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Vol. 17

Volume 17, number 4

October / December 2013

No. 4 Oct./Dec. 2013

International @rchives of Otorhinolaryngology

www.internationalarchivesent.org

Quality, Range, and Legibility in Web Sites Related to Orofacial Functions

Head Position Comparison between Students with Normal Hearing and Students with Sensorineural Hearing Loss

Prevalence of Contralateral Hearing Aid Use in Adults with Cochlear Implants

Allergen-Specific Immunotherapy in Patients 55 Years and Older: Results and Review of Literature

Vector Electronystagmography Analysis in Elderly Individuals with Dizziness Complaint

Characterization of Swallowing Sounds with the Use of Sonar Doppler in Full-Term and Preterm Newborns

Are Histologic Studies of Adenotonsillectomy Really Necessary?

A Descriptive Epidemiology Study of Oral Cleft in Sergipe, Brazil

Tympanometry in Infants: A Study of the Sensitivity and Specificity of 226-Hz and 1,000-Hz Probe Tones

Rehabilitation with Cochlear Implant in Patient with Harboyan Syndrome

Dermoid of the Nasopharynx Causing Neonatal Respiratory Distress

Stage II Chronic Maxillary Atelectasis Associated with Subclinical Visual Field Defect

Human Syngamosis as an Uncommon Cause of Chronic Cough

Adult Rhabdomyoma of the Larynx

Atypical Manifestation of Vestibular Schwannoma

Giant Nasolabial Cyst Treated Using Neumann Incision: Case Report

Cochlear Implants in Children Diagnosed with CHARGE Syndrome


ISSN 1809-9777

International Archives of Otorhinolaryngology Editor

Domingos Hiroshi Tsuji USP, São Paulo, Brazil

Nédio Steffen PUC, Porto Alegre, Brazil

Geraldo Pereira Jotz UFRGS, Porto Alegre, Brazil

Eduardo Crema UFTM, Uberaba, Brazil

Nelson Rosário UFPR, Curitiba, Brazil

Co-Editor

Eliane Schochat USP, São Paulo, Brazil

O. Nuri Özgirgin Başkent University Faculty of Medicine, Ankara, Turkey

Elisabeth Carrara de Angelis Hospital AC Camargo, São Paulo, Brazil

Olivier Sterkers Université Paris Diderot, Paris, France

Fabrizio Ricci Romano USP, São Paulo, Brazil

Onivaldo Cervantes UNIFESP, São Paulo, Brazil

Fayez Bahmad Junior UNB, Brasília, Brazil

Otávio Bejzman Piltcher UFRGS, Porto Alegre, Brazil

Filipe Matuba Agostinho Neto University, Luanda, Angola

Paulo Sérgio Lins Perazzo UNEB, Salvador, Brazil

Fernando Luis Dias INCA, Rio de Janeiro, Brazil

Pedro L. Coser UFSM, Santa Maria, Brazil

Francini Grecco de Melo Pádua UNIFESP, São Paulo, Brazil

Pedro Luís Mangabeira Albernaz UNIFESP, São Paulo, Brazil

Francisco Verissimo de Mello Filho USP-RP, Ribeirão Preto, Brazil

Regina Helena Garcia Martins UNESP, Botucatu, Brazil

Gerson Schulz Maahs UFRGS, Porto Alegre, Brazil

Richard Harvey University of New South Wales, New South Wales, Australia

Aline Gomes Bittencourt USP, São Paulo, Brazil

Associated Editors João Ferreira de Mello Junior USP, São Paulo, Brazil Marcelo M. Hueb UFTM, Uberaba, Brazil Ricardo L. Carrau Ohio State University, OH, USA Luiz Paulo Kowalski H. AC Camargo, São Paulo, Brazil Michiel W. M. Van den Brekel Netherlands Cancer Institute, Amsterdam, Netherlands Marcus Miranda Lessa UFBA, Salvador, Brazil

Giovanni Danesi Ospedali Riuniti di Bergamo, Bergamo, Italy

Robert T. Sataloff Drexel University College of Medicine, Philadelphia, USA

Héctor Rondón Cardoso Universidad Nacional de San Agustín, Arequipa, Perú

Ricardo Ferreira Bento USP, São Paulo, Brazil

Robert Sweet McGill University, Montreal, Canada Robert Vincent Causse Ear Clinic, Colombiers, France

Heinz Stammberger Graz University, Graz, Austria

Roberto Campos Meirelles UERJ, Rio de Janeiro, Brazil

Hélio Lessa UFBA, Salvador, Brazil

Roberto Dihl Angeli UFRGS, Porto Alegre, Brazil

Jacques Magnan Université dAix-Marseille, Marseille, France

Roberto Filipo Sapienza Università di Roma, Roma, Italy

Jair Cortez Mantovani UNESP, Botucatu, Brazil

Rodrigo de Paula Santos UNIFESP, São Paulo, Brazil

Editorial Board

Jeferson S. D’Avila UFSE, Aracajú, Brazil

Ronaldo Frizzarini USP, São Paulo, Brazil

Adriana Brondani da Rocha ULBRA, Canoas, Brazil

Jesús Algaba Guimera Hospital Donostia de San Sebastián, San Sebastián, Spain

Sady Selaimen da Costa UFRGS, Porto Alegre, Brazil

Adriane Teixeira UFRGS, Porto Alegre, Brazil

José Faibes Lubianca Neto UFCSPA, Porto Alegre, Brazil

Salvatore Conticello Università degli Studi di Torino, Turin, Italy

Agrício Crespo UNICAMP, Campinas, Brazil

Jose N. Fayad Keck School of Medicine, USC, California USA

Samir Cahali HSPE, São Paulo, Brazil

Agustin del Canizo Universidad de Salamanca, Salamanca, Spain

Lídio Granato FCMSCSP, São Paulo, Brazil

Shiro Tomita UFRJ, Rio de Janeiro, Brazil

Lilian Muniz Universidade Federal de Recife, Recife, Brazil

Silvia Dornelles UFRGS, Porto Alegre, Brazil

Luiz Antonio Guerra Bernd UFRS, Porto Alegre, Brazil

Silvio Antonio Monteiro Marone PUCCAMP, Campinas, Brazil

Luiz Lavinsky UFRGS, Porto Alegre, Brazil

Silvio da Silva Caldas Neto UFPE, Recife, Brazil

Alexandre Felippu Neto Instituto Felippu, São Paulo, Brazil

Luiz Ubirajara Sennes USP, São Paulo, Brazil

Tania Maria Sih FMUSP, São Paulo, Brazil

Alfio Ferlito Udine School of Medicine, Unide, Italy

Maira Rozenfeld Olchik UFRGS, Porto Alegre, Brazil

Thomas Linder Luzerner Kantonsspital, Luzern, Switzerland

Ana Cristina H. Hoshino USP, São Paulo, Brazil

Manuel Manrique Rodríguez Universidad de Navarra, Navarra, España

Ugo Fisch University Hospital, Zürich, Switzerland

André Luiz Lopes Sampaio UNB, Brasília, Brazil

Marcelo Lazzaron Lamers UFRGS, Porto Alegre, Brazil

Wytske Fokkens Academic Medical Center, Amsterdam, Netherlands

Antonio Celso Nassif Filho PUC, Curitiba, Brazil

Marcelo Ribeiro de Toledo Piza Associação Paparella, Ribeirão Preto, Brazil

Zelita Ferreira Guedes UNIFESP, São Paulo, Brazil

Badr Eldin Mostafa Ain-Shams University, Cairo, Egypt

Márcio Abrahão UNIFESP, São Paulo, Brazil

Bernard Fraysse Hôpital PURPAN, Toulouse, France

Márcio Nakanishi UNB, Brasília, Brazil

Carlos Augusto Pires de Oliveira UNB, Brasília, Brazil

Marcos Vial Goycoolea Clinic of Las Condes, Santiago, Chile

Carlos Curet Universidad Nacional de Córdoba, Córdoba, Argentina

Maria Valéria Schimidt Goffi Gómez USP, São Paulo, Brazil

Carlos Diógenes Pinheiro Neto Albany Medical College, New York, USA

Mario Andréa Lisboa University, Lisboa, Portugal

Ciríaco Cristóvão Tavares Atherino UERJ, Rio de Janeiro, Brazil

Mario Svirsky New York University, New York, USA

Desiderio Passáli University Hospital, Siena, Italy

Maurizio Barbara Sapienza University, SantAndrea Hospital, Rome, Italy

Domenico Cuda Guglielmo da Saliceto Hospital, Piacenza, Italy

Minoru Hirano Kurume University, Kurume, Japan

Priscila Bogar Rapoport FMABC, Santo André, Brazil Marcos Mocelin UFPR, Curitiba, Brazil Richard Voegels USP, São Paulo, Brazil

Alberto Alencar Nuldelmann PUC, Porto Alegre, Brazil Alejandro Rivas Vanderbilt University Medical Center, Tennessee, USA

Librarian Adilson Montefusco

Affiliation

Support


Volume 17, Number 4/2013

International Archives of Otorhinolaryngology Editorial

357

New Challenges for International Archives of Otorhinolaryngology Geraldo Pereira Jotz

Original Articles

358

Quality, Range, and Legibility in Web Sites Related to Orofacial Functions Camila de Castro Corrêa, Deborah Viviane Ferrari, and Giédre Berretin-Felix

363

Head Position Comparison between Students with Normal Hearing and Students with Sensorineural Hearing Loss Renato de Souza Melo, Polyanna Waleska Amorim da Silva, Robson Arruda Souza, Maria Cristina Falcão Raposo, and Karla Mônica Ferraz

370

Prevalence of Contralateral Hearing Aid Use in Adults with Cochlear Implants Cintia Tizue Yamaguchi, Maria Valéria Schmidt Goffi-Gomez

375

Allergen-Specific Immunotherapy in Patients 55 Years and Older: Results and Review of Literature Eduardo Baptistella, Sergio Maniglia, Diego Augusto Malucelli, Daniel Rispoli, Thanara Pruner de Silva, Fernanda Miyoko Tsuru, Renata Vecentin Becker, Gustavo Bernardi, Daniela Dranka, and Bruno Ferraz

380

Vector Electronystagmography Analysis in Elderly Individuals with Dizziness Complaint Lidiane Maria de Brito Macedo Ferreira, Karyna Mirelly O. B. de Figueiredo Ribeiro, André Pestana, Arthur Jorge de Vasconcelos Ribeiro, and Kenio Costa de Lima

383

Characterization of Swallowing Sounds with the Use of Sonar Doppler in FullTerm and Preterm Newborns Hellen Nataly Correia Lagos, Rosane Sampaio Santos, Edna Marcia da Silva Abdulmassih, Liliane Friedrich Gallinea, and Mariangela Langone

387

Are Histologic Studies of Adenotonsillectomy Really Necessary? Giseli Rebechi, Thiago Euênio Pontes, Elias Lobo Braga, Willian Maduel Matos, Fernando Rebechi, and Cícero Matsuyama

Update Article

390

A Descriptive Epidemiology Study of Oral Cleft in Sergipe, Brazil Andrea Luiza, Diego Noronha de Góis, Jadson Alípio Santana de Sousa Santos, Rosany Larissa Brito de Oliveira, and Luiz Carlos Ferreira da Silva

Review Article

395

Tympanometry in Infants: A Study of the Sensitivity and Specificity of 226-Hz and 1,000-Hz Probe Tones Michele Picanço Carmo, Nayara Thais de Oliveira Costa, and Teresa Maria Momensohn-Santos

Thieme Publicações Ltda online

www.thieme-connect.com/ejournals


International Archives of Otorhinolaryngology

Case Reports

403

Volume 17, Number 4/2013

Rehabilitation with Cochlear Implant in Patient with Harboyan Syndrome Lauren Medeiros Paniagua, Maria Elza Kazumi Yamaguti Dorfman, Luiz Lavinsky, and Pricila Sleifer

407

Dermoid of the Nasopharynx Causing Neonatal Respiratory Distress Denise Manica, Cátia Saleh Neto, Cláudia Schweiger, Marcelo Cortina, and Gabriel Kuhl

409

Stage II Chronic Maxillary Atelectasis Associated with Subclinical Visual Field Defect João Mangussi-Gomes, Márcio Nakanishi, Maria Regina Chalita, Fabiana Damasco, and Carlos Augusto Costa Pires De Oliveira

413

Human Syngamosis as an Uncommon Cause of Chronic Cough Janaína Oliveira Bentivi Pulcherio, Eduardo Oliveira Machado da Silva, Daniela Pereira Rezende, Patrícia Bittencourt Barcia Barbeira, Rosane Siciliano Machado, and Marcos Aurélio Baptista de Oliveira

415

Adult Rhabdomyoma of the Larynx Màrcia Monteiro Pinho, Jair de Carvalho e Castro, and Rosana Grandelle Ramos

419

Atypical Manifestation of Vestibular Schwannoma Guilherme Webster, Rui Carlos Ortega Filho, Antonini de Oliveira e Sousa, Márcio Cavalcante Salmito, Mariana Lopes Favero, and Patrícia Maria Sens Marques

421

Giant Nasolabial Cyst Treated Using Neumann Incision: Case Report Alexandre Beraldo Ordones, Larissa Neri, Ingrid Helena Lopes Oliveira, Miguel Soares Tepedino, Fábio de Rezende Pinna, and Richard Louis Voegels

424

Cochlear Implants in Children Diagnosed with CHARGE Syndrome Carolina Costa Cardoso, Michelle Sales de Meneses, Isabella Monteiro de Castro Silva, and Angela Maria Vaccaro Silva Alves

A-1

Instructions to Authors

Copyright © 2013 by Thieme Publicações Ltda Inc. International Archives of Otorhinolaryngology is published four times a year in April, July, and October by Thieme Publicações Ltda, Argentina Building 16th floor, 228 Praia do Botafogo, Rio de Janeiro 22250-040, Brazil. Editorial comments should be sent to journals@thieme.com. Articles may be submitted to this journal on an open-access basis. For further information, please send an e-mail to openaccess@thieme.com. The content of this journal is available online at www.thieme-connect.com/ejournals. Visit our Web site at www. thieme.com and the direct link to this journal at www.thieme.com/iao.

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Some of the product names, patents, and registered designs referred to in this publication are in fact registered trade marks or proprietary names even though specific reference to this fact is not always made in the text. Therefore, the appearance of a name without designation as proprietary is not to be construed as a representation by the Publisher that it is in the public domain. All rights, including the rights of publication, distribution, and sales, as well as the right to translation, are reserved. No part of this work covered by the copyrights hereon may be reproduced or copied in any form or by any means— graphic, electronic, or mechanical, including photocopying, recording, taping, or information and retrieval systems—without written permission of the Publisher. Important Note: Medical knowledge is ever-changing. As new research and clinical experience broaden our knowledge, changes in treatment and drug therapy may be required. The authors and editors of the material herein have consulted sources believed to be reliable in their efforts to provide information that is complete and in accord with the standards accepted at the time of publication. However, in view of the possibility of human error by the authors, editors, or publisher of the work herein, or changes in medical

knowledge, neither the authors, editors, or publisher, nor any other party who has been involved in the preparation of this work, warrants that the information contained here in is in every respect accurate or complete, and they are not responsible for any errors or omissions or for the results obtained from use of such information. Because of rapid advances in the medical sciences, independent verification of diagnoses and drug dosages should be made. Readers are encouraged to confirm the information contained herein with other sources. For example, readers are advised to check the product information sheet included in the package of each drug they plan to administer to be certain that the information contained in this publication is accurate and that changes have not been made in the recommended dose or in the contraindications for administration. This recommendation is of particular importance in connection with new or infrequently used drugs. Although all advertising material is expected to conform to ethical (medical) standards, inclusion in this journal does not constitute a guarantee or endorsement of the quality or value of such product or of claims made by its manufacturer.


Editorial

Editorial

New Challenges for International Archives of Otorhinolaryngology Geraldo Pereira Jotz1 1 Editor-in-Chief, International Archives of Otorhinolaryngology

Int Arch Otorhinolaryngol 2013;17:357.

The International Archives of Otorhinolaryngology is beginning a new chapter in its history; starting with this issue, the journal will be published by Thieme Medical Publishers. Much as impressionism, which emerged in the late 19th century in France, became the starting point for the development of modern art that conveyed the artists’ desire to portray the moment in which a scene took place, the International Archives of Otorhinolaryngology has collaborated with Thieme to better disseminate its content, thereby providing more visibility for its authors. This is an important step in the growth of the journal. The journal will maintain its subsections and will publish supplementary special issues that will accompany the main

journal, the first of which will appear in July 2014. For future issues, we will be changing the system of online submission and review of the papers, which will be managed by ScholarOne (http://mc.manuscriptcentral.com/iaorl). In 2014, we will continue our policy of rewarding articles in the area of meta-analysis that are published in our journal, because these studies form the basis for opinion-making regarding the most varied subjects. In this issue, among the published studies, we highlight the studies related to adenotonsillectomy, rhinoplasty, sensorineural hearing loss, neurotology, allergy, and orofacial issues, seeking challenges, responses, and therapeutic suggestions for several disorders.

Copyright © 2013 by Thieme Publicações DOI http://dx.doi.org/ Ltda, Rio de Janeiro, Brazil 10.1055/s-0033-1354576. ISSN 1809-9777.

357


358

Original Article

Quality, Range, and Legibility in Web Sites Related to Orofacial Functions Camila de Castro Corrêa1

Deborah Viviane Ferrari2

1 Speech-Language Pathologist and Audiologist; Masters Student,

Bauru School of Dentistry, University of São Paulo, Bauru/SP, Brazil 2 Department of Speech-Language Pathology and Audiology, Bauru School of Dentistry, University of São Paulo, Bauru/SP, Brazil

Giédre Berretin-Felix2 Address for correspondence Camila de Castro Corrêa, BS, Octávio Pinheiro Brisola Avenue, 9-75, Bauru/SP 17012-901, Brazil (e-mail: camila.ccorrea@hotmail.com).

Int Arch Otorhinolaryngol 2013;17:358–362.

Abstract

Keywords

► speech ► language and hearing sciences ► breast feeding ► information technologies and communication projects ► education ► distance ► telemedicine ► educational technology

Introduction Plenty of information about health is available on the Internet; however, quality and legibility are not always evaluated. Knowledge regarding orofacial functions can be considered important for the population because it allows proper stimulus, early diagnosis, and prevention of the oral myofunctional alterations during early infancy. Objective The aim was evaluate the quality, legibility, and range of Web sites available in Brazilian Portuguese regarding the orofacial functions. Methods Selected Web sites with information directed to parents/caregivers of babies regarding breast-feeding, feeding after 6 months, deleterious oral habits, and breathing and speech were studied. The Web sites were evaluated through the application of Flesch Reading Ease Test and aspects of the Health on the Net (HON) modified code (HONCode); the range of the subjects addressed was compared with other aspects of infant development. Results From the access of 350 pages of the Internet, 35 Web sites were selected and 315 excluded because they did not meet the inclusion criteria. In relation to legibility, Web sites scored an average of 61.23% in the Flesch Test, and the application of the modified HONCode showed an average of 6.43 points; an average of 2.49 subjects were found per Web site evaluated, with information on breast-feeding being more frequent and subjects such as breathing and speech less frequent. Conclusions Web sites that deal with orofacial functions presented standard legibility classification. Only half of the ethical principles were considered by the modified HONCode in their majority, and most addressed subjects after “breast-feeding” were presented with restricted range.

Introduction The population is researching information about health on the Internet more often. For such, search engines such as Google, Yahoo!, Bing, and Ask.com are frequently used. The importance of these tools in helping the population acquire information about health is highlighted. However, this research must be analyzed, and information that presents indications of scientific evidence should be examined.1

received January 31, 2013 accepted May 3, 2013

The importance of verifying the quality of the information about health on the Internet can be observed in several themes studied, due to their reliability being frequently low.2–4 Concerned about guaranteeing the quality of the information about health offered on the Internet, the Health On the Net Foundation (HON) elaborated the free HONCode certification to advise and to stimulate Internet users to be cautious in their use of medical information obtained on the Internet, according to a set of ethical rules.5 HON is a nonprofit,

Copyright © 2013 by Thieme Publicações DOI http://dx.doi.org/ Ltda, Rio de Janeiro, Brazil 10.1055/s-0033-1351372. ISSN 1809-9777.


Web Sites Related to Orofacial Functions nongovernmental organization, accredited to the Economic and Social Council of the United Nations, that promotes trustworthy online health information and seeks to guide Internet use in an efficient and adequate way.6 Another important characteristic of health Web sites directed to the lay public is the legibility of the available content. The Flesch Reading Ease Index is a formula that evaluates the legibility of a text. Although superficial, it is distinguished as it is the first and only metric of legibility already adapted for Portuguese and able to relate a text with the age group or necessary school level for its reading.7 Legibility and ethical principles are being studied, for example, in Web sites about the Mediterranean diet, indicating the importance of users to seek advice from health professionals due to the poor quality of the information found online.8 Thus, studies evaluating Web sites hosted on the Internet are significant with respect to delineating behaviors of the population, indicating how the people must carry through their searches and what they must do with the collected information. Aiming at evaluating the information available on the Internet specifically regarding speech pathology, studies directed to stuttering and hearing deficiency are presented.9,10 The first study verified that on the five available Web sites, none followed the criteria proposed by the HONCode. The second study, however, through the evaluation of 66 Web sites, pointed out highly changeable quality and legibility and noted that only 14% presented the HONCode certification. In addition, the information on hearing deficiency was, on average, rated at a difficult reading level, requiring high school/college education to be understood. As far as orofacial functions including breathing, sucking, deglutition, chewing, and speech, the availability of information related to the normal development, adequate stimulus, early diagnosis, and prevention of the orofacial myofunction alterations during early infancy is of extreme importance, because it can assist in children’s social and emotional development as well as with their learning.11–16 The objective of this work was to evaluate the quality, the legibility, and the range of Web sites available in Brazilian Portuguese regarding the orofacial functions.

Methods The Google search tool was used with the keywords “site” plus “baby”; the first 10 pages returned were consulted, with

Corrêa et al.

10 Web sites each, in the months of March and April 2012. The first five result pages (50 Web sites) using that tool were also cross-searched for the following words: “breast-feeding” plus “pacifier”; “breast-feeding” plus “baby’s bottle”; “breast-feeding” plus “baby”; “breathing” plus “baby”; “baby” plus “speech.” Thus, 350 pages of the Internet were accessed by a judge, adopting as inclusion criteria Web sites in Brazilian Portuguese that presented information directed to parents/ caregivers of babies about breast-feeding, feeding after 6 months, deleterious oral habits, breathing, and/or speech. Web sites were selected because they contained some subject on the themes, not necessarily contemplating all the topics. To evaluate the legibility level presented by the contents of selected Web sites, Flesch Reading Ease Test was applied, inserting the texts in the Microsoft Office Word tool, where at the end of the orthographic and grammar verification, the program supplies a score. The higher the percentage, the easier to understand the document (►Table 1).7 Moreover, an analysis of the ethical aspects of each Web site was performed, evidencing possible aspects that the objective methods had not identified, using the modified HONCode,6 which is based on the property, intention, qualification of the author, attribution, interactivity, and updates of Web site: • Property: clear indication of property and/or sponsorship of Web site • Intention: identification of the objective of Web site, being educational, commercial, or if this aspect was not clear • Authorship: indication of who supplies the information • Qualification of the author: determination of the professional experience of the author • Attribution: whether it references the basis of the information in regard to references dully cited • Interactivity: availability of an email address or other forms of interaction between the owner and the user • Updates: the frequency or the date of the last update is available Each one of these characteristics was subdivided into items that received a score that varied between 0, 1, or 2 (depending on the evaluated aspect). The higher the score obtained (total of 13 points), the better the quality of the Web site.

Table 1 Levels of legibility of a text, relating to the level of education necessary for its reading Flesch

Classification

Schooling level

100–90

Very easy

Elementary education (1st–4th grade/1st–5th year)

90–80

Easy

80–70

Reasonably easy

70–60

Standard

60–50

Reasonably difficult

50–30

Difficult

High school and college level

30–0

Very difficult

Academic texts—presence of technical terms

Elementary education (5th–8th grade/6th–9th year)

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The range of the subjects on baby orofacial functions was evaluated, verifying if the Web site brought information on aspects of breast-feeding, feeding after 6 months, deleterious oral habits, breathing, and speech, regardless if the information was complete or recent. Finally, it was verified if the contents of the orofacial functions were related to oral health care, highlighting the holistic view of the patient and interdisciplinary approach.

Data Analysis The results were analyzed descriptively, from the study and evaluation of each Web site. The Web site’s particular score was considered as well as the average related to each aspect evaluated, grouping the Web sites evaluated.

Results In view of the access of 350 pages of the Internet, 35 Web sites were selected from the inclusion criteria and evaluated in

regard to range, legibility, and quality. The Internet pages excluded from the study included 61% that were advertisement, 17% that did not present topics about the theme in question, and 12% that were not Web sites (blogs and links to articles). Each one of the 35 Web sites was submitted to the Flesch Ease Reading Test, and the sites obtained the average of 61.23% on the Flesch Index (standard deviation: 8.71). In ►Fig. 1, the index of each Web site evaluated is shown. Considering orthographic verification, 17 (48.57%) Web sites presented orthographic errors. In the modified HONCode the average score obtained was 6.43 points (standard deviation: 2.71). ►Fig. 2 shows scores of each Web site evaluated based on the seven aspects of the modified HONCode. The range of the Web sites follows the themes studied, and a larger amount of information on breast-feeding was found and a smaller amount in subjects such as breathing and speech (►Table 2).

Fig. 1 Flesch Index, in percentage, of each Web site about orofacial functions.

Fig. 2 Score of the modified Health on the Net (HON) code of each Web site about orofacial functions. International Archives of Otorhinolaryngology

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Web Sites Related to Orofacial Functions

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Table 2 Range of Web sites on breast-feeding, feeding after 6 mo, deleterious oral habits, breathing, and speech Themes

Web sites (total ¼ 35)

Percentage

Breast-feeding

34

97.14

Feeding after 6 mo

23

65.71

Deleterious oral habits

19

54.29

Breathing

8

22.86

Speech

4

11.43

Considering how many subjects each Web site presented, among “breast-feeding,” “feeding after 6 months,” “deleterious oral habits,” “breathing,” and “speech,” an average of 2.49 subjects (49.8%) per Web site was presented, with standard deviation of 1.17. The oral health aspects score was observed in 21 Web sites (60%), relating them with the orofacial functions and baby development. In the qualitative analysis of the Web sites, technical terms were not explained through writing or illustrations in 26 (74.29%) of them, along with extensive presentation of the content, formatting making accessibility difficult, forums not mediated by professionals, copy of materials without the citation of original references, information without scientific evidence, and indication of behaviors without highlighting the importance of the medical opinion.

Discussion The evaluation process of the legibility and quality of the information about orofacial functions available on the Internet must be carried through systematically and frequently, due to the fact that many Web sites propose to approach this theme but do not always use language that is accessible to the majority of the population and do not take the ethical principles into consideration. In regard to the Flesch score, the average score of the Web sites found was 61.23%, classifying them as appropriate material considering their legibility. However, the limitations of the Flesch Test must be highlighted; it is an index that evaluates the intelligibility of a text superficially, considering the number of words in sentences and the number of syllables per word but does not allow the verification of the complexity of ideas or their logical order.7 This justifies the contradiction of attaining a high average of the Flesch score while simultaneous having observable grammatical errors, which is ignored by the test, giving an overall high score. Despite approaching different themes, the findings of this study meet the pooling carried through regarding “diabetes” where 51.2% of 41 publications had Flesch scores between 50 and 75, which classifies them as easy reading.17 In regard to the evaluation of educational brochures for parents about the newborn screening, 92% of the materials had low Flesch scores, in addition to having high frequency of outdated contents, being aesthetically unpleasing, and not clearly indicating the most appropriate behavior.18

The average regarding the score of the modified HONCode illustrates that Web sites fulfill only half of the ethical principles proposed. This finding agrees with the literature in several themes, which affirm the low indexes of this certification.2–4,19 In the field of the orofacial motricity specific studies were not found. However, it is observed in dentistry that only 5.3% of the Web sites on dental cavities in children follow the principles of the HON.20 Observing that on average 49.8% of the topics included on the Web sites were investigated, a broader range of orofacial functions can be verified when compared with other themes, which brings rates of 33.75% regarding asthma topics and 8.3% about Chagas disease topics.3,21 However, it is worth noting that the other researchers used more detailed forms, grouping several topics into the different blocks of information checked, which contributes to a smaller percentage of Web sites that consider all required topics in health. Tests and criteria used in this study were important in the attempt to quantify the range and legibility of the Web sites found about orofacial functions. However, in the qualitative analysis, modified aspects were verified that are basic to guarantee the transmission of knowledge and that the objective instruments did not identify, such as exposition of information without scientific evidence or difficult accessibility and usability. Web sites about allergic rhinitis, tegumentary and visceral leishmaniasis, Chagas disease, and obesity also had information that was incomplete, incorrect, and without scientific basis, which brings into doubt the credibility of the content displayed for this technology of information and communication, in addition to possible conflicts of information obtained in medical consultations.22 This way, Web sites that seem trustworthy in certain evaluations can present relevant alterations, such as not supplying higher levels of accurate health content, that can interfere with the informative potential of the Web site.23 As a first review studying existing Web sites on orofacial functions, this study stresses the need to continue guiding the public to Web addresses that can be explored and even the creation of tools that are more appropriate for the acquisition of knowledge. Thus, studies converging on the improvement of information about health available on the Internet can contribute to the reduction of the damages in health of the population,21 with implications favorable to the promotion of health to those who search information on the Web and for other individuals of the community. International Archives of Otorhinolaryngology

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Corrêa et al.

Conclusion

11 Araújo CMT. Uso de chupeta: repercussões no aleitamento ma-

The legibility of Web sites that approach orofacial functions was classified on average as standard, being that they fulfilled half of the ethical principles considered by the modified HONCode. Moreover, the most discussed subjects were “breast-feeding” and “feeding after 6 months,” with restricted range for not relating to the other aspects of the baby’s development of the orofacial functions.

12

13

14

References 1 Wang L, Wang J, Wang M, Li Y, Liang Y, Xu D. Using Internet search

2 3

4 5

6 7

8

9 10

engines to obtain medical information: a comparative study. J Med Internet Res 2012;14:e74 Chang MY, Han DH, Moon IJ, et al. Assessment of allergic rhinitis websites in Korea. Clin Exp Otorhinolaryngol 2010;3:32–36 Park H-W, Min K-U, Kim Y-Y, Cho S-H. Assessing the quality and contents of asthma-related information on the Korean Internet as an educational material for patients. J Korean Med Sci 2004;19: 364–368 Soobrah R, Clark SK. Your patient information website: how good is it? Colorectal Dis 2012;14:e90–e94 Pacios M, Campos CJR, Martha AS, Barra PSC. Os sites de medicina e saúde frente aos princípios éticos da Health on the Net Foundation —HON. Rev Bioética 2010;18(2):483–496 Health on the Net Foundation. Available at: http://www.hon.ch/. Accessed August 4, 2012 Biondo-Simões MLP, Martynetz J, Ueda FMK, Olandoski M. Compreensão do Termo de Consentimento. Rev Col Bras Cir 2007;43 (3):183–188 Hirasawa R, Saito K, Yachi Y, et al. Quality of Internet information related to the Mediterranean diet. Public Health Nutr 2012; 15:885–893 Silveira PCM, Costa AES, Lima CC. Gagueira na web: qualidade da informação. Rev CEFAC [online] 2012;14(3):430–437 Laplante-Lévesque A, Brännström KJ, Andersson G, Lunner T. Quality and readability of English-language internet information for adults with hearing impairment and their significant others. Int J Audiol 2012;51:618–626

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16

17 18

19

20

21

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terno e no desenvolvimento sensório-motor oral. Distúrb Comun 2007;19(2):478–479 Bezerra PKM, Cavalcanti AL, Bezerra PM, Moura C. Maloclusões, tipos de aleitamento e hábitos bucais deletérios em pré-escolares: um estudo de associação. Pesqui Bras Odontopediatria Clín Integr 2005;5(3):267–274 García García VJ, Ustrell Torrent JM, Sentís Vilalta J. Evaluación de la maloclusión, alteraciones funcionales y hábitos orales en una población escolar: Tarragona y Barcelona. Av Odontoestomatol [online] 2011;27(2):75–84 Gimenez CMM, Moraes ABA, Bertoz AP, Bertoz FA, Ambrosano GB. Prevalência de más oclusões na primeira infância e sua relação com as formas de aleitamento e hábitos infantis. Rev Dent Press Ortodon Ortop Facial [online] 2008;13(2):70–83 Maciel CTV, Leite ICG. Aspectos etiológicos da mordida aberta anterior e suas implicações nas funções orofaciais. Pró-Fono R Atual Cient [online] 2005;17(3):293–302 Cepero Sanchez ZJ, Hidalgo-Gato Fuentes I, Duque de Estrada Riveron J, Perez Quiñones JA. Intervención educativa en escolares de 5 y 6 años con hábitos bucales deformantes. Rev Cubana Estomatol [online] 2007;44(4) Moreira MF, Silva MIT. Readability of the educational material written for diabetic patients. Online Braz J Nurs 2005;4(2) Arnold CL, Davis TC, Frempong JO, et al. Assessment of newborn screening parent education materials. Pediatrics 2006;117(5 Pt 2): S320–S325 Barbosa AL, Martins EN. Evaluation of Internet websites about floaters and light flashes in patient education. Arq Bras Oftalmol 2007;70:839–843 Leite F, Correia A. Quality evaluation of websites with information on childhood dental caries in Portuguese language. Rev Odonto Ciênc [online] 2011;26(2):116–120 Malafaia G. Análise de informações sobre a doença de Chagas disponíveis em websites brasileiros na rede mundial de computadores (internet). Arq Bras Ciên Saúde 2009;34(3):188–195 Malafaia G, Castro ALS, Rodrigues ASL. A qualidade das informações sobre doenças disponíveis em websites brasileiros: uma revisão. Arq Bras Ciênc Saúde 2011;36(2):72–78 Kunst H, Groot D, Latthe PM, Latthe M, Khan KS. Accuracy of information on apparently credible websites: survey of five common health topics. BMJ 2002;324:581–582


Original Article

Head Position Comparison between Students with Normal Hearing and Students with Sensorineural Hearing Loss Renato de Souza Melo1 Polyanna Waleska Amorim da Silva2 Maria Cristina Falcão Raposo4 Karla Mônica Ferraz5 1 Universidade Federal de Pernambuco (UFPE), Recife/PE, Brasil 2 Faculdade Integrada do Recife (FIR), Recife/PE, Brasil 3 Associação Caruaruense de Ensino Superior (ASCES), Caruaru/PE,

Robson Arruda Souza3

Address for correspondence Renato de Souza Melo, MD, Rua Avelino Cândido, 32, Salgado, Caruaru/PE, CEP 55018–070, Brazil (e-mail: renatomelo10@hotmail.com).

