Hearing restoration with gene therapy – I knew this day would come

For me, this is a profound moment. It will be even more profound if many people can benefit from it.

Some of you may know that I suffer about an 85% hearing loss, and even with powerful hearing aids I still have very poor hearing which has gotten almost non-functional over the years. It makes me a social hermit since I don’t function well in public. It is part of the reason I became a broadcaster, because I had such a terrible time in college lectures and with language requirements in the school of science. In broadcasting, I only had to talk to the camera or to the microphone. It was a job that was not only a dream come true, it brought me out of my shell that many hearing impaired people live in due to the social isolation it brings.

I started losing my hearing as a child due to being treated with the antibiotic Tetracycline, which is known to be ototoxic. By the time I was 10, I needed hearing aids, but fortunately, I had formed my primary speech skills. Many other people who lose hearing as children aren’t so lucky as I and have speech problems as a result.

I knew this day would come, I predicted that gene therapy to treat cochlear nerve deafness would be coming over 10 years ago. I can only hope I can be able to take advantage of it someday. I won’t hide my own selfishness, I want to be one of those people.

Fully functional <i>(Image: Steve Gschmeissner/SPL)</i>

Fully functional hair cells in the cochlea (Image: Steve Gschmeissner/SPL)

Deaf people get gene tweak to restore natural hearing

People who have lost their hearing will be injected with a harmless virus carrying a gene that should trigger the regrowth of their ears’ sensory receptors

IN TWO months’ time, a group of profoundly deaf people could be able to hear again, thanks to the world’s first gene therapy trial for deafness.

The volunteers, who lost their hearing through damage or disease, will get an injection of a harmless virus containing a gene that should trigger the regrowth of the sensory receptors in the ear.

The idea is that the method will return a more natural sense of hearing than other technologies can provide. Hearing aids merely amplify sounds, while cochlear implants transform sound waves into electrical waves that the brain interprets, but they don’t pick up all of the natural frequencies. This means people can find it difficult to distinguish many of the nuances in voices and music.

“The holy grail is to give people natural hearing back,” says Hinrich Staecker at the University of Kansas Medical Center, who is leading the trial. “That’s what we hope to do – we are essentially repairing the ear rather than artificially imitating what it does.”

There are still many things we don’t know about how the ear works. This is because the delicate machinery of the inner ear is enclosed in the hardest bone in the body, making it difficult to isolate without causing damage.

What we do know is that sound waves are funnelled into the ear, making the ear drum vibrate. These vibrations are transferred to the cochlea in the inner ear via three tiny bones. Thousands of sensory receptors line a part of the cochlea called the organ of Corti, as rows of inner and outer hair cells. Sound waves, amplified by the outer hair cells (shown above right), vibrate the inner hair cells, opening ion channels on their surface that let neurotransmitters flow in. This triggers electrical activity in the cochlear neurons, passing the information to the brain so it can be processed.

Both inner and outer hair cells can be damaged by loud noises, drugs such as some antibiotics and disease, and don’t regrow. A possible fix arose in 2003, when researchers discovered that certain genes can transform the cells supporting the hair cells into both types of hair cell.

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Complete story here.

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milodonharlani

Very good news, indeed, if it pans out in practice. I hope you may benefit.

Curious George

Good luck! We need you.

Janice Moore

Oh, dear Anthony!
This is WONDERFUL news. Thank you for sharing.
A girlfriend of mine (had to quit her job as a cop and was then, like you, “on stage” as a “deaf” trial attorney –tough!) has cochlear implants, but, as the article said, they are good, but not-quite-there… .
(I hope you won’t mind my telling you that I will be praying very hard about this for you.)
With a big smile and HIGH HOPES for you,
Janice

Latitude

…can I get in line right behind you?

Lance Wallace

I wonder if tinnitus might be treatable eventually, by this or similar approaches. My wife and I have a constant “radiator hiss”, which fortunately we are able to tune out much of the time, but others have far more serious cases.

stan stendera

Best news I’ve had in a year. Good luck! I would pray for you if I was a religious person.

David in Michigan

Age related deafness cured??? That would be something special. I remember my grandfather who became progressively more deaf as he aged. It was an obstacle that he hated and, as you pointed out, isolated him from his family and friends. I hope this works!!

Konrad

This is amazing news.
Do you know how soon after the trial this would be available?
I’m guessing they would only do one ear at a time as with cataract operations and such.

PaulH

Wow, I hope this works. 🙂

Anthony, I want you to be part of this first test group that gets the injections. Move to Kansas if you have, too. Give them your house if it will help. Do whatever you have to do to regain your hearing. With normal hearing, your life will be so much happier and more successful. If you need donations to cover the cost, set up an account. Lets make this happen.

ghl

I’m going to inject the top of my head.

Janice Moore

STAN STENDERA!
Hi.
#(:))

Pamela Gray

Freakin cool! I’ve studied ototoxic hearing loss. Mother had it. And many veterans that came through our VA lab had it. This is awesome!

Janice Moore

“If you need donations to cover the cost, set up an account. Let’s make this happen.” John Coleman
YES!
Do, Anthony!

RexAlan

Latitude
…can I get in line right behind you?
And me next please!

John R T

Will it help me learn Central American Spanish lingo?
These seventy-year-old connections deliver neither what I read nor what I remember from four decades ago, in Asturias.
Wear ear plugs and dark glasses for as long as it takes to become accustomed to all those
sounds.
Great news for all of us. . . . thanks

Janice The American Elder

A harmless virus . . . and with my luck, I’d get my hearing back, but all my hair would fall out, and my skin would turn blue.

