Sharper Than They Expected: A Tribute to Nansie Sharpless, Ph.D.

By Neyeah Watson

Women and people with disabilities have been historically underrepresented in science. In 2014, individuals with hearing loss compromised only 1.2% of Scientific & Engineering degree recipients. Women represented only 29% of the science, technology, engineering, and mathematics (STEM) workforce as of the same year.

While these statistics remain low, women and people with disabilities have seen overwhelming growth in opportunity in the past 50 years, which we can attribute changes in policy, personal attitudes, and the success of some exceptional individuals. One such pioneering individual was Nansie Sharpless, Ph.D., a biochemist who lived with bilateral hearing loss. HHF has chosen to highlight her victories this Women’s History Month. Her motivation to push barriers strengthened the confidence society has in women in science today.

Sharpless was born in Pennsylvania in 1932. Though this year saw breakthroughs for women, such as the election of the first female Senator, Hattie Wyatt Caraway, and Amelia Earhart becoming the first woman to fly solo across the Atlantic Ocean, limitations remained. Women’s suffrage had just become a law 12 years prior, and the Equal Rights Amendment, which mandated gender equality and prohibits discrimination on the basis of sex, did not yet exist. During childhood, Sharpless gravitated toward science, mathematics, and education. Tragically, at 14 Sharpless contracted meningitis, a disease that was still considered fatal at this time. Sharpless recovered but was left with a profound hearing loss in both ears.

Credit: American Association for the Advancement of Science

Credit: American Association for the Advancement of Science

Sharpless’ hearing loss did not halt her academic goals despite the lack of accommodations for students with disabilities at this time. The Education of All Handicapped Children's Act—which mandated schools to provide students with learning, mental and/or physical disabilities equal access to education and to protect them from harassment and discrimination—was not passed until 1975.

Still, Sharpless defeated the odds. In 1960, only 35% of the total bachelor’s degrees achieved were women. In comparison to today, over 57.34% of total bachelor’s degrees are women. In 1954 Sharpless earned a bachelor’s degree in zoology from Oberlin College. Although Sharpless thrived in school academically, she struggled to listen in the classroom. She was fortunate to have classmates who assisted with her notetaking.

Sharpless faced similar communicative challenges in her pursuit of a master’s degree in medical technology at Wayne State University. She attended meetings, learned innovative techniques, and developed strategies to alleviate her learning obstacles.

At Wayne, Sharpless’ strong educational performance was unprecedented—and unsupported. Administrators were discouraging; in fact, the director of admissions for Wayne State University warned Sharpless she’d face unusual scrutiny in which she would be expected to achieve a perfect grade point average in order to prove that she could handle the work. Sharpless was undeterred and went on to receive her doctorate from Wayne as well.

After graduation, Sharpless was hired to conduct biochemistry research at Mayo Clinic, where she focused on the correlations between chemicals and mental disorders. Some of her most notable work includes the studying of the L-dopa metabolism in spinal fluid and its relationship with Parkinson’s Disease. Her research showcased the patterns of dystonia, a movement disorder, in response to L-dopa therapy for Parkinson's disease.

Sharpless was also fortunate to defy expectations by joining the Albert Einstein Medical College faculty as an associate professor. She was later promoted to the position of Chief of the Albert Einstein Medical College’s Neuropsychopharmacological laboratory.

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Sharpless passed at the age of 55 while still serving in the Chief position at the Albert Einstein Medical College. In her career, Sharpless published over 50 papers and penned 11 books. Through her triumphs, Sharpless illustrated that hearing loss did not limit her. She was able to utilize her passion for science to become a dominant voice within research and advancement for women and for individuals with disabilities.

March celebrates the journeys of resilient women around the world. Gender and disabilities have presented challenges to individuals, especially before protective laws were in place. Sharpless fought to be outside of the statistic, seeking her purpose within what she loved most—science.

HHF intern Neyeah Watson studies communications at Brooklyn College.

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Hearing Restoration Project Scientific Director to Lead University’s Research Enterprise

By Tamara Hargens-Bradley, OHSU News

OHSU/Kristyna Wentz-Graff

OHSU/Kristyna Wentz-Graff

Peter Barr-Gillespie, Ph.D., will be Oregon Health & Science University’s (OHSU) first chief research officer and executive vice president, effective Jan. 1, 2019. Barr-Gillespie has served as interim senior vice president for research at OHSU since 2017.

