Hearing Loss Is My Superpower

By Frank Barnes III

Teen angst may be a rite of passage for everyone, but for me it was amplified, if you’ll pardon the pun. Despite receiving a cochlear implant at age 22 months, and being mainstreamed into my local public school district from kindergarten, by late middle school into high school I had became bitter and resentful about my dependence on hearing technology. I saw it as a burden.

Angry and frustrated, I felt self-conscious about the external sound processor on my head, believing it was all that people knew and judged about me. By wearing a hearing assistive device, I thought that I was broadcasting, “I need assistance.” I felt incompetent and incapable, and to show that I didn’t need it, I didn’t wear it to my eighth grade graduation party and kept removing it during freshman year of high school. I felt like not leaning into the hearing loss was a better choice for me.

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This meant I also had no interest in actively seeking out the hearing loss community, and instead passively rejected it, not out of any sense of malice but just because it didn’t feel like somewhere I needed to be. I consistently resisted any nudges from my parents to connect with people with hearing loss who also wore cochlear implants.

It may not surprise you to hear that this only sunk me lower, affecting not only my self-esteem but also what had been my high honors/academic excellence, athletic performance, and social relationships.

Age 22 Months

I have a profound hearing loss in both ears, which resulted from bacterial meningitis. My parents first noticed my hearing loss when I was 18 months old. At age 22 months I received a cochlear implant in my right ear. That it was a mere four-month window between the first signs and diagnosis of my hearing loss until implantation is a remarkable tribute to the commitment and efforts of my parents and the professionals all focused on finding the best solution for me and my family. My surgeon was the late John Niparko, M.D., of Johns Hopkins University, a true leader in the field. At the time in 1997 unilateral implantation was the protocol; I don’t use anything in my left ear.

As a toddler, I attended Summit Speech School in New Providence, New Jersey, to develop my speech, auditory, and social skills, and was able to be mainstreamed into the local public schools starting in kindergarten. I was sent to school with a boxy FM receiver that I had to use throughout the day in order to function with my typical-hearing classmates and teachers.

It’s a big responsibility for a kid, from age 6 and up, already struggling to pay attention to the teacher and socialize with other kids, to also have to change an FM box’s batteries and make sure it doesn’t break or get wet. Over time, I resented having to use it and just stopped in ninth grade—not because I was bullied but because I felt like I didn’t need it.

It was during this period of self-isolation from the hearing loss and cochlear implant community when an invitation for a reunion at Summit arrived. Of course I did not want to attend. But at the prodding of my parents, I did go. And that’s where and when I had my epiphany.

Back at Summit, I found myself surrounded by other alumni—all of us now teens and young adults—who shared their stories of living full lives. Listening to them speak, I saw they were each succeeding in school and prospering in the world—while wearing the same hearing devices that I wore. It was an eye-opening experience, one that planted the seed of-self acceptance that would slowly blossom to change everything for me.

Hearing their experiences, I realized, all of a sudden, that my hearing condition in fact gave me a foundation to achieve any goal and to become successful despite the loss of one of my senses. Everyone in that room reminded me of where I have been, and how we all have similar conditions and struggles. I saw the staff, who to this day are still there teaching and educating and loving and supporting—and the positive effects this has had on all of the alumni. I realized I had distanced myself from the very people, teachers and students alike, who made me who I am today.  

Age 22 Years

During college at Stony Brook University in New York, I did an academic year “abroad”—I was not overseas physically but “traveled” emotionally. I attended California State University, Northridge, and developed friendships with D/deaf students, which helped me gain confidence about wearing my external sound processor in new environments. I met people whose experiences nearly mirrored mine, which made me feel empowered and a part of the community.

I also took classes in D/deaf studies: American Sign Language and the hearing sciences (audiology coursework). When I learned about the anatomical framework of the ear/body relationship, I had a newfound appreciation for the astonishing technological capability of cochlear implants and profound work of audiologists and hearing specialists.

I graduated last spring with a degree in the health sciences, and landed a dream job in the field of immersive technologies, or virtual reality. My company crafts shared VR environments that are visualization and simulation experiences. I wear many hats at the company, working in production, delivery, marketing, and business development, and on project management teams.

The immersive experience is a balance of the visual and the auditory. Given my hearing loss, the visual energy that I get from working in 360° VR media has provided some sensory relief! While the case studies that I've reviewed have not been directly related to disabilities, the company's technology actively transforms emotive storytelling media into greater awareness and empathy, making it a remarkable fit for delivering impactful stories of struggle and triumph.

