Hearing Health Summer 2018

hearing health foundation

1958-2018

60 years strong

Summer 2018 A Publication of Hearing Health Foundation hhf.org

The Family Voices Issue Navigating hearing loss journeys with challenges and joy, together

HEAR EVERY WORD

NOT EVERY THIRD. HEAR CLEARLY, SPEAK CLEARLY WITH AMPLIFIED CORDLESS PHONES.

AMPLIFIED CORDLESS PHONES » Volume Booster amplifies call volume up to 50dB » 6-level custom tone settings (for handsets) KX-TGM450S

Up to

50 db

Voice Amplification

ALSO AVAILABLE

KX-TGM430B

KX-TGM420W

» Adjustable ringer volume up to 112dB Up to

112db

Ring Tone Volume

» Slow Talk (Real Time Voice & Answering System Messages) » Bright LEDs light up when phone rings » Large, backlit LCDs, keypads and touch buttons » Talking keypad, phonebook and caller ID » Expands to 6 handsets

shop.panasonic.com/amplified

letter from the ceo

DEAR READERS & SUPPORTERS, as a daughter, sister, and mother of family members with hearing conditions, this “Family Voices” issue has special relevance for me. In sharing my family’s hearing loss journey (see page 10), I see that it echoes that of many in our community of readers—and it underscores the importance of Hearing Health Foundation’s mission to further hearing research, education, and prevention.

In fact our HHF family grows each summer when we welcome a small army of interns who share their time and talent with us at our office in New York City. Please meet: Andrew Guralnick, who is earning a master’s in public administration at Baruch College, and is working on a project to track decibel levels in the city’s subway system.

Elizabeth Crofts, who will be a junior at Boston University studying biomedical engineering, is digitizing our database and writing about published research by HHF-funded scientists (see page 39).

Brandon Dye, who is earning a master’s degree in public health at Hofstra University, is a community education intern exploring how we can help bring back hearing screenings to New York City public schools.

Steven Ciravolo, who will be a sophomore studying film at Marist College, is creating videos for HHF’s communication efforts, including fundraising campaigns and education about noise-induced hearing loss (NIHL).

Meagan Rowley, who will be a senior at Case Western Reserve University studying nutrition, is helping to widen the reach of this magazine and also examining research on links between nutrition and hearing loss.

Anissa Vasquez, who will be a senior at Syracuse University, is an environmental sustainability and policy major. She is researching noise ordinances, work by the Congressional Hearing Health Caucus, and other public sources of noise that may lead to NIHL. Maddy Jaworski, who will be a senior at Fordham University double majoring in communications and Spanish, is helping with communications and development, including researching our list of Centurions, HHF’s longtime medical professional supporters.

Kayleen Ring, who will be a senior in marketing at Providence College, is handling our social media outlets and refining HHF’s Google presence.

HHF CEO Nadine Dehgan with (from left, back row): Andrew Guralnick, Elizabeth Crofts, Brandon Dye, Steven Ciravolo, Meagan Rowley, (front row) Anissa Vasquez, Maddy Jaworski, Kayleen Ring, and (inset) Daniel Krumm.

Daniel Krumm, who will be a senior at Fordham double majoring in American studies and history, is assisting with magazine advertising.

We must also thank past interns Vicky Chan, who contributed two stories in this issue (see pages 20 and 22), and Adrean Mejia, who helped with social media and photographed our thank you gifts (see page 25).

I am truly grateful for the tireless work of our interns, other volunteers, and our staff and researchers. I look forward to sharing more from our interns in future issues!

Nadine Dehgan CEO, Hearing Health Foundation ndehgan@hhf.org

  



 

  



   











a publication of hearing health foundation

summer 2018

3

HEARING HEALTH The Family Voices Issue Publisher Nadine

Summer 2018, Volume 34, Number 3

From parents of toddlers to college students, our community shares stories about their hearing loss journeys, experiencing frustration and anxiety, triumph and joy—together.

Dehgan CEO, Hearing Health Foundation Editor Yishane

Art Director Robin Senior Art Director Senior Editors

06 Family Voices My Magic Ear Kid. Joey Lynn Resciniti 10 Family Voices The Hearing Sense.

Nadine Dehgan 12 Managing Hearing Loss Your Child Has a Hearing Loss.

What Now? Nadine Dehgan 14 Education IEP My Way. Kate Hood 15 Education School Lessons.

Finding a Place for APD in College. Eliza Uberuaga The Best Lesson Yet. Layla Rudy No Excuses. Meghan Bayer 20 Living With Hearing Loss The Listening Project.

Vicky Chan and Lauren McGrath

22 Family Voices Maternal Instinct. Vicky Chan 24 60th Anniversary Connections on Camera.

Jeffery Scott Gould 26 60th Anniversary Faces of Hearing Loss.

28 Living With Hearing Loss Indistinct Chatter. Carol Stoll 29 Music On a Quest. Sue Baker 30 Research Hearing Restoration Project Plans Announced for 2018–19.

Peter G. Barr-Gillespie, Ph.D. 34 Research Recent Research

by Hearing Health Foundation Scientists, Explained.

Departments

Sponsored

03 Letter From the CEO

46 Advertisement Tech Solutions.

42 Roundup Talking Tweens & Teens.

49 Marketplace

50 Meet the Researcher Tenzin Ngodup, Ph.D. Hearing Health Foundation and Hearing Health magazine do not endorse any product or service shown as paid advertisements. While we make every effort to publish accurate information, we are not responsible for the correctness of information herein.

Cover From left: Julia, Joey Lynn, and Tim Resciniti paddle boarding north of their home in Pittsburgh.

Visit hhf.org/subscribe to receive a FREE subscription to this quarterly magazine. hearing health

Kidder

Gael Furbush

Amy Gross, Lauren McGrath

Medical Director David

Features

4

Lee

hhf.org

S. Haynes, M.D.

Staff Writers

Barbara Jenkins, Au.D. Emily L. Martinson, Au.D., Ph.D. Kathi Mestayer Carol Stoll Advertising

advertising@hhf.org, 212.257.6141 Editorial Committee

Peter G. Barr-Gillespie, Ph.D. Robert A. Dobie, M.D. Judy R. Dubno, Ph.D. Melissa E. Heche, Au.D. Anil K. Lalwani, M.D. Joscelyn R.K. Martin, Au.D. Board of Directors

Elizabeth Keithley, Ph.D., Chair Sophia Boccard Robert Boucai Noel L. Cohen, M.D. Col. John T. Dillard, U.S. Army (Ret.) Judy R. Dubno, Ph.D. Ruth Anne Eatock, Ph.D. Jason Frank Roger M. Harris David S. Haynes, M.D. Anil K. Lalwani, M.D. Michael C. Nolan Paul E. Orlin Robert V. Shannon, Ph.D. 363 Seventh Avenue, 10th Floor New York, NY 10001-3904 Phone: 212.257.6140 TTY: 888.435.6104 Email: info@hhf.org Web: hhf.org 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 Tax ID: 13-1882107 Hearing Health magazine (ISSN: 0888-2517) is published four times annually by Hearing Health Foundation. Copyright 2018, Hearing Health Foundation. All rights reserved. Articles may not be reproduced without written permission from Hearing Health Foundation. USPS/Automatable Poly To learn more or to subscribe or unsubscribe, call 212.257.6140 (TTY: 888.435.6104) or email info@hhf.org.

A seamless connection to life - all they have to do is listen.

Give your child access to the latest in hearing technology with the Nucleus® 7 Sound Processor – the industry’s first and only Made for iPhone cochlear implant sound processor.1 Designed specifically for the Nucleus 7 Sound Processor, the Nucleus Smart App allows you to control, monitor and troubleshoot your child’s hearing from the palm of your hand.

You can check device use information and even find a lost sound processor.

®

Find a Hearing Implant Specialist near you: Call: 800 354 1731 Visit: www.IWantYouToHear.com 1. Apple Inc. Use Made for iPhone hearing aids [Internet]. Apple support.

2017 [cited 24 February 2017]. Available from: https://support.apple. com/en-au/HT201466.

2. Cochlear Limited. D1190805. CP1000 Processor Size Comparison.

2017, Mar; Data on file. ©Cochlear Limited 2017. All rights reserved. Trademarks and registered trademarks are the property of Cochlear Limited. The names of actual companies and products mentioned herein may be the trademarks of their respective owners.

©2017. Apple, the Apple logo, iPhone, iPad and iPod touch are trademarks of Apple Inc., registered in the U.S. and other countries.

The Nucleus Smart App is compatible with iPhone 5 (or later) and iPod 6th generation devices (or later) running iOS 10.0 or later.

The Nucleus 7 Sound Processor is compatible with iPhone 8 Plus, iPhone 8, iPhone 7 Plus, iPhone 7, iPhone 6s Plus, iPhone 6s, iPhone 6 Plus, iPhone 6, iPhone SE, iPhone 5s, iPhone 5c, iPhone 5, iPad Pro (12.9-inch), iPad Pro (9.7-inch), iPad Air 2, iPad Air, iPad mini 4, iPad mini 3, iPad mini 2, iPad mini, iPad (4th generation) and iPod touch (6th generation) using iOS 10.0 or later. Apple, the Apple logo, FaceTime, Made for iPad logo, Made for iPhone logo, Made for iPod logo, iPhone, iPad Pro, iPad Air, iPad mini, iPad and iPod touch are trademarks of Apple Inc., registered in the U.S. and other countries. App Store is a service mark of Apple Inc., registered in the U.S. and other countries.

Information accurate as of November, 2017

CAM-MK-PR-335 ISS1 NOV17

My MAGIC EAR Kid After a late hearing loss diagnosis, our daughter thrives and achieves independence (including, as she’ll tell you, out on the ocean).

By Joey Lynn Resciniti

6

hearing health

hhf.org

julia was a full-term baby born exactly one week before her due date. She was healthy and perfect. She passed her newborn hearing screening.

The hospital bassinet had a cabinet underneath where the diapers were stored.

If I wasn’t very careful with the doors, they would slam loudly. Julia would get startled and cry.

At least she can hear, I thought. This would prove ironic to me when Julia showed signs of having problems hearing. When Julia was 15 months old, I became concerned with her speech—or its lack. She’d babbled a little bit as an infant and then didn’t say much until at a year or so when she said hi, once.

No one wanted to admit there was a problem. My husband Tim was even a little defensive about the subject. Grandparents chimed in that she was just a “late talker.” When I mentioned my concerns to the pediatrician, he recommended the state’s early intervention program, which led to our qualifying for twice-weekly speech language pathologist visits.

All this time, no one suspected Julia wasn’t hearing. With the specialist’s help, small gains were made in her speech. She developed a vocabulary of a few dozen words but never progressed to speaking two-word sentences or multisyllabic words.

So by the time we made it to the audiologist over a year later, when she was nearly 3, I had come to terms with Julia having some level of hearing loss. I knew when she turned her back to me, she wouldn’t respond if I called, and that was a big sign to me.

family voices

illustration credit: john tracy clinic, american academy of audiology

A Series of Tests

Sitting on my lap in the soundproof booth, Julia turned toward the speaker that was making a loud sound. But as the sounds got quieter, I got a heavy feeling in my stomach. She stopped turning toward the speaker.

Finally the audiologist leaned into the microphone and told me she was coming over to our room. I willed myself not to cry as she said she’d found a moderate hearing loss in both ears.

The audiologist next used little headphones to transmit sound directly to Julia’s auditory nerve. The results showed Julia’s hearing loss is sensorineural, a nerve problem, and not a mechanical problem like a tube that is too small in the ear. I was told that it is not uncommon for a newborn to pass a hearing screening, like Julia did, and then find something later. The audiologist reassured me that we’d found it sooner rather than later, and that intense speech therapy would catch her up.

The next few months were tough.

We scheduled an auditory brainstem response (ABR) test. For this test, the audiologist sedates the child and plays a series of clicks while measuring brain activity. This test is often done for young children to confirm their hearing loss before they’re fitted for hearing aids.

Our family was not ready to accept the first diagnosis and kept questioning the results. But I was with Julia in the booth, and knew it was correct. So when the ABR confirmed the hearing loss, I set to work managing the appointments and paperwork that would eventually help my daughter speak and thrive.

The first thing the audiologist showed us after the ABR testing was the “speech banana.” This was a confusing bit of information at first.

Banana? Speech? The speech banana is a visual aid for a quick introduction

I was told that it is not uncommon for a newborn to pass a hearing screening, like Julia did, and then find something later. to hearing loss and the varying levels of severity. (See chart, below.)

Normal hearing is in the 0 to 20 decibel (dB) range at the top of the banana. All speech sounds (vowels as well as consonants and consonant pairs) are above where Julia can hear with her 55 dB loss. Theoretically, without hearing aids she can’t hear any of those sounds. Things louder than 55 dB, like a dog barking or a piano, would be accessible for her without hearing aids. But the tricky part is that it isn’t so cut and dry.

Julia wasn’t unable to hear all language, and she also wasn’t always able to hear dogs barking.

Instant Change

We ordered hearing aids and earmolds. The audiologist showed us how to insert the tiny size 13 battery and talked to us about school accommodations and speech therapy as she programmed the little hearing aids for Julia’s specific hearing loss.

Introduced by the audiologist, and while the name can be confusing at first, the “speech banana” acted as a visual aid for a quick introduction to hearing loss and the varying levels of severity.

a publication of hearing health foundation

summer 2018

7

family voices

h earing health foundation

I’d thought about the moment she’d first hear with her new hearing aids. It was going to be the first time she’d hear my voice. Maybe the first time ever. I wanted to say, “I love you.” I wanted to say something nice, something comforting.

The audiologist worked the molds into her ears and clicked the battery doors shut. Julia’s eyes opened wide and her hands clenched on the arms of her chair. She could hear—and she was terrified!

“These are your new magic ears,” the audiologist said.

