On a Data-Driven Mission

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

The annual meeting of Hearing Health Foundation’s (HHF) Hearing Restoration Project was held in Seattle November 11-12, 2018. We used this meeting to update one another on recent progress on HHF-funded projects, discuss in detail the implications of new data, evaluate the directions of ongoing projects, and plan for the next funding period.

As you may recall, in November 2016 the Hearing Restoration Project (HRP) made a deliberate turn toward funding only the highest-impact science that our group leads the world in researching—we have termed this the “Seattle Plan.” We therefore devoted a substantial portion of our efforts to cross-species comparisons that contrast molecular responses to inner ear sensory hair cell damage in species that regenerate their hair cells, especially chickens and fish, with responses in mice, which like other mammals do not regenerate their hair cells. We also have been examining the “epigenetic” structure of key genes in the mouse, as one hypothesis is that epigenetic modifications of the DNA—that is, the inactivation of genes through chemical changes to the DNA—causes mouse (and human) cells of the cochlea to no longer respond to hair cell damage by regenerating hair cells.

  Avian and mammal supporting cell subtypes differ, but Stefan Heller, Ph.D., and team are investigating if an evolutionary homogenous equivalent exists in the organ of Corti, and if this knowledge could be used for hair cell regeneration. Credit: Chris Gralapp / Otolaryngology Head and Neck Surgery (OHNS) - Stanford University School of Medicine

Avian and mammal supporting cell subtypes differ, but Stefan Heller, Ph.D., and team are investigating if an evolutionary homogenous equivalent exists in the organ of Corti, and if this knowledge could be used for hair cell regeneration. Credit: Chris Gralapp / Otolaryngology Head and Neck Surgery (OHNS) - Stanford University School of Medicine

I am happy to report that progress over the past two years on these two major projects has been outstanding. For the cross-species comparisons, Stefan Heller, Ph.D., and Tatjana Piotrowski, Ph.D., reported on single cell analysis of, respectively, chick and fish hair cell organs responding to damage. Using single cell analysis—isolating hundreds to thousands of individual cells and quantifying all of the protein-assembly messages they express—we can determine the molecular pathways by which hair cells are formed during development and regeneration. This approach has always been promising, but this year we have begun to reap the expected benefits, as those projects have given us an unprecedented view of hair cell formation.

The epigenetics project overseen by Neil Segil, Ph.D., has now reached maturity, and using the voluminous data acquired over the past several years his lab has shown how supporting cells (from which we intend to regenerate hair cells) change the epigenetic modification of their DNA so they no longer are able to switch on key genes used for turning them into hair cells. A topic of great interest at the meeting was that of genetic reprogramming: Can we use genes (like transcription factors, proteins that control the transfer of genetic information) or small molecules (which often can be taken orally and still reach their targets) to overcome the epigenetic modification and push supporting cells to turn into hair cells? Preliminary results from Segil’s lab and from others in the field make us optimistic that the reprogramming approach will eventually be part of a regeneration strategy.

We also heard from Seth Ament, Ph.D., a bioinformatics expert we recently recruited to the HRP to explicitly compare our various datasets and find the common threads between them. Ament has used gene expression data from the chick, fish, and mouse, as well as the epigenetic data from the mouse, to hypothesize which genes may be important for hair cell regeneration. As a systems biology specialist, Ament brings a fresh eye to the field of auditory science and has not only identified some of the genes we expected to be important, but new ones as well. His success nicely justifies our cross-species approach, and the bioinformatics comparisons that he has been able to achieve in his initial HRP project have been impressive.

Finally, two other Seattle Plan projects have gone well, including our data-sharing platform called the gEAR (gene Expression Analysis Resource), developed by Ronna Hertzano, M.D., Ph.D., which allows us to analyze our data privately but also to efficiently share data with the public. In addition, John Brigande, Ph.D., reported on his project developing mouse models for testing interesting new genes; his group will be adding several powerful models in the year to come.

The excitement at the meeting extended to our future plans. We agreed that the Seattle Plan was the still the proper course, and we eagerly anticipate more data and results to come from our consortium of researchers. We are truly getting a clearer picture of hair cell regeneration due to the HRP’s efforts. That said, there is a long way to go; our efforts show us how surprisingly intricate biology is, despite knowing from the start that systems like the inner ear are remarkably complex. Nature always has surprises for us, by turns dashing treasured hypotheses while revealing unexpected mechanisms. The HRP is most definitely on track for success, and all of us in the HRP sincerely thank you for your continued support.

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

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My Cautious Gratitude

By Lauren McGrath

  Clockwise from left: Heather, her daughter, her husband, and son.

Clockwise from left: Heather, her daughter, her husband, and son.

Heather Mills never imagined her early adulthood would be interrupted by Ménière's disease, a chronic hearing and balance disorder without a cure. She was diagnosed at 21—just within the typical 20-to-50-year-old range of onset—after a slew of tests and follow-up visits with a specialist at the University of Minnesota.

Heather’s symptoms initially included a unilateral (in one ear) mild low-frequency hearing loss, tinnitus, and some ear pressure and pain. Within a few years, her hearing loss became bilateral and worsened from moderate to severe. She was regularly distressed by intense ear pressure, struggled with her balance, and experienced occasional bouts of vertigo. As Heather learned, Ménière's affects each patient differently. She considered herself fortunate not to face drop attacks (instances of falling to the ground without losing consciousness), one of the most terrifying symptoms associated with Ménière's disease.