In his new role, Barr-Gillespie will be principal adviser to OHSU President Danny Jacobs, M.D., FACS, on research strategy and research resource allocation. He will lead and manage OHSU’s research enterprise—comprising dozens of internationally and nationally acclaimed basic, translational, clinical, and public health research programs—and serve on the president’s executive leadership team.

“Dr. Barr-Gillespie has done a tremendous job leading the OHSU research mission on an interim basis. I’m delighted to appoint him to a new, permanent position that reflects his contributions and capabilities as well as the vital role of research at OHSU,” Jacobs says.

Barr-Gillespie also will collaborate with external academic, industrial and community research partners, and the various funding, regulatory and accrediting bodies. Moreover, he will represent OHSU in research collaborations with other universities in Oregon and the northwest region.

“I am excited to support Dr. Jacobs in developing OHSU’s 2025 strategic plan for research,” Barr-Gillespie says. “To be among the top-ranked research universities for NIH funding in the country and maintain our national reputation for cutting-edge research, we need to empower our researchers to do their best science by smartly investing in people, core resources, and space, and enhancing our graduate programs.”

OHSU/Kristyna Wentz-Graff

OHSU/Kristyna Wentz-Graff

Barr-Gillespie is an internationally recognized scholar, biomedical researcher and visionary academic leader who has been on faculty at OHSU since 1999. He currently holds faculty appointments in the departments of Otolaryngology/Head and Neck Surgery, Biochemistry and Molecular Biology, and Cell and Developmental Biology in OHSU’s School of Medicine and Oregon Hearing Research Center. He also is a senior scientist in the OHSU Vollum Institute.

An NIH-funded investigator, Barr-Gillespie’s research focus, his passion, is understanding the molecular mechanisms that enable our sense of hearing. Specifically, the Barr-Gillespie lab endeavors to determine how sensory cells in the inner ear called hair cells allow humans to perceive sound. Barr-Gillespie will maintain his active research program while serving as chief research officer.

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Barr-Gillespie is also the scientific director of the Hearing Restoration Project (HRP), an international consortium of 14 investigators funded by Hearing Health Foundation. The HRP’s goal is to develop a biological therapy for hearing loss arising from destruction of hair cells, which are not regenerated after damage from noise, ototoxic drugs, or aging.

Barr-Gillespie earned his bachelor’s degree in chemistry from Reed College in 1981, carrying out his senior undergraduate thesis at OHSU after a summer fellowship in OHSU’s biochemistry department. He received his doctorate in pharmacology at the University of Washington in 1988, and completed a postdoctoral fellowship in physiology, cell biology and neuroscience with Jim Hudspeth, M.D., Ph.D., at the University of California San Francisco and the University of Texas Southwestern Medical Center in 1993.

Following his fellowship, he accepted a faculty position in physiology at Johns Hopkins and remained there until accepting the position of scientist at the OHSU Vollum Institute and associate professor of otolaryngology/head and neck surgery in the OHSU School of Medicine in 1999. In 2014, Barr-Gillespie was appointed associate vice president for basic research at OHSU.

As a young investigator, Barr-Gillespie was named a Pew Scholar in Biomedical Sciences, a program that funds research “that shows outstanding promise in science relevant to the advancement of human health.” During his tenure at OHSU, he has been honored with the Faculty Excellence in Education Award and the John A. Resko Faculty Research Achievement and Mentoring Award.

Over his distinguished career, he has published more than 115 scholarly articles, chapters, and reviews, and has been an invited lecturer at dozens of research universities, academic conferences, and scientific events.

Barr-Gillespie and his wife, Ann Barr-Gillespie, D.P.T., Ph.D., live in Portland. She is the vice provost and executive dean of the College of Health Professions at the Pacific University Hillsboro campus. Their children are Aidan Gillespie, 17, and Katie Gillespie, 24, whom Peter and Ann share with their mother, Susan Gillespie. In their spare time, Peter and Ann enjoy cycling and hiking.

This is republished with permission from OHSU News.

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Comfortable

By Joe Mussomeli

Being uncomfortable can be nerve-wracking, strange, and sometimes scary. For my brother, Alex, 14, being uncomfortable is all of these things. Born with a hearing loss, Alex has felt uncomfortable so many times in his life it’s impossible to count them all. He recently found himself in an especially uncomfortable situation when he was invited for a swim and sleepover at a friend’s house.