More than during any other point in my life, I became fully connected to the hearing loss community on the journey toward embracing my hearing loss. Advanced Bionics, the maker of my cochlear implant and Harmony behind-the-ear processor, invited me to share my experiences in New York City and then speak at their headquarters in California. At a Bionic Ear Association meeting in New Jersey, with other CI recipients and their families, I felt grateful to be able to relay words of calm and hope to parents uncertain about post-procedure changes for their child and family.

I have given speeches at, and recently became a trustee of, the Hearing Loss Association of America (HLAA)’s New Jersey chapter, through which I had earned a scholarship in high school. I joined the HLAA’s Walk4Hearing fundraising event and met with legislators in Trenton, New Jersey’s capital, to be present for the Assembly Committee on Education, Science, and Technology because they were voting on Bill 1896, the Deaf Student's Bill of Rights.

The many facets of hearing loss—education, advocacy, empathy, awareness—all have become priorities for me.

Knowing When You Need Support

It’s crazy that I used to think I could succeed without the hearing loss community. They have my back and are forever my tribe. Having a community means you will work together to be your best self for one another. My parents knew how to ask for help, but it took me a little longer to realize the importance of being vulnerable and asking for assistance—and then being able to pay it forward by helping others.

In 5th grade, when I was about 10, I was playing soccer toward the end of the day, with the daylight waning, when the unthinkable happened: I lost my headpiece. No processor, no hearing. I was in the middle of the field, trying to figure out what to do, feeling frustrated and anxious. My soccer coach came over and asked, “What happened?”

I read his lips to know what he was asking, and he explained that the team had to ask more questions to help me find it: “What does it look like? If you don't find it, what will you do? And, how are you?” Which I felt like was a weird question because they wanted to know if I was upset—and I said, yes!

But what they said to me next was, “What can we do to make you feel better?” I really appreciated that they didn’t say “you should” or “you’d better”—they weren't saying hearing loss was a barrier. It was more that they were saying how can we make you feel better, as a person.

We stood in a long line horizontally to cover the field, and slowly stepped across the dirt together, scanning the ground. Amazingly, we found it! I had told myself that if I didn't find it, it would be okay. But everyone else said, “No, we will find this! This is a part of who you are, this is what you need, we’re here for you.” They were saying to me that this is a part of your identity and let's make sure you go home, whole. The team came together for me.

In Turn, Helping Others

Something I had not paid any attention to as a kid is the behind-the-scenes work, the village of hearing healthcare professionals and other experts who work together to create the churning machine that says, “Here are the resources, here are the services so that these kids can succeed.”

I can’t overstate the value of Facebook groups that keep me connected to the community of cochlear implant users, people who have upcoming surgeries or family or friends who do. Get connected through online communities, word of mouth, or introductions by your audiologist or CI manufacturer.

True, I say this like it’s super-easy to send a message to a complete stranger! But I am highly confident that any person with a new diagnosis of hearing loss while find an outpouring of support and friendship from these connections, and will be uplifted and less unnerved after speaking, meeting, and bonding with the vibrant, full-of-life people across the hearing loss spectrum.

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I thoroughly enjoy being a working professional, advocate, and public speaker. In speeches I’ve made to the hearing loss community, I am candid about how I had not realized the value of their community, how I did not want to accept being different. Often people react with surprise or even dismay, but I usually see a few people who are nodding in agreement that we need to share how there are daily struggles and doubts, and that not everything is easy or cheery all the time.

Our journeys must take a measure of self-acceptance about being different, whether we use a cochlear implant, hearing aid, or sign language. Being different from the hearing society is the best thing that I've ever experienced. From the vantage point of my advanced age of 22, I can now see that in your tweens and teens you want to just be like everyone else. It is not until later that you realize that being different, that standing out, is in fact great! My hearing loss is my superpower.

This article originally appeared in the Spring 2019 issue of Hearing Health magazine. Frank is also a participant in HHF’s Faces of Hearing Loss project.

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Veterans Sue Over Defective Hearing Protection

By Joseph Oot

Veterans nationwide are filing lawsuits against the military equipment manufacturer 3M, after a July 2018 verdict concluded the company’s dual-ended Combat Arms Earplugs Version 2 (CAEv2) were defective. The verdict in this whistleblower lawsuit, filed by Moldex-Metric on behalf of the U.S. government, paved the way for service members seeking legal restitution.  

