Spotlight On: Andy Groves, Ph.D.

Andy Groves, Ph.D.

CURRENT INSTITUTION: 

Baylor College of Medicine, Houston, Texas

EDUCATION:

Undergraduate from the University of Cambridge

Ph.D. from the Ludwig Institute for Cancer Research, London

Postdoc at the California Institute of Technology 

This new feature aims to connect Hearing Health Foundation (HHF) supporters and constituents to its Hearing Restoration Project (HRP) consortium researchers. Spotlight On provides an opportunity to get to know the life and work of the leading researchers working collaboratively in pursuit of a cure for hearing loss and tinnitus. 

What is your area of focus?

I am a developmental biologist who uses the ear as a model system to understand the general problem of embryonic development—how do you form something very complicated from very simple beginnings. The inner ear is a tissue that receives extremely precise instructions to form just the right number of cells in the right place at the right time. My lab studies where the ear comes from embryonically, how the cochlea acquires its exquisite pattern, and why sensory hair cells are not replaced in mammals after damage.

Why did you decide to get in to scientific research?

I always enjoyed biology and chemistry as a kid and thought it would be more fun than studying medicine. I had a very enthusiastic high school biology teacher who loaned me books on biology and evolution, which made an enormous impression. When I was an undergraduate at Cambridge, I was lucky to have two professors who both won Nobel Prizes, and during my senior year I had the opportunity to do research with one of them. After that, scientific research seemed like the only game in town….

Why hearing research?

I started to study ear development as a postdoctoral fellow in the 1990s because it had received very little attention for decades. The ear appeals to my love of extremes in biology: It has one of the most elaborate three-dimensional structures of any organ; it possesses cells of astonishing mechanical sensitivity; and it can detect sounds over a trillion-fold power range. It is also remarkable to think that our entire auditory experience—conversation, music, the natural world—is captured by just a few thousand sensory cells in each ear!

What is the most exciting part of your research?

Experiments can take months or years to carry out. But every now and then you find something new, and the thrill of realizing that you have found out something that no one else in the world knows about is quite addictive.

What do you enjoy doing when you’re not in the lab?

I am a huge music fan and have a large CD collection. Right now my playlist includes Beethoven sonatas played on a fortepiano, some rare Miles Davis live concerts from 1965, and Howlin’ Wolf albums. As a grad student, I sang at Cambridge and with the London Philharmonic Orchestra. I also love reading. Despite living in the U.S. for over two decades, I know very little about its history, so I have been trying to educate myself about the Civil War Era. I just finished reading “The Half Has Never Been Told” by Edward Baptist.

What is a memorable moment from your career?

For me, it is the “firsts”—seeing students or postdocs publish their first paper or when someone in my lab gets their first academic position. The nature of science means that most of what is discovered will become obsolete or surpassed, but the achievements and careers of the people who have come through the lab will hopefully last for much longer.

If you weren’t a scientist, what would you have done?

To be honest, I never had a “plan B.” I love teaching, and so if I had to give up research, it might be nice to teach biology to undergraduates.

Hearing Restoration Project

What has been a highlight from the HRP consortium collaboration?

The biggest help has been having collaborators on hand to do experiments that are outside the scope of my own lab. We recently published a paper with another HRP researcher, Stefan Heller, Ph.D., at Stanford, where he helped us analyze gene expression of single cells in the cochlea. We showed that blocking the Notch pathway could cause new hair cells to form in very young animals, but that this approach stops working as animals get older. The explosion of new technology and techniques means it is harder to do all the experiments you want in your own lab—so collaboration is key.

What do you hope to have happen with the HRP over the next year, two years, five years?

I hope we can begin a large-scale testing of candidate drugs or gene manipulations in the next two years. This initial screening will likely be in cell culture systems or in the zebrafish system that some members of the HRP helped to pioneer. In five years, I hope we have lead compounds that have been validated independently in several HRP labs.

What is needed to help make HRP goals happen?

Frankly, funding to keep our research moving forward. A postdoctoral fellow with five to six years of training starts out on a modest salary of about $45,000, plus $12,000 in benefits. So that’s $57,000 before they even pick up a test tube in the lab. Each person will typically use between $15,000-$20,000 a year in supplies and chemicals. Simply maintaining a single cage of mice for one year costs $210, and my lab can use between 300-500 cages of mice for our experiments! HHF and its donors have been extremely generous in their support, however with additional funding the output from the consortium could be significantly greater and accelerate the pace to a cure.

Which scientist or mentor was the most inspirational?

My two postdoctoral mentors at Caltech, David Anderson and Marianne Bronner, were both instrumental in making me the scientist I am today. As I was moving into the ear field, I was also lucky to meet Ed Rubel while he was on a sabbatical at Caltech and now as a fellow member of the HRP. More broadly, my two scientific heroes are Seymour Benzer and Francis Crick. Both were gifted scientists who laid the foundations of modern biology and were able to make seminal contributions to every field they worked in, from developmental and molecular biology to the study of aging, behavior, and consciousness. 

