All Hail Supporting Cells

By Yishane Lee

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

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

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

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

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

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

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

Congratulations to Our Former Emerging Researchers

By Tara Guastella

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

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

It is with great pleasure that we share:

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

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

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

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

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

Isabelle Roux, Ph.D.

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

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

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

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

The Case for Genetic Testing

By Yishane Lee

Genetic causes account for roughly half of hearing loss cases in infants, according to the Centers for Disease Control and Prevention. Many cases of progressive hearing loss that occur after infancy and childhood also have a genetic component.

At least 1,000 mutations in 64 genes linked to hearing loss have been identified. Thanks to rapid advances in genetic sequencing, identifying virtually all the genetic causes of hearing loss may occur within the decade, according to a recent report in the journal Genetic Testing and Molecular Biomarkers.

Researchers are using “targeted resequencing” to locate gene mutations in certain regions in the human genome that are linked to diseases much more quickly than sequencing the entire genome. In our Fall 2013 issue of Hearing Health magazine, Xue Zhong Liu, M.D., Ph.D., reviews the advances in sequencing technology and how this will affect the future treatment of hearing loss.

Because genetics can play such a significant role in hearing loss, genetic testing can answer questions you have about the cause of your or a loved one’s hearing loss. If the testing uncovers a mutation, it can help explain the hearing loss, its severity or progression, and whether other symptoms may become apparent. For instance, a person with Usher syndrome has not only hearing loss but also eventual blindness. You can proactively take steps to manage treatment and outcome. Knowing the genetic cause of a hearing loss can also help you predict whether the condition will be passed along to your children, or whether the children of other family members may have the condition. 

Last summer, Hearing Health magazine presented an overview of genetic causes of hearing loss, including Connexin 26 disorder. This is the most common cause of congenital hearing loss not related to a syndrome (with other symptoms, such as a thyroid problem). Mutations in the GJB2 gene affect development of the cochlea in the inner ear. Everyone carries two copies of the GJB2 gene (which encodes the protein connexin 26), and the mutations are usually recessive. So, two parents with one mutation each can have normal hearing. But if their child gets two faulty copies of the gene, the child will have a hearing loss. In fact, the majority of children born with hearing loss have normal hearing parents.

There are limits to genetic testing, however. For one thing not all the genes are known—yet. Also, a positive result for a mutation does not necessarily mean a person will get the condition associated with the mutation. And a negative result doesn’t mean you won’t get the particular condition, too—it may be that a different mutation in the same gene wasn’t detected, or there could be another mutation in a different gene that may cause the condition.

We have compiled a list of several dozen genetic testing centers nationwide that have specialized testing for hearing loss. Find a testing center near you.

Hearing Health Foundation Names Peter Barr-Gillespie Director of HRP

FOR IMMEDIATE RELEASE
CONTACT:
Nick Carcaterra
Susan Blond, Inc.
212.333.7728 ext. 126
nickc@susanblondinc.com

HEARING HEALTH FOUNDATION
NAMES PETER BARR-GILLESPIE DIRECTOR OF
THE HEARING RESTORATION PROJECT
& ANNOUNCES “GEORGE A. GATES RESEARCH AWARD”

Non-Profit Dedicated To Curing Hearing Loss Through Innovative Research Announces New Director for Their Groundbreaking Hearing Restoration Project

New York, NY (October 24, 2012)—On October 8, 2012, Hearing Health Foundation (HHF) honored George A. Gates, M.D., who, until his recent retirement from Hearing Health Foundation’s Board of Directors, served as the Medical Director for the organization.  As part of the evening, the Foundation announced the “George A. Gates Research Award,” to be presented annually, in perpetuity, to an outstanding Emerging Research awardee. Gates, who has been a key member of the HHF board since 1987, was the founder of the Hearing Restoration Project (HRP), a program designed to accelerate the timeline to a cure for hearing loss. Today, the Foundation is also proud to announce Peter Barr-Gillespie, Ph.D, as the new Director of the HRP.

“We will forever be grateful to Dr. Gates for his perseverance, vision, and commitment to Hearing Health Foundation and especially the Hearing Restoration Project,” says Andrea Boidman, Executive Director of Hearing Health Foundation. “He started us out on the right foot, and we are very excited to see Dr. Barr-Gillespie now at the helm. I am confident that Dr. Barr-Gillespie will help our consortium work toward the goal of a biologic cure for hearing loss; we are very lucky to have him in this leadership role.”

