Fall 2012 Hearing Health

The Ultimate Consumer Resource on Hearing

Hearing Health Fall 2012

Holiday Gift Guide HEARING AIDS FOR ACTIVE LIFESTYLES 10 Signs Your Child Can’t Hear You

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Meet the Grantees

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Hearing Health V O L . 2 8 , N O . 4 , FA L L 2 0 1 2

CONTENTS DEPARTMENTS 4

From the Chairman

6

Hearing Health Foundation News

8

Hearing Headlines

30

44 Arts & Culture 47 Marketplace 50 Meet the Researcher

PEDIATRICS 10 F amily Voices Triple the Fun. A family living by the ocean finally gets to enjoy it to the fullest. Jennifer Lawrence

12 Hearing Health 10 Clues Your Child Has Hearing Loss. A

genetic or progressive loss can be hard to detect. Barbara Jenkins, Au.D.

SENIORS 14 M anaging Hearing Loss Hope to Cope. In some cases, the rare

balance disorder Ménière’s disease may to depression. Mary Alm, Ph.D.

44 Hearing Health magazine (ISSN: 0888-2517) is published four times annually by Hearing Health Foundation. Contact Hearing Health Foundation for subscription or advertising information at: info@hearinghealthfoundation.org or 866.454.3924. Copyright 2012, Hearing Health Foundation. All rights reserved. Articles may not be reproduced without written permission from Hearing Health Foundation. In no way does Hearing Health Foundation nor Hearing Health magazine endorse the products or services appearing in the paid advertisements in this magazine. Further, while we make every effort to publish accurate information, Hearing Health Foundation and Hearing Health magazine are not responsible for the correctness of the articles and information herein.

USPS/Automatable Poly

FEATURES

16

echnology Get Active. Being active is a breeze, thanks to T improvements in hearing aids and accessories. Barbara Jenkins, Au.D.

20

Managing Hearing Loss A Salute to the Veterans. Servicemen discuss their hearing issues, common in the military. Elizabeth Stump

24 Holiday Gift Guide Peace on Earth, Peace of Mind. These select gifts advocate awareness, provide safety, and celebrate the season.

30

esearch The Hearing Restoration Project: What Stops the Inner R Ear From Regenerating? Andy Groves, Ph.D., and Neil Segil, Ph.D.

34 42

Research Presenting the 2012 Emerging Research Grantees.

Living With Hearing Loss Teaching by Example. A carpenter and musician gives hope to children in Africa. Amy Gross WWW.HEARINGHEALTHMAG.COM | FALL 2012 | 3

FROM THE CHAIRMAN

E

ach fall we are thrilled to announce the latest group of exceptional scientists who have been awarded grants as part of Hearing Health Foundation’s Emerging Research program. This year’s crop of 22 talented researchers working to explore new avenues of hearing and balance science is no exception. Read about their work on page 34.

We are also excited to give you an update from Andy Groves, Ph.D., and Neil Segil, Ph.D., two Hearing Restoration Project (HRP) consortium members who are working on hair cell regeneration. On page 30, they share with us their efforts, as part of the HRP’s groundbreaking race to cure deafness in a decade, to encourage the mammalian cochlea to regenerate inner ear hair cells.

While research is a primary focus of Hearing Health Foundation, another is education. On page 12, be sure to read audiologist Barbara Jenkins’s guide to determining whether your child has hearing loss. A new staff writer, Jenkins also reviews technology and accessories that help make sure anyone with hearing loss is not limited when it comes to engaging in physical activity, starting on page 16.

We’ve searched high and low for great holiday gifts that promote hearing loss awareness, education, and safety; the list begins on page 24. And finally, we share with you stories about two young boys with hearing loss and the inspiring efforts the boys’ parents have made on their sons’ behalf. Read about Cormac’s underwater adventures on page 10 and Anthony’s superheroics on page 44. ’Tis the season to hear well. We wish you and your family safe, healthy, and happy holidays!

Warm regards,

Shari S. Eberts

Hearing Health Foundation Chairman, Board of Directors

Hearing Health

V O L . 2 8 , N O . 4 , FA L L 2 0 1 2

Publisher Hearing Health Foundation Andrea Boidman, Executive Director Editor Yishane Lee Art Director Julie Grant Senior Editor Andrea Delbanco Medical Director George A. Gates, M.D. Staff Writers Karen Appold; Samuel R. Atcherson, Ph.D.; Ross Cushing, Au.D.; Amy Gross; Barbara Jenkins, Au.D.; Nannette Nicholson, Ph.D. Advertising Melody Seiling 866.454.3924 advertising@hearinghealthfoundation.org Editorial Committee Stefan Heller, M.D., Ph.D.; Anil K. Lalwani, M.D.; Joscelyn R.K. Martin, Au.D.; Cynthia Morton, Ph.D.; Allen Ryan, Ph.D.; Samuel H. Selesnick, M.D. Board of Directors Shari S. Eberts, Chairman; Mark Angelo; Robert Boucai; Judy R. Dubno, Ph.D.; Rebecca Ginzburg; Roger M. Harris; David S. Haynes, M.D.; Elizabeth Keithley, Ph.D.; Richard Miyamoto, M.D.; Michael C. Nolan; Paul E. Orlin; Peter Steyger, Ph.D.; Clifford P. Tallman, Jr.; Elizabeth Thorp; Nancy M. Williams

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Hearing Health Foundation is a tax-exempt, charitable organization and is eligible to receive tax-deductible contributions under the IRS Code 501 (c)(3).

Federal ID # 13-1882107.

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HHF NEWS

Four-Star Charity We are pleased to announce that Charity Navigator has once again awarded Hearing Health Foundation four out of a possible four stars. Receiving four stars indicates that Hearing Health Foundation adheres to good governance and other best practices that minimize the chance of unethical activities and consistently executes its mission in a fiscally responsible way. America’s premier independent charity evaluator, Charity Navigator helps charitable givers make intelligent giving decisions by providing in-depth, objective ratings and analysis of the financial health, accountability, and transparency of America’s largest charities.

A CNN Close-Up

Researchers: Apply Now Hearing Health Foundation is calling for applications from new research applicants—as well as current grant awardees applying for a second year of funding—who are dedicated to exploring new avenues of hearing and balance science. To learn more about Hearing Health Foundation’s Emerging Research Grants program and submit a letter of intent, see www.hearinghealthfoundation.org/emergingresearch-grants. Read about the nearly two dozen 2012 grantees and the exciting research Hearing Health Foundation is funding on page 34.

New Staff Writer As of this Fall issue, Barbara Jenkins, Au.D., BCABA, has been added to Hearing Health magazine’s masthead as a staff writer. Awarded the 2010 Leo Doerfler Award for Clinical Excellence by the Academy of Doctors of Audiology, Jenkins is Colorado’s first board-certified doctor of audiology and has more than 25 years of hospital and clinical experience in treating patients with hearing loss. See her articles “10 Clues Your Child Has Hearing Loss” on page 12, and “Get Active,” about hearing devices for active lifestyles, on page 16.

Cyndi Lauper in Concert!

Eight-year-old Samantha Brownlie (shown with her brother Sean), a friend of Hearing Health Foundation, was recently featured on a CNN series called the Human Factor, which profiles “survivors who have overcome the odds.” Brownlie was diagnosed with nonsyndromic sensorineural bilateral hearing loss when she was 3 years 6 | HEARING HEALTH | A PUBLICATION OF HEARING HEALTH FOUNDATION

Save the date! A music-filled “Evening with Cyndi Lauper” to benefit Hearing Health Foundation will take place February 6, 2013, in New York City. The colorful ’80s pop singer and MTV pioneering artist is perhaps best known for her hits “Girls Just Wanna Have Fun,” “Time After Time,” and “True Colors.” See www. hearinghealthfoundation.org for more information and to purchase tickets to this exciting event.

PHOTOS COURTESY OF HEARING HEALTH FOUNDATION (LEFT) AND BARBARA JENKINS (RIGHT)

NEWS

old. Brownlie bravely and candidly shared her experience on camera, talking about her hearing loss, wearing hearing aids, and writing her book, “Samantha’s Fun FM Unit and Hearing Aid Book.” Video of the segment can be viewed at www.hearinghealthfoundation.org.

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HEARING HEADLINES

EMBRYONIC STEM CELLS RESTORE GERBIL HEARING Researchers successfully used human embryonic stem cells to restore hearing in gerbils, according to a study published in September in the journal Nature. “We have the proof of concept that we can use human embryonic stem cells to repair the damaged ear,” says lead author Marcelo Rivolta, M.D., Ph.D., a stem-cell biologist at the U.K.’s University of Sheffield.

This is the first time that transplanted human stem cells

have restored hearing in mammals.

The research team injected treated human stem cells into the ears of 18 gerbils with complete deafness in one ear. (The hearing range of gerbils is akin to that of humans.) The gerbils had been exposed to a chemical that damages auditory nerves but not hair cells. Testing 10 weeks later showed that some of the transplanted cells had grown projections that formed connections with the brain stem.

Though the success rate varied, some gerbils showed a 46 percent overall improvement in hearing.

HEARING CAUTION ABOUT PAINKILLERS AND ROCK CONCERTS A study by researchers at Brigham and Women’s Hospital in Massachusetts found that women who took ibuprofen or acetaminophen two or more days a week had an increased risk of hearing loss. Based on data from over 62,000 women, the more often a woman took either medication, the higher her risk for hearing loss.

The risk was greater among women under age 50—especially for those who took ibuprofen six or seven days per week. Aspirin use did not have the same effect on hearing.

The authors, writing in the American Journal of Epidemiology in September, theorize that ibuprofen and acetaminophen reduce blood flow to the cochlea.

HEADLINES

In addition, there is fresh evidence about the danger of loud music on young ears. According to research to be published in the journal Otology & Neurology, a single exposure to loud noise can have long-term effects.

The study found that 72 percent of teenagers tested after attending a loud rock concert experienced temporary, reduced hearing ability.

Of these, one quarter said they were experiencing tinnitus, or ringing in their ears, which they did not have before the concert.

PEOPLE WITH DYSLEXIA MAY HEAR DIFFERENTLY Dyslexia is a disability that occurs when the brain does not properly recognize and process information correctly— transposing letters on a page, for

8 | HEARING HEALTH | A PUBLICATION OF HEARING HEALTH FOUNDATION

Stem cells could eventually broaden the reach of existing therapies. To add to the mounting evidence, gene therapy restored hearing in deaf-born mice, according to study published in the journal Neuron in July.

But more work is necessary to determine safety and long-term effectiveness. The first treatments for people could be at least 15 years off, says Stefan Heller, Ph.D., a stem-cell researcher at Stanford University in California and a member of Hearing Health Foundation’s Hearing Restoration Project consortium.

instance. But there may also be a hearing component to the disorder.

“Any sort of language problem could very well have its roots in perception itself,” says Susan Nittrouer, Ph.D., a child speech and hearing expert at the Ohio State University Wexner Medical Center.

She noticed the role hearing may play in dyslexia after a decade-long study involving more than 100 children who were born deaf or with profound hearing loss. Each child in the study received cochlear implants (CIs), and the CI-wearing children functioned similarly to how children with dyslexia function.

The fact that problems in these children were spotted before they would have been obvious in dyslexic children with normal hearing could lead to a new approach to detecting, decoding, and treating dyslexia.

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FAMILY VOICES

Pediatrics Triple the Fun

With the help of patience, hard work, and technology, a family living by the ocean finally gets to enjoy it to the fullest.

CORMAC WAS BORN AT 31 weeks and five days. He was the third born of the triplets, or Baby C. After Cormac spent 21 days in the hospital feeding and growing, we were told he needed additional hearing tests.

Three sedated auditory brainstem response tests and several otoacoustic emissions tests confirmed Cormac had profound bilateral sensorineural hearing loss; he is profoundly deaf.

I cried a lot after the final diagnosis. I cried because of what I didn’t know: whether he would go to school with his brothers; whether I somehow caused this; or whether he would ever speak or hear my voice.

But when I was told about cochlear implants (CIs), I smiled. I felt hope for Cormac. I was told he was a great candidate and that he would hear, learn to listen, and speak. So at 13 months he received simultaneous bilateral CIs. It was by far the single best decision I have made in my life.

I can’t think about the moment of his initial activation without crying—

this time tears of joy. Cormac asked to hear more sound by signing it.

What most people don’t realize is that the surgery is just the beginning.

You don’t just get implants and then hear. It’s all about the work you and your family put in after the surgery.

It wasn’t just an hour of therapy; it was a way of living. It’s making language and listening the number one priorities in your life.

The next few years were filled with so many amazing moments.

Cormac’s brothers were his greatest supporters. They didn’t know it, but they were his language models.

Before Cormac could hear it was almost like he was a spectator in his own game of life. Once Cormac started hearing, he was part of the team. He was the team captain.

At age 3, Cormac was able to attend preschool with his brothers.

We were thrilled. There was only one thing still not possible at the time: He couldn’t hear in or around the water.

His processors were not waterproof, only water resistant. He even had to take them off for baths.

When he was very young it didn’t seem to be much of an issue, but as he got older it started to bother me. We live near the beach, and I worried about safety. It was dangerous for him to not be able to hear me. Although Ciaran and Colin were his ears for him—they made sure he didn’t miss a helicopter or airplane flying by, and Ciaran, Cormac, and Colin take to the waves. they explained to other

10 | HEARING HEALTH | A PUBLICATION OF HEARING HEALTH FOUNDATION

children that he couldn’t hear but that he could read their lips—a lot of times Cormac played by himself or only with his brothers on the beach.