Your financial support will help ensure we can continue this vital research in order to find a cure for hearing loss and tinnitus in our lifetime. Please donate today to fund the top scientific minds working collaboratively toward a common goal. For more information or to make a donation, email us at info@hhf.org.

Your help provides hope.

Hearing: It Takes Two

By Teresa Nicolson

A major challenge in hearing research is to understand how structures known as ‘hair bundles’ are formed in the cochlea. Hair bundles have a crucial role in the detection of sound and the conversion of mechanical signals (that is, sound waves) into electrical signals. The cochlea contains two types of hair cells – inner and outer – and a hair bundle protrudes from the top of every hair cell. Each hair bundle consists of a collection of smaller hair-like structures called stereocilia that line up in rows within the bundle to form a structure that resembles a staircase (Figure 1). The stereocilia are filled with filaments made of the protein actin.

Figure 1: The roles of the two isoforms of myosin 15 (MYO15) in hair bundles.

Left: Schematic depiction showing the three rows of stereocilia in a normal hair bundle, with the first row (dark green) being the shortest and the third row (pale purple) being the tallest. This difference in height results in a characteristic staircase-like structure. The stereocilia in the first two rows mediate the process of mechanotransduction, and the large isoform of myosin 15 localizes to the tips of these stereocilia; the small isoform is found primarily in the taller stereocilia in the third row. Right: When both isoforms are defective or absent, the stereocilia in the third row do not reach their normal height (top). If the N-terminal extension in the large isoform is absent in mice, hair bundles form normally but some of the stereocilia in the first two rows degenerate in older animals (bottom). The large isoform of myosin 15 has a large extension (shown in orange) at its N-terminus.

Through studies of deaf patients, geneticists have made remarkable progress in identifying genes that are required for hearing (see hereditaryhearingloss.org). Many of the corresponding proteins are important for the function of hair cells and more than a dozen of them have roles in the hair bundle; these proteins include several myosin motor proteins that differ from the conventional myosin motors that are found in muscle cells. Hair cells actually produce two versions (or isoforms) of one of these unconventional myosin motors, myosin 15 (Wang et al., 1998; Liang et al., 1999). One of these isoforms has a large (134kD) extension at its N-terminus, but the role played by this extension in hair cells has long been a mystery.

A clue to the importance of the extension is provided by the fact that mutations in the gene (exon 2) that encodes the additional amino acids in the extension cause deafness in humans (Nal et al., 2007). To explore the role of this extension Jonathan Bird and co-workers – including Qing Fang as first author – have compared mice in which the myosin 15 proteins have the extension (isoform 1) and mice in which they do not (isoform 2; Fang et al., 2015).

Previously our knowledge about the function of myosin 15 was based on studies of mice with a mutant shaker2 gene: this mutation leads to defective hair cells in both the cochlea and the vestibular system, which is the part of the ear that controls balance. (The name shaker was coined to describe the unsteady movements seen in these mice). The shaker2 mutation effects both isoforms of myosin 15 and prevents the stereocilia growing beyond a certain height (Probst et al., 1998). The staircase-like structure seen in normal hair bundles is not seen in the shaker2 mice.

Experiments with an antibody that recognizes both isoforms suggest that myosin 15 is located at the tips of the stereocilia (Belyantseva et al., 2003). The shaker2 phenotype suggests that myosin 15 promotes the growth of stereocilia, presumably by working as an actual motor that interacts with actin filaments (Bird et al., 2014). However, the details of how this happens are not fully understood, although it might depend on proteins that are transported to the growing tip by myosin 15 (Belyantseva et al., 2005; Zampini et al., 2011).

The large isoform of myosin 15 (green) localizes predominately at the tips of short stereocilia (magenta), but not tall stererocilia, in inner hair cells in the cochlea of mice

To examine the role played by the large extension in isoform 1, Fang, Bird and colleagues – who are based at the University of Michigan, the National Institute on Deafness and Other Communication Disorders, and the University of Kentucky – generated an antibody that is specific to this isoform and used it to investigate the effects of deleting the exon 2 gene (Fang et al., 2015). Surprisingly, they found that isoform 1 is restricted to the first two rows of stereocilia in inner hair cells (Figure 1). In outer hair cells, on the other hand, isoform 1 is also found at the tall stereocilia in the third row. As for isoform 2, it is mainly present in the third row in inner hair cells.

Finding the two isoforms in different locations came as a surprise, but it could help to explain why deletion of the N-terminus and shaker2 mutations lead to different phenotypes. Shaker2 mutations affect both isoforms and lead to short hair bundles. Deletion of the N-terminus does not affect the length of stereocilia: rather, the hair bundles develop normally at first, but the first two rows of stereocilia then wither away. This suggests that the large isoform is important for the maintenance of a subset of the stereocilia: in particular, it maintains the stereocilia are involved in converting sound energy into an electrical signal in the inner part of the cochlea.

