Get Moving to Preserve Your Hearing

By Yvonnie Phan

As 2018 begins, many Americans, motivated to improve their physical and mental wellbeing, have already made the popular New Year’s Resolution to exercise more frequently. This commitment has an additional, lesser-known benefit; exercise is proven to preserve hearing health. Engaging in physical activity with proper safety precautions can delay or prevent age-related hearing loss, or presbycusis, which affects a quarter of adults 65-74 and half of those older than 75.  

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Cardiovascular exercise is vital to hearing health as one ages. A person over 50 years old without a genetic predisposition to hearing loss and who engages in cardio for 20-30 minutes five times weekly is more likely to maintain a healthy auditory system than someone with low cardiovascular activity. In a decade-long Miami University study of 1000 subjects of all ages, those over 50 with moderate-to-high cardiovascular fitness levels maintained hearing sensitivity comparable to people in their 30s, effectively delaying presbycusis.

An additional investigation from the University of Florida affirms that routine cardio provides the necessary blood flow, oxygen, and nutrients to maintain the health of important auditory systems within the cochlea. Lead author Shinichi Someya, Ph.D. explains that  “the cochlea, or inner ear, is a high-energy demanding organ.”

Stretching and yoga are healthy alternatives to cardiovascular exercise. These activities facilitate proper blood flow throughout the body and activate the muscles. While stretching or performing yoga poses, it’s important to focus on breathing to increase oxygen and blood flow. There are even yoga poses designed specifically for those with tinnitus.

The hearing health benefits of exercise can be negated by noise exposure or improper ear care, however.

Listening to audio through headphones at a loud volume can increase one’s chances of Music-Induced Hearing Loss (MIHL), as can the music played during exercise classes. Turning down the volume on your device, wearing earplugs, and giving ears time to recover from loud noises can help prevent damage to the auditory system.

Those who swim are encouraged to keep their ears dry. Moisture in the ear allows for bacteria, or even fungi and viruses, to attack the ear canal, which can lead to Swimmer’s Ear and cause temporary hearing loss. Dry ears immediately and do not insert anything, such as cotton swabs, into them.

Health professionals strongly recommend everyone incorporate exercise into their daily routine. There are many benefits in maintaining a consistent exercise regimen and we can now add hearing loss prevention to the list. Before starting a new fitness routine, consult your physician to assure the routine is safe and suitable for your health.

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HHF Partner Hyperacusis Research Shares 14-Year-Old’s Heartbreaking Story to Fight Noise Intolerance

Photo Credit: Hyperacusis Research

Photo Credit: Hyperacusis Research

By Lauren McGrath

Hearing Health Foundation (HHF) Emerging Research Grants (ERG) grant funder Hyperacusis Research—a nonprofit dedicated to developing effective treatments for hyperacusis and to funding research that will eliminate the underlying mechanisms that cause hyperacusis—has a new reason to fight to cure the noise intolerance disorder.

Cindy, 14 years old, has suffered from hyperacusis since she was blasted in the face with an airhorn one year ago. The blast almost immediately prompted “a burst of pain in [her] ear” that made it “feel like someone was stabbing [her].” Six months and several doctors’ visits later, an occupational therapist recognized her symptoms and diagnosed her with the disorder, which causes Cindy to experience pain at low levels of sound relative to what a person with typical hearing can withstand.

Once a happy and social eighth-grader, Cindy now rarely leaves her home. Secluded from the painful sounds of the outside world, her house has become “her sanctuary,” her mother explains. Her intolerance of everyday noises like the school cafeteria and teachers’ voices has forced her to leave public school in exchange for an isolating homeschool experience. “The thing I hate most is that I can’t see friends,” Cindy shares.

Cindy suffers from one of four hyperacusis subtypes called pain hyperacusis. The other three types, according to Hyperacusis Research, are loudness hyperacusis (which causes moderately intense sounds to be perceived as very loud), annoyance hyperacusis (which causes negative emotional reactions to sounds), and fear hyperacusis (which prompts an aversive response to sounds that causes anticipatory response and avoidance behavior). Specific medical treatments, at the moment,  do not yet exist for pain hyperacusis.