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To most teenagers, these activities aren’t uncomfortable; they’re fun. But when Alex received his invitation, he was overcome by anxiety related to his hearing loss. He worried he’d take too long to change into his swimming clothes, which include a shirt that attaches to his waterproof cochlear implant (CI). He dreaded others seeing him wear this. He feared he wouldn’t understand every word the other kids said in the pool. Above all, he was nervous the other kids would distance themselves from him because he was different.

Swimming wasn’t even the most distressing part. It was the mention of a sleepover that troubled Alex most. Just the mention of the word “sleepover” made his stomach tie into a knot. Every night, he removes his CI and hearing aid. He places the devices in a dry and store dehumidifier box, powers on the box, and then, finally, goes to sleep. Carrying out this routine at a sleepover would be, in theory, extremely difficult. Alex would have to keep his hearing devices on while talking to his friends at night. At the conversation’s end, Alex would have to take off his devices, but if it resumed, he would have to go through the hassle of putting his devices back on, or he would have to be left out. The whole situation would be unconventional and nerve-wracking for him. Essentially, it would be uncomfortable.

Alex was so uncomfortable that he almost declined the invitation until our dad pointed him in a different direction. The morning before the sleepover, Alex asked Dad if he should go to his friend's house. My dad told him that he should. Alex was quiet for a moment before he poured out all of his anxiety. He told him about how nervous he was and all of his other discomforts. Dad responded, “I won’t tell you what to do, but I will tell you this: Do you want to be uncomfortable your whole life, or are you willing to take a chance?” He left Alex to think over these words.

In the end, Alex didn’t go to the sleepover, but he went for a swim. Yes, it did take him longer than the other kids to change for the pool, and yes, he didn’t hear every word his friends said in the pool. But he still went, he dove into a pool of discomfort, dealt with it, and to his surprise, he had a bit of fun. He texted my dad an hour later asking if he could stay until at least 9 PM. My dad replied with three words: “If you’re comfortable.” Alex replied with two: “I am.”

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ChEARs to Our Hearing

By Lauren McGrath

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To extend our celebration of World Hearing Day (March 3), Hearing Health Foundation (HHF) has partnered with the innovative Class I hearing aid manufacturer Eargo to raise funds for hearing loss research and awareness.

Eargo launched the ChEARs to Our Hearing Facebook fundraising campaign Monday, March 4, and the company is generously matching all gifts made up to $1,000.

In alignment with HHF’s 2017 survey about barriers to hearing loss treatment, Eargo’s Audiologist, Steve Eagon, says, “Our consumers are most concerned about the price of hearing aids. User-centric innovations driven by HHF’s scientific research—specifically through its Emerging Research Grants (ERG) program—will ultimately advance more possibilities for hearing aid technology,  eventually driving down costs.”

HHF also champions the importance of treating a hearing loss to maintain one’s quality of life, or, as Eargo’s mission statement reads, “hear life to the fullest.” An untreated hearing loss can increase one's risk of experiencing cognitive decline, dementia, falls, social isolation, and depression.

Eargo also shares HHF’s motivation to reduce the stigma of hearing loss. “There always has been, and still is, a lot of stigma around hearing aids. As a society, we're focused on looking and feeling young, and using hearing aids are often seen as a sign of submission to old age. Instead of investing in one of their senses, people often deny their hearing loss—by turning the volume up, asking people to repeat themselves, and unfortunately avoiding the situation rather than dealing with it head on,” Eagon says.

HHF is deeply grateful to Eargo for their decision to support hearing loss research and awareness. HHF kindly invites our readers to make a contribution to ChEARs to Our Hearing on Facebook. Thank you for your consideration and support of our work!

 
 

About Eargo:

Eargo is a direct-to-consumer health tech company that’s destigmatizing hearing loss through consumer-driven innovation. Eargo devices are available for individuals with mild to moderate hearing loss. The Eargo Max model was recognized as one of TIME’s best inventions of 2018.  For more, see eargo.com.

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In Memoriam: Noel Cohen, M.D.

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Hearing Health Foundation (HHF) shares with great sadness the passing of Board of Directors member Noel Cohen, M.D., who dedicated his career to helping people hear. Cohen was a world-renowned cochlear implant surgeon at New York University (NYU) Langone Medical Center whose contributions as a clinician, scientist, and educator will forever enrich hearing health.

Cohen served as a lieutenant in the U.S. Naval Reserve before completing his ENT residency at NYU School of Medicine and Bellevue Hospital in 1962. Cohen held many leadership roles in the years to follow including professor of otolaryngology at NYU; chair of NYU’s department of otolaryngology–head & neck surgery; acting dean at the NYU School of Medicine; and president of the NYU Hospital Center.