This conversion process, which is called mechanotransduction, is largely present in both the shaker2 mutants and in the mice in which the N-terminus has been deleted, albeit with some subtle differences. This phenotype suggests that myosin 15 is not directly involved in mechanotransduction: however, it seems that the large isoform of myosin 15 can recognize and accumulate at sites where this process takes place. The localization pattern of myosin 15 observed in the outer hair cells reinforces the idea that some form of membrane tension is required for accumulation of the large isoform.

A similar result was found with another protein (called sans) that is required for growth of stereocilia: deleting sans after hair bundles had fully formed caused the first two rows of stereocilia to shrink over time (Caberlotto et al., 2011). Sans interacts with the mechanotransduction machinery in hair cells (Lefèvre et al., 2008), and the loss of sans has a more dramatic effect on mechanotransduction than the loss of myosin 15. Nevertheless, these two cases suggest that it is possible to uncouple the different roles of various proteins in development and in the subsequent maintenance of mechanically-sensitive stereocilia in hair bundles. It will be interesting to see whether other short bundle mutants may have a similar phenotype, if given the chance.

Paper Acknowledgements

We thank Dennis Drayna, Lisa Cunningham, Katie Kindt and Melanie Barzik for critical reading; and Stacey Cole, Elizabeth Wilson, Joe Duda, Karin Halsey, Lisa Kabara, Jennifer Benson, Stephanie Edelmann, Anastasiia Nelina and Ron Petralia for expert technical assistance. This research was supported by funds from the NIDCD intramural research program DC000039-18 and DC000048-18 (JEB, IAB, TBF), NIDCD extramural funds R01 DC05053 (SAC, GIF, QF, MM, and AAI), R01 DC008861 (AAI, GIF), P30 DC05188 (DFD), the Hearing Health Foundation (MM) and a University of Michigan Barbour Scholarship and James V. Neel Fellowship (QF). We thank the University of Michigan Transgenic Animal Model Core and grants that support them (P30 CA46592), and the animal care staff at each institution.

This post originally appeared on eLife Science on October 6, 2015 in reference to the scientific publication, "The 133-kDa N-terminal domain enables myosin 15 to maintain mechanotransducing stereocilia and is essential for hearing." For the article's references and citations, please click here. 

HHF Celebrates National Protect Your Hearing Month

By Emily Shepard

October marks National Protect Your Hearing Month, part of the American Academy of Audiology’s (AAA) campaign to raise public awareness about hearing protection. Through extensive research and programming such as the Safe and Sound Program, Hearing Health Foundation has contributed greatly to this awareness. To celebrate National Protect Your Hearing Month, HHF has compiled a list of 5 Must Know Facts about Hearing Loss Prevention.

Fact #1: Noise Induced Hearing Loss (NIHL) can be contracted in a variety of environments.  Around 30 million U.S. workers are exposed to hazardous noise levels. The National Institute on Deafness and Other Communication Disorders (NIDCD) reports that 26 million Americans between the ages of 20 and 69- around 15% of the population- have NIHL due to exposure to loud sounds or noise at work or in leisure activities. 60% of military service members have NIHL or tinnitus, or both. Given this huge percentage, it’s unsurprising that active and veteran service members rank hearing loss and tinnitus as their top health concern.  

Fact #2: NIHL is the most preventable type of hearing loss. The measures needed to prevent NIHL are easy and simple. Just remember the following three words: Walk, Block, and Turn. When exposed to loud sounds, walk away. Block noise by wearing earplugs or other hearing protective devices when involved in a loud activity. Turn down the sound on stereos and mp3 devices. These are some of many ways you can help protect your hearing. Ultimately, the idea is to keep an eye (or an ear) on noises that seem hazardous or alarming.   “For more information about how to protect your hearing, please visit our partner’s page, It’s a Noisy Planet. Protect Their Hearing®.

Fact #3:  Half of classical orchestral musicians experience hearing loss. But that doesn’t mean you should! As stated in our blog post, “The Danger From Noise When It Is Actually Music”, musicians practice or perform up to eight hours a day. Sound levels onstage can reach up to 110 decibels (dB), the equivalent of a jackhammer! Prolonged exposure to 85 dB (the sound of heavy traffic), causes hair cells of the inner ear to be permanently damaged and can lead to hearing loss. With an 85 dB minimum for this risk, musicians exposed to jackhammer-levels are in dangerous territory. Attending an orchestra show or any other musically-vibrant production may not put you at the same risk of musicians, but it is still important to take cautionary measures. Find a seat that isn’t too close to the front of the stage and bring earplugs in case the music gets too loud. If the sound becomes especially loud, it might be worthwhile to leave early. Since soundtracks and recordings of shows are often available for purchase, there’s no need to stay out of fear of missing out. Remember, safety should always come first.  