Brasil 4 Department of Statistics, Universidade Federal de Pernambuco

(UFPE), Recife/PE, Brasil 5 Department of Physical Therapy, Universidade Federal de

Pernambuco (UFPE), Recife/PE, Brasil Int Arch Otorhinolaryngol 2013;17:363–369.

Abstract

Keywords

► ► ► ► ► ►

vestibular diseases deafness posture postural balance ear inner

Introduction Head sense position is coordinated by sensory activity of the vestibular system, located in the inner ear. Children with sensorineural hearing loss may show changes in the vestibular system as a result of injury to the inner ear, which can alter the sense of head position in this population. Aim Analyze the head alignment in students with normal hearing and students with sensorineural hearing loss and compare the data between groups. Methods This prospective cross-sectional study examined the head alignment of 96 students, 48 with normal hearing and 48 with sensorineural hearing loss, aged between 7 and 18 years. The analysis of head alignment occurred through postural assessment performed according to the criteria proposed by Kendall et al. For data analysis we used the chi-square test or Fisher exact test. Results The students with hearing loss had a higher occurrence of changes in the alignment of the head than normally hearing students (p < 0.001). Forward head posture was the type of postural change observed most, occurring in greater proportion in children with hearing loss (p < 0.001), followed by the side slope head posture (p < 0.001). Conclusion Children with sensorineural hearing loss showed more changes in the head posture compared with children with normal hearing.

Introduction Posture can be defined as the position or attitude of the body available in static or harmonic arrangement of body parts in dynamic situations.1 Good posture is a result of the ability of the bones, ligaments, capsules, and muscle to bear the body upright, allowing a person to stay in one position for

received March 3, 2013 accepted May 19, 2013

prolonged periods without discomfort and with low power consumption.2 In the standard posture, the spine has normal curvatures, and the bones of the lower limbs are in perfect alignment for support of body weight. The neutral position of the pelvis leads to the proper alignment of the abdomen, trunk and lower limbs, thorax, and upper spine so that the function of

Copyright © 2013 by Thieme Publicações DOI http://dx.doi.org/ Ltda, Rio de Janeiro, Brazil 10.1055/s-0033-1351685. ISSN 1809-9777.

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364

Head Positions of Students with Sensorineural Hearing Loss the respiratory muscles is favored. The head is upright, balanced, and aligned to minimize burden on the neck muscles that are responsible for their support and position.2 The sense of head position and hence the alignment expressed by body posture are influenced by information from the proprioceptive receptors located in the neck and the sensory information coming from the vestibular system.3 Situated in the inner ear, the vestibular-cochlear system features dual function: the cochlea is responsible for hearing and the vestibule for the regulation of balance and postural control.4 Due to the proximity of the anatomical structures responsible for auditory and vestibular function, it is common to find associated changes in both systems in the case of involvement of the inner ear5; thus it is believed that many children with hearing loss have vestibular problems concomitant with sensorineural hearing loss.6 In this sense, studies have shown that the hypoactivity of the vestibular system is a common finding during otoneurologic assessment in children with sensorineural hearing loss,7–9 altering the sensory information from the vestibular system. If sensory information coming from the vestibular system is inaccurate or there is a failure in integrating it with the central nervous system, changes in head position can occur, resulting in a distorted reference of sense of the position of the head, neck, and possibly body posture.3 Given the above, children with sensorineural hearing loss appear have vestibular deficits concomitant with sensorineural hearing loss as a result of injury to the inner ear,10 and this can change the sense of the position of the head of the children, making them more susceptible to the changes in head posture compared with normally hearing students, justifying the completion of this study, which analyzed the alignment of the head of normally hearing students with sensorineural hearing loss and compared the distribution of changes in head posture between groups, considering genders and age groups in the sample and also the degree of hearing loss in the group of children with hearing loss.

Methods This is a cross-sectional study, which was conducted between August and December 2012. To delimit the sample size of this study, a survey was conducted with the manager of the Center for Rehabilitation and Special Education Rotary to identify the number of children with sensorineural hearing loss enrolled of ages desired by the study and those who fit the criteria for inclusion and exclusion. This school aims to teach children and teens with special needs. Thus, it was possible to pair 48 students regarding gender and age, because there was a predominance of one gender and some age groups, hampering the expansion of sample of this study. The same amount of students was set to form the normally hearing group and the pairing between the two groups. Thus, 96 students, aged between 7 and 18 years, participated in this study, 48 with normal hearing and 48 with sensorineural hearing loss. The students were recruited by a convenience sample, matched for gender and age, and selected by a draw conInternational Archives of Otorhinolaryngology

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Melo et al. ducted by their teachers, who were not aware of the characteristics of this study. The draws were held in their classroom, in front of all students and of researchers of this study. The normally hearing students were recruited from the School Duque de Caxias, geared to the public with normal hearing, and students with hearing loss were recruited from the Center for Rehabilitation and Special Education Rotary. Both are state schools, have similar profiles, and are located in the municipality of Caruaru/PE. The volunteers were divided into two groups: a normally hearing group and a sensorineural hearing loss group. The normally hearing group was composed of 48 students, 24 males and 24 females, with two representatives of both genders by age. Exclusion criteria for this group included the presence of any disability (neurological, hearing, visual, mental), history of trauma in the head and neck, and lower limb discrepancy greater than 2 inches, obtained through measurement test real and apparent extent of the lower limbs, previously performed by evaluators. The sensorineural hearing loss group, formed by 48 students, 24 males and 24 females, also contained two representatives of both genders by age. Exclusion criteria for this group were presence of any other associated disabilities, history of trauma in the head and neck, and lower limb discrepancy greater than 2 inches, obtained through tests measuring real and apparent length of the lower limbs, previously performed by the evaluators. Inclusion criteria for both groups included regular enrollment in one of the schools collaborating with research, within the age group intended for the study, and submission of the term of consent duly signed by the parent or responsible of the volunteer, and exclusively for those with hearing loss, fluency in the Brazilian Sign Language (LIBRAS), submission of medical report of clinical diagnosis of sensorineural hearing loss, and audiometry performed in the last 6 months. There was also a division between the groups of children with hearing loss, according to the degrees of hearing loss presented. The students were divided into two subgroups, one group of mild to moderate loss and the other, severe to profound loss. Students were stratified in this way due to many reports of audiometry already experienced within this diagnosis. It is worth noting that some students had different degrees of hearing loss from one ear to another; however, the differences always were mild to moderate or severe to profound. For data confirmation of the inclusion and exclusion criteria of this study, we considered the information reported by parents of students during the interview with the researchers, together with data obtained in the student’s transcript. The procedures before testing and evaluation of head alignment were previously explained by researchers to normally hearing volunteers orally and volunteers with hearing loss through the LIBRAS by one of the researchers who interpreted LIBRAS. For acquisition of the data posture, individually, each student was assessed on a flat surface, barefoot in a bathing


Head Positions of Students with Sensorineural Hearing Loss suit (shorts for boys and bikinis and hair piled high on the head for girls), at a distance of 20 cm from a wall. As postural reference, we used the standard anatomical points proposed by Kendall et al,11 visualizing the volunteers in the following planes: coronal plane, anterior and posterior and sagittal plane, left and right. These criteria allow assessment of the posture and identification of deviations in head posture, such as the forward, protrusion, tilted, and rotated side of head positions. The anatomical landmarks referenced and demarcated in this study were: glabella, gulp, mental protuberance, acromion, manubrium sternum, spinous process of C7 spinous process, and T3. The postural assessment data were recorded on a standardized form containing the student ID, birth date, grade, school, and changes in head posture observed in each of the planes examined at the same moment by two physiotherapists, as suggested by Polly et al.12 After evaluating posture in the sensorineural hearing loss group, we collected data about the degree of hearing loss by audiometry brought by parents or guardians or in the student’s records. The data are expressed as percentage frequency and were analyzed using the chi-square test and Fisher exact test. The level of significance was set at p < 0.05. For data analysis, we used the Software Statistical Package for Social Sciences (SPSS) version 18.0. This study was approved by the Ethics Committee at the Hospital of the Restoration (CEP/HR) as the protocol number 1793.0.000.102–11, according to Resolution 196/96 of the National Health Council.

Results In the present study, we evaluated head posture of 48 students with normal hearing and 48 students with sensorineural hearing loss; students had a mean age of 12.5  3.5 years in each group, as shown in ►Table 1.

Melo et al.

The results showed a higher occurrence of changes in the head posture in the group of students with hearing loss compared with normally hearing students (normally hearing 20.8%, hearing loss 75%, p < 0.001). According to the analysis of prevalence ratio, the occurrence of changes in head posture in children with hearing loss can be 3.26 times higher than in normally hearing students (confidence interval ¼ 1.95; 5.46), as shown in ►Table 2. Regarding the type of postural change, the forward position of the head was the most common abnormality in both groups, occurring in greater proportion in the students with hearing loss (normal hearing 14.6%, hearing loss 52.1%, p < 0.001), which indicated a prevalence ratio of 2.17 times greater probability of presenting in students with hearing loss compared with normally hearing students in this sample (confidence interval ¼ 1.49; 3.16), shown in ►Table 2. The inclination lateral head was the second postural change most frequently observed in both groups, affecting more students with hearing loss (normal hearing 6.25%, hearing loss 50%, p < 0.001). The likelihood of developing this change was 2.56 times higher in students with hearing loss compared with students with normal hearing (confidence interval ¼ 1.80; 3.62), as shown in ►Table 2. Moreover, the rotated laterally with protruding head posture, although most often observed in the group of children with hearing loss, showed no association with hearing loss, as shown in ►Table 2. According to the genres and changes in head posture, the results showed differences between groups (female: 20.8% normal hearing, hearing loss 79.2%, p < 0.001; male: normal hearing 20.8%, hearing loss 71.4%, p ¼ 0.001). The same occurred with the forward head posture (female: normal hearing 12.6%, hearing loss 54.6%, p ¼ 0.002; male: normal hearing 16.7%, hearing loss 50%, p ¼ 0.014). The same results were found for lateral inclination of head (female: normal hearing 8.3%, hearing loss 41.7%, p ¼ 0.008; male: normal hearing 4.2%, hearing loss 58.3%, p < 0.001; ►Table 3).

Table 1 Characterization of the sample Hearing

p

Hearing loss

n

%

n

%

48

100

48

100

Female

24

50

24

50

Male

24

50

24

50

12.5  3.5

100

12.5  3.5

100

Right-handed

45

93.7

41

85.4

0.181a

Left-handed

03

6.3

07

14.6

Mild to moderate

4

8.3

Severe to profound

44

91.7

Volunteers Gender

Age (mean) Lateral dominance

Degrees of hearing loss

a

Pearson chi-square test. International Archives of Otorhinolaryngology

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Head Positions of Students with Sensorineural Hearing Loss

Melo et al.

Table 2 Occurrence of changes in head position in students with normal hearing and students with sensorineural hearing loss in the sample (n ¼ 96) p

PR

CI

75

<0.001a

3.26

1.95; 5.46

52.1

<0.001a

2.17

1.49; 3.16

50

<0.001

Normal hearing (n ¼ 48)

Hearing loss (n ¼ 48)

n

%

n

%

Head position changes

10

20.8

36

Forward head

7

14.6

25

Lateral inclination head

3

Lateral rotation head

6.25

1

Protruding head

24

2.08

0

6

0

12.5

4

8.3

a

2.56

1.80; 3.62

0.111

b

0.117

b

Abbreviations: CI, confidence interval; PR, prevalence ratio. a Chi-square test. b Fisher exact test.

Table 3 Occurrence of changes in head position in students with normal hearing and students with sensorineural hearing loss, according to the gender of the sample (n ¼ 96) Female (n ¼ 48)

a

Male (n ¼ 48)

Normal hearing (n ¼ 24)

Hearing loss (n ¼ 24)

n

n

%

p

%

p

Normal hearing (n ¼ 24)

Hearing loss (n ¼ 24)

n

%

n

%

5

20.8

17

71.4

0.001a

Head position changes

5

20.8

19

79.2

<0.001

Forward head

3

12.6

13

54.6

0.002a

4

16.7

12

50

0.014a

b

1

4.2

14

58.3

<0.001b

a

Lateral inclination head

2

8.3

10

41.7

0.008

Lateral rotation head

1

4.2

4

16.7

0.348b

0

0

2

8.4

0.489b

Protruding head

0

0

1

4.2

1.000b

0

0

3

12.6

0.234b

Chi-square test. Fisher exact test.

b

Postures with lateral rotation and protruding head showed no relationship with the genders, as seen in ►Table 3. Considering the age and changes in head posture between the groups, the results showed differences only in the age group 7 to 14 years. The changes in head posture showed differences between the age groups 7 to 10 years (normal hearing 6.3%, hearing loss 87.5%, p < 0.001) and 11 to 14 years (normal hearing 25%, hearing loss 93.8%, p < 0.001), as seen in ►Table 4. Similarly, the forward head posture pointed to differences between the age group 7 to 10 years (normal hearing 0%, hearing loss 50%, p ¼ 0.002), with the same result in the group aged 11 to 14 years (normal hearing 18.8%, hearing loss 75%, p ¼ 0.001), as shown in ►Table 4. The lateral inclination posture of the head showed differences between the age groups 7 to 14 years (normal hearing 0%, hearing loss 68.8%, p < 0.001) and 11 to 14 years (normal hearing 12.5%, hearing loss 56.3%, p ¼ 0.009). There was no relation between the position with head rotation and lateral protrusion of the head, as illustrated in ►Table 4. When we considered the changes in the posture in the group of children with hearing loss and the degree of hearing International Archives of Otorhinolaryngology

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loss presented, we observed significant differences between the subgroups (mild/moderate 25%, profound/severe 79.5%, p ¼ 0.043). The results showed no differences between the specific change in head posture and the hearing loss presented, according to ►Table 5.

Discussion This study showed differences between the alignment of the head of students evaluated, and those students with hearing loss showed higher occurrence of changes in head posture compared with normally hearing students. According to the review conducted by the authors, there are no studies evaluating the alignment of the head of students with sensorineural hearing loss and normally hearing students, and this fact hindered the expansion of the discussion of this study. However, research data reinforce the results found in this study. Recent studies have evaluated the postural control of normally hearing children and children with hearing loss and reported that children with hearing loss showed significantly more instability in postural control than


Head Positions of Students with Sensorineural Hearing Loss

Melo et al.

Table 4 Occurrence of changes in head position in students with normal hearing and students with sensorineural hearing loss, according to age group in the sample (n ¼ 96) 7–10 y old (n ¼ 32) Normal hearing (n ¼ 16)

p

Normal hearing (n ¼ 16)

Hearing loss (n ¼ 16)

p

Normal hearing (n ¼ 16)

Hearing loss (n ¼ 16)

p

n

%

n

%

n

%

n

%

n

%

n

%

Head position changes

1

6.3

14

87.5

<0.001a

4

25

15

93.8

<0.001a

5

31.3

7

43.8

0.718a

Forward head

0

0

8

50

0.002b

3

18.8

12

75

0.001a

4

25

7

31.3

1.000b

a

1

6.3

4

25

0.333b

Lateral inclination head

0

0

11

68.8

<0.001

Lateral rotation head

1

6.3

2

12.5

1.000b

Protruding head a

Hearing loss (n ¼ 16)

15–18 y old (n ¼ 32)

11–14 y old (n ¼ 32)

0

0

0

0

a

2

12.5

9

56.3

0.009

0

0

3

18.8

0.226b

0

0

1

6.3

1.000b

18.8

b

0

0

1

6.3

1.000b

0

0

3

0.226

Chi-square test. Fisher exact test.

b

Table 5 Occurrence of changes in head position in children with sensorineural hearing loss, according to the degrees of hearing loss (n ¼ 48) Degree of hearing loss (n ¼ 48) Mild/moderate (n ¼ 4)

a

p

Severe/profound (n ¼ 44)

n

%

n

%

Head position changes

1

25

35

79.5

0.043a

Forward head

1

25

24

54.5

0.338a

Lateral inclination head

0

0

24

54.5

0.109a

Lateral rotation head

1

25

3

6.8

0.425a

Protruding head

0

0

4

8.3

1.000a

Fisher exact test.

normally hearing children; these instabilities could cause changes in the body posture of these children.13–16 In this context, Melo et al, who evaluated the spinal posture of 88 students (44 deaf and 44 normally hearing students, aged 7 to 17 years), reported that the deaf students showed significantly more postural changes in the spine when compared with normally hearing students, as observed in this study.17 Similar to this study, Coelho-Junior et al evaluated and compared the alignment of the head of 60 subjects, 30 with normal vestibular function and 30 with unilateral vestibular hypoactivity.18 The results showed significant differences in the degrees of forward and inclination lateral head position, which were higher in patients with underactive vestibular function compared with subjects with normal vestibular function. This finding corroborates the results of this study, despite not having performed the vestibular assessment in this sample (this is a limitation of the present study, because it is an examination of relatively high cost for the study population, hardly offered public hospitals, and this research did not have sufficient funding for it to be included). However,

studies have shown that children with sensorineural hearing loss often have vestibular disorders,19,20 which could cause changes in head posture in school children such as those found in this study. The forward head posture was the most frequent postural change among schoolchildren, affecting more students with hearing loss. These data agree with Coelho-Junior et al,18 who reported that the angle of forward head posture demonstrated a higher difference between groups, affecting more students in the group with vestibular dysfunction. Sousa et al reported that the instabilities in postural control in their study occurred more commonly in the anteroposterior direction, the same direction as the forward head posture, which may explain our findings.13 The inclination head posture was the second most observed postural change in impaired schoolchildren in this study, occurring in greater proportion in the group with hearing loss. In this context, Prencht reported that individuals with hearing loss and vestibular dysfunction showed lateral inclination posture of the head, to the side of vestibular dysfunction.21 Ganança et al evaluated the posture of 82 International Archives of Otorhinolaryngology

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Head Positions of Students with Sensorineural Hearing Loss subjects with vestibular dysfunction and concluded that it is possible to identify postural patterns typical in these individuals.22 Among these patterns, the most noted was the inclination of the trunk and head to the same side, corroborating the results of this study. Derlich et al reported that in their study, the instabilities in postural control of children with hearing loss occurred more commonly in the medial-lateral direction, the same direction as the lateral head posture, which could trigger such changes in head posture observed in the present study.14 Besides the data between the groups in isolation, some variables were also analyzed in this study; however, one of the most important findings was observed in the age variable. The upper age range of this study was to analyze head posture with increasing age in this population. The results demonstrated that changes in head posture occur in greater proportion in childhood and adolescence until 14 years old. Kavalco believes that these are the most critical periods for the observation of postural changes in students.23 Another important finding was observed in the subgroups of students with variable degrees of hearing loss. The students with severe to profound hearing loss had significantly increased incidence of abnormal head posture in general compared with students with mild to moderate hearing loss (►Table 5). One explanation for this finding may be found in the study of Guilder and Hopkins, who evaluated the function of the vestibular system in children with hearing loss and reported that children with severe to profound hearing loss often had hypoactivity of the vestibular system.24 Similarly, Lavinsky stated that children with profound hearing loss showed a high incidence of vestibular dysfunction in their study.25 These data could explain the findings of this study, given that 91.5% of the children with hearing loss evaluated in this study had severe to profound loss and only 8.5% had mild to moderate loss. According to these data, we found that children and adolescents with sensorineural hearing loss are likely to develop changes in head posture. It is believed that one of the reasons for this may be the stiffness of the neck, which might occur as an attempt to stabilize the head in a determined position to avoid vertigo and/or dizziness.26,27 This event could be associated with postural fixation of the cervical region, leading to muscle tension, pain, limitation in the amplitude joint, muscle shortening, not only in the head but reverberating throughout the body,28 which would justify the findings of this study. Satisfactory postural control is critical to the success of most daily tasks. The knowledge of neurologic and biomechanical aspects of this control has direct implications for physical therapy,29 justifying the importance of future research to address this issue. These data underscore the need to develop preventive and therapeutic measures in school-aged children, preventing the emergence of such changes, restoring postural pattern in students who already have asymmetries, and providing a postural pattern suitable for children with hearing loss. International Archives of Otorhinolaryngology

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Melo et al. The physical therapist can intervene in these disorders in childhood, with the availability of services in schools and institutions that serve this population and in multidisciplinary teams, which reinforces the importance of the presence physical therapists in the school environment.30

Conclusions The students with sensorineural hearing loss had a higher occurrence of changes in head posture in general, compared with normally hearing students. This fact seems to affect more children and adolescents aged 7 to 14 years, of both genders and those with severe to profound hearing loss.

Acknowledgments We thank the manager of the Gerência Regional de Educação do Agreste Centro-Norte—Caruaru, Antonio Fernando Santos Silva, for the consent to conduct the research, and the director’s and the teachers of the Centre for Rehabilitation and Special Education Rotary and of School Duque de Caxias for the space given researchers to collect data. We especially thank the parents who agreed to the participation of their children in this study and students who participated.

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tron. 5th ed. São Paulo, Barueri, Brazil: Manole; 1997 2 Kendall FP, McCreary EK, Provance PG. Músculos: provas e funções.

4th ed. São Paulo, Brazil: Manole; 1995 3 Armstrong B, McNair P, Taylor D. Head and neck position sense.

Sports Med 2008;38:101–117 4 Mangabeira Albernaz PL, Ganança MM, Caovilla HH, Ito YI, Novo

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NF, Juliano Y. Aspectos clínicos e terapêuticos das vertigens. Acta AWHO 1986;5(2 suppl):49–109 Caovilla HH, Ganança MM, Munhoz MSL, Silva MLG. Equilibriometria clínica. 1st ed. São Paulo, Brazil: Atheneu; 2000 Azevedo MG, Samelli AG. Estudo comparativo do equilíbrio de crianças surdas e ouvintes. Rev CEFAC 2009;11:85–91 Kaga K, Shinjo Y, Jin Y, Takegoshi H. Vestibular failure in children with congenital deafness. Int J Audiol 2008;47:590–599 Lisboa TR, Jurkiewicz AL, Zeigelboim BS, Martins-Bassetto J, Klagenberg KF. Vestibular findings in children with hearing loss. Int Arch Otorhinolaryngol 2005;9:271–279 Cushing SL, Papsin BC, Rutka JA, James AL, Gordon KA. Evidence of vestibular and balance dysfunction in children with profound sensorineural hearing loss using cochlear implants. Laryngoscope 2008;118:1814–1823 Melo RS, Silva PWA, Silva LVC, Toscano CFS. Postural evaluation of vertebral column in children and teenagers with hearing loss. Int Arch Otorhinolaryngol 2011;15:195–202 Kendall FP, McCreary EK, Provance PG. Músculos: provas e funções. 5th ed. São Paulo, Brazil: Manole; 2007 Polly DW Jr, Kilkelly FX, McHale KA, Asplund LM, Mulligan M, Chang AS. Measurement of lumbar lordosis. Evaluation of intraobserver, interobserver, and technique variability. Spine 1996;21:1530– 1535, discussion 1535–1536 de Sousa AM, de França Barros J, de Sousa Neto BM. Postural control in children with typical development and children with profound hearing loss. Int J Gen Med 2012;5:433–439


Head Positions of Students with Sensorineural Hearing Loss

Melo et al.

14 Derlich M, Kręcisz K, Kuczyński M. Attention demand and postural

22 Ganança FF, Bushatsky A, Perracini MR, Silva D, Serafini F. Posture

control in children with hearing deficit. Res Dev Disabil 2011;32: 1808–1813 Sousa AMM, Barros JF, Sousa Neto BM, Gorla JI. Avaliação do controle postural e do equilíbrio em crianças com deficiência auditiva. R da Educação Física/UEM 2010;21:47–57 De Kegel A, Dhooge I, Cambier D, Baetens T, Palmans T, Van Waelvelde H. Test-retest reliability of the assessment of postural stability in typically developing children and in hearing impaired children. Gait Posture 2011;33:679–685 Melo RS, Silva PWA, Macky CFST, Silva LVC. Análise postural da coluna vertebral: estudo comparativo entre surdos e ouvintes em idade escolar. Fisioter Mov 2012;25(4):803–810 Coelho-Junior NA, Gazolla JM, Gabilan YPL, Mazzetti KR, Perracini MR, Ganança FF. Alinhamento da cabeça e ombros em pacientes com hipofunção vestibular unilateral. Rev Bras Fisioter. 2010;14: 330–336 Schwab B, Kontorinis G. Influencing factors on the vestibular function of deaf children and adolescents: evaluation by means of dynamic posturography. The Open Otorhinolaryngology Journal 2011;5:1–9 Horak FB, Shumway-Cook A, Crowe TK, Black FO. Vestibular function and motor proficiency of children with impaired hearing, or with learning disability and motor impairments. Dev Med Child Neurol 1988;30:64–79 Prencht W. Recovery of some vestibular and vestibulospinal functions following unilateral labyrynthectomy. Prog Brain Res 1986;64:381–389

in patients with peripheral vestibular syndrome: exploratory study. In: Archives for Sensology and Neurootology in Science and Practice. Porto, Portugal: Neurootological and Equilibriometric Society; 2003 Kavalco TF. A manifestação de alterações posturais em crianças de primeira a quarta séries do ensino fundamental e sua relação com a ergonomia escolar. Rev Bras Fisioterapia 2000;2(4) Guilder RP, Hopkins LA. Auditory function studies in an unselected group of pupils at the Clarke school for the deaf. Laryngoscope 1936;46:190–197 Lavinsky L. Vestibular function in children with severe hearing deficiency. Rev HCPA 1990;10(1):14–26 Horak FB, Shupert C. Função de sistema vestibular no controle postural. In: Herdman SJ, ed. Reabilitação Vestibular. São Paulo, Brazil: Manole; 2002:25–51 Magee DJ. Avaliação da postura. In: Magee DJ, ed. Avaliação Musculoesquelética. 3rd ed. São Paulo, Brazil: Manole; 2002: 723–753 Hall SJ. Biomecânica básica. Rio de Janeiro, Brazil: Guanabara Koogan; 1996 Shumway-Cook A, Woollacott MH. Controle postural normal. In: Shumway-Cook A, Woollacott MH, eds. Controle Motor—teoria e aplicações práticas. 2nd ed. Barueri, Brazil: Manole; 2003: 153–178 Melo RS, Silva PWA, Tassitano RM, Macky CFST, Silva LVC. Balance and gait evaluation: comparative study between deaf and hearing students. Rev Paul Pediatr 2012;30(3):385–391

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Original Article

Prevalence of Contralateral Hearing Aid Use in Adults with Cochlear Implants Cintia Tizue Yamaguchi1

Maria Valéria Schmidt Goffi-Gomez1

1 Division of Cochlear Implants, Department of Audiology, Clinic

Hospital of the Medicine High School of the University of São Paulo (HCFMUSP), São Paulo/SP, Brazil

Address for correspondence Cintia Tizue Yamaguchi, Audiologist, 255 Dr. Enéas de Carvalho Aguiar, 6th floor, Cerqueira César, São Paulo/SP 05403-000, Brazil (e-mail: ct.yamaguchi@uol.com.br).

Int Arch Otorhinolaryngol 2013;17:370–374.

Abstract

Keywords

► ► ► ►

cochlear implantation deafness hearing aids speech perception

Introduction The exclusive use of a cochlear implant (CI) in one ear allows patients to effectively hear speech in a quiet environment. However, in environments with competing noise, the processing of multiple sounds becomes complex. In an attempt to promote binaural hearing in a noninvasive manner, the use of a hearing aid in the nonimplanted ear is suggested for patients with a unilateral CI. Aims To identify the prevalence of hearing aid use in the contralateral ear in adults who already have a CI; to determine the reasons why some patients do not use contralateral hearing aids (CHAs); and to analyze the effects of residual hearing in CHA users. Materials and Methods This is a clinical study in 82 adult patients with CI implants who responded to a questionnaire designed to determine current use of CHA. Results In our patient sample, 70 CHA nonusers were identified. The prevalence of CHA users was determined to be 12% with a 95% confidence interval of 11 to 13%. About 58.2% of the CHA nonusers reported a lack of noticeable benefit even after wearing hearing aids, and 23.6% reported not having received the option to use a CHA. CHA users had a pure tone average of 107-dB hearing level, whereas CHA nonusers had a pure tone average of 117-dB hearing level. Conclusion The prevalence of the use of a CHA is low in our study. We attribute the low use of a CHA to either a lack of residual hearing or to a lack of benefit from the amplification.

Introduction Deafness is a disability that affects individuals on variety of levels: social, emotional, and familial. As a result, deafness leads to significant lifestyle modifications. Individual sound amplification devices (hearing aids) can mitigate hearing loss by amplifying sound input according to the needs of a patient. However, some patients with severe to profound hearing loss do not benefit from the use of hearing aids (due to a lack of sensory input) and cannot appropriately discriminate words and sentences. The most efficient and widely used solution for this type of deficit is the placement of a cochlear implant (CI). A CI is an

received September 4, 2012 accepted June 6, 2013

electronic device that is implanted into the cochlea. CIs are indicated for the treatment of individuals with hearing loss who have severe to profound hearing loss and have not benefitted from the use of traditional hearing aids. The CI is an effective adjunct during the (re)habilitation of prelingual hearing-impaired children and postlingual hearing impaired adults.1,2 The CI is usually implanted unilaterally. However, questions have been raised concerning issues related to unilateral hearing. For instance, unilateral hearing impairs the performance of speech perception in environments with a high level of ambient noise and negatively affects the performance of schoolchildren.3,4

Copyright © 2013 by Thieme Publicações DOI http://dx.doi.org/ Ltda, Rio de Janeiro, Brazil 10.1055/s-0033-1352501. ISSN 1809-9777.


Contralateral Hearing Aid Use in Adults with CIs The exclusive use of a CI in one ear (without the use of a contralateral hearing aid) allows patients to effectively hear speech in a quiet environment. However, in environments with competing noise, the processing of multiple sounds becomes difficult. In an attempt to promote binaural hearing in a noninvasive manner, the use of a hearing aid on the nonimplanted ear has been suggested for patients with a CI. This approach has been referred to as bimodal stimulation.5 The International Consensus on Bilateral Cochlear Implants and Bimodal Stimulation recommends the application of bimodal stimulation in patients with residual hearing.6 It also proposes that bimodal stimulation may offer advantages with respect to speech perception in quiet and noisy environments and may contribute to sound localization. The use of both CIs and hearing aids, rather than each of these alone, has shown an improvement in speech perception in noisy environments.7 Potts et al performed a study evaluating the concomitant use of CIs and hearing aids and the combined effect on speech recognition and localization.8 They suggested that the hearing aid must be designed using digital technology. They concluded that the combined benefits of a CI and a contralateral digital hearing aid are greater than the benefits of using these alone. Fitzpatrick and Leblanc conducted a study exploring the factors influencing the discontinuation contralateral hearing aid use in patients with a CI.9 They proposed that an “enhanced understanding of the factors influencing patients’ decisions may help clinicians to modify their recommendations and counseling and thereby increase patient exposure to the potential benefits of combining acoustic and electrical stimulation.” Cullington and Zeng compared music perception, affective prosody discrimination, speaker identification, and speech recognition in presence of a competing talker in bimodal and bilateral CI users.10 The aim of their study was to identify whether bilateral CI users or those with bimodal stimulation had the best aural performance. The authors found no difference between the groups. However, this approach is not always followed in clinical practice. As a result, the potential stimulation of residual hearing of the opposite ear, which would allow the patients to benefit from binaural hearing, is often not attempted. In the present study, we focus on the reasons why adults with CIs who have residual hearing do not use a hearing aid on the contralateral ear.

Aims To identify the prevalence of the use of hearing aids in adults who received a CI at the Clinic Hospital of Medicine High School of the University of São Paulo (HCFMUSP); to determine the reasons why patients with CIs reported the nonuse of hearing aids and to elucidate the effects that residual hearing has on the decision to use a hearing aid in the contralateral ear.

