Our Impact Invigorated: HHF Visits the NIDCD

By Timothy Higdon

In my role as CEO of Hearing Health Foundation (HHF), most of my time is spent liaising with the individuals who make our groundbreaking work possible—scientists, volunteers, Board members, and donors—from our New York City office. I was fortunate to recently step away from my typical routine to witness the excitement of hearing and balance science at the National Institutes of Health (NIH) on Wednesday, August 21. 

This educational visit was organized by the Friends of the Congressional Hearing Health Congressional (FCHHC), the coalition co-founded by HHF that supports the policy interests of the Congressional Hearing Health Caucus (CHHC), a bipartisan group to committed to increasing hearing health care.

We learned about the latest federally-funded advancements in hearing and balance disorders in a tour of the labs at NIH’s National Institute on Deafness and Other Communication Disorders (NIDCD) Intramural Research Program at the Clinical Center in Bethesda, MD. The nation’s largest hospital devoted to clinical research, the center is one location where the NIDCD supports and conducts research on the normal and disordered processes of hearing, balance, taste, smell, voice, speech, and language with an annual budget of $474 million. 

Members of the FCHHC in the Clinical Center. Photo by Nichole Westin, American Cochlear Implant Alliance.

Members of the FCHHC in the Clinical Center. Photo by Nichole Westin, American Cochlear Implant Alliance.

NIDCD Scientific Director Andrew J. Griffith, M.D., Ph.D., and clinician-scientists Carmen Brewer, Ph.D., and Clint Allen, M.D., hosted a presentation and tour. Griffith noted the importance of animal models in his overview of the hearing and balance functions, a nod to our Hearing Restoration Project’s work with birds, fish, and mice to identify biological cures for hearing loss in humans.

I was very impressed by the state-of-the-art facilities, especially the vestibular testing booth that is used to evaluate hearing and balance patients. Eye movement is observed while the chair or walls of the booth spin rapidly, helping doctors to understand how conditions like vertigo or Ménière's disease are affecting the patient.

The support that is given to patients in clinical trials also inspired me immensely. Clinical trials recruitment can be challenging, but the NIH has a national reach with a database registry for interested patients. The NIH helps with relocation expenses for the patient to minimize disruption while necessary care is provided.

I am tremendously excited by the strong relationship HHF maintains with the NIDCD. The NIDCD’s newly appointed Director, Debara Tucci, M.D., is an alumnus of our Emerging Research Grants (ERG) program and Council of Scientific Trustees. Many of our ERG recipients subsequently qualify for funding from the NIDCD and other constituent institutes of the NIH at the rate of $91 for every $1 invested by HHF. My visit to the NIH was a meaningful reminder of the impact our scientists make at the federal level, while demonstrating that much more work must be done to better the millions who live with hearing and balance conditions. 

How wonderful it was to spend the day with so many individuals committed to hearing health. I look forward to continuing our relationships with the NIDCD and the FCHHC to advance our vision of a world in which people can live without hearing loss.

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A Home for Hearing Research

NIDCD 30 years.jpg

By Neyeah Watson

The National Institute on Deafness and Other Communication Disorders (NIDCD) commemorated 30 years as an institute of the National Institutes of Health in October 2018. Hearing Health Foundation (HHF) is proud to both honor and share in this milestone for the NIDCD, which focuses on biomedical advancements in hearing, balance, taste, smell, voice, speech, and language.

The need for the NIDCD was first championed by Geraldine Dietz Fox, a Philadelphia preschool teacher who, at 27, had developed a sensorineural hearing loss in her left ear from the mumps virus. In her search for resources and treatments, she discovered HHF, at the time known as Deafness Research Foundation, and joined its Board of Directors.

An advocate for hearing loss research, Fox was an influential member of HHF’s board but recognized the need to look beyond its nonprofit resources and toward government funding. Already politically connected by way of her father and husband, who worked on the campaigns of Florida Representative Claude Pepper and U.S. President Ronald Reagan, respectively, Fox headed to Washington, D.C., on behalf of HHF.

