Ask the Scientist: Gene Therapies and Hearing

By Peter G. Barr-Gillespie, Ph.D.

A DNA double helix

National Human Genome Research Institute

Recently, Hearing Health Foundation (HHF) has received several questions regarding the Reuters report on gene therapies for hearing. There are two separate but related topics raised in this article. As the scientific research director of HHF’s Hearing Restoration Project, which since 2011 has been uncovering concrete discoveries toward a biologic cure for hearing loss and tinnitus, I want talk about each individually, and then discuss what I interpret they mean together.

The article first presents the Science Translational Medicine paper from Jeffrey Holt’s lab. This is very much a proof-of-principle report, focused on an animal model and using a time for delivery of the corrected gene that is extremely early in development (equivalent to a 5-to-6-month-old human fetus). It is important to point out that their strategy will only correct one type of genetic hearing loss and genetic hearing loss from mutations in other genes will require related but different strategies. Nevertheless, this is an exciting example of modeling gene therapy in animals, and represents a logical progression toward that goal in humans.

The article then moves on to reference the Novartis trial. For this trial, they are using a similar technical strategy, viral delivery of a gene, but they are targeting people—those who have lost their hearing through non-genetic means, such as noise damage, aging, or infections. The gene they are delivering, known as ATOH1, may stimulate production of new hair cells; it is a gene that is essential for formation of hair cells during development, and in some experimental animal models, delivery of the gene can lead to production of a few hair cells in adult ears.

That said, many people who I have talked to in the field who work with experimental models of hair cell formation using ATOH1, including members of our Hearing Restoration Project consortium, believe that this trial is premature. By and large, the animal models do not support the trial; most suggest that there will be few hair cells formed and little hearing restored. While we can hope for a little bit of hearing recovery, we are concerned about toxic responses to the gene delivery using viruses. Personally, while I think it would be truly fantastic if the Novartis trial works, at this moment in time I don’t think the rewards yet outweigh the considerable risks being imposed on a human (include safety during the procedure and potential side effects afterward).

Still, the Novartis trial will tell us about the safety of viral delivery into the ears of humans, and knowing that is critically important. I think the most likely outcome is that we will learn whether the strategy the Novartis trial used to deliver the gene is safe. Unfortunately, if we don’t see improved hearing, we won’t know why—did the gene not get to the right place, or does it just not work?

Technical aspects of gene delivery are what ties together the Novartis work and the Holt lab work. Both use viruses for delivering genes, and together the results from these and others will let us know, from a procedural standpoint, how we can deliver genes to the ear. I think it is unlikely that delivering just ATOH1 will do the trick of restoring hearing; it may be that we need to deliver other genes or to use drugs to overcome the block we see to making new hair cells.

So while these are exciting reports to hear about, especially that Novartis is actually carrying out a trial in humans, it is still premature to think that this is going to be a viable strategy for restoring hearing. This is why Hearing Health Foundation's Hearing Restoration Project is doing everything possible to accelerate the pace of its research.

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.

Taking Hair Cell Regeneration Down a Notch

By Andy Groves

Hearing Restoration Project (HRP) consortium scientists, Andy Groves and Stefan Heller had their research published in Frontiers in Cellular Neuroscience on March 31, 2015. Below is a summary of their research:

Sensorineural hearing loss is most commonly caused by the death of hair cells in the organ of Corti, and once lost, mammalian hair cells do not regenerate. In contrast, other vertebrates such as birds can regenerate hair cells by stimulating division and differentiation of neighboring supporting cells.

During the development of the inner ear, newly-born hair cells send signals to their neighbors that instruct them to not become hair cells, but to become supporting cells instead. Hair cells are the mechanosensitive cells of the ear. Supporting cells surround them and as their name implies, physically support them and help regulate some of the properties of hair cells. One of these signals is an evolutionarily ancient pathway, the Notch signaling pathway. We and others have shown that if you block the Notch signaling pathway in the cochlea or balance organs of young mice, the supporting cells no longer get the message to stay as supporting cells, and instead they transform into hair cells. This process also happens during hair cell regeneration in birds - supporting cells transform into hair cells, which then send a Notch signal to their neighbors and prevent too many hair cells from being formed.

These observations suggest that it might be possible to block Notch signaling in mature, deafened animals as a means of getting new hair cells to form. We performed a simple experiment to test this in progressively older and older animals. To our surprise, we found that once mice are more than a week old, blocking Notch signaling has no effect on the cochlea any more, and no new hair cells are made.  We showed that this was due in part to components of the Notch signaling pathway being switched off in the ear as the animals get older. Viewed this way, the Notch signaling pathway can be thought of as a “Scaffold” - it is used to allow the cochlea to be built in the first place, but is then dismantled once the cochlea becomes functional.

