HHF Welcomes New Board Members in April 2017

By Nadine Dehgan

Hearing Health Foundation (HHF) is delighted to welcome Bob Shannon, Ph.D., and Ruth Anne Eatock, Ph.D., to our Board of Directors. Their unwavering commitment toward advancing research to better understand hearing loss and its associated disorders make Drs. Shannon and Eatock perfect additions to our leadership team.

Dr. Robert Shannon is a research professor of otolaryngology at the University of Southern California’s Keck School of Medicine with over four decades of experience in researching auditory perception and psychoacoustics. He also serves as an editor and reviewer for several prominent scientific journals and funding agencies and has published more than 100 scientific articles on his research. Most recently Dr. Shannon has been a primary investigator on research studies that advance the technology and effectiveness of the auditory brainstem implant (ABI), an auditory prosthesis for people who have a non-functioning auditory nerve. The ABI is the first device approved by the Food and Drug Administration for prosthetic electrical stimulation of the human brainstem.

“I initially got involved with HHF (then DRF) by joining the Science Review Committee, to ensure the high quality of the research proposals, and later joined the Council of Scientific Trustees,” said Dr. Shannon. “Now I look forward to continued service on the HHF Board of Directors to have an integral role in pushing the Foundation’s research efforts forward.”

Dr. Ruth Anne Eatock is a professor of neurobiology and the dean of Faculty Affairs for the Biological Sciences Division at the University of Chicago. She trained at McGill, Caltech, the Massachusetts Eye and Ear Infirmary, and MIT, and has held academic positions in otolaryngology and neuroscience departments at University of Rochester, Baylor College of Medicine, and Harvard. She has experience mentoring students, fellows and clinical scientists in sensory processing by the inner ear, reviewing federal and private grant applications, editing and reviewing research papers, and organizing hearing research meetings.  These experiences have given her a broad appreciation of the progress and goals driving our diverse hearing research community.

Dr. Eatock notes: “My first independent grant was a Deafness Research Grant (now known as Emerging Research Grants), so I am well aware of the importance of such seed funding in helping new investigators establish themselves and advance hearing research.”

HHF is excited to have Drs. Bob Shannon and Ruth Anne Eatock as new members of our Board of Directors and we look forward to their contributions toward HHF’s mission. Please join us in giving them both a warm welcome!

A Balancing Act Before the Onset of Hearing

By Sonja J. Pyott, Ph.D.

Our ability to hear relies on the proper connections between the sensory hair cells in the inner ear and the brain. Activity of the sensory hair cells (red) and these connections ( green) before hearing begins is essential for the proper development of hearing. The research conducted by Sonja J. Pyott, Ph.D., and colleagues investigated the mechanisms that regulate this activity.

The development of the auditory system begins in the womb and culminates in a newborn’s ability to hear upon entering the world. While the age at which hearing begins varies across mammals, the sensory structures of the inner ears are active before the onset of hearing. This activity instructs the maturation of the neural connections between the inner ear and brain, an essential component of the proper development of hearing. However, we still know very little about the mechanisms regulating the activity of these sensory structures and their neural connections, specifically during the critical period just before the onset of hearing.

In our paper, “mGluR1 enhances efferent inhibition of inner hair cells in the developing rat cochlea,” soon to be published in an upcoming issue of The Journal of Physiology, we investigate the role of glutamate, a neurotransmitter, in regulating activity of the sensory structures and their connections in the inner ear before the start of hearing.

Neurotransmitters assist in the communication between neurons and are typically classified as either excitatory or inhibitory based on their action. Excitatory action results in stimulation; inhibitory action assists in the calming of the brain. Our research found that although glutamate typically excites activity, it also elicits inhibitory activity. This dual role for glutamate occurs because it activates two distinct classes of glutamate receptors: ionotropic glutamate receptors (iGluRs) and metabotropic glutamate receptors (mGluRs).