The goal of the Hearing Restoration Project is to find a cure for hearing loss through innovative research surrounding inner ear hair cell regeneration. Most non-mammals spontaneously regenerate these cells, which allow them to hear, but humans do not and the Hearing Restoration Project aims to understand not only why this is the case, but how we can translate what we know about non-mammals to people. Barr-Gillespie brings a wealth of experience to the HRP, joining an already impressive team of researchers.

After undergraduate studies at Reed College in Portland, Oregon, Peter G. Barr-Gillespie attended graduate school at the University of Washington, where he received his Ph.D. in Pharmacology in 1988. He spent five years as a postdoc with Jim Hudspeth, first at the University of California, San Francisco, then at the University of Texas Southwestern Medical Center. He joined the Department of Physiology at Johns Hopkins University as an Assistant Professor in 1993 and rose to Associate Professor in 1998. In 1999 he joined the Oregon Hearing Research Center as an Associate Professor and the Vollum Institute as a Scientist. He was promoted to Professor in 2004 and granted tenure in 2007.

"It's extremely exciting to be part of the Hearing Restoration Project,” said Peter Barr-Gillespie. “Progress towards a successful strategy for restoring hearing has been hampered by the scattershot approach to the problem taken by individual investigators. In the Hearing Restoration Project, a consortium of outstanding scientists has chosen to work together collaboratively towards this common goal, an approach that should substantially shorten the time needed to devise a way to restore hearing in people with hearing loss. I feel privileged to lead such a distinguished group of investigators."

In his new role, Barr-Gillespie will oversee the work of the entire Consortium, made up of some of the most talented, creative, and inspired researchers in the area of cell regeneration in the ear, whose work has already contributed significantly to the field. In addition to Barr-Gillespie as Director, the Consortium consists of: Dr. John Brigande, Dr. Alain Dabdoub, Dr. Albert Edge, Dr. Andy Groves, Dr. Stefan Heller, Dr. Michael Lovett, Dr. Liz Oesterle, Dr. Tatjana Piotrowski, Dr. David Raible, Dr. Yehoash Raphael, Dr. Edwin Rubel, Dr. Neil Segil, Dr. Jennifer S. Stone, and Dr. Mark Warchol.

For more information please visit: http://hearinghealthfoundation.org/

About Hearing Health Foundation

Since 1958, Hearing Health Foundation has given over $27.8 million to hearing and balance research.  In 2011 Hearing Health Foundation launched the Hearing Restoration Project (HRP), a consortium of scientists working on cell regeneration in the ear.  HRP's goal is a biologic cure for most types of acquired hearing loss. Hearing Health Foundation also publishes Hearing Health magazine, a free consumer resource on hearing loss and related technology, research, and products.

Dr. Edge Speaks at Harvard Club of New York About Search for Hearing Loss Cure

New York, NY, April 19, 2012 -- Dr. Albert Edge, Department of Otolaryngology, Harvard Medical School and Hearing Restoration Project Consortium Member spoke to nearly 100 people at the Harvard Club of New York about the search for a biologic cure for hearing loss.

"Replacing cells in the inner ear is an exciting potential new therapy for deafness," said Dr. Edge.  Hair cells in the inner ear convert sound information into electrical signals that enable the brain to “hear” the outside world. More than 25 years ago, researchers discovered that birds naturally regrow damaged hair cells and regain their hearing. In humans, hearing loss is permanent when the hair cells are damaged.

Now a whole new science has evolved to find a way to trigger hair cell regrowth in humans. This could mean a biologic cure for the nearly 50 million Americans living with various forms of hearing loss.

About Dr. Albert Edge (Harvard Medical School) is currently an Associate Professor in the Department of Otolaryngology. At the Tillotson Unit for Cell Biology in the Eaton-Peabody Laboratory at the Massachusetts Eye and Ear Infirmary, Dr. Edge’s research is focused stem cells and the basic mechanisms of cellular repair in the nervous system. Dr. Edge is also studying the loss of sensory cells in the inner ear that result in deafness due to excessive noise, drugs, disease, or aging.