Despite its prevalence Heather family, hearing loss—once her most debilitating Ménière's symptom—came as a surprise. Her father has lived with a unilateral hearing loss since childhood, while her mother and maternal grandmother both developed high-frequency sensorineural hearing loss in their late 40s. “It never occurred to me that it may one day affect me, too,” reflects Heather, who can recall her ability to hear whispers across her high school classrooms.

Though she followed her doctor’s directions to take diuretics and maintain a low-salt diet for her vestibular symptoms, Heather chose not to purchase hearing aids. Lacking amplification, Heather faced difficulty in her job as a legal project specialist, which required frequent verbal interaction with clients, lawyers, and vendors both on the phone and in person. She found herself increasingly dependent on a close friend and colleague who truly served as her ears by repeating information for her during and after meetings.

Heather’s untreated hearing loss, combined with her constant fear of a sudden vertigo attack, fueled feelings of isolation. Unable to participate in conversations with friends, Heather stopped receiving invites to social outings. Challenges with work and friends began to affect Heather’s mental health. “I became depressed, lonely, and developed anxiety because of two unknowns: not knowing when my vertigo would strike again, and wondering how I’d continue to work to support my family.”

Heather noticed a sharp decline in her job performance when her helpful coworker—her ears—left the law firm. Part of Heather’s role required instructing staff on new software, and she was humiliated to find out that her trainees’ questions went unanswered because they’d not been heard. “This is when I began to lose confidence in myself,” Heather remembers.

Heather realized she had to address her hearing loss. In line with Hearing Health Foundation (HHF)’s findings in a 2017 survey of more than 2300 participants, cost is by far the largest barrier to a hearing aid purchase. Heather delayed taking action for so many years—11 to be exact—because her insurance provided no hearing healthcare coverage.

“All I can say now is I wish I had gotten hearing aids sooner!” exclaims Heather, who, with newfound confidence, no longer struggles in her daily professional communications and social life. Prior to pursuing treatment, her conversations had soured quickly when she constantly had to ask other parties to speak up, repeat themselves, or remind them of her hearing loss. Most painfully, communication without hearing aids often left Heather dismissed by a “nevermind” when she requested repetition. With her new devices, Heather felt her confidence restored.

Now in remission, Heather considers her life happy and her health stable. Hearing aids have somewhat alleviated her tinnitus, her ear pressure has subsided, and the vertigo spells are very rare. She’s sought treatment for her anxiety and depression. Heather credits her husband, Billy, with whom she has two young children, for his support during her more difficult years. Engaging in online Ménière's support groups has been a beneficial coping tool.

Heather is cautiously grateful for her current health, knowing the unpredictability of Ménière's could alter her circumstances at any time. She hopes for scientific advancements in Ménière's research that will one day uncover the causes, more reliable diagnostic procedures, and a cure.

Heather lives in Minnesota with her husband and children. She is a participant in HHF’s Faces of Hearing Loss campaign.

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Detailing the Relationships Between Auditory Processing and Cognitive-Linguistic Abilities in Children

By Beula Magimairaj, Ph.D.

Children suspected to have or diagnosed with auditory processing disorder (APD) present with difficulty understanding speech despite typical-range peripheral hearing and typical intellectual abilities. Children with APD (also known as central auditory processing disorder, CAPD) may experience difficulties while listening in noise, discriminating speech and non-speech sounds, recognizing auditory patterns, identifying the location of a sound source, and processing time-related aspects of sound, such as rapid sound fluctuations or detecting short gaps between sounds. According to 2010 clinical practice guidelines by the American Academy of Audiology and a 2005 American Speech-Language-Hearing Association (ASHA) report, developmental APD is a unique clinical entity. According to ASHA, APD is not the result of cognitive or language deficits.

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In our July 2018 study in the journal Language Speech and Hearing Services in the Schools for its special issue on “working memory,” my coauthor and I present a novel framework for conceptualizing auditory processing abilities in school-age children. According to our framework, cognitive and linguistic factors are included along with auditory factors as potential sources of deficits that may contribute individually or in combination to cause listening difficulties in children.

We present empirical evidence from hearing, language, and cognitive science in explaining the relationships between children’s auditory processing abilities and cognitive abilities such as memory and attention. We also discuss studies that have identified auditory abilities that are unique and may benefit from assessment and intervention. Our unified framework is based on studies from typically developing children; those suspected to have APD, developmental language impairment, or attention deficit disorders; and models of attention and memory in children. In addition, the framework is based on what we know about the integrated functioning of the nervous system and evidence of multiple risk factors in developmental disorders. A schematic of this framework is shown here.

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In our publication, for example, we discuss how traditional APD diagnostic models show remarkable overlap with models of working memory (WM). WM refers to an active memory system that individuals use to hold and manipulate information in conscious awareness. Overlapping components among the models include verbal short-term memory capacity (auditory decoding and memory), integration of audiovisual information and information from long-term memory, and central executive functions such as attention and organization. Therefore, a deficit in the WM system can also potentially mimic the APD profile.

Similarly, auditory decoding (i.e., processing speech sounds), audiovisual integration, and organization abilities can influence language processing at various levels of complexity. For example, poor phonological (speech sound) processing abilities, such as those seen in some children with primary language impairment or dyslexia, could potentially lead to auditory processing profiles that correspond to APD. Auditory memory and auditory sequencing of spoken material are often challenging for children diagnosed with APD. These are the same integral functions attributed to the verbal short-term memory component of WM. Such observations are supported by the frequent co-occurrence of language impairment, APD, and attention deficit disorders.

