Quantifying the Effects of a Hyperacusis Treatment

By Xiying Guan, Ph.D

A typical inner ear has two mobile windows: the oval and 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 studied 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 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 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.

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

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My Magic Ear Kid

By Joey Lynn Resciniti

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 startle 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 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.

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 very quick introduction to hearing loss and the varying levels of severity.

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.

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).

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. 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 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.

This article originally appeared on the cover of the Summer '18 issue of Hearing Health magazine with a supporting story from Julia Resciniti  

Challenges: A Sibling’s Perspective

By Joe Mussomeli

Everyone has challenges in their life; they can be small or big, but they’re still challenges. My brother, Alex, was born with severe hearing loss—the first in my family to have the honor.

Alex’s diagnosis marked the start of very stressful period for our family. It took some time for my mother to process his hearing loss, but both of my parents quickly recognized the importance of helping Alex get access to sound as soon as possible. They equipped Alex with hearing aids before three months old and our journey began.

I don’t remember too much of the details, as I was only two years old at the time, but I was told that my parents spent many nights with Alex, practicing the sounds of letters, and making sure he could distinguish and pronounce each of them correctly.

What I do remember is initially feeling left out as a little kid. At first, all of my parents’ time was occupied by Alex. At 15 years old, this is understandable to me now, but as a little kid it wasn’t. My parents picked up on my feelings and began to make sure I wasn’t left out. They did their best to make sure I was involved and helping Alex. They taught me how to practice sounds with Alex, how to change his hearing aid batteries, and most importantly, how to be there for him when he needed me most.

Today, whenever I think of my brother Alex, I rarely think about his hearing loss. I almost always think of him as just Alex—not Alex with hearing loss or anything like that. I’ve almost always treated him the way any other older brother would treat their younger brother. We roughhouse, tease each other, laugh together (mostly at each other), and most importantly, we care for each other.

Alex has been in my life so much that by now I barely notice his cochlear implant on his right ear, or his hearing aid on his left. To me, they’re just ears, just like Alex is just Alex.

But there are certain times when his hearing loss is very evident to me, like when he takes off his hearing aid and implant and can’t hear my mom call him for dinner or answer a question I might ask him.  

These moments by now are part of our daily routine. They’re small and I don’t think about them often, but when they happen, they remind me how lucky I am. How I’m able to hear our mom call us for dinner without devices. How I can tell my dad I love him back when he says it, without taking the time to put on a hearing aid or implant. Thinking about this doesn’t make me pity Alex, it makes me admire him. I admire his strength and I admire how he doesn’t let hearing loss bring him down.

Alex’s hearing loss started out as a struggle, but it wound up bringing my brother and me closer together. I wouldn’t be as close with Alex as I am today if I never helped him overcome the challenges he faced with hearing loss. Challenges are tough and hard to deal with at times, but overcoming those challenges are even harder. If someone can overcome the challenges that life throws at them, then they can do anything.

Joe Mussomeli is an upcoming 10th-grade student who lives in Westport, CT. His younger brother, Alex, has been featured in Hearing Health magazine and is a participant in HHF’s “Faces of Hearing Loss” campaign.

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Age Effects on Speech Recognition

Age-related changes in perceptual organization have received less attention than other potential sources of decline in hearing ability. Perceptual organization is the process by which the auditory system interprets acoustic input from multiple sources to create an auditory scene. In daily life this is essential, because speech communication occurs in environments in which background sounds fluctuate and can mask the intended message.

Perceptual organization includes three interrelated auditory processes: glimpsing, speech segregation, and phonemic restoration. Glimpsing is the process of identifying recognizable fragments of speech and connecting them across gaps to create a coherent stream. Speech segregation refers to the process where the glimpses (speech fragments) are separated from background speech, to focus on a single target when the background includes multiple talkers. Phonemic restoration refers to the process of filling in missing information using prior knowledge of language, conversational context, and acoustic cues.

Kenneth Vaden, Ph.D.

Judy R. Dubno, Ph.D.