2011 Grant Recipients Announced

FOR IMMEDIATE RELEASE                                                                                                  
July 26, 2011                                                                                                                      

Contact:
Trisha Donaldson
212-257-6143
tdonaldson@drf.org
www.drf.org

DRF Increases grantmaking:  2011 Grant Recipients Announced

Deafness Research Foundation (DRF)’s National Hearing Health Grants Center is excited to announce that it has awarded over $600,000 to 25 outstanding research scientists in the field of hearing and balance science.  For two years in a row, we are excited to announce an increase in our grantmaking.

Each year, DRF awards research grants to young investigators who are exploring new avenues of hearing and balance science. These funds will support research in the following areas:

• Fundamental Auditory Research – development, genetics, molecular biology, physiology, anatomy, and regeneration biology;

• Hearing and Balance Restoration – infants, children and adults

  • cochlear implant, auditory hair cell regeneration, and auditory nerve regeneration;

• Hearing Loss – aging, noise-induced, otosclerosis, ototoxicity, and otitis media;

• Central Auditory Processing Disorder;

• Usher Syndrome; and

• Vestibular and Balance Disorders (dizziness and vertigo, Meniere's disease).

For this year's grants selection, DRF's Council of Scientific Trustees reviewed applications from scientists at renowned research institutions around the U.S.  The selected research projects received detailed peer review for scientific merit and program relevance.  A complete list of the 2011 grant recipients is provided below, including recipients whose research is funded in whole or part by the DRF Centurion Clinical Research Award, the C.H.E.A.R. Endowment Award, Collette Ramsey Baker Research Award, and The Todd M. Bader Research Grant of The Barbara Epstein Foundation, Inc.

FIRST YEAR HEARING & BALANCE RESEARCH GRANT RECIPIENTS
Keith E. Bryan, Ph.D., University of Iowa, Carver College of Medicine
Investigating the role of cabp1 in kcnq4 channel modulation

Brenton G. Cooper, Ph.D., Texas Christian University
Lateralization of acoustic perception in Bengalese finches

Regie Lyn P. Santos-Cortez, M.D., Ph.D., Baylor College of Medicine
Identification of genes that predispose to chronic otitis media in the at population of Bolabog, Boracay island, Philippines

Elizabeth Dinces, M.D., M.S., Albert Einstein College of Medicine
Effects of aging on selective attention in complex multi-source sound environments

Carolyn P. Ojano-Dirain, Ph.D., The University of Florida College of Medicine
Prevention of aminoglycoside-induced hearing loss with the mitochondria-targeted

Sung-Ho Huh, Ph.D., Washington University School of Medicine
Role of fgfs in cochlear sensory epithelium

Albena Kantardzhieva, Ph.D., Massachusetts Eye and Ear Infirmary
Defining the interaction partners of major proteins in the hair cell's synaptic ribbon

Shuh-Yow Lin, Ph.D., The University of California, San Diego School of Medicine
Molecular mechanisms of synaptic transmission in hair cells

Debashree Mukherjea, Ph.D., Southern Illinois University School of Medicine
Targeting inflammation in prevention and treatment of noise induced hearing loss

Erin K. Purcell, Ph.D., The University of Michigan, Kresge Hearing Research Institute
A stem cell-seeded nanofibrous scaffold for auditory nerve regeneration

Zlatka P. Stojanova, Ph.D., House Research Institute
Epigenetic regulation of the atoh1 gene

Jie Tang, Ph.D., Creighton University School of Medicine
Creation of a pendrin with both motor and transport functions

Ellen S. Wilch, Ph.D., Michigan State University College of Human Medicine
Identification of cis-regulatory gjb2 and gjb6 elements by chromosome conformation capture and investigation of potential cis-regulatory variants in persons with hearing loss and monoallelic mutation of gjb2

Hsiao-Huei Wu, Ph.D., The University of Southern California, Keck School of Medicine
Regulation of inner ear development by hgf, the nonsyndromic hearing loss gene, dfnb39

SECOND YEAR HEARING & BALANCE RESEARCH GRANT RECIPIENTS
Edward L. Bartlett, Ph.D., Purdue University
Cellular bases of temporal auditory processing

Soyoun Cho, Ph.D., Oregon Health & Science University
Dynamics of exo- and endocytosis at hair cells

