Cure for Hearing Loss & T

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]

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.

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Educating Children with Hearing Loss and Autism Spectrum Disorder

By Eric Sherman

     Older Brother, Zach, Cole, and Eric (dad)

   Older Brother, Zach, Cole, and Eric (dad)

Gallaudet Research Institute’s 2009-2010 Annual Survey estimates that about 40 percent of children with hearing loss exhibit another disability and notes the prevalence of autism spectrum disorder (ASD) to be 1 in 59.1 Early intervention is critical for the development of speech, language, communication skills, and learning. Some families are fortunate to discover their child’s hearing loss at a young age so an early intervention program can be implemented to help their child stay on track with their hearing peers.

What happens when your child is diagnosed with profound to severe hearing loss at 6 months of age, receives his first cochlear implant at age 1½ (second at before his 5th birthday) and spends a couple of years in auditory-verbal therapy (AVT) or speech therapybut is showing little to no progress?

This was the case with my youngest son. After receiving a second cochlear implant at 1½ and then two years of AVT, my son was nowhere near his hearing peers in communication and language skills. My wife and I knew language development could be a long process, but our gut was telling us that something else was wrong. Our auditory-verbal therapist advised us to seek additional medical evaluations to see if there was something else prohibiting our son’s language development. After having evaluations done, our son at almost 3 years of age was diagnosed on the autism spectrum.

Having a child with hearing loss takes lot of work. Add autism to the mix and it is like trying to solve a puzzle without knowing which pieces are in play.

With intensive behavioral and speech therapy over the years, our son, age 11, has done well. He has become more verbal and can certainly communicate his needs and wants. What is difficult is unlocking the doors into his learning style. There is this blurred line between his autism and hearing disability. When our son has difficulty doing schoolwork, we always question whether he is hearing the information, whether he has a problem processing the information, or whether he is just not interested in the material because it has no real meaning to his everyday life.

Our son has been lucky over the past three years having a teacher who is very creative and skillful in engaging her students. But this has not always been the case. My family’s experiences have taught us that the school district needs more information and training on how to educate a child with both hearing loss and ASD. Service providers need to know how to address each disability individually and collectively understand how they impact a child’s overall education. We have a school audiologist who wanted to create a goal for our son where he tells his aide or teacher when his CI processors are not working. This is a reasonable expectation for a child with hearing loss, but when ASD is added to the mix this may be difficult to achieve.

A child with ASD may have to be taught what it means for their processor not to be working, as well as what to do after they determine they are not working. Furthermore, generalization of whether the processor is “working or not working,” “broken,” or “on or off” may be confusing and difficult to understand. Our son believes his processor is “on” if his headpiece was attached to his head. Also, a child with autism may prefer the silence and not notify anyone their processors are off.

The dual diagnosis of hearing loss and ASD has been documented for the past 20 years; however, research and clinical guidelines on how to find and teach young children with this dual diagnosis are sparse.2 School professionals and educators need more tools and training to better equip the growing number of children afflicted with ASD and hearing loss. Both Advanced Bionics, which makes cochlear implants, and Illinois State University have done work on this issue, calling attention to the need for better diagnostic tools, early intervention, and training of education professionals. Still, significantly more needs to be done.

Eric Sherman is a father of two boys. In addition to advocating for his son and others, he serves as a parent representative on advisory boards on how special education and family support services are implemented in his local school district. To learn more about Eric Sherman visit ciwear.com/Ourstory/index.html

References:

  1. Gallaudet Research Institute (April 2011). Regional and National Summary Report of Data from the 2009-10 Annual Survey of Deaf and Hard of Hearing Children and Youth. Washington, DC: GRI, Gallaudet University.
  2. When It’s More Than Hearing Loss
  3. Autism and Hearing Loss: What You Need to Know to Help Your Families  
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Unraveling Genes Critical for Inner Ear Development

By Albert Edge, Ph.D., and Alain Dabdoub, Ph.D

The goal of the Hearing Restoration Project (HRP) is to determine how to regenerate inner ear sensory cells in humans to eventually restore hearing for millions of people worldwide. These sensory cells, called hair cells, in the cochlea detect and turn sound waves into electrical impulses that are sent to the brain. Once hair cells are damaged or die, hearing is impaired, but in most species, hair cells spontaneously regrow and hearing is restored. The HRP is aiming to enable this ability in humans. 