Furthermore, it is important to note that cognitive-linguistic and auditory systems are highly interconnected in the nervous system. Therefore, heterogeneous profiles of children with listening difficulties may reflect a combination of deficits across these systems. This calls for a unified approach to model functional listening difficulties in children.

Given the overlap in developmental trajectories of auditory skills and WM abilities, the age at evaluation must be taken into account during assessment of auditory processing. The American Academy of Audiology does not recommend APD testing for children developmentally younger than age 7. Clinicians must therefore adhere to this recommendation to save time and resources for parents and children and to avoid misdiagnosis.

However, any significant listening difficulties noted in children at any age (especially at younger ages) must call for a speech-language evaluation, a peripheral hearing assessment, and cognitive assessment. This is because identification of deficits or areas of risk in language or cognitive processing triggers the consideration of cognitive-language enrichment opportunities for the children. Early enrichment of overall language knowledge and processing abilities (e.g., phonological/speech sound awareness, vocabulary) has the potential to improve children's functional communication abilities, especially when listening in complex auditory environments. 

Given the prominence of children's difficulty listening in complex auditory environments and emerging evidence suggesting a distinction of speech perception in noise and spatialized listening from other auditory and cognitive factors, listening training in spatialized noise appears to hold promise in terms of intervention. This needs to be systematically replicated across independent research studies. 

Other evidence-based implications discussed in our publication include improving auditory access using assistive listening devices (e.g., FM systems), using a hierarchical assessment model, or employing a multidisciplinary front-end screening of sensitive areas (with minimized overlap across audition, language, memory, and attention) prior to detailed assessments in needed areas.

Finally, we emphasize that prevention should be at the forefront. This calls for integrating auditory enrichment with meaningful activities such as musical experience, play, social interaction, and rich language experience beginning early in infancy while optimizing attention and memory load. While these approaches are not new, current research evidence on neuroplasticity makes a compelling case to promote auditory enrichment experiences in infants and young children.

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A 2015 Emerging Research Grants (ERG) scientist generously funded by the General Grand Chapter Royal Arch Masons International, Beula Magimairaj, Ph.D., is an assistant professor in the department of communication sciences and disorders at the University of Central Arkansas. Magimairaj’s related ERG research on working memory appears in the Journal of Communication Disorders, and she wrote about an earlier paper from her ERG grant in the Summer 2018 issue of Hearing Health.

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Suffering After Sacrifice

By Lauren McGrath

Every Veterans Day, Hearing Health Foundation (HHF) celebrates the brave individuals who have served and sacrificed to defend our country. We are grateful to our active military members and veterans for their courageous protection of American values and freedoms.

As we honor those who have served in the U.S. Armed Forces, we acknowledge a tragic and troubling health problem. An astounding number of veterans—60% of those returning from Iraq and Afghanistan—live with tinnitus and noise-induced hearing loss. In 2017, the Veterans Administration reported 1.79 million disability compensation recipients for tinnitus and 1.16 million compensation recipients for hearing loss, the number one and two disabilities, respectively. In an HHF video about hearing loss treatment, Retired Army Colonel John Dilliard, Chair-Elect of HHF’s Board of Directors, explains, “The noise from repeated gunfire and high-frequency, high-performance aircraft engines takes its toll on the human hearing mechanisms.” Col. Dillard lives with both tinnitus and hearing loss following 26 years of service.

  John Dillard and fellow soldiers, Fort Irwin National Training Center, 1977.

John Dillard and fellow soldiers, Fort Irwin National Training Center, 1977.

Dr. Bruce Douglas, 93, remembers the moment his hearing became severely compromised while serving in the Navy during the Korean War. “On what was my 26th birthday, after pulling the trigger on the M1 rifle with no protection (none of us had any) multiple times, I was left with tendonitis in both knees—and worse, permanent, chronic tinnitus due to acoustic trauma. My hearing went downhill ever after, and every imaginable kind of sound and sensation has resulted from my tinnitus,” Douglas writes in the Fall 2018 issue of Hearing Health.

Hearing protection training must start as soon as one enters the military. But there is a misconception that hearing protection inhibits vital communication and mission readiness because hearing signs of danger is imperative to survival. “Soldiers want to be able to hear the snap of the twig and want to be able to be situationally. As a result, they are often resistant to wearing hearing protection,” Col. Dillard says.

Fortunately, sophisticated hearing protection technology does exist so that military personnel do not have to choose between protecting their ears or their lives. Examples include noise-attenuating helmets, which use ear cups to protect against hazardous sound, and Tactical Communication and Protective Systems, which protect against loud noises while amplifying soft ones.

The U.S. military continues to work toward safer hearing in the service. The U.S. Army has developed the Tactical Communication and Protective System (TCAPS), which are earbuds that dampen dangerous noises to safe levels using microphones and noise-canceling technology, while also providing amplification of softer sounds and two-way communication systems. An initiative by the U.S. Air Force called Total Exposure Health (TEH), meanwhile, focuses on overall health both on and off the job, will measure cumulative noise exposure over the course of 24 hours. These developments and others, which HHF applauds, are covered in greater detail in Hearing Heath’s Fall 2017 issue.

