Hearing Beyond the Hair Cell

By Yehoash Raphael, Ph.D.

Recently, it became clear that loud signals can also damage the connecting interface between the hair cell and the auditory nerve. This interface is the synapse. When the synapse is disrupted, hearing is impaired even without the loss of hair cells, leading to a condition called synaptopathy.

Experiments using transgenic mice showed that elevating levels of a specific molecule called NT3 in the area of the synapse can heal synaptopathy caused by exposure to loud noise. Since transgenic technology is a research tool not applicable for clinical use on humans, it is now necessary to design methods for elevating NT3 in human ears, leading to repair of synaptopathy. This is an important task, because if left untreated, synaptopathy progresses to include nerve cell death and permanent hearing deficits.

One potential way to increase NT3 concentration in the cochlea is by the use of gene transfer technology, which is based on infecting cochlear cells with viruses that are engineered to secrete NT3 and not cause infections. A potential risk of this method is that the site of NT3 is not restricted to the area of the synapses affected by the synaptopathy; NT3 can influence other types of cells.

In my lab at the University of Michigan, we tested the outcome of injecting such viruses on the structure and function of normal (intact) ears. We determined that the procedure resulted in the deterioration of hearing thresholds, and the auditory nerve and its connectivity to the hair cells were also negatively affected.

This negative outcome indicates that treatment of synaptopathy should be based on a more specific way to provide NT3 in an area restricted to the synaptic region. My work with the Hearing Restoration Project is dedicated to optimization of gene transfer technology in the cochlea, and may assist in finding more detailed methods for NT3 gene transfer that better target affected cells.

More information on Dr. Raphael’s research can be found in his report, “Viral-mediated Ntf3 overexpression disrupts innervation and hearing in nondeafened guinea pig cochleae,” published in the journal Molecular Therapy—Methods & Clinical Development on August 3, 2016.

Yehoash Raphael, Ph.D., is the The R. Jamison and Betty Williams Professor at the Kresge Hearing Research Institute, in the Department of Otolaryngology–Head and Neck Surgery at the University of Michigan.

Greatness Always Has a Price

By Morgan Leppla and Laura Friedman

Signed in 1990, the Americans with Disabilities Act (ADA) is the most contemporary federal legislation related to disabilities, outlining important workers’ rights and their employers’ obligations to provide reasonable accommodations. However, it does raise questions in regards to union contracts under the National Labor Relations Act (NLRA), which was passed in 1935 and protects workers’ rights to unionize, collectively bargain, and take action (e.g. strikes). Thus, portions of the NLRA and ADA conflict with each other, putting strain on union workers who need reasonable accommodations.

The ADA outlaws discrimination against qualified individuals with disabilities, stating that individuals must negotiate with employers for “reasonable accommodations.” On the other hand, the NLRA prohibits union members from negotiating individually. Once the ADA was passed in 1990, it outlawed any part of previously entered collective bargaining contracts that included discriminatory clauses. Additionally, an employer cannot use a collective bargaining agreement as a means to engage in discriminatory practices that are otherwise prohibited by the ADA.

The challenge for employers is balancing their dual obligations to comply with established collective bargaining arrangements while accommodating individual workplace needs.

Many union contacts contain seniority clauses, providing benefits based on how long union employees have been in their position. For example, an employee who is at a company for 10 years may choose their hours before the newest hire. However, if the newest hire has a disability, it may be necessary for them to pick their hours before the more senior worker as a reasonable accommodation. This violates the union contract and NLRA for two reasons: 1) it can be considered direct dealing with an employee, and 2) it overlooks the terms of the contract. Even so, this accommodation does not otherwise pose “undue hardship,” and therefore should be granted under the ADA.

NOT SO FAST: Firstly, the NLRA does not contain language that protects people with disabilities. Secondly, the ADA was meant to expand upon, not replace, the Rehabilitation Act of 1973, which prohibits discrimination against people with disabilities in federal hiring practices and requires affirmative action in hiring for federal agencies, programs that receive federal funding, federal contractors, and subcontractors. However, the ADA does not have an affirmative action requirement, so employers are no longer obligated to give applicants with disabilities preferential treatment throughout the recruitment or hiring process. Furthermore, ignoring the seniority clauses in collective bargaining agreements would be using affirmative action in the hiring of persons with disabilities, and therefore illegal, adding the web of confusion as to which legislation must employers comply with.

Other issues are related to privacy of medical records. In one instance, a union needed workers’ medical histories in order to meaningfully negotiate their contract, which is permitted by the NLRA but prohibited by the ADA. Due to lack of evidence, guidance, and clarity, the court had the parties settle. While in this particular instance the issue was put to bed, the inability to make a decision failed to set a precedent which could address future disputes.