One day, after sticking his head under the faucet in the bathtub, Cormac said, “Mommy, I know that makes a sound when the water goes over my head because I can feel it. I want to know what it sounds like.”

He was 4. A year later, we learned that Advanced Bionics had come out with the first swimmable processor, the Neptune. We waited years for it to get approved and released.

Finally, we got a pair. It was the middle of February, not exactly the right time of year to go swimming, so Cormac and his brothers got in the bathtub. He was so excited. The first thing he did was stick his head under the faucet. I asked him what it sounded like and he said, “Really loud.” He stuck his head under the water while his brothers talked to him. He kept lifting his head up and saying, “I can hear you!”

Now there is no time Cormac can’t hear, unless he is sleeping. Being able to hear with his CIs has made so many things easier. It has given him more independence. I can’t really put into words how amazing it is for Cormac and our family. He no longer misses out on things on the beach. He talks in the pool and the ocean, and he makes new friends all day long.

Jennifer Lawrence lives with her family in Belmar, New Jersey.

Email editor@hearinghealthmag. com to share your parenting and hearing loss story.

PHOTO COURTESY OF JENNIFER LAWRENCE

“

By Jennifer Lawrence

FM for Real Life

Introducing the new Amigo T30/T31

Ask teachers and audiologists what they expect from FM systems and two words are likely to pop up: Simplicity and Reliability. Ask students the same question and they might reply, “hear the teacher clearly”.

Amigo T30 and T31 FM educational transmitters offer these benefits and more:

Keeping it simple – In-the-palm fitting and programming makes PC’s and cables redundant Hearing more speech – Broad 8.5 kHz bandwidth provides more high frequency speech cues Inspiring confidence – Status LEDs in the receiver and transmitter Built to last – Robust construction withstands real life school settings For more information about Amigo FM visit www.amigofm.com or contact your hearing healthcare professional.

Amigo – FM made friendly!

A m WWW.HEARINGHEALTHMAG.COM | FALL 2012 | 11

Pediatrics

HEARING HEALTH

10 Clues Your Child Has Hearing Loss

Being aware of developmental milestones and watching for signs can help you make sure your child’s progress stays on track.

By Barbara Jenkins, Au.D., BCABA

1000 VOCABULARY RANGE AT 36 MONTHS

NUMBER OF WORDS

800

FOR CHILDREN WITH NORMAL HEARING

600

400

200

0

Normal

At Birth

6 Months

2 Years

AGE OF HEARING LOSS IDENTIFICATION 12 | HEARING HEALTH | A PUBLICATION OF HEARING HEALTH FOUNDATION

The Infant and Toddler

Delayed or absent speech development is the most important clue indicating a possible hearing loss in the very young child. Identifying hearing loss in the infant and young child requires watching for critical developmental milestones. Detecting a hearing loss as early as possible and then treating it can help your child’s speech and language develop at a rate comparable with that of children without a hearing loss.

Use the following milestones as a guideline, and always discuss any concerns with your pediatrician.

By 3 months, your baby recognizes your voice, makes cooing noises, and is startled by sudden, loud noises.

By 6 months, your baby recognizes speech sounds and familiar voices, turns his head toward interesting sounds, plays with his own voice, and laughs. Your baby uses his voice to indicate pleasure and discomfort, and has speechlike conversations with caregivers.

By 9 months, your baby understands simple words like “Mommy,” “Daddy,” “no,” “bye-bye,” and his own name.

By 10 months, your baby’s babbling should sound speech-like, with strings of single syllables (“da-da-da-da”).

By 12 months, one or more real, recognizable, spoken words emerge.

By 18 months, your toddler should understand simple phrases and be able to retrieve familiar objects and point to body parts on command (without gestures). Your toddler has a spoken vocabulary of 20 to 50 words and short phrases (“all done,” “go out,” “Mommy up”) and is learning new words every week.

PHOTO CREDIT: @ISTOCKPHOTO.COM/JBRYSON; CHART SOURCE: MARION DOWNS NATIONAL CENTER FOR INFANT HEARING

U

niversal hearing screening for newborns has helped to identify most children with hearing issues quickly and accurately. With simple tests, 80 to 90 percent of hearing loss can be detected, and children can begin early intervention with the best possible outcomes for language development.

However, even if your child passed the newborn screening at birth, hearing loss that is genetic or progressive may not appear until later, when a child is a toddler or older. It’s important to identify the signs that may suggest a possible hearing loss in your child as quickly as possible, so that the next steps can be taken: testing, followed by appropriate treatment and management.

HEARING HEALTH

By 24 months, your toddler’s spoken vocabulary should be 200 to 300 words, and simple sentences can be spoken. Adults who do not spend time with your child on a daily basis can understand your child’s speech. Your child should be able to sit and listen while being read to.

The Older Child

It is more difficult to identify hearing loss in children who have developed speech skills, as they may have unconsciously developed coping techniques to compensate for their loss. Watch for these signs:

1 2 3 4 5 6

Your child seems to hear fine some of the time and then not respond at other times.

Your child wants the TV volume louder than other members of the family.

Your child asks “what?” or says “huh?” more often than he used to.

Your child moves one ear forward when listening, or he complains that he can only hear out of his “good ear.”

Your child’s grades fall, or his teacher notes that he doesn’t seem to hear or respond in the classroom.

Your child says that he didn’t hear you. Many parents assume their children are not paying attention when in fact there may be an unidentified hearing loss.

7 8 9 10

It seems as though your child is just not paying attention.

Your child starts to speak more loudly than previously.

Your child looks at you intently when you speak to him. He may be depending on visual cues.

You just have a feeling. Sometimes you just can’t put your finger on what your concern is.

There are many possible causes of acquired hearing loss that appear months or years after birth. Most hearing loss in children without obvious risk factors (such as premature birth) has a genetic cause. If you have concerns, contact your pediatrician for a referral to an audiologist, the professional who is specially trained to identify hearing loss in children of all ages, for a complete hearing evaluation.

Staff writer Barbara Jenkins, Au.D., BCABA, is Colorado’s first board-certified doctor of audiology. She has more than 25 years of hospital and clinical experience in treating patients with hearing loss. Jenkins serves as Colorado’s professional state commissioner for the deaf and hearing impaired, and was awarded the 2010 Leo Doerfler Award for Clinical Excellence by the Academy of Doctors of Audiology. For more information, see www.advancedaudiology.com.

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MANAGING HEARING LOSS

Seniors

Hope to Cope The inner ear disorder known as Ménière’s disease can lead to depression and anxiety.

By Mary Alm, Ph.D.

14 | HEARING HEALTH | A PUBLICATION OF HEARING HEALTH FOUNDATION

unknown, but symptoms appear when there is a change in the fluid volume inside the inner ear.

Causes of Distress

While many patients may only have minor symptoms, there are several reasons for the higher rates of anxiety and depression among people who have more severe forms of Ménière’s disease. The symptoms of the condition can be unpleasant and ongoing, and although not lifethreatening, they can feel unbearable.

Vertigo may be terrifying and can impact a person’s ability to drive, walk, work, or do anything else. Tinnitus can be so loud that it drowns out conversations, prevents an individual from falling asleep, or wakes them from slumber. The internal ear pressure—a feeling of fullness in the ear—can sometimes be so excruciating that it inhibits the ability to work, think, or sleep.

Hearing ability with Ménière’s may fluctuate, and many sufferers have difficulty with word retrieval and concentration. Nausea and vomiting may also occur during an episode.

The unpredictability of symptoms can also cause anxiety.

Some people experience more intense symptoms with weather changes, stress, or fatigue, but many find that

PHOTO CREDIT: @ISTOCKPHOTO.COM/RELAXFOTO.DE

a

s the new school year is about to start, Becky is worried and sad: Her vertigo attacks seem to be occurring more frequently. Last year she took quite a few sick days, either recovering from these attacks or because her tinnitus was so loud that she could not hear the students she teaches in her second-grade class. Becky loves being a teacher, and she certainly doesn’t want to quit or lose her job, but her Ménière’s disease is negatively affecting her life. Unsure of what to do, Becky feels afraid and helpless; all she knows for sure is that her symptoms are getting worse.

Becky is not alone. Anxiety and depression are common among those with Ménière’s disease, an incurable and sometimes debilitating inner ear disorder characterized by periodic episodes of vertigo along with tinnitus, ear pressure, and progressive hearing loss.

This rare disease is estimated to affect about 615,000 people in the United States, with more than 45,000 new cases diagnosed annually, according to data from the National Institutes of Health. For six in 10 people, symptoms of Ménière’s either improve on their own or the vertigo can be managed, typically through diet, drugs, or devices.

Patients with severe Ménière’s may opt for surgery, but this can affect hearing. Why some people get Ménière’s is

MANAGING HEARING LOSS

A National Institutes of Health–funded

study currently undergoing clinical their vertigo or other symptoms of the various anxiety disorders are trials and led by the University of come without warning. Episodes distinctive. A panic attack is an episode Washington may offer a new way to typically only last a few hours, but of intense fear that can include a racing control a Ménière’s attack. To stop the sensation of dizziness, participants they can occur one after another in heart, shortness of breath, dizziness, wear an implant behind the ear that a short period of time and then be and breaking into a sweat. A person controls abnormal electrical activity followed by several months or even who suffers from agoraphobia is afraid in the nerve that sends balance information to the brain. years of no symptoms. to be in a situation from which escape The potential permanency of may be difficult, such as being in a Ménière’s disease, which generally presents in people crowd. Generalized anxiety disorder is worrying about in their 40s and 50s, is also hard to bear. People with many different things.

Ménière’s disease may become sad and anxious after Some people with Ménière’s show a few signs of postrealizing that they could have the disorder for the rest of traumatic stress disorder (PTSD). Symptoms of PTSD their lives. Their sadness and anxiety may worsen when are triggered by a traumatic event, such as having vertigo. they lose more of their hearing or if the other ear becomes People with PTSD have flashbacks, nightmares, or affected. Experiencing louder tinnitus or more frequent uncontrollable thoughts about the traumatic event. They vertigo attacks can also cause more distress. may be hypersensitive or have difficulty concentrating.

Symptoms of Ménière’s disease can interfere with People with Ménière’s disease may feel sad when they leading a normal life. People with Ménière’s may be reflect about how their illness has impacted their lives. unable to fulfill responsibilities at work, at home, or in They may feel worthless, helpless, hopeless, and in despair, the community, and those unfamiliar with the disease which may indicate depression. People who are depressed may not understand how it impacts the ability to perform feel sad or have lost interest in doing enjoyable activities. common tasks. Bosses may expect employees with They may feel extremely tired and have insomnia or Ménière’s disease to be as productive as their coworkers. sleep too much. Some other signs of depression include Spouses may expect household chores to be done. Friends appetite changes, feelings of guilt, difficulty focusing on may feel slighted when the person is not able to socialize. things, irritability, and thoughts about death or suicide.

People with Ménière’s disease may develop feelings How to Find Help of guilt if they are unable to meet the expectations of If you believe that you or someone you love may be others. Symptoms may lead to reduced responsibilities at exhibiting signs of anxiety or depression, seek help from work, termination from a job, family conflict, and social a mental health professional such as a psychologist. isolation, which can cause much distress. Many people Research shows that talk therapy is an effective way to grieve for the lives they once had and for plans and dreams reduce anxiety and depression and has longer-lasting that can no longer be realized. effects than medication, but a therapist will refer you to a Health Anxiety psychiatrist if you also need medication.

An excessive worry or preoccupation with symptoms To help you succeed in therapy, find a therapist who is may result from having had bad experiences with knowledgeable about Ménière’s disease and who makes symptoms in the past, and the fear that the illness will get you feel comfortable. Ask your health care provider worse. This extreme worry is called health anxiety. People for a referral. Psychology Today, a respected magazine, with health anxiety become very concerned with even and Good Therapy, an association of mental health the slightest changes in their symptoms, and these minor professionals worldwide, both maintain searchable changes can lead to much distress. online listings of psychologists and other mental health If left untreated, health anxiety can spiral to professionals who can offer help. hypochondria, worrying about symptoms or an illness For more on depression and hearing loss, please see the web that one does not actually have. People with Ménière’s extra for this story at www.hearinghealthmag.com. disease are at risk for developing health anxiety because the Mary Alm, Ph.D., is a licensed psychologist who provides disorder may be chronic, degenerative, and unpredictable. therapy for people with vestibular disorders. She is in private The distress of having Ménière’s disease can become practice in Claremont, Calif., and is an adjunct professor in so great that an anxiety disorder develops. Panic attacks, health psychology at Walden University in Minneapolis. For agoraphobia, and generalized anxiety disorder may more information, see www.drmaryalm.com. be seen among those with Ménière’s disease; the signs WWW.HEARINGHEALTHMAG.COM | FALL 2012 | 15

TECHNOLOGY

Get

Active

Thanks to improvements in hearing aid technology and accessories, there’s no reason to shy away from enjoying an active lifestyle.

P

articipating in sports and outdoor activities has always been challenging for people who wear hearing aids because of the potential for moisture damage, loss of the device, and a reduction in sound quality due to wind noise. As a result, you may have sacrificed the ability to hear clearly while on the basketball court or when out for a run.