All cells develop through a chain of events triggered by chemical signals (proteins) from outside the cell. The signals kick off responses inside the cell that can change the cell’s ability to proliferate (grow and divide) and differentiate (take on specialized functions).

The Wnt signaling pathway, a sequence of events triggered by the Wnt protein, helps guide inner ear cell development, including the proliferation of cells that differentiate into the hair cells and supporting cells necessary for hearing and balance. But in mice and other mammals, inner ear cell proliferation does not continue past newborn stages.

Underscoring their importance in evolutionary terms, Wnt signals occur across species, from fruit flies to humans—the “W” in Wnt refers to “wingless”—and Wnt signaling is guided by dozens of genes. Albert Edge, Ph.D., Alain Dabdoub, Ph.D., and colleagues performed a comprehensive screen of 84 Wnt signaling-related genes and identified 72 that are expressed (turned on) during mouse inner ear development and maturation. Their results appeared in the journal PLoS One this February.

The Wnt signaling network has three primary pathways. Two are known to be integral to the formation of the mammalian inner ear, including the determination of a cell’s “fate,” or what type of cell it ultimately turns into. This is particularly significant because the inner ear’s sensory epithelium tissue is a highly organized structure with specific numbers and types of cells in an exact order. The precise arrangement and number of hair cells and supporting cells is essential for optimal hearing.

The relationship between the Wnt-related genes, the timing of their expression, and the various signaling pathways that act on inner ear cells is extremely complex. For instance, the composition of components inside a cell in addition to the cell’s context (which tissue the cell is in, and the tissue’s stage of development) will influence which pathway Wnt signaling will take. It is known that inhibiting the action of Wnt signaling causes hair cells to fail to differentiate.

 

The new research complements previous chicken inner ear studies of Wnt-related genes as well as a recent single-cell analysis of the newborn sensory epithelium in mice (conducted by HRP scientist Stefan Heller, Ph.D., and colleagues). Comprehensively detailing these 72 Wnt-related genes in the mouse cochlea across four developmental and postnatal time periods provides a deeper understanding of a critical component of hair cell development, bringing the HRP closer to identifying genes for their potential in hair cell regeneration.

Your Support Is Needed!

Hair cell regeneration is a plausible goal for eventual treatment of hearing and balance disorders.

The question is not if we will regenerate hair cells in humans, but when.  

However, we need your support to continue this vital research and find a cure!

Please make your gift today.  

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Are Hair Cell Regeneration Genes Blocked?

By Yishane Lee

On March 8, 2016, Hearing Health Foundation hosted a live-video research briefing, as part of an ongoing effort to provide regular updates on our research programs and progress. Through these briefings, our goal is for our attendees to learn new information and achieve a greater understanding of hearing loss, prevention, and to o develop effective therapies for hearing loss and tinnitus.

Peter Barr-Gillespie, Ph.D., the scientific director of the Hearing Restoration Project (HRP), began the webinar with announcing the newest HRP consortium member, Ronna Hertzano, M.D., Ph.D., from the University of Maryland. Ronna is a clinician as well as a research scientist, a rare combination and an asset for the HRP. She also developed a bioinformatics platform, gEAR, that the HRP is using to efficiently compare large, complex genetic datasets between species.

Dr. Barr-Gillespie went on to outline a year in the life of the HRP—how the investigators collaborate, discuss, and develop research projects. He then provided an overview of a currently funded project focused on examining whether genes can be manipulated to overcome a block to hair cell regeneration in mammals, including humans. The advancements in technologies, such as CRISPR gene modification, provides the HRP with the ability to study hair cell regeneration in different species and at a level of detail and manipulation unheard of before.

