Hearing Restoration Project cure for Hearing Loss

Our Mission: Hearing Health Foundation is the largest private funder of hearing research, with a mission to prevent and cure hearing loss through groundbreaking research.  Since 1958 Hearing Health Foundation has given nearly $30 million to hearing and balance research, including work that led to cochlear implant technology.  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 within the next ten years. Hearing Health Foundation also publishes Hearing Health magazine, a free consumer resource on hearing loss and related technology, research, and products.

With your help, we can make hearing loss a thing of the past.

http://hearinghealthfoundation.org/our-research-plan

Join or donate for the upcoming Nov 3, 2013 marathon race in NY for the cure on hearing loss.

Or read their latest Fall magazine.

If cure for hearing loss isn't your thing then perhaps help find a cure for vision loss where blind mice own blindness were successfully restored with stem cells. The restoration of blindness is now within our lifetime.

When hearing aids benefit deaf/hh children

While growing up at around age 6 or 7 years old I received my second hearing aid after having worn out my first one that I had since age 2 1/2 years old. It wasn’t the behind the ear hearing aid but a body aid. I’ve gotten a lot of good mileage out of my old hearing aids. Obviously so because without that hearing aid of mine I wouldn’t be here where I am today. Successful and thankful with what residual hearing I had left though not perfect. It has helped me with my ongoing ragtime piano playing ever since I was seven years old. I am proud of all of my past hearing aids I've worn because each one of them I have used it with great benefit. I even remembered how I enjoyed using my hearing aid when I was little because it gave me access to the world of sound, music and voice around me. It took awhile to realize that hearing is a precious thing and a gift even if I couldn't hear perfectly. It was perfect enough for me with the help from the hearing aid technology. I even have a drawing I did when I was around 5 or 6 years old showing me as a happy kid wearing the body hearing aid. There was a time or two when I didn't like wearing my hearing aid but that phase quickly passed once I realized how important my hearing aids were to me.

Below is a picture of my old body hearing aid inside a protective case . It’s a Zenith. Along with a Battery Membership card, and a special yellow button that has as special message.

It’s so well preserved though I am sure my old hearing aid can work if I put in the right kind of battery.

Pretty fascinating on how hearing aids (even cochlear implants) have helped so many people with hearing loss. Isn’t technology great? And perhaps someday hearing aids will become obsolete with the help of biotechnology that have the ability to restore sensorineural hearing loss. It's not a matter of if but when. That day is coming whether in my lifetime or not.

Stem cells for hearing loss gets closer

We're now at a point where researchers can take your own skin cells and convert them into  regenerative cells to help re-grow damaged hair cells within your own inner ear and restore hearing loss.  Clinical trials begin soon over the next 10 years. And believe me, this will come a lot sooner rather than later with the way medical researchers are advancing at a rapid rate to reach this unprecedented milestone.  Not only would this be hugely beneficial to recipients of hearing loss for millions of children and adults researchers also recognize the extremely lucrative potential when it comes to regenerative medicine.

A cure is worth it....or not?

The now famous news of gerbils whose own deafness were nearly restored using stem cells to treat their hearing loss will probably be the turning point in medical history on the path to restore hearing loss for the human population. It'll be a matter of time until we are able to reverse hearing loss in humans using stem cells. This would bode good news amoung the many 35 million people with hearing loss in the United States. Not to mention a highly lucrative field once it gets going. I would say that 98% of the population with hearing loss would more than likely accept this news as positive and that many would accept this medical procedure for themselves instead of opting for hearing aids or cochlear implants.  The remaining population (2% of the total population of people with hearing loss) consisting of some 600,000 or so culturally deaf people would either accept this news positively, negatively or remain on the fence about it. 

Now, being culturally deaf does not mean all of them would be against this medical choice on choosing to restore hearing loss should a medical procedure actually becomes available.

Being culturally deaf also does not mean a person has to be born with a hearing loss first and learn to sign early on. Many late deafened people are culturally deaf.

Being culturally deaf does not mean a person must not wear hearing aids or cochlear implants. Or that he or she must go "au naturel" instead of relying on hearing aids or cochlear implants. Many culturally deaf people wear hearing aids or cochlear implants.

I would say that those culturally deaf people who wear hearing aids or cochlear implants would be more than likely support for the restoration of hearing loss in people using stem cells than not should it becomes available and a reality. And that it becomes a medical choice and not a law.

Would I take it?

Sure.