Fortunately, hearing aid manufacturers have taken on these issues. Improvements in hearing aid styles, technology, and accessories mean you can wear your hearing aids while engaging in your favorite activities.

–––––––––––– Water Damage ––––––––––––– There are now hearing aids you can use while swimming, without fear of one of the leading causes of hearing aid damage: water. While many hearing aids are waterresistant, the Siemens Aquaris behind-the-ear (BTE) hearing aid is rated to be waterproof up to 3 feet deep and 30 minutes. Advanced Bionics recently introduced its first “swimmable” processor, the Neptune, for people who have cochlear implants.

16 | HEARING HEALTH | A PUBLICATION OF HEARING HEALTH FOUNDATION

Waterproof and water-resistant hearing aids are not only necessary for water sports: You should also consider them if your preferred activity causes you to perspire a lot. The minerals and salts in perspiration can damage a hearing aid that’s not moisture-proof. Most manufacturers now add a special coating to devices that protects against water as well as skin oils and earwax. The Starkey 3 Series hearing aids use a coating like this.

A hearing aid sleeve can go a long way toward protecting your hearing aid from moisture. Sleeves are available in multiple sizes and materials and provide a protective barrier to moisture—similar to a good raincoat. Ear Gear and Hearing Aid Sweat Band are two companies that make sleeves. Although Advanced Bionics Neptune these sleeves are not fully waterproof, they prevent the majority of moisture and perspiration from entering your hearing aids.

No matter which type

PHOTO CREDIT: @ISTOCKPHOTO.COM/PHOTOMORPHIC

By Barbara Jenkins, Au.D., BCABA

TECHNOLOGY

Emergency Care for a Wet Hearing Aid Don’t panic. As soon as you realize what’s happened: 1. Dry off the exterior of the hearing aids with a cloth (not a tissue).

2. Remove the battery and keep the battery door open.

3. Remove the ear hook or earmold if necessary.

4. Put the hearing aid into your dry-aid kit (always keep one in the car) or, if you don’t have one, put your hearing aids on the table next to a table lamp. The mild heat will dissipate the moisture. Do not put them very close to the light bulb or in the oven. The high heat will damage the devices.

5. Wait an hour or so and cross your fingers.

of hearing aid you use, you will need a desiccant container to store them when you are not wearing them. Overnight, these cases will remove moisture from your hearing aids to keep them working well. Some also sanitize and remove earwax buildup, such as Ear Technology Corporation’s Dry & Store. Ask your hearing healthcare professional which one is best for you, and use it every time you remove your hearing aids for the night.

–––––––––––––– Wind Noise ––––––––––––––– One of the most common complaints from outdoor enthusiasts, especially golfers, is wind noise. A completelyin-the-canal (CIC) device sits deeply enough in the ear canal that wind sounds won’t affect it. But it is not appropriate for every type of hearing loss, and fit will depend on the size of your ear canal.

In addition, all hearing aids can automatically reduce this irritant. Ask your hearing healthcare provider to turn your “wind noise reduction” option to maximum in the program you use for outdoor sports. It will not completely eliminate the noise, but it will reduce it significantly.

If you have an in-the-canal (ITC) hearing aid, consider using a tiny wind hood or windscreen over the microphone to physically block the wind from hitting the diaphragm.

The wind hood or windscreen should not interfere with sound production. There are a few BTE devices that now offer wind hoods as well.

–––––––––– Hearing Aid Styles ––––––––––– Some hearing aid styles are more conducive to active lifestyles than others. The use of headbands and custom earmolds can help your hearing aid stay in your ear (retention) and be safe from moisture or sudden motion.

(For a comprehensive guide to hearing aid styles, see “Plenty of Options,” in the Summer 2012 issue, at www. hearinghealthmag.com.)

Extended-wear and mini CICs (also known as IICs, or invisible-in-the-canals): Hearing aids such as the Phonak Lyric and the Siemens Eclipse fit more deeply in the ear canal than traditional CIC hearing aids. They make these the best for retention and wind noise reduction.

This style is available in mild to moderately severe power ranges. Not all ear canals can be fit to them, and the devices are too small to fit Bluetooth or FM technology, which limits wireless or assistive listening capabilities.

(For more about IICs, see “Mini Gems” in the Spring 2012 issue, available at www.hearinghealthmag.com.)

Standard CIC to ITE: These devices can now utilize Bluetooth technology to link to a personal microphone worn by your coach or biking partner. CICs in particular can be worn with a helmet and used with a safety cord and a canal lock to keep it in place. (See “Attachment Issues,” page 18.)

BTE, Slim-Tube BTE (Open-Ear BTE), or RIC: This type of hearing aid may be best for your hearing loss, but any device that fits on top of or behind the ear has inherent drawbacks for the active person. Most notably these are wind noise, retention, and helmet use. The microphone of the device is located where it is most susceptible to wind. Retention is an issue for sports that require a sudden change of orientation or helmet use. Helmets may also fit so tightly that the hearing aid is pressed against the skull, causing pain. Some wearers customize a cutout in the helmet, but be sure to verify that this does not alter the safety of the helmet. RICs can also use a canal lock to help with retention.

This is just a partial list of options for making your current hearing aids or your next set of hearing aids as sportsfriendly as possible. Talk with your hearing healthcare

Because Bad Things Can Happen to Good Hearing Aids Be sure to discuss with your hearing healthcare professional the length and limitations of your warranty and insurance. Warranty usually covers regular wear and tear on the internal components of the hearing aid, and repairs of a malfunctioning device (similar to a car’s typical

repairs and maintenance).

Insurance covers shell damage or loss (similar to body work on a car). New hearing aids come with one to four years of warranty and insurance. With an active lifestyle, consider buying an extended warranty.

WWW.HEARINGHEALTHMAG.COM | FALL 2012 | 17

TECHNOLOGY

Attachment Issues

Phonak Lyric

professional about the best options for you and your activities.

In addition, companies like Adco Hearing Products and Westone have knowledgeable customer support staff to help you make the right decisions. With so many options available, these companies that are accessible online are a great resource for always-changing technology not directly sold by your hearing healthcare provider.

Don’t limit your activities because of your hearing loss.

Research your options, consult others, and get what you need to enjoy your life more fully.

Staff writer Barbara Jenkins, Au.D., BCABA, is Colorado’s first board-certified doctor of audiology. She has more than 25 years of hospital and clinical experience in treating patients with hearing loss. Jenkins serves as Colorado’s professional state commissioner for the deaf and hearing impaired, and was awarded the 2010 Leo Doerfler Award for Clinical Excellence by the Academy of Doctors of Audiology. For more information, see www.advancedaudiology.com.

These products and options help keep your hearing device secure when you are in motion.

HUGGIE AIDS: These fit around your outer ear (pinna) and fit a wide range of BTE hearing aids for both adults and children. They can be a bit cumbersome to measure and fit at first, but once you get the hang of it they slip right on.

SIEMENS SPORTS CLIP: While this new, very effective tool for keeping a BTE hearing aid in place will not work for all hearing aids, it fits snugly to most adult ears without affecting sound quality or feeling heavy. The sports clip has been used successfully with very little movement of the device, even while the wearer is suspended upside down.

EAR BAND-IT: These are worn around your head like traditional headbands to keep any style of hearing aid in place by covering the ears.

They are made of thick Neoprene like the kind used in wetsuits to help prevent water from entering the ear canal.

CANAL LOCK: If you have an RIC or CIC hearing aid, ask your hearing healthcare professional to have a canal lock attached. It is a custom, clear acrylic appendage that rests on the bottom of the pinna.

SAFETY CORDS AND CLIPS: A safety cord and clip can attach hearing aids to each other as well as to clothing for added security. Choose a bright color for added visibility.

AT&T CapTel® you

can see & hear what is being said

Trouble hearing over the phone? AT&T CapTel can help! AT&T’s free captioning service uses the latest in voice recognition technology to display every word the caller says. So you can hear what’s being said and read a captioned version of your conversation at the same time.

Check out the newest AT&T CapTel calling options: • AT&T CapTel® 800i: People can call you directly and both incoming and outgoing calls are automatically captioned.

• AT&T WebCapTel®: Make AT&T CapTel calls through the internet.

No special equipment or software is needed.

CapTel services are NOT for emergency calls. If you use CapTel to call 911, AT&T may not automatically recognize your physical location, and, thus cannot forward your location to the 911 center. In an emergency CapTel users should dial 911 directly. © 2011 AT&T Intellectual Property. All rights reserved. AT&T, the AT&T logo and AT&T marks contained herein are trademarks of AT&T Intellectual Property and/ or AT&T affiliated companies. CapTel is a trademark of Ultratec, Inc. in the United States and/or other countries. All rights reserved.

18 | HEARING HEALTH | A PUBLICATION OF HEARING HEALTH FOUNDATION

to learn more… captel.att.com 1-877-401-8668

Using your smart phone and QR code scanner; scan this to begin your FREE registration for AT&T WebCapTel.

A heAring Aid

thAt cAn dO ALL thiS?

Lyric, the world’s first and only 100% invisible, 24/7 wearable, sweat-proof, shower-proof, for-months-at-a-time * hearing device can.

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Lyric’s unique design and placement works with your ear’s anatomy to deliver exceptional sound quality in quiet and noisy environments.

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Unlike many hearing aids, Lyric can be used during almost all your daily activities, such as exercising, showering, talking on the phone and sleeping.

cALL 1-877-257-7996

for a free informational DVD and a 30-day risk-free trial.† *Individual patient needs may vary. **Lyric is water resistant, not waterproof, and should not be completely submerged under water. †Professional fees may apply. Annual subscription begins the first day of trial. Consumers in NY, NM & VT will receive a 45-day trial. Offer expires 12/31/2012. Lyric is not appropriate for all patients. See a Lyric Provider to determine if Lyric is right for you. Lyric, Distributed by Phonak, LLC ©2012 All rights reserved. HH/Fall-2012

www.lyrichearing.com

MANAGING HEARING LOSS

A Salute to the Veterans Veterans from conflicts both past and current share their stories about hearing problems, the top health issue at military hospitals.

By Elizabeth Stump

20 | HEARING HEALTH | A PUBLICATION OF HEARING HEALTH FOUNDATION

there were 92,260 tinnitus claims and 65,583 hearing loss claims at U.S. Department of Veterans Affairs (VA) medical centers, according to the HCE. Cases of hearing loss, tinnitus, and auditory injury in the military continue to rise by as much as 13 to 18 percent annually.

Military Response

In response to the rise of auditory injuries in the military and their growing costs, the Department of Defense called for the establishment of the HCE in 2009.

Along with the VA, the HCE leads a collaborative effort to address the prevention, diagnosis, mitigation, treatment, research, and rehabilitation of hearing loss and hearing impairment.

“Hearing loss is truly a hidden disability, and our aim is to address significant gaps in the military’s ability to prevent or mitigate and then treat this type of injury,” says Col. Mark Packer, M.D., the interim acting executive

PHOTO CREDIT: @ISTOCKPHOTO.COM/MTMCOINS

T

innitus (ringing in the ear) and hearing loss are the number one and two health complaints, respectively, among military veterans overall, dating from World War I and up to and including current conflicts.

At least 60 percent of returning military from Iraq and Afghanistan have acquired hearing loss or tinnitus due to noise exposure during service. According to the U.S. Department of Defense Hearing Center of Excellence (HCE), the numbers for those injured in the global war on terror effort are more than 350,000 service members with tinnitus and over 250,000 with hearing loss.

The cumulative numbers are even more disturbing.

In the past decade, 840,000 service members have been diagnosed with tinnitus, and just over 700,000 have hearing loss, the HCE says. Combined, the number of cases of tinnitus, hearing loss, and other auditory disorders over the last decade tops 1 million.

In 2010, the most recent year for which data is available,

MANAGING HEARING LOSS

For more about the Department of Defense Hearing Center of Excellence, see its website at hearing.health.mil/Home.aspx.

For more about the Tinnitus Retraining Therapy Trial, see www.clinicaltrials.gov.

director of the HCE and an Air Force neurotologist. “The Army, Navy, and Marine Corps are actively obtaining hearing tests as a readiness protocol, while the Air Force is surveying the ‘at risk’ population in its hearing conservation program.”

Packer says the HCE was set up in part to help track auditory injuries among military personnel, which includes developing data sharing with the VA and a registry to track diagnoses and treatments. The HCE’s hearing loss prevention campaign is scheduled to begin this fall and winter. (For more about the HCE’s mission, see “The Military Paradox” in the Summer 2011 issue, at www.hearinghealthmag.com.)

While the HCE develops analyses, policy, guidelines, and patient and practitioner tools, it does not see or treat patients apart from those at two regional medical centers in Texas. These two centers are participating in a national Tinnitus Retraining Therapy Trial (TRTT). Tinnitus retraining therapy is a treatment for people with chronic tinnitus. The TRTT is a National Institutes of Healthfunded, phase III clinical trial—the “gold standard” for validation of a treatment.

“Tinnitus among veterans is a big, costly problem for the military and the VA medical system,” says Craig Formby, Ph.D., the TRTT study chair and a University of Alabama distinguished graduate research professor in

the Department of Communicative Disorders. “As of early September, we have 53 participants enrolled across six participating sites and will be adding three additional Army/Department of Defense treatment sites in the coming year.” (For more about the TRTT, see “A Landmark Trial” in the Summer 2012 issue, at www.hearinghealthmag.com.)