A July 2018 study in The Journal of the Acoustical Society of America by William J. Bologna, Au.D., Ph.D., Kenneth I. Vaden, Jr., Ph.D. (2015 ERG), Jayne B. Ahlstrom, M.S., and HHF board of directors member Judy R. Dubno, Ph.D. (1986–88 ERG), investigated these components to determine how their declines may contribute to increased speech recognition difficulty with age. As expected, older adults performed more poorly than younger adults. Older adults were less able to make use of limited speech information and reduced continuity. A competing talker created hearing challenges regardless of age. The study concludes, “Taken together, these results suggest that age-related declines in speech recognition may be partially explained by difficulty grouping short glimpses of speech into a coherent message.” —Elizabeth Crofts 

Hearing Loss Film “Hearing Hope” Captures Personal Strength, Scientific Vision

Hearing Health Foundation (HHF) has created a new short film, “Hearing Hope,” to expand awareness of hearing health through the voices of those who benefit from and those who carry out the foundation’s life-changing work.

"It took me longer to talk than most kids. Because I couldn't understand what they were saying so I couldn't copy it," explains Emmy, 7.

The third most prevalent chronic physical condition in the U.S., hearing loss can affect anyone—from first-grader Emmy to retired U.S. Army Colonel John—but its reach is often underestimated. “It’s one of the most common sensory deficits in humans,” explains cochlear implant surgeon Dr. Anil Lalwani. “I think we have to go from it being hidden to being visible.”

Both a hearing aid user and cochlear implant recipient, seventh-grader Alex is doing his part to make hearing loss less hidden. Smiling, he says he wants people to know that hearing with his devices makes him happy. John wishes to be an advocate for veterans and all who live with hearing loss and tinnitus.

When she received her hearing loss diagnosis at 17, NASA engineer Renee never thought she'd be living her dream.

The film also highlights resilience in response to the challenges associated with hearing conditions. Video participant Renee saw her dream of becoming an astronaut halted at 17 when her hearing loss was detected. Now she helps send people to space as an engineer at NASA.

Sophia describes the “low, low rock bottom” she hit when she was diagnosed with Usher Syndrome, the leading cause of deafblindness. Yet she feels special knowing her disability shapes her and sets her apart.

Jason recounts having no resources for hearing loss in children when his son, Ethan, failed his newborn hearing screening. Today he’s grateful for Ethan’s aptitude for language, made possible through his early hearing loss intervention.

With the support of HHF, more progress is made each year. “I’m glad that the doctors are trying to figure out how fish and birds can restore their hearing,” says Emmy.

For the past 60 years, HHF has funded promising hearing science and in 2011 established the Hearing Restoration Project (HRP), an international consortium dedicated to finding biological cures for hearing loss using fish, bird, and mouse models to replicate the phenomenon of hearing loss reversal in humans.

“If [the HRP] can achieve that goal of hearing restoration...that would be a marvelous thing for hearing loss,” reiterates Dr. Robert Dobie.

Through “Hearing Hope,” HHF would like to share its mission and message of hope to as many individuals as possible and reassure those with hearing loss and their loved ones they are not alone. As an organization that channels all efforts into research and education, HHF would greatly appreciate any assistance or suggestions to increase visibility of the film.

Watch the full film at www.hhf.org/video. Closed captioning is available.

Celebrate Founder’s Week Starting August 1

By Nadine Dehgan

This August marks the 100th birthday of Hearing Health Foundation (HHF)’s late founder, Collette Ramsey Baker. She formed our foundation 60 years ago in gratitude for surgery that restored her own hearing. Her legacy lives on as we continue to fund innovative hearing and balance science. HHF exists to better the lives of the millions who live with hearing and balance disorders, and we would like to acknowledge those who are most important to you.

June 1966: Collette Ramsey Baker (left) is presented with an award at the Rotary Club of New York.

To celebrate the spirit of Mrs. Baker’s birthday, HHF will dedicate a week to your loved ones and those connected to hearing loss. When you make a gift to HHF between August 1 and August 7 in honor of, or in memory of someone special, we will notify them (or their family) of your generosity and add their name to our “Honor Wall” page. As you share your names and stories, we will see the many faces of hearing loss.

Commemorate the most extraordinary people in your life by participating in Founder’s Week and dedicating a gift to hearing research in their honor.

As always, 100% of your gift will be invested in life-changing research and awareness programs, and you can choose where to direct your donation. Thank you for supporting our important work - we couldn’t do it without you!