What does this mean? It suggests that inhibiting Notch signaling alone is unlikely to be an effective means of hair cell regeneration in mammals. It is possible that other factors will be required, and some HRP members are busy testing these other pathways right now. It will also be of great interest to understand HOW the Notch pathway is dismantled with age, whether we can exploit this in future therapies.

Read more about this research proposal here: http://hearinghealthfoundation.org/hrp-consortium-projects-groves-segil-stone.

This work was supported by Department of Defense Grant DODW81XWH-11-2-004(AKG) and Hearing Restoration Project consortium grants from the Hearing Health Foundation (AKG and SH), NIH grant DC004563 (SH), NIH grant P30DC010363 (SH, JSO), and NIHR01DC014450 (JSO).

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.

Gene Discoveries May Lead to Regeneration of Cells Needed for Hearing

By Jeffrey Norris

The researchers identified patterns of gene expression that may determine whether the ear’s inner pillar cells can give rise to new hair cells, which are key to hearing.

School of Medicine scientists have discovered biological mechanisms that appear to play a role in the regeneration of cells in the inner ear.

Over a lifetime, these cells often are damaged or die due to oxidative stress, excessive noise exposure or toxic drugs. The accumulated loss can significantly compromise hearing. Nearly one in four people ages 65-74, and half who are 75 or older, are candidates for hearing aids because of disabling hearing loss.

The discoveries could lead to new ways of evaluating, in animal models, experimental drug treatments intended to prevent hearing loss or restore hearing, and might even lead to methods for regenerating vital cells that have been lost, said Stefan Heller, PhD, professor of otolaryngology.

A paper describing the findings, as well as new methods to quickly link changes in cell function during development to molecular changes within cells, was published June 9 in Cell Reports. Heller is the senior author of the paper. Postdoctoral scholars Jöerg Waldhaus, PhD, and Robert Durruthy-Durruthy, PhD, share the lead authorship.

Discoveries by Stefan Heller and his colleagues could lead to new ways of evaluating, in animal models, experimental drug treatments intended to prevent hearing loss or restore hearing. - Steve Fisch

Sound waves striking the eardrum cause vibrations that are transmitted through tiny bones in the middle ear to fluid within the snail-shell-shaped cochlea of the inner ear. Specialized cochlear cells in a region called the organ of Corti use hairlike sensors to detect the vibrations in cochlear fluid and then trigger nerve signals that are sent to the brain.

“Compared to other senses, we know very little about how hearing works,” Heller said. “The cells are rare. We have to crack open a bone to get to them. They perish quickly, so we must work fast.” There are 120 million retinal cells in a mouse eye, Heller said, but only 3,200 hair cells in a mouse ear.

By using new techniques to rapidly and deeply probe individual cells, Heller’s team has begun to close the knowledge gap.

Molecular mysteries

Many of the biophysical properties of hair cells are understood. Different hair cells along the cochlear spiral are tuned to respond to distinct ranges of sound frequency based on differences in their electrical properties. Frequency is encoded by the place and the properties of the cells’ locations in the cochlea. This understanding has led to the development of cochlear implants to restore hearing in deaf people.

However, little is known about the molecular biology that determines how hair cells develop at specific locations and how different electrical properties arise among hair cells specialized to detect different frequencies. This makes it difficult for scientists to envision strategies to regenerate the specialized cells or to prevent their death, particularly in the high-frequency region of the cochlea, where cells are more susceptible to injury.

Once hair cells die in a mature mammal, they are not replaced. But scientists have recently determined that a supporting cell type, called the inner pillar cell, has the potential to regenerate hair cells in newborn mice.

In its new study of 2-day-old mice, Heller’s lab team measured the activity of 192 genes. The researchers determined which genes were turned on, or “expressed,” in each of 808 hair cells and supporting cells from either the apex or base of the organ of Corti. They quantified this gene expression by measuring the amount of RNA produced from each gene.

The researchers identified patterns of gene expression that may determine whether inner pillar cells can give rise to new hair cells. Similarly, they discovered gradual changes in the expression of specific genes across cells that span the organ of Corti from its base to its apex that may be crucial for the establishment and maintenance of a population of hair cells that responds to a range of sound frequencies.

Crunching the data

Using powerful number-crunching software to analyze the large amount of genetic data, Heller’s lab team accurately identified the two known types of hair cells and the seven known types of supporting cells and created a computer-generated map of their locations within the organ of Corti. They did this using only the genetic data, but then used other previously known DNA sequences to independently verify the accuracy of the cell identification and mapping.

The strategy the researchers used to predict the spatial location of cells within the organ of Corti from gene-expression data also should prove useful to biologists who study other types of cells in different organs, Heller said.