We invite you to watch the video with captioning, or read the presentation with summary notes. We are excited to share this discussion of the HRP’s progress to date and our plans for 2016 and beyond.

 

Your Support Is Needed!

Hair cell regeneration is a plausible goal for eventual treatment of hearing and balance disorders.

The question is not if we will regenerate hair cells in humans, but when.  

However, we need your support to continue this vital research and find a cure!

Please make your gift today.

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Hearing Loss vs. Dizziness: If I Could Choose!

By John V. Brigande, Ph.D.

I was about 9 when hearing loss in my left ear was first detected. The audiologist explained to me that as a result, I may not be able to hear birds singing as easily, and that I may need to concentrate more to understand words starting with “sh,” “k,” or “t.” Sensing my alarm, she tried to reassure me by saying it was unlikely that the hearing loss would affect both ears, and if it did, it would likely not be to the same extent.


Managing the loss of a primary sense is all about adaptation. In grade school, I simply tilted my right ear toward sound sources. Over time my hearing loss became bilateral and progressive, and its cause remains unknown. In graduate school I began using hearing aids and later received a cochlear implant in my left ear. I continue to use a hearing aid in my right ear, and thankfully for the past eight years, my hearing has remained stable, if stably poor.


I have always compensated. At Boston College (where I received my undergraduate, Master’s, and Ph.D., all in the biological sciences) I sat in the front seat of my classes, as close to the speaker as possible. I asked my professors and classmates to face me when they spoke so I could use visual cues to enhance oral comprehension. During postdoctoral training in auditory neuroscience at Purdue University, I was given complimentary assistive listening technology upon my arrival to the lab.


While I do not consider my hearing loss to be a profound limitation personally or professionally, it has certainly sculpted my career path. When picking my area of scientific focus, I settled on a career in auditory neuroscience to better understand hearing loss.


I also reasoned that the auditory research conferences and meetings I’d be attending would likely have assistive listening technology to allow me to participate more fully. I have benefited immeasurably from the scientific community that makes up the Association for Research in Otolaryngology, whose meetings have world-class assistive listening technologies and interpreter services plus overwhelming support of members who have hearing loss.


As I entered my 40s, I experienced vertigo for the first time. The clinical data do not fit with a diagnosis of Ménière’s disease, and the link between my vertigo and hearing loss is unclear.


When I have an acute attack of dizziness, my visual field scrolls from right to left very quickly so that I must close my eyes to avoid profound motion sickness and vomiting. I must lie down until the dizziness subsides, which is usually 12 to 16 hours. I honestly cannot do anything—I can only hope to fall asleep quickly.


Vertigo is a profound limitation for me. With no disrespect or insensitivity intended toward the hearing impaired community—of which I am a passionate member—I would take hearing loss over vertigo in a heartbeat. Dizziness incapacitates me, and I cannot be an effective researcher, educator, husband, or father. Some people perceive an aura before their dizziness occurs, but I do not get any advance warning. Unlike hearing loss, I cannot manage my dizziness—it takes hold and lets go when it wants to.


I recall one episode especially vividly. I was invited to give a seminar at the National Institute on Deafness and Other Disorders (NIDCD) and experienced a severe attack just hours before my flight. Vertigo forced me to reschedule my visit, which was tremendously frustrating. That night, I slept in the bathroom (my best solution when vertigo hits). Vestibular (balance) dysfunction is quite simply a game changer.   


A satisfying part of my research involves trying to define treatments for hearing loss and dizziness. Usher syndrome is a condition combining hearing, balance, and vision disorders. In Usher syndrome type 1, infants are born deaf and have severe vestibular problems; vision abnormalities appear by around age 10. In working with a group of dedicated colleagues at various institutions, we have evidence that fetal administration of a drug in mice with Usher syndrome type 1 can prevent balance abnormalities.