As greater preventative technology for our military becomes available, HHF remains dedicated to finding better treatments and cures for tinnitus and hearing loss to benefit the lives of millions of Americans, including veterans, a disproportionately affected group. We hope you will join us in remembering their sacrifices with gratitude and compassion.

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Measuring Brain Signals Leads to Insights Into Mild Tinnitus

By Julia Campbell, Au.D., Ph.D.

Tinnitus, or the perception of sound where none is present, has been estimated to affect approximately 15 percent of adults. Unfortunately, there is no cure for tinnitus, nor is there an objective measure of the disorder, with professionals relying instead upon patient report.

There are several theories as to why tinnitus occurs, with one of the more prevalent hypotheses involving what is termed decreased inhibition. Neural inhibition is a normal function throughout the nervous system, and works in tandem with excitatory neural signals for accomplishing tasks ranging from motor output to the processing of sensory input. In sensory processing, such as hearing, both inhibitory and excitatory neural signals depend on external input.

For example, if an auditory signal cannot be relayed through the central auditory pathways due to cochlear damage resulting in hearing loss, both central excitation and inhibition may be reduced. This reduction in auditory-related inhibitory function may result in several changes in the central nervous system, including increased spontaneous neural firing, neural synchrony, and reorganization of cortical regions in the brain. Such changes, or plasticity, could possibly result in the perception of tinnitus, allowing signals that are normally suppressed to be perceived by the affected individual. Indeed, tinnitus has been reported in an estimated 30 percent of those with clinical hearing loss over the frequency range of 0.25 to 8 kilohertz (kHz), suggesting that cochlear damage and tinnitus may be interconnected.

However, many individuals with clinically normal hearing report tinnitus. Therefore, it is possible that in this specific population, inhibitory dysfunction may not underlie these phantom perceptions, or may arise from a different trigger other than hearing loss.

One measure of central inhibition is sensory gating. Sensory gating involves filtering out signals that are repetitive and therefore unimportant for conscious perception. This automatic process can be measured through electrical responses in the brain, termed cortical auditory evoked potentials (CAEPs). CAEPs are recorded via electroencephalography (EEG) using noninvasive sensors to record electrical activity from the brain at the level of the scalp.

  Cortical auditory evoked potentials (CAEPs) are recorded via electroencephalography (EEG) using noninvasive sensors to record electrical activity from the brain.

Cortical auditory evoked potentials (CAEPs) are recorded via electroencephalography (EEG) using noninvasive sensors to record electrical activity from the brain.

In healthy gating function, it is expected that the CAEP response to an initial auditory signal will be larger in amplitude when compared with a secondary CAEP response elicited by the same auditory signal. This illustrates the inhibition of repetitive information by the central nervous system. If inhibitory processes are dysfunctional, CAEP responses are similar in amplitude, reflecting decreased inhibition and the reduced filtering of incoming auditory information.

Due to the hypothesis that atypical inhibition may play a role in tinnitus, we conducted a study to evaluate inhibitory function in adults with normal hearing, with and without mild tinnitus, using sensory gating measures. To our knowledge, sensory gating had not been used to investigate central inhibition in individuals with tinnitus. We also evaluated extended high-frequency auditory sensitivity in participants at 10, 12.5, and 16 kHz—which are frequencies not included in the usual clinical evaluation—to determine if participants with mild tinnitus showed hearing loss in these regions.

Tinnitus severity was measured subjectively using the Tinnitus Handicap Index. This score was correlated with measures of gating function to determine if tinnitus severity may be worse with decreased inhibition.

Our results, published in Audiology Research on Oct. 2, 2018, showed that gating function was impaired in adults with typical hearing and mild tinnitus, and that decreased gating was significantly correlated with tinnitus severity. In addition, those with tinnitus did not show significantly different extended high-frequency thresholds in comparison to the participants without tinnitus, but it was found that better hearing in this frequency range related to worse tinnitus severity.

This result conflicts with the theory that hearing loss may trigger tinnitus, at least in adults with typical hearing, and may indicate that these individuals possess heightened auditory awareness, although this hypothesis should be directly tested.

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Overall, it appears that central inhibition is atypical in adults with typical hearing and tinnitus, and that this is not related to hearing loss as measured in clinically or non-clinically tested frequency regions. The cause of decreased inhibition in this population remains unknown, but genetic factors may play a role. We are currently investigating the use of sensory gating as an objective clinical measure of tinnitus, particularly in adults with hearing loss, as well as the networks in the brain that may underlie dysfunctional gating processes.

2016 Emerging Research Grants scientist Julia Campbell, Au.D., Ph.D., CCC-A, F-AAA, received the Les Paul Foundation Award for Tinnitus Research. She is an assistant professor in communication sciences and disorders in the Central Sensory Processes Laboratory at the University of Texas at Austin.

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A Muffled Life

By Jim Lynch

A Tricycle Mishap

For a 5-year-old a tricycle is a mini Lamborghini. Whether this particular model belonged to my family or our next-door neighbor has long since faded in memory, but what made it especially attractive was fashioned to its handlebar: a rubber squeeze-bulb and silver metal horn. Jackie Gilroy and I took turns riding it between our houses for hours during the summer before I was scheduled to enter first grade. We were particularly fascinated by the sound of the horn, a noise we could make louder by using two hands to squeeze air into the metal chamber.