I didn’t say anything nice or comforting. I couldn’t help myself, I started to laugh! She looked so adorable, like she was on a roller coaster rather than an office chair. I forgot all about making a grand first speech and instead just beamed at her. Julia’s head swiveled to the ceiling. I noticed an obnoxiously loud fan for the first time.

On the way home, Julia tried to repeat just about everything we said. She could hear above the banana, all the vowel and consonant sounds. She began mimicking speech immediately. Every noisy thing that I had never taken the time to notice before was new and interesting.

We were warned that it might be difficult to get Julia to wear her new magic ears. The audiologist told us to be very firm so she wore the devices during all waking hours.

If she tried to take them out, back in they went.

Eventually, at age 5, Julia learned to insert her hearing aids herself, with the promise of a sleepover once she could show responsibility. She began to take ownership of the aids, poring over earmold colors and designs (striped, swirled, polka-dotted) with the practiced eye of a stylish tween (she wasn’t yet 8). She became a connoisseur of the hand-shaped earmold (great) vs. one that is made through an automated process (not so great).

Surfing

in Silence

As the parent of a child with a hearing loss, Joey began blogging about everyday hearing happenings to keep things in perspective.

Responsibility Shifts

As time goes by, those early years begin to fall into their proper perspective. I used to think it would mean something to me if Julia could someday tell me that she heard me when she was a toddler. Time and distance have shown that she doesn’t remember much of anything from her prelingual years. Her memories start when she was about 4. Everything prior to that comes from pictures and videos.

Some of the videos, like one when she is about 2 showing her fascination with lightning bugs, are painful for me to watch. In the video, you can hear me prompting Julia to say the word “bug” over and over, and watching it now I see plainly that she is confused and cannot hear us saying the word.

I wish I’d realized back then that she needed help. I wish I hadn’t spent a whole year frustrating myself and my baby.

Surfers have long believed that theirs is not just a sport, but a transformative life event. Conquering the ever-changing ocean instills a confidence unmatched by other pursuits.

By Julia Resciniti

8

hearing health

hhf.org

the surf instructor finally said we were ready.

My hearing aids were out before I knew what I was doing. The instructor was still talking, but I didn’t hear.

Out in the ocean, I was on my feet as the wave fizzled around my surfboard.

The smell of salt nipped pleasantly at my nose. I could taste the rushing sea air on my tongue. The inky blue was calming. The board

family voices

The ENT assumed Julia’s hearing loss is genetic, but the markers haven’t been discovered yet. The overwhelming majority of children with hearing loss—more than 90 percent—have typical-hearing parents.

If I had to do it again, I would tell myself to get her hearing tested. And also that she was going to be okay and that in three short years she’d be saying so much more than “bug.”

Life with a 13-year-old hearing aid user is much easier.

Julia is an independent seventh grader who gets straight As. We have as a family weathered ear infections with the potential to wreak havoc on a spelling test, late-night searches for a hearing aid battery among tangled twin sheets, and hearing aids that can’t be worn in the pool.

Now there are whole chunks of time when I don’t think about her ears, a blessing made possible by experience. We agonized when Julia’s hearing ability dropped another 15 dB to 70 dB, putting her in the severe category, and feared her hearing loss would progress even more, but it did not.

At the very first diagnosis, the ENT (ear, nose, and throat specialist, or otolaryngologist) assumed Julia’s loss is genetic, but the markers haven’t been discovered yet.

The overwhelming majority of children with hearing loss—more than 90 percent—have typical-hearing parents.

We just don’t know.

Years later Julia’s audiologist explained the drop: “Sometimes with a change in a child’s ear canal size, it may seem as if there is a 10 to 15 dB change in hearing across the frequency range. As the ear grows, a little more sound pressure may be needed to detect sound. This will result in what looks like a change in hearing but may just be growth

of the ear canal.” This makes sense. After eight years of steady audiograms and this explanation, I am finally able to let go of those lost decibels and my fear of losing more.

Every now and then there’s a head cold, dead battery, or damaged bit of ear tubing, and I am once again that younger mother, riddled with anxiety about taking care of Julia’s hearing. But the shift in responsibility has become hers. Julia is the one taking the lead on troubleshooting her technology at home, school, the pool, wherever she goes. At 13, she is the one always needing to think about her ears. Perhaps that’s what we’ve been working toward all along.

Joey Lynn Resciniti is the author of “Magic Ear Kids,” from which part of this article is excerpted. She lives with her family in Pennsylvania. For more, see magicearkids. com. Julia Resciniti’s article (below) is adapted from handsandvoices.org. For information on newborn tests and early intervention, see hhf.org/pediatrics.

Share your story: Tell us about your family’s hearing loss journey at editor@hhf.org.

Support our research: hhf.org/donate

was steady beneath my feet. The world was silent. I felt at peace as the coast sped closer and closer.

The board began to slow. I toppled off. The brown murk swirled about me when I opened my eyes with a sudden jolt of panic. The sand tickled against my feet. The board passed over me. I felt the panic ebb away as it was replaced with a wonder. Even the board’s shadow was so serene.

I loved it. I felt the leash tug me toward the coast the way an eager dog would.

The board was no longer over me. My lungs began to burn, so I lifted my head above the water. Another wave came, causing me to stumble for the coast. The instructor waded toward me. Before he could open his mouth to utter a sound, I was asking him to do it again.

He made a comment to my parents that I could not hear.

As I scrambled on the board, I didn’t care about anything except surfing again. And so we did.

The instructor kept saying things that I didn’t hear.

I wasn’t bothered by this. Many times in the past after begging to have dialogue repeated to me I found myself strangely disappointed. What I didn’t know was that he was impressed at my eagerness to surf again each time.

The triumph of standing on the board and the silence out in the ocean was amazing.

Without sound, I had nothing to focus on but the calm that only came when the sea raged around me, propelling me swiftly forward. I didn’t mind not being able to hear.

Just for that moment, I was even glad for it. a publication of hearing health foundation

summer 2018

9

Emmy (shown with her big sister Sara, far left) was diagnosed with a hearing loss at age 2.

The Hearing Sense

By Nadine Dehgan

on this lazy saturday, surrounded by legos, chattering children, and rainbow and flower paintings, my home is full of life and a little noisy. As I sit down to write this, I am reminded that my daughters and I have the privilege to be able to communicate orally and experience the various sounds around us, but we also know not to take our “hearing sense” for granted.

My younger brother, Caleb, has a hearing loss and has worn bilateral hearing aids since childhood, but I didn’t fully understand the vital role hearing and communication played in child development until five years ago.

That was when Emmy, my youngest daughter, was 16 months old and I noticed she was not as socially engaged as her older sister, Sara, had been at that age. She preferred to play by herself and seemed content to build and knock down towers on her own. Emmy also didn’t have a vocabulary, whereas Sara was speaking in short phrases at age 1 (which I knew was early). So although in the back of my mind I knew something was wrong, I did everything in my power not to compare my two children, realizing that children develop at different rates.

When Emmy was 19 months old, I finally convinced my husband, a mathematician who appreciates the large realm of what’s considered “normal,” that something was not right. Her pediatrician recommended we get Emmy tested, and we did; she was tested by a private company and by New York State. The results confirmed my private fears: She was definitely developmentally delayed, according to the state testers, but they were unable to label her disability. They hinted to my husband and me that Emmy may be autistic, but that she was too young for them to confirm a diagnosis.

The private company did diagnose her with autism.

Our work started the minute we left the tester’s office: I

10

hearing health

hhf.org

began researching the best early intervention options, the best schools for children on the autism spectrum, and the options if our family needed to relocate and to where. We also started Emmy on intense early intervention therapy.

I felt overwhelmed but determined to do whatever I could for my daughter.

When I took Emmy to her age 2 yearly checkup (months late because I was so focused on her developmental delays) she failed her hearing test—in both ears—twice. It turns out her “severe learning disability” was not autism at all: Emmy has a conductive hearing loss, which is when sound waves are not able to efficiently go through the outer ear canal to the eardrum and the small bones of the middle ear, causing an inability to hear sounds.

Her hearing loss was largely due to eardrum ruptures as a result of the dozens of ear infections she had since she was an infant. Her first ear infection was at 5 weeks old!

These ruptures caused a buildup of scar tissue and fluid that prevented her from hearing well. We were advised that Emmy’s hearing loss could be treated through surgeries, and these have greatly improved her hearing. Nearly immediately after her first surgery Emmy started to babble and then speak. She hasn’t been quiet since.

We caught Emmy’s hearing loss during her critical early development years, and I’m grateful for her surgeons and the research that went into her treatments. This is why I’m so happy to be part of Hearing Health Foundation (HHF), whose mission is to discover better treatments and cures for hearing loss and tinnitus, as well as to be a resource for the 360 million people worldwide living with hearing loss.

Through our awareness and outreach programs HHF aims to educate the public on how to prevent noise-induced hearing loss, the most preventable cause of hearing loss, as

family voices

Know the Signs A recent poll by the American Speech-Language-Hearing Association (ASHA) shows that parents may not be aware of the early warning signs of communication disorders, nor of the benefits of early treatment. Here are the signs, according to ASHA:

›› ›› ›› ›› ›› ›› ›› ››

Lack of attention to sounds (birth to 1 year) Does not respond when you call child’s name (7 months to 1 year) Does not follow simple directions (1 to 2 years) Shows delays in speech and language development (birth to 3 years) Pulls or scratches at his/her ears Difficulty achieving academically, especially in reading and math Socially isolated and unhappy in school Persistent ear discomfort after exposure to loud noise (regular and constant listening to electronics at high volumes)

If you notice any of these symptoms—even if your child has passed the newborn hearing screening—have your child’s hearing tested. In addition, a family history of hearing loss and/or recurrent ear infections are other reasons to have their hearing tested more frequently.

For more, see hhf.org/pediatrics and identifythesigns.org.

I consider myself an involved, informed parent. I took Emmy to medical professionals and grew up with a brother who wears hearing aids, so if Emmy’s hearing loss could be missed by me and our team of medical experts, any child’s hearing loss could go undetected. well as to decrease the stigma surrounding having a hearing issue.

I consider myself an involved, informed parent.

I took Emmy to medical professionals and grew up with a brother who wears hearing aids, so if Emmy’s hearing loss could be missed by me and our team of medical experts, any child’s hearing loss could (unfortunately) go undetected.

Since Emmy passed her newborn hearing screening test at birth I never considered hearing loss as a cause for her developmental delays. Hearing loss can occur at any time of life, with some forms not appearing until a child is a toddler or enters school, or even later.

Illnesses, ear infections, head injury, certain medications, and exposure to loud noise are all potential causes of

hearing loss. Left undetected in children, hearing loss can negatively impact speech and language acquisition, academic achievement, and social and emotional development—but all of this can be diminished and even eliminated through early detection and intervention.

More than 90 percent of babies born with hearing loss are to fully hearing parents and in families with no history of hearing loss.

Five years after that first inkling something was amiss, Emmy is an extremely social, talkative, outgoing, clever, and determined 7-year-old who loves to tell the story of her “ears,” how she and Uncle Caleb both are left-handed, and how both have hearing problems. She is showing an early aptitude for math (we named her in honor of Emmy Noether, one of most important women in the field of mathematics, with her own theorem named after her), and she has already decided she wants to invent bubble gum–tasting ear drops when she is older for all the kids, including herself, who can taste the drops when they’re placed in her ears. I couldn’t be more proud.

HHF CEO Nadine Dehgan lives in New York City with her family. For more, see hhf.org/pediatrics.

Share your story: Tell us about your family's hearing loss journey at editor@hhf.org.

Support our research: hhf.org/donate

a publication of hearing health foundation

summer 2018

11

managing hearing loss

hearing health foundati o n

YOUR CHILD HAS A HEARING LOSS

What Now? hearing loss is a loss. it’s not uncommon to grieve or be concerned after receiving a diagnosis. Take some time to come to terms with the news.

Please know there are professionals ready to help and many resources at your disposal. And there are also countless families who have gone through this same experience and have found success.

Help your child develop a fun, informative answer to educate their friends and others about their hearing loss and assistive devices. This will help them develop self-advocacy skills they will need later in life. For years, Julia, in our cover story, called her hearing aids her magic ears.

When discussing your child’s hearing loss with siblings and extended family, keep it simple.

Let them ask questions and answer them honestly and positively.

Ask your child’s audiologist for recommendations for a speech pathologist, education consultants, and materials to set up your child for success. If sign language is one of (or the only) chosen method of communication, make lessons a family activity. Your child should feel included in all conversations, especially in the home.

Here are some tips you may find helpful: Accept your child and cherish them for who they are. Encourage and foster their inner strength and focus on their character and successes rather than their hearing loss. Their hearing loss doesn’t define them—their heart, soul, grit, courage, personality, and character does (or will!). This will help your child accept themselves and their hearing loss and provide them with the confidence and assurance they will be accepted by their peers 12

hearing health

hhf.org

When your child reaches school age, set up a meeting with the school district’s special education services department and start the individual education plan (IEP) process. Cultivate and maintain the relationships made with special services—your child’s academic success depends on it. (For more about IEP readiness, see page 14.)

Know the laws related to disabilities and special education, so you’re equipped to be your child’s best advocate. Have regular check-ins with your child’s teachers to identify any gaps and ensure your child is developing appropriately for their age, both socially and academically.

You may be feeling overwhelmed and scared with many, many questions.

We understand—and are here to help. By Nadine Dehgan

Use a folder, binder, notebook, and/or online storage such as Google Drive to keep all the paperwork and results for tests and reports and to note the dates of appointments and procedures. Also use it to house warranty information for all hearing devices.

Mishaps happen—keep a positive attitude and laugh them off if possible. “Remember the time the dog thought the hearing aids we left out on the table were the perfect snack?”

There are lessons to be learned from issues that arise from bullying and other social incidences. These interactions can be incorporated into life lessons about diversity and how hearing loss is something to embrace. My daughter equates hearing issues to type 1 diabetes and dyslexia—conditions that her two good friends have.

Nadine Dehgan is the CEO of HHF. Read her story about her daughter’s hearing loss on page 10. For more, see hhf.org/pediatrics.