Rapid advances in single-cell gene-expression analysis are likely to supplant a standard technique called in-situ hybridization, according to Heller. The standard technique relies on labeled genetic probes to target individual genes one by one in order to identify specific cell types. The new approach of measuring hundreds of genes in parallel and reconstructing the organs in the computer appears to be more accurate and powerful.

“Molecular gradients play a key role in developmental biology, but in the past researchers depended on identifying gradients in one molecule at a time,” Heller said. “With these new techniques, we are identifying cells that, for example, have molecular characteristics of stem cells, by analyzing the expression of many genes all at once, and we know precisely where they are located.”

The research was funded by the National Institutes of Health (grants DC006167 and DC012250), the Stanford Initiative to Cure Hearing Loss and Hearing Health Foundation’s Hearing Restoration Project.

Stanford’s Department of Otolaryngology-Head & Neck Surgery also supported the work.

Republished with permission from the Stanford School of Medicine's Office of Communication & Public Affairs.

The Path to a Cure for Hearing Loss and Tinnitus

By Laura Friedman

On May 21, 2015, Hearing Health Foundation hosted its first 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.

During this inaugural research briefing, Dr. Peter Barr-Gillespie, Scientific Director, Hearing Restoration Project presented the Hearing Restoration Project (HRP). 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 14 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. 

1. History of Hearing Health Foundation

   • Founded in 1958, established reputation for pioneering breakthroughs in hearing and balance research.

     • Early supporters of the revolutionary cochlear implant. Today, over 220,000 children and adults benefit.

     • Advocated for the passage of Universal Newborn Hearing Screening legislation in the 1990s. Today, 97% of newborns are tested for hearing loss at birth.

     • The Emerging Research Grants Program provides seed funding for researchers in hearing and balance science such as discoveries in hair cell regeneration, tinnitus, hyperacusis, and Ménière’s research. 

2. The Challenge

   • In the past century, the primary treatment for hearing loss has been hearing aids and cochlear implants. While these have been very successful treatments, they have limitations.

   • For this century, we have a number of different avenues for more effective therapy. 

     • Preventing the damage to the hair cells to preserve hearing. By generating greater awareness of the effects of hearing loss, we aim encourage people of all ages to protect their ears.

     • Gene therapy, targeting those who have lost hearing due to genetic disorders.

     • The majority of people who have lost hearing have done so through noise damage or aging, and may be candidates for hair cell regeneration/restoration.

3. HRP Consortium History & Model

   • One of the key facets of the HRP’s approach is that we use three different animal models for studying hair cell regeneration

     • Two of those models, the chick and the zebrafish, show robust hair cell regeneration.

       • f you damage the hair cells of a chick or a fish, within a short time—only a day or two for the fish, a few weeks for the chick—the hair cells come back; new hair cells are formed.

         • So, that's spectacular, because it tells us that animals are capable of regenerating hair cells.

     • y contrast, the mouse is our other experimental model. Like in the human, the mouse shows no hair cell regeneration after a few days following birth.

       • You can damage the hair cells in the mouse and as far as we can tell, nothing much happens in terms of restoring hair cells. So, if we can figure out how to regenerate hair cells in the mouse, then we will be able to regenerate hair cells in people.

4. HRP Strategic Research Plan

   • Our strategic plan consists of three separate phases. We have already made a lot of progress on Phase 1 and we have initiated Phase 2:

     • Phase 1 – Discovery research:  Compare the fish, chick, and mouse to discover pro- or anti-regeneration pathways and determine supporting cell fates.

     • Phase 2 – Pathway validation: Verify pathways using fish, chick, and mouse models and describe regeneration strategies.

     • Phase 3 – Develop therapies and treatment options: Identify drugs that trigger hair cell regeneration in the mouse model.

5. Progress To-Date

   • Progress on Phase 1: We've identified a variety of candidates for hair cell regeneration and the pathways that are necessary. 

     • We have too many, so we really are continuing to use bioinformatics methods to winnow down and determine which are most important.

     • We have definitively shown, at least in the mouse, the specialized supporting cells remain.

     • We know now what our target cells are for triggering hair cell regeneration. 

   • Phase 2 has begun, but we haven’t stopped Phase 1: 

     • We've got multiple approaches to try and see whether or not we can block regeneration in the fish and chick or stimulate regeneration in the mouse.

   • Phase 3 is in sight:

     • Experimental models from Phase 2 will be used to screen for drugs—using the mouse first

6. The Next Five Years

   • With your help, we can continue to quicken the pace towards a cure. Here’s our plan for the next five years: 

     • Phase 1 will continue: more candidate generation for Phase 2

     • Phase 2 (pathway verification) already initiated in zebrafish, mouse, chick (low throughput)

     • Phase 2 must be scaled up: many more genes, combinatorial approaches; cell lines for screening

     • Phase 3 (drug screening) requires the right screening model, which will come out of Phase 2.