Fact #4: What commonly used portable device is louder than a hair dryer, dishwasher, heavy city traffic, and a subway platform? The correct answer is an MP3 player at maximum volume (105 dB). Listening to your favorite artists or podcasts on blast may seem like a thrill, but there’s nothing fun about subjecting your ears to hazardous noise levels. 1 in 5 teenagers, an age group that frequently uses MP3 players, suffer from hearing loss. The Palo Alto Medical Foundation reports that 12.5% of kids between the ages of 6 and 19 suffer from loss of hearing as a result of using ear phones or earbuds turned to a high volume. So to play it safe, HHF suggests no more than 15 minutes of unprotected exposure at or above 100 decibels.  

Fact #5: Steps to prevent hearing loss should begin the moment someone is born. In 1993, only 5% of newborns were tested at birth for hearing loss. Thanks to HHF’s instrumental role in establishing Universal Newborn Hearing Screening legislation, this percentage increased dramatically. By 2007, 94% of newborns were tested. Early detection of hearing impairments in infants can help to diminish or even eliminate negative impacts that would otherwise harm their future development. Therefore it is important to screen infants for hearing impairments, preferably before they are discharged from the hospital. You can learn about the different types of tests hospitals use to screen infants here.  

HHF Board of Directors Elects Elizabeth Keithley, Ph.D. as its new Board Chair

By Elizabeth Keithley, Ph.D.

Elizabeth Keithley, Ph.D., Chairman, HHF Board of Directors                                            Professor Emeritus, Department of Surgery and Otolaryngology

University of California at San Diego

I have been a scientist who does research on mechanisms of inflammation and aging on the inner ear for more than 30 years. Growing up with a mother who had a hearing loss, I understood of the impact that hearing loss can have on a person’s life. It was quite natural that while in college I became interested in neuroscience and specifically the study of sensory perception. A professor asked me to work in his lab on hearing mechanisms and I have been studying them ever since.

In the 1990s I was asked to review the Emerging Research Grant (ERG) applications and that began my association with Hearing Health Foundation (HHF, and formerly known as Deafness Research Foundation). Soon afterward I was asked to join the Board of Directors. I have remained on the Board since that time.

The ERG program is a very valuable asset for the research community by enabling early-stage researchers to get their careers started. This program allows them to write a proposal describing a series of experiments to test a hypothesis that will increase our understanding of auditory or vestibular (hearing or balance) mechanisms. With data generated during the ERG funding period, the researcher can write an expanded, plausible proposal to address a larger issue. This becomes a proposal for funding from the National Institutes of Health.

In some ways the ERG program is a “dress rehearsal” for a career as an academic scientist. When these scientists receive funding from HHF, they have the opportunity to develop their own ideas. They begin to have some independence from a more senior investigator. The best path to achieving a world where everyone can hear is to continue bringing new people with their innovative ideas into the field of hearing and balance research. A review of the names of HHF-funded researchers over the past half century reveals the American leaders in the fields of hearing and balance research from the mid-1980s on.

As of October 1, 2015, I am the Chair of the HHF Board. I am very pleased to be involved with this important organization. HHF was created almost 60 years ago by a woman who was steadfast in her support of funding for new technologies and treatments for hearing loss. I will do whatever I can to ensure we are able to continue to make a meaningful impact through hearing research. 

It is a goal to see HHF raise enough money to fund the Hearing Restoration Project. The consortium model is a wonderful way to focus the attention of scientists to work together collaboratively and get meaningful results. If we can get to the level of funding $5 million to $6 million for research annually, it will give the scientists the resources to further accelerate the pace of the research and produce advances to prevent, treat, and cure hearing loss. Another goal that is equally as important to me is to be able to return our funding levels for the ERG program to $1 million a year. This was the level of funding when I started 20 years ago and I don’t think it is unreasonable to recommit to that amount in the future.

Hearing and balance research and advancements in hearing devices and technology have come a long way over the past 50 years. Significant outcomes have been achieved, but we still have a lot of work to do. The number of people with hearing loss and other hearing-related conditions is increasing and we need to continue to fund the most cutting-edge research until there is a day when every person can enjoy life without a hearing loss or tinnitus.  

I am interested in getting to know the members of our Hearing Health community.  If you have questions or comments, please don’t hesitate to reach out to me via email.  

I look forward to hearing from you.

World Heart Day

By the Better Hearing Institute

World Heart Day is today, September 29th. In response to a growing body of research showing a link between cardiovascular and hearing health, Hearing Health Foundation and Better Hearing Institute (BHI) are urging you to check your hearing.

Raymond Hull, PhD, professor of communication sciences and disorders in audiology and neurosciences at Wichita State University, recently completed research analyzing 84 years of work from scientists worldwide on the connection between cardiovascular health and the ability to hear and understand what others are saying. Hull’s work, which reviewed 70 scientific studies, confirmed a direct link.
 