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Those inspired to help Cindy can donate to Hyperacusis Research to advance the ontological knowledge of hyperacusis through research grants, including those awarded to HHF’s ERG investigators.

Since 2015, Hyperacusis Research has generously funded grants for a total of five ERG investigators focused on hyperacusis at the University at Buffalo, Oregon Health and Science University, and Massachusetts Eye and Ear Infirmary. You can learn more about our ERG researchers’ efforts to better understand the mechanisms, causes, diagnosis, and treatments of hyperacusis and severe forms of loudness intolerance here.

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Prenatal Intervention May Be Necessary for Usher Syndrome Treatment

By Carol Stoll

Usher syndrome is a hereditary disorder that affects 1 in 20,000 people worldwide and causes concurrent hearing and vision loss. Though currently there is no cure, scientists have begun to understand the molecular mechanisms of hearing loss in Usher syndrome by identifying the specific mutations in genes associated with auditory hair cell malfunction. Gene-specific targeting has been used to target Usher mutations and restore hearing, but the effectiveness and best timing of the treatment is still being investigated in mouse models. Recent research published in JARO by Emerging Research Grants (ERG) recipient Michelle Hastings, Ph.D., and colleagues shows that early administration of a genetic targeting treatment is critically important for repairing outer hair cells and thus rescuing hearing in those with genetic disorders like Usher syndrome.

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Hastings’ research focuses on type 1 Usher, which is the most severe of three subtypes and is associated with six genes. One of these genes, USH1C, contains the instructions to create a protein that localizes to auditory hair cells and helps to maintain their bundle structure and ability to detect sound waves. A mutation in USH1C causes this protein to be cut short and malfunction, and is thus responsible for type 1C Usher in humans. Adding, or “knocking-in,” the mutation to mouse DNA causes symptoms similar to those of human patients with type 1C Usher. These Usher mice exhibit hearing and vision loss as well as deficits in balance, little or no auditory-evoked brainstem response (ABR), and abnormal eye tests called electroretinograms. The hearing loss is linked to defective or missing inner and outer hair cells in the cochlea of the inner ear.

Antisense oligonucleotide (ASO) therapy is a gene-specific targeting therapy previously used by Hastings and her colleagues to rescue hearing in Usher knock-in mice. ASOs are small strands of nucleotides (the building blocks of DNA and RNA) that are specifically synthesized to bind to the disease-causing mutation site of RNA and block it from creating defective RNA and proteins. The ASO therapy targeting the USH1C mutation was administered to the Usher mice a few days after birth. Hearing was rescued and ABR improved, which is indicative of improved inner hair cell function. However, function of the outer hair cells, which surround the inner hair cells and are responsible for amplifying sounds, was not tested.

Hastings’ most recent study, with Jennifer Lentz, Ph.D.’s research group, investigated whether the timing of ASO treatment is important for rescuing outer hair cells in addition to inner hair cells for full hearing rescue. ASO therapy was administered to knock-in Usher mice of varying ages, and then outer hair cell function was tested by measuring distortion product otoacoustic emissions (DPOAEs) in 1-, 3-, and 6-month-old mice. When two tones are presented in the ear canal, outer hair cells that function normally respond by producing amplified sounds known as DPOAEs. In Usher mice, DPOAEs are not detected, which indicates loss of outer hair cell function. ASO treatment was able to recover outer hair cell function measured by DPOAEs when it was administered one day after birth. However, the treatment was not effective if first administered on or after postnatal day five.