This case began three years ago in May 2016 when Moldex-Metric, a California-based company in the military equipment industry, brought charges against their competitor, 3M. The plaintiff claimed that the original manufacturer of the CAEv2 devices, Aearo Technologies which was purchased by 3M in 2008, colluded to manipulate product tests and falsify data in order to achieve government standards and sales. Moldex-Metric was able to present evidence that both Aearo and 3M continued to sell the defective devices for more than 10 years, even though the devices were found to be too short, a defect that made the equipment difficult to properly insert in the ear. As a result, the devices were loose fitting, prone to fall out, and inadequately provided the level of protection claimed by the manufacturer.

After years of litigation, 3M agreed to settle the allegations in July 2018. 3M was ordered to pay the U.S. government $9.1 million in damages—but none of these damages compensated CAEv2 users, and 3M said this settlement was not an admission of liability. However, the verdict against 3M likely sparked the flood of class-action lawsuits filed since then.

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More than 300 lawsuits have been filed by service members seeking restitution. Retired U.S. Marine Capt. Matt Morrison of New Jersey is one such service member who in February 2019 filed his case against 3M. He says the CAEv2 devices were the direct cause of the complete hearing loss he has sustained in his right ear. While deployed between 2007 and 2013, including two tours of Iraq and one of Afghanistan, he was frequently exposed to loud equipment, machinery, gunfire, and explosions.

Along with thousands of other service members, Morrison says he came to rely on the standard-issue hearing protection as much as a bulletproof vest. "The gear you're issued is everything from a helmet to a flak jacket, eye and ear protection. I never thought that, after the fact, the gear would be faulty or defective and cause this kind of injury," Morrison told a local news reporter.

Like Morrison, active duty military members are exposed to machinery, aircraft, and sudden weaponry blasts leaving their ears susceptible to noises as loud as 184 decibels (dBA). Sounds at or above 110 dBA can cause permanent hearing loss and tinnitus instantaneously without hearing protection. The U.S. Department of Veterans Affairs reports tinnitus and hearing loss are the most common disabilities among veteran service members, with 60 percent of those who served in Iraq and Afghanistan living with one or both of the conditions today.

Both the military and hearing loss communities take seriously all reports of defective hearing protection, especially given the prevalence and permanence of hearing loss and tinnitus among veterans. Without a commitment to strict product performance, user testing, and data verification standards, service members will remain at risk.

Joseph Oot is a writer with ConsumerSafety.org, an organization connecting individuals with information on developing lawsuits, court cases, and recent news affecting consumers. As a consumer advocate, Oot works with both individuals and industry professionals to share helpful information surrounding potentially harmful products.

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Stability in an Unstable World

By Timothy S. Balmer, Ph.D., and Laurence O Trussell, Ph.D.

Balmer & Trussell traced the direct and indirect pathways that carry vestibular information to the cerebellum for controlling balance and posture. Shown here is a primary afferent axon (green) expressing the light-gated ion channel, Channelrhodopsin. Postsynaptic cells, in this case a unipolar brush cell (magenta), were recorded from during stimulation of the input axons by light flashes. This technique was used to discover how direct and indirect vestibular pathways are processed in the cerebellum.

Balmer & Trussell traced the direct and indirect pathways that carry vestibular information to the cerebellum for controlling balance and posture. Shown here is a primary afferent axon (green) expressing the light-gated ion channel, Channelrhodopsin. Postsynaptic cells, in this case a unipolar brush cell (magenta), were recorded from during stimulation of the input axons by light flashes. This technique was used to discover how direct and indirect vestibular pathways are processed in the cerebellum.

Mice are helping scientists to understand how the world around us remains looking stable even as we move.

While out jogging, you have no trouble keeping your eyes fixed on objects in the distance even though your head and eyes are moving with every step. Humans owe this stability of the visual world partly to a region of the brain called the vestibular cerebellum. From its position underneath the rest of the brain, the vestibular cerebellum detects head motion and then triggers compensatory movements to stabilize the head, body and eyes.

The vestibular cerebellum receives sensory input from the body via direct and indirect routes. The direct input comes from five structures within the inner ear, each of which detects movement of the head in one particular direction. The indirect input travels to the cerebellum via the brainstem, which connects the brain with the spinal cord. The indirect input contains information on head movements in multiple directions combined with input from other senses such as vision.