Yamaguchi, Goffi-Gomez

Materials and Methods The studied cohort included all of the patients who were implanted with a Cochlear Nucleus 22, Cochlear Nucleus 24, or Medel Combi 40 multichannel CIs from April 12, 1999 to July 18, 2007. Patients who met the following inclusion criteria were enrolled in the study: users of unilateral, multichannel CIs that met the HCFMUSP criteria11; prelingual or postlingual hearing-impaired adults (aged 18 years or older). Three types of methods were used for data collection: inperson interviews (patients who were scheduled for programming of their CI within the period of data collection); telephone interviews; and questionnaires sent via e-mail. In some cases, it was necessary to use more than method of contact. A questionnaire (►Table 1) was prepared and presented to all contacted patients. The questions were designed to encompass the following information: the use or nonuse of hearing aids in the contralateral ear, the reasons for use or nonuse, and whether the patients experienced any

Table 1 Hearing aid and cochlear implant (CI) use questionnaire Name: Age: Gender: Education: CI side: date of CI placement: Pure tone average implanted side: not required Pure tone average opposite side: not required 1. Did you use hearing aids before receiving the CI? If yes, in which ear? Yes ( ) No ( ) Right ear ( ) Left ear ( ) 2. Do you use a hearing aid on the ear other than the one with the CI? Yes ( ). Why? I was oriented to ( ) I have benefited from the hearing aid ( ) It contributes to the CI ( ) Others: ( ) No ( ). Why? Lack of orientation ( ) Lack of benefit ( ) Lack of financial resources ( ) Others: ( ) 3. Do you miss or have difficulty in localizing sounds? Yes ( ) No ( ) Sometimes ( ) ___________________________

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Table 2 Demographic data

n

Group 1

Group 2

72

10

Table 4 Reasons for using hearing aids contralateral to the CI and PTA threshold (in decibels hearing level) of patients who reported the reasons n

%

Mean

SD

Helps to listen better

7

70

103.14

6.36

Instructed to use

1

10

100

Binaural advantage and instructed

2

20

125

Total

10

100

Age (y) Median

42

37

Minimum

18

22

Maximum

70

63

72

10

Median

117.8

107.2

Minimum

93

95

Maximum

125

125

Hearing aid users (n)

PTA (dB)

PTA contralateral (dB)

Abbreviations: CI, cochlear implant; PTA, pure tone average; SD, standard deviation.

Table 5 Between-group statistical analysis of pure-tone average threshold (in decibels hearing level)

Abbreviation: PTA, pure tone average.

difficulties in sound localization. The questionnaire included a section about consent for the use of the data in our study. After completion of the questionnaire, data relating to the pure tone average (PTA) threshold of 500, 1,000, and 2,000 Hz were collected from medical records.12 A value of 125 dB was determined as the frequency threshold absent in audiometry for the calculation of the PTA threshold of the ear contralateral to the CI. In total, 137adults met the initial criteria for entry into our study. Of these, we were unable to contact 32, and 23 patients were excluded on the basis of the exclusion criteria. Therefore, the final total sample size was 82 patients (36 women and 46 men). The cohort was divided into two groups: group 1 comprised CI patients who were nonusers of a contralateral hearing aid; group 2 comprised CI patients who were users of a contralateral hearing aid (►Table 2). The data were analyzed through descriptive statistics. Shapiro–Wilk test was used to assess the normality of the data. When normality was observed, a t test was used; otherwise, parametric Mann–Whitney test was applied. Data were considered statistically significant if p values were < 0.05.

During the interview, the participants were asked about their difficulties with sound localization. In Group 1, a majority of the participants noted difficulty with sound localization in environments with background noise. The difference between groups 1 and 2 was not statistically significant (►Table 4). We observed that 70% of the patients reported improvement in their hearing ability with the combined use of CI and a contralateral hearing aid; 20% of the patients benefited from bimodal stimulation with 125 dB of residual hearing (►Table 5). The Mann–Whitney test showed a significant difference in the PTA thresholds of the two groups (p ¼ 0.0051; ►Table 6).

Results

Discussion

The prevalence of individuals with CIs who were using hearing aids in the contralateral ear was 12% (95% confidence interval, 11 to 13%; ►Tables 2 and 3).

Currently, bimodality (CI combined with a contralateral hearing aid) is fundamental with regard to the possibility of achieving binaural hearing in patients with bilateral, severe to

Group 1

Group 2

p

n

72

10

0.0051

Mean

117.76

107.2

Standard deviation

9.46

10.77

Median

125

103

Table 3 Difficulty of sound localization of studied groups referred during interview Group 1

p

Group 2

Difficulty in the sound localization

n

%

n

%

No

14

19.4

2

20

0.9693

Only in environments

34

47.2

2

20

0.0851

Yes

17

23.6

3

30

0.6988

Data not available

7

9.7

3

30

0.2249

Total

72

100

10

100

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Contralateral Hearing Aid Use in Adults with CIs

Yamaguchi, Goffi-Gomez

Table 6 Distribution of the pure tone average threshold (in decibels hearing level) by intervals in both studied groups Group 1

p value

Group 2

dB

n

%

Main

SD

n

%

Main

SD

90–100

6

8.50

96

2.76

4

40.00

98.75

2.5

0.184

101–110

8

11.30

106,13

3.6

3

30.00

104

2.65

0.3337

111–120

20

28.20

115,7

2.68

1

10.00

115

> 120

37

52.10

124,89

0.66

2

20.00

125

0

0.9075

Total

71

10

Abbreviation: SD, standard deviation.

profound, hearing loss. This combination is generally used in patients who have residual hearing and/or who have a good response with a hearing aid in the nonimplanted ear. Several studies have been conducted on the subject with the aim to expand upon the knowledge already known about bimodality.1,2,5–7 Several researchers have concluded that sound localization can be improved through the use of a CI combined with a contralateral hearing aid.5,8,13 ►Table 2 displays the responses of the participants when questioned about the difficulty of sound localization. The responses relating to sound localization were similar in both groups. Moreover, in our study there were participants who did not describe difficulties in sound localization; despite being unilateral CI users without a contralateral hearing aid. Dunn et al14 published a study in which only 2 of the 12 patients evaluated for sound localization were able to localize sound with a CI combined with a contralateral hearing aid. The authors also reported that patient effort is a factor when it comes to improving the integration of sensory information (and improved sound localization) provided by the combined use of a CI and a hearing aid. Their conclusion concerning the importance of patient effort is supported by the following factors: (1) sound localization is a central auditory nervous system process, and (2) some people are able to integrate information from two ears (improving speech perception and sound localization) and others (with similar, two-ear capabilities) are not. The patient responses concerning the reasons for the use of a CI in conjunction with a hearing aid are presented in ►Table 3. These data show that most patients (70%) reported that sound quality was better with the combined use of a CI and a hearing aid. The data also show that these patients have PTA contralateral to a CI capable of offering acoustic information that contributes to sound quality. Some authors have suggested a theory for how the bimodal condition provides superior information: acoustic amplification through a contralateral hearing aid provides necessary, lowfrequency information, which is not provided by the CI alone.5,15,16 We believe that the level of residual hearing plays an important role in bimodal adaptation. PTA comparison between group 1 and group 2 was statistically significant (p ¼ 0.0051; ►Table 4). The group of patients with better

residual hearing was the group that reported the combined use of a CI with a contralateral hearing aid. Several other authors have suggested that residual hearing contributes to the effectiveness of bimodality.5,6,8,13 However, Ching et al found no statistical significance when comparing the PTA in children on the basis of tests for sound localization and speech perception and questionnaires given to parents of the children.17 The authors mentioned that the contributions from residual hearing were found to be associated with the individual. Therefore, the following questions remain: (1) Is the success of bimodal hearing directly dependent upon the residual hearing of patients? (2) Is the success of bimodal hearing more dependent upon the integration of the central, auditory, nervous system? (3) Is the success of bimodal hearing associated with both of these factors? Potts et al stated that it “is recommended that unilateral CI recipients, with measurable unaided hearing thresholds, be fit with a hearing aid.”8 Continuing with the topic of residual hearing, ►Table 5 shows the distribution of residual hearing according to groups. It was observed that the percentage of subjects with residual hearing up to 110 dB was higher in group 2, and the percentage of individuals with residual hearing up to 110 dB was lower in group 1. Interestingly, all of the patients in group 2 used hearing aids in both ears prior to their CI placement. In addition, most of the patients in group 1 did not use hearing aids in both ears prior to their CI placement. This leads us to conclude that the use of hearing aids prior to the placement of a CI may have some influence on the central auditory nervous system integration, such that, when adapted to bimodality, integration and plasticity of the auditory pathways already exist.

Conclusions In our clinic, we were able to identify that the prevalence of the use of a contralateral hearing aid in adults with CI was 12%. One of the reported reasons for not using a contralateral hearing aid in conjunction with a CI was a lack of perceived benefit from the hearing aid. The residual hearing of the CI patients who use a contralateral hearing aid was higher (107-dB hearing level) than that of the CI patients who do not use a contralateral hearing aid (117-dB hearing level). International Archives of Otorhinolaryngology

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Yamaguchi, Goffi-Gomez

References

9 Fitzpatrick EM, Leblanc S. Exploring the factors influencing dis-

1 Bento FR, Neto RB, Castilho AM, Gómez VG, Giorgi SB, Guedes MC.

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Resultados auditivos com implante coclear multicanal em pacientes submetidos à cirurgia no Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. Braz J Otorhinolaryngol 2004;70:632–637 Bevilacqua MC, Filho OC, Nascimento LT, Banhara MR. Evaluation of the combined use of hearing aid and cochlear implants in adults. Disturb Comun 2004;16:27–33 Feuerstein JF. Monaural versus binaural hearing: ease of listening, word recognition, and attentional effort. Ear Hear 1992; 13:80–86 Lieu JE. Speech-language and educational consequences of unilateral hearing loss in children. Arch Otolaryngol Head Neck Surg 2004;130:524–530 Ching TY, Incerti P, Hill M, van Wanrooy E. An overview of binaural advantages for children and adults who use binaural/bimodal hearing devices. Audiol Neurootol 2006;11(Suppl 1):6–11 Offeciers E, Morera C, Müller J, Huarte A, Shallop J, Cavallé L. International consensus on bilateral cochlear implants and bimodal stimulation. Acta Otolaryngol 2005;125:918–919 Hamzavi J, Pok SM, Gstoettner W, Baumgartner WD. Speech perception with a cochlear implant used in conjunction with a hearing aid in the opposite ear. Int J Audiol 2004;43:61–65 Potts LG, Skinner MW, Litovsky RA, Strube MJ, Kuk F. Recognition and localization of speech by adult cochlear implant recipients wearing a digital hearing aid in the nonimplanted ear (bimodal hearing). J Am Acad Audiol 2009;20:353–373

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continued hearing aid use in patients with unilateral cochlear implants. Trends Amplif 2010;14:199–210 Cullington HE, Zeng FG. Comparison of bimodal and bilateral cochlear implant users on speech recognition with competing talker, music perception, affective prosody discrimination, and talker identification. Ear Hear 2011;32:16–30 Gomez MVSG, Guedes MC, Sant’Anna SBG, et al. Medical and audiological selection criteria and evaluation for cochlear implants candidates: HC-FMUSP protocol. Int Arch Otorhinolaryngol 2004;8: 295–303 Davis H, Silverman RS. Hearing and Deafness. 3rd ed. New York: Holt, Rinehart & Wilson; 1970 Seeber UB, Baumann U, Fastl H. Localization ability with bimodal hearing aids and bilateral cochlear implants. J Acoust Soc Am 2004;116:1699–1709 Dunn CC, Tyler RS, Witt SA. Benefit of wearing a hearing aid on the unimplanted ear in adult users of a cochlear implant. J Speech Lang Hear Res 2005;48:668–680 Kong YY, Stickney GS, Zeng FG. Speech and melody recognition in binaurally combined acoustic and electric hearing. J Acoust Soc Am 2005;117(3 Pt 1):1351–1361 Vandali AE, Sucher C, Tsang DJ, McKay CM, Chew JW, McDermott HJ. Pitch ranking ability of cochlear implant recipients: a comparison of sound-processing strategies. J Acoust Soc Am 2005;117:3126–3138 Ching TY, Psarros C, Hill M, Dillon H, Incerti P. Should children who use cochlear implants wear hearing aids in the opposite ear? Ear Hear 2001;22:365–380


Original Article

Allergen-Specific Immunotherapy in Patients 55 Years and Older: Results and Review of Literature Eduardo Baptistella1 Sergio Maniglia1 Diego Augusto Malucelli1 Daniel Rispoli1 Thanara Pruner de Silva1 Fernanda Miyoko Tsuru1 Renata Vecentin Becker1 Gustavo Bernardi1 Daniela Dranka1 Bruno Ferraz1 1 Centro Médico Especializado Baptistella (CMEB), Curitiba/PR, Brazil

Int Arch Otorhinolaryngol 2013;17:375–379.

Abstract

Keywords

► immunotherapy ► aged ► rhinitis

Introduction Over the years the immune system suffers many morphologic and functional alterations, which result in a peak of function in puberty and a gradual decrease in the elderly. Aim Treat patients 55 years or older with allergic rhinitis with immunotherapy and then analyze the response to allergens. Materials and Methods From June 2009 to July 2010, 104 charts of patients 55 years or older with allergic complaints were evaluated. The patients were selected by anamnesis, physical examination, and otorhinolaryngologic exam. The patients had cutaneous test for mites before and after 1 year of sublingual specific immunotherapy. The cutaneous response was classified as negative (absent), light, moderate, or severe. Results Before vaccination, 42 (40.4%) patients were classified as having a severe form of allergy and 62 (59.6%) as having a moderate allergy. After the specific therapy, 40 (38.4%) patients were classified as negative (absent), 37 (35.6%) as light, 19 (18.3%) as moderate, and 8 (7.7%) as severe responses. Conclusion Immunotherapy, a desensitization technique, is indicated in cases which patients cannot avoid the exposure to allergens and in situations where pharmacologic therapy is not ideal. Specific immunotherapy to treat the allergic rhinitis in elderly patients was efficient and had no collateral effects, and in addition to the clinical benefit, improvement in the cutaneous test could also be observed.

Introduction Over the years, the immune system—responsible for combating allergy symptoms in the organism—suffers many morphologic and functional alterations. The immune system helps the human antigenic constitution. The immune system consists of cells, molecules, and genes that interact with other systems for the protection of the organism. This system is composed of innate immunity, which represents the nonspecific natural resistance of human beings, and adaptive immu-

received November 6, 2012 accepted June 13, 2013

Address for correspondence Fernanda Miyoko Tsuru, CMEB, Eduardo Baptistella Avenida João Gualberto, 1795, Conjunto 01 Juvevê, Curitiba/PR, CEP 80 030-001, Brazil (e-mail: feu.tsuru@yahoo.com.br).

nity (immunity proper)—more specifically, memory and higher-efficiency immunity. The immune system changes and peaks in puberty and then declines gradually in the elderly,1 known as immunosenescence. There is a great interaction between the immune and nervous systems, and this means there is an exacerbation of immune disorders and depression of the normal functions of the immune system.2 Apparently, the elderly are more vulnerable to these events.3

Copyright © 2013 by Thieme Publicações DOI http://dx.doi.org/ Ltda, Rio de Janeiro, Brazil 10.1055/s-0033-1353138. ISSN 1809-9777.

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Allergen-Specific Immunotherapy in Patients  55 Years The decline of immunity is related to changes that occur in each of the cell types that make up the immune system. The T cells responsible for cellular immunity show reduction of early events in their signaling cascades,4,5 resulting in decrease of activity. Cytokines, key mediators of the immune response that control various cellular functions (proliferation, differentiation, and cell death),6 become imbalanced and increase the susceptibility of the organism to infections caused by viruses and extracellular bacteria.7 These defects in regulation of the immune response can also lead to an increase in autoimmune diseases.8,9 B cells are responsible for humoral immunity and show qualitative and quantitative changes in their repertoire, which are characterized by low antibody response and decreased high-affinity antibodies to the aging of the organism.10 Allergic rhinitis is a disorder linked to the immune system and may be associated with other allergic manifestations such as bronchial asthma, dermatitis, conjunctivitis, among others.11 This disease affects, in a greater or lesser degree, all ages, with the prevalence of 25% in the general population.1,12,13 It is even reported among young people; however, with the changing age pattern, it is estimated that there are a greater number of diagnoses of this entity in the elderly. It is also estimated that one third of elderly people suffer from some type of allergic symptoms. Patients with allergic rhinitis have type 1 hypersensitivity reaction, with persistent inflammation of the nasal mucosa. Inflammatory cells such as eosinophils and mast cells, and mediators such as chemokines and cytokines, participate in the pathophysiology. The pattern developed by the immune response in atopic patients is Th2 with production of cytokines such as interleukin (IL)-4, IL-5, and IL-10.14–16 B lymphocytes from individuals previously sensitized to specific allergens produce IgE antibodies that bind to the surface of mast cells and basophils by high-affinity receptors for the Fc portion. After new contact, the allergen binds IgE on the surface of mast cells, promoting the same degranulation, with release of histamine, lipid-derived substances (prostaglandins and leukotrienes), and cytokines. Eosinophils are also recruited to the inflammatory site and release a cationic protein with amplification of the allergic response.17 In other words, allergic rhinitis is an inflammation of the nasal mucosa, induced by exposure to allergens after sensitization, which trigger an inflammatory response mediated by IgE, resulting in chronic or recurrent symptoms.11 The diagnosis is basically clinical. An appropriate otorhinolaryngologic examination identifies without many difficulties signs of rhinitis, such as hypertrophy and pallor of the inferior turbinates and hyaline secretion.18 However, additional tests may help establish the diagnosis, treatment, and follow-up of these patients. Sensitization to allergens is detected using in vitro techniques such as measurement of specific IgE (radioallergosorbent test, or RAST) and also by in vivo tests, such as skin tests, to determine immediate hypersensitivity.19 Several allergens may be tested through cutaneous tests and RAST. However, mites are the main constituents of domiciliary dust and the allergens that cause more awareness International Archives of Otorhinolaryngology

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Baptistella et al. and respiratory symptoms among atopic patients in the city of Curitiba. The main mites found in this city are the Dermatophagoides pteronyssinus (Pyroglyphidae family) and the Blomia tropicalis (Glycyphagidae family). There is a predominance of the D. pteronyssinus (65%) over the B. tropicalis (30%), as shown by the domiciliary dust mites study in which 384 domiciliary dirt samples were examined.20 Other allergens frequently tested are pollen, grasses, fungi, and cat and dog epithelium. The RAST is allergen-specific and IgE immune-mediated. It is highly specific and less sensitive in addition to being more expensive than other types of tests available on the market, for example, the skin prick test. A laboratory blood test identifies IgE in a patient’s blood. Therefore, the more IgE is present, the greater the allergic response to a specific antigen. Because it is a quantitative test, it is useful not only to make the diagnosis of allergy but also to take preventive measures and to monitor the efficacy of treatment and compliance.18 The RAST must be ordered in a rational way, based on history and physical examination, only if it is necessary. The skin test is also very important in differential diagnosis of nasal disease and in determining the pattern of sensitization of the population. It is an important foundation for appropriate treatment of allergic rhinitis, particularly with regard to specific immunotherapy and also in promoting measures to reduce patient exposure to the allergen identified. For this test, patients are examined and treatment objectives are explained to the patient. The medial surface of the volar forearm is assessed to exclude dermal lesions and is wiped with 70% alcohol. A single drop of allergen extract is placed using the dropper, at a distance of 2 cm, in a predetermined allergens sequence. A plastic device is used that limits the degree of penetration into the skin (PUNTOR, Alko do Brasil, Rio De Janeiro, Brazil) for each allergen. After 3 minutes, the excess extract is removed with a paper towel, avoiding contaminating neighboring tests. Reading takes place 15 to 20 minutes after the puncture. The presence of papules with diameters  3 mm indicates a positive reaction, and results are graduated subjectively as light, moderate, or severe. The interruption in the use of the antihistamines is necessary for at least 7 days before a cutaneous test and the RAST.21 Allergic rhinitis treatment consists of an environmental control with withdrawal of the patient from the possible risk factors identified by the cutaneous test. Besides, the treatment may be associated with the application of topic nasal products based on corticoids and also on antihistamines. Another form of therapy is based on the systemic response of the patient. The therapeutic modality focuses on specific immunotherapy and is more active against seasonal allergies. It is based on the administration of increasing doses of allergen-specific substances, and the individual’s hypersensitivity reaction is mediated by IgE.22 This treatment may be effective in patients who persistently present signals and symptoms with traditional treatment. Furthermore, the immunotherapy may be discontinued after 45 years, when the IgE levels become elevated. The prognostic is extremely variable, and the symptoms may persist for up to 10 years in children.18


Allergen-Specific Immunotherapy in Patients  55 Years

Aim The present study included patients 55 years or older with allergic rhinitis who had immunotherapy, and the efficiency of the specific-allergen immunotherapy was analyzed.

Materials and Methods The present study was approved by the Ethics Committee and Research from Centro Médico Baptistella (CMEB) under protocol number 0015/2009.

Design of the Experiment From June 2009 to July 2010, 104 charts of patients treated in CMEB 55 years or older with allergic complaints (including nasal obstruction, rhinorrhea, itching, and sneezing) were evaluated in Curitiba. The patients were selected by anamnesis, physical examination, and otorhinolaryngologic exam. All the patients had the cutaneous test for mites before and after 1 year of sublingual specific immunotherapy. The cutaneous response was classified as negative (absent), light, moderate, or severe.

Sample The final sample included 104 patients aged 55 years or older. The medium age was 64.5 years, with 55 years and 74 years being the minimum and maximum, respectively. There were 73 (70.2%) female and 31 (29.8%) male patients. Patients undergoing treatment for other comorbidities and/or taking other continuous medication were excluded.

Allergic Tests The cutaneous test was used before and 1 year after the beginning of the immunotherapy. It was used a mixture of two mites: D. pteronyssinus and B. tropicalis, both of which are

Baptistella et al.

triggers of allergic symptoms in patients residing in Curitiba. Using a dropper, a single drop of the allergen extract was placed above the medial surface of the foreman at a distance of 2 cm. After 3 minutes, the excess was removed with a paper towel and the reading took place after 15 minutes. The response was measured as negative (no papule), mild (papule < 4 mm), moderate (papule 4 to 6 mm), or severe (papule > 6 mm), according to the local reaction unleashed by the allergen in the patient’s skin.

Allergen-Specific Immunotherapy Based on allergy test, three sublingual vaccine vials in different concentrations were used. Each 8-mL bottle was diluted as 1:100,000, 1:10,000, or 1:1,000. Doses were given three times per week (Monday, Wednesday, and Friday). The ideal treatment lasted 2 years and consisted of drops administered sublingually incrementally on the aforementioned days of the week (►Table 1).

Results The cutaneous test before vaccination showed 42 patients with severe index, the equivalent of 40.4% of the sample, and 62 patients with moderate index, representing 59.6% of the total, as shown by ►Fig. 1. After the specific immunotherapy, 40 patients (38.4%) were classified as negative for the allergen, 37 (35.6%) had light response, 19 patients (18.3%) had moderate response, and 8 (7.7%) had severe response to the allergen, as shown by ►Fig. 2.

Discussion Immunotherapy, a desensitization technique, is indicated in special cases when the patient cannot avoid allergen

Table 1 Schedule of allergen-specific immunotherapy

1 wk

2 wk

3 wk

4 wk

5 wk

Day

Phase 1 (1:100,000), number of drops

Phase 2 (1:10,000), number of drops

Phase 3 (1:1000), number of drops

Monday

1

1

1

Wednesday

2

2

2

Friday

3

3

3

Monday

4

4

4

Wednesday

5

5

5

Friday

6

6

6

Monday

7

7

7

Wednesday

8

8

8

Friday

9

9

9

Monday

10

10

10

Wednesday

11

11

11

Friday

12

12

12

Monday

13

13

13

Wednesday

14

14

14

Friday

15

15

15

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Allergen-Specific Immunotherapy in Patients  55 Years

Baptistella et al. In the present study we noticed the benefits of specific immunotherapy for the treatment of allergic rhinitis in elderly patients. During the evaluation of the 104 patients submitted to this therapeutic modality, there were no side effects. Moreover, the therapeutic evolution could be observed based on the cutaneous test. The divergences found in the literature regarding both contraindications to the various treatment times and allergen-specific administrations and the results achieved in this study show the need for further research in this area to confirm that allergen-specific immunotherapy is an alternative treatment for allergic rhinitis in patients aged 55 years or older in whom conventional drug treatment and environmental control were not sufficient treatment of the chronic disease in question.

References 1 Machado PP. O baú dos sonhos adormecidos: a dimensão simból-

Fig. 1 Skin test response prior to allergen-specific immunotherapy. 2 3

4 5 6

7

8

9

10

Fig. 2 Skin test response after allergen-specific immunotherapy.

exposition and in situations when there is no appropriate response to pharmacologic treatment. Although not including specific vaccination on elderly people in the literature, the present study demonstrates that immunotherapy for allergic rhinitis in this age group is safe and effective. The European Academy of Allergy and Clinical Immunology advises specific vaccination as a relative contraindication for elderly patients with allergic rhinitis. 23 The II Brazilian Consensus about Rhinitis, from 2006, 24 on the other hand, affirmed that, in rare cases, immunotherapy may be used in seniors. However, details about possible specifications were not provided. The Canadian Society of Allergy and Clinical Immunology, 25 in its guidelines for the use of immunotherapy, does not contraindicate this treatment for aged patients. International Archives of Otorhinolaryngology

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ica da rinite alérgica em um estudo de caso. Bol Psicol 2007;57 (126):89–106 McEwen BS. Physiology and neurobiology of stress and adaptation: central role of the brain. Physiol Rev 2007;87:873–904 Graham JE, Christian LM, Kiecolt-Glaser JK. Stress, age, and immune function: toward a lifespan approach. J Behav Med 2006; 29:389–400 Miller RA, Garcia G, Kirk CJ, Witkowski JM. Early activation defects in T lymphocytes from aged mice. Immunol Rev 1997;160:79–90 Pawelec G, Hirokawa K, Fülöp T. Altered T cell signalling in ageing. Mech Ageing Dev 2001;122:1613–1637 Fulop T, Larbi A, Douziech N, Levesque I, Varin A, Herbein G. Cytokine receptor signalling and aging. Mech Ageing Dev 2006;127:526–537 Bonagura VR, Hatam L, DeVoti J, Zeng F, Steinberg BM. Recurrent respiratory papillomatosis: altered CD8(þ) T-cell subsets and T(H) 1/T(H)2 cytokine imbalance. Clin Immunol 1999;93:302–311 Rizzo LV, Belfort R Jr. Ocular autoimmunity. In: Lahita RG, Chiorazzi N, Reeves WH, eds. Textbook of the Autoimmune Diseases. Philadelphia, PA: Lippincott Williams & Wilkins; 2000:515 Amadori A, Zanovello P, Cozzi E, et al. Study of some early immunological parameters in aging humans. Gerontology 1988; 34:277–283 Ewers I, Rizzo LV, Kalil F. Imunologia e envelhecimento. Einstein 2008;6(Suppl 1):S13–S20 Silva TM. Perfil das Citocinas IL4, IL5, IL8e IFN-ã Analisadas por T-PCR em Tecido de Mucosa Nasal de Pacientes Portadores de Rinite Alérgica. [Dissertação (mestrado)]. Belo Horizonte, Brazil: Universidade Federal de Minas Gerais, Faculdade de Medicina; 2007 Ibiapina Cda C, Sarinho ES, Camargos PA, Andrade CR, Cruz Filho AA. Allergic rhinitis: epidemiological aspects, diagnosis and treatment. J Bras Pneumol 2008;34(4):230–240 Megid CBC, Cury PM, Cordeiro JA, Jorge RBB, Saidah R, Silva JBG. Tratamento da Rinite Alérgica: comparação da acunpuntura e corticóide nasal. Acta ORL 2006;24(2):61–66 Benson M, Strannegård IL, Wennergren G, Strannegård O. Interleukin-5 and interleukin-8 in relation to eosinophils and neutrophils in nasal fluids from school children with seasonal allergic rhinitis. Pediatr Allergy Immunol 1999;10:178–185 Mygind N, ed. Allergic and Nonallergic Rhinitis: Clinical Aspects. Philadelphia, PA: Saunders; 1993 Bradding P, Feather IH, Wilson S, et al. Immunolocalization of cytokines in the nasal mucosa of normal and perennial rhinitic subjects. The mast cell as a source of IL-4, IL-5, and IL-6 in human allergic mucosal inflammation. J Immunol 1993;151:3853–3865


Allergen-Specific Immunotherapy in Patients  55 Years

Baptistella et al.

17 Durham SR, Ying S, Varney VA, et al. Cytokine messenger RNA

21 Godinho R, Lanza M, Godinho A, Rodriques A, Assiz TML. Freqüên-

expression for IL-3, IL-4, IL-5, and granulocyte/macrophage-colony-stimulating factor in the nasal mucosa after local allergen provocation: relationship to tissue eosinophilia. J Immunol 1992;148:2390–2394 18 Baptistella E, Trotta F, Silva TP, et al. Revisão da literatura e estudo retrospectivo de 670 pacientes com rinite alérgica. ACTA ORL/ Técnicas em Otorrinolaringologia 2009;27:85–88 19 Lopes MI, Miranda PJ, Sarinho E. Use of the skin prick test and specific immunoglobulin E for the diagnosis of cockroach allergy. J Pediatr (Rio J) 2006;82(3):204–209 20 Rosário Filho NA, Baggio D, Suzuki MM, Thomaz PCP, Sugisawa S, Hanggi V. Ácaros na poeira domiciliar em Curitiba [Abstract]. Rev Bras Alerg Imunopatol 1992;25:15–25

cia de positividade em teste cutâneo para aeroalérgenos. Rev Bras Otorrinolaringol (Engl Ed) 2003;69(6):824–828 Associação Brasileira de Alergia e Imunopatologia e Sociedade Brasileira de Pediatria. Projeto Diretrizes. In: Imunoterapia alérgeno-específica. Projeto Diretrizes. Julho 2011 Mailing H, Weeke B; Position Paper of the European Academy of Allergy and Clinical Immunol. Position paper: immunotherapy. Allergy 1993;48(14, Suppl):9–35 Solé D, Mello Jr JF, Weckx LLM, et al. II Consenso Brasileiro sobre Rinites. Rev Bras Alerg Imunopatol 2006;29(1):29–58 Guidelines for the Use of Allergen Immunotherapy.. Canadian Society of Allergy and Clinical Immunology. Available at: http:// www.csaci.ca/index.php?page=361. Accessed May 22, 2011

22

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Original Article

Vector Electronystagmography Analysis in Elderly Individuals with Dizziness Complaint Lidiane Maria de Brito Macedo Ferreira1 Karyna Mirelly O. B. de Figueiredo Ribeiro2 Arthur Jorge de Vasconcelos Ribeiro4 Kenio Costa de Lima5 1 Department of Otolaryngology, Universidade Fedreral do Rio Grande

do Norte (UFRN), Natal/RN, Brazil 2 Department of Physiotherapy, Universidade Fedreral do Rio Grande do Norte (UFRN), Natal/RN, Brazil 3 Department of Audiology, Universidade Potiguar (UnP), RN, Brazil 4 Division of Neurology, Department of Pediatrics, Universidade Federal do Rio Grande do Norte (UFRN), Natal/RN, Brazil 5 Department of Public Health, Universidade Federal do Rio Grande do Norte (UFRN), Natal/RN, Brazil

André Pestana3

Address for correspondence Lidiane Maria de Brito Macedo Ferreira, Universidade Federal do Rio Grande do Norte, Av. Campos Sales, 682, AP 1302, Natal/RN, Brazil (e-mail: lidianembm@yahoo.com.br).

Int Arch Otorhinolaryngol 2013;17:380–382.

Abstract

Keywords

► aged ► dizziness ► medical examination

Introduction Vector electronystagmography is widely used to evaluate vestibular function; however, elderly patients may show changes not due to vestibular disorders. Aim This study aims to characterize vector electronystagmography tests in the elderly participants in a service station of the Brazilian public health system, the Unified Health System (SUS) in the city of Natal, who presented with dizziness. Methods A cross-sectional and clinical study to characterize the vector electronystagmography in 10 members of an elderly SUS referral center located in the city of Natal with dizziness. Results Of 10 patients interviewed, 9 were women, and just 1 was a man. All complained of dizziness in the last year, and most of them had more than one type of dizziness. The main differential diagnosis was benign paroxysmal positional vertigo. Regarding vector electronystagmography, we found 20% of patients with semispontaneous nystagmus in at least one direction and changes in saccades latency in 100% of patients, with 20% in relation to the accuracy of pendular tracking changes at the frequency of 0.2 Hz, 10% at 0.4 Hz, and 80% at 0.8 Hz, and 10% change in optokinetic gain. No patient had altered caloric test. Conclusion Vector electronystagmography alterations in the elderly do not always indicate disease.

Introduction Vestibular dysfunction, or vestibulopathy, comprises disorders of the vestibular system in the peripheral (labyrinth or cochlear nerve) and/or central (nuclei, pathways, and central nervous system interrelationships). When the condition is determined by impairment of the inner ear (labyrinth), it is called labyrinthopathy.1

received January 10, 2013 accepted June 25, 2013

Among the elderly with chronic vestibular disease, 53.3% fall every year,2 and vertigo is the most common cause, occurring in 25% of episodes.3 It is important to remember that polypharmacotherapy, for various pathologies, also can cause falls among the elderly, either by side effects of drugs (hypotension, sedation, confusion, altered motor reflexes) or by its associations.4

Copyright © 2013 by Thieme Publicações DOI http://dx.doi.org/ Ltda, Rio de Janeiro, Brazil 10.1055/s-0033-1353139. ISSN 1809-9777.