Elizabeth Keithley, Ph.D., the chair of HHF’s board, spoke highly of Cohen’s passion for building the hearing research community by providing opportunities for its youngest members. “He was a strong advocate for funding young investigators through our Emerging Research Grants [ERG] program to help their establishment as academics and scientists,” Keithley says.

Cohen oversaw the ERG grantmaking process as a member of HHF’s Council of Scientific Trustees (CST) prior to joining the board in 2016. Additionally, Cohen and his late wife, Baukje, were committed financial supporters of HHF through their family foundation.

Anil Lalwani, M.D., also a member of HHF’s board and the head of the CST, was a colleague of Cohen’s at NYU He fondly remembers him as “a surgeon instrumental in providing the priceless gift of hearing to countless youngsters and adults who otherwise would still be living in a silent world.”

Cohen will be deeply missed by HHF and the otolaryngology community. We are grateful to Cohen for his immense service to those who study, treat, and live with hearing loss.

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Ears On Fire

by Ben Meltzer 

I attended my first concert, Harry Chapin, at age 3. This was on March 24, 1977 at Painters Mill Music Fair in Owings Mills, Maryland. 14 years later, at the same venue, I saw my first of some 35 Bob Dylan concerts. I was a big concertgoer who wore earplugs only sporadically.

I had a great deal of additional noise exposure in an office where my colleagues and I were subjected to routine fire alarm testing during working hours. And for several weeks, I sat near a squealing printer that ran continuously.

In early 2007, I started feeling fullness in my left ear. Over the next nine months, it worsened. I experienced my first bout of fleeting tinnitus or SBUTT: sudden brief unilateral tapering tinnitus.

An ENT told me aural fullness is a common symptom of temporomandibular joint disorder, or TMJ, so I visited my dentist, who made me a mouthguard. It made my symptoms worse.

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Two weeks later, I awoke to find ordinary sounds were painfully loud. The honk of a car horn was excruciating, as was the roar of a lawnmower.

More debilitating than this knifelike pain from sound, however, was a constant burning pain in the ears that existed independent of sound. This symptom confounded the audiologist and ear doctors I saw. 

The loudness discomfort test I took at a prominent hospital indicated severe hyperacusis, but the audiologist there said she had never encountered a patient with constant ear pain. The institution’s inexperience with my symptoms unnerved me. 

Over the next two years–with time, quiet, and ear protection–I slowly improved. Then, two big noise insults in the same week proved catastrophic. At the dentist, with a dental drill whirring to my right, I felt something break in my right ear. A few days later, I was near the fire station when an air-raid siren blasted, loud enough to alert the whole town’s firefighters.

From then on, my symptoms of noise trauma―aural fullness, tinnitus, hyperacusis, and burning pain―intensified. The trauma turned my right ear red and veiny, and it became sensitive to wind and cold. When my hair grows longer, I feel it on my right ear more acutely than on my left. So I now have a “good ear” and a “bad ear,” with similar symptoms that differ in severity. 

Worst of all, the right ear could no longer tolerate an earplug or being covered with a protective earmuff. I get severe pain from merely covering the ear.  This symptom has mitigated slightly. I can now–more than 10 years post-injury–wear earmuffs for around 5 minutes on a good day and still not at all on a bad one.  Sometimes I feel as though there’s a lit match inside my ear. 

A noise injury worsens readily. For hyperacusis sufferers such as myself, quiet makes the condition better; noise makes it worse. Among sufferers this is indisputable, but medical practitioners bizarrely treat quiet as harmful. Too often they think the patient’s chief concern is hearing loss, rather than an intolerable perception of sound. 

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Two nonprofits currently researching noise-induced pain and pushing the field forward are Hyperacusis Research and Hearing Health Foundation. New discoveries, with scientific tools and techniques only recently available, show that noise is far more destructive than previously believed. 

I plan to donate my ears to the Temporal Bone Registry so that researchers will be able to examine them after my death to better understand hyperacusis, relieve it, and put an end to the misery endured by those like me.

Ben Meltzer is from Baltimore and now lives with his wife in New York.