Despite the increased testing and implementation of earplug use in the military, U.S. troops are still suffering widespread hearing loss, according to the HCE. Hearing protection is limited: Earplugs can protect from noise that reaches 80 to 85 decibels (dB), but can’t protect against explosions and firefights that can reach intensely dangerous levels of up to 180 dB. Additionally, much of the fighting in the two war zones comes on suddenly and unexpectedly, providing little time for soldiers to use their military-issued hearing protection. Powerful roadside bombs lead to ruptured eardrums and broken bones in the ear. And some soldiers do not choose to wear hearing protection due to the fear of dampening their ability to be alert to danger, missing communication between service members, or adding an inconvenience during combat.

To honor the service of military veterans this Veterans Day, Hearing Health asked several to share their stories of living with hearing loss.

John Ayers, 79, Texas

In the 1950s, I volunteered for the U.S. Air Force at an aircraft training base. My job did not require flying as a military combat person; instead, I trained others to prepare the B-47 jet bomber to fly combat missions.

Earplugs were required only for those who worked on the flight line and next to the aircraft. However, when several jet engines are being run up at once for testing, the sounds exceeded the limits for which the earplugs were designed.

My flying experience was as a non-crew member and passenger. Flying at 10,000 feet, the engine roar permeated every part of the body. The droning of the engines made the entire airplane frame vibrate, making it difficult to sleep; hearing other people talk was impossible. It was several days before my hearing returned to normal.

After being discharged from the Air Force, I re-entered college and started working at an aircraft plant, which required my hearing be tested. At age 25, I was informed I had hearing loss. Like most young men, my response was a nonchalant “Oh!” It was not until I was in my late 40s that my hearing loss became more pronounced. In my 50s, I was fitted with hearing aids.

Like many individuals, I did not want others to know I had hearing issues, so I only wore the aids at home. As time progressed, the hearing aids ended up in a jewelry box. When I retired and had a more pronounced need for hearing help, I finally started wearing the aids full-time. In 2004, I lost all hearing in my left ear. I was implanted with a cochlear implant in 2005. In 2007, I lost hearing in the right ear, and received an implant for that side too. They work extremely well, and I am now assisting six major universities conducting research about cochlear implants.

WWW.HEARINGHEALTHMAG.COM | FALL 2012 | 21

MANAGING HEARING LOSS

Nathan Heltzel, 35, New Jersey

I served for a total of 11 years. I first joined the Army active duty as an enlisted Military Police soldier when I was 18 years old in 1995, and I left in July 2000. After 9/11 I voluntarily re-enlisted in the Air Force for six years via the N.Y. Air National Guard in November 2003 as a Security Forces sergeant. I was deployed to the northwest corner of Saudi Arabia—south of Iraq and east of Jordan—in May 2005 and served one tour in the region.

I have 40 percent hearing loss in my left ear and 30 percent in my right ear. I have tinnitus in both ears, especially the left. It was horrible at first. The hearing problems are a direct result of gunfire and loud jet engines on flight line duty. Common sources of noise are gunfire in both training exercises and on deployment as well as the sound of jet engines on the flight line that Security Forces troops secure.

I left the service because of my hearing problems. I was afraid to ever fire a gun again or to be in a situation where I would be exposed to small arms fire or explosions that would make the ringing worse.

I went for treatment while serving as an active guardsman, and I was told by the medical clinic on base that I had to go to the VA Hospital on my off-duty time to address my hearing issues. Since I couldn’t leave work for the appointments, I learned to manage my tinnitus and hearing loss on my own.

I use a white-noise machine, fan, or air-conditioning unit while sleeping to mask the tinnitus. I try to not eat salt or spicy food in excess, or drink too much caffeine or alcohol, as I’m told all of these exacerbate tinnitus. I carry earplugs with me to very loud places.

There were no military requirements for annual hearing tests when I was in the military from 1995 to 2009. While the military provides guidance regarding hearing protection, in practice it’s often a lot harder to implement. You need to be able to hear your radio, your buddy, your team, and especially any notice that can give you a tactical advantage.

Richard Uzuanis, 68, Illinois

I was a major in the U.S. Army Armor. My service lasted 14 years, from 1966 to 1980. I have noise-induced hearing loss of about 50 dB. The damage was initially caused when I was exiting a M60A1 Tank in Grafenwoehr, Germany, during my second year with the 68th Armor, and the tank blew up. Another major explosion, when a nearby tank took a lightning hit that set off its ammunition, also contributed. Like many others in the service who had intentions of making it a career, we did not complain about small injuries—real or perceived. It was pretty easy to cover up hearing loss. Virtually no one during my time in the service complained or pointed out any real or perceived hearing loss.

I did not leave the service due to my hearing loss; it was a family decision.

My service duties were not made particularly difficult by my hearing loss. They involved regular use of radio communications systems with headsets so I could always turn up the volume. Also, I did not seek treatment while in military service because I did not really acknowledge that I had a hearing loss until I was out of the military. I guess I was fairly typical of men my age.

I acknowledged that I had a significant loss only at the urging of my wife. I knew I had a loss but I denied to myself how serious it was. I went to the VA last year for the first time and established my service-connected hearing loss for the record. I use behind-the-ear hearing devices in both ears now.

It was not in the personal culture of the men I was serving with to call attention to anything that was perceived as impacting our ability to do our duties or to complete our missions. If there was a truly serious problem that impacted our ability to perform, we would identify the problem so as to not risk the safety or the lives of others in our teams or units. But we just personally dealt with the less critical issues, and hearing loss was one of these issues.

22 | HEARING HEALTH | A PUBLICATION OF HEARING HEALTH FOUNDATION

MANAGING HEARING LOSS

Bret Weaver, 42, California

I served in the Air Force in Anchorage, Alaska, from 1988 to 1991. My hearing loss was partly from the service and partly from a medical condition that was discovered later. I worked on F-15 jets as a test cell mechanic. There was a jet that blew out three times on different startups while I was under it, and I began noticing ringing in my ears a few nights later. I was removed from the flight line immediately after failing my hearing test and wasn’t allowed to go back, as much as I tried and wanted to.

I was given my first hearing aid before I left active duty. I don’t think I ever wore it for the first year or two. They tested me pretty regularly up to my discharge. My hearing continued to worsen after I got out of the military, so they tested me yearly. After I got out of college, I went to the VA in Minneapolis and finally received an MRI. I found out that I had an acoustic neuroma (brain tumor) that was causing the right ear to gradually worsen. The VA did the surgery for me, so I’m grateful. Now I use a behind-the-ear aid and I work as a civilian with the military. I’ve seen the mobile hearing trucks, so it seems like the military is being more proactive about hearing protection.

I think hearing loss risks are downplayed to a degree because hearing loss can be very hard to deal with on an individual basis. It’s not something that another person can see, like losing a limb. People don’t understand the difficulties of using hearing aids, or how your hearing changes daily or hourly depending on the battery life of your hearing aid, for example.

There will always be guys who feel they are too tough or busy for personal protective wear or devices. I also feel there are some jobs, especially in the military, where communication is very important for safety. You have to hear what you’re being told and be able to repeat or relate information. So being asked to wear double hearing protection can be very difficult. You may compromise one safety area or issue to accommodate another.

Elizabeth Stump is a writer and editor in San Diego. She is the former editor-in-chief of the Hearing Loss Association of America’s Manhattan chapter newsletter in New York City.

WWW.HEARINGHEALTHMAG.COM | FALL 2012 | 23

HOLIDAY GIFT GUIDE

Peace on Earth, Peace of Mind These gifts not only show sensitivity and awareness about hearing loss— they also double as clever safety gear so you and your loved ones can enjoy the holidays and beyond, completely worry-free.

TOYS American Girl Hearing Aid, $14

The American Girl doll company has a long history of creating items that speak to diversity and inclusion, and what little girl wouldn’t want her doll to look just like her? This removable hearing aid is specially made to fit both old and newly purchased 18-inch-tall My American Girl dolls; it can be worn on either ear, or purchased for both.

A permanent piercing at the by-mail Doll Hospital 24 | HEARING HEALTH | A PUBLICATION OF HEARING HEALTH FOUNDATION

ensures the hearing aid is expertly fitted to the doll’s ear for easy placement and removal. (www.americangirl.com) Auditory Training Games, prices vary

Board games and videogames can be effective, informal, and fun ways of sharpening your child’s listening skills. They reinforce such abilities as sound localization, sound sequencing, phonological awareness, binaural listening, and discernment of syllables and phonemes. For early readers, look for games such as Zingo, Super Why ABC,

HOLIDAY GIFT GUIDE

Bling for your cochlear implant transmitter, from Hayleigh’s Cherished Charms (see page 28).

and Hooked on Phonics. Board games for older kids and even adults such as Blurt, Boggle, Pictionary, Rory’s Story Cubes, Scrabble, and Wordplay test comprehension, the ability to follow directions, and creative thinking.

Electronic games such as Simon and Bop It (shown) can help hone reactions to sounds and language commands.

Popular videogames such as Dance Dance Revolution, Guitar Hero, and Wii Music also teach auditory skills under the guise of pure play. (Find anywhere where games are sold.)

Toys “R” Us Differently Abled Guide, free

Each September, Toys “R” Us produces a toy guide for “differently abled” children as a resource for people who care for kids with special needs. The guide features dozens of toys and games from major brands chosen by the National Lekotek Center, a nonprofit dedicated to making play accessible to all children. They are categorized according to the developmental skills they encourage: auditory, language, visual, tactile, gross motor, fine motor, social skills, self esteem, creativity, and thinking. For example, the Fijit Friends toy (shown) interacts with music and spoken commands while also building basic language and social skills. (www.toysrus.com/ differentlyabled)

BOOKS Goodbye Tchaikovsky, $9.99

A 12-year-old violin virtuoso suffers from a sudden, irreversible hearing loss, plunging him into a silent world.

He wonders how to communicate with friends, how to cope with unfamiliar situations, how to complete school, and what his future will now hold. Author Michael Thal says he wrote “Goodbye Tchaikovsky” as a way to heal the pain from his own hearing loss at age 44. The middleschool novel, billed as appropriate for anyone coping with a disability, comes from a small press specializing in books for gifted and homeschooled children. (www.rfwp.com) Healing at the Speed of Sound: How What We Hear Transforms Our Brains and Our Lives From Music to Silence and Everything in Between, $25.95

Can we use sound to improve our lives and achieve our goals? Authors Don Campbell, who wrote the bestselling book “The Mozart Effect,” and Alex Doman, an expert in the practical application of sound and listening, harness over a decade’s worth of research to show how we can use music—and silence—to become more efficient, productive, relaxed, and healthy.

The book provides advice, exercises, playlists, and links to WWW.HEARINGHEALTHMAG.COM | FALL 2012 | 25

HOLIDAY GIFT GUIDE

free, downloadable music on their website to create what they bill as the perfect soundtrack for any goal or task. (www.amazon.com)

SAFETY AND PROTECTION

Hear Your Life: Inspiring Stories and Honest Advice for Overcoming Hearing Loss, $14.95

Able Planet’s LINX Audio technology enhances sound quality and clarity and improves speech intelligibility. It also increases the user’s perception of loudness without increasing the volume, a benefit for people who have hearing loss. The technology also helps protect hearing: When the volume is turned up on your MP3 device and the headphones, the headphones operate at 7 to 10 decibels lower than other noise-canceling headphones. This lightweight model is great for the traveler or avid music lover. (www. ableplanet.com)

Hearing healthcare professional Melissa K. Rodriguez profiles more than a dozen patients she’s worked with to design and fit their hearing aids, ranging in age from young children to centenarians. Their stories show how hearing loss affects quality of life; Rodriguez hopes they will also provide incentive and inspiration to help you or a loved one get help for hearing issues. The book includes resources such as the decibels of common noises, information about hearing aid cost and maintenance, and a hearing self-test. (www.barnesandnoble.com) Let’s Hear It for Almigal, $16.99

Almigal is a happy, spunky little girl with a big personality who feels unlucky because of her hearing loss. Inspired by author Wendy Kupfer’s daughter, Almigal wants to hear “every single sound in the whole entire universe”— and she eventually does, with the help of cotton-candy-pink cochlear implants. Endearing, lighthearted, and informative, this picture book can be enjoyed by children with or without hearing loss and is at a reading level of age 5 and up. (www.barnesandnoble.com) Turning a Deaf Ear, $4.99 (e-book only); A Walk in My Moccasins: Memoirs of a Deaf Physician, $12.99

These two memoirs are from people who did not use hearing devices until they were adults. In the first, Allyson Raphel writes with humor about spending the first three decades of her life in relative silence before getting a cochlear implant. In the second, family doctor Justus Peters, M.D., writes about getting hearing aids at age 19.