She befriended Robert Ruben, M.D., a chairperson for the National Committee for Research in Neurological and Communicative Disorders, a coalition of health agencies and scientists that worked to increase funding for the National Institute for Neurological and Communicative Disorders and Stroke, as it was then known. A four-time Emerging Research Grants (ERG) recipient and otolaryngologist, Ruben had been urging Congress for support of more communication sciences research.

Fox’s new friendship with Ruben and other scientists, combined with her impressive zeal and demeanor as a private citizen with hearing loss, helped her gain an appointment to the advisory committee of the National Institute for Neurological and Communicative Disorders and Stroke in 1986. But Fox was disappointed in the amount of hearing research supported by the institute, and she collaborated with Ruben and Peter Reinecke, a congressional staffer, to move toward crafting a bill for the creation of the NIDCD.

Reinecke worked closely with Pepper, who had a hearing loss of his own, and who teamed up with Iowa Senator Tom Harkin, whose brother had hearing loss. The legislation received bipartisan support and was signed into law by President Reagan in 1988, forming the NIDCD.

HHF’s lasting relationship with the NIDCD has been vital to new discoveries in hearing science. For example, HHF’s ERG program provides seed funding to talented researchers, most of whom go on to expand their research after successfully competing for larger NIDCD research grants. “HHF plays a seminal role in launching the independent research careers of many  scientists in hearing research,” said former NIDCD director James F. Battey, Jr., M.D., Ph.D.

With gratitude to Fox, Ruben, and Reinecke for giving a home to hearing research, HHF is proud to have been associated with the NIDCD’s creation and celebrates the shared commitment to find better cures and treatments for hearing loss and related conditions.

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NIH Researchers Show Protein in Inner Ear Is Key to How Cells That Help With Hearing and Balance Are Positioned

By the National Institute on Deafness and Other Communication Disorders (NIDCD)

Line of polarity reversal (LPR) and location of Emx2 within two inner ear structures. Arrows indicate hair bundle orientation. Source: eLife

Line of polarity reversal (LPR) and location of Emx2 within two inner ear structures. Arrows indicate hair bundle orientation. Source: eLife

Using animal models, scientists have demonstrated that a protein called Emx2 is critical to how specialized cells that are important for maintaining hearing and balance are positioned in the inner ear. Emx2 is a transcription factor, a type of protein that plays a role in how genes are regulated. Conducted by scientists at the National Institute on Deafness and Other Communication Disorders (NIDCD), part of the National Institutes of Health (NIH), the research offers new insight into how specialized sensory hair cells develop and function, providing opportunities for scientists to explore novel ways to treat hearing loss, balance disorders, and deafness. The results are published March 7, 2017, in eLife.

Our ability to hear and maintain balance relies on thousands of sensory hair cells in various parts of the inner ear. On top of these hair cells are clusters of tiny hair-like extensions called hair bundles. When triggered by sound, head movements, or other input, the hair bundles bend, opening channels that turn on the hair cells and create electrical signals to send information to the brain. These signals carry, for example, sound vibrations so the brain can tell us what we’ve heard or information about how our head is positioned or how it is moving, which the brain uses to help us maintain balance.

NIDCD researchers Doris Wu, Ph.D., chief of the Section on Sensory Cell Regeneration and Development and member of HHF’s Scientific Advisory Board, which provides oversight and guidance to our Hearing Restoration Project (HRP) consortium; Katie Kindt, Ph.D., acting chief of the Section on Sensory Cell Development and Function; and Tao Jiang, a doctoral student at the University of Maryland College Park, sought to describe how the hair cells and hair bundles in the inner ear are formed by exploring the role of Emx2, a protein known to be essential for the development of inner ear structures. They turned first to mice, which have been critical to helping scientists understand how intricate parts of the inner ear function in people.

Each hair bundle in the inner ear bends in only one direction to turn on the hair cell; when the bundle bends in the opposite direction, it is deactivated, or turned off, and the channels that sense vibrations close. Hair bundles in various sensory organs of the inner ear are oriented in a precise pattern. Scientists are just beginning to understand how the hair cells determine in which direction to point their hair bundles so that they perform their jobs.