Share your story: Tell us your tips for handling a hearing loss diagnosis at editor@hhf.org.

Support our research: hhf.org/donate

education

h ear i n g health foundation

an iep is designed to help you, the student, but are you ready to attend your IEP meeting? Get ready for the new school year with our IEP Guide!

By Kate Hood, Hearing Our Way

IEP Team: A group of people that meets to create and update the IEP, typically including your parents, teacher of the deaf, case manager, and school principal or director of special education. Other teachers and service providers, such as your audiologist, may be included.

IEP MyWay

By high school, students attend IEP meetings, but many professionals believe involving them in their IEP meetings when younger, even for a short amount of time, helps teach valuable self-advocacy tools.

Here is a guide.

14

hearing health

hhf.org

Individualized Education Plan (IEP): Action steps for you and your teachers to help you achieve your goals.

IEP Meeting: A yearly meeting to discuss your progress, set new goals, and plan accommodations.

When you were younger, you may not have attended your IEP meetings. However, you are the most important member of your IEP team. Talk to your teacher and parent about the right time to start attending your IEP meeting.

Before the IEP meeting: »» Create a list of questions. »» Create a list of possible solutions to problems you have experienced in class. »» Create a list of accommodations including: »» Accommodations you've used in the past and want to keep. »» Accommodations you've used but haven't found beneficial. »» Accommodations you would like to try this year. »» Give a copy of these notes to your case manager or itinerant teacher ahead of time to prepare for the meeting and create an agenda.

Accommodations are changes in how you learn, not what you learn. Some of the most common accommodations for students with hearing loss include preferential seating, FM systems, live captioning (such as CART), provided notes, no oral testing, and resource room/learning center time. In addition, audio/video must

be captioned and teachers must write homework assignments (not just announce them).

During the IEP meeting: »» Introduce yourself with a friendly smile.

Thank everyone for being there. »» Make sure you can hear and see everyone's faces. If not, demonstrate your self-advocacy skills by speaking up and moving your seat. »» As decisions are made, repeat and rephrase to make sure you understand.

Try this: "So, what I understand is that we will do a trail of CART in one class to begin, then add more?" »» Ask questions. The people in the room are your biggest supporters and will be proud to see you take part in the meeting.

After the IEP meeting: »» Ask for a copy of the meeting notes. »» Read and review the new IEP. »» Follow through by using the accommodations you selected and reminding teachers about the accommodations when necessary. »» If a teacher isn't following the IEP, ask your case manager for advice. »» Always remember: Ask for what you need politely (without accusing), and you will find that people are more willing to accommodate you!

This was originally published in Hearing Our Way, a magazine for kids and teens with hearing loss, families, and professionals. Subscriptions $20/year; see hearingourway.com.

Share your story: Tell us your tips for navigating an IEP meeting, at editor@hhf.org.

Support our research: hhf.org/donate

family voices

School Lessons

Three students share their experiences managing their hearing loss, from childhood to college.

FINDING A PLACE FOR APD IN COLLEGE.

By Eliza Uberuaga “it’s best to not draw too much attention to your learning disability in the college process,” my college counselor said to me. One year after his comment, I made my way to Middlebury College with full bags but little confidence. While I had demonstrated that divulging my learning disability, auditory processing disorder (APD), did not hinder my acceptance into my dream school, I needed to prove that I could succeed in this new, challenging environment.

Once I began my life at Middlebury, I was given official documentation of my learning disability and granted extended time for exams. As I met with one of my professors to go over the documents, I was faced with questioning rather than support.

She asked if I truly needed the extra time. As the weight of her comment sunk in, I began to wonder if in fact I needed these accommodations. I had the records right in front of me, but I questioned if I was somehow inferior for needing these arrangements.

Any confidence I had when entering Middlebury dwindled as my professor’s question exacerbated my already nagging doubts.

The question of whether accommodations are warranted is a concept many people with APD find themselves debating. APD can be subtle, but that does not mean that

Coming to understand how I learn and to see pride in my learning difference is an accomplishment I have worked hard to gain. it does not exist. Auditory processing impairs your auditory pathways, making it difficult to process the information.

Having APD is like listening to a voicemail on a busy street while everyone else is listening to it in a quiet space. While most people can block out that background noise, people with APD hear that noise as if it is part of the message itself. In time, that voicemail will be heard and understood; however, the process it takes to get there can be more difficult for someone with APD. Since I was very young, I have been taking notes in class and then reteaching them to myself at home.

This technique allows me to get all of the information I need while allowing me to comprehend it at my own pace. In the end, I understand the information just as well as anyone else in my class; I merely take a different route to get there. And so, examples like these prove that I do indeed need the extended time my professor questioned me about, even though the need is not always evident in the classroom.

Coming to understand how I learn and to see pride in my learning difference is an accomplishment I have worked hard to gain. In questioning student accommodations, educators are questioning the validity of the student’s

difference in learning. In doubting one’s own accommodations, students question a right that they deserve.

Auditory processing disorder has been a barrier in my educational career, causing me to constantly defend how and why I learn differently. However, it has forced me to stand up for what I need and to persevere when I am questioned. I hope that in sharing this story, educators will be resolute in their support, and students will be confident in their pursuit for success.

New York native Eliza Uberuaga will be a sophomore this fall at Middlebury College, Vermont, and is pursuing a double major in education and Spanish.

Share your story: Tell us your hearing loss journey at editor@hhf.org.

Support our research: hhf.org/donate

a publication of hearing health foundation

summer 2018

15

education

h ear i n g health foundation

THE BEST LESSON YET

By Layla Rudy i was diagnosed with bilateral sensorineural hearing loss at age 3, after a series of unsuccessful doctor visits. Two years later, I went to the audiologist to get my hearing aids for the first time. I remember being frightened by a picture in the waiting room of the shiny tube that comes out of the ear molds attached to hearing aids.

My little brother was crying in the stroller as we left the doctor’s office for brunch.

I went to speech therapy for the next four or five years; during school, along with two other kids, I was pulled out of class a few times a week. I remember thinking that I didn’t belong there and that everything was fine with me. But the teachers and administration didn’t know what to do with me—they thought something was wrong with me, and they just threw me in the same boat as the kids with speech problems or ADHD. I was the only kid in my elementary school with hearing loss. While it was great that my mom wanted to mainstream me, looking back, it was clear the school didn’t know how to handle a student like me.

The problem was ignorance more than a lack of resources—although that was an issue, as well. My teachers plopped me in a desk—not always in the front of the room—and, not understanding how important the FM system was for me to hear in their classrooms, they didn’t wear it. I thought the teachers didn’t care about me, but it turns out they just never learned how to deal with students with hearing loss. I was too embarrassed to say anything, so the FM systems often went completely unused. For the same reasons, I was never assigned a note-taker. I was the outlier in a room full of typical children.

Identical issues continued in middle school and high school. I developed anxiety from a young age and, by adolescence, the effects were more severe. In retrospect, a lot of my anxiety stemmed from my worries that my peers were talking about me, or that I was not fully aware of my surroundings. To put it simply, I was anxious because I couldn’t hear.

I realize I’ve subconsciously trained myself not to rely on anyone else. It’s not that I don’t want to ask for help, or that I’m too embarrassed. I know that if I need it, I can ask, unlike when I was in elementary school.

My mom had always told me that college would be easier—I could get a note-taker and finally have the resources I never had. When I applied to Brooklyn College, I found the Center for Student Disabilities Services on their website. I was thrilled to find that they had a section devoted to hearing loss and deafness. Better yet, professors were given a guide to understand how each disability was to be handled and treated.

16

hearing health

hhf.org

education

I have spent my whole life going home and teaching myself what the teacher had taught us in class (and that I could not hear)—taking my own meticulous notes that my classmates begged to borrow. I have always wanted a note-taker, an FM system that gets consistently used, and empathetic teachers. Now that I finally have access to all of this, I don’t need it.

Layla as a high school graduate (top) and with her brother James when they were young children.

So, when I arrived at their offices, I filled out forms and submitted my hearing test results. They told me to come back in a few days, at which point I would have everything I had wished for in elementary school.

Ironically, I never bothered to go back to their offices, and I never bothered with their accommodations.

I have spent my whole life going home and teaching myself what the teacher had taught us in class (and that I could not hear)—taking my own meticulous notes that my classmates begged to borrow. I have always wanted a note-taker, an FM system that gets consistently used, and empathetic teachers. Now that I finally have access to all of this, I don’t need it.

I realize I’ve subconsciously trained myself not to rely on anyone else.

It’s not that I don’t want to ask for help, or that I’m too embarrassed. I know that if I need it, I can ask, unlike when I was in elementary school.

But my hearing loss, my disability, has enabled me to rely on myself, to be aware of my surroundings, and to better communicate with others.

And to me, self-reliance is the best lesson yet.

New Jersey native Layla Rudy will be a sophomore at Brooklyn College and is studying biology to eventually conduct auditory research. She is part of HHF’s Faces of Hearing Loss campaign; see hhf.org/faces.

Share your story: Tell us your hearing loss journey at editor@hhf.org.

Support our research: hhf.org/donate

a publication of hearing health foundation

summer 2018

17

Shown with her dad and brother, who also have a hearing loss, Meghan heads an honor society chapter for students with disabilities.

NO EXCUSES

By Meghan Bayer

o n augu st 1 2, 19 9 6, i cam e sc r e am i ng i n to t h e world.

I had all of my fingers and toes and my parents could not have been more proud of their “perfect” baby girl. I was (and still am) blessed with two very loving parents, and just over two years after my parents had me, I was given the gift of big sisterhood.

As I went through my toddler years, I hit all my developmental milestones.

Around the age of 3 and a half, my parents started noticing that I was always turning my right ear to the person speaking. Something wasn’t right.

I was not reacting to sounds the way the typical hearing child should. My parents took me to my pediatrician who referred us to an audiologist. On September 28, 2000, at age 4, I was diagnosed with bilateral moderate-severe sensorineural hearing loss and immediately fitted with hearing aids. My dad and brother also have congenital hearing loss and wear hearing aids.

When I was first diagnosed, my parents’ worlds were shattered. My dad continued to be in denial, and all my mom could do was hold me and cry.

I think they felt I would somehow be limited in my abilities. As a way to recover and accept the diagnosis, we started taking family sign language classes, which we all enjoyed.

18

hearing health

hhf.org

education

In order to fulfill my high school’s foreign language requirement, they offered to let me take ASL. I politely declined and stated that I would be taking French.

From the start, I was mainstreamed in a private school. I had a very typical childhood; my mom drove me to soccer, dance, gymnastics… you name a sport and I have probably at least tried it. My days were filled with homework, playing outside, and evening swimming lessons. I was a well-behaved student who had earned the respect of teachers, faculty, and staff. I maintained straight As and regularly made the honor roll. At school, I had a hearing support teacher come in for a half hour twice a week to troubleshoot my equipment, review math skills, and occasionally play fun games.

In 5th grade, my family moved two hours away for their jobs. At my new school, I didn’t know anyone and I was very shy. If someone asked me about “those things on my ears” I would stare at the floor silently.

Everything was different. I had a locker now, a bunch of teachers who didn’t fully understand my needs, and a new hearing support teacher. I lived in a new city and making friends seemed like an impossible task. I had to deal with my first real bully who would call me names and physically abuse me. When I defended myself from getting hit by a hockey stick, I got detention.

At the same time, my hearing became progressively worse until I was profoundly deaf in my left ear. I was implanted with bilateral cochlear implants during the summer of 2010, the second one just days before starting my freshman year of high school. With intense therapy, I successfully learned to hear and I was constantly amazed at my new hearing world. My academic success improved dramatically and my confidence soared.

In order to fulfill my high school’s foreign language requirement, they offered to let me take ASL. I politely declined and stated that I would be taking French. My sophomore year, I was given the French II Award for the highest academic average in my class. This goes to show that if you work hard, anything can happen! By senior year of high school I had accrued 800 community service hours, lettered in varsity basketball, and was inducted into the National Honor Society.

Today, I can confidently tell you that hearing loss will never be an excuse for me or any of my family members. I’ll graduate from the University of Pittsburgh in December 2018, on track for summa cum laude, with a major in communication rhetoric with a concentration in

the health sciences and a minor in the administration of justice. I decided to slow my education down a semester so I could do research under one of the top scholars in the field of the rhetoric of medicine. After graduation, I plan on taking a gap year to work before graduate school for a dual master’s degree in disaster relief/emergency management and homeland security.

I’m the president of our school’s chapter of the Delta Alpha Pi International Honor Society, an honor society for students with disabilities. This has allowed me to help others learn how to advocate for themselves and educate the academic community on how they can best assist a student with any disability. The chapter is very young, but I accomplished the goal I set as a freshman to leave the university more accessible for people with hearing loss than before. For instance, after a yearlong effort, captions are now included on videos that appear on digital bulletin boards and TVs around campus.

Looking back on the journey, I am thankful that my speech was completely unaffected by my severe inability to hear during the prelingual period. I am grateful to my parents, and I would not be where I am today without the help of each and every single person on my journey.

Pittsburgh native Meghan Bayer will graduate from the University of Pittsburgh this winter. For more, see megsmiracles.com.

Share your story: Tell us your hearing loss journey at editor@hhf.org.

Support our research: hhf.org/donate

a publication of hearing health foundation

summer 2018

19

family voices

h earing health foundation

THE LISTENING PROJECT In a new documentary, a longtime pediatric audiologist reviews her patients’ challenges and successes through their use of hearing technology.

By Vicky Chan and Lauren McGrath “most people still assume that if a person is deaf, they’re not able to speak,” narrates Jane Madell, Ph.D., in the opening moments of her documentary film “The Listening Project,” released in March 2018. Her statement sets the tone for the following 38 minutes of personal stories that shatter stigmas about hearing loss.

A New York City pediatric audiologist, speech language pathologist, and auditory verbal therapist, Madell created the documentary with award-winning filmmaker Irene Brodsky to reveal how technology has improved communication— and life—for people with hearing loss.