This amounts to a murky legal landscape. While some of the language has been interpreted by courts, there is a lot employers and individuals need to navigate on their own. Such uncertainty and lack of clarity further hurts the disabled individual because they have to take extra strides to ensure that they receive reasonable accommodations and are not subjected to discrimination based on disability by either the terms of a collective bargaining agreement or the actual employer.

Have a personal experience with discrimination in the workplace or with negotiating reasonable accommodations with an employer? 

Please share it with HHF by emailing info@hhf.org today!

FOOTNOTES:

• http://digitalcommons.ilr.cornell.edu/cgi/viewcontent.cgi?article=1300&context=edicollect

• https://www.eeoc.gov/laws/regulations/backqround_and_summary.cfm

• http://www.americanbar.org/content/dam/aba/events/labor_law/basics_papers/nlra/collective_bargaining.authcheckdam.pdf

Audiology Awareness Month

By Morgan Leppla

October is Audiology Awareness Month and Hearing Health Foundation would like to thank audiologists for all they do in diagnosing, managing, and treating hearing loss and other hearing disorders.

Pioneering ear, nose, and throat physiologist, Hallowell Davis may have coined the word audiologist in the 1940s when he decided that the then-common term “auricular training” sounded like a way to teach people how to wiggle their ears. Fortunately, their role in promoting health is far more important than that.

Audiologists diagnose and treat hearing loss, tinnitus, and balance disorders. Some of their main responsibilities include:

• Prescribing and fitting hearing aids

• Being members of cochlear implant teams

• Designing and implementing hearing preservation programs

• Providing hearing rehabilitation services

• Screening newborns for hearing loss

They also work in a variety of settings that include private practices, hospitals, schools, universities, and for the government, like in VA hospitals (run by the U.S. Department of Veterans Affairs). Audiologists must be licensed or registered to practice in all states, the District of Columbia, and Puerto Rico.

Becoming an audiologist requires post-secondary education. One could earn a doctor of audiology (Au.D.), a master’s degree (M.A. or M.S.), or if interested in pursuing a research doctorate, a Ph.D.

The American Academy of Audiology provides a code of ethics that ought to structure audiologists’ professional behavior.  As in other medical professions, audiologists should strive to act in patients’ best interests and deliver the highest quality care they can while not discriminating against or exploiting whom they serve.

Audiologists are principal agents in hearing health. Their contributions to preserving hearing and preventing hearing and balance diseases are crucial to the well-being of millions.

Learn more about hearing healthcare options at “Looking for Hearing Aids? Find the Right Professional First.”

8 Reasons to Put a Hearing Test at the Top of Your To-Do List

By Better Hearing Institute

Of all the life hacks for better living, taking care of your hearing is among the smartest and most economical.

From pilfering away at your relationships and quality of life, to putting you at risk for other health conditions, untreated hearing loss is a silent thief. Here are eight reasons why you should get a hearing test today.

1. It may help your pocketbook. A study by the Better Hearing Institute (BHI) shows that using hearing aids reduces the risk of income loss by 90 to 100 percent for those with milder hearing loss, and from 65 to 77 percent for those with severe to moderate hearing loss, and lost as much as $30,000 annually.
    

2. Your mind may benefit. Research shows a link between hearing loss and dementia. Leading experts to believe that addressing hearing loss may at least help protect cognitive function.
    

3. It could boost your job performance. Most hearing aid users say it has helped their performance on the job. That's right. Getting a hearing test could benefit all those employees (a whopping 30 percent) who suspect they have hearing loss but haven't sought treatment.
    

4. Life’s challenges may not seem so intimidating. Research shows people with hearing loss who use hearing aids are more likely to tackle problems actively. Apparently, hearing your best brings greater confidence.
    

5. Your zest for life might get zestier. Most people who use hearing aids say it has a positive effect on their relationships. They’re more likely to have a strong social network, be optimistic, feel engaged in life, and even get more pleasure in doing things.
    

6. It could protect you against the blues. Hearing loss is linked to a greater risk of depression in adults, especially 18 to 69-year-olds.
    

7. You’ll probably be more likely to get the drift. The majority who bought their hearing aids within the past five years say they’re pleased with their ability to hear in the workplace, at home with family members, in conversations in small and large groups, when watching TV with others, in lecture halls, theaters or concert halls, when riding in a car, and even when trying to follow conversations in the presence of noise.
    

8. Your heart and health may benefit. Some experts say the inner ear is so sensitive to blood flow that it’s possible that abnormalities in the cardiovascular system could be noted here earlier than in other less sensitive parts of the body.

So do it for your health. Do it for your happiness. Get a hearing test.

To take a free, quick, and confidential online hearing check to help determine if you need a comprehensive hearing test by a hearing health care professional, visit www.BetterHearing.org

The content for this blog post originated in a press release issued by The Better Hearing Institute.