Vector Electronystagmography Analysis in Elderly Individuals Being multifactorial and closely related to systemic changes and to the patient’s habits (drinking, smoking, stimulant use, or hyperkalemic diet), vestibulopathy is difficult to diagnose; it is often diagnosed by exclusion, and therefore treatment is difficult. Accurate diagnosis is not always achieved, and only the syndromic diagnosis will guide clinical practice. Clinically, there are several ways to detect the imbalance and seek its causes to prevent complications such as falls. Vertigo is evaluated by anamnesis and otoneurologic clinical exam (with evaluation of the cranial nerves, cerebellum, and static and dynamic balance), laboratory tests (such as biochemical tests), audiological tests (audiometry, immittance, otoacoustic emissions, and brainstem evoked potentials), vestibular auxiliary exam (vector electronystagmography), and, if necessary, imaging studies (computed tomography and magnetic resonance image). It should be remembered that, depending on the patient’s comorbidities, other laboratory tests are needed for diagnosis and/or monitoring of base pathologies. The vector electronystagmography is a widely used exam in clinical practice; however, it has limitations related to the health condition of the patient (patients too debilitated may not undergo the test), especially the elderly, with their fragility and comorbidities. Moreover, the geriatric patient often presents degeneration of the vestibular, oculomotor, or proprioceptive systems, which generate abnormal results in vector electronystagmography but which in reality do not reflect the pathologic condition but physiology. The objective of this study is to characterize vector electronystagmography exams in elderly participants from a service station of the Brazilian public health system, the Unified Health System (SUS) in the city of Natal, who complain of dizziness.

Methods A sectional study was conducted to characterize the vector electronystagmography of 10 elderly patients with dizziness. These patients are participants at a SUS referral center located in the city of Natal. The subjects were chosen at random when questioned for the occurrence of dizziness in the last year. Subjects 60 years or older, with good cognitive level, able to walk without walking aids, and with dizziness complaint in the last year were included. Subjects with decompensated diseases and those who did not have exams by the scheduled date were excluded. Patients underwent a clinical history and otoneurologic physical examination and then had the vector electronystagmography test. Equipment used for vector electronystagmography included the computed electronystagmography system and an otoneurologic stimulator e-96 (Contronic, Brazil). The steps of the exam were performed according to criteria proposed by Mangabeira-Albernaz et al5: calibration of ocular movements, spontaneous and semispontaneous nystagmus registration, analysis of saccadic movements, analysis of pendular tracking, optokinetic gain, and analysis of preand post–caloric test (with water) nystagmus.

Ferreira et al.

Dizziness was classified according to the classification of Drachman and Hart.6 Physical examination consisted of otoscopy, evaluation tests of static balance (Romberg and Romberg-Barrè test), dynamic balance (proof of Fukuda and Babinski-Weil), evaluation tests of coordination (finger-nose and finger-nose-finger tests and diadochokinesis), evaluation of spontaneous and semispontaneous nystagmus, evaluation of vestibulo-ocular reflex, dynamic visual acuity, head shake, head impulse, vibration-induced nystagmus, roll test, and Dix-Hallpike maneuver. We also evaluated the cervical region through physiotherapy maneuvers to assess cervical causes for the dizziness. The etiologic diagnosis was made based on this physical examination and blood pressure control, along with the history of comorbidities and medications used. The study was approved by the Ethics Committee under number 309/2012 of the institution, and all patients signed an informed consent form prior to data collection. It was made a descriptive statistics for data analysis.

Results Of the 10 patients interviewed, 9 were women and only 1 was a man. All complained of dizziness in the last year, and most of them had more than one type of dizziness (►Table 1). The most common etiologic diagnosis of dizziness was benign paroxysmal positional vertigo (BPPV; ►Table 2). Regarding vector electronystagmography, we found that changes in saccades and pendular tracking were the most common findings, and no patient had abnormal caloric proof (►Table 3).

Discussion The study group consisted of elderly patients with dizziness in the last year, not necessarily vertigo, so we can conclude that not all had labyrinthopathy. This became clear when considering the types of dizziness: several patients had presyncope, floating sensation, and imbalance, which refer, in most cases, to causes of dizziness not associated to the labyrinth. However, in general, vertigo was the most frequent type, alone or combined. This finding is confirmed by the literature,

Table 1 Classification of types of dizziness in elderly individuals Type of dizziness

Number of patients

Vertigo

1

Disequilibrium

2

Presyncope

1

Floating sensation þ presyncope

1

Vertigo þ disequilibrium

2

Vertigo þ floating sensation

1

Vertigo þ floating sensation þ presyncope

1

Vertigo þ disequilibrium þ presyncope

1

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Vector Electronystagmography Analysis in Elderly Individuals Table 2 Etiologic diagnosis of dizziness in elderly individuals Diagnosis

Number of patients

BPPV

6

Cervical syndrome

2

Cardiovascular alterations

2

Abbreviation: BPPV, benign paroxysmal positional vertigo.

Table 3 Findings in the vector electronystagmography exams of elderly individuals Semispontaneous nystagmus in at least one direction: 20% of the patients Changes in saccadic movements: 100% of the patients regarding latency, 20% in relation to the precision Changes in pendular tracking: 20% in the frequency of 0.2 Hz; 10% in the frequency of 0.4 Hz; 80% in the frequency of 0.8 Hz Changes in optokinetic gain: 10% of the patients Changes in caloric proof: 0

in which vertigo is identified as corresponding to 60.2% of cases.7 When evaluating the possible causes of dizziness, four patients had nonvestibular causes (two had cervical problems and two, cardiovascular) and most had BPPV, diagnosed by the Dix-Hallpike maneuver. This is a pathology of the labyrinth, with involvement of posterior semicircular canal in most cases. The vector electronystagmography is a diagnostic test of vestibular disorders; its main stage is the caloric test, which diagnoses lesions of the two labyrinths separately. However, this test is sensitive to changes of the lateral semicircular canals only. According to our findings, not all patients with BPPV will show changes in the vector electronystagmography, because the topography of the lesion is in different locations from the initial search. In this study, no patient had abnormal caloric test because we obtained nonvestibular disorders as causes of dizziness, and the only vestibular cause chosen was BPPV, which shows no change in this examination. Felipe et al found normal caloric tests in 72.5% of their elderly patients.7 This is reflected in typical changes of electronystagmography in elderly patients, which occur due to degeneration of

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the physiologic systems—in other words, caused by oculovestibular and neurologic age-related modifications and reduction of response time and reflexes. As such, the results of this study can be generalized to elderly without vestibular disorders. Gushikem et al in 2003 found abnormal tests in 68% of an elderly group without vestibular disease.8 In the sample of this study, 100% had abnormal tests, but it is noteworthy that it was a reduced sample. The change in saccades latency in 100% of the elderly justifies these changes, and it is due to the slowness of oculomotor responses, not exactly to vestibular disorders. The micrograph was not found in any examination, although this is described in the literature as a common finding in elderly.8

Conclusion Most of the evaluated patients had BPPV as etiologic diagnosis for their dizziness, and the most prevalent alteration observed in the vector electronystagmography was the change in latency of saccades (in 100% of the tests). No patient had altered caloric proof.

References 1 Ganança MM, Caovilla HH. Desequilíbrio e reequilíbrio.

2

3

4 5

6 7

8

In: Ganança MM, ed. Vertigem tem cura? O que aprendemos nestes últimos 30 anos. São Paulo, Brazil: Lemos Editorial; 1998:13–19 Gazzola JM, Ganança FF, Aratani MC, Perracini MR, Ganança MM. Clinical evaluation of elderly people with chronic vestibular disorder. Braz J Otorhinolaryngol 2006;72:515–522 Gazzola JM, Ganança FF, Aratani MC, Perracini MR, Ganança MM. Circumstances and consequences of falls in elderly people with vestibular disorder. Braz J Otorhinolaryngol 2006; 72:388–392 Fabricio SCC, Rodrigues RAP, Costa Júnior ML. Quedas acidentais em idosos institucionalizados. Acta Paul Enf 2002;15:51–59 Mangabeira-Albernaz PL, Ganança MM, Pontes PAL. Modelo operacional do aparelho vestibular. In: Mangabeira-Albernaz PL, Ganança MM, eds. Vertigem. 2nd ed. São Paulo, Brazil: Moderna; 1976:29–36 Drachman DA, Hart CW. An approach to the dizzy patient. Neurology 1972;22:323–334 Felipe L, Cunha LCM, Cunha FCM, Cintra MTG, Goncalves DU. Presbivertigem como causa de tontura no idoso. Pró Fono R Atual Cient 2008;20:99–104 Gushikem P, Caovilla HH, Ganança MM. Avaliação otoneurológica em idosos com tontura. Acta ORL 2003;21:1–7


Original Article

Characterization of Swallowing Sounds with the Use of Sonar Doppler in Full-Term and Preterm Newborns Hellen Nataly Correia Lagos1,2 Rosane Sampaio Santos1,3 Liliane Friedrich Gallinea2,5 Mariangela Langone2 1 Department of Internal Medicine, Federal University of Paraná

(UFPR), Curitiba/PR, Brazil 2 Department of Orofacial Motricity: Dysphagia, Scope Hospital, University Tuiuti of Paraná (UTP), Curitiba/PR, Brazil 3 Department of Speech Pathology, University Tuiuti of Paraná (UTP), Curitiba/PR, Brazil 4 Department of Speech Therapy, Pathology Specialist Hospital Speech, The Pontifical Catholic University of Paraná (PUC), Curitiba/ PR, Brazil 5 Department of Child and Adolescent, Federal University of Paraná (UFPR), Curitiba/PR, Brazil

Edna Marcia da Silva Abdulmassih1,4

Address for correspondence Hellen Nataly Correia Lagos, MSc, Rua Santa Catarina, 427, ap24, Bairro Água Verde, Curitiba, Paraná, Brazil (e-mail: hellennataly@gmail.com).

Int Arch Otorhinolaryngol 2013;17:383–386.

Abstract

Keywords

► ► ► ►

deglutition infant newborn Doppler effect

received April 22, 2013 accepted June 11, 2013

Introduction Technological advances have provided a large variety of instruments to view the swallowing event, aiding in the evaluation, diagnosis, and monitoring of disturbances. These advances include electromyography of the surface, dynamic video fluoroscopy, and most recently sonar Doppler. Objective To characterize swallowing sounds in typical children through the use of sonar Doppler. Method Thirty newborns participated in this prospective study. All newborns received breast milk through either their mother’s breasts or bottles during data collection. The newborns were placed in either right lateral or left lateral positions when given breast milk through their mother’s breasts and in a sitting position when given a bottle. There were five variables measured: initial frequency of sound wave (FoI), frequency of the first peak of the sound wave (FoP1), frequency of the second peak of the sound wave (FoP2), initial intensity and final sound wave (II and IF), and swallowing length (T), the time elapsed from the beginning until the end of the analyzed acoustic signal measured by the audio signal, in seconds. Results The values obtained in the initial frequency of the babies had a mean of 850 Hz. In terms of frequency of first peak, only three presented with a subtle peak, which was due to the elevated larynx position. Conclusion The use of sonar Doppler as a complementary exam for clinical evaluations is of upmost importance because it is nonintrusive and painless, and it is not necessary to place patients in a special room or expose them to radiation.

Copyright © 2013 by Thieme Publicações DOI http://dx.doi.org/ Ltda, Rio de Janeiro, Brazil 10.1055/s-0033-1353369. ISSN 1809-9777.

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Introduction

Methods

Eating is a vital function in any age and especially for a newborn. Eating orally for newborns is a complex activity that requires coordination of sucking, breathing, and swallowing functions so that it does not risk aspiration.1 Efficient and secure eating requires not only sucking, but also coordination in breathing with sucking and swallowing. This involves a combination of functions from the lips, jaw, tongue, pharynx, larynx, and esophagus.2 Therefore the coordination of sucking, swallowing, and breathing cannot be completely acquired in premature babies when oral eating is introduced.3 Premature babies have a high risk of eating difficulties. Their neurologic immaturity, abnormal muscular tone, disabled oral reflexes, general weakness, and difficulties in autoregulation could lower the quality of their oral motor abilities, interfering in the synchronization of sucking, swallowing, and breathing, which is a primary oral motor mechanism, and the amount ingested.4,5 Technological advances have provided a large variety of instruments to view the swallowing event, aiding in the evaluation, diagnosis, and monitoring of disturbances. These advances include electromyography of the surface,6 sonograms of swallowing action,7 swallowing endoscopies, dynamic video fluoroscopy of swallowing,8,9 virtual endoscopy through a computerized tomography with reconstructive software,10 cervical auscultation through a microphone and accelerometer,11 and most recently sonar Doppler.12–15 These procedures are not invasive and are used to contribute to the evaluations of swallowing. Experiments with analytical computations of swallowing sounds have been made, and these studies demonstrate the usefulness of digital sounds of swallowing for duration measurements and the amplitude of sound waves.13,16,17 The technical development of sonar Doppler as a method for evaluating swallowing makes comprehension of the findings in swallowing disorders necessary. Studies on the use of sonar Doppler in evaluating swallowing up to now show normal swallowing sound waves in all age groups without pointing to findings referring to swallowing disturbances.12–15 Sonar Doppler of the larynx is a noninvasive technique that causes no pain or exposure to radiation. There is no need for sedatives, and it is quick and easy to use in infants. Results can be recorded with sound analysis software. The equipment is of good quality and has a widespread availability—it is available in most hospitals and clinics. There is no need for further investment in new equipment because the equipment already exists for use in other parts of the body. The equipment is also easy to transport. Sonar Doppler does not substitute for video fluoroscopy and video endoscopy of swallowing, but it could help in the evaluation of functional swallowing and in the monitoring of treatment as a biofeedback, in which muscular activity can be converted into an audio stimulus, visual stimulus, or both. The objective of this study was to characterize swallowing sounds in typical children using sonar Doppler.

The present observational study occurred March 2010 through October 2011. The study was approved by the Ethics and Research Committee through the registration of CEP: 2099.266/2009-11, on March 12, 2010. Participants included 30 premature newborns. Guardians were informed of the study and were solicited for the authorization of participation of newborn babies. Once they accepted, they were asked to sign terms of agreement and authorization. Criteria for inclusion in study was premature newborn of either sex, born in a public institution in Curitiba city, with adequate sucking and swallowing, substantiated through previous speech evaluations. Babies who showed breathing difficulties in coordination of breathing and swallowing or any other structural and/or functional alterations that could interfere with swallowing were excluded. Newborns with any diagnosis of neurologic problems or associated syndromes and high-risk newborns with pathologies that could complicate alterations in the stomatognathic system were also excluded. To record the sound, a portal ultrasonic detector was used (model DF-4001, Martec brand), with a plain disc transducer, of only one crystal, providing interface to the sonar Doppler. The ultrasound frequency was 2.5 Hz, with output 10 mW/ cm2 and an output power of 1 W sound. The Doppler equipment was coupled with a standard IBM microcomputer, with a Pentium 200-MHz processor, 32 MB of RAM, video plate with high color support (16 bits; 800  600), CD-ROM drive of 8  , sound plate, and speakers. The operating system was Windows 98 and VOX metria Software was used.18 All newborns received breast milk through either their mother’s breasts or bottles during data collection. The newborns were placed in either right lateral or left lateral positions when given breast milk through their mother’s breasts and in a sitting position when given a bottle. As an indicator for the best location of the positioning of the transducer, a presentation by Takahashi et al was used,19 which showed the best adaptation of the transducer and the best capture of sonorous signal, location 11, above the lateral region of the trachea, inferior to the cricoids cartilage. The transducer was adjusted onto the neck using a gel contact to facilitate the contact of the transducer and skin. The transducer’s bundle was positioned to form an angle of 30 to 60 degrees. Five variables were measured: initial frequency of sound wave (FoI), frequency of the first peak of the sound wave (FoP1), frequency of the second peak of the sound wave (FoP2), initial intensity and final sound wave (II and IF), and swallowing length (T), the time elapsed from the beginning until the end of the analyzed acoustic signal measured by the audio signal, in seconds.

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Results Of the babies evaluated, 50% were male and 50% were females; they all were 48 hours old.


Characterization of Swallowing Sounds Table 1 Mean initial frequency (Hz) in newborns Consistency

Newborns

Liquid

850

Table 2 Mean frequency of first peak in newborns Consistency

Newborns

Liquid





Not obtained peak value, was a subtle peak.

Table 3 Mean frequency (Hz) of the second peak in newborns Consistency

Newborns

Liquid

1087

The values obtained in the initial frequency had a mean of 850 Hz as shown in ►Table 1. In terms of frequency of first peak, only three presented with a subtle peak, due to the elevated larynx position (►Table 2). ►Table 3 shows the frequency of the second peak standards found in newborns. The mean of the initial intensity of adults and newborns are shown in ►Table 4. Finally, ►Table 5 shows swallowing time in the group studied.

Discussion Technological advances have brought about instruments with great variability in applications, aiding in the search of evaluations, diagnostics, and monitoring of swallowing disorders. Tools that detect with precision alterations in the swallowing process are important not only scientifically but also clinically. In the research field, these tools validate objective data by greatly reducing subjectivity in the individuals doing the evaluations. In clinics, they could facilitate decisions made by therapists, indicating which therapeutic maneuvers should be used or which eating consistency is the safest to digest.17

Table 4 Mean of initial and final intensity (dB) in newborns Intensity

Newborns

Adults

Initial

72

51

Final

91

91

Table 5 Mean swallowing time (s) in newborns

According to some authors,20 the mean initial frequency of the swallowing sound in adults is FoI 567 Hz; in the present study it was observed that in newborns the mean found was FoI 850 Hz, showing a more acute frequency compared with adults. Findings in first peak in adults are characterized by a weak signal associated with laryngeal lift, and the passage of cake through the pharynx21 was FoP1 905 Hz; of the 30 newborns analyzed only three presented with sufficient initial peak. This can be explained through the elevated position of the larynx. Frequency of the second peak represents a strong sound associated with the opening of the cricopharyngeal21; in adults, the mean is FoP2 1.087 Hz, very close frequencies between the two peaks. The mean of initial and final intensities found in adults was, respectively, 51 dB and 91 dB; in newborns the mean was 72 dB and 91 dB. Finally, the times found in swallowing standards in adults were T1: 1.33 seconds; T2: 1.67 seconds; T3: 1.85 seconds; and in newborns they were T1: 0.53 seconds, T2: 1.04 seconds, T3: 2.08 seconds. As found in these data, sonar Doppler can be used as a method of evaluation for swallowing, giving more support during evaluations, treatment, and diagnosis of a dysphasic patient.

Conclusion The use of sonar Doppler as a complementary exam for clinical evaluations is of upmost importance because it is nonintrusive and painless, and it is not necessary to place patients in a special room or expose them to radiation. Not only is it unnecessary to sedate the patient, the time of use is short, making it easy to use in infants. The findings could be recorded in analytic sound software, linked in any portal computer, using portal, cost-effective equipment.

References 1 Silva-Munhoz LF, Bühler KEB. Achados fluoroscópicos da degluti-

2

3

4

5

Time

Newborns

T1

0.53

T2

1.04

T3

2.08

Lagos et al.

6

7

ção: comparação entre recém-nascidos pré-termo e recém-nascidos de termo. Jornal Soc Bras Fonoaudiol 2011;23:206–213 Mizuno K, Ueda A. The maturation and coordination of sucking, swallowing, and respiration in preterm infants. J Pediatr 2003; 142:36–40 Quintella T, Silva AA, Botelho MIMR. Distúrbio da deglutição (e aspiração) na infância. In: Furkim AM, Santini CS, eds. Disfagias Orofaríngeas. Carapicuíba, Brazil: Pró-Fono; 1999:61–96 Morris SE, Klein MD. Pre-Feeding Skills: A Comprehensive Resource for Feeding Development. Tucson, AZ: Therapy Skill Builders; 1987 Silva RNM. Efeitos da Sucção não-nutritiva no Prematuro. In: Lopes SMB, Lopes JM. Follow up do recém-nascido de Alto Risco. Rio de Janeiro, Brazil: Medsi; 1999:309–316 Crary MA, Baldwin BO. Surface eletromyographic characteristics of swallowing in dysphagia secondary to brainstem stroke. Dysphagia 1997;12:189–197 Brown PB, Sonies BC. Diagnostic methods to evaluate swallowing other than barium contrast. In: Perlman AL, Schulze-Delrie K, eds. International Archives of Otorhinolaryngology

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8

9

10 11 12 13

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Deglution and Its Disorders. San Diego, CA: Singular Publishing Group; 1997:227–253 Macedo-Filho ED. Estudo comparativo entre a Videoendoscopia da Deglutição (VED) e a Videofluoroscopia (VFC) na Avaliação da Disfagia Orofaríngea Pós-Acidente Vascular Cerebral (AVC) [Tese de doutorado]. Curitiba, Brazil: Universidade Federal do Paraná; 2001 Hartnick CJ, Hartley BE, Miller C, Willging JP. Pediatric fiberoptic endoscopic evaluation of swallowing. Ann Otol Rhinol Laryngol 2000;109:996–999 Burke AJ, et al. Evaluation of airway obstruction using virtual endoscopy. Laryngoscope 2000;1110:23–29 McKaig TN. Ausculta: Cervical e Torácica. In: Furkin AM, Santini CS, eds. São Paulo, Brazil: Pro-Fono; 1999:171–187 Santos RS, Filho EDM. Sonar Doppler as an instrument of deglutition evaluation. Int Arch Otorhinolaryngol 2006;10(3):182–191 Bernardes TG, Jukiweski A, Santos RS. Uso do sonar doppler como biofeedback da deglutição em pacientes com doença de Parkinson [Dissertação de mestrado]. Curitiba, Brazil: Universidade Tuiuti do Paraná; 2008

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14 Cagliari CF, Jurkiewicz AL, Santos RS, Marques JM. Doppler sonar

15

16 17 18 19 20 21

analysis of swallowing sounds in normal pediatric individuals. Braz J Otorhinolaryngol 2009;75:706–715 Sória FS. Padrão de Normalidade da Deglutição em Idoso através do Sonar Doppler [Dissertação de mestrado]. Curitiba, Brazil: Universidade Tuiuti do Paraná; 2010 Youmans SR, Stierwalt JAG. An acoustic profile of normal swallowing. Dysphagia 2005;20:195–209 Spadotto AA. Análise Quantitativa do Sinal da Deglutição. [Tese de doutorado]. São Carlos, Brazil: Universidade de São Paulo; 2009 Behlau M. VoxMetria: Software para Análise de Voz e Qualidade Vocal. São Paulo: CTS Informática; 2003 Takahashi K, Groher ME, Michi K. Methodology for detecting swallowing sounds. Dysphagia 1994;9:54–62 Cichero JAY, Murdoch BE. Detection of swallowing sounds: methodology revisited. Dysphagia 2002;17:40–49 Hamlet SL, Patterson RL, Fleming SM, Jones LA. Sounds of swallowing following total laryngectomy. Dysphagia 1992;7: 160–165


Original Article

Are Histologic Studies of Adenotonsillectomy Really Necessary? Giseli Rebechi1 Thiago Euênio Pontes1 Cícero Matsuyama1

Elias Lobo Braga1

1 Setor de Otorrinolaringologia, Instituto CEMA, São Paulo/SP, Brasil

Int Arch Otorhinolaryngol 2013;17:387–389.

Abstract

Keywords

► tonsillar neoplasms ► tonsillectomy ► adenoidectomy

Fernando Rebechi1

Address for correspondence Giseli Rebechi, Avenida Indianópolis, 740, Bairro, Moema, CEP 04062-001, São Paulo/SP, Brasil (e-mail: girebechi@gmail.com).

Introduction In most ear, nose, and throat services, it is routine to send the material extracted from tonsillectomy for histologic study to research malignancy, to analyze suspect material, or to provide medical-legal documentation. Recent studies have shown that this routine analysis is dispensable. Objective To evaluate the actual need and perform a cost–benefit analysis of routine histopathologic examination in tonsillectomy with no signs or symptoms of malignancy. Methods A retrospective observational study evaluated the charts of patients undergoing adenotonsillectomy, tonsillectomy, or adenoidectomy from January 2008 to September 2009 at the Institute of Otorhinolaryngology CEMA-SP. Costs of this test for the public health system were analyzed and the literature reviewed. Results We studied 281 patients between 2 and 22 years of age; 142 (50.5%) were male and 139 (49.5%) were female. Of the surgeries, 201 were adenotonsillectomies (71.5%), 41 were tonsillectomies (14.5%), and 39 were adenoidectomies (14%). The most common indication for surgery was recurrent infection (63.3%). None of study patients had clinical suspicion of malignancy. The tests showed a cost of R$20.03 per tonsil analyzed. Conclusion Routine histopathologic examination in patients undergoing adenotonsillectomy with no signs or symptoms of malignancy is dispensable and increases the cost of the surgeries.

Introduction Adenotonsillectomy is surgery to remove the tonsils and adenoids, and it is one of the most common and most frequently performed surgical procedures in the world.1,2 Its different indications can be divided into therapeutic, diagnostic, as well as access to other surgeries. Sleep apnea syndrome, hypertrophic tonsils and adenoids, chronic tonsillitis, halitosis, and suspicion of malignancy are indications for adenotonsillectomy, but the most frequent indication is recurrent tonsillitis. Most otolaryngology services routinely send adenotonsillectomy specimens for histopathologic examination, whether

received June 8, 2013 accepted July 11, 2013

Willian Maduel Matos1

for malignancy investigation, analysis of suspect material, or medicolegal documentation of surgical removal.1 Recent studies have shown that routine histopathologic analysis of the tonsil is dispensable, because they have a very low probability of diagnosing occult malignancies. Unfortunately, this risk is still not zero, so the need for routine histopathology is still controversial.

Objective Define the real need for routine histopathologic examination of adenotonsillectomy specimens and perform a cost–benefit analysis of its use in patients without risk factors for malignancy.

Copyright © 2013 by Thieme Publicações DOI http://dx.doi.org/ Ltda, Rio de Janeiro, Brazil 10.1055/s-0033-1353441. ISSN 1809-9777.

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Materials and Methods

Table 1 Results of histopathologic examination of the tonsils

This retrospective observational study evaluated the records of all patients who had adenotonsillectomy, tonsillectomy, or adenoidectomy from January 2008 to September 2009 at the CEMA Institute of Otorhinolaryngology, São Paulo, Brazil. This project was approved by the Ethics and Research Institution under the protocol 17.205/2009. Patients or their guardians signed an informed consent form, shown in Appendix 1. Data analysis was performed using descriptive statistics, and the results are presented in absolute numbers. We excluded patients with malignancy symptoms and signs. All patients underwent general anesthesia and tonsillectomy by extraction dissection technique. The pharyngeal tonsils were removed with a Beckmann curette. Specimens were immediately placed in sterile glass with 10% formalin and sent for histologic analysis. The tonsils were fixed in 10% formalin and embedded in paraffin, and sections were stained with hematoxylin-eosin. We studied the results of the histopathologic examinations, regardless of age, sex, and indication for surgery. We also analyzed the cost of this test for the public health system, and we reviewed the literature.

Results A total of 281 patients were recruited, between 2 and 22 years old. Of them, 142 (50.5%) were male and 139 (49.5%) were female. Most patients had tonsil hypertrophy grade III according to the Brodsky classification and pharyngeal tonsil hypertrophy documented by nasofibrolaryngoscopy. Of the surgeries, 201 were adenotonsillectomies (71.5%), 41 were tonsillectomies (14.5%), and 39 were adenoidectomies (14%). The most common surgical indications were recurrent tonsillitis (63.3%) and obstructive sleep apnea syndrome (38.7%). None of the patients in our study had clinical suspicion of malignancy. Results of the pathologic examinations of tonsils and adenoids are shown in ►Tables 1 and 2. The tests cost R$20.03 per piece analyzed; the adenotonsillectomy surgery has three pieces, reaching a total of R$60.09 per surgery.

Discussion Tonsils with malignant processes present different aspects than benign lesions on clinical examination; physical examination and medical history can be important guides. These lesions may start with nonspecific symptoms and insidious symptoms, and they are usually diagnosed in patients over 60 years old. Smoking and alcoholism are risk factors. According Beaty et al,3 risk factors for tonsillar malignancy include history of head and neck cancer, tonsillar asymmetry, visible lesion, ulcerated or hard consistency on palpation of the tonsil, unexplained weight loss or constitutional symptoms, and cervical lymphadenopathy. Usually the patient starts with dysphagia without signs of acute infection with ipsilateral otalgia, difficulty in mobility of the tongue, nasal voice, halitosis, and nasal reflux. Symptoms such as changes in tone of voice, drooling, bloody saliva, and trismus indicate deep infiltration of the tumor. International Archives of Otorhinolaryngology

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Exam result

Number of tonsils

Percentage

CT with LH

282

58.3

CT with LH and suppurative foci

96

19.9

CT with LH with areas of surface erosion

44

9

CT with LH with colonies of actinomyces

46

9.6

CT with LH and submucous fibrosis

2

0.4

CT with recent hemorrhage

4

0.8

Nonspecific CT

6

1.2

Papilloma squamous cells

4

0.8

Total

484

100

Abbreviations: CT, chronic tonsillitis; LH, lymphatic hyperplasia.

Table 2 Results of histopathologic examination of the pharyngeal tonsils Exam result

Number of tonsils

Percentage

Luschka pharyngeal adenoid hypertrophy

237

98.75

Lymphoid tissue of reactive pattern

2

0.84

Malpighian mucosa with vascular ectasia

1

0.41

Total

240

100

Many authors have reported that tests with positive results for malignancy had a suspected diagnosis before surgery. The result of histopathologic exams on tonsil specimens correlates well with preoperative clinical impressions, and their findings rarely change the management of the patient.4 Randall et al reported a prevalence of malignancy of 0.087% in routine examinations. In these patients, 88% had preoperative suspicion of malignancy. Among the tests, 0.011% had a positive result but no risk factor; the authors concluded that routine examination was unnecessary if there was no suspicion of malignancy.1 Garavello et al noted a 0.18% incidence of positive histopathologic analysis without clinical suspicion in children, concluding that routine examinations were unnecessary.5 Felix et al found a 0.19% incidence of positivity; however, all of the patients had some risk factor for tonsillar malignancy. The examinations did not locate any hidden malignancies.6 Williams and Brown found 4,070 tonsils with malignancy on histopathology, and all of them had been diagnosed during preoperative evaluations.7 A study by DellAringa et al showed no malignancy in the patients analyzed, finding a negative cost–benefit ratio for routine histopathologic exams.8 Younis et al presented


Are Histologic Studies of Adenotonsillectomy Really Necessary? research that showed none of the 2,099 pediatric patients undergoing tonsillectomy had malignancy found on histopathologic exam, but this incidence differed from the adult population.9 Mohamad et al also found in their study that routine examination is not necessary in the pediatric population.10 Many authors have showed that histopathologic exam of specimens from children is superfluous11 and that increasing age is a risk factor to be considered.12 This fact is due to very different indications for tonsillectomy in different age groups; in adults, the incidence of excisional biopsies and symptoms of malignancy are much higher than in pediatric patients. Spending with microscopic examinations by tonsil varies greatly in the studies, ranging from US $12.85 to US $90.00. Annual spending in the United States is approximately US $35,467,080.00. For the Brazilian government, spending on each piece is R$20.03.

References 1 Randall DA, Martin PJ, Thompson LDR. Routine histologic exami-

2 3 4

5

6

7

8

Conclusion Routine histopathologic examination in pediatric adenotonsillectomy specimens is dispensable, with a negative cost–benefit ratio. Despite all these studies showing that such tests burden the public purse as well as private health systems, surgeons are required to apply for the pathologic study because health plans require its result for the payment of the surgery. We emphasize the importance of a thorough history and clinical examination in all patients undergoing adenotonsillectomy. In children, the chance of occult malignancy is very low. In patients with risk factor present in clinical ear, nose, and throat examination, this exam is indispensable. With more careful preoperative assessment, we could save millions of dollars per year.