Balmer & Trussell traced the direct and indirect pathways that carry vestibular information to the cerebellum for controlling balance and posture. Direct projections from the vestibular inner ear (green) and indirect projections from the brainstem (magenta) were shown to target different populations of neurons in the cerebellum.

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By studying the mouse brain, Balmer and Trussell have now mapped the direct and indirect circuits that carry sensory information to the vestibular cerebellum. Both types of input activate cells within the vestibular cerebellum called unipolar brush cells (UBCs). There are two types of UBCs: ON and OFF. Direct sensory input from the inner ear activates only ON UBCs. These cells respond to the arrival of sensory input by increasing their activity. Indirect input from the brainstem activates both ON UBCs and OFF UBCs. The latter respond to the input by decreasing their activity.

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The vestibular cerebellum thus processes direct and indirect inputs via segregated pathways containing different types of UBCs. The next step in understanding how the cerebellum maintains a stable visual world is to identify the circuitry beyond the UBCs. Understanding these circuits will ultimately provide insights into balance disorders, such as vertigo.

A 2017 Emerging Research Grants (ERG) scientist who received the Les Paul Foundation Award for Tinnitus Research, Timothy Balmer, Ph.D., is a postdoctoral fellow at the Oregon Hearing Research Center at Oregon Health & Science University (OHSU). Laurence Trussell, Ph.D., a 1991 ERG recipient, is a professor of otolaryngology–head and neck surgery at OHSU.

This research summary was repurposed with permission from eLife with permission.

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Hair Cell Eulogy

By Dawn Doig

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People don’t seem to understand the damage they’ll incur
The way that birds’ songs, music, voices, all become a blur -
When the ears are not protected from loud music, shotgun blasts;
The birdsong they heard yesterday may have been their last...

The problem is maybe ignorance, lack of info about the ear
And how its “innards” function when it’s enabling us to hear.
The inner ear is lined with hair cells that when healthy will respond
To incoming sound energy from the ear canal and beyond.

Some hair cells dance to drum beats, some dance to jingling bells,
But regardless of the dance they do, they know their dances well.
The mighty “Endo” (Endolymph) taught them all from the very start
To learn the dance for hearing’s sake; to learn it all by heart.

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Endolymph is a fluid that courses through the inner ear
And when Endo starts to move too fast, the hair cells cringe with fear -
Fear of death impending if Endo moves too fast
For hair cells fall like blades of grass as Endo rages past.

What causes Endo’s rages? - for a warning’s never there
And the hair cells least expecting are the ones who never fare.
Amidst a dance of merriment, the rage takes them by surprise
And one by one the hair cells die before the others’ eyes.

One day a shotgun blast went off and downed a startled moose
Another day another blast downed an unsuspecting goose.
Both moments enraged Endo, who pounded in his fury
And lunged upon some hair cells who would otherwise be merry.

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The dance to birds’ songs dwindled first as yet another hair cell died;
The remainder knew their fate too well and curled up and cried -
They wept for all their comrades who had lost their lives in vain,
If only muffs or earplugs were worn, they would not have such pain.

They had to strain for spoken words, some still tried to dance,
But numbers now as they were, the hair cells didn’t have a chance.
How hard they tried to make new sounds as loud and clear as before,
But with their numbers now so low, they couldn’t do it anymore.

Farm machinery, music blaring at volumes much too high,
Snowmobiling, off-roading - they all make hair cells die.
Why risk the chance of losing a hair cell dance or two?
Wear ear protection and smother Endo’s rage -

THE DECISION’S UP TO YOU!!

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Dawn Diog is a clinical audiologist. Originally from Victoria, British Columbia, Canada,, she resides in Cameroon, West Africa where she is the English Language Learners (ELL) coordinator for the American School of Yaounde.

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Doing My Best

By India Mattia

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My hearing loss appeared suddenly, at age 61, when I woke up for work unable to hear in my right ear. With no history of difficulty hearing, I was completely stunned. Did I, I wondered, damage my ear with loud music in my headset yesterday?

In a panic, I requested an immediate appointment with my primary care doctor. He prescribed antibiotics and recommended I see an ear, nose, and throat doctor (ENT, or otolaryngologist) if my hearing did not return.

The antibiotics did not help my hearing, and upon the visit to the ENT I learned I had become completely deaf in my right ear and had developed a small hearing loss in my left ear. She suggested injecting steroids into my right ear to restore my hearing. I was apprehensive about having a needle stuck in my ear, but the doctor assured me that the novocaine would numb any pain. I agreed, and the procedure made me feel dizzy.  