New Player Identified in Hair Cell Development

By Betty Zou, Sunnybrook Research Institute

Sensory hair cells (red) and supporting cells (green) are intricately organized in the developed cochlea. Supporting cells have high levels of the Kremen1 protein, which is stained with a green fluorescent marker here. [Image courtesy of Dr. Alain Dabdoub]

There are roughly 37.2 trillion cells in the human body, each of which can be categorized into one of about 200 different types. What’s remarkable about this immense number and diversity of cells is that they all came from a microscopic cluster that comprises the embryo. Many of these early progenitor cells start out the same, but they receive different programming instructions along the way that enable them to replicate and differentiate to form various tissues and organs.

Signalling pathways are cellular communication systems that govern whether a cell keeps dividing or stops, where it goes and, ultimately, what it becomes. One such pathway is Wnt (pronounced “wint”) signalling, a group of signal transmission networks that play a critical role in embryonic development. Dr. Alain Dabdoub, a scientist in Biological Sciences at Sunnybrook Research Institute, is studying how Wnt signalling affects inner ear development and hearing. A new study by his team has shown for the first time that Kremen1, a poorly understood member of the Wnt network, plays a direct role in the formation of the cochlea, a spiral-shaped auditory sensory organ in the inner ear.

“We know that initially at the very early stages [of development], Wnt signalling pushes cells to proliferate,” says Dabdoub. “Then division stops and cell differentiation occurs. We’re trying to find out what promotes this high level of Wnt and also what decreases it.”

Kremen1 is a protein that sits on the cell surface where it receives and transmits signals to the cellular machinery inside. Previous studies have shown that it blocks Wnt signalling, so Dabdoub and his team decided to investigate whether Kremen1 is involved in cell differentiation in the cochlea.

The researchers found that at an early embryonic stage Kremen1 was present in the precursor cells that give rise to hair cells and supporting cells. Shortly thereafter, Kremen1 was only found in the supporting cells that surround hair cells. When the researchers forced the precursor cells to overproduce Kremen1, fewer of them went on to become hair cells and more became supporting cells. In contrast, knocking down levels of Kremen1 resulted in more hair cells. The results were published in August 2016 in the journal Scientific Reports.

The cochlea contains tens of thousands of hair cells, which have hair bundles on their surface to detect and amplify sound. In mammals, when these cells are damaged or destroyed, they are not replaced and hearing loss results. Supporting cells, on the other hand, remain abundant during an individual’s lifetime and do not appear to be affected by the insults that batter hair cells.

Dabdoub’s research seeks to understand how the cochlea and hair cells form, as well as how these sensory cells can be replenished to restore hearing. “If you think about regeneration, where are the cells that you’re going to regenerate coming from?” he says.

The survival of supporting cells makes them excellent candidates from which to regrow hair cells, but they must first replicate to ensure there are enough to maintain a stable number of supporting cells and form new hair cells. Dabdoub thinks that exploiting the proliferation-enhancing properties of Wnt signalling will help achieve this. His finding that Kremen1 plays an important role in cell fate decisions in the cochlea will be critical to future efforts to regenerate hair cells. “This is a molecule that we should keep an eye on as we work towards regeneration,” he says.

Funding for this study came from the Hearing Health Foundation’s Hearing Restoration Project, Koerner Foundation and Sunnybrook Hearing Regeneration Initiative.

This blog was reposted with the permission of Sunnybrook Research Institute.
 

We need your help in funding the exciting work of hearing and balance scientists.

To donate today to support HHF's groundbreaking research,

please visit hhf.org/donate.

A Balancing Act

By Morgan Leppla

Balancing is pretty great. Not needing to think about how to stand upright is something I yield great benefit from, but is something that occurs without conscious effort or thought. I am fortunate, but many are not. This week is Balance Awareness Week, and HHF is highlighting the the inner ear and its mechanics!

The inner ear is a tiny but notable body part; not only is it important to hearing, but it is also where the balance organs and nerves are located.

The basic components of the inner ear include semicircular canals, the cochlea, the utricle, the saccule, and the vestibulocochlear nerve. The cochlea and one half of the vestibulocochlear nerve (the cochlear nerve) are in charge of hearing. The remaining semicircular canals, utricle, saccule, and vestibular nerve are responsible for balance.

There are three semicircular canals that contain fluid to activate sensory hair cells, which are arranged at ninety degree angles and detect different kinds of movement: up and down, side to side, and tilting. The utricle connects the semicircular canals to the saccule, which also detect motion. They are located in the vestibule inside of the labyrinth, which is the bony outer wall of the inner ear. All of this is the vestibular system.

But it is not only the vestibular system that assists with balance. Vision and sensory receptors (muscles, joints, skin, etc.) all transmit messages to the brain that work together and voila! balance.