I can’t remember which of us made the suggestion, but one day we discovered that if we placed our ears next to the horn, the sound was louder still. Therefore, in the impulsive and thoughtless manner of children, we took turns blaring that explosive clangor into each other’s ears at point blank range for a good part of the afternoon. We laughed at our discovery and discovered that the effect lasted even longer, with ringing in our ears.

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When I woke the next morning and came downstairs, my mother was at the stove finishing scrambled eggs for my breakfast. As she put my plate before me, I saw her lips moving, but I heard nothing. I put my hands to my ears and began to cry as she tried without success to converse with me. Not only couldn’t I hear her, but I also couldn’t hear my own words, or even the sound of my crying. Overnight, I had become completely deaf.

Facing It

Over the next few days, some muted hearing gradually returned. After I informed her of my squeeze-bulb horn activity, she made an appointment for us with an audiologist. After explaining to him what I had done, and undergoing what passed for extensive testing in midcentury (I remember a series of tuning forks and having to turn my head at various angles and respond to his whispered questions), he informed her that I had permanently damaged the nerve endings at the higher frequency range of hearing in both ears. I remember him telling her that what had happened to me was akin to a soldier’s hearing when a grenade goes off in close proximity. While I didn’t suffer physical injury, the hearing loss was the same.

Even if there were hearing aids available during that era, two things became readily clear: my family would not have been in a financial position to afford them, and, given the type of hearing loss I had sustained, they wouldn’t have helped. Whatever the quality or degree of auricular attenuation I had sustained, it was permanent, and would last for the rest of my life. At five years of age, however, I was simply happy to have regained a measure of hearing. Whatever consequences suffered by Jackie Gilroy are lost to memory.

At that point in my young life, I had little trouble understanding my parents, siblings and friends who were in close proximity. They sometimes had to get my attention if my head were turned (my brothers would often yell, “Hey Beltone!”), but face-to-face conversation was possible. Even so, my parents decided to postpone enrolling me in first grade that September, with the hope that things might somehow improve before I would need to function in a classroom environment.

Starting first grade a year later, I began a long auditory adjustment that paralleled any and all social interaction. My hearing difficulty often appeared to teachers and fellow students as indifference, disrespect or stupidity. High-frequency loss also made it impossible to hear the syllables of some words, and therefore difficult to pronounce them as well.

The “ed” on the past tense of “ask,” for example completely disappeared. Sibilant syllables vanished from spoken words, and the susurration of whispers made them indecipherable. Embarrassment and mockery are stern but effective teachers, however, and they provided remarkable motivation for a trial-and-error approach to the demands of a wider world.

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And Faking It

I soon ascertained that there were many compensatory methods to bring to bear on my degraded hearing. I quickly learned, for example, that the first hint of what others were saying lay in their facial expressions. A frown, scowl or smile provided a starting point for what was to come.

Tone of voice was also a powerful indicator. A flat, staccato grouping of words coupled with a stern expression were causes for apprehension, while a soft, lilting tone combined with an open face often indicated harmony or agreement. If a speaker’s inflection turned up at the end of his sentence, he was likely posing a question.

I further adapted to a system of filling in the gaps when some of the words in a sentence went unheard because of distance, volume or pronunciation. “In what____ was the _______Armada ________ by Great _______?” From the back of the class, such an obvious question (upward inflection at end of sentence) could be understood in sufficient time by a student with hearing loss who had read the assigned history chapter. Those strategies worked with a modicum of success in a classroom where one person spoke at a time. In a noisy environment, however, sounds grew more remote and understanding more problematic.

When as an adult I had an extensive and more sophisticated evaluation done by an audiologist, I discovered that my hearing levels were 70% of normal in the left ear, and 72% in the right. Because of years of adapted strategies, however, my range of understanding registered in the low 90% level for both ears in a quiet, isolated environment.

Lingering Difficulties and Treatment at Last

Nevertheless, song lyrics and movie dialogue continued to pose problems. Because the usual strategies often failed in such circumstances, I often relied on imagination to provide meaning. With resourceful creativity, I used the melody of songs, and the tone of cinematic dialogue, as well as body language of the actors, to provide sufficient clues to the overall context of songs and movies. I sometimes think that my imagination provided better lyrics and dialogue than the lyricist or scriptwriter.

Not until 2005 did technology become available to augment my adaptive methodology. The devices I now use improve my hearing marginally, but I still rely on a lifetime of learned maneuvers to interact with others. Although the sounds of previously difficult sibilant syllables became somewhat crisper, other problems remain or were created.

A moderate wind sounds like a typhoon when it blows over the device’s microphone. In addition, ambient noise levels can still totally negate any level of discernment. At a social gathering such as a wedding reception, for example, the murmur and babble of guests make understanding people directly across a table hit-and-miss. When the band or DJ begins, I must cease conversation altogether, except to respond to the person to my immediate right or left, and then with considerable difficulty.

In the classroom, my disadvantage created a different approach to interaction with students. Because I was fortunate to teach in an atmosphere of deference and tranquility, the majority of conversations with students proceeded nicely. Sometimes, however, soft-spoken or rapid-speaking students, or those in the rear of the class could pose problems. If a request for a repeated question or comment failed to generate clarity, years of learned compensatory techniques usually facilitated sufficient comprehension.