It'd be for my right ear only since that is the only ear that can hear well and it wouldn't take much for my ear adjust to the new clarity of sound in its natural splendor without my hearing aid for the first time in my life. As for my left ear I don't wear a hearing aid since I do not derive any real benefit due to the severe/profound hearing loss I have. Although the possibility does exist that I could "train" my left ear for years with a hearing aid and then opt for the procedure. Who knows?  I'll just bide my time and if this medical procedure does happen during my lifetime then I'll have the opportunity to make an important decision.

I'd say it's worth it for myself to see that my hearing loss in my right ear be restored and be fully functional. Others will have a different opinion about it and many would personally be against it. So, for those who are shaking their heads, if there's a cure for blindness, then please do not opt for it for yourself or for your baby if you have a serious vision problem. After all, you wouldn't want to look like a hypocrite for being against on the restoration of hearing but accept the restoration of sight?  Can't have one's cake and eat it, too.

Tale of a hypocrisy and Marlee Matlin

News is still reverberating about the latest development on the treatment for hearing loss when gerbils in a laboratory were deliberately made deaf had their hearing loss reversed using human embryonic stem cells.

The stem cells were used to make immature nerve cells. Those were then transplanted into the deaf ears of 18 gerbils.  

Ten weeks later, the rodents' hearing ability had improved by an average of 46 percent, with recovery ranging from modest to almost complete, the researchers reported.

Not totally a cure yet but certainly in that direction that hearing loss or nerve deafness can certainly be reversed.  A key conceptual working model demonstrated here using the medical knowledge and technique that can readily be applied on humans, and that adult stem cells would be used as well.

With that news Marlee Matlin was asked about the medical break through with stem cells.

“Think about this. What if someone told you that you could’ve been made different than the content person you are with genetics. Would you? Now think how millions of deaf people who lead productive lives, would feel when told that babies born deaf can be ‘cured’.”  

Matlin also told followers, “Be proud of who you are, regardless of what people think is a “handicap” or “normal.” Normal is what you want to be. Don’t let anyone tell you who or what you should be.”

The problem here in reference to the yellow highlighted part above is that parents of deaf or hard of hearing babies have the parental and legal right to make an informed decision regarding the use of stem cells to reverse their baby's hearing loss once that procedure becomes safely available. They can either go forward with the treatment or reject it. And odds are quite high that most parents will prefer to opt for the stem cells treatment than to forego it. This would be absolutely true when we have someday stem cells that can reverse blindness or low vision condition would parents opt for that treatment for their blind or low vision babies. Chances are high that culturally deaf parents of a deaf/blind baby would stop at nothing to see that their baby get the stem cells treatment for blindness but not deafness when they value more on vision than hearing. Just as any parents would see any other medical condition would they like to see reversed for their babies. A hypocrisy in action folks. In fact, there wouldn't be any outcry from the culturally deaf community if there was a stem cells treatment that could reverse vision loss in babies, children, teens and adults.

But here's another "hypocrisy" about Marlee Matlin's. She champions for the donation of free hearing aids to deaf and hard of children around the world with the Starkey Hearing Foundation. I think this is great! In fact she regularly attends this annual gala event called, "So the world may hear." This year's gala 2012 event raised a record $7.6 million dollars and had many notable celebrities like:

President Bill Clinton, Maria Bello, Robin Williams, Marlee Matlin, Billy Crystal, Forest Whitaker and Sammy Sosa attended this year's Awards Gala, which raised a record $7.6M. But the biggest star was the power of hearing.

And yet she warns us about stem cells treatment for hearing loss? What's her stance on stem cells treatment for curing blindness or low vision in babies?

The power of hearing. Yes, that's a strong draw and reason for people to support on finding ways to alleviate hearing loss. Even Lou Ferrigno (who received his Esteem Envoy implantable hearing aid) and Gary Busey (Marlee Matlin confronted Busey his hearing loss that he needs to get a hearing aid seen in the Celebrity Apprentice show by which $1 million dollar was raised for Starkey Hearing Foundation) who were at the gala event as well. It won't be long until celebrities support for the cure for hearing loss through stem cells treatment.

Now, here's the irony.

Marlee Matlin is perhaps unwittingly helping people understand and value the power of hearing for several years by attending the "So the World May Hear" gala annual event that began in 1984 and supports the distribution of free hearing aids to children around the world. Deaf and hard of hearing children around the world with their free hearing aids are getting a taste of that power of hearing and experience the joy of being able to hear sound.  And with that taste in mind already experienced they certainly would want to opt for stem cells treatment to reverse their own hearing loss if they had that choice and the means to do so.