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First Study to Examine Cognitive Development in Deaf Babies Finds Differences Begin in Infancy

By The Ohio State University Wexner Medical Center

Deaf children face unique communication challenges, but a new study shows that the effects of hearing impairment extend far beyond language skills to basic cognitive functions, and the differences in development begin surprisingly early in life. Researchers at The Ohio State University Wexner Medical Center are the first to study how deaf infants process visual stimuli compared to hearing infants and found they took significantly longer to become familiar with new objects.

 “This is somewhat counterintuitive because a lot of people assume that deaf children compensate for their lack of hearing by being better at processing visual things, but the findings of the study show the opposite,” said Claire Monroy, post doctorate otolaryngology fellow at The Ohio State University Wexner Medical Center and co-author of the study.

Macey Kinney plays with her 10-month-old son Zealand, who was born deaf. A new study shows that developmental differences in deaf babies extend beyond language and hearing, and begin surprisingly early in life. Credit: Ohio State University Wexner Medical Center

Macey Kinney plays with her 10-month-old son Zealand, who was born deaf. A new study shows that developmental differences in deaf babies extend beyond language and hearing, and begin surprisingly early in life. Credit: Ohio State University Wexner Medical Center

To test their visual processing skills, researchers showed infants different objects on a screen. When a baby has successfully encoded the object, they will lose interest and look away. This familiarization is what researchers call habituation. “Deaf infants took longer to habituate to the objects and looked away from them less than hearing infants,” said Derek Houston, associate professor of otolaryngology at Ohio State. “These results were surprising because you wouldn’t expect there to be such profound differences in a test that really has nothing to do with hearing.”

However, researchers say the results don’t necessarily mean that deaf children are learning at a slower pace. “Because they use vision to process the world around them, they may pay closer attention to visual objects,” said Houston. “They might actually be processing more about each object.”

Future research will examine why these differences in visual learning exist so that each child is taught in a way that works best for them and leads to healthy development. “Understanding the source of these differences can really help us tailor interventions specifically for these children,” said Monroy. “And the earlier that happens, the better.”

This article was republished with permission from the Ohio State University Wexner Medical Center. See the original press release here.

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Becoming a Champion

By Rose DuMont

Leaving home for college with a severe to profound hearing loss was difficult. I graduated high school with high honors and had a lot of friends despite receiving only minimal support from my behind-the-ear hearing aids.

The inadequacy of my devices, which provided only a “soft” introduction to sound, soon caught up to me when I began my undergraduate studies. I struggled to hear my professors, and I was overwhelmed by the amount of responsibility I was required to take on. Among my many classmates in large lecture halls, I felt invisible and became lazy about attending classes. I was unable to fully participate in conversations with others.

In the first semester, I developed an ear infection that caused my right eardrum to burst. This immediately caused vertigo, which I wrote off as a one-time, horrible experience. I was in my dorm, where I remained on the floor for an hour until the world stopped spinning. It felt as if the floor had been ripped out from under me and nothing would be right ever again. I didn't throw up that first time, but I have thrown up nearly every time since.

Over the next two years, I experienced vertigo with increasing frequency. Eventually I was having attacks every few days. Every vertigo episode seemed never ending. Once the spinning stopped, there was no relief; just a feeling of impending doom. My tinnitus―which already affected me throughout my life―became more pronounced than ever before. The incessant white noise sometimes makes me feel like I am trapped in a huge indoor stadium with thousands of people talking at once.  I consulted an ENT at Mass Eye and Ear who administered three sets of vestibular tests, two MRIs, and multiple hearing tests over an 18-month period. Finally, at age 21, I received a Ménière's disease diagnosis and I searched and found a specialist at UMass Worcester for treatment.

Being given a definitive reason for my debilitating vertigo brought immense relief. At last, I could give a name to the source of my misery and take appropriate doctor-directed measures. I reduced my sodium intake, kept my weight in check, and tried my best to reduce stress.

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I was prescribed a low dose of Klonopin (taken daily) and Ativan (when I felt an attack coming on), which I took diligently for nearly 3 years before I decided I would rather not be reliant on drugs for the rest of my life. I voluntarily gave up driving for 2 years, not knowing when an episode would hit. Klonopin allowed me to lead a somewhat normal life and kept my vertigo at bay. Taking that daily, I was able to go to my college classes most of the time and began to drive a car again. Weaning myself off of Klonopin was one of the most difficult things I’ve ever had to do. It took me 9 months, but my body was able to compensate for the change as I slowly took my reliance off the drug and onto my own vestibular system. I took up yoga, started running marathons, encouraged the positive relationships in my life to outweigh the negative ones, and have continued my low-sodium diet for over a decade. My life is infinitely better becoming consistent with these practices.