In the parts of the inner ear where hair cells and their hair bundles convert sound vibrations into signals to the brain, the hair bundles are oriented in the same direction. The same is true for hair bundles involved in some aspects of balance, known as angular acceleration. But for hair cells involved in linear acceleration—or how the head senses the direction of forward and backward movement—the hair bundles divide into two regions that are oriented in opposite directions, which scientists call reversed polarity. The hair bundles face either toward or away from each other, depending on whether they are in the utricle or the saccule, two of the inner ear structures involved in balance. In mammals, the dividing line at which the hair bundles are oriented in opposite directions is called the line of polarity reversal (LPR).

Using gene expression analysis and loss- and gain-of-function analyses in mice that either lacked Emx2 or possessed extra amounts of the protein, the scientists found that Emx2 is expressed on only one side of the LPR. In addition, they discovered that Emx2 reversed hair bundle polarity by 180 degrees, thereby orienting hair bundles in the Emx2 region in opposite directions from hair bundles on the other side of the LPR. When the Emx2 was missing, the hair bundles in the same location were positioned to face the same direction.

Looking to other animals to see if Emx2 played the same role, they found that Emx2 reversed hair bundle orientation in the zebrafish neuromast, the organ where hair cells with reversed polarity that are sensitive to water movement reside.

These results suggest that Emx2 plays a key role in establishing the structural basis of hair bundle polarity and establishing the LPR. If Emx2 is found to function similarly in humans, as expected, the findings could help advance therapies for hearing loss and balance disorders. They could also advance research into understanding the mechanisms underlying sensory hair cell development within organs other than the inner ear.

This work was supported within the intramural laboratories of the NIDCD (ZIA DC000021 and ZIA DC000085).

Doris Wu Ph.D. is member of HHF’s Scientific Advisory Board, which provides oversight and guidance to our Hearing Restoration Project (HRP) consortium This article was repurpsed with permission from the National Institute on Deafness and Other Communication Disorders. 

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HHF's Field Trip to NIDCD's New Research Center

By Nadine Dehgan

Nadine Dehgan, HHF's CEO

Nadine Dehgan, HHF's CEO

This August, I had the pleasure of visiting the National Institutes of Health (NIH) and attended a laboratory tour hosted by the National Institute on Deafness and Other Communication Disorders (NIDCD), which is one of the 27 Institutes and Centers that makes up the NIH. Organized by the Friends of the Congressional Hearing Health Caucus (FCHHC) and in the company of a select group of individuals including Congressional staff members, other hearing organizations, and NIH staff, we first met in the Porter Neuroscience Research Center in Bethesda, Maryland. The research center’s name honors former U.S. House of Representative member John Edward Porter, a huge supporter of biomedical research. He was largely responsible for leading the charge to double the NIH budget from 2003-2011. Rep. Porter was also the vice chairman of the Foundation for the NIH, and still holds many other public service roles.

James Battey, Jr., M.D., Ph.D., the NIDCD Director, reviewed NIDCD operations and showed how the research funding supports seven mission areas in hearing, balance, taste, smell, voice, speech, and language.  He also mentioned the recently released National Academy of Sciences (NAS) Hearing Health Care Consensus Report (whose recommendations HHF supports). Dr. Battey was warm and approachable and accompanied the visitors throughout the tour answering questions.

Andrew Griffith, M.D., Ph.D., the NIDCD Scientific Director and Chief of the Molecular Biology and Genetics Section, provided us with a detailed explanation of the NIDCD’s intramural research program.  “Intramural” refers to the internal research conducted on the NIH campus and usually is only 10% of an Institute’s entire budget.  Dr. Griffith underscored the benefits of this unique funding environment that allows the investigators to conduct both long-term and high-risk, high-reward science that would otherwise be difficult to undertake in academia and private industry.

The NIDCD is one of ten neuroscience Institutes with labs housed in the newly constructed Porter Neuroscience Building.  Prior to the building’s construction, these labs were spread across eight separate locations. Now, the labs are organized by scientific research topic to allow researchers to share resources and allow for easy collaboration.  Research includes basic and clinical neuroscience research, including investigating Parkinson’s and Alzheimer’s. (See the detailed listof topic areas that comprise more than 800 scientists in 85 labs.)

