Untreated Hearing Loss Puts 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.

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.

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.

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.

Empower the Hearing Restoration Project's life-changing research. If you are able, please make a contribution today.

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.

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.

Genome Editing Protects Hearing in Mice

Massachusetts Eye and Ear/Harvard Medical School associate professor Zheng-Yi Chen, D.Phil. (1995 ERG), and colleagues delivered a CRISPR/Cas9 genome editing complex directly into the inner ear hair cells of mice, preventing hearing loss in an animal model of genetic progressive deafness. The CRISPR/Cas9 therapy disabled a mutated form of the gene Tmc1, the first time that a gene editing protein has been ferried directly into the relevant cells to halt progression of genetic hearing loss.

A single-letter mutation in the gene Tmc1 and carrying only one of two copies of the mutated gene both lead to progressive hearing loss in mice and humans. With the mutated gene disabled, the inner ear hair cells survive, and mice otherwise genetically destined to become deaf retained a portion of their hearing. In a report published in Nature in December 2017, the team says that at four weeks, untreated mice were unresponsive to sound below an average of 80 decibels, while treated mice responded to sound at approximately 65 decibels. At eight weeks, treated mice also retained their instinctive physical “startle” response to sudden loud sound, while the untreated mice did not respond. The researchers said delivering the Cas9 protein itself locally, instead of DNA elements that the cell can use to build Cas9, improved the DNA specificity and potential safety of the treatment. —Massachusetts Eye and Ear

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.

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.

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.

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. 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. 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.

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.

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. 

D.J. Demers: Hearing Loss Awareness Through Comedy

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.”

Tuning In

By Laura Friedman

As my three-year work anniversary approaches, I’ve been reflecting on my learnings as a team member of Hearing Health Foundation (HHF). There is one that sticks out the most: As someone who has only known her life as being one with hearing loss and wearing hearing aids (I was diagnosed at age 3 ½), I don’t know what I’m missing, compared with my typical hearing peers. I have learned from those who have acquired hearing loss later in life, even as young adults, that they are acutely aware of the difference in their hearing experience even with assisted listening devices. They aren’t enjoying music like they once did, they have increased difficulties hearing conversational speech around the dinner table and at restaurants, and they are missing things in meetings that the “old” them would never miss.

Ok, I lied. There are actually two major learnings. The second is, which is something I’ve experienced within my own family, is the prevalence of hearing loss denial and the resistance to treating one’s hearing loss. For those who do acknowledge their hearing loss, many do not wear their hearing devices, further isolating them from a world they were previously a part of.

As a young child when I visited my grandfather, who was notorious for not wearing his hearing aids, I told him, “If I have to wear mine, you have to wear yours!” He would give me his signature smirk and appease his granddaughter. But I knew once I left, back in the drawers those hearing aids went.

Denying the existence of an ailment or resisting treatment is not unique to hearing loss; this is true for many diagnosed with other serious and life-changing conditions. However, when it comes to hearing loss, almost one-third of an estimated 40 million U.S. adults with hearing difficulties haven’t even taken the first step to see a specialist for help. What’s even more troubling is today it is estimated that 360 million people worldwide have hearing loss, with 1.1 billion people at risk for acquiring it, according to the World Health Organization.

In the U.S. nearly 25 percent of those ages 65 to 74 and half of those older than 75 have a disabling hearing loss. An even more astounding fact is that over 70% of adults in the U.S. who have hearing loss and who would benefit from a hearing aid don’t have one.

Studies show that untreated mild to moderate hearing loss is linked to increased levels of loneliness and isolation, depression, dementia, and medical conditions like heart disease and diabetes. It can even hurt earning potential and career mobility. Treating hearing loss can significantly offset and decrease the risk of acquiring these consequences. So the question is: If treating hearing loss deters the onset of detrimental health conditions, why aren’t more people taking preventative measures to protect their hearing or taking actionable steps to treat it?

For those in the U.S. there is unfortunately little to no insurance and Medicare coverage for hearing aids, meaning those who need them most are unable afford them. While there’s still a lot left to do, policy is slowly moving in the right direction: In August 2017 the Over-the-Counter Hearing Aid Act was signed into law, paving the way for a new class of hearing aids to enter the marketplace. This will provide greater access to hearing technologies for those with mild-to-moderate hearing loss at a fraction of the price.

I can’t imagine how frustrating it must be to know what you’re missing; it is my wish that nobody has to experience that feeling. This is why I chose to work for an organization dedicated to funding research to develop better technologies, therapies and cures for hearing and balance disorders. Discoveries are the only way to better the listening experience of those with hearing loss and bring more options to the market.

I ask you to do this one thing: Get your hearing tested and encourage your friends and loved ones to do the same. If necessary, treat your hearing loss. Treating hearing loss has been a life-changer for me, as well as millions of others around the world, who choose to tune in, ultimately benefiting our health, work, and life.

Laura Friedman is the Communications and Programs Manager of Hearing Health Foundation. Read her hearing loss story in the Spring 2016 issue of Hearing Health magazine.