At 23, I received a cochlear implant in my left ear at the recommendation of my audiologist. The day after my surgery, I had vertigo for 18 hours straight. Fortunately, I’ve never experienced something like that since. And, after getting my implant programmed and hearing my audiologist ask, “Can you hear me?” I knew it was the right decision. Cochlear implantation has enabled me to become more independent and, therefore, happier. I’m still excited by the new sounds I discover each day.

I started running long distance when I was 29, and am hooked on how good running makes me feel. Since I’ve started, my vertigo happens far less than it ever did (only 3 or 4 times a year!), and I know I am the one in control, instead of feeling as if Ménière's disease controls me. I’ve run 5 half marathons and 6 marathons, with my 7th in March in Washington, D.C. and 8th in June in Portland, OR.

At this point I can say Ménière's disease and my initial negative experiences in undergraduate school have impacted my life for the better. Ménière's is a lonely condition but it’s forced me to become much more self-reliant―an important trait to finding both work and friends wherever I go, as I’ve moved around the country. Before I could depend on myself, I’d look to everyone else to try to do things just like them. In college, for example, I tried to listen and take notes at the same time during classes before realizing, at age 30, that I’m not able to learn the same way as someone with typical hearing. Once I realized that, my Masters degree was a breeze, and I was able to easily earn my 3.9 GPA.

Ménière's disease does not affect all people the same way.  Not everyone has the same symptoms so it can be difficult to diagnose. Also, there isn’t one specific treatment. If one method doesn’t help, then you need to try another. Be patient and realize you are stronger than you think.

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If I could, I’d tell my younger self―just after my Ménière's diagnosis―this: “You are stronger than you think you are.” The same goes for anyone becoming acquainted with the condition. I’d tell them, “You’re a champion.” I’m not yet a running champion; I have never placed in a race. Though I can run a marathon in under 4 hours, my dream is to reduce my time by 20 minutes to qualify for the Boston Marathon. There I can compete alongside some of the best runners in the world, doing what keeps me balanced, relying only on myself.

Rose DuMont lives in Arizona where she works as a teacher of the deaf. She was diagnosed with hearing loss at age 5 and is a participant in HHF’s “Faces of Hearing Loss” project.

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Your Concerns About Cochlear Implants, Answered

By René H. Gifford, Ph.D, CCC-A, and David S. Haynes, M.D., FACS

“If you qualify for a cochlear implant (CI) but do not have one, please indicate reasons you have not pursued.”

This question was one of three dozen on hearing conditions posed in Hearing Health Foundation’s 2017 Reader Survey, which was administered through the pages of this magazine, online, and also with Hearing Loss Association of America, through their magazine and online. More than 2,300 people responded to the survey. Those who responded to the CI question above gave the following answers, and they were able to check off as many as applied. In order of popularity, the responses were: 1) not convinced of improvement, 2) surgery complications, 3) waiting for biological cure, 4) concerns about sound quality, 5) cosmetic, and 6) fear of discomfort.

Although “other” with a fill-in option was offered, age did not appear to be a primary concern. In fact, many older adults with severe to profound hearing loss whose hearing aids are no longer are beneficial have found success with CIs, as demonstrated by Barbara Sinclair (page 20), who received an implant 17 years ago at age 72, and our cover story author Bruce Douglas (page 6), who had implantation surgery at age 91.

Part of the survey’s purpose is to better understand the needs of our community of readers and supporters, and so, as cochlear implant surgeons, we wanted to address these concerns.

Source: University of Illinois Hospital

Source: University of Illinois Hospital

Not convinced of improvement

All CI centers, including ours at Vanderbilt University Medical Center, perform extensive presurgical testing to determine if a CI is the right option for a patient, versus the continued use of hearing aids. The testing, based on data and experience, answers this question with an incredible degree of accuracy. Our goal is to reach a level of hearing that dramatically outperforms the best hearing aid outcomes for a given individual. Expectations are much higher than this, however, and it is extremely rare for a patient who is wearing their implant full-time not to experience much better preoperative hearing performance. The benefit has been so pronounced that Vanderbilt and other CI centers are working to expand implantation criteria so that this technology reaches people with milder forms of hearing loss.