Now, I'm sure some in the culturally deaf community would express their shock and outrage over this but let me remind some of the culturally deaf people that the bulk of their community do wear hearing aids and cochlear implants. Look around whenever you are with a group of deaf and hard of hearing friends. How many of them are wearing hearing aids and cochlear implants? Why are they wearing hearing aids? Why cochlear implants? What makes you think they would not want to opt for stem cells treatment to reverse or simply improve their own hearing?  What makes you think they would not support parental rights to make an informed decision to provide stem cells treatment to reverse hearing loss for their deaf/hh babies?

So, who is really showing the hypocrisy here?

Is the death of culturally deaf community that far behind? Or is it just plain paranoia? 

The New Stem Cells Generation - going from deaf to hearing.

The stuff of science fiction has now arrived where Madeline becomes the first of many toddlers born deaf to get their first cord- blood stem cells treatments in the effort to restore their sensorineural hearing loss. This procedure continues to show success and Madeline's hearing continues to improve.

Madeline Cooper, who is nearly 2 years old, is the first person in the nation to take part in a new Food and Drug Administration-approved study of stem cell treatment of sensorineural hearing loss.

Children’s Memorial Hermann Hospital in Houston and California-based Cord Blood Registry will eventually enroll another nine children in this first phase of the study.

Unlike some advertised stem cell treatments that may cost patients tens of thousands of dollars — and, according to critics, are little more than quackery — the Conners paid nothing to take part and have been promised no specific results.

The process is simple: Doctors inject the children with stem cells from their own stored umbilical cords. Because it is the patients’ own blood, there is little chance the treatment will produce side effects.

In theory, the treatment will adjust patients’ immune systems to encourage their bodies to repair themselves. In truth, researchers have no idea if it will work.

Earlier Italian studies on mice concluded that stem cells may help the body repair damaged cells in the ear and restore some hearing in these types of cases, according to a 2008 published report in the University of South Florida journal Cell Transplantation.

Madeline's hearing loss has gone from extreme to moderate hearing loss since undergoing the treatment. A definite improvement. This stem cell treatment will continue and may take a few years to help fully restore hearing loss. We will not know how fast this rate of restoration will take place over the next several months to a few years. We will know more once the Cooper family head back to Texas in July, and again in January of 2013, to get a more detailed assessment of Madeline’s improvement.

This will be the start of a new generation of cord-blood stem cell babies and toddlers born deaf to become, in theory, hearing with the help of their own blood-cord stem cells. An exciting era in regenerative medicine for parents of deaf children. This new cord-blood stem cell procedure was covered in my blog piece in January 2012 for your review.

Once proved successful parents of deaf babies will see this as the preferred treatment to help restore their child's hearing loss. This will certainly be a much preferable route (and cheaper, too) than to choose cochlear implant.  Now the question becomes, will this eventually destroy the cochlear implant industry? Only time will tell.

FDA Approves Phase I Stem Cells for Babies with Hearing Loss

UPDATE: Check out one of the deaf children's progress here.

There's a new FDA approved cord blood stem cell safety study to be done on deaf children age 6 weeks to 18 months who acquired sensorineural hearing loss (nonsyndromic deafness). This is a year long study.

Children’s Memorial Hermann Hospital and Cord Blood Registry® (CBR) are launching the first FDA-approved, Phase I safety study on the use of cord blood stem cells to treat children with sensorineural hearing loss.

The study, which will use patients’ stem cells from their own stored umbilical cord blood, is the first-of-its-kind, and has the potential to restore hearing. This follows evidence from published laboratory studies that cord blood helps repair damaged organs in the inner ear.

The year-long study will follow 10 children, ages 6 weeks to 18 months, who have sustained post-birth hearing loss. Children who are deaf as a result of a genetic anomaly or syndrome are not eligible. To ensure consistency in cord blood stem cell processing, storage, and release for infusion, CBR is the only stem cell bank providing clients for the study.

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Researchers will obtain and process the patients’ stored cord blood for treatment. The cells then will be given to the patients via IV infusion, and patients will be observed for several hours in the hospital.

Patients will return to the hospital to repeat all tests except the MRI at one month and one year, and all tests with an MRI at six months.

“This study is exciting because it might offer a non-surgical option for some children with profound loss,” Linda Baumgartner said. “More importantly, this is the first treatment with the potential to restore normal hearing.”