As part of HHF’s Hearing Restoration Project (HRP) consortium, I have been working on testing gene candidates in mice for their ability to trigger hair cell regeneration. This research is exciting as it is leading the HRP into phase 2 of its strategic plan, with phase 3 involving further testing for drug therapies. The probability is that manipulating a single gene will not provide lasting hearing restoration, and that we will need to figure out how to manipulate multiple genes in concert to achieve the best therapeutic outcomes.
It is an exciting time to be a neuroscientist interested in trying to find ways to help patients with hearing loss and balance issues. I am hopeful that we will make progress in defining new ways to treat and even prevent vertigo in the near future and ultimately to discover a cure for hearing loss and tinnitus.

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.

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 development@hhf.org

Your help provides hope.

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A Special Message from Claire Schultz, CEO of HHF

Optimism is the faith that leads to achievement.
Nothing can be done without hope and confidence.

-Helen Keller

We are so very grateful for your support of our research to cure hearing loss and tinnitus, and for your interest in prevention and hearing health. The following are some of our achievements during 2015:

Emerging Research Grants (ERG) - HHF awarded 10 grants in Tinnitus, Ménière's, Central Auditory Processing Disorder, and Hyperacusis. The longstanding commitment to funding the highest quality research projects remains an important priority. Learn more about how to fund a named grant, here.

Hearing Restoration Project (HRP) Published Research - HRP consortium members had their HHF-funded research published in renowned scientific publications, a big accomplishment that shows progress toward our goal and reinforces the value provided by our collaborative research approach. Check out our blog in 2016 for more updates on their path to find a cure for hearing loss and tinnitus. 

Research Briefings and Events - Our new series of live-video research updates offers an exclusive look at progress being made by our HRP Consortium members. These successful webinars, as well as HRP research events in local cities around the U.S., will continue in 2016. Watch our most recent briefing, here.

Safe and Sound - HHF and Puro Sound Labs, a consumer headphone company have partnered to spread the word about responsible listening, hearing loss prevention, and the importance of hearing health. Puro Sound's headphones have a built-in sound control device to help monitor music decibel levels and allow listeners to enjoy music while simultaneously protecting their hearing. Buy your pair today!

 

HHF Took Action - In August, the Centers for Disease Control and Prevention (CDC) issued a report on the prevalence of disability in the U.S. that excluded hearing loss. HHF took action and asked others to join our efforts; 8,500 people signed our petition which we sent to White House representatives. The result was positive; the CDC issued a statement that changes are underway.

Your contribution today ensures we continue advancing the research in 2016. Together, we will make the dream of cure a reality.

Thank you in advance for your generosity. May the New Year bring you good health and happiness.  

 

Sincerely yours,

Claire Schultz, CEO

Any donation you send before December 31st will be instantly

doubled thanks to a generous matching gift from one
of our supporters - you will make twice the IMPACT!

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Scientists restore hearing in noise-deafened mice

By the University of Michigan Health System

Scientists have restored the hearing of mice partly deafened by noise, using advanced tools to boost the production of a key protein in their ears.

  This microscope image of tissue from deep inside a normal mouse ear shows how ribbon synapses (red) form the connections between the hair cells of the inner ear (blue) and the tips of nerve cells (green) that connect to the brain.     Credit: Corfas lab - University of Michigan

This microscope image of tissue from deep inside a normal mouse ear shows how ribbon synapses (red) form the connections between the hair cells of the inner ear (blue) and the tips of nerve cells (green) that connect to the brain. 

Credit: Corfas lab - University of Michigan

By demonstrating the importance of the protein, called NT3, in maintaining communication between the ears and brain, these new findings pave the way for research in humans that could improve treatment of hearing loss caused by noise exposure and normal aging.

In a new paper in the online journal eLife, the team from the University of Michigan Medical School's Kresge Hearing Research Institute and Harvard University report the results of their work to understand NT3's role in the inner ear, and the impact of increased NT3 production on hearing after a noise exposure.

Their work also illustrates the key role of cells that have traditionally been seen as the "supporting actors" of the ear-brain connection. Called supporting cells, they form a physical base for the hearing system's "stars": the hair cells in the ear that interact directly with the nerves that carry sound signals to the brain. This new research identifies the critical role of these supporting cells along with the NT3 molecules that they produce.

