Genome Editing Protects Hearing in Mice

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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

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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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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.

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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.

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Help Us Move Beyond Grateful

By Nadine Dehgan

Thank you for your partnership as we progress toward our dream of cures for hearing loss and tinnitus.

Our researchers are hard at work discovering how reptiles, birds, and fish are able to restore their hearing after being deafened so they can translate this knowledge into cures for mammals and humans. 

When better treatments and cures are discovered, I know Jamie—pictured below with her four children—will be incredibly grateful for the opportunity to have her hearing restored. We will all be grateful.

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Jamie's life changed one year ago when her daily activities were suddenly compromised. Words turned into mere muffled sounds—and then silence. She found herself increasingly dependent on lip-reading to avoid asking people to repeat themselves, a request that embarrassed her.

Her fears were confirmed when her doctor determined that Jamie, 32, has severe hearing loss in both of her ears. The doctor was astonished by the intensity of the decline in Jamie's hearing.

Jamie is fortunate to have a supportive and loving husband and family. But she lives in fear she may never be able to hear her beautiful children and other important sounds in her life.

Can you help bring us closer to better treatments and a cure for hearing loss for Jamie and 48 million other Americans with hearing loss?

Please, if you are able, give to HHF today. 100% of your generous gift will be directed to the area of your designation. 

Thank you and happy holidays!

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The Gap Between Self-Reported Hearing Loss and Treatment Patterns

By Carol Stoll

Hearing loss is one of the most prevalent chronic conditions in the U.S. and has been associated with negative physical, social, cognitive, economic, and emotional consequences. Despite the high prevalence of hearing loss, substantial gaps in the utilization of amplification options, including hearing aids and cochlear implants (CI), have been identified. Harrison Lin, M.D., a 2016 Emerging Research Grants recipient, along with colleagues, recently published a paper in JAMA Otolaryngology–Head & Neck Surgery that investigates the contemporary prevalence, characteristics, and patterns of specialty referral, evaluation, and treatment of hearing difficulty among adults in the U.S.

Unlike this man who is having his hearing tested, a large number of individuals in the U.S. who experience hearing difficulties are not seeking treatment. Photo source: Bundesinnung Hörakustiker, Flickr.

Unlike this man who is having his hearing tested, a large number of individuals in the U.S. who experience hearing difficulties are not seeking treatment. Photo source: Bundesinnung Hörakustiker, Flickr.

The researchers did a cross-sectional analysis of responses from a nationwide representative sample of adults who participated in the 2014 National Health Interview Survey and responded to hearing health questions. The data collected included demographic information as well as self-reported hearing status, functional hearing, laterality (hearing ability in each ear), onset, and primary cause (if known) of the hearing loss. In addition, the team analyzed specific data regarding hearing-related clinician visits, hearing tests, referrals to hearing specialist, and utilization of hearing aids and CIs.

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Overall, 36,690 records were included in the analysis, which extrapolated to an estimated 239.6 million adults in the U.S. Nearly 17 percent indicated their hearing was less than “excellent/good,” ranging from “a little trouble hearing” to “deaf.” Approximately 21 percent of respondents had visited a physician for hearing problems in the preceding five years. Of these, 33 percent were referred to an otolaryngologist and 27 percent were referred to an audiologist. Of the adults who indicated their hearing from “a little trouble hearing” to being “deaf,” 32 percent had never seen a clinician for hearing problems and 28 percent had never had their hearing tested.

The study shows that there are considerable gaps between self-reported hearing loss and patients receiving medical evaluation and recommended treatments for hearing loss. Increased awareness among clinicians regarding the burden of hearing loss, the importance of early detection and medically evaluating hearing loss, and available amplification and CI options can contribute to improved care for individuals with hearing difficulty. Future studies are warranted to further investigate the observed trends of this study.

Harrison W. Lin, M.D., is a 2016 Emerging Research Grants recipient. His grant was generously funded by funded by The Barbara Epstein Foundation, Inc.

We need your help supporting innovative hearing and balance science through our Emerging Research Grants program. Please make a contribution today.

 
 
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HHF Launches Faces of Hearing Loss Campaign

Think of someone you know who has hearing loss. Who do you see?

You envision a relative, but you are not thinking of your 4-year-old niece. A neighbor comes to mind, but not the 32-year-old who lives across the street.

This is a trick question. Hearing loss—and related conditions like tinnitus, Ménière's disease, and hyperacusis—can affect anyone, anywhere. Hearing loss is your 4-year-old niece, your 32-year-old neighbor, your colleague in her mid-20s. Hearing loss affects every age, race, ethnicity, and gender.

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No one is immune from developing a hearing and balance disorder—and hearing loss has no single face. To refute common misconceptions that it only affects older adults, HHF has collected images of individuals living with a hearing condition to capture the diversity of its impact across the country. These are HHF’s “Faces of Hearing Loss.”

Participants shared their picture, current age, state of residence, type of hearing condition, and the age at onset or diagnosis. Among the tens of millions of Americans with hearing loss are an 11-year-old boy in Oregon, an 80-year-old woman living in Washington, and a 47-year-old man in North Dakota. These individuals may never meet, but “Faces of Hearing Loss” connects them through their shared experiences.

If you or a loved one has hearing loss, please consider participating in “Faces of Hearing Loss” by completing this brief form, sending in picture, and answering a few basic questions. If you are the parent of a child under 18, you may sign a release form on their behalf.

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I Want You to Know About My Hearing Loss

By Lauren McGrath

Maureen “Marzi” Wilson is an author, illustrator, and self-described introvert. As one who goes about life with the tendency to speak less and listen more, she fittingly calls her latest collection of artwork Introvert Doodles.

Marzi does not have hearing loss. “As someone with typical hearing, I believe that I and others like me have a lot to learn from those with hearing loss. We need to pay attention to their experiences,” Marzi writes.

Marzi Wilson's "I'm Deaf/Hard of Hearing" gives individuals with hearing loss a way to voice their experience.

Marzi Wilson's "I'm Deaf/Hard of Hearing" gives individuals with hearing loss a way to voice their experience.

“I’m Deaf/Hard of Hearing” is Marzi’s latest relatable masterpiece about hearing loss in her “I Want You to Know” series. “I Want You to Know” is Marzi’s honest attempt to educate her viewers about hearing loss and other conditions that can be misunderstood or stigmatized. She knows that even the most well-meaning people have misconceptions about what does not affect them firsthand.

Since Marzi does not fully know what it is like to be hard of hearing, her work represents the feelings and experiences of real people with whom she’s connected online. To gather inspiration for her “I Want You to Know” pieces, Marzi engages with individuals who are personally affected. They describe the biases that interfere with their lives and offer practical solutions to their typical counterparts. This process provides “an opportunity for them to voice their experiences—I just illustrate them,” Marzi says, humbly.

In addition to spotlighting hearing loss in “I Want You to Know,” Marzi has previously created doodles on autism, grief, and obsessive-compulsive disorder and plans to craft future illustrations about chronic illness, dyslexia, and miscarriage.

She understands her introverted nature as a creative advantage. In her words, shyness goes hand in hand with being “perceptive, creative, and thoughtful”—the very characteristics needed to compassionately capture important pieces of human experience.

Receive updates on life-changing hearing research and resources by subscribing to HHF's free quarterly magazine and e-newsletter.

 
 
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