Importantly, this dual activation balances excitatory and inhibitory activity of the sensory structures, a balance of which is likely important in the final refinement of the neural connections between the inner ear and brain prior to the onset of hearing.

As part of future research, we will further investigate the role of mGluRs, one the distinct classes of glutamate receptors, in the development of hearing. We will also investigate if mGluRs balance excitatory and inhibitory activity in the adult inner ear, similar to its role prior to the onset of hearing. Insights into these mechanisms may identify new ways to modulate activity and prevent congenital or acquired hearing loss.

Study coauthor Sonja J. Pyott, Ph.D., was a 2007 and 2008 Hearing Health Foundation Emerging Research Grants recipient.

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

What's That Noise?

By Laura Friedman

In honor of World Tinnitus Day April 18, Hearing Health Foundation (HHF) wants to draw attention to the effects and challenges associated with tinnitus.

The U.S. Centers for Disease Control estimates some 15% of Americans—about 50 million people—have experienced tinnitus. Roughly 20 million people struggle with chronic tinnitus, while 2 million have extreme and debilitating cases. It is also a top war wound among active U.S. military personnel and veterans.

Tinnitus is defined as the perception of sound when there is no external, acoustic source. Individuals with tinnitus may describe the noise as buzzing, hissing, whistling, swooshing, or clicking. Roughly 90 percent of tinnitus cases occur with an underlying hearing loss.

Tinnitus can be either intermittent or chronic. People who experience intermittent tinnitus occasionally hear sounds in their ears that can last from minutes to hours after being exposed to excessively loud noises. An example would be someone sitting near the fence of a NASCAR race without wearing hearing protection. People with chronic tinnitus, on the other hand, often experience noise more frequently, which can last for more than three months.

The impact of tinnitus on everyday life differs from person to person. Researchers found that most people with chronic tinnitus are not too bothered by it. Many of these people prefer to only see a doctor for assurance that their tinnitus is not an indication of a serious disease or impending deafness. People who were bothered by their tinnitus reported that it was annoying, invasive, upsetting, and distracting in daily life. In a small tinnitus self-help group, some members frequently describe having problems sleeping, understanding speech, poor concentration, inability to relax, and depression.

People with age-related hearing loss, or presbycusis, may also experience a ringing, hissing, or roaring sound in the ears. Presbycusis progresses over time and is generally more severe in men than in women and the risk increases with age, as shown in epidemiological surveys.

Although there is no cure for tinnitus, there are available treatments that can minimize tinnitus symptoms. Tinnitus Activities Treatment (TAT), cognitive behavioral therapy (CBT), and Tinnitus Retraining Therapy (TRT) are sound therapies that can lessen the effects of tinnitus, often times very helpful in combination with counseling. Furthermore, by using hearing protection and noise reduction technologies, and by avoiding excessive noise, many people can prevent significant hearing problems.

Taking care of your hearing should always be part of keeping healthy overall. If you suspect a hearing loss or tinnitus, HHF recommends getting your hearing checked. If you do have a hearing loss or tinnitus, talk with your hearing healthcare professional about available treatments. For more information, visit hhf.org/tinnitus or email us at info@hhf.org.

Laura Friedman is the Communications and Programs Manager of Hearing Health Foundation.

John Brigande provides commentary: Hearing in the mouse of Usher

Oregon Health & Science University

The March issue of Nature Biotechnology brings together a set of articles that provide an overview of promising RNA-based therapies and the challenges of clinical validation and commercialization. In his News and Views essay, “Hearing in the mouse of Usher,” John V. Brigande, Ph.D., provides commentary on two studies in the issue that report important progress in research on gene therapy for the inner ear.

One in eight people in the United States aged 12 years or older has hearing loss in both ears. That figure suggests that, if you don’t have hearing loss, you likely know someone who does. Worldwide, hearing loss profoundly interferes with life tasks like learning and interpersonal communication for an estimated 32 million children and 328 million adults worldwide. Inherited genetic mutations cause about 50 percent of these cases.