The results of this study indicate that there is a developmental window of time when USH1 gene expression is needed to properly develop auditory hair cells, and thus early genetic treatment is essential for hearing rescue of those with Usher syndrome. In humans, hair cell development occurs early in pregnancy, and thus ASO treatment would likely require very early prenatal intervention. ASOs have been approved for clinical use for a number of different diseases in humans, but more animal research is necessary before moving to clinical trials for ASO therapy for Usher syndrome. Hastings has also published research on testing ASO therapy on prenatal mice, and found that injecting ASOs in the amniotic cavity of pregnant mice can in fact access the cochlea. Hastings’ research has improved the scientific community’s understanding of the functions of inner and outer hair cells and brings us closer to developing a cure for Usher syndrome.

Michelle Hastings, Ph.D., was a 2009 and 2011 Emerging Research Grants scientist. For more, see “Rescue of Outer Hair Cells With Antisense Oligonucleotides in Usher Mice Is Dependent on Age of Treatment” in The Journal of the Association for Research in Otolaryngology.

WE NEED YOUR HELP IN FUNDING THE EXCITING WORK OF HEARING AND BALANCE SCIENTISTS. DONATE TODAY TO HEARING HEALTH FOUNDATION AND SUPPORT GROUNDBREAKING RESEARCH: HHF.ORG/DONATE.

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Untreated Hearing Loss Put Overall Health at Risk

Hearing Health Foundation (HHF) CEO Nadine Dehgan’s “Treating Hearing Health for Better Overall Health” was published online to My Prime Time News following its original print appearance in The American Legion’s December 2017 issue.

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The article details how the state of the inner ear impacts other critical functions, like the heart and the brain. Cited are the various conditions that can arise as a result of untreated hearing loss, including cardiovascular disease, dementia, diabetes,  depression, and falls. When the auditory system is functioning well, however, the risk for these ailments declines.

Additionally, hearing loss is also linked to other medical conditions and drugs. People with anemia are twice as likely to have hearing loss. According to Peter Steyger, Ph.D., a scientific adviser to HHF. Further, certain cancer-fighting chemotherapy drugs, such as cisplatin, may permanently harm hearing.

While the relationship between hearing health and overall health is always significant, the publicity of “Treating Hearing Health for Better Overall Health” is an especially timely and helpful follow-up to ERG recipient Harrison Lin, M.D.’s new findings concerning the gaps between self-reported hearing loss and patients evaluation and treatments for hearing loss, which appeared in this month’s issue of JAMA Otolaryngology—Head & Neck Surgery.

Individuals who believe they may have a hearing loss are encouraged to consult an audiologist or ENT, and can learn more about the relationship between hearing health and overall health in the full article on My Prime Time News.

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New Method Enables Systematic Study of Hair Cell Loss and Regeneration in Chickens

By Carol Stoll

Most forms of hearing loss are permanent because damage to inner ear sensory hair cells is irreversible in mammals, including humans. Mammalian vestibular hair cells have the potential to regenerate albeit at a low rate, but the hair cells of the adult mammalian cochlea are not regenerated. Birds, however, have a robust regenerative response to hair cell damage and are able to restore structure and function in inner ear organs. Consequently, the study of the molecular mechanisms that trigger the onset of avian hair cell regeneration in the balance organs as well as in the cochlea is important and may lead to therapies for hearing loss in humans.

This image shows the undamaged and damaged utricle, an inner ear balance organ, in a chicken. HRP researchers have devised a new method to study the precise timing of hair cell regeneration in chickens using a single surgical application of an ototoxic drug. Photo by Amanda Janesick, Ph.D.

This image shows the undamaged and damaged utricle, an inner ear balance organ, in a chicken. HRP researchers have devised a new method to study the precise timing of hair cell regeneration in chickens using a single surgical application of an ototoxic drug. Photo by Amanda Janesick, Ph.D.

Past experiments that investigate these regeneration mechanisms in living chickens required multiple injections of a drug to induce hair cell loss, making it difficult to determine the exact timing of the regeneration response. A collaboration of two Hearing Restoration Project researchers, Stefan Heller, Ph.D. and Jennifer Stone, Ph.D., and two talented postdoctoral fellows from their laboratories was recently published in Journal of the Association for Research in Otolaryngology identifying a potential solution to this problem. They developed an experimental framework that uses a single ototoxic drug application, enabling them to study the precise onset and timing of hair cell regeneration in vivo.