It Made Me a Better Teacher

In retrospect, I suspect that my auditory deficit, and the changes it wrought, made me a better teacher than I would have been with typical hearing. Because I had to utilize alternate methods and techniques (with a visible keenness of focus) to interact with students, my interest in their opinions and evaluations must have conveyed an unusual intensity. As I strained to comprehend their questions, concerns and comments, my physical demeanor emphatically registered the genuine value I placed on understanding their questions and comments in class discussions.

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While a reduction in the ability to hear does not rise to the level of a significant physical disability, it changes the manner in which one must approach life. Such changes, although onerous, can also foster unforeseen advantages. My career as an educator was predicated on an adaptive approach to classroom procedure and management. Without a youthful hearing injury, I may not have gravitated toward teaching at all, or have enjoyed four decades of participation in that noble profession.

Jim Lynch was a high school English teacher for nearly four decades in the Wilkes-Barre, Pennsylvania area, as well as an adjunct English instructor at area universities and a community college. In retirement, he resides in Fleetwood, Pennsylvania with his wife of 51 years and two cats.

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Sudden Hearing Loss Is a Medical Emergency

By Donna Rohwer

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Mondays are always bad, right? I awoke deaf in my left ear—completely deaf. I asked my husband if he thought it was anything to worry about and he said, “Not yet.” I thought the same and appreciated the confirmation. We didn’t know then that sudden hearing loss is a medical emergency.

Sudden Sensorineural Hearing Loss (SSHL)

Damage to the inner ear, the cochlea, or related nerve pathways cause SSHL. A loss of 30 decibels or more in three consecutive frequencies in one or both ears within several days is considered SSHL. Immediate treatment can make partial or total recovery more likely. Unfortunately, many medical professionals— from emergency room to waiting room—don’t recognize SSHL or know how to treat it. As a result, many patients lose the opportunity for recovery because they don’t get the right treatment within the critical time. In my case, I received treatment within a week—it wasn’t optimal, but better than many. I had no significant recovery.  

SSHL Is LOUD, Isolating, and Devastating

The shocking thing to SSHL patients is how LOUD everything becomes. Sounds distort and blend together, sound direction is lost, and every sound seems magnified. The tinnitus is sudden and loud, with whooshing, popping, and other sounds. The tinnitus often worsens with activity or background noise, and goes long into the night. Some people don’t feel well, see well, sleep well, or balance well. SSHL strains relationships and many people simply stop participating in activities. I felt as if I had lost my life.  

SSHL Can Be Life-Threatening

I consoled myself at first that my condition wasn’t life-threatening. Within weeks, however, I no longer wanted to go on living. I later learned that many people respond this way. Physicians recognize the psychological impact when someone loses a limb. Losing the sense of hearing, suddenly, is not dissimilar. I didn’t know how to live with SSHL, or where to turn for support. I felt abandoned until I received the mental health support I urgently needed.

Alone at the Table

I have slowly reclaimed my life through the support of family, friends, and several Facebook groups. I also have used a cognitive therapy course for tinnitus, antidepressants (briefly), and months of working through the process. But there are still moments. My passion is recreational poker. I recently played with a mixed group, some with typical hearing, some with hearing loss. The hearing people were talking, but the background noise kept me from understanding them, and I don’t know ASL. I felt alone at the table—caught somewhere between the hearing world and the deaf world.

What Do We, as SSHL Patients, Want?

We want non-ENT medical professionals to learn about SSHL and treat it as a medical emergency. We want ENT doctors to recognize the psychological aspects of SSHL and refer us to appropriate resources. We want hearing loss advocates to see that SSHL has unique challenges different from other kinds of hearing loss. Lastly, we want a cure.

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Accomplishments by ERG Alumni

By Elizabeth Crofts

Progress Investigating Potential Causes and Treatments of Ménière’s Disease

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Gail Ishiyama, M.D., a clinician-scientist who is a neurology associate professor at UCLA’s David Geffen School of Medicine, has been investigating balance disorders for nearly two decades and recently coauthored two studies on the topic. While not directly funded by HHF, Ishiyama is a 2016 Emerging Research Grants recipient and also received a Ménière’s Disease Grant in 2017.

Ishiyama and colleague’s December 2018 paper in the journal Brain Research investigated oxidative stress, which plays a large role in several inner ear diseases as well as in aging. Oxidative stress is an imbalance between the production of free radicals and antioxidant defenses. The gene responsible for reducing oxidative stress throughout the body is called nuclear factor (erythroid-derived 2)-like 2, or NRF2. Ishiyama’s study looked at the localization of NRF2 in the proteins in the cells of the human cochlea and vestibule. It was found that NRF2-immunoreactivity (IR) was localized in the organ of Corti of the cochlea. Additionally, it was observed that NRF2-IR decreases significantly in the cochlea of older individuals. The team postulates for future studies that modulation of NRF2 expression may protect from hearing loss that results from exposure to noise and ototoxic drugs.

In a January 2018 report in the journal Otology & Neurotology, Ishiyama and team researched endolymphatic hydrops (EH), a ballooning of the endolymphatic fluid system in the inner ear that is associated with Ménière’s disease. Symptoms include fluctuating hearing loss, as well as vertigo, tinnitus, and pressure in the ear.