The new Phase 1 study already began in April 2011 and will end April 2015 although the collection on final study results will end in April 2014. This study will use stem cells from cord blood saved at a cord bank after a baby is born. Stem cells from the deaf baby's cord blood will be done via IV transfusion back into the child. Parents whose deaf children qualify for the trial study will decide whether to try the new procedure or not.

Since this news came out it didn't take long for an outcry in the form of a petition for culturally deaf people and others to decry the IV transfusion stem cell study as:

....inhumane and unethical and condemn this hospital, corporation, other collaborators and the FDA for treating our Deaf infants in this experiment as their guinea pigs.

The original petitioner went so far as to claim such procedure as dangerous and that it carries "long lasting risks" but never elaborated on exactly what those "long lasting risks" are. However, there is a similar but previous safety study using cord blood stem cells on children with cerebral palsy age 1 to 12 years old that began in January 2010. Final data collection on outcome ends in February 2012 which is next month.  Ironically, there appears to be no objection from any particular protest group for this IV transfusion safety study for babies and older on reversing their cerebral palsy condition.

The MCG team follows on the heels of a pilot study at Duke University which is exploring if cord blood can be readily applied to infant’s with palsy like symptoms in the first two weeks after birth. Both teams are looking to verify the anecdotal evidence that cord stem cells can be used to ‘cure’ CP children. If successful, the work at MCG and Duke University may provide parents with hope that storing a child’s cord blood could be an effective shield against cerebral palsy.

Cord blood IV transfusions have been done in children and adults.

To be safe, most cord blood transplants done so far have been in children and smaller adults. Researchers are now looking for ways to use cord blood for transplants in larger adults.

This first ever FDA approved study on hearing restoration (sensorineural hearing loss) for deaf children describes the study:

Acquired sensorineural hearing loss is characterized by a loss of functioning hair cells in the Organ of Corti, with greater hair cell loss correlating with more severe hearing impairment. Children with sensorineural hearing loss experience difficulty developing normal language which usually leads to poor academic and social development. Currently, there are no reparative therapeutic options available, and treatments are designed to augment the diminished function of the injured Organ of Corti.


Pre-clinical data suggest progenitor cell infusions may enhance intrinsic repair mechanisms in the Organ of Corti which may restore hair cells. This treatment could ultimately lead to hearing improvement. Human umbilical cord blood (hUCB) is an available, autologous, stored progenitor cell population available for potential therapeutic use. The primary objective of this study is to determine the safety of autologous hUCB infusion in children with acquired hearing loss. The secondary objective is to determine if functional, physiologic and anatomic outcomes are improved following hUCB treatment in this patient population.

This may turn out to be a promising result since it says that pre-clinical data suggest that such a procedure may restore hair cells thus lead to hearing improvement. This is obviously a much more natural process, if proved successful, involving the baby's own stem cells from the baby's umbilical cord (cord blood) could ultimately turn out to be much better option than cochlear implants. In fact, if successful would shorten the prediction time scale to seveal years on a reversing sensorineural hearing loss instead of a few decades away. Over a year and half a ago in my blog I commented that in 2004 a prediction was made that an application for hearing loss rather than a cure could be at least twenty years away (or by 2024) by Dr. Rubel. In another article in 2004 Dr. Rivolta said a cure could be possible in 10 to 15 years away (by 2014 to 2019). In 2009 in the Department of Otolaryngology University of Miami Ear Institute's newsletter quoted believing they could be 10 years away (or 2019) from a cure at the earliest possible timeline. But most scientists seem to concur that a cure or a very applicable treatment on reversing hearing loss would be seen within their (the scientists') lifetime...and that's not very far off folks.

Dream Team of Scientists Collaborate on Cure for Hearing Loss in Ten Years

Looks like a goal is set by a dream team of stem cell scientists and researchers for hearing loss that sensorineural (nerve deafness) hearing loss would be cured through biological means in ten years from now.

The Hearing Restoration Project (HRP) is a Consortium made up of some of the most talented, creative, inspired researchers in the area of cell regeneration in the ear, both mid-level and senior-level investigators whose work has already contributed significantly to the field. The Consortium is an alliance of scientists working collaboratively and interactively with the goal of developing a biologic cure for severe sensorineural hearing loss in the next decade. At the Summit, experts discussed an area of particular promise: hair cell regeneration in humans.


"Hearing Health Foundation understands that it is important to invest in researchers who are working in hair cell regeneration as this will be an important component in the next wave of hearing treatments," said Andrea Boidman, Executive Director of Hearing Health Foundation. "Hearing research needs to be a national priority, and people need to know that there are treatments on the horizon that could really impact their lives."