Heller, Stone, and colleagues performed their experiments on a total of 75 chickens. At seven days of age, the chickens were anesthetized and underwent surgery to eliminate hair cells in the inner ear organs. During the surgery, streptomycin (an ototoxic antibiotic) was delivered to the chicken’s inner ear. At various time points after the surgery, two sensory organs—the utricle, a vestibular organ; and the basilar papilla, the hearing organ—were dissected, labeled for various cellular markers, and analyzed under a microscope. Hair cells and their surrounding supporting cells were counted and observed for damage. EdU, a marker of cell division, was administered to the chickens to determine whether or not new hair cells were generated by cell division. These techniques enabled the researchers to quantitatively characterize the regenerative response of the utricle after damage.

The results of the study demonstrate that surgical application of a single streptomycin dose is a feasible approach to elicit hair cell loss and regeneration in the chicken utricle and basilar papilla. Just hours after streptomycin delivery, hair cell numbers significantly declined and DNA replication was activated. The team was then able to record specific events of the regeneration process, which get initiated around 12 hours after streptomycin-induced hair cell loss, and continue over the course of several days.

Supporting cells produce new hair cells either by converting into a hair cell (direct transdifferentiation), or by dividing, usually asymmetrically, into a supporting cell and a hair cell.  Throughout this regenerative response, supporting cell numbers and density in the utricle remain relatively constant, suggesting that there is a mechanism that responds to specific levels of damage and coordinates the individual events of the regeneration process.

The study establishes a framework for the refined study of the two modes of hair cell regeneration in the chicken utricle. The next steps of the work will focus on understanding the exact timing and mechanism of coordination of the regeneration response. With only a single application of streptomycin necessary to induce near-complete hair cell loss in hearing and balance organs, the new animal model allows for study of the entire process including initiation, realization, and termination. The fundamental understanding of the avian regenerative mechanisms may lead to future development of therapies for loss of hearing and balance in humans.

WE NEED YOUR HELP IN FUNDING THE EXCITING WORK OF HEARING AND BALANCE SCIENTISTS. DONATE TODAY TO HEARING HEALTH FOUNDATION AND SUPPORT GROUNDBREAKING RESEARCH: HHF.ORG/DONATE.

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2018: Hear's to You

By Nadine Dehgan

From every one of us at Hearing Health Foundation (HHF)—scientists, staff, and volunteers—thank you for your support in 2017 and best wishes for 2018.

Because of folks like you, 2017 was an incredible year in which HHF:

  • Increased funding for Hearing Restoration Project & Emerging Research Grants by 35%
  • Launched Ménière's Disease Grants program to better understand & treat this condition
  • Began to fund critical Ototoxic Drug Research so cancer survivors won't have to live with hearing loss as a result of their life-saving treatments
  • Advocated for universal newborn hearing screenings, resulting in the passage of the Early Hearing Detection and Intervention Act
  • Endorsed the Over-the-Counter Hearing Aid Act, which will provide a more affordable and accessible treatment option for adults with mild to moderate hearing loss
  • Created Faces of Hearing Loss to show that hearing and balance disorders affect all of us
  • Received Top Ratings from all Charity Watchdogs including Consumer Reports and BBB

With hearts full of gratitude, we look forward to the work to be done in 2018—HHF’s 60th anniversary year.

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With your help, HHF will continue to fund groundbreaking discoveries for the tens of millions of Americans with hearing loss and tinnitus—among whom is Ethan, 6, born with bilateral (in both ears) hearing loss and fortunate to receive early intervention.

Ethan is a first-grader who loves his sisters, soccer, reading, math, and martial arts. Until a cure for hearing loss is realized, he will be dependent on hearing aids or other treatments.

New scientific findings in 2018—empowered by you—can change the future of hearing loss for Ethan and so many others. I look forward to updating you on progress made.