The Future is Very Bright – But we need your support!

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.  

Hearing Health Foundation at Partnering for Cures 2014

By Pallavi Bharadwaj

Convened by FasterCures, the Washington, D.C.-based center of the Milken Institute, the conference will bring together nearly 1,000 medical research leaders, investors and decision-makers to forge the collaborations needed to speed and improve outcomes-driven R&D. The ongoing promise of hair-cell regeneration is closer to reality than ever.

Hearing Health Foundation’s Hearing Restoring Project consortium has identified major roadblocks that have stymied the field, and has designed rational approaches to overcome these barriers.

Partnering for Cures is designed to facilitate informed investments and cultivate relationships, adapting the outcomes-oriented approach of investor conferences, and building on the networking opportunities at industry partnering meetings. In addition to innovator presentations, it also features panels that spotlight solutions to long-standing challenges in medical research.

 “We are very pleased to be present and participate in this conference. It is a unique opportunity to raise visibility for hearing loss and the path to a cure among an important audience.” says Claire Schultz, CEO HHF.

Hearing Health Foundation is one of 30 innovators presenting their cross-sector research collaboration to potential partners and funders at the conference.  Selected through a competitive proposal process, each partnership is aimed at reducing the time and cost of getting new medical solutions from discovery to patients.

“These collaborations address some of the thorniest issues in medical research using models that can be scaled and translated across diseases,” said FasterCures’ Executive Director Margaret Anderson.

“From re-imagining clinical trial infrastructure to improving and expanding data sharing, to creating the tools and resources needed to translate basic science into cures, they are accelerating the path from lab to market for novel – and needed – therapies.”

For more information and to register for the conference, go to www.partneringforcures.org

To know more on HHF’s Hearing Restoration Project presentation at Parterning for Cures 2014, please click here. 

The Danger From Noise When It Is Actually Music

By Yishane Lee

Les Paul Ambassador

John Colianni

Noise-induced hearing loss affects anyone exposed to very loud or chronic noise. It doesn’t matter if the “noise” is actually music. It has been estimated that up to half of classical orchestral musicians have hearing loss because of their work in music, practicing or performing up to eight hours a day. Sound levels onstage, no matter the music genre, can reach up to 110 decibels (dB), although it is not usually continuous. That is equivalent to a jackhammer—even if there’s a melody behind it.

Researchers at the Nofer Institute of Occupational Medicine in Poland measured the exposure for classical musicians as 81 to 90 dBA (A-weighted decibels, a unit of measure for how humans perceive sound) for 20 to 45 hours a week. In their study published in the International Journal of Occupational Safety and Ergonomics, they estimated that this exposure over the course of a career increases the risk of a hearing loss of 35 dB by 26 percent. At the greatest risk for hearing loss are those in the brass section—horn, trumpet, tuba—as well as those playing percussion, the study found.

Prolonged exposure at 85 dB (the sound of heavy traffic) will permanently damage the delicate hair cells of the inner ear, leading to hearing loss. Tinnitus, or ringing in the ears, is another potential problem. Roughly 90 percent of tinnitus cases occur with an underlying hearing loss.

Not surprisingly, rock and jazz musicians are not immune. Indeed, there are a number of well-known rock and pop musicians who have publicly discussed their hearing loss and/or tinnitus, among them Sting, Eric Clapton, Neil Young, Phil Collins, and Will.i.am.

But hearing loss due to noise (or music) is completely preventable. A related study by the Polish scientists determined that brass players benefitted the most from the use of custom-molded, silicone earplugs with acoustic filters that reduced sound levels. Woodwind, percussion, and string players also benefited.

In 2013, the Les Paul Foundation and HHF teamed up to launch the Les Paul Ambassadors program. Guitar great Les Paul was determined to find a cure for hearing loss and tinnitus, and through his foundation’s support of HHF’s Hearing Restoration Project, an international research consortium of top hearing scientists, we have the opportunity to find a cure. Learn about the program and the first Ambassador, Lou Pallo, as well as our other Ambassadors saxophonist Chris Potter and jazz pianist John Colianni.

Learn more about NIHL and its risk factors, treatment, and prevention in our new Summer issue of Hearing Health magazine.

Protect Your Ears This July 4!

By Tara Guastella

The Fourth of July is a great time for barbecues, trips to the beach, and spending time with friends and family. But fireworks and firecrackers, ubiquitous to many Independence Day celebrations, not only are a potential fire hazard, they also can do permanent damage to one's ears.

These beautiful spectacles measure between 140 and 165 decibels. This means that even one minute of exposure to them can cause immediate, permanent hearing loss.  