The challenge in developing gene therapy for the inner ear isn’t a lack of known genes associated with hearing loss, but a lack of vectors to deliver DNA into cells. Brigande, associate professor of otolaryngology and cell, developmental, and cancer biology at the OHSU School of Medicine, provides perspective on companion studies that demonstrate adeno-associated viral vectors as a potent gene transfer agent for cochlear cell targets.

The first study demonstrates safe and efficient gene transfer to hair cells of the mouse inner ear using a synthetic adeno-associated viral vector that promises to be a powerful starting point for developing appropriate vectors for use in the human inner ear. The second study demonstrates that a single neonatal treatment with this viral vector successfully delivers a healthy gene to the inner ear to achieve unprecedented recovery of hearing and balance in a mouse model of a disease called Usher syndrome. Individuals with Usher syndrome type 1c are born deaf and with profound balance issues and experience vision loss by early adolescence. The research teams were led by scientists from the Harvard School of Medicine.

Brigande sees these new studies as potentially spurring investment and kickstarting the development of new approaches to correct a diverse set of deafness genes. 

Hearing Restoration Project consortium member John V. Brigande, Ph.D., is a developmental neurobiologist at the Oregon Hearing Research Center. He also teaches in the Neuroscience Graduate Program and in the Program in Molecular and Cellular Biology at the Oregon Health & Science University. This blog was reposted with the permission of Oregon Health & Science University.

Urgent Call to Action: Proposed Cuts to Hearing Research

By Nadine Dehgan, Elizabeth Keithley, Ph.D., and Peter Barr-Gillespie, Ph.D.

Hearing Health Foundation (HHF) is deeply concerned to learn the Trump administration has proposed an 18% cut to the budget for the National Institutes of Health (NIH).

Such a cut would be devastating for all medical research - including hearing research. As an advocate for the millions of Americans who have hearing loss we are especially troubled.

A drastic decrease to the funding of hearing research would disrupt the efforts of the many hearing researchers who dedicate their lives to finding cures and treatments for hearing loss, tinnitus and balance disorders.

HHF and the NIH are partners in funding research. HHF’s two research programs—the Emerging Research Grants and the Hearing Restoration Project—both rely on NIH support. HHF's funding alone cannot support these labs.

Private funding of hearing research is dwarfed by NIH support, and these proposed cuts could harm the research program of each and every hearing research lab, including those supported by the HHF.

As people with hearing loss, parents of those with hearing loss, children of those with hearing loss and as the leadership of the Hearing Health Foundation we ask your support. Financial support is always needed and welcome - but in this case we are specifically asking for you to contact your representatives to let them know that you oppose cuts to the NIH (and in fact support increases to the NIH’s budget).

If you are passionate about funding the research that will lead to cures for hearing loss and balance disorders, now is the time to act.

Please join us in contacting your Senators and House Representative's offices today.

With our sincere thanks,
Nadine Dehgan | CEO of HHF
Elizabeth Keithley | Chair of the Board
Peter Barr-Gillespie | HRP Scientific Director

Low Income at Disproportionate Risk for Hearing Loss

By Morgan Leppla

Hearing loss affects people of every age, race, and socioeconomic level. However, there are circumstances that put different people in danger of acquiring it. While some causes of hearing loss are avoidable or controllable, others are are not easily escaped. Low income people are much more likely to suffer from hearing loss than those who earn higher salaries, witnessed in both children and adults.

There are a number of reasons for this disproportional risk. Access to regular and preventative health care can be scarce so the prevalence of health problems tends to be higher overall, including that of hearing loss. Middle ear infections are more common, as is lead poisoning and malnutrition. Such detriments affect physical as well as mental development in children, and impact socio-emotional and academic performance. This disparity is heightened within underfunded school systems where sufficient accommodations for all disabilities, including hearing loss, may not be available.  