Both books detail how they learned to cope, and then adapt. (www.smashwords.com, www.amazon.com) 26 | HEARING HEALTH | A PUBLICATION OF HEARING HEALTH FOUNDATION

Able Planet True Fidelity Headphones, $129.99

Active

Noise-Canceling

Bellman & Symfon Safe Fire Safety Solution, $229.95, and Care Home Alerting System, $199.95

Missing a smoke alarm alert in the event of a fire is a serious risk for people who remove their hearing aids before going to sleep. The Safe Fire Safety Solution is a smoke detector connected to an alarm clock and bed shaker. The detection of smoke triggers an extra loud alarm signal, flashing lights, and bed vibrations. While missing the doorbell may not be life-threatening, it can be inconvenient and frustrating. The Care Home Alerting System includes a doorbell that is wirelessly connected to a flash receiver which in turn produces bright flashing lights to let you know someone has rung the doorbell. (www.bellman.com) Clarity Pal, $99

This is a simplified cell phone aimed at older adults. Features such as amplified sound, a large dial pad, and a onetouch emergency button are all standard with the phone. It provides 25 decibels of amplification as well as an adjustable tone control. A talking caller ID, vibrating and flashing visual

HOLIDAY GIFT GUIDE

ringers, and a “talk back” feature that reads the phone number you are dialing to ensure accuracy are additionally helpful. Compatible with hearing aids, Pal is available as an unlocked GSM phone, so you can use the wireless phone provider you prefer. (www.claritypal.com) Energizer Micro Sport Headlight, $24.99, and LED Safety Flasher, $5.99

For people who like to take walks after the sun goes down and when it is cooler, these wearable lights can help you see and be seen, which is particularly important for those who may not hear approaching cars or cyclists. Both are water-resistant and lightweight (headlight shown). (www.energizer.com) ReSound Unite Mini Microphone, $440

With the Mini Microphone, you won’t need a front-row seat at your child’s piano recital. Simply place the Mini Mic right on the piano for perfect clarity of sound. The Mini Mic also helps with single-sided deafness. If your conversational partner wears the Mini Mic, he can stand on the side of your “bad” ear or even with his back to you—and you’ll have no problem hearing. These are just some of the uses for this small, discreet mic that has a range of up to 20 feet and a battery operating time of more than 8 hours. It can also stream music or sound from your laptop, radio, or TV. (www.gnresound.com)

STOCKING STUFFERS AuDBling Hearing Aid Awareness Bracelets, $15

These multistrand bracelets, each including a signature cochlear heart charm, promote awareness about hearing loss and encourage dialogue about its treatment.

Founded by Florida hearing healthcare professional Noël Crosby, Au.D., AuDBling offers a wide variety of cochleathemed jewelry and gifts that are natural conversation starters about hearing loss. (www.audbling.com) 28 | HEARING HEALTH | A PUBLICATION OF HEARING HEALTH FOUNDATION

Ear Gear Hearing Aid Protectors, from $25.95 per pair

For people who want to show off their hearing aids, these colorful, water-resistant sleeves are made of a spandex-nylon blend that protects hearing instruments from dirt, sweat, moisture, and loss.

They’re available with or without a cord, and in a variety of sizes to accommodate virtually all hearing device types and sizes, including cochlear implants and bone-anchored hearing aids. They come in a neutral tan color, too. (www. gearforears.com) Energizer EZ Turn & Lock Batteries, $7 to $10 for a pack of eight

Convenient, easy-to-use packaging keeps your hearing aid batteries secure yet accessible. Turn the dial to get a new battery; unused batteries stay “locked” in the package. A longer tab attached to each battery makes it simpler to handle and to place in your hearing aid, too. The perforated top of the package makes it smaller for tucking into bags and purses. (www.energizer.com) Hayleigh’s Cherished Charms Cochlear Bling, from $8

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RESEARCH

What Stops the Inner Ear From Regenerating?

The mammalian cochlea seems to start out with an intrinsic capacity to repair itself, but this capacity disappears as the cochlea matures.

This is the second of four articles about current projects under way in the Hearing Restoration Project (HRP).

This piece explains “Molecular profiling of purified subpopulations of mouse inner ear hair cells and supporting cells,” the project of HRP consortium members Andy Groves, Ph.D., an associate professor at Baylor College of Medicine in Houston, and Neil Segil, Ph.D., the executive vice president of research at House Research Institute in Los Angeles. We thank the researchers for contributing this story to the magazine.

T

he cochlea contains about 15,000 hair cells—the tiny hairlike projections that stick up from each cell’s surface like organ pipes. As sound waves travel through the fluid within the cochlea, they stimulate these projections, which causes the hair cells to become electrically active.

Each hair cell is connected by a nerve cell to the brain, which then interprets these electrical signals as sound.

Hair cells are incredibly mechanically sensitive. It has been estimated that their hairlike projections only have to be moved by the diameter of a few atoms in order for the cell to become stimulated. This sensitivity allows us

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to hear extremely soft sounds such as a whisper, a pin dropping, or an orchestral pianissimo.

This exquisite sensitivity of hair cells comes at a price, however. They are extremely vulnerable to damage. In addition to the aging process, a variety of insults and injuries can damage hair cells—principally loud noises, but also chemicals, such as certain kinds of antibiotics or chemotherapy drugs.

The hearing loss that results from the death of hair cells can be gradual, as is commonly seen in the wear and tear of old age, or immediate and catastrophic, such as deafness resulting from explosions or gunshots.

In either case, the hearing loss is permanent, as the lost hair cells are never replaced. But although humans cannot replace their lost hair cells, this is not true for other animals. It has been known for more than 20 years that birds, frogs, and fish can regenerate their hair cells naturally.

A bird that has been deafened is able to grow back almost its entire complement of hair cells and hear again almost perfectly in a matter of weeks. However, mammals apparently lost the ability to regenerate hair cells at some point over the past 300 million years.

PHOTOS COURTESY OF HOUSE RESEARCH INSTITUTE

By Andy Groves, Ph.D., and Neil Segil, Ph.D.

RESEARCH (Opposite) The newborn mouse organ of Corti, in the cochlea, photographed via a fluorescence microscope. In this genetically engineered mouse, the supporting cells are labeled fluorescent green. The hair cells are shown in fluorescent purple.

HOW DO BIRDS DO IT? Every bird hair cell is surrounded by four to eight “supporting cells” to form a repeating mosaic pattern.

The death of a bird hair cell somehow triggers one of its neighboring supporting cells to divide to create two daughters. One of the daughter cells then turns into a hair cell, and this process of division followed by transformation into a hair cell restores the mosaic of hair cells and supporting cells.

However, although mammals also have supporting cells surrounding their hair cells, there is almost no regeneration of hair cells after damage.

Why are mammals unable to do something that birds do so well? There are a number of possible explanations.

For example, mammalian supporting cells might simply have lost the ability to divide and make hair cells at some point in our evolution. Alternatively, they might still be able to regenerate hair cells, but the signal to trigger regeneration might be blocked or missing.

To test these possibilities, our labs carried out a conceptually simple but technically difficult experiment.

We developed ways of purifying supporting cells from the ears of newborn mice, an age where all the cell types of the cochlea are formed and in place. We also devised ways of keeping the supporting cells alive in a culture dish to study their ability to divide and make hair cells.

After several years of trial and error, we were able to show that newborn mouse supporting cells could behave like those of birds, at least in a culture dish. They were able to divide, and their daughter cells were able to turn into hair cells.

The snag with these results was that the experiments were all done in newborn mice, and unlike humans, mice do not start to hear until about two weeks after birth. When our labs repeated their experiments in 2-week-old mice, the purified supporting cells were now unable to divide.

These experiments tell us that the potential of the inner ear to regenerate hair cells changes quickly with age; therefore, understanding the biological basis of these changes is crucial for understanding how to regenerate hair cells.

THE NOTCH SIGNALING PATHWAY Although cell division is very important for regeneration, the ability to make new hair cells is also

extremely important. A number of labs, including ours, have been studying an evolutionarily conserved way in which cells communicate with one another called the Notch signaling pathway. Notch signaling is frequently used to create mosaic patterns of different cell types, such as the mosaic-repeating pattern of hair cells and supporting cells.

In this scheme, supporting cells make a protein on their cell surfaces. This is called the Notch receptor. Hair cells make proteins on their cell surfaces that bind to the Notch receptor, like a key fitting into a lock. Binding to the Notch receptor is believed to actively inhibit supporting cells from turning into hair cells. As a result, the alternating pattern of hair cells and supporting cells remains stable (see illustration, page 32).

The top surface of a single hair cell in the cochlea. The hairlike projections sticking up from the surface of this cell form a characteristic “W” shape. It is the movement of these tiny projections that cause the hair cell to become electrically active.

(Left) The surface of a mammalian cochlea, showing three rows of outer hair cells, and a single row of inner hair cells.

(Right) A mammalian cochlea that has been damaged by sound. Many of the hair cells have been destroyed, and will never regenerate, leading to permanent hearing loss.

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If this model is correct, it suggests that blocking Notch signaling between hair cells and supporting cells would remove the barriers that normally prevent supporting cells from turning into hair cells. Our labs and others have used both genetic and pharmacological approaches to accomplish this.

We find that supporting cells can indeed actively transform into hair cells when Notch signaling is blocked.

In some experiments, at least 50 percent of supporting cells transformed into hair cells.

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What is especially intriguing is that recent evidence suggests that the Notch pathway is also used when birds regenerate their hair cells, suggesting that manipulating this signaling pathway with drugs could conceivably be used to make new hair cells in mammals.

However, as in the example with dividing supporting cells, the story is far from simple. Our two labs have recently shown that the ability to make new hair cells by blocking Notch signaling only works in very young mice.

Once again, the mammalian cochlea seems to start out with an intrinsic capacity to repair itself, but this capacity disappears as the cochlea matures.

CONCLUSION How can we understand these barriers that seem to be placed in the path of mammalian hair cell regeneration?

Our labs are being supported by Hearing Health Foundation to start exploring this question. Using stateof-the-art gene sequencing technology, we are comparing

ILLUSTRATION COURTESY OF HOUSE RESEARCH INSTITUTE

When bird hair cells (HC) are killed, some of the supporting cells (SC) are triggered to divide (shown in gray). One of the two supporting cells turns back into a hair cell, restoring the system back to normal. In damaged mammalian ears, the supporting cells do not divide or make new hair cells.

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the genes that are switched on in young mouse supporting cells with older supporting cells that have lost the ability to regenerate.

Similarly, we are studying the genes that are switched on or off in supporting cells after inhibiting the Notch pathway with drugs—again, by comparing young mice where these drugs are effective with older mice in which the drugs have no effect.

We do not think it is likely that a single “magic bullet”

exists—a genetic switch that if flipped will restore regeneration. However, we are hoping to use their results to identify pathways in supporting cells, made up of many different interacting genes, that are changing as the animals age.

The hope is that in the future some of these pathways might be targeted with drugs to help reset supporting cells to a state where they are able to regenerate like those in birds.

gene mapping may make it possible to trigger hair cell regrowth in humans.

Today, about a dozen laboratories in the United States are working on ways to translate to humans what we already know about hair cell regeneration in The groundbreaking Hearing chickens. One approach is to stimulate Restoration Project brings together existing stem-like cells in the human new focus, new science, and new hope for a cure for deafness. The project is inner ear to regenerate hair cells, by delivering molecules that stimulate based on the discovery funded by new hair cell production. Studies in Hearing Health Foundation that birds can regenerate the hair cells necessary animals that spontaneously regenerate hair cells, such as birds and fish, are for hearing when those cells become unveiling important molecules needed damaged; humans can’t. But recent discoveries in stem cell research1 and Patient.Ad.Quarter.Page.pdf 5/29/12to reach 2:57 PMthis goal.

A second approach is to transplant stem-like cells into the damaged ear that can give rise to new hair cells.

Different labs are working on different pieces of this puzzle: some are working on gene therapies, some are working on stem cell therapies, and others are working on possible ways to integrate both approaches.

When these labs are successful at solving the puzzle of regeneration, there will be—for the first time ever—a biologic cure for deafness. Hearing Health Foundation is at the center of this important work.

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RESEARCH

Presenting the

2012

Grantees Hearing Health Foundation announces its latest group of exceptional Emerging Research scientists, whose pioneering efforts in hearing research continues a tradition begun more than 50 years ago. ince its inception in 1958, Hearing Health Foundation has awarded over $27.8 million through more than 2,000 scientific grants in hearing and balance research.

This money has led to dramatic innovations that increase options for those living with hearing loss, as well as protecting those at risk. With the potential for hearing restoration through regeneration biology, the scope of Hearing Health Foundation-funded research has expanded enormously through the Hearing Restoration Project. Hearing Health Foundation supports research in the following areas:

S

• Fundamental auditory research: development, genetics, molecular biology, physiology, anatomy, and regeneration biology • Hearing and balance restoration in infants, children, and adults • Cochlear implants/surgical therapy for otosclerosis; hair cell regeneration; hearing aids; medical therapy • Hearing loss from aging, noise, otosclerosis, viral infection (sudden deafness), ototoxicity, temporal bone pathology, otitis media, cholesteatoma, and tumors • Vestibular and balance disorders (dizziness and vertigo, Ménière’s disease) • Tinnitus (ringing in the ears) and hyperacusis (decreased tolerance of sound) Hearing Health Foundation’s Scientific Review Committee and Council of Scientific Trustees reviewed applications from 34 | HEARING HEALTH | A PUBLICATION OF HEARING HEALTH FOUNDATION

scientists at renowned research institutions throughout the United States. All research projects underwent competitive peer review for scientific merit and program relevance.

FIRST-YEAR GRANT RECIPIENTS Renjie Chai, Ph.D., Stanford University After receiving his Ph.D. at Baylor University in Texas, Chai is a postdoctoral fellow in the Department of Otolaryngology at Stanford University in California.