When exposed to sounds that are too loud or loud sounds that last a long time, such as a fireworks display, delicate cells in our inner ear can be damaged, causing noise-induced hearing loss (NIHL). These sensory hair cells in the inner ear convert sound energy into electrical signals that travel to the brain. Once damaged, our hair cells cannot grow back.

While you're enjoying summertime get-togethers, remember to pack ear protection, such as earplugs or earmuffs, and don't forget a pair for the kids! Also remember to Walk, Block, and Turn:

Walk away from loud sounds.

Block loud sounds with ear protection.

Turn the volume down (when you can control it).

Learn more about how loud is too loud today.

We wish you and your family a happy, healthy, and safe Fourth of July holiday!

Stay tuned for more about NIHL—its symptoms, prevention, treatment, and related conditions such as tinnitus—in the upcoming Summer issue of Hearing Health magazine. Get a free subscription by signing up here. We also wish to salute and thank our military service members, who are disproportionately affected by hearing loss and tinnitus as a result of their service, on this American holiday.

What Does a Chicken Have to Do with Hearing Loss?

http://aubankaitis.com/2014/05/14/chicken-and-hearing-loss/

What would you think if someone told you that a baby chick holds the cure for hearing loss? One of the keys to restoring normal hearing in humans is cochlear hair cell regeneration, something that most animals other than mammals, including chickens, can do.  The Hearing Health Foundation recently launched a new public service announcement (PSA) called “Chirp the News” which features a baby chick with hearing loss who goes on to live a happy, normal-hearing life. After viewing it, my curiosity was piqued. I had an opportunity to ask Shari Eberts, Chairman of the HHF’s Board of Directors, a couple of questions and wanted to share what I learned.

Question: For those that are not familiar with your organization, what is the Hearing Health Foundation and/or what is the Foundation’s mission?

Hearing Health Foundation (HHF) is the largest private funder of hearing research, with a mission to prevent and cure hearing loss and tinnitus through groundbreaking research. Since 1958 HHF has given away millions of dollars to hearing and balance research, including work that led to cochlear implant technology and now through the Hearing Restoration Project is working on a cure for hearing loss and tinnitus. Hearing Health Foundation also publishes Hearing Health magazine, a free consumer resource on hearing loss and related technology, research, and products.

Question: Shari, it is my understanding that you acquired a hearing loss in your late 20′s.  Can you tell me a little bit about how your hearing loss was identified, the cause of your hearing loss, and how it has impacted your personal and professional lives?  

I first noticed my hearing loss in business school. Students were participating in class, and I would sometimes miss their comments, particularly the funny ones that were made almost as an aside. My father and my grandmother both had a hearing loss, so I figured I should get tested. It turns out that I had a mild hearing loss in both ears. The loss is genetic and is centered in the mid-range or speech frequencies. Luckily, my high pitch hearing is almost perfect. My loss has gotten progressively worse each year since business school, but I am able to manage it with hearing aids and by advocating for myself. At first, I didn’t want to admit that I had a hearing loss, and I hid it from others, but eventually I began to realize how much better my life could be if I used my hearing aids, and I began wearing them all the time. I am glad that I do.

As someone who lives with hearing loss everyday, I am personally thrilled with the prospects for a cure. Life with hearing loss can be frustrating.  Sometimes you miss the joke when everyone else is laughing and sometimes you miss important information because you don’t hear it. Supportive family and friends can make living with hearing loss easier, but a genuine cure would be life changing. After having met and worked with our consortium scientists for these past two years, I am confident that we will have a cure in my lifetime. I am counting the days.

Question: Knowing that you acquired a hearing loss in your late 20′s, it makes sense that you would be passionate about educating people about hearing loss and learning about various research focusing on a cure. With so many different organizations dedicated to hearing loss, what made you specifically gravitate toward Hearing Health Foundation? What makes this organization so unique?

HHF’s approach to research is unique and I believe it will shorten the timeline to a cure. For years, scientific research has been conducted in relative isolation—one researcher or one institution working alone to tackle a major health issue. HHF developed the HRP Consortium model to do things differently. Our HRP scientists work on research projects together, share their unpublished data and tools, and collaborate on the development and refinement of the HRP’s strategic research plan. The group meets bi-annually in person, monthly by conference call, and communicates frequently by email. This continual dialogue is helping to eliminate repetitive work across the team, saving time and research dollars, and most importantly, accelerating the timetable to a cure.

Our HRP Consortium is the dream team of hair cell regeneration, comprising the best auditory scientists at leading institutions worldwide such as Harvard and Stanford. With more than 200 years of combined experience in hearing research, the HRP Consortium publishes widely (over 400 published papers among them) and have well established labs (receiving over 600 NIH grants combined). We have every confidence we have the right team in place, and the right model to accelerate the timeline to a cure.