The facilities are bright, state-of-the-art, and energy efficient. It is the most energy-efficient science lab in the entire world! It uses solar panels, geothermal wells, and has a special chilled beam air-conditioning system that requires a fraction of energy regular systems use.  At 50,000 sq. ft, it is also one of the largest research buildings in the world dedicated to studying the brain.

Doris Wu, Ph.D.(Slide images from Bissonnette & Fekete, 1996; Morsli et al, 1998)

Doris Wu, Ph.D.(Slide images from Bissonnette & Fekete, 1996; Morsli et al, 1998)

The tour took us to the labs of Doris Wu, Ph.D., Chief of the Sensory Cell Regeneration and Development Section, who discussed her studies of the development of the inner ear in mice and chickens, in particular her work to identify the molecular processes involved. Dr. Wu is also a member of HHF’s Scientific Advisory Board, which provides oversight and guidance to our Hearing Restoration Project (HRP) consortium of researchers.

She paint-filled an embryonic mouse inner ear and let us view it. I put on a pair of gloves and saw how tiny it was in the petri dish (less than 2mm in length) and then what it looked like magnified. As the day went on, I grew more and more impressed with the technical aspects of scientific hearing research.

In Dr. Griffith's lab, he discussed how his team helps those with genetic hearing loss. By identifying specific genes that are mutated in families, in certain cases, he can develop personalized therapies to address the cause of the hearing loss and prevent it.  Dr. Griffith also discussed exciting research from another NIDCD lab that is using CRISPR/Cas9 genome editing technology to create and test therapies. This amazing editing tool has been touted as being faster, cheaper, and more accurate than previous gene editing technologies; HRP researcher John Brigande, Ph.D., is also using it in his current HRP project. 

It was a super impressive tour—the scientists and administration are all friendly, smart, and most importantly dedicated to advancing hearing science. It’s so refreshing to meet so many people who are committed to the advancement of humankind and to uncovering discoveries that will lead to improvements in the quality of life and health of so many.
HHF is very happy to partner with the NIDCD and its research goals, which Dr. Battey wrote about in the Summer 2016 issue of Hearing Health magazine. We are also very proud the majority of early-career scientists we support through our Emerging Research Grants program go on to earn additional funding from the NIH, underscoring the importance of the innovative research both our institutions believe is worthy.

Congressional staff and hearing advocates at FCHHC’s 2016 NIDCD tour

Congressional staff and hearing advocates at FCHHC’s 2016 NIDCD tour

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Help Mom Hear Better This Mother's Day

By Yishane Lee

Give the gift of hearing this Mother’s Day by taking your mom to get a hearing screening, and getting one yourself. One in five adults has a hearing loss—including adolescents—and the rate increases with age, with one in three seniors experiencing a hearing loss. But the average time between being diagnosed with a hearing loss and getting a hearing aid is seven years. That’s a long time to miss parts of conversations, misunderstand television dialogue, or be unable to fully enjoy a family gathering.

Now a new study puts more urgency into the need to check hearing. Researchers from the National Institute of Deafness and Other Communication Disorders (NIDCD) found a link between hearing loss and an increased risk of depression and published the results in the journal JAMA Otolaryngology–Head & Neck Surgery. The scientists examined data for 18,000 people and found that a decline in hearing more than doubled the risk for depression when compared with those who said they had excellent hearing.

Women ages 70 and older were particularly susceptible to depression with even a moderate hearing loss of 35 to 50 decibels. And when every level of hearing loss was considered, 14.7 percent of women of all ages were more likely to feel sad and depressed, compared with 9 percent of men with any degree hearing loss. The link between depression and hearing loss remained even when the researchers controlled for factors such as vision problems.

The NIDCD study underscores the importance of getting your hearing checked and treated, and of getting treated for depression as well in the event of a diagnosed hearing loss. However, and unfortunately, the researchers also found that depression was higher among those using hearing aids.