Brodsky captured interviews of 15 individuals with hearing loss, most of whom Madell treated when they Richard, a software engineer who wears a were children. cochlear implant, is among the 15 people Madell says filming interviewed about their hearing loss. allowed her to reconnect with her former patients to “see what they had to say about growing up with a hearing loss and what advice they might have for parents of newly identified children with hearing loss.”

The subjects of “The Listening Project” are vibrant young adults living and working all over the world— connected by their gratitude for the technologies and treatments that enable them to hear and talk. The majority are cochlear implant recipients, while the remainder wear hearing aids. They experienced similar anxieties, including not being able to hear everything in social settings, disclosing hearing loss to new acquaintances, and accepting their hearing loss.

If not for modern medical progress, the film’s subjects may not ever have been able to overcome these hurdles.

When Madell began her career in audiology 45 years ago, hearing loss treatments were very restrictive. Only 20

hearing health

hhf.org

The subjects of the documentary are vibrant young adults living and working all over the world— connected by their gratitude for the technologies and treatments that enable them to hear and talk. children with mild to moderate hearing loss could hear well with hearing aids, and the Food and Drug Administration had not yet approved cochlear implants.

Such limitations challenged Madell emotionally early in her practice. Though she smiled and appeared optimistic in front of her patients and their families following a hearing loss diagnosis, she knew how hard they would need to work with the then-inadequate accommodations for their children to succeed.

Madell’s former patients and millions of others are fortunate that changes in hearing technology and policies in recent decades have been dramatic. “We are so lucky we live now and not 30 years ago, 40 years ago,” says one.

Advancements have allowed children with hearing loss to learn spoken language, which Madell believes is critical for educational, social, and professional development and gives them options they would not have otherwise.

Madell hopes the personal stories in “The Listening Project” will help parents of newly diagnosed children, as well as legislators, educators, and healthcare workers.

“Parents of children with hearing loss have told me that if they had seen the film before the diagnosis, it would have been easier to deal with,” she says. It shows parents that with the resources and hearing technology available today, hearing and speech are possible for every child.

Vicky Chan is a Brooklyn College graduate from New York City.

Lauren McGrath is HHF’s marketing manager. To learn more about the film for either personal or educational use, see thelisteningprojectfilm.org.

Share your story: Tell us about your experience with hearing technology at editor@hhf.org.

Support our research: hhf.org/donate

Every Word Counts When hearing loss makes phone calls difficult, rely on CapTel® Captioned Telephones to show you captions of everything your caller says. With different models to fit your lifestyle, you’ll never miss another word of the conversation.

CapTel 2400i includes Bluetooth® connectivity and Speaker phone.

CapTel is a registered trademark of Ultratec. Bluetooth® is a trademark of Bluetooth SIG, Inc. CapTel Captioned Telephone is designed exclusively for individuals with hearing loss and is funded and regulated by the FCC.

www.CapTel.com l 1-800-233-9130

family voices

h earing health foundation

Maternal Instinct A parent’s intuition leads to a diagnosis of— and treatment for—hearing loss in her children.

3 out of every 1,000

The number of children born in the U.S. with detectable hearing loss in one or both ears, with more losing their hearing throughout childhood.

97 percent

newborns in the U.S. who have their hearing tested at birth. In 1993, only 5 percent of newborns were tested.

More than 90 percent

of children born with hearing loss have typical-hearing parents.

22

hearing health

hhf.org

like most moms, brandy moon has always been a champion for her three sons, Anthony, 12, Andersyn, 10, and Ayden, 7. Her sons each have bilateral sensorineural hearing loss and enlarged vestibular aqueduct syndrome, which means the damaged nerves in the inner ears are unable to transmit sound to the brain.

Moon’s journey as a parent-advocate had a difficult start. She was completely unfamiliar with hearing loss in children before she became a mother, and accessing proper treatment for the trio was a challenge.

Moon juggled numerous audiological appointments that were a five-hour round-trip drive from home. And her oldest child Anthony’s hearing loss diagnosis was delayed till he was 2 years old.

Anthony had typical speech development and passed all his first and second year wellness and hearing check-ups at the pediatrician. When he was 2, Anthony fell and hit his head. Moon suspected the trauma had caused either hearing loss or a cognitive disorder, but the doctors assured her Anthony suffered no permanent damage and took no action for him.

Moon’s instincts were correct. When her second child, Andersyn, was diagnosed with hearing loss at birth a few months after Anthony’s head injury, she insisted Anthony receive a detailed hearing evaluation. Born in 2005, Anthony had never received a newborn screening despite the passage of federal legislation mandating the practice six years earlier.

Backed by Hearing Health Foundation (HHF) and like-minded organizations throughout the 1990s, the Newborn and Infant Hearing Screening and Intervention Act of 1999 was enacted to facilitate early communication intervention for children with hearing loss. Prior to advocacy by HHF and others, the national rate for newborn hearing screening coverage was just 5 percent.

The legislation quickly improved newborn hearing screening tests around the country. In 2005 when Anthony was born, 94.2 percent of babies in the U.S. were screened, but some states lagged behind. In 2005 in Tennessee, where the Moon family lives, only 66.9 percent of newborns were tested—the lowest in the nation.

Unfortunately, Anthony was among the nearly one third of infants in the state who did not receive hearing tests in 2005. However, by his brother Andersyn’s birth two years later, the state’s rate had increased to 91 percent.

Anthony was eventually diagnosed with severe bilateral hearing loss and fitted with hearing aids. It was only due to Moon’s perseverance that her oldest son was ultimately given a comprehensive exam. “It was a little frustrating for us,” she says. “We knew something wasn't right with him so we had it rechecked.”

image credit: istockphoto.com; statistics graphic information from the cdc/niosh

By Vicky Chan

family voices

Anthony was among the nearly one third of infants in Tennessee who did not receive hearing tests in 2005. However, by his brother’s birth two years later, the rate had increased to 91 percent.

Moon’s message is that newborn screening is vital. “If your child has hearing loss, it is best to start intervention as soon as possible and have your child fitted with hearing aids or cochlear implants if they need them,” she says.

Robert Dobie, M.D., HHF’s senior scientific trustee, agrees. “Speech and language development depends on the brain’s responses to hearing in the first two years of life,” he says. “Now, with the ability to detect hearing loss at birth, and restore hearing in infancy with hearing aids, cochlear implants, and other technology, nearly all children with hearing loss are equipped to develop strong speech and language and succeed in school.”

With hearing aids, Anthony at age 2 was newly fascinated by all the sounds he could hear—including the squishy sound of his mom’s flip-flops as the pair walked through a parking lot. Moon realized it was likely that Anthony, like Andersyn, was born with hearing loss, but it only became detectable to her after his head injury.

Andersyn was given a newborn hearing test so Moon knew immediately that he had complete hearing loss in his left ear and, in his right ear, profound hearing loss. At one point, an audiologist suggested Andersyn wasn’t benefiting from his hearing aids, but Moon knew differently; she saw that with Andersyn’s hearing aids turned up, a sound as subtle as crinkling paper near his ears would startle him.

Andersyn now does exceptionally well with hearing aids, as does his little brother Ayden, Moon’s youngest child. Ayden was also born with severe hearing loss in both ears, which is also being treated with bilateral hearing aids. The boys’ doctors have cited a genetic connection of unknown cause.

Today, hearing loss is an ordinary part of life for her three boys, thanks to their mom’s tireless advocacy. With help from FM systems and speech therapy, they all receive a mainstream education and enjoy a variety of sports including baseball, basketball, swimming, and fishing.

HHF continues to advocate for newborn hearing screening—especially as federal funding for testing repeatedly appears on the budget chopping block. In 2017, HHF and others successfully prevented its elimination, resulting in the enactment of the 2017 Early Hearing Detection and Intervention (EHDI) Act. But the federal government’s proposed 2019 budget threatens to cut funding for EHDI once again. Brandy Moon’s experience, and that of millions of other families across the U.S., underscores the importance of providing newborn hearing screenings for the future health and success of all children.

Vicky Chan is a Brooklyn College graduate from New York City. Brandy Moon is part of HHF’s Faces of Hearing Loss campaign; see hhf.org/faces. For references, see hhf.org/summer2018-references.

Share your story: Tell us about your or your family’s hearing loss journey at editor@hhf.org.

Support our research: hhf.org/donate a publication of hearing health foundation

summer 2018

23

research

he a r i ng h e a lt h fo u ndat i o n

CONNECTIONS on CAMERA

An accomplished cinematographer reflects on a work in progress that captures the emotions and aspirations of those who affect, and are affected by, Hearing Health Foundation (HHF).

By Jeffrey Scott Gould i was 15 when my mother handed me my grandfather’s 8mm camera. Once I shot my first roll of film, I knew I had discovered my passion. Within a year, I had directed my first documentary, about a New Jersey traffic cop.

More than four decades later, with an established focus on documenting people living with various health conditions, I was honored when HHF approached me about a video project. Bryan Pollard, whose nonprofit organization has funded several Emerging Research Grants for HHF investigating hyperacusis (loudness 24

hearing health

hhf.org

intolerance), provided the introduction. We had worked on an awareness video about a teenager suffering from hyperacusis, and prior to working with Bryan, I directed a 93-minute documentary called “Quiet Please…” about people with misophonia, a neurological disorder that triggers adverse reactions to specific sounds.

The opportunity to capture hearing loss on film intrigued me. My first question was: Who will I film?

HHF, as I have come to learn, is a catalyst for enormous progress in hearing and balance science. As a visual storyteller, I know that this important work is best communicated through the heartfelt stories of the people involved.

When I began to work with HHF CEO Nadine Dehgan and marketing manager Lauren McGrath, we determined the video would include 11 subjects who live in six different states. The participants benefit from the work of HHF, or they influence positive change

60th anniversary

photo credits: jeffrey scott gould and chris insignares

When faced with their unthinkable circumstances, Nada and Jason had the choice between being victims or confronting challenges head on.

Both chose the latter in coping with their sons’ hearing loss. within the organization, or both. Our interview subjects include members of HHF’s Board of Directors, Council of Scientific Trustees, its funded researchers, and doctors, as well as individuals with hearing and/or balance disorders—and many who fall into multiple categories.

In April I met two children with hearing loss: 7-year-old Emmy and 13-year-old Alex. Emmy, Nadine’s daughter, has conductive hearing loss that has been largely remedied through surgeries, while Alex’s sensorineural hearing loss is treated with a hearing aid and cochlear implant. While not a parent myself (I’m an uncle to many bright and personable children), I could see that Emmy and Alex stand out as particularly confident and insightful. They exude a contagious sense of acceptance, strength, happiness, and positivity.

Particularly moving were my interviews with parents, who I’ve observed tend to approach adversities differently than their children who are affected. Parents go into what I call “protect mode” to make sure their child has the best chance in the world. Alex’s mother, Nada Alsaigh, M.D., who was devastated by Alex’s hearing loss diagnosis at birth, became a pillar of strength for her son and her whole family.

Likewise, Jason Frank, whose young son, Ethan, has hearing loss, is so dedicated to awareness about hearing loss that he now serves as a member on HHF’s board. He speaks passionately about the importance of newborn hearing screening and early intervention. When faced with their unthinkable circumstances, Nada and Jason had the choice between being victims or confronting challenges head on. Both chose the latter in coping with their sons’ hearing loss, which I find commendable.

With Andy Groves, Ph.D., of HHF’s Hearing Restoration Project (HRP), and Robert Dobie, M.D., HHF’s Senior Scientific Trustee, the conversations became more technical but remained vibrant. It may sound odd, but the best way I can describe Andy, an accomplished researcher and professor of developmental biology, is like a kid in a candy shop. He truly loves coming into work at Baylor College of Medicine, in Houston, every single day, and

that positive mindset trickles down to his colleagues, staff, and students.

Robert gives thoughtful responses to every prompt; after decades as a medical professional, he is completely entrenched in the world of otolaryngology. Observing his animated interactions with members of the board made me realize that HHF is involved in every type of hearing and balance condition.

My most recent shoots were with Michael Nolan and Sophia Boccard, who are both HHF board members and who live with hearing conditions. I met with Michael in his office at JP Morgan Chase. He has a way of making his listener feel comfortable, engaged, and validated. He speaks not only about his own hearing loss but also that of his mother, sharing a story about accompanying her to the audiologist and how important it is to him that she finally pursued treatment.

When I met Sophia, I knew within seconds that there’s something incredible about her. From her cover story in the Spring 2018 issue of this magazine, I already knew that she didn’t let her Usher syndrome diagnosis define her, but I never expected her to be so full of life, fun, and witty.

We all live with something, whether it’s visible or invisible, and to see someone like Sophia so happy, thriving, and making a difference in other people’s lives, is remarkable.

As I near the end of the shooting phase, I’m grateful to have been able to meet amazing people who are changing lives, to capture and share their stories. I reflect on the project often, whether I’m filming one of the many standout participants or conversing with friends and colleagues. Working with such a diverse group of eloquent, inspirational, and outright brilliant people, I can’t help but feel that I am part of something special, to be linked with passionate as well as compassionate individuals who bring their talents and energy to this hard-working organization.

Jeffrey Scott Gould is the director, cinematographer, and editor of Action Media Productions, a video production company in Long Branch, New Jersey.

For more, see actionmedia.tv.

Please watch the completed video at hhf.org/video.

Share your story: Tell us your hearing loss journey at editor@hhf.org.

Support our research: hhf.org/donate a publication of hearing health foundation

summer 2018

25

60th anniversary

hearing health foundation

FACES OF

hearing loss hearing loss, tinnitus, and related conditions are shared by tens of millions of Americans and hundreds of millions around the world. Our Faces of Hearing Loss participants come from diverse backgrounds across the U.S. but are connected by their shared experiences. Together, the portraits dispel the notion that anyone is immune from developing a hearing or balance issue. Here is a selection of children, teens, and young adults taken from the online gallery (hhf.org/faces).