According to Hull, “Our entire auditory system, especially the blood vessels of the inner ear, needs an oxygen-rich nutrient supply. If it doesn't get it due to cardiovascular health problems, then hearing can be affected."  
 
While there are many possible causes of hearing loss, cardiovascular disease appears to exaggerate the impact of those causes and intensify the degree of hearing decline, says Hull. This compounded effect not only increases the difficulty a person experiences in perceiving what has been said, but also diminishes their ability to make sense of what they hear with speed and accuracy.

Could hearing loss be an early sign of cardiovascular disease?

Research is ongoing, but a number of findings suggest that keeping track of your hearing may help you monitor your cardiovascular health as well.

“The inner ear is so sensitive to blood flow that it is possible that abnormalities in the cardiovascular system could be noted here earlier than in other less sensitive parts of the body,” according to David R. Friedland, MD, PhD, Professor and Vice-Chair of Otolaryngology and Communication Sciences at the Medical College of Wisconsin in Milwaukee.

In Dr. Friedland’s own 2009 study, published in The Laryngoscope, he and fellow researchers found that audiogram pattern correlates strongly with cerebrovascular and peripheral arterial disease and may represent a screening test for those at risk. They even concluded that patients with low-frequency hearing loss should be regarded as at risk for cardiovascular events, and appropriate referrals should be considered.

More recently, a 2014-published study by researchers at the University of Wisconsin in Madison found that the risk of hearing impairment was significantly greater in people with underlying atherosclerosis, or hardening of the arteries, than in those without vessel abnormalities, suggesting that hearing loss may be an early sign of cardiovascular disease in apparently healthy people, according to an article in The Wall Street Journal. The study involved a large cohort of middle-aged participants and showed that hearing loss is common in people in their forties. 

3 Heart-Healthy Reasons to Get a Hearing Test

1. Six decades of research points to heart-hearing health link: Specifically, the study authors concluded that the negative influence of impaired cardiovascular health on both the peripheral and central auditory system—and the potential positive influence of improved cardiovascular health on these same systems—have been found through a sizable body of research.
   

2. The ear may be a window to the heart: Some experts find the evidence showing a link between cardiovascular and hearing health so compelling that they say the ear may be a window to the heart. They encourage collaboration between hearing care providers, cardiologists, and other healthcare professionals. Some even call on hearing care professionals to include cardiovascular health in patient case history and to measure their patients’ blood pressure.
   

3. The same lifestyle behaviors that affect the heart impact hearing. A higher level of physical activity is associated with a lower risk of hearing loss in women. Another revealed that smokers and passive smokers are more likely to suffer hearing loss. And a third found that regular fish consumption and higher intake of long-chain omega-3 polyunsaturated fatty acids are associated with a lower risk of hearing loss in women. Coincidence? Or does it all come back to blood flow to the inner ear? Research is ongoing.

The content for this blog post originated in a press release issued by The Better Hearing Institute on September 15, 2015.

Meet the Researcher: Samira Anderson, Au.D., Ph.D.

By Tine Aakerlund Pollard

Samira Anderson, Au.D., Ph.D.  received her Ph.D. from Northwestern University, and also holds an Au.D. from the University of Florida. Anderson is an assistant professor in the Department of Hearing and Speech Sciences at the University of Maryland and is a 2014 Emerging Research Grant recipient.

My experience as a clinical audiologist inspired my research. I worked for 26 years as an audiologist before deciding to pursue a Ph.D. Part of my motivation came from working with patients who struggled with their hearing aids. I was frustrated that I was unable to predict who would benefit from hearing aids based on the results of audiological evaluations.

Two people who have identical audiograms and who are fit with the same advanced hearing aids may experience vastly different results when hearing in the presence of noise. I wanted to study the way the brain processes sound, and how deficits in this process may impact the accuracy of the auditory signal reaching the brain.

To examine the neural processing of auditory input across the life span, I study the development of speech sound differentiation in infants, and the relationship between speech encoding and later language development. This information may lead to earlier identification and treatment of language-based learning impairments. 

In older adults, I am looking at the effects of aging and hearing loss on the ability to understand speech in complex environments. As we age, we begin to notice a gradual decrease in our ability to process incoming stimuli, in part due to slower speed of processing. These changes are exacerbated by hearing loss and deficits in cognitive abilities, such as memory and attention. 

Specifically in the future, I hope to determine the effects of manipulating hearing aid settings on the ability of the brain to accurately encode speech. Understanding the effects of amplification on the brain’s processing of speech means that better hearing aid processing algorithms can be developed. I would also like to compare changes in the brain’s processing of sound after wearing hearing aids alone vs. wearing hearing aids and using auditory training.

Studying language development made me interested in hearing science. My mother immigrated to the U.S. from Lebanon just before I was born, and I grew up hearing both English and Arabic. This exposure led to an interest in languages and how we first acquire spoken language as children. I was born in Southern California but grew up all over the U.S. as my father was a career Marine.