Don’t leave your mom out of the conversation. Book a hearing screening for both of you in honor of Mother’s Day. She’ll thank you, we promise!

Learn more about finding the right hearing health professional and taking care of your ears from the Spring issue of Hearing Health magazine:

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All Hail Supporting Cells

By Yishane Lee

We have the ability to hear, thanks to the sensitive hair cells located in our ears. They are called hair cells because of their hair-like shape, long and thin (resembling the hairs on your head yet having nothing to do with them). When these hair cells die or are damaged, it is permanent. And so is the resulting loss of hearing. Unlike in other species—such as birds, fish, and amphibians—in mammals including humans, once these cells die, they don’t grow back or repair themselves, which makes it even more critical to keep them alive.

Supporting cells, as their name suggests, support hair cells both structurally and nutritionally. But a new study of supporting cells in the inner ears of mice reveals yet another role—one that is hugely important for researchers working to restore hearing in mammals.

Writing in July in an online edition of the Journal of Clinical Investigation, scientists at the National Institute on Deafness and Other Communication Disorders (NIDCD), found that supporting cells can actually actively help repair damaged sensory hair cells.

Supporting cells and a chemical they produce called heat shock protein 70 (HSP70) appear to play a critical role in protecting damaged hair cells from death in the ears of mice. Senior study author Lisa Cunningham, Ph.D., said, “Our study indicates that when the inner ear is under stress, the cell that responds by generating protective proteins is not a hair cell, but a supporting cell.” Cunningham and her team are collaborating with a clinical team at the NIDCD to design a clinical trial. It will look at ways to induce the production of HSP70 in the inner ear.

Further, our Hearing Restoration Project consortium members Albert Edge, Ph.D. of Harvard Medical School, Stefan Heller, Ph.D. of Stanford University, and Elizabeth Oesterle, Ph.D. of University of Washington are trying to figure out what happens to supporting cells after hair cells die or are damaged. Their project, “Supporting Cell Fate Mapping,” has so far found that some promising news regarding supporting cells and their function. In “Making a Map,” an article in the Fall issue of Hearing Health, Dr. Oesterle writes:

“After some severe insults (damage), the nonsensory supporting cells in the [auditory] epithelium [the organ of Corti] can retain some normal cellular identity for long periods of time. This is encouraging because in non-mammals it is the supporting cells that give rise to new replacement hair cells after hair cells are lost. After various severe insults, our data suggest that the supporting cells die and neighboring cells—cells that are normally abutting the sensory epithelium—move in.”

Both recent research results show that while we still have more to learn about the roles of supporting cells, their support they provide may be crucial to the search for a cure for hearing loss and tinnitus.

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Congratulations to Our Former Emerging Researchers

By Tara Guastella

The primary goal of our Emerging Research Grant (ERG) program is to prepare scientists new to hearing and balance research to earn funding through the National Institutes of Health (NIH). It is with that in mind that we are thrilled to congratulate the latest crop of ERG alumni who have received NIH support.

For the past 55 years, we have proudly provided thousands of hearing researchers with the seed funding to make it possible to compete successfully for NIH awards and further their research careers. With the tightening funding climate in Washington, it is truly a remarkable achievement to obtain these awards.

It is with great pleasure that we share:

2012 Emerging Researcher, Wei Min Chen, Ph.D., University of Virginia, received two awards from National Human Genome Research Institute (NHGRI) for work in complex genetics research identifying genetic predictors of certain diseases.

2012 Emerging Researcher, Sung Ho Huh, Ph.D., Washington University, received a National Institute on Deafness and Communication Disorders (NIDCD) award studying cellular and molecular functions of cochlear development.

2012 & 2013 Emerging Researcher, Israt Jahan, M.B.B.S, Ph.D., University of Iowa, received a NIDCD award for her work in hair cell regeneration.

2011 & 2013 Emerging Researcher, Carolyn Ojano-Dirain, Ph.D., University of Florida, received a NIDCD award for her work in aminoglycoside-induced ototoxicity.

2012 & 2013 Emerging Researcher, Lina Reiss, Ph.D., Orgeon Health & Science University, received a NIDCD award for her work in binaural hearing loss and hearing devices.