One week and two injections later, my hearing had not come back. The ENT could not identify the cause with certainty. She thought it might be an infection. But I couldn’t think of ways I would have gotten infected, and to me I didn’t see any obvious signs of an infection.

Soon my hearing loss was accompanied by vertigo, tinnitus, and ear pain. Every time I moved a certain way, I felt my head spin. I relied on the keep the radio to block out my tinnitus and took Tylenol to dull the pain. My ENT referred me to a specialist at John Hopkins Hospital, but the first appointment I could secure was two months away, in August.

Meanwhile, I began to adapt to the challenges of hearing loss in my daily life. No longer able to hear my alarm clock to wake up for work, I switched to a vibrating pillow alarm. I watched TV with Bluetooth headphones to avoid disturbing my family with the high volume I needed to hear. I couldn’t manage noisy spaces, though. Restaurants, outdoor events, and loud traffic were unbearable to me.

I began to feel embarrassed and ashamed of having to ask people to speak louder or repeat themselves. I was afraid to tell my coworkers at the real estate office where we worked, but confided in my supervisor, who was sympathetic. Outside of work, my husband often advocated for me by telling others about my hearing loss.

When I thought circumstances couldn’t get worse, I learned my office was to close in July, leaving me without a job. I hoped to make ends meet with the severance pay and unemployment compensation while looking for work.

The John Hopkins doctor confirmed an infection had caused permanent sensorineural hearing loss in my right ear. He recommended a hearing aid, but I cannot afford one.

I remain without a job and my unemployment compensation has ended. I have had a few job interviews—but have kept my hearing loss a secret—and have done my best understand the interviewers’ spoken questions. Aside from my hearing loss, age discrimination makes job hunting difficult. I don’t qualify for social security disability benefits because my hearing loss is only in one ear.

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All this said, my hearing loss has led me to make some positive lifestyle changes. I read more often than before and have swapped rock and roll for soft music. I’ve reduced my sodium intake and eliminated caffeine which is supposed to improve blood flow to the ear. I ride an exercise bike and have taken up t’ai chi to stay physically active.

I applied for early social security retirement since I will be 62 in June. I am also applying for social security disability for both my hearing loss and asthma, which I have had since my late 20s. I am hopeful something will come through. My story may not have an inspiring ending, but I’m doing my best and looking toward better days.

India Mattia lives in Maryland.

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Prevent the Preventable

By Neyeah Watson

International Noise Awareness Day (INAD) is observed annually on April 24 to redirect our focus to the invisible: hearing loss caused by noise, or noise-induced hearing loss (NIHL). More than 48 million Americans live with a form of hearing loss, and one third of all hearing loss cases can be attributed to noise exposure. In honor of INAD, sponsored by the Center for Hearing and Communication, Hearing Health Foundation (HHF) urges the public to zealously protect the hearing of adults and children from the dangers of loud noise.

Loud noise can harm from exposure to one sudden, disturbing, blast-like sound (an impulse noise) or a series of loud sounds over time. Impulse noises include fireworks, explosions, car horns, gunfire, and thunder. These noises reach the outer ears unexpectedly before conversion to sound, sometimes causing immediate trauma and NIHL.

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Gradual NIHL, on the other hand, occurs over time. Sources include loud music and occupational sounds from construction sites or airport runways. Gradual NIHL can also result from hobbies, like playing in a band, attending group fitness classes, and snowmobile riding.

The irreversible damage of NIHL physically takes place when hair cells within the cochlea of the inner ear are damaged. This results in what’s called sensorineural (permanent) hearing loss.

Fortunately, NIHL is 100% preventable. For adults, protecting your ears doesn’t require a lot of effort. When going about your busy lifestyle, keep these simple techniques in mind.

  • Always keep a pair of earplugs with you. Whether you ride public transportation as a passenger or employee, loud noises accompany you on your commute.

  • Purchase noise-cancelling headphones, which are safer for your ears because they prevent the user from needing to raise the volume to block out external sounds.

  • Listen to music and television at medium volumes.

  • Most importantly—give your ears time to recover. Quiet time is one of the best healing experience you can give to your ears.

Protecting your children’s hearing is easy, and begins in the home. Here’s what you can do:

  • Practice the 60/60 rule: Only listen to music at 60% of the volume for 60 minutes.

  • Inspire their preference for quiet toys such as puzzles, dolls, coloring books, and building blocks. When considering electronic toys, look for for products with volume control settings.