For the study, patients with EH and vestibular schwannoma were tested to evaluate the clinical outcome of patients when EH is treated medically. Vestibular schwannoma, also known as acoustic neuroma, are benign tumors that grow in the vestibular system of the inner ear, which controls balance. Often when patients develop episodic vertigo spells and have a known diagnosis of vestibular schwannoma, surgeons recommend surgical intervention, as they attribute the symptoms to the vestibular schwannoma. However, a noninvasive treatment may hold promise. Through the use of high-resolution MRI scans, the researchers found that when EH coexists with vestibular schwannoma in a patient, and the patient also experiences vertigo spells, a medical treatment for EH—that is, the use of diuretics to relieve inner ear fluid buildup—may alleviate the vestibular symptoms.

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A 2016 ERG scientist funded by The Estate of Howard F. Schum, Gail Ishiyama, M.D., is an associate professor of neurology at UCLA’s David Geffen School of Medicine. She also received a 2017 Ménière’s Disease Grant.


New Insights Into Aging 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, and creates 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.

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., Jayne B. Ahlstrom, M.S., and Judy R. Dubno, Ph.D., investigated these three components of perceptual organization to determine the extent to which their declines may be the source of increased difficulty in speech recognition with age. Younger and older adults with typical hearing listened to sentences interrupted with either silence or envelope-modulated noise, presented in quiet or with a competing talker.

As expected, older adults performed more poorly than younger adults across all speech conditions. The interaction between age and the duration of glimpses indicated that, compared with younger adults, older adults were less able to make efficient use of limited speech information to recognize keywords. There was an apparent decline in glimpsing, where interruptions in speech had a larger effect on the older adult group.

Older adults saw a greater improvement in speech recognition when envelope modulations were partially restored, leading to better continuity. This demonstrated that with age comes a poorer ability to resolve temporal distortions in the envelope. In speech segregation, the decline in performance with a competing talker was expected to be greater for older adults than younger adults, but this was not supported by the data.

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A 2015 Emerging Research Grants scientist, Kenneth I. Vaden, Jr., Ph.D., is a research assistant professor in the department of otolaryngology–head and neck surgery at the Medical University of South Carolina.

A 1986–88 ERG scientist, Judy R. Dubno, Ph.D., is a member of HHF’s Board of Directors. The study’s lead author, William Bologna, Au.D., Ph.D., is a postdoctoral research fellow at the National Center for Rehabilitative Auditory Research in Portland, Oregon.

A 2018 HHF intern, Author Elizabeth Crofts is a junior at Boston University studying biomedical engineering. For our continually updated list of published papers by ERG alumni, see hhf.org/erg-alumni.

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The Bridge Between Two Worlds

By Vicky Chan

Disability rights attorney Jared Allebest was born with a bilateral profound hearing loss. He was diagnosed at age 1 and fitted for hearing aids a year later. Today, Jared uses both hearing aids and ASL to communicate.

The son of a lawyer, Jared was determined to follow in his father’s footsteps and his hearing loss never deterred him. Throughout his education, he remained inspired by his favorite elementary school teacher, Ms. Marquardt, who taught him one of the most invaluable lessons: Having a hearing loss isn’t a barrier to success. “[Hearing loss] has affected my outlook to fight harder and to push myself to accomplish the things that I want to do in my life,” Jared explains.

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After his graduation from Thomas Jefferson School of Law in 2009, Jared founded a law firm that advocates for people with hearing loss and speaking disabilities. The firm focuses on empowering their clients through education, advocacy, and lobbying. He works with clients with both typical hearing and hearing loss and takes on cases relating to disability rights or discrimination, as well as employment, marriage/divorce, and criminal law.

Jared admits that he faces auditory challenges in his profession. During trials, he has to be exceptionally attentive to all parties. He also receives assistance from an ASL interpreter in the courtroom so he doesn’t miss anything being said.

Despite some difficulty, Jared believes that his hearing loss is an advantage. His clients are more comfortable with him because they know he can empathize with them. People listen carefully when he speaks about issues concerning hearing loss. “By fighting for the rights of those who live with hearing loss, I am advocating for myself as well. I think of myself as the bridge between two worlds,” Jared says.

Jared’s strong reputation as a dedicated lawyer stems from his sincerity and passion for helping others with legal issues that are deeply personal to him. The most rewarding part of his profession is knowing that his clients are satisfied with his commitment.

Jared’s advocacy for the hearing loss community outside goes beyond the courtroom. He is the former chairman of Loop Utah—an advocacy group dedicated to educating people in Utah about the benefits of loop technology. He currently serves as a community representative on the Advisory Council to the Utah Division of Services to the Deaf and Hard of Hearing (USDB Advisory Council).

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Of course, Jared knows he can’t be an advocate for all people with hearing loss, as much as he would like to be. He can’t be the connection between the legal world and the hearing loss world for everyone. Jared maintains that the most important part of living with hearing loss is effective self-advocacy. “Being assertive about your needs will help you to hear better, be more productive, and be happier.”

Jared lives and practices law in Utah. He is a participant in HHF’s Faces of Hearing Loss campaign.

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Eight Pairs of Earplugs in Four Noisy Settings: My Hearing Protection Experiment

By Kayleen Ring

Before my 2018 summer internship at Hearing Health Foundation (HHF) in New York City, I underestimated the importance of protecting my ears, often leaving myself at risk for damage from noise at concerts, sporting events, and other loud places. I took my typical hearing for granted until learning that hearing loss is largely caused by noise exposure and can negatively impact the brain function of young adults, even in its mildest forms. But I was also encouraged to discover noise-induced hearing loss (NIHL) is preventable. Earplugs in particular are a convenient, low-cost tool for hearing preservation.