In fact, a summit was held in New York city today.

Hearing Restoration Initiative 2011 Summit
The Promise of Cell Regeneration
On Monday, October 3, 2011, DRF will host a Summit on Hearing Restoration at the New York Academy of Medicine, at 103rd Street and 5th Avenue in New York City. As a part of DRF's Hearing Restoration Initiative, this landmark summit brings together the leaders in the field of cell regeneration research in the ear to discuss what it is, where it is going and how one day soon a therapy for cell regeneration in the ear will be a reality.


Doors open at 12pm.
Exhibit Hall hours 12pm to 6pm


Exhibitors include:
- AARP - Caption Call - Caption First


This event is open to the public. Online registration has closed, but you will be able to register on-site.


ASL and CART services will be provided.
For more information, please contact Melody Seiling at mseiling@drf.org.

That's pretty ambitious to say that a biological cure using stem cells for sensorineural hearing loss would happen in ten years time. Certainly would be loads better than an Esteem implantable hearing aid.

The timeline predictions on the cure for hearing loss

In a recent news about stem cells that were made grow into ear hair cells in mouse got me thinking on the scientists/researchers attempt to predict when a cure or treatment for hearing loss in humans would take place. Ever since the discovery of birds' ability to regrow and restore their own hearing loss in 1986 researchers have used the terms "decades away" on when an application for hearing loss restoration may take place. In this recent news it was quoted that a possible cure could be at least a decade away noting that people who already benefit from hearing aids would be the most likely targeted group. But lets go back in time on how these predictions changed over time.

In 2004 it was predicted that an application for hearing loss rather than a cure could be at least twenty years away by Dr. Rubel. In another article in 2004 Dr. Rivolta said a cure could be possible in 10 to 15 years away. In 2009 in the Department of Otolaryngology University of Miami Ear Institute's newsletter quoted believing they could be 10 years away from a cure at the earliest possible timeline. But most seem to concur that a cure or a very applicable treatment for hearing loss would be seen within their (the scientists') lifetime.

Often times these revelations on stem cells discoveries on hearing loss are made in jumps rather than something that unfolds smoothly over time. Once discoveries or breakthroughs are published in scientific journals researchers from all over the world pour over the data and details and apply the discovered knowledge to theirs. And thus increasing the likelihood of another jump in evolutionary discovery on the path for the cure or restoration of hearing loss in humans. The revelation in this latest news out of California was about the fact that when the bundles of lab grown ear hair cells were mechanically stimulated or moved mimicking how sound moves ear hair cells, the cells were able to generate electrical currents that resemble those produced by young hair cells. It was not simply growing ear hair cells but seeing that the electro-mechanical qualities and properties in a lab grown ear hair cells worked.

It'd be wise to take the word "cure" loosely and see it from a restorative/treatment point of view in the very near future. But then again, with scientists and researchers from all over the world working on the cure for hearing loss, a cure could be very well within our grasp a lot more sooner rather than later.

Make cure for hearing loss mandatory by law for deaf babies if stem cell prove successful?

According to one Deaf person in a deaf forum he felt that deaf babies and toddlers ought to be cured for their hearing loss if stem cell proved successful and see that a law making it mandatory for them to be treated.

stem cell consistently proves to be 100% successful, I will have unwavering support for it and will want to see a law making it mandatory for toddlers, not adults.

Agree or disagree? That day may actually come, and it'd simply be a matter of time. And this is a contention that worries many people.

"Deaf group attacks stem cell researcher for his work on the cure for deafness"

"Arrests were made against a small group of Deaf people who attacked a stem cell researcher for his work on finding a cure for hearing loss. The attack took place at the researcher's home where he sustained multiple injuries. He was treated at a nearby hospital and released. The attack occurred while he was home alone. Graffiti was spray painted on the living room walls and elsewhere in the victim's house with words like 'Audism is wrong,' and 'Deaf babies do not need your help,' along with several epithets. The group, based on the graffiti, named themselve as 'Deaf Crusaders.' "

Could this shocking news conceivably happen someday?