Question: The Hearing Health Foundation was established in 1958 and had been seeking donations from the public to help fund “groundbreaking research” for the prevention of and cure for hearing loss. Can you provide a historical synopsis of some of the more significant research achieved by the Foundation since its inception?

HHF’s founder, Collette Ramsey Baker, was steadfast in her support of funding for new technologies and treatments for hearing loss. For example, back in the 1960s, HHF began funding research into cochlear implant technology. HHF’s founder, Collette Ramsey Baker, prevailed despite objections and doubts from supporters that she was wasting money. Cochlear implants have proven to be a valuable treatment option for people with profound hearing loss, benefiting 125,000 people in the U.S. and 300,000 people worldwide. HHF has also research that led to the development of many of today’s standard treatments for otosclerosis (abnormal bone growth in the ear) and ear infections.   In the 1990s, HHF was a leader in advocating for Universal Newborn Hearing Screening legislation, which increased testing from 5% of newborns to 94% by 2007. In 2011, HHF launched our most important project yet, the Hearing Restoration Project (HRP),  which aims to discover a biological cure for hearing loss and tinnitus.

Question: What research is the Foundation currently working on that is anticipated to have a significant and/or practical impact on hearing loss prevention and/or cure within the next 10 years?

HHF officially launched its Hearing Restoration Project (HRP) in 2011 and is currently funding 5 projects from its consortium scientists, but the initial discovery that led to the HRP came many years before. Many types of hearing loss result from damage to the delicate hair cells of the inner ear. Humans can’t regrow these cells—but in a game-changing breakthrough in 1987, HHF-funded scientists discovered that birds can. While studying how drugs that are known to cause hearing damage affect the tiny sensory cells in the ear, these scientists needed to permanently damage a chicken’s hair cells. For 10 days, research assistants administered a common antibiotic, known to cause hearing loss, to laboratory chickens. On day 11 many of the hair cells were lost and a few days later, even more were lost. Surprisingly, when the scientists looked three weeks later, almost all the hair cells had returned.  They didn’t believe these results so they did the experiment again and again. Sure enough, chickens can naturally regenerate their inner ear hair cells, restoring their hearing after damage.

The amazing thing is that regeneration happens naturally and very robustly in almost all animals – mammals are the exception. This makes HHF and the researchers confident that we will find a way to stimulate this regeneration in mammals, including humans.

The HRP consortium of scientists has developed a strategic research plan to develop a cure for hearing loss and tinnitus in 10 years. This three-phase plan starts with discovery research and culminates in clinical trials. The plan, developed specifically by the HRP scientists and updated to incorporate new findings and approaches, is a living document meant to guide but not limit the work. Relevance to this strategic plan is one of the criteria for a project to receive HRP funding.

The HRP is currently in Phase I of its strategic research plan (years 1-5). This first phase focuses on searching for the genes or series of genes that trigger natural regeneration of hair cells in animals such as birds and zebrafish. This phase will also examine which genes in mammals prevent the natural regeneration of hair cells. Finally, Phase I will determine the types of cells in mammals’ ears that could serve as available targets for regeneration therapies. Phase II (years 3-8) starts with the residual cells that remain in a mammal’s inner ear after hearing loss and uses the genes identified in Phase I to trigger hair cell regeneration. In Phase III (years 8-10), the HRP Consortium will partner with a pharmaceutical or other company to develop drugs that mimic the identified genes, resulting in a regenerative therapy.

Question: How can audiologists and other hearing health care providers get involved with the Hearing Health Foundation?

HHF is always eager to partner with hearing health care providers! In fact, we have developed a brochure specifically for use by hearing health care providers that includes important information for their patients about how hearing works, the types of hearing loss, and common treatment solutions. It also lets patients know about the resources HHF can offer, like its free quarterly magazine. Hearing Health Magazine is the award-winning leading consumer publication on hearing loss filled with the latest on research breakthroughs, strategies to manage hearing loss, personal stories, hearing technologies and products and features on seniors, pediatrics, parents, musicians, veterans and more!   Please feel free to contact us at info@hhf.org if you are a hearing health care professional and would like copies of our patient brochure or magazine.

Question: How can the general public support the mission and goals of the Hearing Health Foundation?

There are lots of ways for people to learn more about HHF and help support our research for a cure for hearing loss and tinnitus.

1. Visit our website to learn more

2. Stay up to date on all the latest news by liking us on Facebook and following us on Twitter

3. Sign up for our informative monthly e-newsletter

4. Subscribe to Hearing Health Magazine, our award-winning leading consumer publication on hearing loss. Get the latest on research breakthroughs, strategies to manage hearing loss, personal stories, hearing technologies and products, and features on seniors, pediatrics, veterans, musicians and more.