Low income caretakers of children experience additional external burdens due to a host of factors, such as long work schedules, literacy level, and language proficiency. These factors also make it difficult for caretakers to advocate on behalf of their children, or possibly notice their children’s developmental delays as a result of potential hearing loss.

Low income individuals who have emigrated from developing nations are also more likely to have already experienced some degree of permanent hearing damage. They are also often ushered into low-paying jobs that can include occupational workplace hazards like dangerously loud noises or exposure to chemicals.

People of color have  a higher risk for acquiring hearing loss. While non-Hispanic Whites still constitute the largest single group of Americans living in poverty, ethnic minority groups are overrepresented (27.4 percent African-American; 28.4 percent American Indian and Alaskan Native; 26.6 percent Hispanic, and 12.1 percent Asian and Pacific Islander compared with 9.9 percent non-Hispanic White). 

Lastly, men of all races, in low income jobs that may include physical labor, are even more at risk than other men. (If you didn’t already know, men are more likely than women to have a hearing loss, regardless of income levels).

Preventing hearing loss requires effort communicating with groups that are more at risk. If we are to end hearing loss as an epidemic, it makes sense to promote and extend hearing health care to populations who could use it most.

Under Normal Circumstances

By Morgan Leppla

March is Disability Awareness Month. In honor of this important awareness month, Hearing Health Foundation is raising awareness and celebrating all of our different abilities and doing our part to reduce the stigma of living with hearing loss and its associated disorders.

Whether we like it or not, people compare themselves to others. Maybe contemporary culture brings it out in us, or perhaps that impulse is rooted in Darwinism ideology of survival of the fittest, reminding us of competitive advantages. Who is taller, more intelligent, faster?

Possibly, it also has to do with how we conceptualize normalcy. In the mid-1800s, Belgian statistician Adolphe Quetelet introduced the idea of “l’homme moyen” (average man) when he realized that human traits are distributed over a bell curve. So the average man would have the mean of all human traits in a single abstract person.

“Normal” entered English vocabulary in 1840 and has since been used to describe bodies and behavior. However, before society focused on the “the norm” it concerned itself with “the ideal.” Take the most coveted parts of bodies and traits that exist and combine them, and that would be the ideal person.

So why is this distinction meaningful? Because every living person is non-ideal, since by definition it cannot exist in one person, whereas people (bodies and traits) can be “normal.” On the contrary, normalcy is attainable on an individual level. And society’s reactive effect to the creation of normal humans was the production of their dichotomous counterparts: the extremes or deviants at the tail ends of the bell curve, the abnormal.

However, a collision of the normal and the ideal occurred when English statistician Francis Galton decided to rank human traits, created quartiles on an intelligence bell curve, and ordered them one to four. One was lowest intelligence and least desirable while four was highest intelligence and most desirable. He reoriented the human ideal using the norm. And now, I would say, it is “normal” to want to be the smartest, most athletic, most attractive, etc.?

The latter half of the 19th century employed pseudo-empirical justifications for describing how bodies should be in fairly clear terms. And to focus on distribution of differences warps the way society approaches normalcy as a concept. It allows us to draw lines where perhaps they ought not exist.

Thus we arrive at the construction of disability. Anyone who does not physically look like others or does not act like others is perceived as deviant or abnormal because they are at the wrong end of the bell curve. Beyond the initial construction of the human normal, barriers that are literal, educational, communicational, and attitudinal further maintain “disability” since nonexistent or poor accommodations along with stigma exacerbate “disabling” differences.

Hearing Health Foundation is encouraging everyone to think about how “norms” have molded our preferences and attitudes and whether that translates to treating people differently. Life may be more arbitrary than you think, and more can be going on than what meets the eye.

HHF is committed to spreading awareness of hearing loss and its associated disorders as well as reducing the stigma attached to them. If you’d like to share your story and experiences with our community, please email us at info@hhf.org.