Brendan Born with a profound hearing loss and lives in Oregon (shown age 11).

Brook Born with a severe hearing loss and lives in Michigan (shown age 20).

Aaron Born with a severe bilateral hearing loss and lives in New York (shown age 24).

Harper Born with a bilateral sensorineural hearing loss. and lives in California (shown age 3).

Alex Born with a bilateral sensorineural hearing loss and lives in Connecticut (shown age 12).

RECEIVE A GIFT WHEN YOU SUPPORT HHF’S GROUNDBREAKING RESEARCH thank you for supporting hearing health foundation’s life-changing research, awareness, and education work. Every gift counts and we pledge to use each dollar wisely. We are proud that major independent charity watchdog groups as well as Consumer Reports have recognized us for our fiscal responsibility.

In gratitude for your contribution of $50 or more, we will send you your choice of a scarf or tie, a unique thank you gift that we hope you’ll wear proudly. Each of the three silk scarves (near right, 36 inches square) are multicolored, featuring imagery of cochlear hair cells courtesy of the labs of Hearing Restoration Project members John Brigande, Ph.D., Stefan Heller, Ph.D., and Edwin Rubel, Ph.D. For the silk ties (opposite page), nine colors and designs with an inner ear pattern are available.

The scarves and ties showcase biology’s beautiful vibrancy, and underscore our sincere appreciation of your help funding innovative hearing and balance science.

26

hearing health

hhf.org

Joe Developed tinnitus and hyperacusis at age 20 and lives in New Jersey (shown age 23).

Scarves

Cochlea Cobalt

Restoration Red

Inner Ear Indigo

60th anniversary

Miranda

Julian

Born with a bilateral hearing loss and lives in New Jersey (shown age 21).

Born with a moderate to severe sensorineural hearing loss and lives in California (shown age 4).

Shane

Kaelynn

Diagnosed with a bilateral hearing loss at age 4 and lives in Texas (shown age 6).

Born with a moderate to severe bilateral sensorineural hearing loss and lives in California (shown age 5).

Kate

For more, see hhf.org/faces.

Born with a profound sensorineural hearing loss and lives in Oregon (shown age 8).

Share your story: To add your photo to our growing gallery, see hhf.org/spread-awareness.

Moaz Diagnosed with a hearing loss at age 3 and lives in Michigan (shown age 23).

Support our research: hhf.org/donate

photo credit: adrean mejia (ties)

Ties Black Lelo

Black Lido

Grey Lido

Purple Grey Kubo

Blue Lelo

Burgundy Lelo

Burgundy Polka

Green Grey Kubo

Green Lelo

Please note quantities are limited. To make a donation and receive a thank you gift, please see hhf.org/thank-you-gifts; send a check using the attached envelope; call 212.257.6140; or email info@hhf.org. Please include the gift numbers (as indicated on the envelope) of your top three preferences in order, and we will do our best to accommodate your choice. Thank you for your support of HHF’s mission.

a publication of hearing health foundation

summer 2018

27

living with hearing loss

hearing health foundati o n

INDISTINCT CHATTER

D.J. Demers is raising awareness about hearing loss through lots of laughs.

By Carol Stoll

D.J. Demers likes to interact with the audience but sometimes has difficulty hearing them from the stage. Instead of getting discouraged, he simply makes it a part of the show. example, Demers says he tells his girlfriend “last call” on final thoughts for the day and then takes out his hearing aids to go to sleep. He brags about sleeping like a baby with no distracting noises and says, “It really makes you wonder, who has the disability?”

Demers earned the 2013 Toronto Comedy Brawl and the 2014 Homegrown Comics Competition at the Just for Laughs comedy festival in Montreal, and was named 28

hearing health

hhf.org

“Best Breakout Artist” at the 2015 Canadian Comedy Awards. Besides appearing on “America’s Got Talent” in 2016, he appeared twice on “Conan,” and his debut album “[Indistinct Chatter],” released in 2017, earned a Juno Award nomination for comedy album of the year.

This past October and November, Demers performed across the country on the Here to Hear Comedy Tour, sponsored by hearing aid manufacturer Phonak in order to raise awareness about hearing loss through the power of laughter.

If you are just getting a hearing loss diagnosis, Demers has some advice: Figure out and implement the necessary tools needed to help you in your day-to-day life; surround yourself with positive people; and don’t be afraid to advocate for yourself. If you are passionate about comedy or any type of performing arts, Demers says, “Just do it.

That’s it. If it excites you, do it. Don’t impose any fictional limits on yourself.”

Carol Stoll is a staff writer. For more, see djdemers.com.

Share your story: Has having a hearing loss informed your career? Tell us at editor@hhf.org.

photo credit: troy conrad

after his first audition on “america’s got Talent,” D.J. Demers was described as funny, original, and likeable by the tough and at times brutally honest judge Simon Cowell. Demers is now an award-winning stand-up comedian and has appeared multiple times on the late-night talk show “Conan.” He also happens to have profound hearing loss.

As an infant, Demers had many ear infections and eventually was diagnosed with severe to profound bilateral sensorineural hearing loss. He started wearing hearing aids at age 4. Not wanting to be defined by his disability, D.J. turned to making others laugh, doing stand-up at age 21. “I was instantly hooked. Best decision I ever made,” he says.

Demers’s comedy makes light of heavy situations. He wants his audience to feel “free from concern,” he says.

He likes to interact with the audience but sometimes has difficulty hearing them from the stage. Instead of getting discouraged, he simply makes it a part of the show. “It’s never a negative experience because I never make it one,” Demers says. “An audience follows your lead, and my aim is to always keep it positive.”

Now 31, Demers doesn't shy away from proudly calling out his hearing aids and describing the unique, humorous situations he faces because of his hearing loss. For

music

ON A QUEST Les Paul, who would have turned 103 in June, experimented endlessly to find the perfect sound.

By Sue Baker from his earliest days, the concept of sound consumed musician and inventor Les Paul. How did sound work? Why did the record player produce sounds different from the player piano? Why does the sound of the train change as it moves down the tracks? Why did the body of his acoustic guitar vibrate when he plucked the strings?

How could he make just the strings vibrate?

Although best known for his solid body electric guitar and industry-changing recording inventions, for Les the quest always came back to sound, even in his later years.

“I’ve spent my life looking for the perfect sound, trying to build the perfect guitar to play the perfect note,” he wrote in his 2005 autobiography, “Les Paul in His Own Words.”

Les Paul was a tinkerer by nature and necessity, always wanting to invent something to fill a void or to improve what was available.

In the 1960s, Les’s eardrums were ruptured due to playful roughhousing. The resulting infection and, later, mastoidectomy surgery, left him with a hearing loss.

He wasn’t happy about the hearing aids’ sound quality for music.

I met Les when I was the executive director at a museum in his hometown of Waukesha, Wisconsin. We were creating an exhibit about his career. Over the course of what would be the last decade of his life, our friendship grew. Two years after Les passed away at age 94 in 2009, his business manager Michael Braunstein asked me to work at the Les Paul Foundation.

During one of my visits to Les’s home in 2001, I asked him about an unusual piece of equipment in a corner.

“Oh, it’s just an experiment I was doing,” he said. “I was trying to replicate how the human ears work.” He was a tinkerer by nature and necessity, always wanting to invent something to fill a void or to improve what was available.

Musician Jon Paris says Les’s audiologist (whom he met at New York City’s Iridium Jazz Club, where Les performed every Monday night) told him that Les “drove him nuts—in

Guitar legend Les Paul (left) enlisted the help of Marty Garcia of Future Sonics to improve his hearing aids.

a good way—constantly demanding better quality from his hearing aids.”

Another friend, Chris Lentz, says that Les worked with Marty Garcia of Future Sonics to improve his hearing aids. In a note to Chris, Marty wrote, “Throughout our years together, Les validated just about every voice coil transducer Future Sonics developed.”

In a 2008 interview in Audiology Today, Les talked about how he wanted to improve hearing aids for music.

He cited the importance of extending the audio range to capture more of the harmonic structure than what is needed for speech. Les also wanted hearing aids that could be worn in the shower and would work optimally when using the telephone.

Les Paul’s 103rd birthday would have been this June 9.

He would have been gratified to see how far hearing aid technology has come.

Sue Baker is the program director for the Les Paul Foundation. For more, see lespaulfoundation.org.

Learn about 2018 Emerging Research Grants recipient Tenzin Ngodup, Ph.D., who received the Les Paul Foundation Award for Tinnitus Research, on page 50.

Share your story: What’s a dream feature you’d like to see in future hearing aids? Tell us at editor@hhf.org Support our research: hhf.org/donate a publication of hearing health foundation

summer 2018

29

research

h e ar i n g health foundation

Hearing Restoration Project Plans Announced for 2018–19

By Peter G. Barr-Gillespie, Ph.D.

the goal of the h ea ri ng resto rat i o n p r oje ct ( h r p ) i s to determine how to regenerate inner ear sensory cells in humans to restore hearing.

These sensory hair cells detect and turn sound waves into electrical impulses that are sent to the brain for decoding. Once hair cells are damaged or die, hearing is impaired, but in most species, such as birds and fish, hair cells spontaneously regrow and hearing is restored. The HRP is tasked with uncovering how to replicate this regeneration process in humans.

The HRP is the first international research consortium focused on investigating hair cell regeneration as a cure for hearing loss and tinnitus in humans. The overarching principle of the HRP consortium is cross-discipline collaboration: open sharing of data and ideas. By having almost immediate access to one another’s data, HRP scientists are able to perform follow-up experiments much faster, rather than having to wait years until data is published.

Kavli Prize winner James Hudspeth, M.D., Ph.D., the head of the Laboratory of Sensory Neuroscience at New York’s Rockefeller University—and my former mentor as well as a 1979 and 1980 Emerging Research Grants scientist—praises the collaborative approach. “During the past few decades, our understanding of the molecular and genetic causes of hearing loss has advanced to a remarkable extent: so much so that we can now envision reversing hearing loss by replacing the sensory hair cells whose damage usually underlies that condition,” says Hudspeth, who was awarded the prestigious prize in neuroscience by the Norwegian Academy of Science in May 2018. “The HRP expedites this process by providing financial support to pathfinding investigations. Perhaps even more importantly, it promotes collaborations among hearing researchers, thus reducing redundant efforts and rapidly propagating new results through a larger community of investigators.”

HRP scientific director Peter G. Barr-Gillespie, Ph.D., is the interim senior vice president for research and a professor of otolaryngology at the Oregon Hearing Research Center, and a senior scientist at the Vollum Institute, all at Oregon Health & Science University. For more, see hhf.org/hrp.

30

hearing health

hhf.org

“[The HRP] promotes collaborations among hearing researchers, thus reducing redundant efforts and rapidly propagating new results through a larger community of investigators.”

—James Hudspeth, M.D., Ph.D., a 2018 winner of the Kavli Prize in Neuroscience.

research

FIRST-YEAR PROJECTS Comparison of three reprogramming cocktails Andy Groves, Ph.D., Baylor College of Medicine

Epigenetics of the mouse inner ear

Stefan Heller, Ph.D., Stanford University

Michael Lovett, Ph.D., Imperial College London; David Raible, Ph.D., University of Washington; Jennifer Stone, Ph.D., University of Washington

Chicks regenerate hair cells in auditory and vestibular organs after damage, making them a valuable animal model to study the signals controlling hair cell regeneration.

This project aims to identify changes in gene expression after hair cell loss in the chick cochlea and vestibular system. The collected data will be comprehensive because it will cover all detectable expressed genes.

Subsequent data analysis will focus on establishing a sequence of gene expression changes that we hypothesize will correlate with important steps of the hair cell regeneration process. These steps include the signals that initiate, execute, sustain, and ultimately terminate the regenerative process.

Comparison among the different organs and across species through collaborations with other HRP investigators will allow us to draw conclusions about species-specific specialized mechanisms as well as more general processes that control hair cell regeneration.

Inner ear supporting cells from newborn mice harbor a latent capacity for some regenerative responses, but these disappear within the first few weeks of life. This observation provides an experimental window that this proposal will exploit to address fundamental questions about the failure of hair cell regeneration in mammals. Specifically, we propose experiments to identify those changes in the genetic material, the chromatin, that are responsible for orchestrating the differentiation of new hair cells within the perinatal organ of Corti; and investigating the changes in the chromatin, the epigenome, that lead to the failure of regeneration in the adult inner ear.

photo credit: jane g photography

Each cell type in the human body is defined by its activation of a unique combination of genes that endow each cell type with specific properties. The activation of these genes is achieved by special proteins known as transcription factors. These “switches� are responsible for turning on appropriate genes in one cell type and preventing inappropriate genes from being activated. In recent years, investigators have identified a number of these transcription factors that lead to the formation of hair cells in the inner ear. The goal of this project is to rigorously test the extent to which a cocktail of transcription factors is able to reprogram supporting cells of the inner ear to turn into hair cells.

Transcriptome changes in single chick cells

a publication of hearing health foundation

summer 2018

31

research

h e ar i n g health foundation

Signaling molecules controlling avian hair cell regeneration Jennifer Stone, Ph.D., University of Washington HRP members have spent the past three years gathering information about genes that are turned on or off after inner ear hair cell damage in the chick, fish, and mouse. Some of these genes may encode therapeutic agents that can be applied to stimulate hair cell regeneration in humans. Our HRP studies and others have found that five signaling pathways (Wnt, VEGF, BMP4, Notch, and FGF) are important regulators of hair cell regeneration in the chick cochlea (the basilar papilla).

The expression and activity of these pathways change significantly after hair cell damage, and the experimental manipulation of activity in each pathway either boosts or dampens hair cell regeneration.

Furthermore, each pathway shows distinct regional expression patterns in the basilar papilla, which implicates it in either mitotic regeneration or non-mitotic regeneration—two distinct ways in which hair cells are replaced after damage. Studies in other growing tissues demonstrate that these five pathways regulate one another in temporally and spatially restricted patterns, in order to coordinate cell growth, differentiation, and patterning. Thus, it is likely that any therapy leading to safe and stable hair cell regeneration will require coordinated manipulation of more than one gene or pathway in the cochlea. In this study, we propose to begin to determine how these five powerful pathways interact to enable and control hair cell regeneration in the chick basilar papilla after hair cell damage.