Let's hope not. But with researchers working around the clock all across the United States (and the world) on the cure for hearing loss (see here, here, here, here and here) it would be a matter of time until a cure could be found for various kinds of hearing losses. For some Deaf people they would consider these scientific advancements as a threat to their Deaf culture (see "Will Deaf Culture Die Out Someday - Part I and Part II) should a cure finally be found. And certainly among those few who do view a cure for hearing loss as a threat there could be potentially smaller subset of Deaf extremists who may resort to the use of violence against stem cell researchers on the cure for hearing loss. As with any group there is always a few bad apples who will resort to violence as their means of protesting (see vandalism question at Gallaudet protest). Researchers have been attacked in the past before for their medical work.

Police said several students from the University of California, Santa Cruz (UCSC) attacked a researcher, who uses mice to study breast cancer, at her home Feb. 24. (2008)

The biology professor, who declined to give her name to protect herself and her family from more violence, was at home with her children and husband when the alleged assailants attacked her home just before noon. The suspects attacked the professor’s husband and then fled the scene in a vehicle. Because the incident happened in daylight, the husband, who suffered minor injuries, was able to write down the license plate of the car.

If a bunch of students went after a professor for her medical work on mice to the point of terrorizing and injuring a researcher then it wouldn't be inconceivable that a Deaf person (or a small group of Deaf people) with extremist tendencies to attack a researcher working on a cure for hearing loss. Or for that matter supposingly a cure has been found would a research center get vandalized and broken into in the attempt to "stop" the cure on deafness from getting out. There is always that possibility. It may be small or even non-existant but certainly a possibility based on fear and frustration.

The above news is a hypothetical one but the fact remains that there are always somebody with extremist tendencies who will go further, even to the point of injuring, or heaven forbid, murder a researcher in the name of stopping medical advancements, for example, such as for the cure on hearing loss.

Promising research continues for cure on hearing loss

For 2010, the American Hearing Research Foundation will be funding six grants. This year, we received the highest number of grant proposals in our history—41 proposals in all. The following grants will begin on January 1, 2010, and will be funded for $20,000 for one year.

Douglas Cotanche, Ph.D.
Boston University School of Medicine, Massachusetts
“Cell fate regulation in cochlear development and regeneration”

Birds can regenerate hair cells in the cochlea once they are lost – an ability that mammals do not possess. When hair cells are lost in birds, following loud noise exposure or administration of ototoxic drugs in the lab, the supporting cells around the hair cells turn into new hair cells. Cotanche and colleagues hope that by studying this process in birds, they will be able to induce the mammalian cochlear cells to undergo a similar regeneration. Specifically, the researchers will study the genetics involved in supporting cell transduction into hair cells in chicks and mice. They hope to gain understanding of the molecular processes involved in hair cell regeneration in order to cause the same regeneration in mammalian cells.

Saima Riazuddin, Ph.D.
Cincinnati Children’s Hospital Medical Center, Ohio
“Generation and characterization of a mouse model of DFNB26”

More than 100 gene locations have been indentified as contributing to hearing loss in its various forms. However, knowledge on how genes affect hearing remain inadequate. Researchers need to be able to study and develop animal models to study disease causing mutations, and to define the molecular pathways leading to hearing loss to ultimately develop therapeutic approaches to treating deafness. Riazuddin and colleagues have recently mapped a deafness causing gene called DFNB26 in an extended family. They also discovered another gene that that suppresses the deafness-causing gene. The researchers hope to study how these two genes work to cause and prevent deafness among the members of this family by developing mice with these genes. The study will provide valuable models for defining the role of DFNB26 in the inner ear and to develop treatment strategies for this disease.

Angelika Doetzlhofer, Ph.D.
Johns Hopkins University School of Medicine, Baltimore, Maryland
“Response of supporting cell to hair loss in the mammalian cochlea”

Doetzelhofer and colleagues hope to uncover the molecular signals activated in supporting cells after hair cell loss, and what pathways may inhibit hair cell regeneration. In mammals, hair cells only develop during embryonic development, and when hair cells are lost, they are not replaced. However, in birds, when hair cells are lost, supporting cells can transform into new hair cells. Researchers believe that supporting hair cells, even in mammals, have the ability to transform into hair cells, but that signals from adjacent cells and/or dying hair cells block supporting cells from becoming new hair cells. Doetzlhofer and colleagues will investigate these inhibitory pathways and will look at changes in gene expression in supporting cells as hair cells are lost. Understanding the molecular mechanisms that prevent hair cell regeneration in mammals is a crucial first stem in developing successful hair cell replacement therapies.

Researchers identify protein needed to develop auditory neurons

Loss of spiral ganglion neurons or hair cells in the inner ear is the leading cause of congenital and acquired hearing impairment. Researchers at the University of California, San Diego, School of Medicine and the National Institutes of Health found that Sox2, a protein that regulates stem cell formation, is involved in spiral ganglion neuron development. The study was published in the January 13 issue of the Journal of Neuroscience.