  • Set the TV or game consoles to reasonable volumes.

  • Make sure your child does not sit too close to the television or other noisy electronics.

  • Use plush furnishings such as curtains, carpeting, and pillows to allow for sound to be absorbed.

  • Always set an example for your children. Your use of earplugs and low volumes will allow your children to learn a behavior that will become part of their hearing lifestyle.

Life’s enjoyments don’t necessarily need to be limited by diminished hearing. We all have the power to protect our ears.



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A Woman's Canine Ears

By Barbara M.      

I always knew I wanted to work with people after high school. Though I struggled throughout my education, I graduated, miraculously, thanks to my supportive family and hearing aid technology. With a high school degree I was eligible for Vocational Nurses (VN) school, a career I maintained for over 23 years.

My sensorineural hearing loss was diagnosed at around age 4, well before newborn hearing screenings were commonplace, in 1954. “Barbie needs to see your face when you talk, Mommy,” my sister announced one day, cluing my parents into a possible hearing problem.

Barbara and her most recent hearing dog (HD), Fallon.

Barbara and her most recent hearing dog (HD), Fallon.

I wore behind-the-ear hearing aids and equally relied upon speech-reading, but academics were a constant challenge. Math was the most difficult for me because my teachers often faced the chalkboards, not us students, while writing and explaining equations. My family spent many hours tutoring math to me to ensure I passed.

After high school, I was employed as a nurse’s aide in a long-term care facility. I enjoyed working with and helping the elderly patients, which encouraged me to apply to VN training school. I entered with strong professional references and my two instructors were aware of my hearing loss, so I felt secure.

During the last week of the six-week academic training, which immediately precedes clinical duty training, one of the VN instructors requested a private meeting. She had concerns about my hearing loss. “What if you’re in a situation where you could not hear a patient call/cry out?” she pressed.

Devastated, I reasoned with her hoping she would allow me to begin clinical duty on a trial basis to prove my ability to meet their expectations. I suggested this even though I knew in my heart it would personally difficult to withstand 17 additional months of required training under her surveillance. She pushed back, expressing I could be jeopardizing a patient's life or she could lose her job. I was crushed! I ultimately chose to withdraw from this VN school, knowing I’d be accepted to another school. I was—and graduated to go on to pass the California State Board for licensure.

Some time after my licensure, I was driving past my first VN school and a thought occurred to me; I needed to share my good news with my once doubtful instructor. The instructor with whom I spoke remembered me and congratulated me on my achievement. We chatted amicably before she revealed that the 'other' instructor had since resigned—due to hearing loss she experienced the year after I withdrew from the VN program. I was in disbelief! I hoped she, too, had not faced discrimination. Empathetically, I expressed my concern that no one should be discriminated against because of their disability.

About halfway into my nursing career, in the 1980s, I found myself with the need to to hear more and relied more on speech-reading. I often felt tired and mentally overloaded in noisy environments. One of my biggest challenges during this time was caring for my son, born in 1987. My husband, a firefighter who often worked 48-to-72-hour shifts away from home, cleverly devised a sound-activated light system to alert me when our son made noise or cried in his crib. This was a perfect solution, I thought—except in the case of power outages.

I needed to find another way to alert me to my son's voice at night. It came to me the following year while reading National Geographic, where I saw an advertisement about Canine Companions for Independence (CCI), a nonprofit organization that enhances the lives of people with disabilities by providing them with service dogs and ongoing support. I applied and was paired up with Ronnie, my first Hearing Dog (HD), who helped me immensely by alerting me to important sounds within my home.

Gradually and for unknown reasons my hearing loss continued to decline and I found myself avoiding noisy establishments & places. In 1999 I made the decision to resign from employment so as to ensure quality patient care I no longer was able to auscultate (hear) important lung sounds using the stethoscope. With my skills as a nurse, I chose to transition to be a care provider for my aging parents-in-law, and later, my mother.

I am grateful I was able to remain productive after my resignation, largely because of the help of Ronnie and, later, his successor, Fallon, a Golden Retriever/Labrador mix. Fallon, who passed away in 2014, alerted me to ringing doorbells, whistling tea kettles, and ringing telephones. Though I’m no longer helping people, I’m so happy that Fallon was able to help me.

Ronnie and Fallon helped me to overcome my hidden handicap, a personal milestone towards acceptance of my hearing loss.

Barbara lives in Oregon with her husband, Dan.