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9

10

11

12

nation is unnecessary for tonsillectomy or adenoidectomy. Laryngoscope 2007;117:1600–1604 Cinar F. Significance of asymptomatic tonsil asymmetry. Otolaryngol Head Neck Surg 2004;131:101–103 Beaty MM, Funk GF, Karnell LH, et al. Risk factors for malignancy in adult tonsils. Head Neck 1998;20:399–403 Ikram M, Khan MAA, Ahmed M, Siddiqui T, Mian MY. The histopathology of routine tonsillectomy specimens: results of a study and review of literature. Ear Nose Throat J 2000;79:880–882 Garavello W, Romagnoli M, Sordo L, Spreafico R, Gaini RM. Incidence of unexpected malignancies in routine tonsillectomy specimens in children. Laryngoscope 2004;114:1103–1105 Felippe F, Gomes GA, de Souza BP, Cardoso GA, Tomita S. Evaluation of the utility of histopathologic exam as a routine in tonsillectomies. Braz J Otorhinolaryngol 2006;72:252–255 Williams MD, Brown HM. The adequacy of gross pathological examination of routine tonsils and adenoids in patients 21 years old and younger. Hum Pathol 2003;34:1053–1057 Dell’Aringa AR, Juares AJ, Melo Cd, Nardi JC, Kobari K, Perches Filho RM. Histological analysis of tonsillectomy and adenoidectomy specimens–January 2001 to May 2003. Braz J Otorhinolaryngol 2005;71:18–22 Younis RT, Hesse SV, Anand VK. Evaluation of the utility and costeffectiveness of obtaining histopathologic diagnosis on all routine tonsillectomy specimens. Laryngoscope 2001;111:2166–2169 Mohamad I, Hassan S, Salim R. The routine histopathological examination of tonsillectomy specimens at hospital universiti sains Malaysia—retrospective study and its implication. Malays J Med Sci 2007;14:19–21 Strong EB, Rubinstein B, Senders CW. Pathologic analysis of routine tonsillectomy and adenoidectomy specimens. Otolaryngol Head Neck Surg 2001;125:473–477 Erdag TK, Ecevit MC, Guneri EA, Dogan E, Ikiz AO, Sutay S. Pathologic evaluation of routine tonsillectomy and adenoidectomy specimens in the pediatric population: is it really necessary? Int J Pediatr Otorhinolaryngol 2005;69:1321–1325

Appendix 1 Model of the term of consent CEMA Institute (Department of Otorhinolaryngology) Term of consent We invite the Sr _______________________________________ to participate in the survey “Are histologic studies of adenotonsillectomy really necessary?” under the responsibility of the researcher Dra Giseli Rebechi, to analyze the need for routine histopathologic studies on the products of adenotonsillectomy in patients without risk factors for malignancies and to analyze the costs and benefits of the studies. Your participation is voluntary and will be through the authorization and release of medical records. If you agree to participate, you will contribute to analysis of the real need for routine checkups and lower expenditures of public and private health care. If after consenting to participation you decide to stop participating, you have the right and freedom to withdraw consent at any stage of the research, either before or after the collection of data, regardless of the reason, and without any prejudice. You will not have expense and you will not receive any remuneration. The search results will be displayed, but your identity will not be disclosed and will be kept secret. For any other information, you can may contact the researcher at the address Indianapolis Avenue, 740, Moema, São Paulo/SP, Brazil, by phone (11) 5082 3420, or you may contact the Committee on Ethics in Research-Institute of Otorhinolaryngology CEMA, Pascoal Moreira street, 450, Mooca, São Paulo/SP, Brazil, phone (11) 2602 4000. Informed consent I, ___________________________________________________________, was informed about what the researcher wants to do and why she need my collaboration, and I understand the explanation. Therefore, I agree to participate in the project, knowing that I will not gain anything and I can leave whenever I want. This document is issued in two copies to be signed both by me and the researcher, providing a copy to each of us. _________________________________ Signature of Participant _________________________________ Signature of Researcher Principal

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Update Article

A Descriptive Epidemiology Study of Oral Cleft in Sergipe, Brazil Andrea Luiza1 Diego Noronha de Góis1 Jadson Alípio Santana de Sousa Santos1 Rosany Larissa Brito de Oliveira1 Luiz Carlos Ferreira da Silva1 1 Department of Dentistry, Federal University of Sergipe (UFS),

Aracaju/SE, Brazil

Address for correspondence Dr. Luiz Carlos Ferreira da Silva, PhD, Department of Dentistry, University Hospital, Claudio Batista, s/n–Sanitarium, Aracaju/SE 49060-100, Brazil (e-mail: lcsilva@ufs.br).

Int Arch Otorhinolaryngol 2013;17:390–394.

Abstract

Keywords

► cleft lip ► cleft palate ► epidemiology

Introduction The nonsyndromic orofacial cleft is the fourth most common birth defect, but in Brazil, data about the cleft epidemiology are not accurate. Objective This study aimed to describe the epidemiologic characteristics of oral cleft cases at Specialized Society Attending Cleft Patient in Sergipe State. Methods Data were obtained from patients’ medical records in relation to the following characteristics: age; gender; race; origin; cleft type; additional malformations and/or complications; prenatal accomplishment; treatment applied. For diagnosis analysis, it was noted if mothers had received prenatal care and if they had ultrasonography performed and if the cleft was viewed in it. Results We observed a prevalence of male gender (54%). Age between 0 and 4 years old was most prevalent (53%), and pheoderma race was observed in 47%. Transincisive foramen cleft was found in 52.3% of the individuals. The prevalence of pre- and transincisive foramen cleft was higher in men (66.3 and 55.7%), women accounted for 65.0% of postincisive foramen, and atypical facial cleft (0.3%) occurred in one case. Associated malformations and complications were present in 12% of patients. Prenatal care was reported by 48% of the mothers. Conclusion In this study male gender was the most affected, and 0 to 4 years was the most frequent age group. Transincisive foramen cleft type was most frequently encountered. Prenatal care was reported by most mothers. So, this study found that early treatment is a reality in SEAFESE (Service Specializing in Cleft Care of Sergipe), and consequently the chances of successful integration of the child to society will be better.

Introduction The nonsyndromic orofacial cleft is the fourth most common birth defect, besides being the most observed craniofacial malformation.1,2 Although its etiology still remains unclear, most likely both exogenous (teratogenic) and endogenous (genetic) factors contribute to this embryopathy in humans.3,4 In addition, it is a major oral health problem in the world and its treatment requires specialized multiprofessional and integrated actions.5

received October 22, 2012 accepted June 6, 2013

Several studies of the epidemiologic patterns of cleft deformities show a marked variability in the frequency of this anomaly, ranging from 1 in 500 to 1 in 2,500 live births.5 In Brazil, data about cleft lip and/or palate epidemiology are not accurate; however, the incidence of this malformation is 0.36 in 1,000 live births. In northeastern Brazil, the incidence is 0.39 in 1,000 live births.6,7 In Sergipe, approximately 22 new cases of cleft lip and/or palate occur each year.8 The SEAFESE (Service Specializing in Cleft Care of Sergipe) is a public interest civil social organization formed by a

Copyright © 2013 by Thieme Publicações DOI http://dx.doi.org/ Ltda, Rio de Janeiro, Brazil 10.1055/s-0033-1352502. ISSN 1809-9777.


Oral Cleft in Sergipe, Brazil Table 1 Surgical treatment protocol followed by SEAFESE Patient age

Treatment

0–3 mo

Initial assessment

3 mo

Cheiloplasty

12 mo

Palatoplasty

10–12 y

Secondary surgeries

12–16 y

Secondary surgeries (rhinoplasty)

>17 y

Orthognathic surgery

SEAFESE, Service Specializing in Cleft Care of Sergipe.

multidisciplinary team comprised of 13 professionals in the following specialties: plastic surgery, maxillofacial surgery, nurse, speech therapy, dentistry, orthodontics, psychology, social work, and anesthesiology. All surgeries performed within SEAFESE follow a protocol created by its professionals to obtain the best results for patients with cleft (►Table 1). According to that, the most detailed information about these birth defects in populations is essential to recognize and prevent the problem, and, consequently, to plan assistance policies in general. Thus, the aim of this study was to characterize the cases of cleft lip and palate based on the medical records of patients treated at the SEAFESE.

Methods For the purpose of this study, a descriptive cross-sectional design was adopted, based on medical records of patients treated at the SEAFESE. It included all the medical records of patients attended at SEAFESE from October 2003 to June 2007. Data not reported on medical record were considered as “not defined.” All medical records were analyzed, regarding (1) demographic characteristics (gender, race, age, origin); (2) cleft diagnosis and classification (cleft type, associated malformations and complications, prenatal accomplishment); and (3) treatment applied. The variable age was divided into 6 groups with an interval of 4 years for each group according to the classification made by the Brazilian Institute of Geography and Statistics, Population Census, 2000.9 Patients were classified according to race as leukoderma (white), pheoderma (brown), xanthoderma (yellow), and melanoderma (black); according to the origin, they were grouped based on where they live (metropolis, countryside, and other states). Cleft classification was based on the work of Spina et al,10 which defines four groups: preincisive foramen cleft (uni- or bilateral, median); transincisive foramen cleft (uni- or bilateral); postincisive foramen clefts; and atypical (rare) facial clefts. For diagnosis analysis, it was noted if mothers received prenatal care and if they had ultrasonography done and if the cleft was viewed in it. Regarding treatment, only surgeries to repair cleft lip and palate (lip repair and palatoplasty, respectively) were considered in this study; additional surgeries

Luiza et al.

performed in patients with cleft were included as “other surgeries.” The collected data were tabulated and calculated in Excel for Windows 2003, and the data are presented in absolute frequencies and percentages. This study was approved by the Committee on Ethics in Research Involving Humans, from Federal University of Sergipe (CEP-UFS/CAAE No.: 0082.0.107.000–07).

Results A total of 350 medical records were achieved. The general analysis of the data allows tracing back the profile of participants, as shown in ►Table 2. In relation to gender, 54.0% of patients who comprised the sample were male; the distribution of patients by age focused predominantly between 0 and 14 years (77.4%), with a higher prevalence in the age group of 0 to 4 years. Regarding race, 165 patients (47.2%) were pheoderma and 146 (41.7%) leukoderma. Finally, most of them were from the countryside (58.0%). ►Table 3 shows patient distribution according to cleft type and gender. The most frequent type of cleft was the transincisive foramen, with 183 cases (52.3%), and this cleft was more frequent in males, with 102 cases (55.7%). The second most frequent type of cleft was the preincisive foramen, with 89 cases (25.4); this cleft was also more frequent in males,

Table 2 Distribution of oral cleft cases by gender, age group and race n

%

Male

189

54.00

Female

161

46.00

<4

188

53.70

5–9

44

12.60

10–14

39

11.10

15–19

31

8.90

20–24

24

6.90

>25

24

6.90

Pheoderma

165

47.20

Leukoderma

146

41.70

Xanthoderma

0

0.00

Melanoderma

14

4.00

Not defined

25

7.10

Countryside

203

58.00

Metropolis

133

38.00

Other states

10

2.90

Not defined

4

1.10

Gender

Age group (y)

Race

Origin

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Table 3 Distribution of patients according to cleft type and gender Cleft type

Gender

Total (%)

Male

%

Female

%

Preincisive foramen

59

66.3

30

33.7

89 (25.4)

Postincisive foramen

27

35.0

50

65.0

77 (22.0)

Transincisive foramen

102

55.7

81

44.3

183 (52.3)

Atypical (rare) facial

01

100.0

00

0.0

01 (0.3)

with 59 cases (66.3%). The third most frequent type of cleft was the postincisive foramen, with 77 cases (22.0%), and this cleft occurred more often in females with 50 cases (65.0%). Finally, the atypical (rare) facial cleft was less frequent, with only 1 case (0.3%), a male. Among the 350 medical reports analyzed, only 41 (12.0%) showed data regarding malformations and additional complications in the patients, without specifying. Prenatal care was reported by almost half the sample (168; 48.0%), and only 26 mothers (7.0%) reported on prenatal care. In 156 records (45.0%), this information was missing. According to information provided by patients to fill the medical record, only in 7 cases (4.2%) of 168 referred to prenatal care did the ultrasonography reveal the cleft; in 107 cases (63.7%), nothing was detected, and in 54 (32.1%) there was no information (not defined). Associated malformations and complications occurred in 41 individuals, more frequently in men (26 subjects; 63.4%). The associated malformations and complications can be seen in ►Table 4. Since 2003, when the service was available, 173 surgeries were performed in patients with cleft (►Table 5): 77 cheiloplasty, 79 palatoplasty, and 17 other surgeries (fistuloplasty, rhinoplasty, otoplasty). The highest rate was recorded in 2004 (69 surgeries) and the lowest in 2007 (3 surgeries). This decrease in the number of surgeries was due to a change in SEAFESE head office. ►Table 6 shows the distribution of patients by age at initial assessment in SEAFESE and treatment accomplished. Demand for service was greater in the first 4 years of life; consequently, due to surgical protocol adopted by the institution, lip repair was the most performed surgery.

Discussion This study showed a demand for an early access to the service, especially during the first 4 years of life (►Table 1); the literature is in consensus that the primary surgery for cleft lip and/or palate repair is usually performed in all children before 2 years of age.11 The high concentration of patients attending in this service within this age group suggests that people are getting more conscious about the services directed to cleft treatment. Moreover, the early access to specialist services allows the structuring of a safer and easier treatment plan with a more favorable prognosis, as well as consequent International Archives of Otorhinolaryngology

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Table 4 Associated malformations and complications Associated malformations and complications

Gender Male

Female

Premature

4

3

Limbs deformity þ premature

1

0

Treacher Collins syndrome (ears deformity)

1

0

Cerebral palsy

1

1

Limbs deformity þ anemia

1

0

Diabetics

1

0

Lacrimal duct obstruction

1

0

Apert syndrome þ syndactyly (hands and feet)

1

0

Telecanthus, ozena syndrome

1

0

Delay in bone growth

1

0

Congenital cataract—left eye

1

0

Supernumerary finger—left hand

1

0

Developmental delay

0

1

Breathing problems at birth

2

1

Hydrocephalus þ neurologic problems

3

0

Ptosis

1

0

Congenital heart disease

0

1

Syndromic face

1

0

Epilepsy

1

0

Tessier syndrome þ asthma þ visually impaired

0

1

Displacement of gestational sac

0

1

Polydactyly associated with syndactyly þ Parenteral consanguinity

0

1

Agenesis of the right forearm and hand

0

1

Visually impaired

0

2

Neurological problems

0

1

Malformation widespread

3

1

Total

26

15


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Table 5 Distribution of patients according to the treatment over the years Year

a

Surgical treatment

Total a

%

Cheiloplasty

%

Palatoplasty

%

Other

2003

11

52.4

10

47.6

21

2004

32

46.4

32

46.4

05

7.2

69

2005

15

35.7

19

45.2

08

19.1

42

2006

16

42.1

18

47.4

04

10.5

38

2007

3

100.0

3

Total

77

69

17

173

Fistuloplasty, rhinoplasty, otoplasty.

Table 6 Patient’s age at initial assessment in SEAFESE and treatment accomplished Age group (y)

Surgery

Total

Lip repair

Palatoplasty

Othera

<4

30

13

01

44 (21.5%)

5–09

21

14

35 (17.1%)

10–14

25

09

34 (16.6%)

15–19

22

15

03

40 (19.5%)

20–24

15

07

01

23 (11.2%)

>25

15

11

03

29 (14.1%)

SEAFESE, Service Specializing in Cleft Care of Sergipe. a Fistuloplasty, rhinoplasty, otoplasty.

and significant aesthetic and functional gains that will resonate on the quality of life, reflecting on the family. The demographic profile of the population studied is an individual male (54.0%), pheoderma (47.2%), between 0 and 14 years (77.4%), who lives in the countryside (58.0%). In other studies similar results were observed, where the majority of subjects with oral clefts were males,12–14 lived in metropolitan areas, and were brown.15 The data showed the prevalence of transincisive foramen cleft. These results are similar to a study conducted in Recife, where a predominance of the transincisive foramen cleft (49%) was observed, followed by postforamen (27%) and preforamen (24%) clefts.15 Freitas et al observed in São Paulo that the most common types of cleft lip and/or palate were trans- and postforamen (31.7%), followed by preforamen (28.4%) and rare fissure (3.8%).16 In other countries a similar prevalence was observed. In Germany, Kramer et al found that the transforamen (42.4%) cleft was most often observed, followed by preforamen (28.8%) and postforamen (28.8%) clefts.11 Sagheri et al, also in Germany, found that the transforamen (45.9%) cleft was observed most often, followed by postforamen (41%) and preforamen (9.8%) clefts and PierreRobin syndrome (3.3%).17 The cleft lip and/or palate may be present as isolated deformities (or nonsyndromic clefts) or within the phenotype of a syndrome, called syndromic clefts.15 Several studies suggest that 30 to 40% of cases occur as a pattern of multiple malformations and are classified as a known syndrome related to chromosomal alterations, Mendelian disorder, or

exposure to a known teratogen.18 In this research, associated malformations and complications occurred in 41 individuals, and the data found in this study agree with the findings of Baptista,15 Milerad et al,19 Ellis,20 and Nunes et al.21 Data about prenatal care outlined in this study showed accompanying of almost half the analyzed sample and only 7.0% stated no kind of accompanying. This prevalence is lower than that found in other countries. In France the prenatal diagnosis of cleft occurs in 62.8% of cases,22 in the United States the prevalence is 16%,23 and in England the prevalence is 30%.24 Most parents who have a prenatal diagnosis felt that the diagnosis prepared them psychologically for the birth of the child with cleft.24 Such data suggest that the primary health care policy adopted by the government, at least in terms of awareness, has been characterized by reforming the potential of primary care in favor of better health outcomes, which caused significant changes in health practice, even though 156 (45.0%) medical records do not have report data about prenatal care. Although the increased demand for care in SEAFESE is by children between 0 and 4 years, with a high rate of newborns who have not been subjected to any type of surgery, there was a high number of patients who reported at first visit having already had previous surgery to correct cleft lip and/or palate in another institution. Besides, it was found that in the 0- to 4year group, 44 surgeries had already occurred, followed by 40 surgeries in the 15- to 19-year group. These data confirm that the demand for treatment of cleft patients is taking place at the right time, which is in the first months/years of life. International Archives of Otorhinolaryngology

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Conclusions

11 Kramer FJ, Gruber R, Fialka F, Sinikovic B, Hahn W, Schliephake H.

In conclusion, the few epidemiologic studies on cleft in Brazil highlight the importance and need for studies that address determinants of developmental defects, such as oral cleft. In this study, male gender was the most affected, and the age group 0 to 4 years was the most frequent. Transincisive foramen cleft type was most frequently found. Prenatal care was reported by most mothers. So, this study found that early treatment is a reality in SEAFESE; therefore the chances of successful integration of the child to society will be better.

12

13

14

15

References 1 Cobourne MT. The complex genetics of cleft lip and palate. Eur J

Orthod 2004;26:7–16 2 Munz SM, Edwards SP, Inglehart MR. Oral health-related quality of

3

4

5 6

7

8

9

10

life, and satisfaction with treatment and treatment outcomes of adolescents/young adults with cleft lip/palate: an exploration. Int J Oral Maxillofac Surg 2011;40:790–796 Donkor P, Plange-Rhule G, Amponsah EK. A prospective survey of patients with cleft lip and palate in Kumasi. West Afr J Med 2007;26:14–16 Poletta FA, Castilla EE, Orioli IM, Lopez-Camelo JS. Regional analysis on the occurrence of oral clefts in South America. Am J Med Genet A 2007;143A:3216–3227 Tan KB, Tan KH, Yeo GS. Cleft deformities in Singapore: a populationbased series 1993–2002. Singapore Med J 2008;49:710–714 Freitas e Silva DS, Mauro LDP, Oliveira LB, et al. Estudo descritivo de fissuras lábio-palatinas relacionadas a fatores individuais, sistêmicos e sociais. RGO 2008;56(4):387–391 Rodrigues K, Sena MF, Roncalli AG, Ferreira MA. Prevalence of orofacial clefts and social factors in Brazil. Braz Oral Res 2009;23: 38–42 Ministério da Saúde. Brazil: DATASUS. Available at: http://tabnet. datasus.gov.br/cgi/tabcgi.exe?sinasc/cnv/nvuf.def. Accessed March 22, 2012 IBGE. Instituto Brasileiro de Geografia e Estatística. Regiões de Influência das Cidades. 2007. Available at: http://www.ibge.gov. br/home/geociencias/geografia/regic.shtm. Accessed May 5, 2012 Spina V, Psillakis JM, Lapa FS, Ferreira MC. Classificação das fissuras lábio-palatinas. Sugestão de modificação. Rev Hosp Clin Fac Med Sao Paulo 1972;27:5–6

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17

18

19

20 21

22

23 24

Quality of life in school-age children with orofacial clefts and their families. J Craniofac Surg 2009;20:2061–2066 Ajike SO, Adebola RA, Efunkoya A, Adeoye J, Akitoye O, Veror N. Epidemiology of adult cleft patients in North-western Nigeria: our experience. Ann Afr Med 2013;12:11–15 Martelli DR, Machado RA, Swerts MS, Rodrigues LA, Aquino SN, Martelli Júnior H. Non syndromic cleft lip and palate: relationship between sex and clinical extension. Braz J Otorhinolaryngol 2012;78:116–120 Costa CH, Diniz LV, Lacerda RH, Forte FD, Sampaio FC. Prevalence of dental anomalies in patients with cleft lip and palate, Paraiba, Brazil: clinic and radiographic study. Acta Odontol Latinoam 2012;25:181–185 Baptista EVP. Malformações congênitas associadas à fissura labial e/ou palatal em pacientes atendidos em um serviço de referência para tratamento de defeitos da face: um estudo de série de casos [dissertation]. Recife, Brazil: Instituto Materno Infantil Prof. Fernando Figueira; 2007:67 Freitas JA, Dalben GdaS, Santamaria M Jr, Freitas PZ. Current data on the characterization of oral clefts in Brazil. Braz Oral Res 2004;18:128–133 Sagheri D, Ravens-Sieberer U, Braumann B, von Mackensen S. An Evaluation of Health-Related Quality of Life (HRQoL) in a group of 4-7 year-old children with cleft lip and palate. J Orofac Orthop 2009;70:274–284 Dixon MJ, Marazita ML, Beaty TH, Murray JC. Cleft lip and palate: understanding genetic and environmental influences. Nat Rev Genet 2011;12:167–178 Milerad J, Larson O, PhD D, Hagberg C, Ideberg M. Associated malformations in infants with cleft lip and palate: a prospective, population-based study. Pediatrics 1997;100(2 Pt 1):180–186 Ellis E. Cirurgia Oral e Maxilofacial Contemporânea. 4th ed. Rio de Janeiro, Brazil: Elsevier; 2005 Nunes LMN, Queluz DP, Pereira AC. Prevalência de fissuras labiopalatais no município de Campos dos Goytacazes-RJ, 1999–2004. Rev Bras Epidemiol 2007;10(1):109–116 Guyot A, Soupre V, Vazquez MP, et al. [Prenatal diagnosis of cleft lip with or without cleft palate: retrospective study and review]. J Gynecol Obstet Biol Reprod (Paris) 2013;42:151–158 Matthews MS, Cohen M, Viglione M, Brown AS. Prenatal counseling for cleft lip and palate. Plast Reconstr Surg 1998;101:1–5 Davalbhakta A, Hall PN. The impact of antenatal diagnosis on the effectiveness and timing of counselling for cleft lip and palate. Br J Plast Surg 2000;53:298–301


Review Article

Tympanometry in Infants: A Study of the Sensitivity and Specificity of 226-Hz and 1,000-Hz Probe Tones Michele Picanço Carmo1

Nayara Thais de Oliveira Costa1

1 PhD student in Speech-Language Pathology and Audiology, Pontifical

Catholic University of São Paulo (PUCSP), São Paulo/SP, Brazil 2 Full Professor of the Department of Clinical Speech Therapy, Pontifical Catholic University of São Paulo (PUCSP), São Paulo/SP, Brazil

Teresa Maria Momensohn-Santos2

Address for correspondence Michele Picanço Carmo, Av. Diógenes Ribeiro de Lima, 2000/8,6, Alto de Pinheiros, São Paulo/SP, CEP 05458001, Brasil (e-mail: micheledocarmo@hotmail.com).

Int Arch Otorhinolaryngol 2013;17:395–402.

Abstract

Keywords

► speech language and hearing sciences ► hearing ► acoustic impedance tests ► otitis media with effusion ► spontaneous otoacoustic emissions

Introduction For infants under 6 months, the literature recommends 1,000-Hz tympanometry, which has a greater sensitivity for the correct identification of middle ear disorders in this population. Objective To systematically analyze national and international publications found in electronic databases that used tympanometry with 226-Hz and 1,000-Hz probe tones. Data Synthesis Initially, we identified 36 articles in the SciELO database, 11 in the Latin American and Caribbean Literature on the Health Sciences (LILACS) database, 199 in MEDLINE, 0 in the Cochrane database, 16 in ISI Web of Knowledge, and 185 in the Scopus database. We excluded 433 articles because they did not fit the selection criteria, leaving 14 publications that were analyzed in their entirety. Conclusions The 1,000-Hz tone test has greater sensitivity and specificity for the correct identification of tympanometric curve changes. However, it is necessary to clarify the doubts that still exist regarding the use of this test frequency. Improved methods for rating curves, standardization of normality criteria, and the types of curves found in infants should be addressed.

Introduction Tympanometry is effective in the early identification of changes in the external ear (EE) and middle ear (ME), and its use is well established for children and adults. However, its use for newborns and infants is still controversial. The literature recommends 1,000-Hz tympanometry for infants under 6 months, which has a greater sensitivity for the correct identification of ME dysfunction in this population.1,2

Literature Review ME alterations have a high incidence in children. The presence of otitis media with effusion, cerumen, vernix, or other causes

received November 20, 2012 accepted February 12, 2013

of conductive hearing loss contribute to a large number of false-positive results in neonatal hearing screening and may also delay the completion of diagnostic audiology. Therefore, it is important to identify hearing screening failures caused by transient changes in the ME and EE through an instrument that allows quick, accurate, and noninvasive assessment.1,2 One of the assessment tools used in routine audiology of the ME is the measurement of acoustic impedance, which is a general term related to the transfer of acoustic energy, either by facilitation (admittance) of or opposition (impedance) to the passage of sound energy. This is influenced by the amount of sound energy that is absorbed and reflected by the tympanic membrane (TM). When it is more compliant, the

Copyright © 2013 by Thieme Publicações DOI http://dx.doi.org/ Ltda, Rio de Janeiro, Brazil 10.1055/s-0033-1351678. ISSN 1809-9777.

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TM absorbs more energy and therefore allows greater admittance of the system. On the other hand, the more rigid the TM, the more energy will be reflected, creating greater system impedance.3,4 Tympanometry is a measure of the variation of the acoustic impedance of the tympanic ossicular system caused by pressure variations introduced into the external auditory canal and indicates the condition of the ME and auditory pathways.4 In audiology, the most commonly used probe tone has a low frequency of 226 Hz; however, more modern equipment includes additional probe tones such as 660, 678, and 1,000 Hz. Tympanometry is a common practice in children and adults, and its specifications are very well established. However, there are many uncertainties regarding the specificity and sensitivity of the probe tones used in the evaluation of neonates and infants. The literature has shown that in neonates and infants, tympanograms obtained with a low-frequency probe tone can be recorded as normal, even in the presence of altered ME function.2–6 High-frequency probe tones, in particular 1,000 Hz, have a higher sensitivity for the identification of ME changes and therefore should be used in neonates and infants under 6 or 7 months of age if possible.2,4–8 The objectives of this study were to systematically analyze national and international scientific publications on the application of tympanometry in infants using 226-Hz and 1,000-Hz test tones and to compare sensitivity and specificity between these two test tones. To investigate the number of publications on this topic, two researchers independently conducted a bibliographic survey in the SciELO, Latin American and Caribbean Literature on the Health Sciences (LILACS), MEDLINE, Cochrane, Scopus, and ISI Web of Knowledge electronic databases. For the search strategy, the Descriptors in Health Sciences (MeSH, created by the National Library of Medicine) term acoustic impedance tests was combined with the words neonate and infant. National and international articles published in the past 10 years were examined, delimiting the period between January 2001 and December 2011. The inclusion criteria for the full analysis of texts were as follows: original article or case report; infants up to 7 months old as the research subjects; use of 1,000-Hz probe tone as tone test during tympanometry; description of tympanometric curves obtained; and published in Portuguese, English, or Spanish. The articles that met the inclusion criteria were selected for review in full, analyzed, and organized in a database with the following parameters: author(s), title, source, year, research type, sample characteristics, test tones used, main findings, and classification of tympanometric curves.

The route taken for selection and analysis of text is shown in ►Fig. 1. The 14 selected publications were analyzed considering the source and year of publication. It was found that in the 10-year period investigated (2001 to 2011), the number of studies performed on tympanometry with high-frequency probe tones was not significant, but the number of publications increased from the year 2006 (►Table 1). A thorough analysis of the publications was performed considering the characteristics of the sample, the test tone frequencies used in tympanometry, the main findings identified, and the classification of tympanometric curves (►Table 2). This information is presented and discussed hereafter, with the assistance of other theoretical publications considered relevant for the purpose of better understanding the topic in question.

Test Frequency and Age In neonates and infants, growth entails changes in the ear that generate mechanical alterations, which can influence tympanogram recordings. These changes include increased EE, mastoid, and ME cavity; mass reduction of the ME due to changes in bone density; and bone formation in the wall of the external auditory canal.4 In addition, the resonance of the ME is modified according to age; the resonance frequency of the ME is lower in babies when compared with the values of

Discussion Initially, 447 articles were identified. Thirty-six articles were found in the SciELO database, 11 in the LILACS database, 199 in MEDLINE, 0 in the Cochrane database, 16 in ISI Web of Knowledge, and 185 in the Scopus database. We excluded 433 publications because they did not fit the selection criteria, leaving 14 publications that were analyzed in their entirety. International Archives of Otorhinolaryngology

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Fig. 1 Route taken for selection and analysis of text.


Tympanometry in Infants

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Table 1 List of studies on tympanometry with high- and low-frequency probe tones from 2001 to 2011 Author Kei et al

1

Title

Source

Year

High-frequency (1,000 Hz) tympanometry in normal neonates

J Am Acad Audiol

2003

Margolis et al2

Tympanometry in newborn infants, 1-kHz norms

J Am Acad Audiol

2003

Baldwin7

Choice of probe tone and classification of trace patterns in tympanometry undertaken in early infancy

Int J Audiol

2006

Calandruccio et al31

Normative multifrequency tympanometry in infants and toddlers

J Am Acad Audiol

2006

Swanepoel et al29

Infant hearing screening at immunization clinics in South Africa

Int J Pediatr Otorhinolaryngol

2006

Alaerts et al15

Evaluation of middle ear function in young children: clinical guidelines for the use of 226- and 1,000-Hz tympanometry

Otol Neurotol

2007

Silva et al27

Tympanometry in neonates with normal otoacoustic emissions: measurements and interpretation

Rev Bras Otorrinolaringol

2007

Swanepoel et al8

High-frequency immittance for neonates: a normative study

Acta Otolaryngol

2007

Shahnaz et al6

Multifrequency tympanometry in neonatal intensive care unit and well babies

J Am Acad Audiol

2008

Garcia et al22

Acoustic immittance measures in infants with 226- and 1,000-Hz probes: correlation with otoacoustic emissions and otoscopy examination

Rev Bras Otorrinolaringol (Engl Ed)

2009

Zhiqi et al25

Tympanometry in infants with middle ear effusion having been identified using spiral computerized tomography

Am J Otolaryngology

2010

Camboim et al23

Comparative analysis of otoacoustic emissions with tympanometry in 0- to 6-mo infants

Revista CEFAC

2012

Tazinazzio et al24

Otoacoustic emissions and acoustic immittance measurements using 226-Hz and 1,000-Hz probe tones in neonates

Revista CEFAC

2011

Lewis et al30

A comparison of tympanometry with 226-Hz and 1,000-Hz probe tones in children with Down syndrome

Int J Pediatr Otorhinolaryngol

2011

an adult.9 Sagging of the ear canal can also cause movement of the wall of the ear canal.10,11 In the literature, there are notable divergences in the age at which the high-frequency probe test is recommended. There are indications for its use in children under 7 months of age,2,4–8 less than 6 months old,12,13 less than 4 months old,14 and under 5 months of age.7 Other authors claim that for use of 1,000-Hz test tones in infants up to 3 months old and between 3 and 9 months of age, ME evaluation must be performed in two stages. Initially, the 1,000-Hz test tone should be used and in cases of failure with this tone, testing should then be performed with a 226-Hz probe tone. The use of a low test frequency of 226 Hz has also been indicated for the assessment of infants over 9 months.15

Classification of Tympanometric Curves According to the literature, tympanometric curves are usually classified as follows: type A (normal curve having a single peak of admittance between 150 and 100 daPa and a volume

of 0.2 to 1.8 mL); flat or type B (flat curve with no admittance peak); type C (admittance peak shifted to negative pressures); As (curve with low admittance); Ad (interval between the two branches of the curve equal to or greater than 100 daPa)16; DP (double peak curve)17; ASS (asymmetrical curve with peak at high positive pressure); and I (inverted curve with an inverted configuration compared with the normal curve).18 However, there are also other less common types of tympanometric curves.19–21 Among the studies analyzed in the current review, it was observed that there was a consensus that the type A curve indicated a normal ME,1,15,17–19 and B, C, As, and Ad curves implied an altered ME.15,22–25 Regarding the DP curve, also called a type D curve, five studies showed that this curve indicates normality of the ME in the case of neonates.15,22–24,26 According to one author,3 this type of tympanogram is most often identified in the neonate population when tympanometry testing is performed with a 226-Hz probe tone. This is because such International Archives of Otorhinolaryngology

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Table 2 Main characteristics of studies with test tones of 226- and 1,000-Hz Author

Sample characteristics

Probe tones

Main findingsa

Classification of curves

Kei et al1

122 neonates, aged 1–6 d with OAEs present

226 Hz; 1,000 Hz

1,000 Hz: type 1, 225 ears; type 2, 14 ears; type 3, 3 ears; other atypical forms, 2 ears. 226 Hz: single peak, 115 ears; DP, 116 ears; multiple peak, 9 ears; invalid curves, 3 ears.

1,000 Hz: type 1 (A), normal; types 2 and 3 (B, DP), altered 226 Hz, single peak: normal; DP, altered

Margolis et al2

65 infants, average age of 3.9 wk

1,000 Hz

Single peak curves found in the majority of infants. Authors did not state the number of curves encountered.

Single peak: normal

Baldwin7

104 children between 2 and 19 wk with normal hearing and 107 infants aged 2–21 wk with temporary conductive hearing loss

226 Hz; 678 Hz; 1,000 Hz

Infants with abnormal ME and normal tympanograms (type A): 94.9% with 226 Hz, 1.3% with 678 Hz, and 0% with 1,000 Hz.

Positive peak: normal Flat, descending negative peak, or undetermined: altered

Calandruccio et al31

33 children between 4 wk and 2 y and 33 adults with a mean age of 30.3 y

226 Hz; 630 Hz; 1,000 Hz

226 Hz: higher proportion of type 1B1G; 23.1% type 3B1G in children aged 4–10 wk and 6.8% in children aged 11–19 wk. 1,000 Hz: children showed greater variability of types, with 1B1G and 3B1G having equal distribution.

Type 1B1G, 3B1G, 3B3G, and 5B3G tympanograms (Vanhuyse et al21)

Swanepoel et al29

510 infants, aged 0–12 mo

226 Hz; 1,000 Hz

87% of tympanograms displayed a peak, DP type curves present in 4.5% of cases.