Surgical complications

Cochlear implantation has one of the most favorable risk–benefit ratios of any surgical procedure in the U.S., offering significant communicative benefit while incurring little risk. Our center performs nearly 300 implants per year, and we monitor and track all procedures, outcomes, and complications. As with any operation your surgical team will provide a list of potential complications in order to be comprehensive, but the actual incidence of CI surgery complications ranges from under 1 percent to 3 percent. If any do occur, they are considered minor and temporary, such as postoperative taste disturbances and dizziness. At most CI centers, implantation is completed as an outpatient procedure and generally performed in 1 to 1.5 hours. We recently completed cochlear implantation on a 96-year-old patient who went home on the same day of surgery.

Waiting on a biological cure
The field of hearing restoration through hair cell regeneration—some of which is being conducted by HHF scientists, through the Hearing Restoration Project—is still in its earliest phases. While there have been exciting advances in gene therapy, current technology via cochlear implants can provide people with severe to profound hearing loss immediate access to sound, and all the benefits that this brings. In addition, improved success with CIs is linked to implantation that occurs closer to the onset of hearing loss, as auditory pathways in the brain need to be stimulated or they weaken. Otherwise the resulting permanent changes in the brain’s auditory centers may limit the ability of a patient to hear, even with a perfectly intact cochlea.

Concern about sound quality
Despite CIs being a mechanical device, the voice sound quality has the potential to be no less electronic sounding than that from a telephone, computer, or television. Often the abnormal sound is due to the stimulation of an ear that hasn't heard for many years (or an ear that has never heard). If this occurs, it typically dissipates with continued use of the CI and the stimulation of auditory pathways. Signal processing technology also continues to advance at a rapid rate, allowing for personalized programming for the best hearing outcomes, and—especially with any neural changes with age—programming is important to do at regular intervals.

Cosmetic
The thin internal portion of the CI is designed to sit flush with the skull and is not visible. The visible external components (the battery, sound processor, microphone, and transmitting coil) mostly fit behind the ear, not much larger than a standard behind-the-ear hearing aid. The latest sound processors are self-contained in a single unit about the size of a half dollar coin. These “off-the-ear” processors do not have an over-the-ear component, but rest directly over the magnet that is behind the ear and within the hairline. Eventually we expect that all implanted systems will be compatible with these smaller, off-the-ear processors, and nanotechnology and battery miniaturization will further reduce processor size. (And, the
boom in wearable consumer technology makes visible devices even more mainstream.)

Fear of discomfort
Implantation incisions behind the ear heal quickly, and the drilling of the bone required to place the
implant is a simple mastoidectomy. It is a component of most ear procedures and is not painful. Our center performs over 1,200 mastoidectomies per year across various different ear procedures. Postoperative discomfort is a rare complication and easily managed with over-the-counter medications such as acetaminophen (Tylenol).

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Do You Qualify?
If you have a hearing loss that prevents you from talking on the phone without visual cues (such as needing video calls or caption calling); are unable to understand television programs without closed captioning; and/or are actively avoiding large group gatherings for fear of conversational difficulty, talk to your hearing healthcare professional to see if you may be a CI candidate. CIs are the most successful sensory restoration prostheses to date and have been successfully placed in more than half a million individuals worldwide. The wonders of this technology vastly improve hearing, speech understanding, and overall quality of life.  

René H. Gifford, Ph.D, CCC-A, is a professor in the department of hearing and speech sciences with a joint appointment in the department of otolaryngology at Vanderbilt University, Tennessee. She and HHF medical director David S. Haynes, M.D., FACS, direct the Cochlear Implant Program at the Vanderbilt Bill Wilkerson Center.

This article also appeared in the Fall 2018 issue of Hearing Health. For references, see hhf.org/fall2018-references.

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Shared Knowledge Is Power

By Lauren McGrath

Each February, thousands of hearing and balance scientists join their colleagues from around the world at the Association for Research in Otolaryngology (ARO) Mid-Winter Meeting. It is one of the premier international conferences for those in the field. I was fortunate to attend this year’s 42nd meeting, held in Baltimore, on behalf of Hearing Health Foundation (HHF), along with Emerging Research Grants (ERG) awardees past and present, Hearing Restoration Project (HRP) consortium scientists, and HHF scientific committee members—all of whom are integral to our mission to advance the prevention, treatment, and cures of hearing and balance conditions.

ARO provides auditory and vestibular researchers opportunities present their latest findings and engage in meaningful conversations with one another. If one scientist presents an idea to an audience of 100 scientists, she’s just created the possibility for 100 new ideas will form. Even one novel suggestion following a presentation at ARO can be invaluable to science.