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Improved TMC1 Gene Therapy Restores Hearing and Balance in Mice

By Christopher Geissler, Ph.D.

Half of all inner ear disorders, which have a negative impact on hearing and/or balance, are caused by genetic mutations. A study published in January 2019 in Nature Communications demonstrates the effectiveness of a gene therapy targeting one specific gene mutation, TMC1 (transmembrane channel-like 1). The research was conducted by Carl A. Nist-Lund in the Harvard Medical School lab of Gwenaëlle S. Géléoc, Ph.D., and Jeffrey R. Holt, Ph.D., with contributions from colleagues including 2017 Emerging Research Grants (ERG) recipient Jennifer Resnik, Ph.D., and her ERG co-principal investigator Daniel B. Polley, Ph.D., both also of Harvard Medical School.

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So far, 35 TMC1 mutations have been identified in humans, including several that are responsible for moderate to severe hearing loss, representing between 3 to 8 percent of cases of genetic hearing loss. This TMC1 gene therapy has had an encouraging level of success in mice and may prove capable of addressing similar genetic mutations in humans in the future.

Previous studies targeting this gene were only moderately successful in restoring function in inner hair cells, with little or no success in outer hair cells. Both types of hair cell are necessary for hearing.

The team decided to look at improving the mechanism that encodes TCM1 in affected mice, using a synthetic delivery vehicle they hoped would be more effective than the conventional one used in previous studies. In mice with this TCM1 mutation, hair cells begin to die when the mouse reaches 4 weeks of age. The treated mice in this study showed improved rates of survival in both inner and outer hair cells.

Most importantly, the improvement in hearing in the mice that received this intervention occurred primarily in the lower frequencies. Human speech is at the low to mid frequency range of the auditory spectrum, so if future human trials are able to replicate the success of this study, speech perception may improve.

The study additionally provided evidence of improved responses in the brain of the treated mice. This indicates that treatment of the cochlea by injection had knock-on effects in the auditory cortex, the part of the brain that plays an important role in hearing.

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Finally, the team recorded improved balance function in the mice that received the gene therapy. While only very young mice experienced better hearing, even older mice showed improvement in balance. The team writes that this improvement in balance function in mature mice may contribute to eventually developing a way to treat balance disorders in humans.

Jennifer Resnik, Ph.D., is a postdoctoral fellow in the Polley Lab, part of the Eaton Peabody Laboratories, Massachusetts Eye and Ear/Harvard Medical School. Her 2017 Emerging Research Grant was generously funded by Hyperacusis Research Ltd. Christopher Geissler, Ph.D., is HHF’s director of program and research support.

Empower groundbreaking research toward better treatments and cures for hearing loss and tinnitus. If you are able, please make a contribution today.

 
 
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HHF Endorses California Pediatric Hearing Aids Bill

A young girl speaks at the podium during Assemblyman Bloom’s press conference to introduce his bill to require insurance companies to cover pediatric hearing aids. Credit: Richard Bloom

A young girl speaks at the podium during Assemblyman Bloom’s press conference to introduce his bill to require insurance companies to cover pediatric hearing aids. Credit: Richard Bloom

Hearing Health Foundation (HHF) has formally endorsed AB 598, a bill in California calling for the expansion of hearing aid insurance coverage for children.

California resident Elizabeth Keithley, Ph.D., Chair of HHF’s Board of Directors, recently wrote a letter of support to Assemblywoman Lorena Sanchez, who has stopped earlier versions of the bill. You can read her letter below.

If you live in California and would like to identify and contact your representative about AB 598, you may do so here.


Letter of Support from Elizabeth Keithley, Ph.D.

Dear Assemblywoman Lorena Gonzalez,

Thank you for the leadership you provide to San Diego. I write to you as the Chair of the Board of Hearing Health Foundation (HHF) and Professor Emerita of Surgery/Otolaryngology Head and Neck Surgery at the University of California, San Diego, School of Medicine. I am requesting your support for AB 598, introduced by Assemblyman Richard Bloom, which will require insurance coverage for children’s hearing aids in our state.

HHF is the nation’s largest nonprofit funder of hearing and balance research. Our scientists’ work has led to development of cochlear implants, innovative ear treatments, and progress toward biological cures for hearing loss. We work tirelessly to better the lives of the 50 million Americans with hearing loss.