32

hearing health

hhf.org

Fish epigenomics and enhancer screening Tatjana Piotrowski, Ph.D., Stowers Institute for Medical Research Many genes are turned off by chemical modifications (epigenetic marks) that silence genes and prevent their activation.

This inactivation often occurs at enhancers, which are regions of DNA that control the activation of genes. In this project, Piotrowski will use the ATAC-seq and H3K27ac ChIP-seq methods, which were successful for the mouse inner ear, to find enhancers that are active during hair cell regeneration in fish. Identification of regeneration enhancers will enable the HRP to examine epigenetic marks comparatively—to determine whether regenerating species, such as the zebrafish and chick, utilize different enhancers than non-regenerating species like the mouse, or whether these enhancers are inactive in mammals.

SECOND-YEAR PROJECT Integrated systems biology of hearing restoration Seth Ament, Ph.D., University of Maryland School of Medicine The goal of this proposal is to support the HRP through data integration and systems biology. We propose two related goals for 2018, based on our preliminary network modeling results and discussions with other HRP investigators. We will (a) predict regulatory genes driving cell fate decisions in the developing mouse cochlea using refined transcriptional regulatory networks, gene coexpression networks, protein-protein interaction networks, and related methods. We will (b) extend these analyses to the zebrafish and chick models by projecting networks from the mouse cochlea onto data from these other species. Our goal is to generate testable predictions about driver genes and perturbations (deviations) that could influence hearing restoration.

“The question is not if we will regenerate hair cells in humans, but when.”

—Peter G. Barr-Gillespie, Ph.D., the HRP's scientific director.

research

THIRD-YEAR PROJECT

FOURTH-YEAR PROJECT

Implementing the gEAR for data sharing within the HRP

Mouse functional testing

photo credit: jane g photography

Ronna Hertzano, M.D., Ph.D., University of Maryland School of Medicine The HRP takes a multi-investigator, multi-species, multi-omic (methods of tracking gene expression, or instructions), and cell type–specific approach to define the underpinnings of differences among hair cell regeneration in the chick, fish, and mouse with the aim of identifying keys for hair cell regeneration in mammals. Consequently, the consortium generates large amounts of data that are difficult to visualize, conceptualize, and analyze.

The gEAR portal (gene Expression Analysis Resource, umgear.org) allows for simple visualization of multi-omic, multi-species datasets in the public or private domain—without the need for advanced informatics skills. In the first two years of funding from the HRP, we focused primarily on developing tools for multi-omic, multi-species data upload and visualization. Numerous features were added, and all available HRP datasets were uploaded for sharing within the consortium. In parallel, all tools and features developed for the consortium were made available in the public domain—leading the gEAR to be a primary portal for multiomic data sharing and visualization within the field. With the next two years of funding committed (years three and four), the vast majority of our efforts will be focused on (a) the continued upload of HRP and public datasets, and (b) the development and integration of analysis tools.

John Brigande, Ph.D., Oregon Health & Science University The conceptual framework of this project wrestles with a persistent challenge facing the HRP consortium: We must verify that the candidate genes we advance as regenerative genes actually perform as advertised. Is our altering of the gene expression of a candidate gene truly the trigger that turns supporting cells into hair cells?

Our solution is to devise a mammalian model system that meets several definitive criteria.

First, we need deafened adult mammalian inner ears to detect the production of new hair cells; we achieve this genetically by specifically killing hair cells that are uniquely sensitive to a bacterial toxin.

Second, we need a way to turn on or turn off the candidate gene after the hair cells are dead; we achieve this by chemically activating a gene that in turn unmasks the expression of the proposed candidate. Third, we need a way to detect newly produced hair cells in the cochlea; we achieve this by using a tissue clearing and staining procedure developed with HRP funding that allows us to detect hair cells produced from supporting cells.

This entire approach is called a model system for validating candidate genes for hair cell regeneration. But one size does not fit all, and we need to continually adapt the core model system to achieve full functionality. In this proposal, we aim to test our model system in healthy ears to see if tweaking our candidate genes can produce hair cells from supporting cells in the absence of widespread hair cell death.

The idea here is to make sure that the bacterial toxin–mediated destruction of hair cells is not interfering with our candidate gene activity and new hair cell production.

Our second goal is to test a new virus delivery system that will allow us to evaluate larger candidate genes.

Presently, we can only express very small genes with the virus we are using, restricting candidate gene verification. Our final goal is to evaluate a modified viral vector that is encased in lipid membranes to learn if it can express candidate genes more efficiently in supporting cells.

The benefit of this approach is that viral production is quick, inexpensive, and requires no special training or expertise. Successful completion of this proposal will establish a comprehensive, costeffective approach to aggressively validate candidate genes for hair cell regeneration.

Support our research: hhf.org/donate

a publication of hearing health foundation

summer 2018

33

research

h e ar i n g health foundation

Recent Research by Hearing Health Foundation Scientists, Explained A Powerful New Bioinformatics Tool

For references, see hhf.org/ spring2018-references.

our paper describing a new bioinformatics tool—and to showcase the software, a very detailed investigation as to how inner ear hair cells assemble their hair bundles—appeared in Cell Reports on June 5, 2018.

The creation of the CellTrails tool was supported in part by Hearing Health Foundation’s Hearing Restoration Project (HRP); moreover, it is the product of recognizing existing limitations of data analysis, going back to the drawing board multiple times, and finally getting to a “product” that is going to be the workhorse to analyze a good part of the bioinformatics data that the HRP has been accumulating for years.

The idea for the tool came from conversations between HRP scientific director Peter G. Barr-Gillespie, Ph.D., and me, and our getting stuck with trying to make sense of all the data; thus the tool is the direct product of interactions through the HRP. It follows on our work utilizing single-cell gene expression analysis to examine the genetic instructions allowing individual cells to differentiate (change) into other types of cells, such as inner ear supporting cells that turn into hair cells in species other than mammals, thereby restoring hearing.

The tool helps us pinpoint where specific single cells are located in an organ, and their trajectories as they undergo transformations—information that was lost or fuzzy before.

With it we can create a more robust, visually rendered gene expression landscape. Two postdoctoral fellows in my lab were instrumental in CellTrails: bioinformatics researcher Daniel Ellwanger, Ph.D., the tool’s primary developer, and Mirko Scheibinger, Ph.D., who validated its predictions.

I hope many researchers make use of CellTrails, accessible online, to analyze their own mountains of data. As I told Stanford’s SCOPE Blog, “Single cell transcriptome analysis and reconstruction of spatial and temporal relationships among cells is an exploding new technology. A lot of labs are faced with the challenge of analyzing the data from single cells. This study is a rather extensive study that goes beyond the inner ear field because it provides a new way to analyze single cell transcriptomic data.”

I truly feel that the seeds that were planted years ago are now growing into sizable plants—we have a massive "chick regeneration inner ear plant” that is starting to thrive!

—Stefan Heller, Ph.D. Stefan Heller, Ph.D., a Stanford University professor of otolaryngology–head and neck surgery, is a member of HHF’s Hearing Restoration Project, where Oregon Health & Science University’s Peter G. Barr-Gillespie, Ph.D., is the scientific director.

Find the CellTrails tool at hellerlab.stanford.edu/celltrails.

34

hearing health

hhf.org

research

Uncovering a Signaling Molecule That Modulates Avian Hair Cell Regeneration mammals including humans cannot regenerate hair cells, but other species such as birds and fish readily regenerate hair cells after damage to restore auditory function. The gene ATOH1 produces a protein that pushes supporting cells—cells that neighbor hair cells—to either directly convert into a hair cell or divide and form a new hair cell. However, ATOH1 expression (when the gene is turned on) does not guarantee that hair cells develop in birds or mammals, which suggests that there are factors that prevent supporting cells from changing into hair cells. Identifying these factors in birds may help us better understand the lack of hair cell regeneration in mammals.

We examined the avian auditory system to characterize a potential inhibitor to ATOH1 during hair cell regeneration: bone morphogenetic protein 4 (BMP4). Bone morphogenetic proteins are secreted signaling molecules that regulate cellular processes in many regions of the body, including the nervous system. We found that BMP4 localizes to hair cells of the mature avian hearing organ and disappears when hair cells die or sustain damage. From this, we hypothesized that BMP4 may prevent ATOH1 expression in supporting cells, and loss of BMP4 when hair cells die may enable ATOH1 to be expressed in supporting cells, driving them to convert into hair cells.

When we exposed avian auditory organs to BMP4 after selectively killing hair cells, this prevented ATOH1 expression and hair cell regeneration. When we antagonized BMP4 using an inhibitor, we found a

generally opposite result: an increase in the number of regenerated hair cells.

We conclude that BMP4 is a potent inhibitor of ATOH1 and therefore suppresses hair cell regeneration. We recommend that BMP4 be explored further in studies of mammalian hair cell regeneration.

Published in Hearing Research on May 2, 2018, this study detailing BMP4’s negative effect on ATOH1 expands our knowledge of signaling molecules that suppress hair cell regeneration in birds and may also modulate hair cell regeneration in humans. —Rebecca M. Lewis, Au.D., Ph.D., and Jennifer S. Stone, Ph.D.

Rebecca M. Lewis, Au.D., Ph.D. (far left), is a clinical audiologist and auditory neuroscientist at Massachusetts Eye and Ear, Harvard Medical School, in Boston. HRP researcher Jennifer S. Stone, Ph.D., is the director of research in the department of otolaryngology– head and neck surgery at the Virginia Merrill Bloedel Hearing Research Center at the University of Washington.

For more, see hhf.org/hrp.

This schematic depicts current ideas as to how BMP4 regulates ATOH1 expression and therefore hair cell regeneration in the avian hearing organ. It shows (from left) typical hair cells, hair cell damage, and hair cell regeneration. Typical hair cells secrete BMP4. When hair cells die, BMP4 signaling is reduced, which allows ATOH1 to be expressed in supporting cells and pushes them to turn into hair cells. The newly regenerated hair cells secrete BMP4, suppressing ATOH1 in supporting cells and restoring the typical condition.

a publication of hearing health foundation

summer 2018

35

research

h e ar i n g health foundation

EMERGING RESEARCH GRANTS

For references, see hhf.org/ spring2018-references.

Novel Drug-Delivery Method to the Inner Ear researchers have developed a new approach to be able to repair cells deep inside the ear. The study, conducted by scientists at University of Southern California (USC) and Harvard University, demonstrates a novel way for a future drug to zero in on damaged nerves and cells inside the ear.

“What’s new here is we figured out how to deliver a drug into the inner ear so it actually stays put and does what it’s supposed to do, and that’s novel,” says study co-author Charles E. McKenna, Ph.D., a chemistry professor at the USC Dornsife College of Letters, Arts, and Sciences. “Inside this part of the ear, there’s fluid constantly flowing that would sweep dissolved drugs away, but our new approach addresses that problem. This is a first for hearing loss and the ear.”

The paper, published in Bioconjugate Chemistry on April 4, 2018, is also coauthored by lead researcher Judith S. Kempfle, Ph.D., a 2011 and 2012 Emerging Research Grants scientist, and Hearing Restoration Project member Albert Edge, Ph.D., who are both at Harvard Medical School and The Eaton-Peabody Laboratories in Boston.

There are caveats. The study was conducted on animal tissues in a petri dish, and it has not yet been tested in living animals or humans. Yet the researchers are hopeful given the similarities of cells and mechanisms involved. McKenna says since the technique works in the laboratory, the findings provide “strong preliminary evidence” it could work in living creatures.

The team figured out a new way to deliver a drug into the inner ear so that the drug actually stays put and does what it’s supposed to do.

The groundbreaking drug-delivery method targets the cochlea, where due to age or noise, sensory hair cells and bundles of neurons that transmit their vibrations can break down, as do ribbon-like synapses, which connect the cells, leading to hearing loss.

The scientists designed a molecule combining 7,8-dihydroxyflavone, which mimics a protein critical for development and function of the nervous system, and bisphosphonate, a type of drug that sticks to bones. This pairing delivered the breakthrough solution, as neurons responded to the molecule and regenerated synapses in mouse ear tissue. This led to the repair of the hair cells and neurons, which are essential to hearing.

“We’re not saying it’s a cure for hearing loss,” McKenna says. “It’s a proof of principle for a new approach that’s extremely promising. It’s an important step that offers a lot of hope.” —Gary Polakovic, USC News A 2011 and 2012 Emerging Research Grants scientist, Judith Kempfle, M.D., is a senior research fellow at Massachusetts Eye and Ear, Harvard Medical School, where Hearing Restoration Project member Albert Edge, Ph.D., is a professor of otolaryngology.

36

hearing health

hhf.org

research

A Pressure Relief Valve in the Inner Ear the inner ear senses sound in order to hear as well as sensing head movements in order to balance. Sounds or body movements create waves in the fluid within the ear. Specialized cells called hair cells, because of their thin hairlike projections, are submerged within this fluid. Hair cells bend in response to these waves, with channels that open in response to the bending. The makeup of the ear’s internal fluid is critical because as it flows through these channels its contents encode the information that becomes a biochemical and then a neural signal. The endolymphatic sac of the inner ear is thought to have important roles in stabilizing this fluid that is necessary for sensing sound and balance.

While imaging transparent zebrafish, my team and I found a pressure-sensitive relief valve in the endolymphatic sac that periodically opens to release excess fluid, thus preventing the tearing of tissue. In our paper published in the journal eLife June 19, 2018, we describe how the relief valve is composed of physical barriers that open in response to pressure. The barriers consist of cells adhering to one another and thin overlapping cell projections that are continuously remodeling and periodically separating in response to pressure.