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You’re Invited: Comprehensive Hearing Health Experience in February 2018

By Lauren McGrath

In honor of our 60th anniversary, Hearing Health Foundation (HHF)’s Board Chair, Elizabeth Keithley, Ph.D., is hosting Hearing360—an educational and social forum at the San Diego Central Library—in early 2018. Hearing360 seeks to engage HHF’s San Diego area constituents with the latest updates in hearing research and everyday guidance for hearing health.

Featured event speakers include scientists Ronna Hertzano, M.D. and Andy Groves, Ph.D. from HHF’s Hearing Restoration Project (HRP), the world’s first international consortium dedicated to identifying a permanent biological cure for hearing loss. Their individual presentations on the regeneration of hair cells in the inner ear will be followed by a brief Q&A session.

Ronna Hertzano, M.D.

Ronna Hertzano, M.D.

Andy Groves, Ph.D.

Andy Groves, Ph.D.

Hearing360 will also honor the generosity of longtime HHF supporters Frank and Chris De Francesco, who shared their experiences raising a child with profound hearing loss in “Why We Believe in Hearing Health Foundation,” which appeared in the Fall 2017 issue of Hearing Health magazine.

Scheduled for Sunday, February 11 from 3:00 - 5:00 PM in the San Diego Central Library’s Shiley Special Event Suite, Hearing360 will be fully accessible with a t-coil loop system and open captioning. Light refreshments and wine will be available.

Limited space is available and an RSVP is required for all guests. We look forward to meeting you in San Diego. Please contact HHF at events@hhf.org with any questions regarding Hearing360.

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New Grantees Will Advance Understanding of Ménière's Disease

By Lauren McGrath

Hearing Health Foundation's (HHF) newly established Ménière's Disease Grants (MDG) program will significantly advance our understanding of the mechanisms of Ménière's Disease. In 2018, two innovators will have the opportunity to investigate the disorder's diagnosis and treatment.

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Ménière's Disease is a disorder of the inner ear that causes episodes of vertigo as a result of fluid that fills the tubes of the inner ear. In addition to dizziness and nausea, Ménière's attacks can cause some loss of hearing in one or both ears and a constant ringing sound. The causes of Ménière's remain unknown and a cure has yet to be identified. The National Institutes of Health estimates that 615,000 individuals in the United States live with the disorder.

Two grants have recently been awarded for 2018 for innovative Ménière's Disease research. Both grantees were also previously funded by HHF’s Emerging Research Grants (ERG) program.

Gail Ishiyama, M.D.

Gail Ishiyama, M.D.

Gail Ishiyama, M.D. of UCLA David Geffen School of Medicine is focusing on cellular and molecular biology of the microvasculature in the macula utricle of patients diagnosed with Ménière’s disease. Her project will provide greater information on the blood labyrinthine barrier and allow for the development of interventions that prevent the progression of hearing loss and stop the disabling vertigo in Ménière’s disease patients.

Ian Swinburne, Ph.D.

Ian Swinburne, Ph.D.

Ian Swinburne, Ph.D. of Harvard Medical School is classifying the endolymphatic duct and sac's cell types and their gene sets using high-throughput single-cell transcriptomics. His work will generate a list of endolymphatic sac cell types and the genes governing their function, which will aid in Ménière's diagnosis (which can be delayed due to the range of fluctuating symptoms) and the development of a targeted drug or gene therapy.

HHF is grateful for the opportunity to fund Drs. Ishiyama and Swinburne. If you are interested in naming a research grant in any discipline within the hearing and balance space, please contact development@hhf.org.

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You Can Change Lives

By Nadine Dehgan

On behalf of folks like John—Thank you for your continued support of Hearing Health Foundation (HHF), the largest U.S. nonprofit funder of hearing loss and tinnitus research in America.

We are dedicated to discovering better treatments and permanent cures.

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John—a Retired U.S. Army Colonel—served during the Cold War. Constant exposure to gunfire and high-pitched helicopter engines took a toll on his hearing. He was diagnosed with tinnitus and hearing loss in 1996. His diagnosis fueled his desire to improve the lives of active duty personnel and veterans since.