Both of my parents have hearing loss, so I have witnessed firsthand their struggles with hearing. My mother’s father was an agronomist and had a large farm in Damascus, Syria. When visiting him in Syria I would hear street vendors calling out that they had “Miqdadi cucumbers”—Miqdadi was his last name. I believe that my interest in the scientific field came from him as well as from my mother.

Read Anderson’s first-person account of her switch from the clinic to the lab and details about her research in “A Closer Look,” in the Winter 2014 issue of Hearing Health.

Samira Anderson, Au.D., Ph.D., is a General Grand Chapter Royal Arch Masons International award recipient. Hearing Health Foundation would like to thank the Royal Arch Masons for their generous contributions to Emerging Research Grantees working in the area of central auditory processing disorders (CAPD). We appreciate their ongoing commitment to funding CAPD research.

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

Go for It, Live It, Experience It

By Nina Poersch

Have you ever wanted to do something so badly, fulfilling a dream, but the next minute you’re terrified? Then back to feeling like you’re ready, but then again thinking, “What have I gotten myself into?” And finally settling with: If I don’t do this now, I may never get a chance again, so you go for it.

That was me when I was chosen for Season 30 of the reality TV show “Survivor.”

I was not the first person with a disability on the show. But then, being deaf and a cochlear implant recipient wasn’t always who I was. I was born with typical hearing and didn’t lose it until I was 44. I call it a “gradual sudden hearing loss” that took place over the span of about five years. The doctors are not sure why I lost my hearing. All I wanted was to get my hearing back.

After two months of wearing hearing aids that were not helping, I knew I wanted cochlear implants and got one in my right ear. I am not one to sit back and wonder why something has happened to me, or to worry about the future, but instead I embraced my situation and began a journey of educating others about cochlear implants. It’s what I talk about with every new person I meet. I also volunteer for Cochlear Americas as a mentor with the company.

After the implant, I didn’t need a captioned phone anymore. I also threw away my note pad, which had become my ears for people to write on when I couldn’t understand what they were saying to me. Driving became less horrifying. It’s amazing what you hear in your everyday life that you didn’t realize you were hearing.

But all of this didn’t come easily. I struggled with my family. It took time for everyone in my life to understand that while I do use hearing technology, there are limitations to how well I hear.

For six years I only had one implant. It wasn’t until I was called for a possible chance to be on “Survivor” that I considered going bilateral. It was a definite life-changer and I still kick myself for waiting so long.

I’m not sure whether these challenges prepared me for “Survivor,” but I believe everything happens for a reason. “Survivor” is very much a social game, one that I could have mastered before I lost my hearing. Even with cochlear implants, people with hearing loss don’t always catch everything and in the game, you can’t always ask people to repeat themselves. Nighttime was especially hard because I had to take off my sound processor.

“Survivor” finished airing in May 2015. Looking back, I know that being on the show was a learning experience as well as a teaching experience.

I learned that not everyone knows how to react to someone who has hearing loss. It can make people feel uneasy and create misunderstandings. When I look in the mirror, I don’t see a person who is deaf, or a person who wears cochlear implants. People I meet don’t see that either. They just need to be taught how I hear and how to talk to me—and that it’s okay. It’s very simple and I find most people are very interested.

Playing the game gave me insight and a voice to teach people what it was like for me to lose my hearing and what it means to have a cochlear implant. The challenges on the show that I participated in were not ones that required a lot of hearing, but I felt 100 percent confident that my implants would not have failed me had I had the chance to compete in more challenges. Instead, because of a combination of reasons—my hearing loss, my age, maybe being in the wrong tribe—I was voted off on day eight.

“Survivor” remains one of the most amazing things I have ever done. But it’s not the only thing. The biggest lesson I have learned since losing my hearing is to never let your hearing loss hold you back. Always go for your dreams. And remember that going for your dreams doesn’t always mean that you’ll succeed, but if you don’t try then you’ve failed yourself. In the end you’ll be happy that you did and you’ll find that you’ve inspired others to go for their dreams.

My motto is “go for it, live it, experience it.” It’s how I live my life and it has never failed me.

Nina Poersch lives in California. For more, see ninapoersch.com.

Have a story like Nina's that your would like to share? If so, please email info@hhf.org! 

Distilling the Data

By Michael Lovett, Ph.D.

The burgeoning field of bioinformatics allows the Hearing Restoration Project to analyze and compare large genomics datasets and identify the best genes for more testing. This sophisticated data analysis will help speed the way toward a cure for hearing loss and tinnitus.

Since its launch in 2011, the Hearing Restoration Project (HRP) is focused on identifying new therapies that will restore inner ear hair cell function, and hence hearing. Within the consortium, smaller research groups engage in separate projects over the course of the year, to move the science along more quickly.