Make a Sound Investment

By Frankie Huang

In honor of World Hearing Day, which takes place on March 3 every year, Hearing Health Foundation is joining forces with the World Health Organization (WHO) to draw attention to the economic impact of hearing loss and the importance of treating hearing loss.

Did you know the economic cost for unaddressed hearing loss is estimated to be $750 billion globally? In the U.S. individuals with untreated severe to profound hearing loss are expected to cost society $270,000 each over the course of their lifetimes. Most of these costs are due to reduced productivity in the workplace, although the use of special education resources among children and other social services are also factors.

Lifetime earnings for those with untreated hearing loss average 50 to 70% less than their typical-hearing peers in the U.S., and has been shown to negatively impact household income up to $12,000 per year, on average, depending on the degree of hearing loss, according to the Better Hearing Institute. This is largely due to having fewer opportunities for promotions, reduced job performance, and decreased earning power.

Beyond economic losses, untreated hearing loss can significantly impact a person’s quality of life. Researchers have found that individuals with untreated hearing loss are more likely to develop depression, anxiety, and feelings of inadequacy. They may also avoid or withdraw from social situations. Left undetected in children, hearing loss can negatively impact speech and language acquisition, academic achievement, and social and emotional development.

Prevention, screening for early identification, early intervention, and rehabilitation through hearing devices are among the strategies that mitigate hearing loss and its consequences. Those who treat their hearing loss with hearing aids and/or cochlear implants show improvement in social, emotional, and psychological well-being. Interventions can significantly decrease isolation, increase self-esteem, and lead to better employment opportunities and earnings—all of which will benefit society as a whole.

For World Hearing Day 2017, the WHO has joined forces with Mimi Hearing Technologies. To raise awareness of hearing loss, Mimi hopes to have 1 million people test their hearing. To do this, they are offering the Hearing Test app on iOS free for everyone. If you suspect you or a loved one may have hearing loss, this is a great opportunity to test your hearing with Mimi’s Hearing Test, which is an initial online assessment. The results may require a follow-up appointment with a hearing health professional. However, by detecting signs of hearing loss early on the benefits of treating hearing loss far outweigh the consequences if left untreated.

Celebrating Hearing Innovations

By Frankie Huang

On Feb. 25, Hearing Health Foundation is celebrating International Cochlear Implant Day to raise awareness of this life-changing technology. Cochlear implants greatly enhance the lives of individuals with severe to profound hearing loss and individuals who don’t benefit from the use of hearing aids. Did you know that as of November 2012, there are 324,000 cochlear implants in use worldwide, and that number is growing daily!

Cochlear implants (CI) are electronic medical devices that are implanted via a surgical procedure. Although implants replace the function of the damaged inner ear, it is important to remember that CIs do not restore normal hearing but work by bypassing damaged structures in the inner ear and stimulating the auditory nerve. This sends signals to the brain, allowing the user to perceive sounds.

Researchers found that children 5 years or older with bilateral severe or profound hearing loss who are implanted with CIs have better speech perception and development over time than children treated with hearing aids. In addition, children with profound hearing loss who used CIs showed greater development of preverbal behavior than those using hearing aids.

Other researchers found that children receiving CIs before 24 months of age greatly benefit in terms of their overall language development. Levels of spoken language in children implanted before age 24 months were on par with their typical hearing peers by age 4.5, but those implanted after age 24 months did not “catch up” with hearing peers by age 4.5. It’s important to note the study didn’t evaluate language development or ongoing delays after age 4.5.

HHF is proud to have supported research in the 1970s that led to the development of cochlear implants. Since then the technology has continued to evolve and improve in order to increase the benefits yielded from having a cochlear implant and to reduce risks associated with an invasive surgical procedure. By further improving the design and the function of CIs, researchers may find a way to maximize all the possible benefits for the patient, to preserve residual hearing, and to improve the health of the inner ear.

If you’re interested in funding research related to hearing loss technology,
please consider donating today at hhf.org/donate or contact us at development@hhf.org.