Frances Hannan, Ph.D., New York Medical College
The role of diaphanous in the auditory cytoskeleton

Michelle Hastings, Ph.D., Rosalind Franklin University of Medicine and Science
Therapeutic correction of ush1c splicing in a mouse model of usher syndrome

Zhengqing Hu, M.D., Ph.D., Wayne State University School of Medicine
Innervation of in vitro-produced hair cell by neural progenitor-derived glutamatergic neurons

Judith S. Kempfle, M.D., Massachusetts Eye and Ear lnfirmary
Influence of bone morphogenetic protein 4 and retinoic acid on differentiation of inner ear stem cells

Neeliyath A. Ramakrishnan, Ph.D., Wayne State University School of Medicine
Molecular interactions of the hair-cell afferent synapse

DRF CENTURION CLINICAL RESEARCH AWARD RECIPIENT

James E. Saunders, M.D., Dartmouth Hitchcock Medical Center
Genetic hearing loss in remote Nicaraguan families

This research award is funded by the Centurions of the Deafness Research Foundation. DRF has partnered with CORE Grants Program of the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) to offer a one-year DRF Centurion Clinical Research Award (CCRA) for clinical research in hearing and balance science.

DRF C.H.E.A.R. ENDOWMENT GRANT RECIPIENT

Patricia White, Ph.D., University of Rochester School of Medicine and Dentistry
1st year grant recipient
The role of foxo3 in hearing protection

The C.H.E.A.R. endowment was created to support an annual sensory-neural Deafness Research Grant. C.H.E.A.R. (Children Hearing Education and Research) was absorbed into DRF in 1991, and we are very proud to continue their legacy of funding research in sensory-neural deafness.

COLLETTE RAMSEY BAKER RESEARCH AWARD RECIPIENT

Kirill Vadimovich Nourski, Ph.D., M.D., University of Iowa Hospitals and Clinics
1st year grant recipient
Temporal processing in human auditory cortex
This research award is made in memory of Deafness Research Foundation’s founder, Collette Ramsey Baker.

THE TODD M. BADER RESEARCH GRANT OF THE BARBARA EPSTEIN FOUNDATION, INC., RECIPIENT

Marcello Peppi, Ph.D., Massachusetts Eye and Ear lnfirmary
2nd year grant recipient
Molecular mechanisms of dexamethasone-mediated protection from acoustic trauma
This research award is funded by The Todd M. Bader Research Grant of The Barbara Epstein Foundation, Inc.

###

Deafness Research Foundation is the leading national source of private funding for research in hearing and balance science.  Research made possible by DRF grants has resulted in dramatic innovations that have increased options for those living with hearing and balance disorders, as well as protected those at risk.  Since our inception in 1958, we have awarded over $26.6 million through more than 2,000 scientific research grants to researchers who are dedicated to exploring new avenues of hearing and balance science.  With the potential of hearing restoration through regeneration biology, the scope of DRF-funded research has expanded enormously.  Since 1972, DRF has funded close to 40 research grants that have been instrumental in the development, evaluation and improvement of cochlear implants.  Approximately, 188,000 implant procedures have been completed worldwide with beneficial results, particularly when the procedure is undertaken in infants.  DRF also publishes the award-winning Hearing HealthMagazine.

www.drf.org
Voice (212) 257-6143 • Toll-Free (866) 454-3924 • TTY (888) 435-6104 • Fax (212) 257-6139

Research on Cell Regeneration Highlighted in the New York Times

Two research reports published Friday offer novel approaches to the age-old dream of regenerating the body from its own cells. 

Animals like newts and zebra fish can regenerate limbs, fins, even part of the heart. If only people could do the same, amputees might grow new limbs and stricken hearts be coaxed to repair themselves. 

But humans have very little regenerative capacity, probably because of an evolutionary trade-off: suppressing cell growth reduced the risk of cancer, enabling humans to live longer. A person can renew his liver to some extent, and regrow a fingertip while very young, but not much more. 

In the first of the two new approaches, a research group at Stanford University led by Helen M. Blau, Jason H. Pomerantz and Kostandin V. Pajcini has taken a possible first step toward unlocking the human ability to regenerate. By inactivating two genes that work to suppress tumors, they got mouse muscle cells to revert to a younger state, start dividing and help repair tissue. 

See entire article here.