"These findings may provide the first step toward regenerating spiral ganglion neurons, the nerve cells that send sound representations to the brain," said Alain Dabdoub, PhD, co-investigator and assistant professor of surgery with the...

See - http://www.newsrx.com/newsletters/Stem-Cell-Week/2010-01-25/39012520101SC.html

Growing an Ear Where No Ear Has Grown Before

Deep inside the inner ear, two types of nerve pathways shuttle their messages to and from the brain. Afferent nerves carry messages from the ear to the brain about the sounds your ear hears as well as balance information. Efferent nerves carry information from the brain back to the inner ear to help the ear make necessary adjustments. Researchers have known that the efferent nerves in the inner ear are modified motor neurons that have evolved from neurons that innervate muscles in the face. NIDCD-funded researchers from the University of Iowa wanted to know if other motor neurons in the body could be rerouted to innervate the ear if it were placed in their way. Using tadpoles of an aquatic frog (Xenopus laevis), the researchers transplanted the tadpole ears into the tadpole’s upper side (trunk) or eye region and then watched what happened. They noticed that out of 109 transplanted ears, 73 developed with necessary inner ear structures, including sensory cells, called hair cells, which connect to the nerve fibers. Using a dye that labels only motor neurons, they first found spinal motor neurons had populated the ears in the tadpole’s trunk and cranial motor neurons had populated the ears in the eye region. Next, using antibodies, they demonstrated that connections were indeed forming between the efferent nerves and the hair cells. Finally, using electron microscopy, they showed that structures associated with afferent and efferent nerves were both present in the transplanted ear. Because the afferent nerves travel to the brain directly from the eye region or indirectly from the trunk region by way of the spinal cord, the researchers now want to find out what happens if the inner ear information travels to a non-auditory part of the brain and how that information will be processed.

The poster "Transplantation of Xenopus Laevis Ears Reveals Ubiquitous Rerouting of Motor Neurons to Become Efferents" (#552) takes place Monday, Feb. 8, at 1:00 p.m. PT in the Disneyland Exhibit Hall.

http://www.nih.gov/news/health/feb2010/nidcd-04.htm

Noise-Damaged Hair Cells Can Regrow Stereocilia in Mammals if Rescued in Time

Scientists have been trying to regenerate hair cells — sensory cells in the inner ear named for the hairlike bundles jutting from their tops — out of surrounding non-sensory cells as a way to make up for hair cell loss in ears damaged by noise, some medicines, and other sources. (Unlike other vertebrates, mammals aren’t able to grow new hair cells on their own once the cells are dead or damaged.) But new NIDCD-funded research from Creighton University, Nebraska, and the Chinese PLA General Hospital, Beijing, says that hair cells damaged by noise may have more life in them than we once thought. We may be able to regenerate new hair bundles — called stereocilia — from the original hair cells. The researchers subjected guinea pigs to an impulse noise simulating gunfire, resulting in severe hearing loss. Using scanning electron microscopy, they showed extensive stereocilia damage had occurred throughout the inner ear. However, when the researchers injected math1, a key gene for the development of hair cells, into the inner ear within one week of damage, they found that the stereocilia were able to grow back. Furthermore, key measurements showed that the new stereocilia were able to convert sound vibrations into electrical signals, which is how the brain interprets sound. The researchers caution that there is a window of time when the hair cells are still viable following noise damage. If the hair cells are not rescued within that time frame, they will die. But if researchers are able to intervene early enough — within 10 days — the hair cells can possibly be saved. The researchers plan to conduct tissue culture studies to confirm whether these findings are supported at the cellular level.

The talk "Regeneration of Stereocilia of Cochlear Hair Cells by Math1 Gene Therapy" (#484) takes place Sunday, Feb. 7, at 5:00 p.m. PT in the Disneyland South Ballroom.

http://www.nih.gov/news/health/feb2010/nidcd-04.htm

Creighton professor strives to reverse hearing loss

Here's a recent article that came out last week about a Creighton University professor working on a cure for hearing loss.

It’s a bit of a stretch to describe Kirk Beisel’s research as something akin to seeking “Rogaine for the inner ear.”

But if his work with gene therapy pays off, the Creighton University professor and his team may aid untold numbers of people suffering from hearing loss by restoring the tiny sensory cells, the “hair cells”, inside their ears.