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To improve my own hearing health and to create awareness about NIHL, I experimented with different types of earplugs in various loud settings. Expecting no more than a handful of foam options, I was excited to learn what an assortment of earplugs is available—each with different shapes, sizes, and features. Previously, my earplug experience had been limited to basic foam pairs to drown out my college roommates’ snoring!

I evaluated each personal earplug use experience with a 1 to 10 rating—10 being highest—for effectiveness, comfort, and ease of use. The Noise Reduction Rating (NRR) metric indicates how much noise is blocked out by the pair of earplugs.

Setting: Concerts

Just one loud concert (decibel levels up to 120 dB) can cause permanent damage to your ears. I tested earplugs at two musical events.

1. Eargasm High Fidelity Ear Plugs

  • NRR: 16 dB

  • Effectiveness: 10

  • Comfort: 10

  • Ease of Use: 10

At first, I worried wearing earplugs at a performance by one of my favorite artists would negatively affect my concert experience, but this pair allowed me to hear and enjoy the music perfectly at a reduced volume. They were so comfortable I forgot they were in my ears! They were easy to remove using the pull tab and I  also liked the carrying case they come in, because it fits in my small bag and keeps the earplugs hygienic for reuse.

2. Moldex Pocket Pak Squeeze

  • NRR: 27 dB

  • Effectiveness: 8

  • Comfort: 9

  • Ease of Use: 9

The triple-flange design, neck cord, and carrying case provided a secure earplug experience at an even louder concert where sound levels spiked to 120 dB. Unprotected exposure to noise at this level, which is equivalent to that of ambulance sirens or thunderclaps, can damage hearing in seconds. Fortunately, the ridged edges on the earplugs I used made inserting them far easier and faster than foam earplugs that need to be shaped prior to use.

Setting: Group Fitness

At a popular group fitness class, I recorded sound decibel levels and the results showed extremely loud and dangerous levels of noise. The average was 91 dB and the max was 119 dB over the one-hour class period. For a healthier workout, I wore earplugs.

3. Mack’s Blackout Foam Earplugs

  • NRR: 32 dB

  • Effectiveness: 9

  • Comfort: 10

  • Ease of Use: 9

These were excellent because I was able to hear the music and the trainers’ instructions, just at a lower volume. Less distracted by the loud music than usual, I was able to focus more carefully on my workout and form. They fit snugly and stayed in place over the course of the 60-minute, high-intensity session.

4. EarPeace “HD” High Fidelity Earplugs

  • NRR: 19 dB

  • Effectiveness: 10

  • Comfort: 10

  • Ease of Use: 8

I was particularly impressed that this pair included three set of filters offering different levels of protection. I used the highest decibel filter, 19 dB, and found the class music was still clear and enjoyable. My only challenge was properly inserting the very small filters.

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Setting: Restaurants

When I didn’t intern at HHF this summer, I worked at a restaurant on Long Island, New York, that was always busy, sometimes bursting with chatty customers waiting three hours for service. Beyond the crowds, the restaurant had live musical performances that amplified an already loud environment. This is dangerous for workers and patrons alike. Here, the earplugs I wore still allowed me to hear clearly and hold a conversation.

5. Etymotic ER20XS High-Fidelity Earplugs (NRR: 13 dB)

  • NRR: 13 dB

  • Effectiveness: 8

  • Comfort: 8

  • Ease of Use: 9

The Etymotic earplugs had the positive qualities of the typical high-fidelity earplugs and included three interchangeable eartips, a hygenic carrying case, and a neck cord, providing a secure and effective earplug experience.

6. EarPeace “S”High Fidelity Earplugs

  • NRR: 19 dB

  • Overall Effectiveness: 10

  • Comfort: 10

  • Ease of Use: 10

This pair was great. They reduced the noise perfectly so it was at a comfortable yet still audible volume. The dual-flange design and soft silicone material made the earplugs fit well, were comfortable and easy to use.

Setting: New York City Subway

Decibel levels on the subway platforms trains are extremely high and can cause hearing damage, especially for frequent riders and employees. For my tests, I sat inside the 34th St-Penn Station 1/2/3 subway station across the street from the HHF office, where I was greeted by screeching trains, talkative tourists, and a steel drums player.

7. Moldex Sparkplugs

  • NRR: 33 dB

  • Overall Effectiveness: 9

  • Comfort: 10

  • Ease of Use: 9

The Sparkplugs blocked out noise while allowing me to hear conversations and train announcements. They were easy to mold into my ears, allowing for optimal noise reduction. The pattern on the earplugs is colorful and fun, making them appealing for children, and easily locatable in your bag.

8. Alpine Plug & Go

  • NRR: 30 dB

  • Overall Effectiveness: 8

  • Comfort: 8

  • Ease of Use: 8

These foam earplugs reduced volume but the noise was muffled. Consequently, these would be a great option for more sedentary activities, like sleeping and flying, where you are aiming to block out all noise. The foam was comfortable and fit snugly in my ears, but was challenging to mold.

The reviews and ratings here are based on my individual experiences and are not intended to encourage or discourage anyone’s use of specific earplugs. High ratings are not product endorsements. As someone newly informed about the dangers of noise, it is my hope my summer intern experiment for HHF will raise awareness and inspire others to investigate hearing protection that best meets their needs.

Kayleen Ring is a former marketing and communications intern at HHF. She studies marketing in the honors program at Providence College in Rhode Island.

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