NT3 is crucial to the body's ability to form and maintain connections between hair cells and nerve cells, the researchers demonstrate. This special type of connection, called a ribbon synapse, allows extra-rapid communication of signals that travel back and forth across tiny gaps between the two types of cells.

"It has become apparent that hearing loss due to damaged ribbon synapses is a very common and challenging problem, whether it's due to noise or normal aging," says Gabriel Corfas, Ph.D., who led the team and directs the U-M institute. "We began this work 15 years ago to answer very basic questions about the inner ear, and now we have been able to restore hearing after partial deafening with noise, a common problem for people. It's very exciting."

Using a special genetic technique, the researchers made it possible for some mice to produce additional NT3 in cells of specific areas of the inner ear after they were exposed to noise loud enough to reduce hearing. Mice with extra NT3 regained their ability to hear much better than the control mice.

Now, says Corfas, his team will explore the role of NT3 in human ears, and seek drugs that might boost NT3 action or production. While the use of such drugs in humans could be several years away, the new discovery gives them a specific target to pursue.

Corfas, a professor and associate chair in the U-M Department of Otolaryngology, worked on the research with first author Guoqiang Wan, Ph.D., Maria E. Gómez-Casati, Ph.D., and others in his former institution, Harvard. Some of the authors now work with Corfas in his new U-M lab. They set out to find out how ribbon synapses -- which are found only in the ear and eye -- form, and what molecules are important to their formation and maintenance.

Anyone who has experienced problems making out the voice of the person next to them in a crowded room has felt the effects of reduced ribbon synapses. So has anyone who has experienced temporary reduction in hearing after going to a loud concert. The damage caused by noise -- over a lifetime or just one evening -- reduces the ability of hair cells to talk to the brain via ribbon synapse connections with nerve cells.

Targeted genetics made discovery possible

After determining that inner ear supporting cells supply NT3, the team turned to a technique called conditional gene recombination to see what would happen if they boosted NT3 production by the supporting cells. The approach allows scientists to activate genes in specific cells, by giving a dose of a drug that triggers the cell to "read" extra copies of a gene that had been inserted into them. For this research, the scientists activated the extra NT3 genes only into the inner ear's supporting cells.

The genes didn't turn on until the scientists wanted them to -- either before or after they exposed the mice to loud noises. The scientists turned on the NT3 genes by giving a dose of the drug tamoxifen, which triggered the supporting cells to make more of the protein. Before and after this step, they tested the mice's hearing using an approach called auditory brainstem response or ABR -- the same test used on humans.

The result: the mice with extra NT3 regained their hearing over a period of two weeks, and were able to hear much better than mice without the extra NT3 production. The scientists also did the same with another nerve cell growth factor, or neurotrophin, called BDNF, but did not see the same effect on hearing.

Next steps

Now that NT3's role in making and maintaining ribbon synapses has become clear, Corfas says the next challenge is to study it in human ears, and to look for drugs that can work like NT3 does. Corfas has some drug candidates in mind, and hopes to partner with industry to look for others.

Boosting NT3 production through gene therapy in humans could also be an option, he says, but a drug-based approach would be simpler and could be administered as long as it takes to restore hearing.

Corfas notes that the mice in the study were not completely deafened, so it's not yet known if boosting NT3 activity could restore hearing that has been entirely lost. He also notes that the research may have implications for other diseases in which nerve cell connections are lost -- called neurodegenerative diseases. "This brings supporting cells into the spotlight, and starts to show how much they contribute to plasticity, development and maintenance of neural connections," he says.

In addition to Corfas, Wan and Gómez-Casati, who now works in Argentina, the research was performed by Angelica R. Gigliello, and M. Charles Liberman, Ph.D. director of the Eaton-Peabody Laboratories of the Massachusetts Eye and Ear Infirmary. The research was supported by the National Institute on Deafness and Other Communication Disorders (DC004820, DC005209) and by the Eunice Kennedy Shriver National Institute of Child Health and Human Development (HD18655), both part of the National Institutes of Health, and by the Hearing Health Foundation.