Or, he says, by preventing these hair cells from dying in the first place and thereby slowing down what is the leading cause of deafness in humans.

“The commercial potential is huge because by the time most people reach their 60s, nearly a third of them have hearing loss because these hair cells have died,” says Beisel, whose lab is in the Department of Biomedical Sciences at Creighton.

“When they reach their mid-70s, half have some kind of deafness,” he adds. Beisel is working closely with Lee Fenicle, director of Intellectual Resources Management, who has applied for several patents relating to Beisel’s research.

Click on the link above to read the rest of the article.

Stem cells on hearing loss for returning soldiers

Here's a stem cell development in the state of Washington for soldiers who lost some of their hearing during war.

Regenerative Medicine for Acute Deafness
Recipient: FATE Therapeutics
Amount: $3,000,000
Location: Seattle, WA

This funding would use advanced stem cell research and developmental biology to develop therapeutic treatments for acute hearing loss among Army soldiers. This would advance safe and effective treatments for acute hearing loss, which affects many men and women of the Army on return home from war.

Fate Therapeutics, a company that has made some important leaps in producing induced stem cells without embryos or gene therapy.

Dr. Rivolta on stem cells cure for hearing loss and making hearing aids and cochlear implants obsolete someday soon

Here's a subtitled video and article (transcript) on Dr Marcelo Rivolta who is the Senior Research Fellow at the University of Sheffield where he explains how his stem cells research could make hearing aids and cochlear implants a thing of the past in his quest to find a cure for deafness in the field of sensorineural hearing loss.

On the video be sure to click on the "S" button for "subtitle" on the left of the screen to get the English subtitles with the video. There is the option of choosing video itself only, video and subtitles, video with a British Sign Languageinterpreter, or video, subtitles and BSL.

Hattip: Neuro

Stem Cells for hearing loss live chat tomorrow night, November 19, 2009!

Stem Cells for hearing loss live chat tomorrow night, November 19, 2009!

(Errata: The topic heading said November 18. Correct date is November 19. It has been corrected)

Get ready and dial in at 9 PM Eastern Time (8PM - Central time, 7PM Mountain time, or 6 PM West Coast time)


Topic: "Development and regeneration of hair cells – the latest news in research"

Guest Speaker: Dr. Douglas A. Cotanche

Date & Time: November 19, 2009 at 9 PM

Live Webchat Schedule

Hearing Loss Association of America is proud to present live moderated chats,
featuring the nation's leading experts in hearing loss. This is your opportunity
to ask a question and learn more about hearing loss from leaders in a number of
medical disciplines associated with hearing loss.

How to Participate (no
need to register):
1. Go to www.myhearingloss.org/ (5-10mins before
webchat is scheduled)
2. Click on the "Log in to our Chat Room" button
3. Enter a username to use for the chat
4. Click on the "Connect" button
5. Click on "Rooms" tab (found on the right column)
6. Then double click
on "Guest_Chat" to join the webchat

NOTE: The webchat is a moderated
chat to allow the guest speaker to answer all the questions that have been
submitted in advance, which means you will not be able to type in your question
until after all these questions have been answered.

Here are my questions that I submitted to Dr. Cotanche.

Dr. Cotanche,

I run a blog (Kokonut Pundit) and I do try and keep abrest of these news and developments regarding stem cells and gene therapy for the restoration/cure of hearing loss, mostly in the arena of nerve deafness.

Have you heard recently the latest development about an 18 year old woman Chloe Sohl who had her hearing partially restored (so far - see captioned video http://kokonutpundits.blogspot.com/2009/11/video-interview-caption-with-first.html) by using her own adult stem cells? And if so, is this an indication of how rapidly the field of regenerative medicine using stem cells for the cure/restoration of hearing loss? Instead of a few decades away are we looking at within the next 5 or even 10 years to see this become a common procedure? If not in the United States but what about in other countries (due to FDA lagging behind?).

Thank you

Stem cell history on search for cure for hearing loss

Over at The Stem Cell Blog where David Granovsky compiled a summary with links on the history of stem cells research for the search (more like a race right now) on the cure for hearing loss from January 2001 to November 2009. It is interesting to see just how fast research has evolved over a relatively short amount of time to the point where an 18 year old woman whose hearing has basically recovered (so far) in less than 2 months once given her own adult stem cells to treat her hearing loss.

For the rest of Kokonut Pundit blogs on stem cells for the cure on hearing loss go here.

Hattip: Granovsky.