Tympanogram with peak: ME normal DP type curve: ME altered

Alaerts et al15

110 infants up to 9 mo and 15 adults between 17 and 27 y with normal hearing

226 Hz; 1,000 Hz

226 Hz: number of cases of type A increased with increasing age; number of cases of type D decreased with increasing age. 1,000 Hz: number of cases of type D increased with increasing age.

Types A and D: normal Types B, C, and Du: altered Types 1 and 4u: normal Types 2, 3, and 4u: altered

Silva et al27

110 neonates between 6 and 30 d with TEOAEs present

226 Hz; 678 Hz; 1,000 Hz

226 Hz: 47.7% single peak; 52.3% DP. 678 Hz: 25.4% single peak; 67.3% ASS; 7.3% I. 1,000 Hz: 70.9% single peak; 28.2% ASS; 0.9% I.

Single peak and DP: normal ASS and I: normal or altered

Swanepoel et al8

143 infants between 0 and 4 wk of age

1,000 Hz

8% of tympanograms showed no peak.

Tympanogram with peak: ME normal

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Table 2 (Continued) Author

Sample characteristics

Probe tones

Main findingsa

Classification of curves

6% were DP-type curves. 57% sensibility. 95% specificity.

DP-type curve: ME altered

Shahnaz et al6

33 neonates from ICU and 16 healthy infants between 21 and 28 d; 42 infants between 6 d and 23 wk and 33 preterm neonates between 32 and 51 wk with BAEPs present compared with 16 adults between 18 and 32 y with normal hearing

226 Hz; 1,000 Hz

Newborns and infants showed a great variability of complex tympanometric patterns with 226 Hz. With increasing test frequency, the ratio of single peak curves increased and with 1,000 Hz the rate was 64%.

Model: Vanhuyse et al21 226 Hz: 13% 1B1G and 85% complex multiple peak tympanograms 1,000 Hz: predominantly 3B1G (50%) and 1B1G (38%)

Garcia et al22

60 infants between 0 and 4 mo in the absence and presence of OAEs

226 Hz; 1,000 Hz

OAEs present: 226 Hz: 61% A; 27% D; 8.3% B; 3.7% C. 1,000 Hz: 60% A; 11.6% D; 13.4% I; 5% B; 10% C. OAEs absent: 226 Hz: 53.3% A; 31.7% D; 13.3% B; 1.7% C. 1,000 Hz: 20% A; 2% D; 0% I; 70% B; 8% C.

Types A, D, and I: normal Types B and C: altered Type ASS: normal or altered

Zhiqi et al25

52 infants between 42 d and 6 mo

226 Hz; 1,000 Hz

226 Hz: Group with normal ME: 51.06% single peak; 44.68% DP; 2.13% flat; 2.13% with negative pressure. Group with ME effusion: 77.19% single peak; 19.30% DP; 3.51% flat. 1,000 Hz: Group with normal ME: 97.87% single peak. Group with ME effusion: 98.25% flat.

Single peak: normal tympanogram Flat: altered (ME effusion)

Tazinazzio et al24

52 infants between 11 and 51 d with OAEs present and absent; at 1,000 Hz, the number of ears was less because blocked curves were excluded (occurrence of the occlusion effect)

226 Hz; 1,000 Hz

OAEs present and normal curves: 226 Hz, 82 ears; 1,000 Hz, 66 ears. OAEs present and altered curves: 226 Hz, 0 ears; 1,000 Hz, 6 ears. OAEs absent and normal curves: 226 Hz, 19 ears; 1,000 Hz, 8 ears. OAEs absent and altered curves: 226 Hz, 3 ears; 1,000 Hz, 13 ears.

Types A and D: normal Types As, B, and flat: altered

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Table 2 (Continued) Author

Sample characteristics

Probe tones

Main findingsa

Classification of curves

Lewis et al30

26 children with Down syndrome between 6 and 18 mo

226 Hz; 1,000 Hz

226 Hz: ME no fluid: type B, 7 ears; type A, 17 ears. ME with fluid: type A, 0 ears; type B, 2 ears. 1,000 Hz: ME no fluid: type A, 24 ears; type B, 0 ears. ME with fluid: type B, 2 ears; type A, 0 ears.

Type A: normal Type B: altered

Camboim et al23

118 infants between 0 and 6 mo with OAEs present and absent

226 Hz; 1,000 Hz

1,000 Hz: high correlation between normal curves (type A and DP) and presence of OAEs, as well as altered curves (not type A or DP) and absence of OAEs.

Types A and DP: normal Types C, Ad, As, and B: altered

Abbreviations: BAEPs, brainstem auditory evoked potentials; ICU, intensive care unit; ME, middle ear; OAEs, evoked otoacoustic emissions; TEOAEs, transient evoked otoacoustic emissions. a Information taken from the Results section of publications.

registration occurs when measurement is performed at the resonant frequency of the ME and in the case of this population, this frequency is shifted to lower values. Regarding the I and ASS curves, it was found that there are disagreements with regards to their normal standards and which parameters should be used for classification of a normal or altered curve. The ASS curve and the I curve can be identified in subjects with and without alteration of the ME and can be regarded as normal or altered.22,27 One publication stated that the I curve should be classified as normal in infants.22 Besides the ambiguity that exists in the classification of curves with a 1,000-Hz probe tone, another issue regarding high-frequency tympanometry in neonates is the high number of unclassified or indeterminate curves obtained by the use of classification systems designated for the low-frequency tone test in adults and older children.7 This has created more doubt and questions regarding curve classification and the parameters that define categorization as normal or abnormal. In a study using 1,000-Hz testing, the authors classified curves as follows: type 1 (similar to type A), type 2 (similar to type B), type 3 (similar to DP), and atypical forms. With a 226-Hz tone test, in addition to using the classification of DP, the authors also used the nomenclature single-peak tympanogram, multiple-peak tympanogram, and invalid tympanogram. From this perspective, the authors concluded that there is no direct correspondence between ME condition and tympanometry curve type. However, the type 1 curve, which was obtained in 92.3% of neonates, indicated an ME without alterations.1 It has been hypothesized that changes in the patterns of tympanometry may also vary according to the age of the individuals assessed. In one study using a 226-Hz probe tone, the percentage of type A curves increased with increasing age, International Archives of Otorhinolaryngology

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and the percentage of DP curves decreased.15 Furthermore, the use of the 1,000-Hz tone decreased the prevalence of flat tympanograms and increased DP curves in a group of children. However, it should be mentioned that the sample surveyed included no children with ME alterations. Changes in the quantitative values of tympanometry can be justified by the rapid anatomical growth of the ear in neonates during the first 6 weeks of life.28 An increase in resonance frequency occurs in the ear of neonates, and this shifts the type of tympanometric curve from DP to single peak. This alteration can be explained by anatomical evidence showing that the physical structure of the ear changes from a state allowing positive reactance (dominated by mass) to one allowing negative reactance (dominated by stiffness). When the ME reaches negative reactance, the low-frequency tone test becomes an appropriate tool to evaluate the peripheral auditory system. That is, with the evaluation of ear reactance, it is possible to identify the resonance frequency, and if this is low, the use of a high-frequency tone is sufficiently sensitive. However, if this frequency approaches the frequency of the adult, the conventional 226-Hz tone should be used. Such changes in resonance frequency may be due to intrasubject variability or true evolution of ME function with age.26

Relationship between Tympanometry and Other Audiological Evaluations Other procedures used to identify hearing loss in infants include measurement of otoacoustic emissions (OAEs) and brainstem auditory evoked potentials. It is possible to correlate the results of these procedures and tympanometry traces to better assess hearing condition. The literature indicates that the 1,000-Hz probe tone has a higher correlation with the responses of transient otoacoustic emissions (TOAEs) and distortion product otoacoustic


Tympanometry in Infants emissions.23,24,29 The 1,000-Hz tone displays this correlation in both the evaluation of normal ears and ears with ME alterations. Tympanometry with a 226-Hz tone provides results consistent with assessment of OAEs in the presence of a normal ME only.23 For infants with TOAEs present, both probe tones have good specificity but, for infants with TOAEs absent, the 1,000-Hz tone is more sensitive for the identification of possible alterations in the ME.22 From this perspective, the use of tympanometry with a 1,000-Hz tone in infants is the most appropriate procedure for detecting changes in the ME, especially when TOAEs are absent.24 On the other hand, different tympanometry curve types can be identified even in the presence of different OAE recordings. The reason for the detection of heterogeneous curves even with the presence of OAEs, which suggest normal ME function, is unclear. It may be due to the fact that light dysfunction of the ME is not able to prevent OAE recording or may even be caused by inadequate sealing of the probe, resulting in movement-generated artifacts.1 The relationship between responses to 226- and 1,000-Hz probe tones and OAE recordings was also analyzed in a study of children with Down syndrome, showing a strong correlation between 1,000-Hz tympanometry and OAE results.30

they do not exist) and false-negative results (when changes are not detected even in their presence). However, both falsepositives and false-negatives are less frequent with the 1,000-Hz probe tone. For this reason, the test frequency of 1,000 Hz is more sensitive and specific than the frequency of 226 Hz for the assessment of ME alterations in neonates. Therefore, it is concluded that this probe tone is most appropriate for evaluating infants less than 6 months of age. Although the literature confirms that the 1,000-Hz tone test is the most appropriate for assessment in this population, further studies are needed to clarify the doubts that still exist regarding the use of tympanometry with a high-frequency tone. In addition, better systems for the classification of curves and normality criteria should be investigated.

References 1 Kei J, Allison-Levick J, Dockray J, et al. High-frequency (1000 Hz)

2

3

Test Frequency Most Suitable for Evaluation of Infants Despite the fact that it has been used for decades, the 226-Hz tone test is not the most appropriate for assessing infants,1,2,5–8,22–24,27 mainly because, as already mentioned, this population has maturational characteristics that are distinct from children older than 6 months of age and adults. Among the sources analyzed, were identified three studies that used testing tones of 678 and 630 Hz in addition to 226 and 1,000 Hz. In all three studies, the authors observed that the 1,000-Hz tone test showed better results for the characterization of tympanograms when compared with other frequencies.7,27,31 Studies have shown that the efficacy of the 1,000-Hz tone in relation to the 226-Hz and 678-Hz probe tones is justified by the fact that mass components are larger at high-frequency probe tones and lower at low-frequency probe tones, which modifies the resonance characteristics of the ME.1,2 Changes in the wall of the external auditory canal, ME mobility, and tympanometric characteristics are observed in the first months of life. This is because the ME of children is dominated by the component of mass, and the resonance frequency tends to be lower when compared with normal adults, in whom the system is controlled by stiffness.14 A Brazilian study investigating resonance frequency reported a value of 250 Hz in children between 2 and 12 days and a value of 385 Hz in children between 72 and 84 days.32

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After the analysis of several publications, it was found that both the test tones of 226 and 1,000 Hz show false-positive results (indicating the presence of alterations in the ME when

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tympanometry in normal neonates. J Am Acad Audiol 2003; 14(1):20–28 Margolis RH, Bass-Ringdahl S, Hanks WD, Holte L, Zapala DA. Tympanometry in newborn infants—1 kHz norms. J Am Acad Audiol 2003;14(7):383–392 Carvallo RM. Medidas eletroacústicas imitância acústica. In: Carvallo RM, ed. Fonoaudiologia Informação para a Formação. Procedimentos em Audiologia. Rio de Janeiro, Brazil: Guanabara Koogan; 2003:1–22 Margolis RH, Hunter LL. Timpanometria: princípios básicos e aplicações clínicas. In: Musiek FR, Rintelman WF eds. Perspectivas Atuais em Avaliação Auditiva. São Paulo, Brazil: Manole; 2001: 85–126 Sininger YS. Audiologic assessment in infants. Curr Opin Otolaryngol Head Neck Surg 2003;11(5):378–382 Shahnaz N, Miranda T, Polka L. Multifrequency tympanometry in neonatal intensive care unit and well babies. J Am Acad Audiol 2008;19(5):392–418 Baldwin M. Choice of probe tone and classification of trace patterns in tympanometry undertaken in early infancy. Int J Audiol 2006;45(7):417–427 Swanepoel W, Werner S, Hugo R, Louw B, Owen R, Swanepoel A. High frequency immittance for neonates: a normative study. Acta Otolaryngol 2007;127(1):49–56 Weatherby LA, Bennett MJ. The neonatal acoustic reflex. Scand Audiol 1980;9(2):103–110 Keefe DH, Bulen JC, Arehart KH, Burns EM. Ear-canal impedance and reflection coefficient in human infants and adults. J Acoust Soc Am 1993;94(5):2617–2638 Gravel JS, Hood LJ. Avaliação audiológica infantil. In: Musiek FE, Rintelmann WF, eds. Perspectivas Atuais em Avaliação Auditiva. São Paulo, Brazil: Manole; 2001:301–322 Joint Committee on Infant Hearing. Year 2007 Position Statement: Principles and Guidelines for Early Hearing Detection and Intervention Programs. Pediatrics 2007;120(4):898–921 Newborn Hearing Screening and Assessment. Tympanometry in babies under 6 months. A Recommended Test Protocol [cited November 20, 2012]. Available at: http://hearing.screening.nhs. uk/audiologyprotocols#fileid10753 Holte L, Margolis RH, Cavanaugh RM Jr. Developmental changes in multifrequency tympanograms. Audiology 1991;30(1):1–24 Alaerts J, Luts H, Wouters J. Evaluation of middle ear function in young children: clinical guidelines for the use of 226- and 1,000Hz tympanometry. Otol Neurotol 2007;28(6):727–732 Jerger J. Clinical experience with impedance audiometry. Arch Otolaryngol 1970;92(4):311–324 International Archives of Otorhinolaryngology

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Laryngol Otol 1969;83(6):507–520 Margolis RH, Smith P. Tympanometry in infants: state of art. In: Harford E, Klein J eds. Impedance Screening of Middle Ear Disease in Children. New York, NY: Grune and Stratton; 1978 Sutton G, Baldwin M, Brooks D, Gravel J, Thornton R. Tympanometry in neonates and infants under 4 months: a recommended test protocol. Manchester, England 2002 [cited November 20, 2012]. Available at: http://hearing.screening.nhs.uk/ Marchant CD, McMillan PM, Shurin PA, et al. Objective diagnosis of otitis media in early infancy by tympanometry and ipsilateral acoustic reflex thresholds. J Pediatr 1986;109:590–595 Vanhuyse VJ, Creten WL, Van Camp KJ. On the W-notching of tympanograms. Scand Audiol 1975;4(1):45–50 Garcia MV, Azevedo MF, Testa JR. Accoustic immitance measures in infants with 226 and 1000 hz probes: correlation with otoacoustic emissions and otoscopy examination. Braz J Otorhinolaryngol 2009;75(1):80–89 Camboim ED, Correia AMN, Vasconcelos D, Torres R, Scharlach RC, Azevedo MF. Comparative analysis of otoacoustic emissions with tympanometry in 0-6 month infants. Rev CEFAC-Speech, Language, Hearing Sciences and Education Journal [online] 2012; 14(3):403-412 [cited November 20, 2012]. Available at: http:// www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-184620 12000300004&lng=en&nrm=iso Tazinazzio TG, Diniz TA, Marba STM, Colella-Santos MF. Otoacoustic emissions and acoustic immitance measurements using 226 Hz and 1000 Hz probe tones in neonates. Rev CEFAC-Speech, Lan-

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guage, Hearing Sciences and Education Journal [online] 2011; 13 (3):479-488 [cited November 20, 2012]. Available at: http://www. scielo.br/scielo.php?script¼sci_arttext&pid¼S151618462011000300011&lng¼pt&nrm¼iso Zhiqi L, Kun Y, Zhiwu H. Tympanometry in infants with middle ear effusion having been identified using spiral computerized tomography. Am J Otolaryngol 2010;31(2):96–103 Meyer SE, Jardine CA, Deverson W. Developmental changes in tympanometry: a case study. Br J Audiol 1997;31(3):189–195 Silva KA, Novaes BA, Lewis DR, Carvallo RM. Tympanometry in neonates with normal otoacoustic emissions: measurements and interpretation. Braz J Otorhinolaryngol 2007;73(5):633–639 Mazlan R, Kei J, Hickson L, et al. High frequency immittance findings: newborn versus six-week-old infants. Int J Audiol 2007;46:711–717 Swanepoel W, Hugo R, Louw B. Infant hearing screening at immunization clinics in South Africa. Int J Pediatr Otorhinolaryngol 2006;70(7):1241–1249 Lewis MP, Bradford Bell E, Evans AK. A comparison of tympanometry with 226 Hz and 1000 Hz probe tones in children with Down syndrome. Int J Pediatr Otorhinolaryngol 2011;75(12): 1492–1495 Calandruccio L, Fitzgerald TS, Prieve BA. Normative multifrequency tympanometry in infants and toddlers. J Am Acad Audiol 2006;17(7):470–480 André KD, Sanches SGG, Carvallo RMM. Middle ear resonance in infants: age effects. Int Arch Otorhinolaryngol 2012;16(3): 353–357


Case Report

Rehabilitation with Cochlear Implant in Patient with Harboyan Syndrome Lauren Medeiros Paniagua1,2

Maria Elza Kazumi Yamaguti Dorfman3

1 Doctor of Science in Children’s and Teenager’s Health, Federal

University of Rio Grande do Sul-UFRGS, Porto Alegre/RS, Brazil 2 Professor of Speech-Language Pathology, Fatima’s University (RS), Rio de Janeiro/RJ, Brazil 3 Speech-Language Pathologist, Voice Specialist, Hospital de Clinicas de Porto Alegre, Porto Alegre/RS, Brazil 4 Department of Otorhinolaryngology, University Hospital of Porto Alegre, Porto Alegre/RS, Brazil 5 Graduate Program in Medicine: Surgery, Medical School, Federal University of Rio Grande do Sul, Porto Alegre/RS, Brazil 6 Audiologist, Professor of Federal University of Rio Grande do Sul (UFRGS), Porto Alegre/RS, Brazil

Luiz Lavinsky4,5

Pricila Sleifer6

Address for correspondence Lauren Medeiros Paniagua, Avenida João Wallig, 1705/627, 91340001, Porto Alegre/RS, Brazil (e-mail: lmedeirospaniagua@yahoo.com.br).

Int Arch Otorhinolaryngol 2013;17:403–406.

Abstract

Keywords

► ► ► ►

cochlear implants hearing loss sensorineural rehabilitation of speech and language disorders

Background Harboyan syndrome, defined as congenital corneal dystrophy associated with progressive sensorineural hearing loss, was first described by Harboyan in 1971. It is a hereditary disease manifested by eye lesions consistent with corneal endothelial dystrophy and progressive sensorineural hearing loss. There is bilateral symmetric progressive hearing loss, which may be either dominant or recessive. Objective To report a case of a patient with a diagnosis of Harboyan syndrome. Case Report A 25-year-old woman with profound bilateral sensorineural hearing loss, showing poor hearing performance while using a personal sound amplification device, underwent hearing rehabilitation with a cochlear implant. Conclusion Rehabilitation was imperative in this case. The cochlear implant has proven to be the best therapeutic option, providing the patient with a better quality of life.

Introduction Harboyan syndrome, defined as congenital corneal dystrophy associated with progressive sensorineural hearing loss, was first described by Harboyan et al in 1971. It is a hereditary disease manifested by eye lesions consistent with corneal endothelial dystrophy and progressive sensorineural hearing loss.1 There are two different types of congenital hereditary corneal dystrophies: dominant malformation, which seems to be related to traits of other constitutional anomalies, and recessive degeneration, which is the most common type of this disorder in families with consanguinity.2 The dominant variant affects only 12% of cases of corneal dystrophy, where-

received April 22, 2011 accepted April 15, 2013

as 1% of cases are reported as X-linked recessive inheritance disorder.2 Patients usually have bilateral symmetric progressive hearing loss after birth, which is suggestive of an acquired disease. Cawthorne and Hinchcliffe suggested that hearing loss may occur at any time from birth to 30 years of age. The authors classified this type of disease as a heredodegenerative disorder.3 The association of congenital hereditary corneal dystrophy with progressive sensorineural hearing loss suggests the diagnosis of Harboyan syndrome.1 The scarcity of data related to this disease, especially regarding the degree of involvement of the auditory function,

Copyright © 2013 by Thieme Publicações DOI http://dx.doi.org/ Ltda, Rio de Janeiro, Brazil 10.1055/s-0033-1351669. ISSN 1809-9777.

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Rehabilitation with CI in Patient with Harboyan Syndrome prompted us to report the case of a patient diagnosed with Harboyan syndrome. This patient had profound bilateral sensorineural hearing loss and she showed poor hearing performance while using a hearing device. She underwent successful aural rehabilitation with a cochlear implant (CI).

Review of the Literature Hearing and vision are two major sensory modalities related to the development and achievement of successful communication. Several diseases that affect hearing and vision, such as Cogan syndrome and Harboyan syndrome, have been described in the literature. The latter is a rare inherited disease manifested by eye lesions and progressive sensorineural hearing loss.1,2 Patients with diseases that affect different sensory organs need to be followed by a multidisciplinary team with the purpose of prevention, diagnosis, and treatment. Thus, speech therapists and otolaryngologists should be members of such a team so that clinical and therapeutic effectiveness could be achieved to improve the patients’ quality of life.3,4 There is a scarcity of data on Harboyan syndrome, especially with regard to hearing impairment and possibility of rehabilitation. We could not find another report of a case of hearing loss due to Harboyan syndrome treated with CI in the literature.

Case Report A 25-year-old woman from Porto Alegre, Rio Grande do Sul, Brazil, had an uneventful gestational history and was born by vaginal delivery at term. Her 5-minute Apgar score was 9. She was referred to the Hospital de Clínicas de Porto Alegre (HCPA) for progressive hearing loss. The patient reported that the onset of the disease occurred after 2 years of age, with decreased visual acuity. She started to show progressive hearing loss at the age of 10 years. Otolaryngologic examination showed normal findings on otoscopy. Physical examination of the head and neck also did not reveal any abnormalities. Audiologic evaluation showed symmetrical profound bilateral sensorineural hearing loss, type A tympanometric curves, and absent acoustic reflexes in both ears. Additionally, we found no otoacoustic emissions in both ears and absence of response of brain stem auditory evoked potentials. Additional tests, including STORCH (syphilis, toxoplasmosis, rubella, cytomegalovirus, and herpes simplex), electroencephalogram, brain and temporal bone computed tomography scan, chromosome analysis, urine chromatography for mucopolysaccharidosis, and metabolic screening, did not show any changes. Given the patient’s progressive hearing loss, she had a well-established oral language code. After otolaryngologic and audiologic examination, according to the protocol of the Department of Otolaryngology of the HCPA, the patient underwent aural rehabilitation with the use of bilateral personal sound amplification device (10 hours a day) without success. In mid 2005, we performed a speech examination including communication, language, speech, International Archives of Otorhinolaryngology

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Paniagua et al. and voice.10 In addition, auditory discrimination of vowels, disyllables, trisyllables, and sentences was checked. These sentences were evaluated using either lip-reading or not, based on visual support from gesture and facial expressions. Auditory discrimination was also evaluated using two different methods: open set (predefined speech activity) and closed set (predictable or predefined speech activity). The patient was able to communicate using oral language and the Brazilian Sign Language (Libras). She also demonstrated spontaneous vocabulary, normal speech, and adapted voice (typical of deaf individuals). Nevertheless, she failed all specific tests (discrimination of vowels, disyllables, trisyllables, and sentences) when there was no lip-reading (i.e., the patient could not understand what was being said without visual support from mime and facial expression). Based on these data and because she demonstrated a wellestablished oral language code, the patient was considered a candidate for CI; however, she refused such treatment. In 2006, the patient returned to the Department of Otolaryngology of the HCPA because she decided to undergo hearing rehabilitation according to the treatment proposed. She was then included in the multidisciplinary assessment protocol of the HCPA and the Care Program for Profound/Severe Deaf Patients. The multidisciplinary team consisted of speech therapists, otolaryngologists, psychologists, and social workers. The surgery was performed at the HCPA on May 26, 2006. The procedure was uneventful, and the patient was implanted with a Nucleus 24 CI, ESPrit 3G processor in the left ear. Electrode activation was performed 45 days after the date of the surgery. Then, the patient underwent aural rehabilitation provided by the team of speech therapists. She was followed by the otolaryngologists according to the protocol of the institution. The patient did not have any complications during the follow-up period. During preoperative audiologic procedures, the following results were found in both ears: (1) pure tone audiometry: profound bilateral sensorineural hearing loss; (2) speech audiometry: no voice detection threshold and speech recognition index of 0%; (3) distortion product otoacoustic emissions: absent; (4) brainstem auditory evoked potential: absent; (5) measurements of acoustic impedance: type A tympanometric curves (according to Jerger classification, 1970) and absent acoustic reflexes. We detected proper electrode impedance and presence of response on neural telemetry of all electrodes tested during perioperative procedures. During postoperative audiologic procedures, we found the lowest and highest hearing thresholds of 30 dB and 45 dB, respectively, using freefield audiometry. Postoperative speech examination for communication showed the following results: communication was performed mainly by means of oral language and, less frequently, through sign language. The patient had spontaneous vocabulary, normal speech, adapted voice, and open set communication (i.e., without agreeing with the examiner on what to say). The patient showed improved hearing results, reaching 75% accuracy in sentences without using lip-reading in open


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set tasks and 100% accuracy in sentences without lip-reading in closed set tasks.

Discussion Currently, CI is an excellent option for aural rehabilitation of patients with profound bilateral sensorineural hearing loss, provided that they are eligible for the procedure. When the patient has more than one irreversible sensory deficit in addition to hearing loss, hearing rehabilitation becomes a priority. Puga et al described a progressive worsening of visual and hearing impairment, and sensorineural hearing loss may initially reach high frequencies.5 Chiari et al described a case study of Cogan’s syndrome with associated deafness and blindness.4 The authors reported that professionals from diverse areas should classify individuals with simultaneous visual and hearing loss as “deafblind” people. Such condition cannot be considered a simple sum of sensory losses; instead, it is a multiplying factor by means of which the combination of sensory losses results in serious communication and development problems. Differential diagnoses of the Harboyan syndrome are Hurler disease, congenital glaucoma, Cogan syndrome, and syphilitic interstitial keratitis.6 These disorders were ruled out by additional exams. Inherited hearing loss can be either dominant or recessive, but the latter is the most prevalent type. The pathogenesis of progressive sensorineural hearing loss is not well established. Paparella et al in 1969 described familial progressive sensorineural hearing loss associated with histopathologic findings compatible with marked degeneration of the stria vascularis and reduced ganglion cells mainly in the basal cochlear turn.6,7 However, the audiologic evaluation of these patients usually reveals no recruitment and discreet presence of positive tone decay. These findings are consistent with a greater chance of lesions in the organ of Corti and the auditory nerve (peripheral lesion). Such lesions can be treated with hearing rehabilitation with CI, which partially explains the success of this type of treatment in our patient.

Conclusion As, to the best of our knowledge, this is the first case report of hearing loss caused by Harboyan syndrome treated with CI, it was not possible to perform a comparative analysis of our results with those of other authors. At the time of preparation of this report, the patient has completed 12 months of speech and auditory rehabilitation and is able to recognize 75% of sentences without lip-reading in open set tasks. The patient’s performance in sentence recognition with lip-reading before and after CI according to the proposed speech activity, either in open or closed set tasks, is shown in ►Fig. 1. The patient’s performance in sentence recognition without lip-reading, either in closed or open set tasks, is shown in ►Fig. 2. The rate of 100% means all sentences were recognized. So far, the patient has attended 15 sessions of speech therapy based on auditory-verbal therapy, which is aimed at the development

Fig. 1 Percentage of correct recognition of sentences with lip-reading (WLR) before and after cochlear implant. WLR: visual support from mime and lip-reading. Open set: understanding speech activity without agreeing with the examiner on what to say.

Fig. 2 Percentage of correct recognition of sentences without lipreading (WOLR) before and after cochlear implant. WOLR: without visual support from mime and lip-reading. Open set: understanding speech activity without agreeing with the examiner on what to say. Closed set: understanding speech activity after agreeing with the examiner on what to say.

of auditory and language skills according to Auditory Verbal International.8,9 The deprivation of sight and hearing can cause drastic changes in the person’s access to information and development. Patients with hearing loss who use bilateral personal sound amplification device or CI, according to the individual selection criteria, must be followed in an auditory (re)habilitation program designed to develop or restore the ability of auditory perception with activities that stimulate auditory skills, among them auditory comprehension.4,5,10–13 Auditory rehabilitation reduces the difficulties arising from hearing loss that restrict participation in social activities and that consequently result in a poorer quality of life.13 It is noteworthy that the auditory-verbal therapy focus on rehabilitation benefits individuals with or without genetic syndromes, blindness, and low vision.13–15 Patients with progressive hearing loss seem to respond better to CI than patients with sudden deafness as to the duration of hearing loss.10–12 Because our patient had visual International Archives of Otorhinolaryngology

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and hearing loss, rehabilitation treatment was imperative, and CI has proven to be the best therapeutic option to improve the patient’s quality of life thus far.

8 Easterbrooks SR, O’Rouke C. Gender differences in response to

9 10

References 1 Harboyan G, Mamo J, Karam F, Karam FA. Congenital corneal

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dystrophy. Progressive sensorineural deafness in a family. Arch Ophthalmol 1971;85:27–32 Judisch GF, Maumenee IH. Clinical differentiation of recessive congenital hereditary endothelial dystrophy and dominant hereditary endothelial dystrophy. Am J Ophthalmol 1978;85(5 Pt 1): 606–612 Cawthorne TE, Hinchcliffe R. Familial perceptive deafness. Pract Oto-rhino-laryng 1957;19:69–83 Chiari BM, Bragatto EL, Nishihata R, Carvalho CAF. Perspectivas da atuação fonoaudiológica diante do diagnóstico e prognóstico da surdocegueira. Distur Comun São Paulo 2006;18(3):371–382 Puga ACS, Nogueira AHH, Félix TM, Kwitko S. Congenital corneal dystrophy and progressive sensorineural hearing loss (Harboyan syndrome). (Letter) Am J Med Genet 1998;80:177–179 Paparella MM, Sugiura S, Hoshino T. Familial progressive sensorineural deafness. Arch Otolaryngol 1969;90:44–51 Schuknecht HF. Auditory and cytocochlear correlates of inner ear disorders. Otolaryngol Head Neck Surg 1994;110:530–538

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auditory verbal intervention in children who are deaf or hard of hearing. Am Ann Deaf 2001;146:309–319 Eriks-Brophy A. Outcomes of auditory-verbal therapy: a review of the evidence and a call for action. The Volta Review 2003;104:21–53 Goffi-Gomez MVS, Guedes MC, Sant’ana SBG, et al. Critérios de seleção e avaliação médica e audiológica dos candidatos ao implante coclear: HC-FMUSP. Arq Otorrinolaringol 2004;8: 303–323 Guedes MC, Brito RV, Goffi-Gomes MVS, et al. Telemetria de resposta neural intra-operatória em usuários de implante coclear. Rev Bras Otorrinol 2005;71:660–667 Bento RF, Brito Neto RV, Castilho AM, Goffi-Gomez VM, Giorgi SB, Guedes MC. Resultados auditivos com implante coclear multicanal em pacientes submetidos a cirurgia no Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. Rev Bras Otorrinolaringol (Engl Ed) 2004;70:632–637 Freire KGM. Reabilitação de deficientes auditivos adultos. In: Bevilacqua MC, Martinez MAN, Balen SA, Pupo AC, Reis ACMB, Frota S, eds. Tratado de Audiologia. Santos: Ed Santos; 2012:761– 774 Boothroyd A. Adult aural rehabilitation: what is it and does it work? Trends in Amplification 2007;(6):63–71 Política Nacional de Atenção à Saúde Auditiva. Available at: http:// dtr2001.saude.gov.br/sas/PORTARIAS/Port2004/PT-587.htm. Accessed July 22, 2013


Case Report

Dermoid of the Nasopharynx Causing Neonatal Respiratory Distress Denise Manica1,2

Cátia Saleh Neto1,2

Cláudia Schweiger1,2

1 Department of Otolaryngology, Hospital de Clínicas de Porto Alegre,

Porto Alegre/RS, Brazil 2 Universidade Federal do Rio Grande do Sul, Porto Alegre/RS, Brazil

Marcelo Cortina1

Gabriel Kuhl1,2

Address for correspondence Denise Manica, MD, MSc, Avenida João Pessoa, 1051/408, Bairro Cidade Baixa, Porto Alegre/RS, Brazil (e-mail: denisemanica@gmail.com).

Int Arch Otorhinolaryngol 2013;17:407–408.

Abstract Keywords

► ► ► ► ►

dermoid cyst infant newborn nasopharynx nasopharyngeal neoplasms ► respiratory distress syndrome

The dermoid is a rare and benign malformation of bigerminal origin. It is seen frequently in the neonatal period, and the clinical presentation depends on the site and on the size of the lesion, and the classical clinical picture is of a tumor derived from the naso- or oropharynx, leading to respiratory distress and/or feeding disorders. A female newborn was born cyanotic requiring intubation. The oroscopy revealed a large smooth mass. Transoral surgery was performed with successful extubation. Seven months after, there are no signs of recurrence of the lesion. This report adds another case of this rare pathology that can be life-threatening condition in the neonate. It emphasizes the importance of dermoid in the differential diagnosis of the naso- or oropharyngeal lesions, especially in neonates.