Research area: hair cell regeneration Characterization of Wnt-responsive progenitor cells in the mammalian cochlea: Hearing loss is a common sensory disorder affecting nearly 50 million adults in the United States alone. The majority of hearing loss is caused by the loss of the cochlear sensory hair cells, which, in mammals, lack the ability to regenerate.

We aim to gain insights into the regenerative potential of mammalian cochlear hair cells. The Wnt signaling pathway plays a crucial role in maintaining the stem cell population in several organ systems. Recently our laboratory has found transient expression of active Wnt signaling in the mouse cochlea, and found two inner ear progenitor cell populations marked by two Wnt downstream target genes. This project is designed to systematically investigate the role of the Wnt pathway in maintaining these two Wnt-responsive progenitor cell populations.

Long-term goal: to use hair cell regeneration and cell-based therapy to treat patients with sensorineural hearing loss.

Wei-Min Chen, Ph.D., University of Virginia Chen is an assistant professor at the University of Virginia Department of Public Health Sciences and Center for Public Health Genomics. He received his Ph.D. at the Johns Hopkins School of Public Health. His research focuses on the design and statistical analysis of human gene mapping data.

Research areas: otitis media, genetics Susceptibility to chronic otitis media: translating gene to function: Inflammation of the middle ear (ME) is known as otitis media (OM) in children. Some children develop chronic middle ear infections known as chronic otitis media with effusion and/or recurrent otitis media (COME/ROM).

This disease is very important to study because it is the number one reason children are prescribed antibiotics; it is the number one reason for deafness in children; and because the cost of pediatric and emergency room visits for infants and young children and lost parental wages is estimated at $5 billion annually in the U.S. Our goal is to find genetic factors that

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increase risk for COME/ROM in children. The discovery of causal variants would increase knowledge of novel genes and pathways involved in COME/ROM pathogenesis.

Long-term goal: to improve the clinical prevention of chronic infections and as a result decrease pediatric antibiotic use, surgery, and childhood deafness.

cells and neuronal integrity in adult cochlea. The objective of this research is to identify how the loss of Chd7 influences the susceptibility to acoustic trauma in the mature cochlea.

Long-term goal: to help identify novel genes and molecular pathways involved in protection from NIHL and to provide a rationale for designing new therapies.

Yoojin Chung, Ph.D., Massachusetts Eye and Ear Infirmary Chung received a Ph.D. in biomedical engineering from Boston University in Massachusetts. She is a postdoctoral fellow at Massachusetts Eye and Ear Infirmary and Harvard Medical School.

Research areas: neural coding of cochlear implant stimulation, central auditory plasticity Restoring binaural hearing with cochlear implants in early-onset deafness: Many profoundly deaf people wearing cochlear implants (CIs) still face challenges in everyday situations such as understanding conversations in crowds. Even with CIs in both ears, they have difficulty making full use of subtle differences in the sounds reaching two ears to identify where the sound is coming from. This problem is especially acute in children with congenital deafness.

We will study how perceptual training can help the brain to develop the circuitry for processing this precise information in animals with early-onset deafness, as well as examine how neural mechanisms for binaural processing are altered by auditory deprivation during development and whether these effects can be reversed by CI stimulation.

Long-term goal: to improve treatments for children with early-onset deafness, including the development of sound processors and rehabilitation strategies specifically adapted for bilateral CIs.

Israt Jahan, Ph.D., University of Iowa Jahan received her Ph.D. in medical sciences from Gifu University in Japan.

She began postdoctoral training in inner ear neurosensory development at Creighton University in Nebraska before moving to the University of Iowa to continue her work.

Research area: hair cell regeneration Misexpression of Neurog1 combined with delayed deletion of Atoh1 provides a novel model: Contemporary research is focusing on the regeneration of hair cells in hearing loss using Atoh1. The reconstitution of the organ of Corti requires the proper organization of two types of cochlear hair cells (inner and outer hair cells) as well as associated supporting cells. Recent data showed that the level of Atoh1 determines the degree of survival of different types of hair cells.

Our previous work demonstrated the survival of some organ of Corti–like cells without Atoh1 if replaced by a closely related transcription factor Neurog1. Now we want to investigate the effect of Atoh1 substitution with Neurog1 combined with a delayed loss of Atoh1 expression in viable animals. This combined mutant offers for the first time a critical test of presumed causalities of molecular mechanism that regulate the patterning of the organ of Corti, including hair cell and supporting cell differentiation.

Long-term goal: to define the correct dose and duration of Atoh1 expression for type-specific hair cell development in order to provide novel insights into hair cell regeneration.

Elizabeth A. Hurd, Ph.D., University of Michigan Hurd received her Ph.D. in biochemistry from the University of Bristol in the U.K. She completed 10 years of postdoctoral training at the University of Michigan in the Departments of Obstetrics/Gynecology and Pediatrics. She is a research investigator in the Department of Pediatrics at the university.

Research area: noise-induced hearing loss (NIHL) Investigating the role of Chd7 during noise-induced hearing loss: Mice born with the loss of Chd7, the gene mutated in CHARGE syndrome, exhibit middle ear defects and resistance to acoustic trauma. Preliminary results show that the deletion of Chd7 in adult mice (using a tamoxifeninducible Cre line) also results in a variable resistance to acoustic trauma, even in the absence of middle ear defects.

This suggests important functions for Chd7 in regulating hair

Kelvin Y. Kwan, Ph.D., Rutgers University Kwan received his Ph.D. in biochemistry from Harvard University in Massachusetts. He completed his postdoctoral training in auditory neuroscience at Harvard Medical School where he investigated the process of haircell mechanotransduction. He is an assistant professor and the Duncan and Nancy MacMillan Faculty Development Chair in the Life Sciences in the Department of Cell Biology and Neuroscience at Rutgers University in New Jersey.

Research area: hair cell regeneration Identification of transcription factors for hair cell regeneration: In mammals, when hair cells die from ototoxic drugs or loud noises, they are not replaced. Our efforts are focused on identifying transcription factors that promote WWW.HEARINGHEALTHMAG.COM | FALL 2012 | 35

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repopulation and replacement of lost sensory hair cells.

Transcription factors are DNA-binding proteins that play crucial roles in global gene regulation. We will use a cocktail of small molecules that activate transcription factors normally expressed during hair cell development to promote regeneration by controlled cell division and differentiation into hair cells.

Long-term goal: to promote functional auditory hair cell regeneration and alleviate hearing loss.

Sean Eric Low, Ph.D., Rockefeller University Low received his Ph.D. in neuroscience from the University of Michigan and completed postdoctoral work at the University of Montreal in Canada, where he examined the role of a piezo protein in cutaneous mechanotransduction.

These studies evolved into an interest in mechanotransduction processes in general, and a second postdoctoral position at Rockefeller University in New York.

Research area: fundamental auditory research Ascertaining the contribution of piezo proteins to mechanotransduction in zebrafish hair cells: The proteins that mediate the transformation of mechanical forces into electrical signals within sensory cells that convey the senses of hearing and balance have yet to be identified. This lack of knowledge has undoubtedly hindered the identification of therapeutic compounds capable of alleviating the complications that arise from disorders in hearing and balance, such as deafness and vertigo.

Recently, a member of the novel piezo protein family has been shown to contribute to cutaneous mechanosensation, raising the possibility that related family members may contribute to hearing and balance. We will utilize the zebrafish to test this hypothesis.

Long-term goal: to identify therapeutic agents that can restore normal hearing and balance in people.

This research award is funded in part by the Todd M. Bader Research Grant of the Barbara Epstein Foundation, Inc. Lina Reiss, Ph.D., Oregon Health & Science University Reiss received her doctorate in biomedical engineering from Johns Hopkins University in Maryland and completed postdoctoral work at the University of Iowa, where she conducted research in hybrid cochlear implant clinical trials. She is an assistant professor in the Department of Otolaryngology–Head and Neck Surgery at Oregon Health & Science University.

Research area: cochlear implants (CIs) Changes in residual hearing in a hearing-impaired guinea pig model of hybrid CIs: The goal of the current study is 36 | HEARING HEALTH | A PUBLICATION OF HEARING HEALTH FOUNDATION

to understand mechanisms of hearing loss with “hybrid” or “electro-acoustic” CIs, a new type of CI designed to preserve low-frequency hearing and allow combined acoustic-electric stimulation in the same ear.

Hybrid CI users perform significantly better than standard CI users on musical melody recognition, voice recognition, and speech recognition in the presence of background talkers.

However, approximately 10 percent of hybrid CI patients lose all residual hearing, and another 20 percent lose 20 to 30 decibels after implantation.

We hypothesize that in addition to surgical trauma, electrical stimulation through the hybrid CIs also damages cochlear cells, leading to the residual hearing loss. The first aim is to determine the contribution of electrical stimulation to the residual hearing loss in hybrid CI guinea pigs with noise-induced, steeply sloping, high-frequency hearing loss. The second aim is to examine the effect of electrical stimulation on the cochlear pathology. The findings will guide the development of strategies to prevent hearing loss with electrical stimulation, and allow extension of the hybrid concept to all CI recipients with usable residual hearing.

Long-term goal: to improve residual hearing preservation with hybrid CIs. Susan M. Robey-Bond, Ph.D., University of Vermont and State Agricultural College Robey-Bond received her Ph.D. in toxicology from the University of Rochester in New York. She conducted postdoctoral research at Cornell University in New York and the University of Vermont on antioxidants and DNA repair mechanisms. She is a research associate in biochemistry at the University of Vermont.

Research area: Usher and Usher-like syndrome deafness The role of a mutation in histidyl-tRNA synthetase in Usher-like syndrome deafness: An Usher-like syndrome— comprising deafness, blindness, and fever-induced hallucinations—was recently discovered, caused by recessive inheritance of a mutation in histidyl-tRNA synthetase (HARS).

The HARS enzyme is required for protein production in cells; it attaches the amino acid histidine to a transfer ribonucleic acid (RNA) molecule which activates and transports the amino acid to the ribosome for protein synthesis. We will measure the effects of this mutation on the molecules required for protein synthesis.

Preliminary results suggest HARS may be chemically modified by the cell, and that mutant HARS is modified differently, which is evidence HARS may have roles in the cell separate from its known function in protein synthesis. We additionally propose to determine the interactions of HARS and mutant HARS with other cellular proteins, specifically in cells derived from embryonic mouse inner ears, as a first step in

RESEARCH

elucidating a different role for HARS in hearing.

Long-term goal: to discover potential avenues for therapy to prevent or alleviate symptoms of Usher and Usher-like syndromes.

Isabelle Roux, Ph.D., Johns Hopkins University Roux received her M.S. degrees in genetics and human genetics from the Paris VII Denis-Diderot University in France. She received her Ph.D. in human genetics from the same university, studying how mutations of the gene OTOF could lead to the human congenital deafness DFNB9.

She is a postdoctoral fellow at the Johns Hopkins School of Medicine in Maryland, where she is studying cochlear physiology.

Research area: synaptic transmission in the inner ear Mechanisms involved in efferent synapse formation and maintenance in cochlear hair cells: This research aims to understand the molecular mechanisms that underlie the formation and maintenance of the connections between the sensory hair cells and efferent nerve fibers that provide feedback from the brain to the ear. Such fibers are important modulators of inner ear activity. Our investigation includes different approaches—electrophysiology, confocal microscopy, and mouse genetics—in parallel.

Our aim is to understand how connections between hair cells and efferent nerve fibers that bring information from the brain to the inner ear are formed. Correct synapse formation during development is critical for the proper functioning of synapses in the mature hearing organ.

Long-term goal: to understand the developmental machinery in the inner ear, which can lead to the ability to treat deficits in their function.

Bradley J. Walters, Ph.D., St. Jude Children’s Research Hospital Walters received his Ph.D. in integrative biology at Lehigh University in Pennsylvania. He is a postdoctoral research fellow in the Department of Developmental Neurobiology at St. Jude Children’s Research Hospital in

Tennessee.

Research area: hair cell regeneration Potential regeneration of auditory hair cells in the opossum (Monodelphis domestica): Many non-mammalian vertebrates, such as fish, amphibians, reptiles, and birds, naturally regenerate the inner hair cells whose damage or death results in hearing loss. This suggests that humans lost this regenerative potential at some point during mammalian evolution.

By discovering the critical differences between the hearing

organs of non-mammals and humans, we may be able to manipulate the human ear to allow human auditory cells to regenerate. But since there are many differences between the human ear and the ears of birds, identifying the most important differences represents a daunting task.

Marsupials like the gray short-tailed opossum represent an intermediate group, sharing many similarities of both humans and non-mammals. Supporting cells in the opossum inner ear retain the ability to proliferate well after birth, an indication that these marsupials may possess some regenerative ability.

We will determine whether opossums are capable of hair cell regeneration or recovery of hearing, and characterize the extent of supporting cell proliferation that occurs both during development and after hearing loss.

From this, comparisons will be possible among the various model species (e.g., chickens, opossums, humans) to gain a better understanding of which differences among the various hearing organs are essential for regeneration and which differences are detrimental or unrelated.

Long-term goal: to identify requirements for regeneration to occur in the mammalian cochlea and ultimately begin to develop drugs and/or gene therapies for preclinical testing to encourage regeneration.