Tragically, John's circumstances are not unique. Tinnitus is the most common physical ailment for returning military personnel, followed by hearing loss. 60% of Iraq and Afghanistan veterans suffer from one or both of these conditions.

Many Veterans, even those who pass their hearing test, have trouble understanding speech. This condition, known as auditory processing disorder, is often caused by blast exposure.

Today John teaches at the Naval Postgraduate school where he often counsels young military officers as they cope with their tinnitus, as tinnitus can cause significant sleep, concentration, and mood issues.

Can you help bring us closer to better treatments and cures for tinnitus for John and the 65 million other Americans with tinnitus, many of whom are also veterans? 

PLEASE, IF YOU ARE ABLE, GIVE TO HHF TODAY. 100% OF YOUR GENEROUS GIFT WILL BE DIRECTED TO THE AREA OF YOUR DESIGNATION. 

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D.J. Demers: Hearing Loss Awareness Through Comedy

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By Carol Stoll

After his first audition on America’s Got Talent, D.J. Demers was described as funny, original, and likable by the tough, at times brutally honest judge Simon Cowell. Demers performed a stand-up routine that included witty banter with the judges and had the audience in stitches. Demers is now an award-winning stand-up comedian and has appeared multiple times on the late-night talk show Conan. He also happens to have profound hearing loss.

As an infant, D.J. had many ear infections and when his parents noticed that he wasn’t hearing well, they took him to an audiologist. He was diagnosed with severe-profound bilateral sensorineural hearing loss and started wearing hearing aids in both ears at the age of four. Not wanting to be defined by his disability, D.J. turned to making others laugh. He dreamed of being a comedian since childhood, and finally gave stand-up a shot when he was 21. “I was instantly hooked. Best decision I ever made,” he explained.

D.J. Demers is not your typical cynical comedian; he always has a positive outlook and makes light of heavy situations. His goal is to make his audience feel “free from concern,” as put by his comedian idol Jim Carrey. Demers likes to frequently interact with the audience, but sometimes has difficulty hearing them from the stage. Instead of getting discouraged, he simply makes it a part of the show. “It’s never a negative experience because I never make it one,” Demers said. “An audience follows your lead and my aim is to always keep it positive.”

Demers has also found a perfect middle ground with regards to discussing his hearing loss during his comedy shows. His jokes cover a variety of everyday topics, but he doesn't shy away from proudly calling out his hearing aids and describing the unique, humorous situations that he faces because of his hearing loss. For example, he points out the quick transition between pillow talk with his girlfriend and silence when he takes out his hearing aids to go to sleep. He asks her for a “last call” on final thoughts for the day and then goes to sleep in silence. He brags about sleeping like a baby with no distracting noises and says in a sarcastic deadpan voice, “It really makes you wonder...who has the disability?”  

D.J., now 31 and residing in Los Angeles, has been recognized for his comedic talent with multiple awards. He won the 2013 Toronto Comedy Brawl and the 2014 Homegrown Comics Competition at the Just For Laughs comedy festival in Montreal. He was named “Best Breakout Artist” at the 2015 Canadian Comedy Awards. He appeared on season 11 of America’s Got Talent in 2016, and he has performed a stand-up routine twice on Conan, in 2014 and 2017.

This past October and November, Demers performed across the country on the Here to Hear Comedy Tour. The tour, sponsored by Phonak, aimed to “shatter stigmas and raise awareness about hearing loss through the power of laughter.” Demers was grateful for the opportunity to connect with hard-of-hearing people and valued all of the conversations he had with those he met along the way.

Demer’s pointers for those who are just getting a hearing loss diagnosis include: figure out and implement the necessary tools needed to help you in your day-to-day life, surround yourself with positive people, and don’t be afraid to advocate for yourself. For those with hearing loss who are passionate about comedy or any type of performing arts, Demer advises, “Just do it. That’s it. If it excites you, do it. Don’t impose any fictional limits on yourself.”

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