The unexpected discovery of a physical relief valve in the ear emphasizes the need for further study into how the ear controls fluid pressure, volume, flow, and ion homeostasis (balance of ions) in development and disease. It suggests a new mechanism underlying several hearing and balance disorders characterized by pressure abnormalities, including Ménière’s disease.

Watch a time-lapse video (youtube.com/watch?v=Hz0VlUVjYfI) of the endolymphatic sac, with the sac labeled “pressure relief valve” at 0:40.

—Ian Swinburne, Ph.D. This illustration shows the major inhibitory and excitatory, ascending and descending, neurotransmitter connections of subcortical pathways.

A 2017 Ménière’s Disease Grants scientist (and a 2013 Emerging Research Grants recipient), Ian Swinburne, Ph.D., is a researcher at Harvard Medical School.

The unexpected discovery of a physical relief valve in the ear emphasizes the need for further study into how the ear controls fluid pressure, volume, flow, and ion homeostasis (balance of ions) in development and disease. a publication of hearing health foundation

summer 2018

37

research

h e ar i n g health foundation

EMERGING RESEARCH GRANTS

The three Emerging Research Grants scientists on these pages were each generously funded by the General Grand Chapter Royal Arch Masons International, for their innovative work investigating difficulties with how the brain processes sound, a condition known as central auditory processing disorder (CAPD).

38

hearing health

hhf.org

Hearing Aids Improve Brain Function and Memory in Older Adults one of the most prevalent health conditions among older adults, age-related hearing loss, can lead to cognitive decline, social isolation, and depression. However, new research from the University of Maryland’s department of hearing and speech sciences (HESP) shows that the use of hearing aids not only restores the capacity to hear, but can improve brain function and working memory.

The research team monitored a group of first-time hearing aid users with mild-tomoderate hearing loss over a period of six months. The researchers used a variety of behavioral and cognitive tests designed to assess participants’ hearing as well as their working memory, attention, and processing speed. They also measured electrical activity produced in response to speech sounds in the auditory cortex and midbrain.

Hearing aid use can at least partially restore deficits in cognitive function and auditory brain function in older adults.

At the end of the six months, participants showed improved memory, improved neural speech processing, and greater ease of listening as a result of the hearing aid use.

Findings from the study were published in two journals, Clinical Neurophysiology and Neuropsychologia, in June 2018.

“Our results suggest that the benefits of auditory rehabilitation through the use of hearing aids may extend beyond better hearing and could include improved working memory and auditory brain function,” says HESP Assistant Professor Samira Anderson, Ph.D., who led the team. “In effect, hearing aids can actually help reverse several of the major problems with communication that are common as we get older.”

According to the National Institutes of Health, as many as 28.8 million Americans could benefit from wearing hearing aids, but fewer than a third of that population actually uses them. Several barriers prevent more widespread use of hearing aids—namely, their high cost and the fact that many people find it difficult to adjust to wearing them.

A growing body of evidence has demonstrated a link between hearing loss and cognitive decline in older adults. Aging and hearing loss can also lead to changes in the brain’s ability to efficiently process speech, leading to a decreased ability to understand what others are saying, especially in noisy backgrounds.

The UMD researchers say the results of their study provide hope that hearing aid use can at least partially restore deficits in cognitive function and auditory brain function in older adults.

“We hope our findings underscore the need to not only make hearing aids more accessible and affordable for older adults, but also to improve fitting procedures to ensure that people continue to wear them and benefit from them,” Anderson says.

The researchers are working on developing better procedures for fitting people with hearing aids for the first time.

—University of Maryland Department of Hearing and Speech Sciences

A 2014 Emerging Research Grants scientist, Samira Anderson, Au.D., Ph.D., is an assistant professor in the department of hearing and speech sciences at the University of Maryland, College Park, where she is also the director of its Hearing Brain Lab. Anderson’s two new papers and her prior research published in the journal Ear and Hearing all stemmed from her 2014 ERG project related to CAPD.

research

Improving Speech Recognition in Bilateral Cochlear Implant Users beyond developing new technology to assist with hearing loss, researchers also improve on existing technologies. University of Wisconsin–Madison’s Alan Kan, Ph.D., has been investigating how to improve listening for users of bilateral cochlear implants (BiCls).

Frequently it is challenging for BiCI users to focus on a target speaker in noisy situations. As published in the journal Trends in Hearing on April 30, 2018, Kan devised a new way to use currently existing technology to alleviate this issue.

Based on the finding that BiCI users often have a dominant ear, Kan created an algorithm to refine CI signal processing, so that the implant in the better ear picks up the target speaker’s speech, while ambient noise is sent to the implant in the less-dominant ear. During his study on 11 participants, he found an average improvement of a 4.4 decibel signal-to-noise ratio in the BiCI user’s speech reception threshold when using the better ear strategy, and no listener demonstrated a decrease in performance.

The study notes that this effect can likely be reproduced even without the signal processing, simply by making sure the target voice is speaking on the side of the BiCl user’s dominant ear. This is promising as it demonstrates that clinician advice may greatly enhance a BiCI user’s experience. The paper adds that this approach may also prove beneficial in bilateral hearing aid users.

—Elizabeth Crofts

Based on the finding that bilateral CI users often have a dominant ear, Kan created an algorithm to refine CI signal processing, so that the implant in the better ear picks up the target speaker’s speech, while ambient noise is sent to the implant in the less-dominant ear.

A 2013 Emerging Research Grants scientist, Alan Kan, Ph.D., is an assistant scientist in the Binaural Hearing and Speech Lab at the University of Wisconsin–Madison.

HHF intern Elizabeth Crofts will be a junior at Boston University studying biomedical engineering.

Children’s Speech Perception in Noise listening to speech in noisy environments is particularly challenging for individuals who have central auditory processing disorder (CAPD). In both children and adults, speech perception-in-noise (SPIN) tests are therefore used as part of hearing evaluations.

Previous studies have suggested that cognitive abilities such as working memory capacity and language abilities contribute significantly to SPIN. However, in our study in the Journal of Speech, Language, and Hearing Research in May 2018, my colleagues and I observed no significant association between school-age children’s performance on the widely used Bamford-Kowal-Bench Speech in Noise (BKB-SIN) test and a range of standardized language measures, attention, and working memory measures.

The 83 children ages 7 to 11 who participated represented a broad range of cognitive ability and had typical hearing and nonverbal IQ levels. Our findings align with those studies that report either weak or no

associations between cognitive-linguistic factors and SPIN. While further research is needed to identify the underlying mechanisms that are crucial to SPIN in children, our study results show that children’s performance on the BKB-SIN test is not significantly influenced by their memory and language abilities. Hence, the utility of BKB-SIN as a clinical tool to assess SPIN is supported. —Beula Magimairaj, Ph.D.

A 2015 Emerging Research Grants scientist, Beula Magimairaj, Ph.D., is an assistant professor in the department of communication sciences and disorders at the University of Central Arkansas.

a publication of hearing health foundation

summer 2018

39

research

h e ar i n g health foundation

Quantifying the Effects of a Hyperacusis Treatment a typical inner ear has two mobile windows: the oval and the round window (RW). The flexible, membrane-covered RW allows fluid in the cochlea to move as the oval window vibrates in response to movement from the stapes bone during sound stimulation.

Superior canal dehiscence (SCD), a pathological opening in the bony wall of the superior semicircular canal, forms a third window of the inner ear. This structural anomaly results in various auditory and vestibular symptoms. One common symptom is increased sensitivity to self-generated sounds or external vibrations, such as hearing one’s own pulse, neck and joint movement, and even eye movement. This hypersensitive hearing associated with SCD has been termed conductive hyperacusis.

Recently, surgically stiffening the RW is emerging as a treatment for hyperacusis in patients with and without SCD. However, the postsurgical results are mixed: Some patients experience improvement, while others complain of worsening symptoms and have asked to reverse the RW treatment. Although this “experimental” surgical treatment for hyperacusis is increasingly reported, its efficacy has not been examined scientifically.

In the present study, we experimentally tested how RW reinforcement affects air-conduction sound transmission in the typical ear (that is, without a SCD). We measured the sound pressures in

40

hearing health

hhf.org

Conductive hyperacusis is associated with an increased sensitivity to self-generated sounds or external vibrations, such as hearing one’s own pulse, neck and joint movement, and even eye movement. two cochlear fluid–filled cavities—the scala vestibuli (assigned the value “Psv”) and the scala tympani (“Pst”)— together with the stapes velocity in response to sound at the ear canal.

We estimated hearing ability based on a formula for the “cochlear input drive” (Pdiff = Psv – Pst) before and after RW reinforcement in a human cadaveric ear.

We found that RW reinforcement can affect the cochlear input drive in unexpected ways. At very low frequencies, below 200 hertz (Hz), it resulted in a reduced stapes motion but an increase in the cochlear input drive that would be consistent with improved hearing. At 200 to 1,000 Hz, the stapes motion and input drive both were slightly decreased.

Above 1,000 Hz stiffening the RW had no effect.

The results suggest that RW reinforcement has the potential to worsen low-frequency hyperacusis while causing some hearing loss in the mid-frequencies. Although this preliminary study shows that the RW treatment does not have much effect on air-conduction hearing, the effect on bone-conduction hearing is unknown and is one of our future areas for experimentation.

—Xiying Guan, Ph.D.

A 2017 Emerging Research Grants scientist funded by Hyperacusis Research Ltd., Xiying Guan, Ph.D., is a postdoctoral fellow at Massachusetts Eye and Ear, Harvard Medical School, in Boston.

research

EMERGING RESEARCH GRANTS ERG Alumni Making Headlines hhf’s cornerstone for six decades has been funding innovative, early-career hearing and balance researchers through its Emerging Research Grants (ERG) program. Many go on to earn prestigious National Institutes of Health funding. These ERG alumni recently earned media attention for their scientific breakthroughs: Roland Eavey, M.D., a 1987–88 ERG scientist: The chair of the otolaryngology department at Vanderbilt University coauthored a report in The Journal of Nutrition on May 11, 2018, that showed women with healthier diets had a lower risk of hearing loss. The healthier diets emphasized fruits, vegetables, fish, seafood, nuts, beans, legumes, and olive oil over dairy, meat, and poultry. The longitudinal study spanning 22 years and including more than 70,000 women showed those with a better eating habits cut their risk for moderate or worse hearing loss by 30 percent.

Richard Kopke, M.D., 1996 ERG: The CEO of Oklahoma’s Hough Ear Institute and a 26-year U.S. Army veteran, Kopke coauthored a March 2018 paper in Molecular Therapy about the use of small interfering RNAs (siRNAs) to block the activity of the Notch signaling pathway gene Hes1. Hes1 has been shown to itself block supporting cells from converting into hair cells. The team found that a sustained release of siRNAs in mouse cochleae through nanoparticles after deafening resulted in the recovery of some hearing ability, measured using auditory brainstem responses. Compared with the control mice, the injected mice showed less overall hair cell loss and early signs of immature hair cell development, which the authors say may signal hair cell regeneration.

John Oghalai, M.D., 1996–97 ERG: Oghalai, of the University of Southern California, coauthored a May 7, 2018, study in the Proceedings of the National Academy of Sciences showing promise for preventing noise-induced hearing loss. Using a mouse model, the investigators found that in addition to immediate hair cell death after loud noise exposure, a fluid buildup in the inner ear occurs, eventually leading to nerve cell loss. Because the extra fluid shows a high potassium level, the researchers saw a method to rebalance the fluid by injecting a salt and sugar solution into the ear. Nerve cell loss was reduced by 45 to 64 percent, which the team says may preserve hearing. The team sees future applications for military service members exposed to blast trauma and patients with the hearing and balance disorder Ménière’s disease.

Anthony Ricci, Ph.D., 1999–2000 ERG, and Alan Cheng, M.D., 2002–03 ERG: Ricci, a professor of otolaryngology–head and neck surgery at Stanford University, and Cheng, a Stanford associate professor of otolaryngology, are developing a new type of aminoglycosides, a widely used, life-saving class of antibiotic that fights a broad range of serious infections and diseases such as cystic fibrosis and tuberculosis, but that also has the side effect of hearing loss in one in five patients. The pair have been collaborating since 2008, leveraging Ricci’s knowledge of mechanotransduction (how sound wave vibrations are converted into electrical signals) and ion channels. Of the 18 potential replacement antibiotics they created, three show the most promise for preserving hearing while remaining effective in killing bacteria and will be tested further. —Yishane Lee

For more, see hhf.org/research. For references, including to all the papers cited on pages 34–41, see hhf.org/summer2018-references.

Support our research: hhf.org/donate

a publication of hearing health foundation

summer 2018

41

roundup

h e ar i n g h ealth foundation

Researchers and role models alike discuss hearing loss among young people.

Talking

Tweens & Teens

From the role models

42

hearing health

“You don’t have to be good at singing.

You don’t have to be good at dancing or acting. If you like to do it, if you genuinely enjoy doing it, then do it. No one should stop you.” —Millie Bobby Brown, age 14, who plays Eleven in the Netflix series “Stranger Things,” on being born with partial hearing loss in one ear that worsened to the point where she is unable to hear herself in one ear.

“I used to have tinnitus around the age of 7 or 8. I was reading the Oliver Sacks book ‘Musicophilia,’ and that made me think that this would be a condition that Baby suffers from and encourages him to listen to music 24/7.” —Edgar Wright, the writer and director of the film “Baby Driver,” on the main character of his film, a teenage getaway driver named Baby who has tinnitus that he masks with constant music. People with tinnitus and medical experts have praised the action film for raising awareness about the condition.

a publication of hearing health foundation

“I hope seeing people like me on screen inspires more people to chase their own dreams, and shows deaf kids anything is a possibility for them, because I really don't feel like my deafness was an obstacle or should be a big deal.” —Millicent Simmonds, age 15, who stars in the movies “Wonderstruck” and “A Quiet Place.” With her hearing loss the result of ototoxic drug exposure as an infant, Simmonds uses a cochlear implant and communicates primarily through sign language (whose use was a key plot point in “A Quiet Place”).