Yasheng Yuan, Ph.D., Massachusetts Eye and Ear Infirmary Yuan is a postdoctoral fellow in the laboratory of Albert Edge, Ph.D., at Massachusetts Eye and Ear Infirmary with an academic appointment at Harvard Medical School. He is an ENT surgeon and an associate professor at Shanghai ENT Hospital, Fudan University, China. Albert Edge, Ph.D., is also a consortium member of Hearing Health Foundation’s Hearing Restoration Project.

Research areas: sensorineural hearing loss, stem cell regeneration Regeneration of auditory neurons using stem cells: We have shown that neural progenitor cells derived from mouse embryonic stem (ES) cells transplanted into the auditory nerve send out neural dendrites that extend to the sensory hair cells and axons to the cochlear nucleus. We will specifically use a new mouse ES cell line to trace the transplanted cells and new procedures to detect synapses between these transplanted cells and hair cells. We will connect the location of the new synapses with auditory function throughout the frequency range of the cochlea.

Our study is composed of two related, specific aims. In the first aim we will assess auditory function after cell transplantation.

In the second aim we will connect the synaptic counts to functional improvement in specific frequency regions. The overall goal of this research is to develop a cell-based therapy to replace auditory neurons.

Long-term goal: to find biologic treatments for hearing loss.

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SECOND-YEAR GRANT RECIPIENTS Keith Bryan, Ph.D., University of Iowa Bryan received a M.S. in biochemistry and a Ph.D. in biochemistry from the University of Iowa. He is a postdoctoral fellow in the Department of Molecular Physiology and Biophysics at the University of Iowa.

Research area: fundamental auditory research Investigating the role of CaBP1 in KCNQ4 channel modulation: KCNQ4 potassium channels play an important role in controlling the responsiveness of auditory hair cells to sound stimulation. The mutation of the gene encoding this channel causes deafness in humans, which is typically due to improper functioning of these channels in the ear. We have identified a novel interaction between Ca2+ binding protein 1 (CaBP1), which is highly expressed in auditory hair cells, and KCNQ4. The goal of this research is to evaluate the functional consequences of this interaction on the cellular localization and biophysical properties of KCNQ4 channels in auditory hair cells.

Long-term goal: to understand at the molecular level how hair cells function normally in sound detection and develop novel therapeutic strategies for treating patients with inherited forms of hearing loss.

Elizabeth A. Dinces, M.D., M.S., Albert Einstein College of Medicine Dinces received an M.D. and an M.S. in clinical research from the Albert Einstein College of Medicine in New York. Dinces is board-certified in otolaryngology–head and neck surgery and has a subspecialty certification in neurotology. She is active in resident education, clinical otology, and neurotology, and in research at the Albert Einstein College of Medicine. Her clinical activities include an academic practice in the Bronx, New York, dealing with ear disease and skull base tumors.

Research area: fundamental auditory research Effects of aging on selective attention in complex, multisource sound environments: Our research focuses on understanding how the brain processes sounds into meaningful language. It includes auditory scene analysis in the elderly, sound intensity processing in children, and development of auditory processing after cochlear implantation. The value of learning the role of attention and understanding the active and passive processes of stream segregation in aging populations will help develop therapeutic strategies for aging adults to improve listening and understanding in noisy sound environments.

Long-term goal: to explain mechanisms of auditory scene analysis, which is how the auditory system processes sound into meaningful elements, that break down with aging.

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Sung-Ho Huh, Ph.D., Washington University Huh received his Ph.D. from Washington University in Missouri in 2009. Since then he has been working as a postdoctoral fellow at Washington University School of Medicine.

Research area: hair cell regeneration Role of fibroblast growth factors (Fgfs) in cochlear sensory epithelium: Congenital sensorineural hearing loss is one of the most common hereditary disabilities, affecting 1 in 1,000 children. Fgf20-null mice have congenital hearing loss associated with loss of sensory cells, and the inactivation of both Fgf9 and Fgf20 result in a shortened cochlea. The goal of our research is to understand how Fgf signaling regulates the development, maintenance, and repair of sensory hair cells and supporting cells in the cochlea. Due to lack of regenerative ability in humans after the loss or damage of hair cells, it is critical to identify signals that can reactivate developmental pathways and thus permit repair and regeneration of the damaged cochlea. Studying the mechanisms that regulate cochlear development will provide valuable clues about molecules that can be tested for regenerative activity.

Long-term goal: to determine signaling pathways to induce hair cell regeneration in adult humans.

Kirill Vadimovich Nourski, M.D., Ph.D., University of Iowa Nourski received an M.D. from Saint Petersburg State University in Russia and a Ph.D. in neuroscience from the University of Iowa, where he completed three years of postdoctoral training in the Department of Neurosurgery. He is a research assistant professor in the same department, focusing on the functional organization of the human auditory cortex and temporal auditory processing.

Research area: fundamental auditory research Temporal processing in the human auditory cortex: The auditory cortex is the hearing center in the brain. Some neurosurgical patients undergo an operation in which arrays of electrodes are temporarily implanted in the brain for clinical diagnostic purposes. This provides a unique opportunity to study how the auditory cortex works, by measuring brain waves directly from the brain. We measure the brain’s responses to the timing information of sounds and the ability of the brain to accurately follow this timing, and then use this information to build a coherent percept of the environment.

Patients with CIs are largely dependent on timing and rhythm cues to understand speech and communicate, and people who have auditory processing disorders may be impaired in their ability to process that kind of information. To devise ways of assisting people with auditory processing disorders, it’s

RESEARCH

important to understand where and how, specifically, timing cues are processed in the auditory cortex—in other words, to understand how the timing and rhythm of speech is usually handled by the brain.

Long-term goal: to contribute to finding new and/or improved solutions for people with hearing loss and auditory processing disorders.

Regie Lyn P. Santos-Cortez, M.D., Ph.D., Baylor College of Medicine Santos-Cortez received her M.D. from the University of the Philippines Manila College of Medicine–Philippine General Hospital, where she also did a residency in otorhinolaryngology. She studied genetic epidemiology in Erasmus Medical Centre in the Netherlands and did most of her Ph.D. work on the genetics of nonsyndromic hearing impairment at Baylor College of Medicine in Texas. She is an assistant professor at the Center for Statistical Genetics in the Department of Molecular and Human Genetics at Baylor College of Medicine.

Research areas: otitis media, genetics Identification of genes that predispose to chronic otitis media in an indigenous population: The study aims to identify genes predisposing to otitis media by studying gene variants that are identified from a complex pedigree within an

LOSS • DAMAGE • FAILURE

indigenous population that has a high prevalence of chronic otitis media. The study population is ideal for gene mapping due to the limited number of founders and marriages only within the indigenous population. Next-generation sequencing will be performed in order to quickly and cost-effectively detect the causal genetic variants for otitis media that fall within the mapped genomic region. The discovery of gene variants predisposing to otitis media opens great possibilities toward increased knowledge of pathophysiology, prediction of the likelihood of otitis media through genetic diagnosis, and development of innovative treatments for otitis media.

Long-term goal: to help with the development of new diagnostic and treatment strategies for otitis media.

Zlaka P. Stojanova, Ph.D., House Research Institute Stojanova received her Ph.D. in human genetics from the University of Utah. She is a postdoctoral scientist at the House Research Institute in California.

Research areas: hair cell regeneration, genetics Epigenetic regulation of the Atoh1 gene during development and regeneration of the mammalian organ of Corti: The Atoh1 gene is both necessary and sufficient for auditory hair cell formation during normal development.

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1.800.525.7936 www.soundaid.com WWW.HEARINGHEALTHMAG.COM | FALL 2012 | 39

RESEARCH

It is also one of the first genes to be upregulated during regeneration in non-mammalian vertebrates. The project is investigating novel mechanisms of Atoh1 gene regulation that involve epigenetic modifications (not due to changes in DNA sequence). The study will analyze the mechanistic links between the discovered epigenetic state of the Atoh1 gene and the Atoh1 gene expression.

Long-term goal: to better understand how the Atoh1 gene is regulated in order to reverse the failure of hearing regeneration in the mammalian organ of Corti.

HEARING HEALTH FOUNDATION C.H.E.A.R. ENDOWMENT GRANT RECIPIENT Rebecca Seal, Ph.D., University of Pittsburgh First-Year Grant Recipient Seal received her Ph.D. in neuroscience at Oregon Health & Sciences University and completed her postdoctoral training in sensory circuits at the University of California, San Francisco. She is an assistant professor in the Department of Neurobiology at the University of Pittsburgh.

Research area: fundamental auditory research Role of outer hair cell glutamate release in cochlear function and dysfunction: Outer hair cells are vital for normal hearing. Although the cells are known to amplify the cochlear response to sound using an electromotile mechanism, they also signal to type II spiral ganglion neurons through the regulated release of glutamate. However, the function of this signaling remains unknown. Similar to inner hair cells, glutamate signaling by outer hair cells may influence sound transmission as well as the maintenance of spiral ganglion afferents. In the adult, cholinergic efferents play a critical role in maintaining outer hair cell viability and the innervation pattern of these fibers may also be influenced by the released glutamate.

Thus, there are several potential mechanisms by which loss of glutamate signaling by outer hair cells could cause hearing loss.

Long-term goal: to provide new information about the role of hair cell signaling in hearing and in disorders of the auditory system including hearing loss in order to help treat hearing loss caused by abnormal cochlear function.

The C.H.E.A.R. endowment was created to support an annual Sensorineural Deafness Research Grant. C.H.E.A.R. (Children Hearing Education and Research) was absorbed into Hearing Health Foundation in 1991, and Hearing Health Foundation is very proud to continue its legacy of funding research in sensorineural deafness.

LITERATURE REVIEW ON HYPERACUSIS Richard S. Tyler, Ph.D., University of Iowa Tyler was trained both as a clinical audiologist and as a psychoacoustician.

He has served on the National Academies Institute of Medicine Committee on “Noise and Military Service: Implications for Hearing Loss and Tinnitus”; the World Health Organization Panel on “The Burden of Disease from Environmental Noise and Tinnitus”; and the Veterans’ Affairs Committee on “The Revision of the Veterans Administration Schedule for Rating Disabilities (Hearing Loss and Tinnitus).”

He is a professor in the Department of Otolaryngology and in the Department of Communication Sciences and Disorders at the University of Iowa.

Research areas: cochlear implants, tinnitus Literature review on hyperacusis, recruitment, misophonia, phonophobia, and mechanisms: We will complete a thorough review of the literature, documenting causes, mechanisms, measurement, and treatments. The review is intended to provide a comprehensive document that clinicians and researchers will be able to use to understand hyperacusis and to improve current and suggest future treatments.

Long-term goal: to provide a systematic, comprehensive review of the entire field of hyperacusis in order to direct research to find cures.

STRIAL ATROPY/DEVELOPMENT PROJECT Andy Groves, Ph.D., Baylor College of Medicine Groves received his Ph.D. at the Ludwig Institute for Cancer Research at University College London in the U.K., where he studied the early development of the nervous system. He did his postdoctoral training at the California Institute of Technology, where his research focus became the development and regeneration of the inner ear. Groves is an associate professor in the Department of Molecular and Human Genetics as well as in the Department of Neuroscience at Baylor College of Medicine in Texas.

Research areas: the development and regeneration of the inner ear, stria vascularis development Development of biomarkers to study strial development and degeneration: The sensory hair cells of the cochlea are able to detect sound vibrations. Hair cells need a source of

Call for Applications: Hearing Health Foundation requests applications from new research applicants and current Hearing Health Foundation research grant awardees applying for a second year of funding, who are dedicated to exploring new avenues of hearing and balance science. To learn more about Hearing Health Foundation’s Emerging Research Grants program and submit a letter of intent, visit www.hearinghealthfoundation.org/emerging-research-grants.

40 | HEARING HEALTH | A PUBLICATION OF HEARING HEALTH FOUNDATION

RESEARCH

potassium that helps them to convert sound energy into electrical energy that is sent to the brain. Hair cells in the human cochlea are bathed in a potassium-rich fluid called endolymph, and the potassium is constantly pumped into the endolymph by a specialized group of cells in the cochlea called the stria vascularis. As humans get older, the stria vascularis can degenerate; when it does the “battery” that supplies potassium to the cochlea runs down, and hearing ability diminishes. The goal is to understand how the stria vascularis develops and to devise ways of looking at changes in this structure with age.

Long-term goal: to repair or slow damage to the cochlea and lessen the effects of age-dependent hearing loss.

Kevin K. Ohlemiller, Ph.D., Washington University Ohlemiller received his Ph.D. in neuroscience from Northwestern University in Illinois. After completing postdoctoral work at Washington University in Missouri, he became a research associate professor at the Central Institute for the Deaf, now merged with the Department of Otolaryngology, at Washington University School of Medicine.

Research areas: auditory physiology/pathophysiology, cell biology of hearing and deafness Cellular and genetic bases of age-associated strial

Listen With Your Heart

degeneration and EP decline in NOD congenic mice: The electric currents that run through cochlear sensory cells are largely driven by a specialized cochlear structure called the stria vascularis. The work of the stria requires a lot of energy, so that it is densely vascularized. Loss of strial blood vessels is thought to be a common cause of age-related hearing loss (presbycusis).

Not everyone shows signs of this kind of pathology; however, some genes have alleles that predispose people to presbycusis.

People who carry these genes may be more likely to experience loss of strial blood vessels, and ultimately loss of the stria itself.