Isabelle Roux, Ph.D.

Isabelle Roux, Ph.D.

2012 Emerging Researcher, Isabelle Roux, Ph.D., Johns Hopkins University, received a NIDCD award for her research in hair cells and their interaction with nerve fibers that provide feedback from the brain to the ear.

2012 Emerging Researcher, Rebecca Seal, Ph.D., University of Pittsburgh, received two National Institute of Neurological Disorders and Stroke (NINDS) awards for work studying the central nervous system.

2009 Emerging Researcher, Ruili Xie, Ph.D., University of North Carolina, Chapel Hill, received an award from the NIDCD for research on age-related hearing loss and noise-induced hearing loss.

We congratulate these researchers for their extraordinary research efforts and look forward to learning of their progress into the future.

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Eight Reasons to Get Your Hearing Checked This May

By Elizabeth Thorp

Did you know that nearly 50-million Americans have some sort of hearing loss? I'm one of them—I was born deaf in my left ear from genetic nonsyndromic senorineural hearing loss.

Hearing loss is actually the country's most common birth defect. In fact, two to three of every 1,000 children born in the United States are deaf or hard-of-hearing. And ninety percent of those kids have parents who can hear, like me. I wasn't fully diagnosed until I was a teen.

Perhaps even more interesting, the National Institute on Deafness and Other Communication Disorders (NIDCD) says that only 20% of the staggering number of people who could benefit from hearing aids are actually using them. Hearing research and technology have made huge leaps and bounds since I was a child, and the 40-million people not taking advantage of them are missing an opportunity to hear much better.

So in honor of Better Hearing and Speech Month—which continues through the end of May—here are eight reasons to get a hearing check now:

1. You've probably noticed a hearing problem already but done nothing about it. Don't worry, you're not alone. People generally wait seven to ten years between the time that they notice a hearing problem and the time they actually make an appointment with an audiologist or ENT.

2. Even if you've had regular physicals and appear to be in good health, you could have a hearing issue. Only 16% of physicians routinely screen for hearing loss.* Since a hearing exam is not a standard part of most examinations, you typically have to make a separate appointment—and you may not have known to do so since many general practitioners don't suggest it.

3. If you are a recent veteran, chances are your hearing was damaged during your service. 60% of veterans returning from Iraq and Afghanistan come home with hearing loss and/or tinnitus.

4. Hearing loss can cause learning delays, and your child might be among the 20% of preschoolers to fail a hearing screening*, but the earlier the problem is caught, the better.

5. Hearing loss can lead to depression and social isolation—it can affect nearly every aspect of your life. Treating hearing loss can help people re-engage with their communities and even be able to stay more involved with their families.

6. A recent study out of Johns Hopkins showed that people with mild hearing loss are twice as likely to develop dementia—a likelihood that increases with the severity of the hearing loss. Researchers are still searching for reasons for this correlation, but one hypothesis is that the isolation and depression caused by untreated hearing loss may contribute to cognitive decline. It's possible that, by treating hearing loss, we may be able to stave off dementia.

7. One in five teenagers now has a hearing loss. The supposition is that this is caused by toxic levels of noise from mp3 players. While parents have for years been encourage their teens to turn the music down (listening at maximum volume for more than 15 minutes a day can cause a permanent hearing loss!), it's also important to ask if they're having trouble hearing and get their hearing checked.

8. If you pledge to get your hearing checked, you can help the Hearing Health Foundation raise money. For each online pledge up to 10,000, will donate a dollar to the Hearing Health Foundation to help fund hearing research. And a bonus: the Foundation will help you find local audiologist and otolaryngologist and provide information about what questions you should be asking when you visit.

Elizabeth Thorp is a family travel expert and writer. She is the founder of Poshbrood, a curated catalog of mom-tested, upscale, family-friendly vacation properties. She has been navigating public affairs and communications in Washington for 20 years. Elizabeth lives in Bethesda with her husband, Almus, and three young daughters Isabelle, Lucy, and Penelope.

*Statistic provided by Center for Hearing and Communication, from data collected in New York City.

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