Vestibular disorders can have a big effect on one’s equilibrium. People might experience dizziness, vertigo, or imbalance, as well as other inner ear-related issues. A commonly diagnosed  balance disorder is Meniere’s disease, which is one focus areas for our Emerging Research Grant (ERG) recipients.

Balance disorders can disrupt everyday life for those who experience them. It is also fairly common - in fact, about 69 million Americans or 35% of adults aged 40 and up have experiences vestibular dysfunction at some point in their life!

While it might be hard to believe something as tiny as the inner ear can affect a person’s ability to participate fully in daily life, HHF is fully committed to funding research that explores hearing and balance health.

Donate today to support groundbreaking research! 

We need your help in funding the exciting work of hearing and balance scientists.

We're Partnering With The Mighty!

By Benjamin Sherman

We're thrilled to announce a new partnership that will bring Hearing Health Foundation’s (HHF) resources in front of The Mighty's wide-reaching readership. HHF is excited to share with you our partner page on The Mighty and our logo will appear next to many stories on the site.

For those who don’t know, The Mighty is a story-based health community focused on improving the lives of people facing disease, disorder, mental illness and disability. More than half of Americans are facing serious health conditions or medical issues. They want more than information. They want to be inspired. The Mighty publishes real stories about real people facing real challenges.

HHF is dedicated to helping people with hearing loss, tinnitus, and other hearing conditions live their lives to the fullest. With this partnership, we'll be able to help even more people.

Interested in partnering with Hearing Health Foundation?

Learn more here: http://hearinghealthfoundation.org/become-partner

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

By Nadine Dehgan

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)

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

Labor Day: A Reflection of Disability in America's Workplace

By Morgan Leppla

Did you know that the first Labor Day in the U.S. was celebrated on September 5, 1892, to commemorate the achievements of the labor movement? More than a century later, we still celebrate our workforce for their contributions and successes. Mandatory safety regulations in the workplace, anti-discrimination policies, and establishing minimum wage are some of the noteworthy milestones accomplished.

Labor Day exudes an inclusive spirit. 

But what about individuals who have a disability?

The Americans with Disabilities Act of 1990 (ADA) mandates that employers must make reasonable accommodations for qualified candidates with disabilities in the workplace. However, many people do not speak up or are ashamed to disclose their disability and needs to their employer, including those with hearing loss.

The employment rate for people who are deaf or hard of hearing in the U.S. is 50 percent, compared with 70 percent among workers who do not have disabilities. There is also a stark disparity between annual income for those who have a substantial hearing loss or are deaf, compared with their typical hearing peers: $38,000 per year vs. $50,000 annually in 2011.

A hearing loss may affect one’s ability to remain communicative and productive in the workplace, as the majority of today’s jobs require some form of verbal correspondence. Whether that is listening to instructions on a construction site, providing medical expertise, or receiving feedback from a supervisor, the words people say tend to be important. Gaps in understanding lead to gaps in accuracy, productivity, and performance.

This is not just an individual worker’s problem either. When people stop working at optimal capacity, bottom lines shrink. And considering that 67 to 86 percent of the 48 million Americans who have hearing loss do not have hearing aids, more dollars than you’d expect could be lost.

Workers’ general wellness includes knowing about, managing, and treating hearing loss, as well as feeling comfortable asking for reasonable accommodations without fear of discrimination. Eradicating the stigma surrounding hearing loss is key to addressing it as an epidemic medically, economically, and socially—and we at HHF are working hard to eliminate that stigma through building education and awareness.

Labor Day is about honoring the workforce. Please join Hearing Health Foundation in celebrating the progress made for American workers, as well as acknowledging the obligation to improve the livelihood, both in and out of the workplace, of our fellow citizens. 

Conference Calls-Now With Captions

By Kathi Mestayer

That’s right, it’s not a typo! Where I live, Virginia Relay has just announced availability of captions for conference calls. No more worrying about that caller who is always multitasking and talking into their speakerphone from two feet away. Virginia Relay’s new Remote Conference Captioning service provides the captioning free of charge to Virginia residents, who can view the internet-based captions on their laptop during the conference call.

I learned about this new service from Clayton Bowen, the director of Virginia Relay, a program of the Virginia Department for the Deaf and Hard-of-Hearing (VDDHH). Virginia Relay provides communications access to people who are deaf, hard-of-hearing, and deaf-blind, through a number of traditional and high-tech programs.

If you live in Virginia, visit the Virginia Relay website, where there’s also more information on the conference-call captions.

Which other states offer conference-call captioning? So far, it’s just a handful, but these states do:

• Arizona

• Colorado

• Florida

• Hawaii

• Maine (starting July 1)

• Montana

• New Jersey

• North Carolina

…and the federal government, for their employees.

To learn more about captioning, read my story on real-time captioning, which benefits from a blend of human input and voice recognition, in the Spring 2016 issue of Hearing Health.