Make your tribute gift at www.hhf.org/foundersweek beginning August 1, where you will have the opportunity to tell us who you are recognizing and why and to submit their photo.

Understanding a Pressure Relief Valve in the Inner Ear

By Ian Swinburne, Ph.D.

The inner ear senses sound to 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.

This study helps unravel how a valve in the inner ear's endolymphatic sac acts to relieve fluid pressure, one key to understanding disorders affected by pressure abnormalities such as Ménière’s disease.

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 organs control 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.

Here is a time-lapse video of the endolymphatic sac, with the sac labeled “pressure relief valve” at 0:40.

2017 Ménière’s Disease Grants scientist Ian A. Swinburne, Ph.D., is conducting research at Harvard Medical School. He was also a 2013 Emerging Research Grants recipient.

In Memoriam: David J. Lim, M.D.

By Nadine Dehgan

Credit: UCLA Head and Neck Surgery

We recognize with profound sadness the recent passing of David J. Lim, M.D., who was pivotal to the establishment of Hearing Health Foundation (HHF) and remained committed to our research throughout his life.

As a member of our Council of Scientific Trustees (CST)—the governing body of HHF’s Emerging Research Grants (ERG) program—and as a Centurion donor, Lim worked tirelessly to ensure the most promising auditory and vestibular science was championed.

Prior to his appointment to the CST, “Lim contributed to our understanding of the mechanics of hearing through his excellent scanning electron micrographs of the inner ear,” says Elizabeth Keithley, Ph.D, Chair of the Board of HHF. Lim pursued this critical work in 1970 through his first of many ERG grants.

“Lim was also one of the founding members of the Association for Research in Otolaryngology (ARO) and served as the historian of this esteemed scientific organization,” says Judy Dubno, Ph.D., of HHF’s Board of Directors. “Along with HHF, he cared deeply about ARO and will be missed by many.”

Most recently, Lim was a surgeon-scientist and a director of the UCLA Pathogenesis of Ear Diseases Laboratory, where he was considered an authority on temporal bone histopathology, morphology and cell biology of the ear, and the innate immunity of the middle and inner ear.

We, the HHF community, are grateful to have known and to have benefited from Lim’s wisdom, good humor, and kind spirit. HHF will honor his legacy by continuing our mission, knowing we are indebted to his leadership and dedication to advancements in hearing health.

ReSound HearSay: Be The Voice of Hearing

By Tom Woods

"A journey of a thousand miles begins with a single step.” For many individuals who know—or suspect—they have a hearing loss, the first step in their journey to better hearing can prove difficult.

It took more than two years for Francine Murphy of Peoria, Arizona to take action. She says, “I was in denial and I was concerned that it would not help, especially if the sound quality was poor. Start with acknowledging that there may be an issue and start with your family doctor. The best resource I found was my audiologist.”

ReSound hearing aid user Francine Murphy.

Francine is clearly not alone. For many, the delay is due to uncertainty, apprehension, and lots of questions. In the U.S. alone, more than 25 million people who could benefit from hearing aids have yet to take that first step. 

We believe that hearing is fundamental to life. When it starts to decline, it’s imperative that everyone understands, and has access to, the best hearing technology.

That’s why we created ReSound HearSay, an online resource that gives people who are successfully managing their hearing loss an opportunity to lend their voice to educate and inspire others to seek care.

We think that peer-to-peer information sharing is critical in this learning process.

“Get your hearing tested now,” urges John Chynoweth from Orlando, Florida. “Determine exactly what your hearing is like now (get a baseline). Work with a hearing specialist to determine the environments where you struggle to hear. Try different types of hearing aids to find the right ones for you.”

I’m reaching out to readers of this blog to share their hearing journey. Just like Francine and John, you can help those who are just starting to realize hearing loss or considering a hearing aid, and may be hesitant or unsure where to start.

Through posts, you’ll encourage others into action by addressing common concerns and questions, giving them practical advice to help navigate the process, from diagnosis to hearing aids. And you’ll help them understand the important role of the hearing care professional.

Be the “Voice of Hearing” and help others on the path to better hearing. Please take time today to visit ReSoundHearSay.com to share your insights and experience.

Tom Woods is President, ReSound North America.