Over the past decade my group, and the group led by my collaborator Mark Warchol, Ph.D., have worked to identify genes that are potential targets for drug development or for gene therapies to cure hearing loss. Our approach has been to determine the exact mechanisms that some vertebrates—in our case, birds—use to regenerate their hair cells and thus spontaneously restore their hearing. We have been comparing this genetic “tool kit” with the mechanisms that mammals normally use to make hair cells.

Unlike birds, mammals cannot regenerate adult hair cells when they are damaged, which is a leading cause of human hearing and balance disorders. Our working hypothesis is that birds have regeneration mechanisms that mammals are missing—or that mammals have developed a repressive mechanism that prevents hair cell regeneration.

In either case, our strategy has been to get a detailed picture of what transpires during hair cell regeneration in birds by using cutting-edge technologies developed during the Human Genome Project (the international research collaboration whose goal was the complete mapping of all the nuclear DNA in humans). These next-generation (NextGen) DNA sequencing methods have allowed us to accurately measure changes in every single gene as chick sensory hair cells regenerate.

The good news is that this gives us, for the first time, an exquisitely detailed and accurate description of all of the genes that are potential players in the process. The bad news is that this is an enormous amount of information; thousands of genes change over the course of seven days of regeneration.

Some of these will be the crucially important—and possibly game-changing—genes that we want to explore in potential therapies, but most will be downstream effects of those upstream formative events. The challenge is to correctly identify the important causative needles in the haystack of later consequences.

We already know some important genetic players, but we are still far from understanding the genetic wiring of hair cell development or regeneration. For example, after decades of basic research, we know that certain signaling pathways, such as those termed Notch and Wnt, are important in specifying how hair cells develop. These chemical signaling pathways are made of multiple protein molecules, each of which is encoded by a single gene.

However, the Notch and Wnt pathways together comprise fewer than 100 genes and, despite being intensively studied for years, we do not completely understand every nuance of how they fit together.

It also may seem surprising that—more than a decade after the completion of the Human Genome Project and projects sequencing mouse, chick, and many other species’ genomic DNA—we still do not know the exact functions of many of the roughly 20,000 genes, mostly shared, that are found in each organism. This is partly because teasing out all of their interactions and biochemical properties is a painstaking process, and some of the genes exert subtly different effects in different organs. It is also because the genetic wiring diagram in different cells is a lot more complicated than a simple set of “on/off” switches.

All of this sounds a bit dire. Fortunately, we do have some tools for filtering the data deluge into groups of genes that are more likely to be top candidates. The first is to extract all of the information on “known” pathways, such as the Notch and Wnt mentioned earlier. That is relatively trivial and can be accomplished by someone reasonably well versed in Microsoft Excel.

That leaves us with the vast “unknown” world. Analyzing this requires computational, mathematical, and statistical methods that are collectively called bioinformatics. This burgeoning field has been in existence for a couple of decades and covers the computational analysis of very large datasets in all its forms. For example, we routinely use well-established bioinformatic methods to assemble and identify all of the gene sequences from our NextGen DNA sequence reads. These tasks would take many years if done by hand, but a matter of hours by computational methods.

In the case of our hair cell regeneration data, our major bioinformatic task is to identify the best genes for further experimental testing. One method is to computationally search the vast biological literature to see if any of them can be connected into new networks or pathways. There are now numerous software tools for conducting these types of searches. However, this really is not very helpful when searching through several thousand genes at once. The data must be filtered another way to be more useful.

We have used statistical pattern matching tools called self-organizing maps to analyze all of our data across every time point of hair cell regeneration. In this way we can detect genes that show similar patterns of changes and then drill down deeper into whether these genes are connected. This has provided us with an interesting “hit list” of genes that have strong supporting evidence of being good candidates for follow-up.

An additional approach is to compare our chick data to other datasets that the HRP consortium is collecting. The logic here is that we expect key genetic components to be shared across species. For example, we now know a great deal about what genes are used in zebrafish hair cell regeneration and the genes that specify mouse hair cells during normal development. We can conduct computational comparisons across these big datasets to identify what is similar and what is different. Again, this has yielded a small and interesting collection of genes that is being experimentally tested. 

Our final strategy has been to extract classes of genes that act as important switches in development. These transcription factors control other genetic circuits. We have identified all of these that change during chick hair cell regeneration. As a consortium the HRP now has a collection of about 200 very good candidate genes for follow-up. However, software and high-speed computation are not going to do it all for us. We still need biologists to ask and answer the important questions and to direct the correct bioinformatics comparisons.

Hair cell regeneration is a plausible goal for the treatment of hearing and balance disorders. The question is not if we will regenerate hair cells in humans, but when. Your financial support will help to ensure we can continue this vital research and find a cure in our lifetime! Please help us accelerate the pace of hearing and balance research and donate today. Your HELP is OUR hope!

If you have any questions about this research or our progress toward a cure for hearing loss and tinnitus, please contact Hearing Health Foundation at info@hhf.org.

Michael Lovett, Ph.D., is a professor at the National Lung & Heart Institute in London and the chair in systems biology at Imperial College London.