Beyond research, HHF has been a longtime advocate for Universal Newborn Hearing Screenings, federally mandated in the 1990s. Identifying hearing loss at birth enables parents to promptly pursue intervention for their child. The first six months of a child’s life are the most critical in forming auditory pathways in the brain to hear.

The majority of individuals with sensorineural (permanent) hearing loss, including children, can benefit from hearing aids as treatment to communicate, learn, and develop healthily. A pair of pediatric hearing aids can burden a family by as much as $6000 per pair, which generally must be replaced every three to five years. This is an out-of-pocket expense of over $40,000 before a child reaches 21.

This immense financial barrier to treatment result in absence of treatment that then inhibits children’s social, speech and language development, and academic performance. For an individual child who does not receive intervention, the estimated cost of special education and loss of productivity is $1M.

Right now, California urgently needs the help of leaders like you to relieve families from the stress of choosing between hearing aids for their children and other health necessities. The strength of the future CA workforce depends on it.

Thank you for your consideration. I truly hope you will act to support California’s children through AB 598.

Sincerely,

Elizabeth M. Keithley, Ph.D.

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The Blast

By Jane Prawda

It was a crisp fall day when I was confronted by a catastrophic blast that changed my mental health, and life, forever. The violent noise caused permanent ear damage―and finding the right treatment has been a constant battle.

Auditory experts agree an untreated hearing condition can cause psychiatric disorders like depression and anxiety. At the time of the noise trauma, I had already been living with depression for decades, since age 17, making my particular circumstances quite difficult and emotionally devastating.

On that day I will never forget, I was alone on the sidewalk of Manhattan’s Upper East Side walking home. I had no warning when, suddenly, I heard a tremendous explosion at one of the notoriously noisy construction sites on Second Avenue. At the time, New York City was enlarging the “Q” subway line by more than 30 blocks, a project that left us residents subject to years of dangerous noise.

Construction site in Manhattan

Construction site in Manhattan

Without a place to turn for help following the blast, I continued walking home. By evening, in the silence of my apartment, I could hear a faint twinkling sound in my ears: tinnitus.

It wasn’t surprising that the tinnitus quickly worsened my mental wellbeing. Frightened by the ringing in my ears, I phoned my psychiatrist. He prescribed an anti-anxiety medication.

The tinnitus soon went away, but then months later it returned. Was I experiencing lingering effects of the blast, or was it the medication provoking these disturbing sounds? With their latest re-emergence, the sounds had become louder. I was scared and felt empty inside.

I went from clinician to clinician trying all sorts of new remedies, including lipoflavinoids, neurofeedback, acupuncture, and tinnitus retraining therapy, and found no relief. The constant ringing brought me to the verge of suicide―prevented by my younger brother. He understood my agony, and I am grateful for his empathy.

In 2014 I began an experimental treatment called transcranial magnetic stimulation (TMS), which uses highly-focused pulsed magnetic waves to stimulate nerve cells in the area of the brain that is thought to control mood. With the first treatment, the objective was to relieve tinnitus. Subsequent treatments were to relieve depression. Unfortunately, the one instance of the procedure was performed incorrectly, which not only worsened my hearing 30 dB, a mild hearing loss, but made the ringing present 24/7. It also caused transitory hyperacusis which, thankfully, I no longer live with today.

I was warned before the procedure that there was a slight chance I would lose hearing, but not that my tinnitus would become more persistent. With all symptoms worsened, I felt I’d arrived at another dead end and remained desperate for a solution.

Following the TMS treatments, I developed neuroplasticity, the brain’s formation of neural connections to adjust to injury. My audiologist believes neuroplasticity is what caused the hyperacusis to disappear and the tinnitus to subside considerably.

The tinnitus has come and gone according to my stress levels, at times even completely disappearing. With the help of my psychiatrist, I no longer struggle with depression. I’ve come to accept that a cure does not yet exist for tinnitus, so I cope in the best ways I can. Listening to the sounds of birds in the early morning, ocean waves, and babbling brooks using Resound Relief iPhone app always brought me great comfort.

Jane Prawda Headshot.png

I’ve also adapted to my mild hearing loss. I inform people I meet that I have a hearing loss and to face me when they speak; that works for me.

Through all the trauma I consider myself to be a survivor, as I am the daughter of a Holocaust survivor. It is there I draw my strengths.

Jane Prawda MA, OTR, MS/Ed has been published for her expertise in occupational therapy, including Surviving 9/11: Impact and Experiences of Occupational Therapy Practitioners. She lives in New York City.

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