“Being someone who is hearing impaired is day in, day out.

It is so much more than just not being able to hear as well. I have to work hard to not let life pass me by.

It certainly is a challenge. I’m proud of what I’ve achieved and what I’ve overcome.” —Tufts University graduate Zach Wallace, an AllAmerican swimmer and four-time school record-holder, who will be starting a master’s degree in bioinformatics, also at Tufts, this fall.

roundup

“There were so many words I wasn’t hearing, that I just knew I couldn’t hear and make out, that I just started to feel sorry for myself. I felt lonely. I felt disconnected. I felt inept. And that’s rough. But you deal with that and you move on—or you use it as fuel to work harder.”

—Composer Alex Lacamoire, diagnosed at age 3 with a hearing loss, who was mainstreamed into his local Los Angeles school, skipping first grade, on feeling embarrassed about his hearing aids as a teen. He went on to become the Tony Award–winning music director of the Broadway hit “Hamilton.”

From the researchers “Hearing loss, even minor deficits, can take a toll in young people—they’re using cognitive resources that could be preserved until much later in life.

Most concerning, this early hearing loss could pave the way for dementia.”

—Ohio State University assistant professor Yune S. Lee, Ph.D., the lead researcher of a May 21, 2018, paper in eNeuro that found adults ages 18 to 41 with minor hearing deficits showed signs of right frontal cortex activity to process speech, instead of only the left hemisphere, suggesting that a subtle hearing decline already induces the brain to compensate to understand speech and which may later affect other brain functions such as memory.

“Signs of noise-induced hearing loss (NIHL) may already be present in children aged 9 to 11 years old and may be associated with portable music player use prior to exposure to known noise hazards, such as club and concert attendance.” —Dutch researcher Carlijn M. P. le Clercq, the lead author of a cross-sectional, population-based study published in JAMA Otolaryngology–Head and Neck Surgery on June 14, 2018, which found 1 in 7 children showed high frequency hearing loss and audiometric notches; children who used portable music players at least one day a week were nearly three times as likely to have high frequency hearing loss than those who didn’t. To help prevent NIHL, use volume-limiting headphones and set maximum device volumes via settings or apps.

“Hearing is not the ear. It’s the brain.

And it takes time for the brain to understand what those sounds mean. [I found] that there were so many... social determinants of health outside the operating room that were impeding how my patients did.” —University of Chicago cochlear implant surgeon Dana Suskind, M.D., who launched the Thirty Million Words Initiative to support a language-rich environment for lower-income children, after realizing that the correlation between the use of language and the hearing success of new cochlear implant users is applicable to the use of language and developmental success among all children.

See hhf.org/summer2018-references.

Share your story: How have you overcome a childhood hearing loss?? Tell us at editor@hhf.org.

Support our research: hhf.org/donate a publication of hearing health foundation

summer 2018

43

can a

chicken help

CURE

hearing loss and tinnitus?

The answer, incredibly, is YES! Learn more at Hearing Health Foundation’s website, hhf.org.

Hearing Health Foundation's (HHF) mission is to prevent and cure hearing loss and tinnitus through groundbreaking research and to promote hearing health. HHF is the largest nonprofit funder of hearing and balance research in the U.S. and a leader in driving new innovations and treatments for people with hearing loss, tinnitus, and other hearing disorders.

HHF’s Hearing Restoration Project (HRP) is the first international research consortium focused on investigating hair cell regeneration as a cure for hearing loss and tinnitus.

The overarching principle of the HRP consortium is cross-discipline collaboration: open sharing of data and ideas. By having almost immediate access to one another’s data, HRP scientists are able to perform follow-up experiments much faster, rather than having to wait years until data is published.

Thanks to the generosity of our donors, our researchers have achieved significant advancements. But much more must be done to propel the discoveries of cures for hearing loss, tinnitus, and related conditions. Support our life-changing research at hhf.org/donate.

a publication of hearing health foundation

summer 2018

45

h earing health foundation

TechSolutions

advertisement

46

hearing health

hhf.org

This collection of sponsored products represents current trends in hearing technology.

CapTel® Captioned Telephone

Hear Every Word, Not Every Third

Enjoy the phone again with confidence! Ideal for people with hearing loss, the CapTel® Captioned Telephone shows captions of every word the caller says over the phone.

You can listen to the caller, and read the written captions in the CapTel display screen. Only CapTel gives you several models to choose from, including contemporary touch-screen options and traditional telephone styles. All CapTel phones include a large display screen, adjustable font sizes and colors, and a builtin answering machine that shows captions of your messages. CapTel gives you the confidence to reconnect over the phone, knowing you won’t miss a word!

Visit CapTel.com.

The easy-to-use Panasonic KXTGM430B amplified phone is ideal for anyone affected by hearing loss.

Bright LEDs signal incoming calls.

Boost caller volume up to 40 decibels (dB) and ringer volume up to 100 dB. Slow down fast talkers while conversing/checking messages with the slow talk feature. Sync up to two smartphones to make/take amplified cell calls from anywhere in the house. Includes enlarged dial pads, speakerphone, bilingual talking caller ID and call block.

Expandable to six handsets.

Visit shop.panasonic.com/amplified.

These featured products are paid advertisements.

To advertise in Hearing Health magazine, please email advertising@hhf.org or call 212.257.6141.

Better hearing, better learning

Get a free ConnectClip with your purchase of Oticon Opn hearing instruments ordered for children ages 0-18!* NEW

Making one-to-one communication easier With Oticon ConnectClip, children and teens can hear more of what’s happening around them — especially listening in the classroom, hearing the coach on the field, or keeping up with a dance instructor.

Clarity in the classroom By simply attaching the Oticon ConnectClip to the teacher’s shirt, students can now hear their teacher’s voice loud and clear directly in their Oticon Opn™ hearing aids.

Get even more with Oticon Opn and ConnectClip ConnectClip’s excellent sound quality, 65 ft. range and extended bandwidth can keep children and teens connected at home, play and school.

*Some exclusions may apply. See your hearing care professional for more information. Expires 10/31/18

• Make safe, hands-free calls, streamed to both ears • Wirelessly stream music & videos in stereo • Compatible with thousands of Bluetooth devices

Learn more about Oticon Opn and Oticon ConnectClip at www.oticon.com/opn

advertisement

h earing health foundation

EarDial: The Invisible Smart Earplugs for Live Music Live music goers: Save your hearing while enjoying your music at full fidelity with the world's first invisible earplugs with a companion mobile app. »» High fidelity, precise noise filtration: Hear the music and chat with your friends while protecting yourself from harmful sound decibels. »» Low-profile design, high transparency: Almost invisible when you put them in your ears. »» Soft, hypoallergenic silicon: Wear them comfortably for long hours, with exclusive earwax protection. »» Compact aluminum case: Easy to store, and to carry around at all times. »» Companion mobile app: Know the sound level around you, and how long you can stay safely.

Visit eardial.com.

These featured products are paid advertisements.

To advertise in Hearing Health magazine, please email advertising@hhf.org or call 212.257.6141.

Innovative Real-Time Captioning for Your Smartphone Calls

Find more information online:

INNOCAPTION.COM

48

hearing health

hhf.org

marketplace

SUPPORTERS OF HEARING HEALTH Cochlear™

Captioned Telephone

At Cochlear, we strive to connect your life to a world full of sound.

We’re committed to delivering revolutionary cochlear implant and bone-anchored hearing technologies to help you enjoy, connect to, and interact with a world of sound.

CapTel captioned telephone shows word-for-word captions of everything a caller says over the phone. Like captions on TV—for your phone. Helps people with hearing loss enjoy phone conversations, confident they’ll catch every word.

You no longer need to miss out on what was said during a telephone call. Visit hamiltoncaptel.com to learn about a free service that provides captions for your telephone conversations.

cochlearamericas.com 800.523.5798

captel.com 800.233.9130

hamiltoncaptel.com 888.514.7933

page 5

pages 21, 46

page 51

This smartphone app provides captioning for incoming and outgoing calls within one to two seconds with 95 percent accuracy, using stenographers, not voicerecognition technology. FCC-funded, InnoCaption is free to use for those with hearing loss.

The Les Paul Foundation inspires innovative and creative thinking by sharing the legacy of Les Paul through support of music education, recording, innovation, and medical research related to hearing.

By advancing the field of hearing implant technology, MED-EL’s people and products connect individuals around the globe to the rich world of sound.

lespaulfoundation.org 212.687.2929

medel.com 888.MEDEL.CI (888.633.3524)

page 48

pages 29, 50

page 52

“A better future for every child with hearing loss” is our mission, reflected in our BrainHearing approach to support a child’s cognitive development. The Sensei instrument family provides access to a full range of sound with the highest safety and reliability.

Hear and be heard, loud and clear.

Panasonic’s amplified cordless phone systems are ideal for everyone affected by hearing loss. Among many features, the Volume Booster amplifies the call volume up to 50 decibels.

innocaption.com 703.865.5553

oticon.com peoplefirst@oticonusa.com page 47

shop.panasonic.com/amplified

pages 2, 46

ReSound offers hearing aids for any listening environment and lifestyle.

Find the perfect hearing aid for your needs from one of the world’s largest manufacturers.

resound.com 800.248.4327 page 13

a publication of hearing health foundation

summer 2018

49

meet the researcher

hearing health foundation

EMERGING RESEARCH GRANTS

Meet the Researcher

Emerging Research Grants (ERG) As one of the only funding sources available for innovative research, HHF’s ERG program is critical. Without our support, scientists would not have the needed resources for cutting-edge approaches toward understanding, preventing, and treating hearing and balance disorders.

Tenzin Ngodup, Ph.D.

Oregon Health & Science University

Ngodup earned his Ph.D. in neuroscience from the State University of New York at Buffalo and is now a postdoctoral fellow at Oregon Hearing Research Center, Oregon Health & Science University.

A 2018 Emerging Research Grants scientist, Ngodup received the Les Paul Foundation Award for Tinnitus Research.

In His Words neuron (nerve cell) hyperactivity is believed to lead to tinnitus, or experiencing ringing or buzzing in the ears without an external sound source. While many studies on hyperactivity have focused on the dorsal cochlear nucleus, an auditory processing region in the brainstem, very little attention has been given to the ventral cochlear nucleus (VCN). This is surprising since the VCN is home to neurons that receive direct inputs from auditory nerve fibers, and the majority of VCN neurons convey excitatory (stimulating) signals to higher auditory regions. one of the likely causes of hyperactivity is an imbalance between excitatory and inhibitory (dampening) neuronal connections, or synapses. However, so far only a single inhibitory cell type, called D-Stellate, has been identified in the VCN. With the use of genetically modified mice, we now see that the diversity of inhibitory cell types and connections in the VCN is far richer than previously described. the quantification of all inhibitory neurons in the VCN can be used to examine inhibition in typical vs. tinnitus models, especially after noise exposure. We want to understand how a loss of inhibition (less dampening) could lead to the hyperactivity associated with tinnitus in order to help prevent and treat tinnitus. as a tibetan, officially I am stateless, but I was born and raised in India. I have always been interested in science, biology in particular. I am fascinated by how very complicated and different organ systems in our body work together so efficiently. Science classes were especially fun-filled with hands-on learning through field trips and lab experiments, an active and highly engaging style of teaching that had a profound impact on my interest in science.

50

hearing health

hhf.org

if i were not a researcher, I would be a science teacher. Actually, after earning my bachelor’s degree in biology and education, I taught biology to Tibetan monks in a Tibetan Buddhist monastic school in southern India. i am the first scientist in the family—perhaps one of the first Tibetan neuroscientists in the exiled Tibetan community! I am working on a cookbook about traditional Tibetan dishes, a cuisine that reflects the lifestyle of Tibetans living at more than 10,000 feet above sea level.

For instance, rice is a rare commodity so a dish like dre-si (sweet rice) is served mainly during special occasions like the Tibetan New Year.

Tenzin Ngodup, Ph.D., received the Les Paul Foundation Award for Tinnitus Research. We thank the Les Paul Foundation for its support of innovative research that will increase our understanding of the mechanisms, causes, diagnosis, and treatment of tinnitus. Read more about Les Paul on page 29.

We need your help in funding the exciting work of hearing and balance scientists. Please consider donating today to Hearing Health Foundation to support groundbreaking research.

Visit hhf.org/how-to-help.

HamiltonCapTel.com/apps Voice and data plans may be required when using Hamilton CapTel on a smartphone or tablet.

061918

FEDERAL LAW PROHIBITS ANYONE BUT REGISTERED USERS WITH HEARING LOSS FROM USING INTERNET PROTOCOL (IP) CAPTIONED TELEPHONES WITH THE CAPTIONS TURNED ON. IP Captioned Telephone Service may use a live operator. The operator generates captions of what the other party to the call says. These captions are then sent to your phone. There is a cost for each minute of captions generated, paid from a federally administered fund. To learn more, visit fcc.gov. • Hamilton CapTel may be used to make 911 calls, but may not function the same as traditional 911 services. For more information about the benefits and limitations of Hamilton CapTel and Emergency 911 calling, visit HamiltonCapTel.com/911. • Copyright © 2018 Hamilton Relay. All rights reserved. Hamilton is a registered trademark of Nedelco, Inc. d/b/a Hamilton Telecommunications. CapTel is a registered trademark of Ultratec, Inc.

Struggling with simple conversations and social situations?

YOUR HEARING AIDS MAY NOT BE ENOUGH. MED-EL offers the most advanced portfolio of hearing solutions to help thousands of people like you hear again.

We can help you take the next step.

VISIT

JourneyToBetterHearing.com FOR YOUR FREE WELCOME KIT!

Most reliable system

Superior performance

Most natural hearing

JourneyToBetterHearing.com COCHLEAR IMPLANT SYSTEMS

|

ELECTRIC- ACOUSTIC SYSTEMS

|

MIDDLE EAR IMPLANT SYSTEMS

For information on potential risks and contraindications relating to implantation, please visit www.medel.com/us/isi-cochlear-implant-systems/