5. Inspire others by sharing your personal story and draw comfort from the stories of others

6. Create a fundraising event or giving page

7. Make a tribute gift to honor a loved one with hearing loss or a favorite audiologist

8. Support our work with a tax-deductible donation

Shari Eberts is Chairman of the Board of Directors at the Hearing Health Foundation, an organization whose mission is to prevent and cure hearing loss and tinnitus via collaborative, groundbreaking research. She received her BS from Duke University in 1990 and MBA from the Harvard Business School in 1995. Previously employed by Goldman Sachs and McKinsey & Company, Shari spent 13 years at J.P. Morgan in the capacity of a senior equity analyst (broadlines retail) and, most recently as Associate Director of U.S. Equity Research.  This mom of two and former Wall-Streeter joined HHR in 2010 and has committed herself to supporting the search for a cure for hearing loss and tinnitus.

10 Fun Easter Facts From Our Mascot, the Baby Chick

By Tara Guastella

As you’ve seen on our previous blog posts, a baby chicken may hold the key to a cure for hearing loss and tinnitus. Chickens have the remarkable ability to restore their own hearing naturally by regrowing inner ear hair cells that, once they are damaged, lead to hearing loss.

In fact, most animals other than mammals can regenerate these delicate cells, regaining their hearing after the cells are damaged by noise, age, or ototoxic drugs. This is the basis for the work of our Hearing Restoration Project, which is aiming to translate this remarkable capacity to humans.

So with Easter right around the corner we thought our mascot, the baby chick, would provide you with 10 fun Easter facts:

1) Easter is the celebration of the resurrection of Christ three days after his death. It is the oldest Christian holiday and the most important day of the church year.

2) Like rabbits and hares, eggs and chicks are often associated with Easter because, in pagan times, they were signs of fertility and new life.

3) After Halloween, Easter is the biggest candy-consuming holiday. About 120 million pounds of candy are bought each year, enough to fill four dump trucks.

4) Roughly 90 million chocolate bunnies, 91.4 billion eggs, and 700 million Peeps (sugar-coated marshmallow sweets) are produced each year in the United States.

5) About 76 percent of people eat the ears on chocolate bunnies first.

6) Half of the states in the United States have banned the practice of dyeing live chicks for Easter.

7) To help Americans in need, egg farmers across the country are donating more than 11 million eggs to food banks across the nation.

8) The White House hosts an Easter Egg Roll on its Front Lawn each year. This tradition was started by President Rutherford B. Hayes in 1878.

9) Like many holidays, Easter also has its own catchy tunes or carols such as “Here Comes Peter Cottontail” and “Easter Parade.”

10) Some 16 billion jelly beans are made specifically for Easter, which is enough to fill a plastic egg the size of a nine-story building.

If you celebrate Easter this coming Sunday and enjoy chick-shaped marshmallows or other fun Easter candy, remember that it is a little chick that is helping us to achieve the next great medical breakthrough: curing hearing loss and tinnitus.

Is A Cure for Hearing Loss Possible?

By Shanna Groves

When Hearing Health Foundation approached Lipreading Mom about doing a story about curing hearing loss, I admit I was skeptical. Is a cure really possible? And if it is, would it benefit those who have embraced their deafness? I have many friends who haven’t been able to hear all their lives, and they lead very full lives. So why the need to change that?

Lipreading Mom recently conducted an email interview with Shari Eberts, Chairman of the Hearing Health Foundation’s (HHF) Board of Directors, about HHF’s research into hearing loss.

When did research into a hearing loss ‘cure’ first get support from the Hearing Health Foundation?

HHF’s founder, Collette Ramsey Baker, was steadfast in her support of funding for new technologies and treatments for hearing loss, despite objections and doubts from supporters and those in the industry. Because of that commitment, HHF has been a leader in driving new innovations and treatments for people with hearing loss for more than fifty years. This includes funding research that led to the development of cochlear implants and many of today’s standard treatments for otosclerosis (abnormal bone growth in the ear) and ear infections. Today, HHF continues to support groundbreaking research in hearing, through the search for a biological cure for hearing loss and tinnitus through its Hearing Restoration Project (HRP).

HRP officially launched in 2011 and is currently funding five projects from its consortium scientists, but the initial discovery that led to the HRP came many years before. Many types of hearing loss result from damage to the delicate hair cells of the inner ear. Humans can’t regrow these cells—but in a game-changing breakthrough in 1987, HHF-funded scientists discovered that birds can.