Tenzin Ngodup, Ph.D., represents his HHF-funded tinnitus project at ARO.

Tenzin Ngodup, Ph.D., represents his HHF-funded tinnitus project at ARO.

One forum through which scientists share their knowledge at ARO is in the poster hall. ERG grantees including Tenzin Ngodup, Ph.D., and Samira Anderson, Au.D. Ph.D., stood proudly alongside large poster board displays ready to answer questions about their respective projects. Ngodup, who is currently funded by HHF and based at Oregon Health and Science University, used his poster to visually explain his progress investigating neuronal activity in the ventral cochlear nucleus (VCN) in order to prevent and treat tinnitus. “It was previously thought that there were a few hundred inhibitory glycinergic cells called D-stellate cells in the VCN, but we found a surprisingly large population of glycinergic cells— approximately 2,700—that are physiologically and morphologically distinct from D-stellate cells,” Ngodup says. By quantifying inhibitory neurons in the VCN he aims to examine inhibition in typical vs. tinnitus models, especially after noise exposure.

University of Maryland’s Anderson, a 2014 ERG grantee, represented an impressive half dozen informational poster boards with her colleagues. The titles included: “Aging Effects on the Auditory Evoked Cortical Potentials in Cochlear Implant Users”; “Mutual Information Analysis of Neural Representations of Speech in Noise in the Aging Midbrain”; and “Age-Related Degradation Is More Evident for Speech Stimuli With Longer Than With Shorter Consonant Transitions.” A clinician who transitioned to research, Anderson graciously thanked HHF for funding her first-ever scientific grant, and was thrilled to tell me her work had just been cited by the Wall Street Journal in an article called “Better Hearing Can Lead to Better Thinking,” published February 6, 2019, about the importance of hearing loss treatment in older adults.

Outside of the poster sessions in lecture halls, ARO attendees conduct topic-specific seminars to seated audiences. Elizabeth McCullagh, Ph.D., of University of Colorado Denver, a 2016 ERG grantee, led a symposium called “Mechanisms of Auditory Hypersensitivity in Fragile X Syndrome” in which she and other speakers, including Kelly Radziwon, Ph.D. (2017 ERG), and Khaleel Razak, Ph.D. (2018 ERG), presented their novel findings related to Fragile X syndrome: a genetic model for autism, difficulties in sound localization, and overstimulation by sound in mouse models.

2018 ERG grantees Joseph Toscano, Ph.D., A. Catalina Vélez-Ortega, Ph.D., and David Jung, M.D., Ph.D.

2018 ERG grantees Joseph Toscano, Ph.D., A. Catalina Vélez-Ortega, Ph.D., and David Jung, M.D., Ph.D.

Achim Klug, Ph.D., a volunteer ERG grant reviewer, remarked during the Council of Scientific Trustees (CST) reception—a gathering to formally honor our ERG 2018 grantees—how critical McCullagh’s ERG grant has been to her work as an early-career scientist. With seed funding from HHF, McCullagh was able to investigate and publish information about a previously underfunded topic and deepen understanding within the hearing research field, he said. Allen Ryan, Ph.D., another member of the CST, added the program is “immensely valuable for helping young scientists advance to receive a Research Project Grant [R01] from the National Institutes of Health.” Every dollar invested in ERG grantees yields $91 from the NIH.

The HRP consortium also convened at ARO to deliver updates on five active projects following their most recent Seattle meeting. Bioinformatics and epigenetics were major focal points with Ronna Hertzano, M.D., Ph.D., showcasing updates to the gEAR database that she created (“Gene Expression Analysis Resource”) and Neil Segil, Ph.D., reporting on gene changes in the mouse inner ear, a project he works on with fellow HRP scientists Michael Lovett, Ph.D., David Raible, Ph.D., and Jennifer Stone, Ph.D.

Stefan Heller, Ph.D., who spoke about his Stanford lab’s work on transcriptome changes in single chick cells, noted: "The investments in the HRP are truly paying off, especially in the last one to two years. HRP investigators had major papers published and obtained National Institutes of Health support with the help of funding for the HRP consortium. Regarding my laboratory’s work, HRP support has given us the chance to focus on getting the highest possible quality of data—in my mind, the most important foundation for future work."

HHF looks forward to work to come from Ngodup, Anderson, McCullagh, and other ERG grantees, as well as the collaborative efforts of the HRP to advance a biological cure for hearing loss. We sincerely thank our generous donors and supporters who make this life-changing work possible.

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