Introduction The dermoid, or hairy polyp, is a rare and benign malformation of bigerminal origin with both ectodermal and mesodermal elements. It is seen frequently in the neonatal period, but occasionally it can be found in older children.1–3 The clinical presentation depends on the site and on the size of the lesions, and the classical clinical picture is of a tumor derived from the naso- or oropharynx, leading to respiratory distress and/or feeding disorders. This case report presents one case of dermoid causing respiratory difficulties at birth.

Case Report A 3,050-g female patient was born by spontaneous vaginal delivery at 35 weeks’ gestation. The child was born hypotonic and cyanotic, with Apgar scores 5, 6, and 7. The child required intubation and remained in mechanical ventilation. The mother, 38 years old, attended nine prenatal consultations without gestational problems and with negative serology results, except toxoplasmosis IgG reagent. On physical examination, cardiac, pulmonary, and abdominal examinations were normal. Oroscopy revealed a large

received November 4, 2012 accepted December 28, 2012

smooth mass (►Fig. 1). On the flexible fiberoptic laryngoscopy, it could be seen that the lesion protruded from the left nasopharyngeal wall. Magnetic resonance imaging showed an expansive lesion with signal intensity similar to fat tissue without significant contrast enhancement (►Fig. 2). Transoral surgery with complete excision of the lesion was performed. The histologic analysis described a polyp composed by adipose tissue, fibrosis, skeletal muscle, mature cartilage, and skin compatible with dermoid. The child was extubated in the third day of surgery and remained without respiratory distress. Oral feeding was initiated after 1 week with good acceptance. Now this patient is 7 months old without any signs of recurrence of the lesion.

Discussion The dermoid was first described by Brown-Kelly in 1918.3 One hundred thirty-five cases were reviewed by Kelly et al in 1996.4 After this review, Yilmaz et al in 2012 reviewed 35 more cases, increasing the number of patients reported in the literature to 170 cases.2

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Manica et al.

Dermoids occur six times more frequently in females. The most common location is in the naso- or oropharynx, in the left side. The reason for predilection for females and left side, as in the described case, is unknown.2,3 Dermoids are rarely associated with other congenital abnormalities such as cleft palate, absent uvula, auricle deformities, ankyloglossia, facial hemihypotrophy, atresia of left carotid artery, and osteopetrosis. However, it is important to investigate other congenital malformations. The present case did not present any such associated problems. The differential diagnosis of a neonatal nasopharyngeal mass includes teratoma (different than teratoma in that the dermoid does not have endodermal structures), encephalocele, intranasal glioma, neuroblastoma, hemangioma, and thyroglossal or lingual cyst. The first diagnostic procedure is the flexible fiberoptic laryngoscopy that allows establishment of the size, origin, and extension of the mass. Radiologic studies like computed tomography and magnetic resonance imaging are essential for the surgical planning.3 The initial management focuses on maintaining a secure airway, frequently with endotracheal intubation. The definitive treatment is surgical excision. The histologic analysis is important for the prognosis and follow-up. The dermoid prognosis is excellent, and there were no cases of recurrence or malignant transformation.2,3

Fig. 1 Transoral view of the lesion.

Conclusion This report adds another case of this rare pathology that can be a life-threatening condition in the neonate. It emphasizes the importance of dermoid in the differential diagnosis of the naso- or oropharyngeal lesions, especially in neonates.

Fig. 2 Sagittal magnetic resonance image demonstrating soft tissue lesion in nasopharynx.

References 1 Chakravarti A, Vishwakarma SK, Arora VK, Singh I. Dermoid (hairy

polyp) of the nasopharynx. Indian J Pediatr 1998;65:473–476 2 Yilmaz M, Ibrahimov M, Ozturk O, Karaman E, Aslan M. Congenital

Dermoids are histologically composed by stratified keratinized epithelium with cutaneous structures like hair and sebaceous and sudoriparous glands.2,3 The classical clinical presentation is the visualization of a pedunculated lesion in the pharynx,5 as seen with the patient described here. The respiratory distress can be dramatic with asphyxia at birth or can be subtle with intermittent obstruction. Polyhydramnios during the pregnancy can be seen because of the obstruction of the swallowing mechanism. There can also be vomits, feeding disorders, hemoptysis, eustachian tube disorder, and increased unilateral nasal secretion.6

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hairy polyp of the soft palate. Int J Pediatr Otorhinolaryngol 2012;76:5–8 Kalcioglu MT, Can S, Aydin NE. Unusual case of soft palate hairy polyp causing airway obstruction and review of the literature. J Pediatr Surg 2010;45(12):e5–e8 Kelly A, Bough ID Jr, Luft JD, Conard K, Reilly JS, Tuttle D. Hairy polyp of the oropharynx: case report and literature review. J Pediatr Surg 1996;31:704–706 Roh JL. Transoral endoscopic resection of a nasopharyngeal hairy polyp. Int J Pediatr Otorhinolaryngol 2004;68:1087–1090 Agrawal N, Kanabar D, Morrison GA. Combined transoral and nasendoscopic resection of an eustachian tube hairy polyp causing neonatal respiratory distress. Am J Otolaryngol 2009;30:343–346


Case Report

Stage II Chronic Maxillary Atelectasis Associated with Subclinical Visual Field Defect João Mangussi-Gomes1 Márcio Nakanishi1 Carlos Augusto Costa Pires De Oliveira1

Maria Regina Chalita2

1 Department of Otorhinolaryngology, Head and Neck Surgery,

University Hospital of Brasília, University of Brasília, Brasília/DF, Brazil 2 Department of Ophthalmology, University Hospital of Brasília, University of Brasília, Brasília/DF, Brazil

Fabiana Damasco1

Address for correspondence João Mangussi-Gomes, MD, Department of Otorhinolaryngology, Head and Neck Surgery, University Hospital of Brasília, University of Brasília, SQN 409, Bloco Q, Apto 106, Asa Norte, Brasília/DF 70857-170, Brazil (e-mail: joaopauloemt@gmail.com).

Int Arch Otorhinolaryngol 2013;17:409–412.

Abstract

Keywords

► paranasal sinus diseases ► maxillary sinus ► visual fields ► maxillary diseases

Introduction Chronic maxillary atelectasis (CMA) is characterized by a persistent decrease in the maxillary sinus volume due to inward bowing of its walls. According to its severity, it may be classified into three clinical-radiological stages. Objective To report a case of stage II CMA associated with subclinical visual field defect. Case Report A 34-year-old woman presented with a 15-year history of recurrent episodes of sinusitis and intermittent right facial discomfort for the past 5 years. She denied visual complaints, and no facial deformities were observed on physical examination. Paranasal sinus computed tomography (CT) demonstrated a completely opacified right maxillary sinus with inward bowing of its walls, suggesting the diagnosis of stage II CMA. A computerized campimetry (CC) disclosed a scotoma adjacent to the blind spot of the right eye, indicating a possible damage to the optic nerve. The patient was submitted to functional endoscopic sinus surgery, with drainage of a thick mucous fluid from the sinus. She did well after surgery and has been asymptomatic since then. Postoperative CT was satisfactory and CC was normal. Discussion CMA occurs because of a persistent ostiomeatal obstruction, which creates negative pressure inside the sinus. It is associated with nasosinusal symptoms but had never been described in association with any visual field defect. It can be divided into stage I (membranous deformity), stage II (bony deformity), and stage III (clinical deformity). The silent sinus syndrome is a special form of CMA. This term should only be used to describe those cases with spontaneous enophthalmos, hypoglobus, and/or midfacial deformity in the absence of nasosinusal symptoms.

Introduction Chronic maxillary atelectasis (CMA) is characterized by a persistent decrease in the maxillary sinus volume due to inward bowing of its antral walls.1 Some confusion between CMA and silent sinus syndrome (SSS) exists in the literature. It is suggested, however, that SSS is a form of CMA.2,3 Indeed, according to its severity, CMA may be classified into three successive clinical-radiological stages: stage I, membranous

received November 25, 2012 accepted April 29, 2013

deformity; stage II, bony deformity; and stage III, clinical deformity.1 Although CMA cases are always accompanied by nasosinusal symptoms, the term silent sinus syndrome should only be used when CMA presents with spontaneous enophthalmos, hypoglobus, and/or midfacial deformity in the absence complaints related to nose and paranasal sinuses.2 Some authors affirm that CMA and SSS are never associated with visual field impairment.4 We present a very

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Mangussi-Gomes et al. illustrative case of stage II CMA associated with subclinical visual field impairment, which completely resolved after surgery.

Case Report

Fig. 1 Coronal computed tomography of the nose and paranasal sinuses demonstrates total opacification of the right maxillary sinus. Inward bowing of the medial, superior, and posterolateral walls may also be seen.

A 34-year-old woman presented to the otolaryngology clinics complaining of recurrent episodes of maxillary acute rhinosinusitis, accompanied by facial pain, nasal congestion, and rhinorrhea, for more than 15 years. For the past 5 years, nasosinusal symptoms had partially improved but intermittent and progressive right facial discomfort had developed. General and otolaryngologic physical examinations were unremarkable, except for hyperesthesia to light touch on right malar region. Computed tomography (CT) of the paranasal sinuses was therefore performed (►Figs. 1 and 2). Despite not complaining of visual symptoms, a complete ophthalmologic evaluation was also undertaken. Computerized campimetry (CC) disclosed a scotoma adjacent to the blind spot of the right eye ( ►Fig. 3). Clinical enophthalmos, hypoglobus, and/or facial deformity were not present and the remainder of the ophthalmologic assessment was normal. Based on her clinical and radiologic presentation, she was diagnosed with stage II CMA. She was submitted to functional endoscopic sinus surgery, which included right uncinectomy, bullectomy, and maxillary antrostomy. During the procedure, a thick mucous fluid was aspirated from inside the affected sinus. She did well after surgery and radiologic controls were satisfactory (►Fig. 4). The visual field defect completely resolved, and the patient has been asymptomatic during a 3-year follow-up period.

Discussion Fig. 2 Axial computed tomography of the nose and paranasal sinuses demonstrates total opacification and contraction of right maxillary sinus.

CMA is an acquired and unusual condition that refers to a persistent and progressive decrease in maxillary sinus volume due to an inward bowing of its antral walls.1,2 It seems to affect patients in their late 30s or early 40s, with no gender preference and similar incidences in left and right sides.2

Fig. 3 Computerized campimetry shows a scotoma adjacent to the blind spot of the right eye, possibly indicating damage to the ipsilateral optic nerve. International Archives of Otorhinolaryngology

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Stage II CMA Associated with Subclinical Visual Field Defect

Fig. 4 Postoperative coronal computed tomography of the nose and paranasal sinuses. Wide maxillary antrostomy can be appreciated.

The etiology and physiopathology of CMA are not completely elucidated. It is thought that CMA is an acquired and progressive phenomenon.5,6 The process is probably initiated by a sustained obstruction of the maxillary sinus at the level of the ostiomeatal complex. The resulting hypoventilation of the maxillary sinus leads to gas absorption by the sinus mucosa, generating negative pressure inside the sinus. Consequent accumulation of mucus and chronic subclinical inflammation of the mucosa are responsible for the resorption and remodeling of the adjacent bony walls. Maxillary sinus atelectasis would then occur as a result of the created gradient of pressure acting over osteopenic sinus walls.3,7–9 Clinical presentation of CMA varies according to duration and severity of the disease.1,5 In initial phases, clinical deformities are absent and the diagnosis of CMA is only suggested by characteristic CT findings, which include obstruction of ethmoidal infundibulum and total or near total opacification of sinus content. Variable degrees of inward bowing of sinus walls are also seen.1,5,9–11 In advanced cases, however, CMA presents with evident facial deformity, enophthalmos and/or hypoglobus as major complaints. CT scans reveal the associated severe changes to the maxillary sinus anatomy in these situations. When enophthalmos and/or hypoglobus are present, important orbital floor displacement into the sinus lumen and orbital volume enlargement are found on CT images. 5,9,10

Mangussi-Gomes et al.

Considering its clinic-radiological evolution, CMA can be broken down into three stages, which represent the spectrum of the disease1: stage I, membranous deformity with lateralized soft medial wall; stage II, bony deformity with inward bowing of superior, anterior, and posterolateral osseous walls; stage III, clinical deformity with apparent facial asymmetry, enophthalmos, and/or hypoglobus. CMA is also accompanied by symptoms related to the nose and paranasal sinuses, including facial pain, discomfort or pressure, headaches, congestion, and/or rhinorrhea. Interestingly, nasosinusal symptoms are inversely related to the severity of maxillary sinus deformity.1,5 Although early stages of CMA (stages I and II) are usually associated with history of nasosinusal symptoms, late-stage CMA (stage III) is often asymptomatic at the time of the diagnosis. It is theorized that in advanced cases the absence of important nasosinusal symptoms in initial phases and in the course of the disease would explain the delayed diagnosis and more pronounced bony and aesthetical deformities.1,2,5,12 Considering these aspects, when CMA presents with progressive development of facial asymmetry, enophthalmos, and/or hypoglobus, without past or present history of symptoms related to the nose and sinuses, it should be called silent sinus syndrome. Since this term was coined, SSS and CMA have been described independently in the literature, as if it were completely different conditions.13 Despite that, recent reviews of the literature have indicated that SSS and stage III CMA have the same proposed etiology, pathophysiology, and radiologic features.10,11 Hence, SSS should be considered a quite singular and special form CMA and should be included in the spectrum of the disease, as shown in ►Table 1.2,3 In the case we reported, because the patient presented with subclinical maxillary sinus contraction and opacification, evidenced by CT images, along with past and present history of nasosinusal symptoms, she was better diagnosed with stage II CMA. The diagnosis of SSS would not fit for this case. Interestingly, subclinical visual field impairment was objectively evidenced by CC in the case presented. We hypothesize that because of the contraction of the maxillary sinus there is a consequent descent of the ipsilateral orbital floor and a possible transmission of the negative pressure from the sinus into the orbit. This could impair the vasa nervorum blood flow and subsequently cause a transient ischemic injury to the optic nerve.14 Visual field defect resolved completely after surgery and this would corroborate this

Table 1 Spectrum of the disease CMA/SSSa CMA Terminology

Stage I

Stage II

Stage III

SSS

Deformity

Membranous (soft medial wall)

Bony (osseous walls)

Clinical (enophthalmos, hypoglobus, facial asymmetry)

Nasosinusal symptoms

þþþ

þþ

þ

Abbreviations: CMA, chronic maxillary atelectasis; SSS, silent sinus syndrome. a The diagnosis and differentiation of both conditions are based upon history, physical examination, and characteristic radiological findings.1–3

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Stage II CMA Associated with Subclinical Visual Field Defect hypothesis. This is the first case of CMA so far to be described in association with some sort of visual field impairment and possible damage to the ipsilateral optic nerve.4 CMA/SSS is better managed in a two-step manner. It is first mandatory to relieve the obstruction of the maxillary sinus and restore its normal ventilation. This is usually achieved via functional endoscopic sinus surgery. During the procedure, as the orbital floor and the uncinate process are usually lower and more lateralized, respectively, special attention must be kept not to damage orbital content.3,4,8 The second step consists of correcting clinical deformities whenever present. Reconstruction of the orbital floor is the most commonly performed procedure and various techniques are available for this purpose. As spontaneous resolution of clinical deformity may occur after adequate aeration of the sinus, waiting a few months between the two surgical approaches is highly recommended.2,4,15

Mangussi-Gomes et al. 2 Brandt MG, Wright ED. The silent sinus syndrome is a form of

3 4 5 6

7

8

9 10

Conclusion

11

We described a very illustrative case of stage II CMA. It seems to be the first case of CMA associated with any sort of visual field impairment to be reported in literature. SSS is considered a form of CMA. We highlight the importance of the correct diagnosis and differentiation between these terms.

12

13

14

References

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1 Kass ES, Salman S, Rubin PA, Weber AL, Montgomery WW. Chronic

maxillary atelectasis. Ann Otol Rhinol Laryngol 1997;106: 109–116

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chronic maxillary atelectasis: a systematic review of all reported cases. Am J Rhinol 2008;22:68–73 Hunt SM, Tami TA. Sinusitis-induced enophthalmos: the silent sinus syndrome. Ear Nose Throat J 2000;79:576, 579–581, 584 Annino DJ Jr, Goguen LA. Silent sinus syndrome. Curr Opin Otolaryngol Head Neck Surg 2008;16:22–25 Ende K, Mah L, Kass ES. Progression of late-stage chronic maxillary atelectasis. Ann Otol Rhinol Laryngol 2002;111:759–762 Davidson JK, Soparkar CN, Williams JB, Patrinely JR. Negative sinus pressure and normal predisease imaging in silent sinus syndrome. Arch Ophthalmol 1999;117:1653–1654 Kass ES, Salman S, Montgomery WW. Manometric study of complete ostial occlusion in chronic maxillary atelectasis. Laryngoscope 1996;106:1255–1258 Numa WA, Desai U, Gold DR, Heher KL, Annino DJ. Silent sinus syndrome: a case presentation and comprehensive review of all 84 reported cases. Ann Otol Rhinol Laryngol 2005;114:688–694 Gaudino S, Di Lella GM, Piludu F, et al. CT and MRI diagnosis of silent sinus syndrome. Radiol Med (Torino) 2013;118:265–275 Illner A, Davidson HC, Harnsberger HR, Hoffman J. The silent sinus syndrome: clinical and radiographic findings. AJR Am J Roentgenol 2002;178:503–506 Wise SK, Wojno TH, DelGaudio JM. Silent sinus syndrome: lack of orbital findings in early presentation. Am J Rhinol 2007;21:489–494 Borin A, Cruz OLM, Figueiredo CR. [Síndrome do seio silencioso, uma entidade pouco conhecida: relato de dois casos clínicos e revisão de literatura]. Braz J Otorhinolaryngol 2005;71:26–31 Soparkar CN, Patrinely JR, Cuaycong MJ, et al. The silent sinus syndrome. A cause of spontaneous enophthalmos. Ophthalmology 1994;101:772–778 Thawley SE. The otolaryngologist-ophthalmologist relationship: an historic perspective. Otolaryngol Clin North Am 2006;39:845–853 Sivasubramaniam R, Sacks R, Thornton M. Silent sinus syndrome: dynamic changes in the position of the orbital floor after restoration of normal sinus pressure. J Laryngol Otol 2011;125: 1239–1243


Case Report

Human Syngamosis as an Uncommon Cause of Chronic Cough Janaína Oliveira Bentivi Pulcherio1 Eduardo Oliveira Machado da Silva1 Daniela Pereira Rezende1 Patrícia Bittencourt Barcia Barbeira1 Rosane Siciliano Machado1 Marcos Aurélio Baptista de Oliveira1 1 Department of Otorhinolaringology, Central Hospital of Military

Police of Rio de Janeiro, Rio de Janeiro/RJ, Brazil Int Arch Otorhinolaryngol 2013;17:413–414.

Abstract

Keywords

► cough ► parasites ► larynx

Address for correspondence Janaína Oliveira Bentivi Pulcherio, Department of Otorhinolaringology, Central Hospital of Military Police of Rio de Janeiro, Rua Estácio de Sá, número 20, Estácio, Rio de Janeiro/ RJ 20211-270, Brazil (e-mail: janabentivi.orl@gmail.com).

Introduction Chronic cough may represent a diagnostic challenge. Chronic parasitism of upper airways is an unusual cause. Objective To describe a case of human syngamosis as an uncommon cause of dry cough. Case Report An endoscopic exam performed in a woman suffering of chronic cough revealed a Y-shaped worm in the larynx identified as Syngamus laryngeus. Discussion This nematode parasitizes the upper respiratory tract of many animals including humans. The diagnosis is performed by the examination of the worm expelled by cough or by endoscopy. Endoscopic exam is easy to perform and is essential in the diagnosis of causes of chronic cough, even uncommon entities. Removal is the only efficient treatment.

Introduction The cough is symptom of a great variety of pulmonary and extrapulmonary diseases. It produces several alterations in the individual lifestyle; it is one of the most important causes of search for medical attention, and it may result in high costs on diagnostic exams and medications.1 The dry cough is difficult to diagnose and chronic cough (when it lasts longer than 8 weeks) is even a diagnostic challenge.1,2 Brazilian and international guidelines lead the investigation to the most common causes: asthma, reflux disease, and rhinopathies.1,3 Among several uncommon causes of chronic cough can be parasitism, through the pulmonary cycle or chronic parasitism of upper airways, a rare condition.2 We introduce the case of a patient who presented chronic cough due to parasitism of upper airways.

her throat for 5 months. She had no other symptoms. In this period she had visited many physicians and used several types of medication without improvement of her condition. There was no history of contact with birds, cattle, or even pets. The physical examination showed no abnormalities. In the video laryngoscopy we observed a reddish vermiform organism with active movements, 15 mm in length. It had one end attached to the base of the epiglottis (►Fig. 1). The organism was removed by direct laryngoscopy. It was a Y-shaped worm. The material was sent to biological analysis. It was identified as a couple of Mammomonogamus (Syngamus) laryngeus, a nematode from Syngamidae family. At the 30-day follow-up appointment, the patient had no symptoms. A new laryngoscopy exam was performed and was normal (►Fig. 2).

Discussion Case Report A 41-year-old Caucasian Brazilian woman presented to our hospital with a chronic cough and a foreign body sensation in

received December 28, 2012 accepted February 20, 2013

Nematodes of the family Syngamidae parasitize the upper respiratory tract of cattle, buffaloes, goats, and wild and domestic birds. Humans can be accidentally infected.4,5

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Fig. 1 Video laryngoscopy showing a vermiform organism on the larynx.

Pulcherio et al.

animals through contaminated water and food.7,8 Humans are parasitized at this same way, and the worms develop themselves in larynx or trachea, causing laryngotracheal irritation that results in cough with or without hemoptysis and bronchospasm. It is not clear if these parasites migrate from pharynx to the larynx or if they lodge in the airways after going through the gastrointestinal tract/bloodstream/lung/ larynx cycle.5,8 The blood cell count does not reveal relevant eosinophilia.4,7 The diagnosis is usually made when the worms are expelled during an episode of cough or when visualized in an endoscopic exam.5,7 The treatment consists in the removal of the worm and it is the only effective approach. Some studies report the use of mebendazole and thiabendazole, with favorable outcome.4,7

Conclusion Otolaryngologic causes must be considered in initial management of patients with chronic dry cough after you can eliminate the most common diagnoses. The endoscopic exam of the larynx is easy to perform and is essential in differential diagnosis of a great variety of diseases that curses with chronic cough including parasitism of upper airways. This is a rare entity but must be considered in Brazil where many cases where described.

References 1 II Diretrizes Brasileiras no Manejo da Tosse Crônica. J Bras Pneumol

2006;32(6 Suppl):403–446 2 Neto FXP, Ramos CF, Silva AMT, Santos KAN, Azevedo ACG, Palheta

Fig. 2 Video laryngoscopy after treatment. 3

The species Syngamus laryngeus (Mammomonogamus laryngeus) was first demonstrated in 1899 by Railliet. The first case of human disease caused by this organism was reported in 1913 by Leiper in the West Indies. In Brazil, Travassos described the first case of human disease in Salvador (state of Bahia) in 1921.4,6 There are 100 cases of human infection, and almost all of them were originated in the Caribbean Islands and Brazil.5,6 S. laryngeus is a reddish nematode found in couple, in permanent copula, which is why the pair appears as a Y. The female measures 8 to 23 mm and the male measures 3 to 6 mm.6,7 The life cycle of the parasite includes the elimination of eggs in the soil by coughing or by the stool (when they are swallowed). These eggs hatch and release larvae, which infect

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ACP. Tosse Crônica na Rotina Otorrinolaringológica. Int Arq Otorrinolaringol 2011;15:231–240 Irwin RS, Baumann MH, Bolser DC, et al; American College of Chest Physicians (ACCP). Diagnosis and management of cough executive summary: ACCP evidence-based clinical practice guidelines. Chest 2006;129(1, Suppl):1S–23S Weinstein L, Molavi A. Syngamus laryngeus infection (syngamosis) with chronic cough. Ann Intern Med 1971;74:577–580 Rosen MJ. Chronic cough due to tuberculosis and other infections: ACCP evidence-based clinical practice guidelines. Chest 2006;129 (1, Suppl):197S–201S Freitas AL, De Carli G, Blankenhein MH. Mammomanogamus (Syngamus) laryngeus infection: a new Brazilian human case. Rev Inst Med Trop Sao Paulo 1995;37:177–179 Marinho AC, Tavares W. Rotinas de diagnóstico e tratamento das doenças infecciosas e parasitárias. 2 ed. Rio de Janeiro, Brazil: Atheneu; 2007 Pontes P, Gadelha ME, Gregório LC, Behlau M, Steffen N. Singamose laríngea: apresentação de dois casos. Braz J Otorhinolaryngol 1992;58:294–297


Case Report

Adult Rhabdomyoma of the Larynx Márcia Monteiro Pinho1

Jair de Carvalho e Castro1

1 Department of Otorhinolaryngology, 2nd Infirmary of Santa Casa da

Misericórdia do Rio de Janeiro, Rio de Janeiro/RJ, Brazil 2 Fluminense Federal University, Niteroi/RJ, Brazil

Rosana Grandelle Ramos2 Address for correspondence Dra. Márcia Monteiro Pinho, MD, Rua Lagoa das Garcas 200/706, Barra da Tijuca, Rio de Janeiro/RJ 22793400, Brasil (e-mail: marciapinho@mls.com.br).

Int Arch Otorhinolaryngol 2013;17:415–418.

Abstract

Keywords

► immunohistochemistry ► laryngeal neoplasms ► rhabdomyoma

Introduction Rhabdomyoma is a rare benign tumor derived from skeletal muscles. Laryngeal rhabdomyomas are even rarer, with only approximately 40 reported cases in world literature. Laryngeal rhabdomyomas usually are seen as masses covered by mucosa. They are often solitary asymptomatic tumors, but symptoms such as hoarseness can occur. The radiologic features are usually those typical of benign neoplasms, showing well-delineated borders. The differential diagnoses for laryngeal masses include cysts, laryngoceles, and benign and malignant neoplasms. The diagnosis is usually made using histopathologic findings, but in some cases some difficulties can be found. Immunohistochemical staining is of great value in the differentiation of similar tumors. Treatment of rhabdomyoma is surgical excision. Objective To describe a case of rhabdomyoma of the larynx attended at Santa Casa de Misericórdia do Rio de Janeiro. Case Report A 35-year-old man presented with progressive hoarseness in the preceding year. Laryngoscopy showed a large submucosal tumor at the supraglottic region of the larynx. The neck computed tomography scan confirmed the submucosal mass, with distinct borders. The patient was taken to the operating suite, where endoscopic extirpation of the mass was performed. Initial histologic diagnosis was suggestive of paraganglioma, which was not confirmed by studies with immunohistochemical markers, and diagnosis was changed to adult-type rhabdomyoma. The patient recovered well. His voice returned to normal after 3 months. Discussion Although muscle tumors of the larynx are very rare, rhabdomyoma should be considered when there is a submucosal mass in the larynx.

Introduction Rhabdomyomas (RMs) are rare lesions of striated muscle origin that can be found in cardiac and in extracardiac locations. The latter can be divided into two distinct categories: adult and fetal. Of these two, in the head and neck, the adult type is more common. Laryngeal RMs are extremely rare, with only 40 cases being reported worldwide.1 Adult-type RMs show an absolutely benign behavior and never metastasize.2 The treatment is excision. Recurrences are rare.

received November 16, 2011 accepted March 18, 2012

Review of Literature with Differential Diagnosis Two forms of RM exist: cardiac and extracardiac. Extracardiac RMs represent true rare neoplasms that contain muscle elements. Histologically, these tumors are subcategorized into adult and fetal forms according to their degree of cellular differentiation and maturity. Adult-type tumors are confined to the head and neck region and usually originate from musculature of the pharynx, oral cavity, or less commonly the larynx. RM occurs more frequently in men than women

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(4:1). The reported ages of affected patients have ranged from 16 to 76 years.3 Most reported cases of extracardiac RM have involved solitary masses. Approximately 3 to 10% of adult RMs may be multiple.4 RM virtually has no malignant potential. RMs of the larynx usually appear as mucosal covered masses that vary in size. They frequently are asymptomatic solitary lesions but progressive hoarseness, a foreign-body sensation, and airway obstruction may occur. Dysphagia is also a possible symptom. The radiologic features are usually those typical of benign neoplasms, with well-circumscribed margins with no invasion of surrounding tissue.1 In a few cases, computed tomography (CT) findings may mimic the appearance of the malignant lesions with indistinct borders and blending with adjacent isodense muscle tissue. Magnetic resonance imaging is better at delineating RM from surrounding structures. The differential diagnosis for laryngeal masses includes cysts, laryngoceles, malignant neoplasms such as squamous cell carcinoma and rhabdomyosarcoma, as well as benign neoplasms such as hemangiomas, lipomas, amyloidomas, neurofibromas, and granular cell tumors.5 Histologic analysis is essential for diagnosis. Adult RM have been confused with many other benign tumors as hibernoma, oncocytoma, tumor of salivary glands, paraganglioma, and others,6 but mainly with granular cell tumor.1 Microscopically the adult RM is characterized by large, closely packed, elongated to polygonal cells with pink to bright eosinophilic and finely granular cytoplasm. The nuclei are round to oval, vesicular, usually peripherally located, but may be centrally located. Prominent nucleoli are frequently noted. Cross-striations are characteristic but are not an obligatory feature in light microscopic evaluation.2 They are more easily seen with phosphotungstic acid hematoxylin staining. Ultrastructural studies showed actin filament and Z bands. The diagnosis of RM is histopathologic and can generally be well defined, but some cases can cause difficulties. Immunohistochemistry (IHC) reactions are of particular value in differentiating the similar lesions. The IHC features of RM are identical to those of normal skeletal muscle cells because it shows positivity to MSA (muscle-specific actin), desmin, and myoglobin.6 The positivity of these muscular markers is consistent with RM, as well as the positivity for vimentin, a marker of mesenchymal tissues.2 The main lesion to be differentiated from RM is the granular cell tumor that morphologically consists mainly of smaller cells, has no crossstriations, and is consistently positive for S-100 protein but negative for all myogenous markers IHC preparations.2 RM treatment is surgical resection. This procedure should be as conservative as possible. Local recurrence has been reported and usually results from incomplete resection.5

Fig. 1 The laryngeal bulky lesion.

of the larynx, a large submucosal mass was noted in the left supra glottic larynx (►Fig. 1). The contrast-enhanced CT of the neck revealed a well-demarcated diffusely hyperattenuating submucosal mass on the left supraglottic space (►Fig. 2). The patient was taken to the operating room where the mass was endoscopically dissected. The histopathologic diagnosis was of polygonal cell neoplastic favorable to paraganglioma. The pathologist strongly suggested the complementation with IHC studies. IHC stains for desmin (►Fig. 3) and vimentin (muscle and mesenchymal markers respectively) were positive. Markers for epithelium membrane tumors and S-100 protein for neuroectoderm tumors were negative, thus changing the diagnosis to RM adult type. At a careful revision of the slides, the hematoxylin and eosin sections revealed neoplastic closely packed polygonal cells with deeply eosinophilic vacuolated cytoplasm (►Fig. 4). Nuclei were round, vesicular, centrally located, with prominent nucleoli. Intracytoplasmic and intranuclear hyaline inclusions were seen. Cross-striations, not noted

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Fig. 2 Computed tomography revealed a well-demarcated diffusely hyperattenuating submucosal mass.


Adult Rhabdomyoma of the Larynx

Fig. 3 Strong positive staining for desmin of adult rhabdomyoma ( 400).

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Fig. 6 The larynx aspect 15 months after endoscopic removal of the lesion.

Discussion

Fig. 4 Histology of rhabdomyoma showing closely packed polygonal cells with deeply eosinophilic vacuolated cytoplasm. (Hematoxylin and eosin,  400.)

before, were present in few cells (►Fig. 5). Mitotic activity and necrosis were absent. The patient recovered well. His voice was normal after a period of 3 months. He is free of recurrence at 15 months of follow-up (►Fig. 6).

Fig. 5 Scarcely seen cross-striations. (Hematoxylin and eosin,  400.)

Certainly the adult-type RM of the larynx is an extremely rare benign tumor of myoblastic origin. A literature review allows us to say that only 40 well-documented cases have been reported until today worldwide. In the past, RM was confused with other similar tumors, especially with granular cell tumors. This confusion is due to granular eosinophilic cytoplasm that is common to both diagnoses, although they have a completely different histogenesis. Another important observation of the histologic point of view is that the RM should not be confused with rhabdomyosarcoma, which has a completely different prognosis. The histologic diagnosis should be no problem if adequately prepared slides are available, but as these tumors are very rare and many pathologists are not familiar with this type of tumor, there are reports of substantial difficulties to diagnose them on the first try. We present another case of laryngeal RM in a patient whose diagnosis was not initially set. Although the clinical and pathologic findings should be taken into consideration, we emphasize that IHC plays an important role in the differential diagnosis. Symptoms of laryngeal RM do not differ from those caused by other benign tumors of the larynx, but one should keep in mind the possibility of local airway obstruction when the patient complains of breathing difficulties. The tumor is usually described as a submucosal mass with a smooth surface that can resemble the appearance of a cyst. Laryngoscopy and imaging exams help locate and define the limits of the tumor, but the definitive diagnosis will always depend on the histopathologic studies. The clinical outcome of laryngeal RM adult type is benign and no evidence of metastasis was reported in the literature. Treatment should always be surgical removal, via either endoscopic or external approach, but always preserving the adjacent structures such as the vocal folds and the swallowing apparatus. Recurrence can happen but usually is due to incomplete excision of the tumor. International Archives of Otorhinolaryngology

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Conclusion

2 Hamper K, Renninghoff J, Schäfer H. Rhabdomyoma of the larynx

Although muscle tumors of the larynx are very rare, the RM should be considered when there is a submucosal mass in the larynx. Because the establishment of definitive diagnosis can be difficult with routine histopathology, it is important to consider the realization of IHC studies.