While studying how drugs that are known to cause hearing damage affect the tiny sensory cells in the ear, these scientists needed to permanently damage a chicken’s hair cells. For 10 days, research assistants administered a common antibiotic, known to cause hearing loss, to laboratory chickens. On day 11 many of the hair cells were lost and a few days later, even more were lost. Surprisingly, when the scientists looked three weeks later, almost all the hair cells had returned. They didn’t believe these results so they did the experiment again and again. Sure enough, chickens can naturally regenerate their inner ear hair cells, restoring their hearing after damage.

Our HRP Consortium is the dream team of hair cell regeneration, comprising the best auditory scientists at leading institutions worldwide such as Harvard and Stanford. With more than 200 years of combined experience in hearing research, the HRP Consortium publishes widely (over 400 published papers among them) and have well established labs (receiving over 600 NIH grants combined). We have every confidence we have the right team in place, and the right model to accelerate the timeline to a cure.

For years, scientific research has been conducted in relative isolation—one researcher or one institution working alone to tackle a major health issue. HHF developed the HRP Consortium model to do things differently. Our HRP scientists work on research projects together, share their unpublished data and tools, and collaborate on the development and refinement of the HRP’s strategic research plan. The group meets bi-annually in person, monthly by conference call, and communicates frequently by email. This continual dialogue is helping to eliminate repetitive work across the team, saving time and research dollars, and most importantly, accelerating the timetable to a cure.

Even though we are in the early stages of the research, we think it is very important that the public learn about our efforts. We want them to know that there is hope for a cure, and that there are researchers who consider curing hearing loss and tinnitus to be their life’s most important work. We hope our marketing efforts will help bring attention to the issue, raise awareness of the prospects for a cure and inspire other scientists and laypeople to join us in our support of this important research, so that we can find the cure as soon as possible.

But what about people with different forms of hearing loss: How can these research findings affect people born with hearing loss? People with late-in-life hearing loss?

There are two broad forms of hearing loss:

Conductive Hearing Loss is caused by any condition that blocks or impedes the conveyance of sound through the outer or middle ear. The result is a reduction in the sound intensity that reaches the cochlea. Common causes include ear infections, a perforation in the eardrum, or even buildup of earwax. Generally, conductive hearing loss can be treated with a complete or partial improvement in hearing.

Sensorineural Hearing Loss commonly occurs due to an injury or degenerative change in the inner ear and is currently permanent.

Damage to the hair cells in the inner ear is the most common type of sensorineural hearing loss. The Hearing Restoration Project is focused on the cure for sensorineural hearing loss, through the regeneration of these damaged inner ear hair cells, and with it the regeneration of hearing. Most types of age-related hearing loss and noise-induced hearing loss are caused by damage to these hair cells, making the HRP research applicable to most types of acquired hearing loss.

The amazing thing is that regeneration happens naturally and very robustly in almost all animals – mammals are the exception. This makes HHF and the researchers confident that we will find a way to stimulate this regeneration in mammals, including humans. While ten years may seem like a long time, and it is for someone like myself who lives with hearing loss every day, it is realistically within my lifetime, and that gives me hope and excites me for the future. While we wait for the cure, we encourage people with hearing loss to seek treatment for the condition through hearing aids or other means, so that they can enjoy the highest quality of life possible, while they wait.

What about tinnitus: How can these findings help the millions of people worldwide with ringing in their ears?

With 90% of tinnitus (ringing in the ears) occurring with an underlying hearing loss, a cure for hearing loss is also likely to be a cure for tinnitus. In a recent article in Hearing Health magazine, HHF’s free quarterly publication on all things related to hearing loss, this topic was discussed in detail. Some of the high points are detailed below:

There is certainly evidence that the restoration of hearing can alleviate tinnitus. For example, tinnitus can be induced by exposure to loud sounds that result in the temporary loss of hearing, which is experienced by many after attending a loud event like a rock concert. In most such cases, the tinnitus disappears as the hearing recovers.

Another example is the response of patients for whom otosclerosis (an abnormal bone growth in the middle ear) has induced a conductive hearing loss. Many such patients with this hereditary condition experience tinnitus. However, when their hearing is restored through a surgical procedure, many report an improvement in tinnitus.

Finally, there have been several reports that patients, after receiving a cochlear implant, experience a significant reduction in their tinnitus. Interestingly, in some cases this tinnitus suppression continues for several hours after the implant is turned off. Though the mechanism by which cochlear implants may suppress tinnitus is not clear, these observations all suggest that restoring function to the auditory system may be very helpful for tinnitus in a variety of ways.

There may be skepticism about ‘curing’ hearing loss. How would you respond to criticism toward curing hearing loss or deafness? For example, the Deaf community has a strong cultural identity shaped by living with deafness. Some would argue that a person’s deafness is what makes him/her unique, so why change it?

Hearing Health Foundation respects everyone’s individual choices and beliefs as relates to their hearing loss. For those interested in a biological cure, we are proud to be working on one.

This article was republished with permission.