The above post is reprinted from materials provided by University of Michigan Health System

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

To donate today to Hearing Health Foundation and support groundbreaking research, visit hhf.org/name-a-grant.

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Unlocking the Potential for Hair Cell Regeneration

By Laura Friedman

On November 5, 2015, Hearing Health Foundation hosted its second live-video research briefing as part of our effort to provide regular updates on our research programs and progress. Through these briefings, our goal is for our attendees to obtain new information and understanding about hearing loss, prevention and research toward a cure.


Dr. Andy Groves, Hearing Restoration Project consortium member, presented recent research advances and new discoveries, the use of new technology, and our future plans to prevent and cure hearing loss and tinnitus. The HRP was founded in 2011 and is the first and only international research consortium focused on investigating hair cell regeneration as a cure for hearing loss and tinnitus. The overarching principle of the consortium is collaboration: open sharing of data and ideas. The HRP consortium consists of 13 of the top investigators in the audiological space, as well as a scientific director, Dr. Barr-Gillespie.

We wanted to share with you highlights from the presentation, which is available to watch with live captioning or to read with notes summarizing each slide.

Your Support Is Needed!

Hair cell regeneration is a plausible goal for eventual treatment of hearing and balance disorders. 

The question is not if we will regenerate hair cells in humans, but when.  

However, we need your support to continue this vital research and find a cure! Please make your gift today. 

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#HearTheHope This Holiday Season

By Laura Friedman

#GivingTuesday 2015, an international day of giving that kicks off the holiday giving season, is just around the corner on December 1st.

Hearing Health Foundation (HHF) wants to thank you for your continued support of our mission and programs, such as the Hearing Restoration Project (HRP) and Emerging Research Grants (ERG). Your support matters and has, and will continue to, enhance the lives of millions of Americans. Here are some of our successes, dating back to our founding in 1958:

  • HHF is the largest private funder of hearing research in the U.S.
  • HHF funded research has led to:
    • The development of cochlear implants
    • Treatments for otosclerosis (abnormal bone growth in the ear) and ear infections.
  • In 1985, scientists funded through the ERG program discovered that chickens regenerate their inner ear hair cells after damage and mammals do not. This study led to the development of the HRP in 2011.
  • In the 1990s HHF advocated for Universal Newborn Hearing Screening legislation, to detect hearing loss at birth.
    • Today, 97% of newborns are tested (up from 4% in 1994).

The work doesn't stop there. Your support will continue to impact the course of hearing and balance science and help us find a cure for the 50 million Americans living with hearing loss and tinnitus. The question of finding a cure for hearing loss is not if, but when. Making a financial commitment to HHF is an investment in our future. But we need YOUR help. Here are some ways you can #HearTheHope this holiday season:

  • Make a donation to HHF in honor or in memory of someone close to you.
  • Post on social media, such as Facebook or Twitter, encouraging your friends to donate to HHF.
    • The average person has 300 friends on Facebook which means that if each of your friends donates just $1 on Giving Tuesday, you can raise $300 in one day—it’s that easy!
  • Contribute to an item on our Wish List and give our researchers the tools they need.
  • You can make gifts of appreciated stocks or a planned gift!   
  • Let your talents and interests lead you to your own fundraiser for HHF through our website! No event is too large or small. Here are some ideas for inspiration:

    • Host a potluck and invite your guest to join you by bringing a dish and making a donation to HHF.

    • Hold a bake sale or golf outing and advertise that the proceeds will be donated to HHF.

    • Burn excess Thanksgiving calories and go for a run, swim (indoors of course!), or bike ride, fundraising for every mile accomplished.

Have other ideas or questions for us? E-mail us at Development@hhf.org.

Any donation you send before December 31st will be instantly doubled thanks to a generous matching gift from one of our supporters with hearing loss — and you will make twice the IMPACT!

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Spotlight On: 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.

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