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1 Farboud A, Pratap R, Helquist H, Montgomery P. An unusual cause

of obstructive sleep apnoea. J Laryngol Otol 2009;123:e22

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recurring after 12 years: immunocytochemistry and differential diagnosis. Arch Otorhinolaryngol 1989;246:222–226 Jensen K, Swartz K. A rare case of rhabdomyoma of the larynx causing airway obstruction. Ear Nose Throat J 2006;85:116–118 Zacharia TT, Som PM. Multiple adult rhabdomyomas of the oropharynx, base of the tongue, and floor of the mouth: magnetic resonance findings. Arch Otolaryngol Head Neck Surg 2008;134:892–894 Brys AK, Sakai O, DeRosa J, Shapshay SM. Rhabdomyoma of the larynx: case report and clinical and pathologic review. Ear Nose Throat J 2005;84:437–440 Pichi B, Manciocco V, Marchesi P, et al. Rhabdomyoma of the parapharyngeal space presenting with dysphagia. Dysphagia 2008;23:202–204


Case Report

Atypical Manifestation of Vestibular Schwannoma Guilherme Webster1 Rui Carlos Ortega Filho1 Antonini de Oliveira e Sousa1 Márcio Cavalcante Salmito1 Mariana Lopes Favero1 Patrícia Maria Sens Marques1 1 Department of Otorhinolaryngology, Hospital do Servidor Público

Municipal de São Paulo, São Paulo/SP, Brazil Int Arch Otorhinolaryngol 2013;17:419–420.

Abstract

Keywords

► ► ► ►

neuroma acoustic dizziness hearing loss

Introduction Vestibular schwannoma (also known as acoustic neuroma) is a benign tumor whose cells are derived from Schwann sheaths, which commonly occurs from the vestibular portion of the eighth cranial nerve. Furthermore, vestibular schwannomas account for 8% of intracranial tumors in adults and 80 to 90% of tumors of the cerebellopontine angle. Its symptoms are varied, but what stands out most is a unilateral sensorineural hearing loss, with a low index of speech recognition. Objective Describe an atypical manifestation of vestibular schwannoma. Case Report The 46-year-old woman had vertigo and binaural hearing loss and fullness, with ear, nose, and throat examination suggestive of cochlear injury. After 6 months, the patient developed worsening of symptoms and onset of right unilateral tinnitus. In further exams the signs of cochlear damage remained, except for the vestibular test (hyporeflexia). Magnetic resonance imaging showed an expansive lesion in the right cerebellopontine angle. Discussion This report warns about the atypical manifestations of vestibular schwannoma, which must always be remembered in investigating and diagnosing hearing loss.

Introduction The acoustic neuroma is a benign tumor of the eighth cranial nerve, which commonly affects the portion of the vestibular nerve.1 This tumor represents 8% of all intracranial tumors in adults and 80 to 90% of tumors in the cerebellopontine angle.1,2 The incidence of acoustic neuroma is 1:100,000 people per year,3 but with the evolution of imaging that number has increased because asymptomatic lesions are detected. Its pathogenesis is characterized by a hyperproliferation of Schwann cells, often insidious.1,3 The vestibular schwannoma can occur in two types: sporadic or associated with neurofibromatosis type 2. In the first, the tumor is unilateral and represents 95% of cases, whereas, when associated with neurofibromatosis type 2, the tumor is bilateral and represents the other remaining 5% of cases.4

received February 5, 2012 accepted April 22, 2012

Address for correspondence Guilherme Webster, MD, Hospital do Servidor Público Municipal de São Paulo, Rua Castro Alves, 355—Apto 95, Aclimação, CEP 01532-001, São Paulo/SP, Brazil (e-mail: guilhermewebster@uol.com.br).

The most common manifestation of acoustic neuroma is a unilateral and gradual loss of hearing, and it may be associated with tinnitus.1,4,5 Due to the slow tumor growth, there is enough time for the occurrence of vestibular compensation and adjustment, causing infrequent vertigo symptoms.4 For the diagnosis, it is necessary to use imaging methods during the investigation, especially computed tomography and magnetic resonance imaging, both with emphasis on cerebellopontine angle to check the internal auditory canal— the main location of emergence of vestibular schwannoma. Referring to the differential diagnosis of this disease, meningiomas can be cited; the main differential diagnosis includes Meniere disease, metastases, and tumors of other cranial nerves.4 The aim of this study is to report an atypical manifestation of vestibular schwannoma.

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Fig. 1 Magnetic resonance imaging showing lesion occupying the entire length of the right internal auditory canal (5  6  14 mm) with lateral margin establishing contact with the bottom of the canal and labyrinth without extension.

Case Report The patient, a 46-year-old woman, presented with 2-month history of symptoms of dizziness that lasted several hours. She reported associated nausea, sense of fullness, and binaural hearing loss during the attacks. On physical examination, no change was found. Audiometry showed sensorineural loss at 8 kHz in the right ear, with speech recognition rate of 100% at 50 dB and stapedius muscle reflex present but without recruitment. The otoneurologic examination showed right hyporeflexia. The auditory brainstem response (ABR) showed no signs of retrocochlear disease, and electrocochleography showed signs of endolymphatic hydrops in the left ear. Six months after symptom onset, the patient’s condition worsened, with the appearance of constant tinnitus in right ear and normal ear, nose, and throat examination. A new audiometry showed moderate to severe sensorineural loss, flat, between 1 and 8 kHz, with speech recognition rate of 70% at 45 dB in the right ear and recruitment in stapedial reflex. Magnetic resonance imaging was suggestive of acoustic neuroma, with dimensions of 5  6  14 mm, in the right internal auditory canal (►Fig. 1).

which would also take away the possibility of vestibular schwannoma. In ABR of vestibular schwannoma, we would find an increased interval between waves I and III above 2.3 milliseconds, an increased interval between waves I and V above 4.4 milliseconds, an interaural difference of wave V above 0.4 milliseconds, as well as absence of wave I.4 However, ABR performed in the patient did not show any suggestive changes. The result of the vestibular test case is reported in accordance with the literature, because it showed hyporeflexia on the affected side.4 Thus, due to inconclusive electrophysiologic examination, we turned to magnetic resonance imaging—the gold standard in detecting tumors of the cerebellopontine angle—for help diagnosing the patient.2,3,5

Conclusion This report has warned us about the atypical manifestations of vestibular schwannoma, which should always be remembered in research and diagnosis of hearing loss.

References 1 Park JK, Black PM, Vernick DM, Ramakrishna N. Vestibular

Discussion The vestibular schwannoma is the most common tumor in the region of the internal auditory canal and is often associated with asymmetrical and sensorineural loss associated with a higher prevalence in females. The literature shows that the more frequent hearing change in vestibular schwannoma is a sensorineural loss in high frequencies, associated with absence of acoustic reflex, and a low rate of recognition speech. 1–5 However, as can be seen in the case presented, it has been detected in the presence of stapedial recruitment, which would speak in favor of a cochlear etiology. Moreover, the first consultation revealed a 100% recognition rate of monosyllables,

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schwannoma (acoustic neuroma). Available at: http://www. uptodate.com/contents/vestibular-schwannoma-acoustic-neuroma. Accessed January 28, 2012 Zanoni A, Cruz OLM. Schwannoma vestibular. In: Ganança FF, Ponte P, eds. Manual de otorrinolaringologia e cirurgia de cabeça e pescoço. 1st ed. São Paulo, Brazil: Editora Manole; 2011:577–589 Lin D, Hegarty JL, Fischbein NJ, Jackler RK. The prevalence of “incidental” acoustic neuroma. Arch Otolaryngol Head Neck Surg 2005;131:241–244 Swensson RC, Swensson RP, Pizzini FEC, Boldorini PR, Jorge Júnior JJ. An uncommon presentation of a VIII nerve tumor. Braz J Otorhinolaryngol 2008;74:628–631 Coca Pelaz A, Rodrigo JP, Llorente JL, Gómez JR, Suárez C. [Unusual clinical presentations of vestibular schwannomas]. Acta Otorrinolaringol Esp 2008;59:187–189


Case Report

Giant Nasolabial Cyst Treated Using Neumann Incision: Case Report Alexandre Beraldo Ordones1 Larissa Neri1 Ingrid Helena Lopes Oliveira1 Fábio de Rezende Pinna1 Richard Louis Voegels1 1 Department of ENT, University of Sao Paulo School of Medicine, São

Paulo/SP, Brazil

Miguel Soares Tepedino1

Address for correspondence Alexandre Beraldo Ordones, MD, Rua Dr. Eneas de Carvalho Aguiar, 255/6o andar, 6167, Cerqueira Cesar, São Paulo/SP, Brazil (e-mail: ordones.alexandre@gmail.com).

Int Arch Otorhinolaryngol 2013;17:421–423.

Abstract

Keywords

► cysts ► nasal obstruction ► nasal cavity

Introduction A nasolabial cyst is an ectodermal development cyst. It presents as a fullness of canine fossa, nasal ala, or vestibule of the nose. It is rare and usually small. Treatment consists of complete surgical excision or transnasal endoscopic marsupialization. Objective To describe a giant nasolabial cyst case treated using Neumann incision. Case Report A 37-year-old man was referred to the otolaryngology department with nasal obstruction and nasal deformity. Computed tomography showed a nasal cystic lesion 4  4.5  5 cm wide. Surgical excision using Neumann incision was performed. Discussion Neumann incision provides wide access to the nasal cavity and may be useful in nasolabial cyst treatment.

Introduction Nasolabial cysts are also known as nasoalveolar cyst or Klestadt cyst and were described in 1882 by Zuckerkandl.1,2 They are rare,3 affecting 1.6 per 100,000 persons per year.2 They occur more frequently in females (4:1), especially among African Americans, in the fourth and fifth decades of life.2,4,5 In 90% of the cases, they are unilateral.5 Their growth is slow and painless, so they are often underdiagnosed.1–3,6 Patients typically complain of deformity and nasal obstruction. Signs and symptoms include nasal obstruction, local pain, swelling, and facial deformity.1,2,4,7 Usually, there is a fullness of the canine fossa, the nasal ala, and nasal vestibule. The bulging reaches the nasal cavity beneath the anterior third of the inferior turbinate, resulting in obliteration of the nasolabial fold and elevation of the alae of the nose.2 Because of its close anatomical relation to the nasal cavity and teeth, it may become infected easily. When it is infected, it grows quickly and may be painful.1 A nasolabial cyst is diagnosed by clinical examination and is confirmed by histopathologic study.1,2 Usually, the nasolabial fold is obliterated. The cysts must be palpated bimanually with one finger in the floor of the nasal vestibule and another

received May 18, 2012 accepted August 19, 2012

in the labial sulcus. Imaging tests, like computed tomography (CT) of the paranasal sinuses and nuclear magnetic resonance (NMR), may be useful.1,8 CT shows a cystic lesion located anterior to the piriform aperture; its contents may be homogeneous. Cysts are hyperintense on T1 and isointense with cerebrospinal fluid on T2-weighted images at NMR, without changes after fat suppression.1,8 Differential diagnosis includes cysts of the nasopalatine duct, periapical inflammatory lesions (granuloma, cyst, or abscess), and epidermoid or epidermal inclusion cysts.1 Complete surgical excision of the nasolabial cyst is the best treatment. The most used incision is the sublabial.2–4,7 Our aim is to describe the Neumann incision to treat a giant nasolabial cyst.

Case Report A 37-year-old black man had bilateral nasal obstruction, which improved with saline nasal lavage and nasal corticosteroids. Two years later, he presented with bulging of the nasal vestibule, nasal deformity and asymmetry, and worsened nasal obstruction. Hyposmia, dysgeusia, tenderness, and headache were related.

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Puncture of the right nasal vestibule was performed, removing 60 mL of serous liquid, relieving the symptoms. However, the lesion recurred with worsening of symptoms. CT was performed and a cyst lesion anterior to the right pyriform aperture, 4  4.5  5.5 cm wide, was found, pushing the nasal septum to left and bulging the palate (►Fig. 1). Complete surgical excision of the cyst was performed using the Neumann incision (►Fig. 1). Histopathologic study revealed squamous and respiratory epithelium with chronic inflammatory process associated with histiocytic reaction. Culture of the secretion isolated Streptococcus viridans. The patient currently has no evidence of recurrent disease at 2 years postoperatively (►Fig. 2).

Discussion The origin of nasolabial cysts is controversial. There are two main theories about its growth. The first suggests that the cysts derive from inclusion cysts, secondary to mesenchymal cells after the fusion of medial and lateral nasal prominences to the maxillary prominence during facial skeleton formation. The other theory suggests the cyst is an epithelial remnant of the nasolacrimal duct, running between lateral nasal and maxillary prominences.1,2 The most common histologic type is pseudostratified columnar epithelium, followed by squamous stratified epithelium and simple cuboidal epithelium.7 Su et al assessed 10 cysts on electronic microscopy and noticed the cyst had a highly plicated mucosa and were made of nonciliated stratified columnar epithelium, including basal and goblet cells, structurally different from the ciliated

Fig. 1 Computed tomography (1 and 4) and surgical aspects (2 and 3).

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columnar epithelium of the paranasal and nasal sinuses and airways.9 The treatment of nasolabial cysts consists of complete removal, aiming to prevent infections, define histologic type, and improve esthetics. Fine needle aspiration and cauterization are other treatment options available, but these techniques carry a high recurrence rate.1 As reported in the case, cyst puncture for pain relief should always be considered. Endodontists currently use Neumann incision in performing alveoloplasties. It became popular as an alternative to approaching the maxillary sinus in 1970, replacing CaldwellLuc technique.3 It consists of incision in the free edge of the gingiva in the region of the interdental papillae, from the medial portion of the lateral incisor to the lateral portion of the second premolar and first molar, with two vertical extensions, one medial and other lateral to the gingiva and labia, with subsequent elevation of the flap created, allowing full access to the pyriform aperture (►Fig. 2). After the intervention, the mucosal flap is put back into its original position, and the papillae are sutured with absorbable sutures and atraumatic needle, as well as the vertical extensions. The incision considers the vessels and nerves in the region; therefore, the local sensory disturbances such as bleeding are minimal. The potential complications are facial swelling, insensitive gingiva, teeth numbness, and surgical site infection.3 The patient must be oriented to blow his or her nose and use a toothbrush on the surgical site. Diet should be reintroduced slowly for the first week. Dental prostheses can be used immediately after the surgery.3


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Ordones et al.

Fig. 2 (1) Postoperative aspect. (2 to 4) Three months after the surgery.

Choi et al described a series of cases in which the cysts varied in size from 1  1 cm to 3  5 cm.7 In the case reported, the cyst measured 4  4.5  5 cm, and during follow-up, no signs of recurrence or complications were noticed.

Conclusion Despite its rarity, nasolabial cysts should be considered in differential diagnosis when there is swelling of the floor of the nasal cavity or vestibule. The Neumann incision permits good access to the pyriform aperture and the complete cyst excision.

3 Lee JY, Baek BJ, Byun JY, Chang HS, Lee BD, Kim DW. Comparison

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References

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1 Yuen HW, Julian CY, Samuel CL. Nasolabial cysts: clinical features,

diagnosis, and treatment. Br J Oral Maxillofac Surg 2007;45: 293–297 2 el-Din K, el-Hamd AA. Nasolabial cyst: a report of eight cases and a review of the literature. J Laryngol Otol 1999;113:747–749

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of conventional excision via a sublabial approach and transnasal marsupialization for the treatment of nasolabial cysts: a prospective randomized study. Clin Exp Otorhinolaryngol 2009;2: 85–89 Marcoviceanu MP, Metzger MC, Deppe H, et al. Report of rare bilateral nasolabial cysts. J Craniomaxillofac Surg 2009;37: 83–86 Aquilino RN, Bazzo VJ, Faria RJ, Eid NL, Bóscolo FN. Nasolabial cyst: presentation of a clinical case with CT and MR images. Braz J Otorhinolaryngol 2008;74:467–471 Tiago RSL, Maia MS, do Nascimento GMS, Correa JP, Salgado DC. Nasolabial cyst: diagnostic and therapeutical aspects. Braz J Otorhinolaryngol 2008;74:39–43 Choi JH, Cho JH, Kang HJ, et al. Nasolabial cyst: a retrospective analysis of 18 cases. Ear Nose Throat J 2002;81:94–96 Neves-Pinto RM, Lima PE. Incisão na borda livre da gengiva para o acesso ao seio maxilar: um comentário após oito anos de experiência. Braz J Otorhinolaryngol 1981;47:53–58 Su CY, Huang HT, Liu HY, Huang CC, Chien CY. Scanning electron microscopic study of the nasolabial cyst: its clinical and embryological implications. Laryngoscope 2006;116:307–311

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Case Report

Cochlear Implants in Children Diagnosed with CHARGE Syndrome Carolina Costa Cardoso1 Michelle Sales de Meneses1 Angela Maria Vaccaro Silva Alves3 1 Audiologist; Speech Therapy, University Center Federal District

(UNIPLAN), Brasília/DF, Brazil 2 Audiologist; Speech Therapy, University of Brasília (UnB), Brasília/DF, Brazil 3 Audiologist; Speech Therapy, Pontifical Catholic University (PUCSP), São Paulo/SP, Brazil

Isabella Monteiro de Castro Silva2

Address for correspondence Carolina Costa Cardoso, 22 South Street, Lot 08 apartments 1706, Machado de Assis, Clear Waters, CEP 71925720, Brazil (e-mail: cardoso_carol@yahoo.com.br).

Int Arch Otorhinolaryngol 2013;17:424–428.

Abstract

Keywords

► ► ► ►

charge syndrome cochlear implants hearing language

Introduction The CHARGE association (coloboma of the eyes; heart disease; atresia of the choanae; retarded growth and development; genital hypoplasia/genitourinary anomalies; ear anomalies and/or hearing loss) was first described in 1979 by Hall, and among its main features is hearing loss. This study presents a case aiming to establish relationships between performance on Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS) and Meaningful Use of Speech Scales (MUSS) tests and the analysis of hearing and language categories of a patient diagnosed with CHARGE syndrome, before and after cochlear implant (CI) surgery. Case Report A 7-year-old girl was diagnosed with CHARGE. She had severe sensorineural hearing loss and was a prelingual unilateral CI user. We analyzed data from the patient’s medical records regarding therapies and video recordings. Results The patient showed positive results in all evaluations after CI. IT-MAIS rose from 5 to 90% following the use of CI. MUSS also rose, from 75 to 72.5%, after use of CI. Classification of Auditory Skills changed from category 1 before use of CI to category 6 after use of CI. Classification of Language Skills changed from category 1 before use of CI to category 3 after use of CI. The CI is an aid but there are many factors in the therapeutic process, and great heterogeneity in individuals diagnosed with CHARGE should be investigated. Conclusion The development of listening and language skills after CI use was demonstrated by IT-MAIS and MUSS tests, and categorization of speech and hearing in this child with a diagnosis of CHARGE syndrome shows that CI can be an effective technological resource to provide information on hearing as one source for language construction.

Introduction and Literature Review The CHARGE association, or CHARGE syndrome, was first described in 1979; in 1981 the acronym CHARGE (coloboma of the eyes; heart disease; atresia of the choanae; retarded growth and development; genital hypoplasia/genitourinary

received November 23, 2011 accepted March 18, 2012

anomalies; ear anomalies and/or hearing loss) was proposed to portray this set of findings.1 The auditory sensory deficit may jeopardize learning, especially due to injury in the acquisition and development of oral language, which varies depending on the type and degree of hearing loss. Even a mild loss can interfere with oral

Copyright © 2013 by Thieme Publicações DOI http://dx.doi.org/ Ltda, Rio de Janeiro, Brazil 10.1055/s-0033-1352500. ISSN 1809-9777.


CIs in Children Diagnosed with CHARGE Syndrome language development of children and their academic success. There is no doubt, however, that children diagnosed with severe and profound hearing loss are more susceptible to significant lags in language acquisition and in the educational process.2,3 Cochlear implants (CIs) are electronic devices of high biomedical technology, developed to perform the function of cochlear hair cells that are damaged or missing. CIs provide direct electrical stimulation of the remaining nerve fibers in individuals with severe and profound hearing loss, allowing the transmission of electrical signal to the auditory nerve to be decoded by the cerebral cortex. The CI provides the feeling of listening and the quality required for the perception of speech sounds.4 Considering the use of CIs in patients diagnosed with CHARGE syndrome, it is important to pay attention to variation of temporal bone anomalies and anatomical variations of facial nerve pathway, factors that may hamper or prevent surgery.5 CI centers are gaining more experience and application criteria for surgery are in expansion, but children with additional disabilities continue to be topic of discussion. Many centers perform implant surgery in children with additional disabilities, but this population is very diverse and presents unique challenges. The literature suggests that 30 to 40% of children with sensorineural hearing loss have an additional disability.6 Therefore, it is vitally important to monitor the progress and assess the evolution in these cases, as well as to disseminate the scientific results, to aid in decision making of challenging cases with multiple disabilities. To verify the hearing abilities in very young children, we used the Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS) questionnaire, adapted by Castiquini and Bevilacqua.7 This is a substantial auditory integration scale for young children, which also searches children’s spontaneous auditory behaviors in daily life situations, using examples in three different areas of the auditory skills development. These areas include changes in vocalization associated with device usage, being alert to environmental sounds, and assigning meaning to sounds. Using information provided by parents, the examiner scores each question by the frequency of behavior occurrence that ranges from 0 (“Never shows this behavior”) to 4 (“Always shows this behavior”). For evaluation of development of language skills, we used an adaptation of the Meaningful Use of Speech Scales (MUSS), which is an oral language assessment questionnaire with closed questions whose main objective is to evaluate the spoken language use by the child.8 The experience of using CIs in children with CHARGE syndrome is still incipient and the international literature

Cardoso et al.

reports few clinical cases, as in the studies of Bauer et al,9 Lanson et al,10 Southwell et al,11 and Meinzen-Derr et al.12 This study presents a case aiming to establish relationships between the performance in IT-MAIS and MUSS tests and the analysis of hearing and language categories of this patient with a diagnosis of CHARGE syndrome, before and after CI surgery, in an attempt to contribute to these results about the expectations for other similar cases.

Case Report This project was approved by the ethics committee and research at the Catholic University of Brasilia, under protocol 241/2010. Upon approval, data collection was conducted during March and April 2011. The subject of this study was a 7-year-old girl diagnosed with severe sensorineural hearing loss, which occurred before language acquisition—in other words, she was a prelingual unilateral CI user and diagnosed with CHARGE syndrome. The main way of communicating with the survey participant was through spoken language. The survey participant and her guardian consented to take part in the survey. At the time the research was performed the participant attends regular school and is in her second year (old third grade of elementary school) and is monitored twice a week. The patient has speech therapy sessions twice a week, lasting 1 hour, with family participation. Data from medical records of patient, comprising the record of the therapies and video recordings of therapy sessions, were analyzed. The participant and the family answered questions on the IT-MAIS and the MUSS.7,8 Upon collection, the data gathered before and after cochlear implantation were compared. The hearing and language development were classified according to categories of hearing and language.13,14 Data collected were predominantly descriptive because it is a cross-sectional study comparing the data obtained prior to cochlear implantation with the data obtained after a certain time of CI use.

Results In analyzing the records, the patient has most features of CHARGE syndrome (►Table 1). The patient medical records included the date the CI was activated, sensorial privation time (from birth), and time of auditory development as described in ►Table 2. The results of applying the assessment before surgery (2007) and the most current data (2011) are presented in ►Tables 3 and 4. Regarding the evolution of language categories (►Table 5), in 2007, without CI, the patient’s performance was rated category 1, and in 2011, using CI, the performance was rated

Table 1 CHARGE syndrome main features Coloboma

Cardiac alteration

Atresia of the choanae

Retarded growth and development

Genital hypoplasia

Hear alteration

Yes

Yes

Yes

Yes

No

Yes

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Table 2 Survey records Current age

Age at CI activation

Sensorial privation time

Time of auditory brain development

7 y, 9 mo

4 y, 4 mo

4 y, 4 mo

3 y, 5 mo

Abbreviation: CI, cochlear implant.

category 3 (►Table 5). In the categories of hearing (►Table 6), in 2007, without the CI, the patient was in category 1, and in 2011, with the use of CIs, the patient was in category 6. With a hearing age (time of use/CI activation) of 3 years and 5 months, the patient is currently in the highest category of hearing, category 6, recognition of open-set word. This child is able to hear words out of context and extract sufficient phoneme information, and she can recognize words solely through hearing, which differs from results found in 2007 without the use of CI, when the patient was in category 1, detection. This child detects presence of the speech signal.

Discussion The child in this study presented positive results in all evaluations after cochlear implantation, as expected from the literature with nonsyndromic cases. The child made significant gains in the IT-MAIS, which is a scale designed to access the significance of hearing loss for the child in the use of sounds in a daily life situation; her scores rose from 5 to 90% following the use of CIs. Bauer et al studied six patients diagnosed with CHARGE syndrome who used CIs; the IT-MAIS was used in only two patients, one of whom progressed within 6 months from 7.5 to 77.5% and the other patient from 7.5 to 15% after 6 months of implantation.9 Lanson et al, in study with 11 children diagnosed with CHARGE syndrome, concluded that the patients had varying results and some limited degrees of hearing benefit from CIs.10 These authors also emphasized the importance of counseling parents regarding realistic expectations from CIs. Regarding the MUSS, which is a structured interview with parents and covers information about the frequency with which the child shows significant oral language behaviors in his or her daily routine, the results post–cochlear implantation were lower compared with the results of IT-MAIS, rising from 7.5 to 62.5%, demonstrating that the patient had greater oral communicative abilities upon activation of the CI. The patient reached category 6 of hearing after cochlear implantation, which is equivalent to a step more advanced listening. Comprehension is the more refined hearing ability,4

because it requires that the individual understands the meaning of the message. To understand, the subject must possess the domain of listening skills mentioned earlier, such as detection, discrimination, and recognition. The patient must be able to hear words out of context and extract sufficient phoneme information and to recognize the word solely through hearing. Bauer et al, in a study of children diagnosed with CHARGE syndrome, found a patient who after a year of CI advanced to the recognition ability and another patient who after 4 years of CI use progressed to the comprehension skill in an open hearing, equivalent to the category 6.9 In the present survey, the patient, after 3 years and 5 months of CI use, progressed to the hearing comprehension in open set. As seen in ►Table 6, the patient progressed in oral language, although the gains were small, leaving category 1 (“This child does not speak and can present undifferentiated vocalization”) and moving into category 3 (“This child builds sentences”). The child in this study has deficits including several factors cited as features of CHARGE syndrome, which may justify the slow progress in the language category. The development of listening and language skills depends on several factors, such as15: • degree and time of hearing loss • age at detection and intervention • the child’s characteristics: cognitive style, ability to build language, psychological aspects (memory and attention), and emotional development • family characteristics: attitudes and skills of parents and siblings, suitable environment, acoustic environment at home and school context, which favors the development of listening skills • therapist and/or teacher properly performing work In hearing impaired patients, additional disabilities may impact the development of language as related to the use of CIs,16 and children with multiple disabilities are more prone to additional use of Total Communication,17 which is an educational philosophy for the deaf that has an oral and manual code (such as spontaneous gestures, manual alphabet, Portuguese flagged, and others), compared with children with only one additional disability.

Table 3 Ranking of the IT-MAIS after 3 y and 5 mo of cochlear implant use Table 4 MUSS score 2007, without cochlear implant

2011, using cochlear implant

5%

90%

Abbreviation: IT-MAIS, Infant Toddler Meaningful Auditory Integration Scale. Source: Castiquini and Bevilacqua.7 International Archives of Otorhinolaryngology

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2007, without cochlear implant

2011, using cochlear implant

7%

62.5%

Abbreviation: MUSS, Meaningful Use of Speech Scales. Source: Nascimento and Bevilacqua.8


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Table 5 Classification of language skills Categories

2007

1

Without cochlear implant

2011

Language development This child does not speak and can present undifferentiated vocalization.

2

This child speaks only isolated words.

3

This child constructs simple sentences of 2 or 3 words (after cochlear implants).

4

This child constructs sentences with 4 or 5 words and uses connecting elements (pronouns, articles, prepositions).

5

With the cochlear implant

This child constructs sentences of more than 5 words, using connecting elements, conjugating verbs, plurals, etc. She is fluent in oral language.

Source: Proposed by Bevilacqua et al.14

Table 6 Classification of auditory skills Categories

2007

2011

0 1

Auditory ability This child does not detect speech in situations of normal conversation (speech detection threshold of > 65 dB).

Without cochlear implant

Detection: This child detects the speech signal.

2

This child differentiates between words by suprasegmental features (duration, tone, etc.); example: hand versus shoe, house versus boy.

3

Starting word identification: This child differentiates between closed-set words based on phonetic information. This pattern can be demonstrated with words that are identical in length but contain multiple spectral differences; example: refrigerator versus bike, cat versus home.

4

Identification of words through vowel recognition: This child differentiates between closed-set words that differ primarily in the vowel sound; example: foot, powder, shovel; hand, my, me.

5

Identification of words through consonant recognition: This child differentiates between closed-set words that have the same vowel sound but contain different consonants; example: hand, bread, so, dog, floor.

6

With cochlear implant

Recognition of open-set word: This child is able to hear words out of context and extract sufficient phoneme information, and recognize the word solely through hearing.

Source: Geers.13

The patient receives speech therapy by the Aurioral method, which prioritizes hearing as described by Bevilacqua and Formigoni.15 As the patient of this study shows characteristics other than the hearing disability, the Total Communication method could assist her in developing listening skills and language. The CI is an aid, but there are many factors in the therapeutic process, and great heterogeneity in individuals diagnosed with CHARGE syndrome should be investigated, not only to enable the development of appropriate auditory behavior but to ensure that this influence more positively impacts the development of language.

Conclusion The development of listening and language skills were demonstrated by gains in IT-MAIS and MUSS and categorization of speech and hearing in this child with a diagnosis of CHARGE syndrome; this study shows that CIs can be an effective technological resource to provide information on hearing as one source for language construction.

References 1 Pagon RA, Graham JM Jr, Zonana J, Yong SL. Coloboma, congenital

heart disease, and choanal atresia with multiple anomalies: CHARGE association. J Pediatr 1981;99:223–227 International Archives of Otorhinolaryngology

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2 Fortunato CAU. RDLS: Uma opção para analisar a linguagem de

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crianças surdas usuárias de implante coclear [dissertation]. São Carlos, Brazil: Universidade Federal de São Carlos; 2003:109 Verdu ACMA. Funções simbólicas em pessoas submetidas ao implante coclear: uma análise experimental do ouvir [dissertation]. São Carlos, Brazil: Universidade Federal de São Carlos; 2004:214 Costa OA, Bevilacqua MC, Amantini RCB. Considerações sobre o implante coclear em crianças. In: Bevilacqua MC, Moret ALM, eds. Deficiência Auditiva: Conversando com familiares e profissionais de saúde. 1st ed. São José dos Campos, Brazil: Pulso; 2005:123–137 Wright CG, Brown OE, Meyerhoff WL, Rutledge JC. Auditory and temporal bone abnormalities in CHARGE association. Ann Otol Rhinol Laryngol 1986;95(5 Pt 1):480–486 Roberts C, Hindley P. The assessment and treatment of deaf children with psychiatric disorders. J Child Psychol Psychiatry 1999;40:151–167 Castiquini EAT, Bevilacqua MC. Escala de integração auditiva significativa: procedimento adaptado para a avaliação da percepção da fala. Rev Soc Bras Fonoaudiol 2000;6:51–60 Nascimento LT, Bevilacqua MC. Uma proposta de avaliação da linguagem oral [master’s thesis]. Bauru, Brazil: Universidade de São Paulo; 1997 Bauer PW, Wippold FJ II, Goldin J, Lusk RP. Cochlear implantation in children with CHARGE association. Arch Otolaryngol Head Neck Surg 2002;128:1013–1017 Lanson BG, Green JE, Roland JT Jr, Lalwani AK, Waltzman SB. Cochlear implantation in Children with CHARGE syndrome:

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therapeutic decisions and outcomes. Laryngoscope 2007;117: 1260–1266 Southwell KE, Bird PA, Murray DP. Cochlear Implantation in children with CHARGE syndrome. Cochlear Implants Int 2010;11:170–183; erratum in: Cochlear Implants Int 2010; 11:241 Meinzen-Derr J, Wiley S, Grether S, Choo DI. Children with cochlear implants and developmental disabilities: a language skills study with developmentally matched hearing peers. Res Dev Disabil 2011;32:757–767 Geers AE. Techniques for assessing auditory speech perception and lip reading enhancement in young deaf children. The Volta Review 1994;96:85–96 Bevilacqua MC, Delgado EMC, Moret ALM. Estudos de casos clínicos de crianças do Centro Educacional do Deficiente Auditivo (CEDAU), do Hospital de Pesquisa e Reabilitação de Lesões LábioPalatais—USP. In: Costa OA, Bevilacqua MC, eds. XI Encontro Internacional de Audiologia. Anais. 1996 Mar 30 to April 2; Bauru, Brasil; 187 Bevilacqua MC, Formigoni GMP. Audiologia Educacional: Uma opção terapêutica para a criança deficiente auditiva. 3th ed. Barueri, Brazil: Pró-Fono; 2003 Wiley S, Meinzen-Derr, Choo D. Additional disabilities and communication mode in a pediatric cochlear implant population. Int Congr Ser 2004;1273:273–276 Ciccone M. Comunicação Total: Introdução, estratégia, a pessoa surda. 1st ed. Rio de Janeiro, Brazil: Cultura Médica; 1990


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