Getting a Hearing Test May Be Good for Your Memory

By the Better Hearing Institute

If you want to help your memory and cognitive performance, you may want to get a hearing test and treat hearing loss. In response to a growing body of research that shows a link between unaddressed hearing loss and cognitive function, the Hearing Health Foundation (HHF) and the Better Hearing Institute (BHI) are encouraging people to get their hearing checked by a healthcare professional in recognition of World Alzheimer’s Month in September.

According to Brandeis University Professor of Neuroscience, Dr. Arthur Wingfield, who has been studying cognitive aging and the relationship between memory and hearing acuity for many years, effortful listening due to unaddressed hearing loss is associated with increased stress and poorer performance on memory tests.
 
His research shows that even when people with unaddressed hearing loss perceive the words that are being spoken, their ability to remember the information suffers—likely because of the draw on their cognitive resources that might otherwise be used to store what has been heard in memory. This is especially true for the comprehension of quick, informationally complex speech that is part of everyday life.
 
“Even if you have just a mild hearing loss that is not being treated, cognitive load increases significantly,” Wingfield said. “You have to put in so much effort just to perceive and understand what is being said that you divert resources away from storing what you have heard into your memory.”
 
How hearing loss affects cognitive function
Our ears and auditory system bring sound to the brain. But we actually “hear” with our brain, not with our ears.
 
According to Wingfield, unaddressed hearing loss not only affects the listener’s ability to perceive the sound accurately, but it also affects higher-level cognitive function. Specifically, it interferes with the listener’s ability to accurately process the auditory information and make sense of it.
 
In one study, Wingfield and his co-investigators found that older adults with mild to moderate hearing loss performed poorer on cognitive tests of memory than those of the same age who had good hearing.
 
In another study, Wingfield and colleagues at the University of Pennsylvania and Washington University in St. Louis used MRI to look at the effect that hearing loss has on both brain activity and structure. The study found that people with poorer hearing had less gray matter in the auditory cortex, a region of the brain that is necessary to support speech comprehension.
 
Wingfield has suggested the possibility that the participant’s hearing loss had a causal role. He and his co-investigators hypothesize that when the sensory stimulation is reduced due to hearing loss, corresponding areas of the brain reorganize their activity as a result.
 
“The sharpness of an individual’s hearing has cascading consequences for various aspects of cognitive function,” said Wingfield. “We’re only just beginning to understand how far-reaching these consequences are.”
 
As people move through middle age and their later years, Wingfield suggested, it is reasonable for them to get their hearing tested annually. If there is a hearing loss, it is best to take it seriously and treat it.
 
Hearing loss and dementia

A number of studies have come to light over the last few years showing a link between hearing loss and dementia.  Specifically, a pair of studies out of Johns Hopkins found that hearing loss is associated with accelerated cognitive decline in older adults and that seniors with hearing loss are significantly more likely to develop dementia over time than those who retain their hearing. 
 
A third Johns Hopkins study revealed a link between hearing loss and accelerated brain tissue loss. The researchers found that for older adults with hearing loss, brain tissue loss happens faster than it does for those with normal hearing.
 
Some experts believe that interventions, like hearing aids, could potentially delay or prevent dementia. Research is ongoing. 
 
Staying connected

A number of studies indicate that maintaining strong social connections and keeping mentally active as we age might lower the risk of cognitive decline and Alzheimer's disease, according to the Alzheimer’s Association website.
 
Interestingly, BHI research shows that people with hearing difficulty who use hearing aids are more likely to have a strong support network of family and friends, feel engaged in life, and meet up with friends to socialize. They even say that using hearing aids has a positive effect on their relationships.

For more information about hearing health and finding a healthcare professional, please visit: http://hearinghealthfoundation.org/find-a-hearing-health-professional.

The content for this blog post originated in a press release issued by The Better Hearing Institute on September 8, 2015.

Emerging Research Grants: 2016 Application Period is Now Open

By Laura Friedman

We are excited to inform you that the application period for Hearing Health Foundation's 2016 Emerging Research Grants program is now open. 

Through ERG, HHF, the largest non-profit funder of hearing and balance research, awards emerging researchers grants to conduct novel investigations of auditory and vestibular function and dysfunction. HHF wishes to stimulate research that leads to a continuing and independently fundable line of research. Only research proposals in the topic areas listed in our announcement, including basic, translational, and applied clinical research, will be considered:

We encourage you to review our announcement and Policy on Emerging Research Grants. If you are eligible to apply for this program, please make note of the deadlines below and review the instructions for submitting a letter of intent (LOI).

2016 HHF-ERG Timeline for Applicants:

• LOI deadline: October 26, 2015 by 5pm ET

• Full Application opens: Early November, 2015

• Full Application deadline: December 7, 2015 by 5pm ET

If you have any questions about the ERG program and process, please contact us at grants@hhf.org. 

Please forward and share this information with your colleagues.