In 2008 we discovered that a particular breed of mice (NOD mice) began with a normal stria, but then showed loss of strial vessels, followed by loss of the stria from both ends of the cochlea and progressing toward the middle. These changes were accompanied by other distinctive anatomic features that may or may not reveal something about the process. After crossing NOD mice with another strain that does not show pathology, we aim to determine which pathologic features are inherited together (caused by the same genes), how many genes are involved, and their approximate locations. Any gene(s) that are found may have human counterparts. While finding “risk” genes may not point directly to cures or allow us to predict who will lose their hearing, identifying the genes, gene networks, and gene products will help pinpoint key reactions that can be tweaked pharmacologically.

Long-term goal: to develop gene therapies for presbycusis.

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WWW.HEARINGHEALTHMAG.COM | FALL 2012 | 41

LIVING WITH HEARING LOSS

Alan Shearer spoke about the benefits of hearing aids and hearing aid maintenance at a workshop in Kenya in January.

Teaching by Example A carpenter and musician gives hope to children in Africa by showing them he can talk.

By Amy Gross nonprofit organization Kupenda for the Children. Kupenda, a Swahili word for “to love,” is based in New Hampshire and helps children with disabilities in Kenya by providing financial assistance, school supplies, special needs facilities, and advocacy and awareness.

“When I met Alan a few years ago in church, I realized that many of the children I work with in Kenya had better hearing than him, yet were in classes for the [profoundly] deaf and were only communicating in sign language,” Bauer says. Because

42 | HEARING HEALTH | A PUBLICATION OF HEARING HEALTH FOUNDATION

Shearer never learned sign language and was a musician, Bauer believed he could inspire the kids in Africa just by sharing his life story.

Gathering Support Shearer’s purpose for making the trip to South Africa was twofold.

A carpenter by trade, he was tasked with fixing old or broken furniture in several of the schools served by Kupenda. A 28-year-old deaf student named Shehe, who graduated from a carpentry trade school sponsored

by Kupenda, assisted Shearer in the

PHOTOS COURTESY OF ALAN SHEARER

M

assachusetts native Alan Shearer has been wearing hearing aids since he was a toddler. Diagnosed with mild to severe sensorineural hearing loss at age 2, the 34-year-old has been through his share of hearing aids, watching the technology develop and improve over the years.

Even so, the carpenter and musician was surprised when he was asked to serve as the “hearing aid expert” on a mission trip to Africa this January by Cynthia Bauer, the founder and director of the

LIVING WITH HEARING LOSS

repairs while the group was stationed in the Malindi District of Kenya.

“I got to work with Shehe and mentor him,” Shearer says. “We worked entirely with hand tools, and it was hard work because we used whatever wood was around— amazingly, it was usually beautiful, fresh-cut mahogany.” Because Shehe was completely deaf and used Kenyan Sign Language, Shearer says he had to learn some sign language himself.

Shehe will be taking a final exam this fall for a diploma in carpentry.

The other reason Shearer was invited to participate in the mission trip was to consult with Bauer’s Kenyan director regarding hearing health services and programming for students with hearing loss.

“We set up sessions to meet the children, ask questions, observe, and basically just see what was going on on the ground there,” Shearer says.

“The main problem was that many of the kids had some hearing, but had limited oral communication skills. Even if they had some hearing, they were being treated as if they were profoundly deaf—being taught to sign instead of speak.”

Shearer notes that although many students had been given hearing aids by a Western hearing aid company, there was no follow-up on how to use them. “The aids might actually be working, but the batteries were dead,” he says. “Or if the aids had stopped working, the instructions, written in English, sent the user to a phone number in the United States.”

After seeing for himself the issues that the students were facing, Shearer says he realized that a comprehensive program had to be put in place to teach the proper wearing and maintenance of hearing aids—a program which included access to a local hearing healthcare professional, and even more importantly, support

from the parents of these children.

Kupenda held several workshops and invited parents of children with hearing loss. Shearer acted as the keynote speaker at these seminars, explaining through a translator into Swahili and the local dialect that with the help of hearing aids, he was able to do everything a hearing person could, including talk and make music. “They loaned me a guitar, and I played some music and sang for them,” he says. “Showing them what I could do with my hearing loss was my most powerful way to help.”

At Shearer’s urging for professional

“When they’re not receiving services, these disabled kids lose heart.

We wanted to provide some inspiration.” assistance, Kupenda invited an audiologist from a deaf school 25 miles away (an hour-and-a-half drive) to do a seminar on hearing aids. During the workshop, Shearer helped demonstrate with his own hearing aids, going so far as to show parents and students how to break them down into their four parts—hearing aid, tube, ear mold, battery—and stressing that all four parts must be present and in working order for the aids to work properly.

One student, a 19-year-old girl named Dama, attended the meetings with her father. Clearly bright, Dama nevertheless struggled with limited communication skills.

According to Bauer, after meeting Shearer and seeing him talk, play guitar, and sing, Dama has been

speaking much more, and her speech has significantly improved. “She says he made her believe that she can do the same one day,” Bauer says.

According to Kupenda, in many African countries, people with disabilities are considered to be cursed by God. Shearer says he saw evidence of this during his visit.

“The perspective on disabilities is that even if you’re only partially disabled, you’re not capable of doing anything,” he says. “When they’re not receiving services, these disabled kids lose heart. We wanted to provide some inspiration.”

Future Plans The mission trip to Kenya was not Shearer’s first time working with young people. An avid sailor, he ran a summer youth sailing program in Dorchester, Mass., and coached a high school sailing team in Gloucester, Mass. He also worked for a summer camp and spent a year in Florida working for AmeriCorps.

Shearer is an accomplished musician who not only plays guitar but also piano, mandolin, and accordion. He also writes his own songs and performs in coffeehouses near his home. Given the chance, Shearer says he’d like to make another trip to Africa with Kupenda.

“I’d like to help them follow up if I can,” says Shearer. “The audiologist Kupenda brought in had been given resources by other NGOs [nongovernmental organizations], but like the kids themselves, he needs encouragement, too.”

“What Alan did had not been done before and seems to already be having an impact,” Bauer says.

“It was the beginning of something that we hope will touch the lives of many children who are hard of hearing.”

Amy Gross is a staff writer.

WWW.HEARINGHEALTHMAG.COM | FALL 2012 | 43

ARTS & CULTURE

Marvel Makes

When her 4-year-old son Anthony resisted the use of his bright blue hearing aid, Christina D’Allesandro did what any parent would do: She resorted to a little white lie. Captain America wears them, she said. Anthony expressed his doubts, so D’Allesandro turned to the Internet for backup. She found a generic form to contact Marvel Comics and sent an email message asking if any superheroes existed who had hearing problems.

What happened next was nothing short of heroic. “Out of all the letters we received that day, [D’Allesandro’s] truly touched my heart and demanded action,” says Bill Rosemann, the editor at Marvel who first intercepted her email. “As Stan Lee and Spider-Man taught us, ‘With great power, there must also come great responsibility!’”

Rosemann alerted D’Allesandro to Hawkeye, an Avenger who temporarily wore a hearing aid after damaging his ear (while saving someone, of course).

Rosemann sent her a photo of a 1984 Marvel cover featuring Hawkeye. But he took it a step further, getting artists Nelson Ribeiro and Manny Mederos involved.

The Blue Ear was born. The artists created images casting Anthony as his own character, with a call to action: “When DANGER makes a sound, The Blue Ear answers the call.”

Here, D’Allesandro tells the story, in her own words: 44 | HEARING HEALTH | A PUBLICATION OF HEARING HEALTH FOUNDATION

A

nthony has an underlying genetic condition called Mosaic Trisomy 22. In his case it is characterized by multiple, right-sided abnormalities inhis ear, lung, and heart, and mild facial asymmetry. Parts of his body are affected, but not all, and it is very difficult to predict what the true impact will be.

Most cases of MT22 in children are discovered when severe challenges manifest themselves. We are lucky that Anthony is really only very mildly impacted. He is cognitively normal, physically in the range of normal, and has minor challenges in terms of fine motor skills.

The biggest educational impairment has been, and remains, his hearing loss. Anthony has a complete loss on the right side. He wears a Cochlear Baha Softband—a bone-anchored hearing aid (BAHA)—on his right ear when he’s in school. He has mild loss on the left and wears a blue Phonak aid on his left ear.

The BAHA is significant because although Anthony’s

ILLUSTRATIONS COURTESY OF MARVEL COMICS; PHOTO COURTESY OF CHRISTINA D’ALLESANDRO

Magic

ARTS & CULTURE

Christina D’Allesandro smiles with her husband, Kevin Smith, and sons Dominic and Anthony (at right).

hearing loss in the left ear is mild, persistent fluid in his right ear causes him to test in the severe to profound range of loss.

Starting at age 6 months, Anthony has had multiple tubes in his left ear, most of which have clogged within weeks of placement.

We’ve seen better success with tubes since he turned 3, but we do not have full confidence in his left ear.

The Phonak is essential because, although the BAHA gives the biggest benefit, the band is painful.

With the Phonak we have an aid he can wear all the time that works with his FM listening system.

My heart broke when Anthony said that superheroes don’t wear “blue ears.”

So I lied. I hate lying to my kids, but I was desperate to find him an answer that would make him feel better.

I was searching Marvel’s website when I found an email address and I thought, why not? I may as well give it a try.

I was surprised when I heard back

was so happy to see that there was a superhero wearing a hearing aid.

Then it just got better and better as more things kept coming in. We were amazed.

In the drawing from Nelson [above] the hearing aid was even clearer.

Anthony loved it, as did all of his buddies who play superhero games every day.

Then Manny’s drawing came [opposite page].

Anthony pointed to the little Blue Ear and said: “That is me!”

From that point forward, he was The Blue Ear. After the local news covered the story, people would ask him if he was The Blue Ear. He would reply, with a completely straight face, “Of course I am!” He wears a costume all over town while helping people and beating up bad guys.

—Andrea Delbanco, Senior Editor

“I was surprised when I heard back from them. My faith in humanity was restored.” from them. My faith in humanity was restored.

When you ask for help about something that is so important to you and your family but so miniscule in the grand scheme of everyone else’s lives—and yet someone still takes note and the time to respond— it is amazing.

I thought Bill was a great guy to take the time. Anthony loved the first cover we got from him. He

WWW.HEARINGHEALTHMAG.COM | FALL 2012 | 45

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WWW.HEARINGHEALTHMAG.COM | FALL 2012 | 49

MEET THE RESEARCHER

NAME: Kirill Nourski, M.D., Ph.D., University of Iowa Hospitals and Clinics BIO:

Nourski received an M.D. from Saint Petersburg State University in Russia and a Ph.D. in neuroscience from the University of Iowa. A research assistant professor in the Department of Neurosurgery at the University of Iowa Hospitals and Clinics, he works in the Human Brain Research Laboratory. He is a second-year Hearing Health Foundation grant recipient, and received the Collette Ramsey Baker Research Award his first year in 2011.

The function of the auditory cortex—the hearing center in the brain—is my research area, and I am lucky to be able to work with human subjects. At our hospital, patients with epilepsy may undergo an operation in which surgeons open the skull, insert arrays of electrodes, and wait for epileptic seizures to occur so they can pinpoint their source. In the days, or even weeks, that the patients wait for seizures to occur, some consent to become the subjects of our research. While the electrodes are in the brain, we have the unique opportunity to study how the brain works, recording brain waves directly from the brain.

My project of the past three years deals with measuring the brain’s responses to the timing information of sounds and the ability of the brain to accurately follow this timing and use the information. I want to understand where and how, specifically, timing cues are processed in the auditory brain. For people with cochlear implants, the timing and rhythm of speech is one of the great tools they have to make sense of speech. On the other hand, people who have auditory processing disorders may be impaired in their ability to process that kind of information.

I was born in Leningrad (now Saint Petersburg), Russia, in 1979. I have returned home every year. Luckily, since I’m able to visit in the summer, I can visit the many drawbridges that open along the Neva River on the “white nights”—when daylight lasts nearly around the clock—to allow ships to pass through the city.

50 | HEARING HEALTH | A PUBLICATION OF HEARING HEALTH FOUNDATION

neuroscientist. It was really thanks to him that I became interested in hearing science. During my second year of medical school, I joined his lab as an assistant and my interests changed from clinical medicine to basic science. I wanted to make some contribution to society with my life’s work. As a young child I was not particularly interested in science. I was a child actor in a drama theater and was most interested in the performing arts and my international travels with the company to such places as San Diego and Japan.

I am a big fan of the music of the 1960s. I have a collection of almost 2,000 LPs and 1,000 45s. On weekends and in my travels, I always seek out record stores and resale shops like Goodwill or the Salvation Army. I once found an obscure British cast recording of the rock musical “Hair” for $2 in Melbourne, Australia.

I have great respect for the people who volunteer to be subjects for the experiments in our lab. One patient came to us for surgical assistance with epilepsy was also deaf and had cochlear implants in both ears. I was very impressed by her generosity and willingness to assist in many research studies. I was also touched by her spirit and sense of humor. She was fun to work with. Our patients must shave their heads before surgery, and when she expressed discomfort with her upcoming haircut, I shaved my head too, out of respect and a sense of solidarity.

—Yishane Lee, Editor

PHOTO COURTESY OF KIRILL NOURSKI

IN HIS WORDS:

A family friend was a prominent Russian auditory

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