Pete

Thank you for sharing the above information. My ear doc has informed me of minor nerve damage requiring a special adjustment for my hearing aids. Knowing of the Atohl1 gene gives me something to talk with him about.
Knowing the frustrations inherent with poor hearing, I do wish you well.
Pete

Bill Illis

I too have a hearing problem. Unlike Anthony’s case, it is not as severe and I find it manageable. Gene theapy, stem cells, activating the body’s systems at the molecular level has always been the most promising way forward. Just think if this actually worked.
Reminds me of the days when people complained of ulcers. Many people in fact. Then an Australian researcher suggested it might be caused by bacterial infection and anti-bacterial therapies might cure the condition. Ridiculed for many years, it was not the consensus position. Massive profits were being made on ulcer medications that didn’t really work at all. But now, no one talks about ulcers anymore because it is a condition of the past. Real science, real objective science. Real science.

Larry C

Hoping it turns to reality soon!

I don’t know Anthony. With the current backlash against GMOs in the food industry, do you think the blogosphere is ready for a host who’s a Genetically Modified Organism?

Peter Miller

Best of luck, I sincerely hopes this proves to be real science and not the equivalent of ‘climate science’.

May I humbly suggest waiting for the second or third test groups? There’s a lot to be said for not being first with the latest car, cell phone, or other technology. Let them work the kinks out first. 🙂

Gary Hladik

“…a harmless virus carrying a gene that should trigger the regrowth of their ears’ sensory receptors…a group of profoundly deaf people could be able to hear again…”
Works in mice, but if it had been up to me, I would have made the announcement after a successful human trial, lest false hopes be raised and dashed. Best wishes for success.

Paul Westhaver

This is good AND ethical science at work. Viral gene therapy, with adult experimentation with all the appropriate permissions in place and positive results. Nobody is exploited. Nobody’s body parts were taken without permission.
Fantastic.
Only one question, If the virus carries a gene to convert hair support cells inti the cilia, what is preventing the gene from entering hair support cells elsewhere on the body. – side effect, cure for male pattern baldness. Chaching!!!

Rick K

Anthony, just sending best wishes to you and to all who may benefit. I hope this treatment works!

Darrin

Keeping my fingers crossed for you.
This comes after last week they announced 4 for 4 in restoring function to people with spinal cord injuries using a type of electrical stimulation. The kicker is they were working on something else and a side effect was restoring function to there test subjects.

You have my best wishes! I feel quite positive about this kind of approach to physiological issues.
Bill Illis made a damned good point. The so-called ‘science-based’ medicine which some people champion with dangerous fanaticism has caused all manner of problems, often worse than the disease. I wonder how many doctors take seriously the Hippocratic Oath.
Death to science-based medicine. Here’s cheers to solutions that might actually work. 🙂

Agree with Ric Anthony – it sounds very exciting, but don’t be in the first test group.
Gene therapy with viruses has a patchy history, for example there have been cases of severe allergic reactions, even deaths, in response to introduction of the gene transport virus.
I would also ask some very careful questions about the effect of adding this gene – adding some kind of growth factor sounds awfully close to what happens when a normal cell mutates into a cancerous tumour.
http://pubs.acs.org/subscribe/archive/mdd/v03/i08/html/lesney.html

Jules Hancock

I am totally deaf in my right ear due to a fractured skull. Apparently my ear mechanism works but does not if you understand my meaning.
This is exciting stuff!

starzmom

This sounds terrific for those with a hearing loss. If you are lucky enough to get into the trials, and you need a place to stay in Kansas, we’re here!!
PS I got bionic eyes (Lens implants for cataracts) last summer and am vision correction free for the first time in almost 50 years. I hope the same for you with your hearing.

Cal Smith

I have ben resisting getting a hearing aid but my daughter this week advised me of studies which have shown a high degree of correlation between hearing loss and dementia as well as decreased cognitive function. My wife’s 98 year old mother lives with us and we have to deal with her dementia on a daily basis. What ever it takes to regain your hearing please do it – we certainly do not want you to join the ranks of the cagw people with cognitive dissonance disorders.

Lance

Worked a few years in a saw mill while a young man, next to the trimming saws. Even though we wore hearing protection, I know now, that I suffer from Tinnitus, 24/7 ringing in my ears. I too am slowly having more and more hearing loss, and its not fun at all. Hope for the best Anthony.

KevinK

Well, I certainly hope this treatment can help those in need, including Anthony. If only we had directed more of our spending to efforts like these that may solve (or at least minimize) real problems.
Perhaps it will help, I sure hope so.
Think of what all those billions wasted on “fixing” the unbroken climate could have done to alleviate Alzheimer’s, Parkinson’s, cancer, hearing loss, eyesight…….
Anthony, I sure do hope this may someday help you, if even in a small way.
Cheers, Kevin

JohnWho

Hear! Hear!

Mac the Knife

Anthony,
My sincerest hope is this trial proves viability for this gene therapy method and you will have your hearing restored soon!
Best wishes to you and prayers for success in the gene therapy development and application,
Mac

jolly farmer (richard)

Very best wishes with this, Anthony.
Richard

Evan Jones

You could fly again.

Power Engineer

Anthony-
You better hope they don’t develop a cure for hearing loss or your readership will drop to zilch. All your readers have hearing loss . We can’t interact normally so we find our friends online.
Cheers.
Reddy Kilowatt

This is great news! Hope these trials work well! (I’m rated severe loss in my left ear and profound in my right.)
If this really works, this has the potential to help a ton of people while destroying the current hearing aid market. I wonder what this type of treatment will cost?
This also indicates huge progress on gene therapy in general.
Could this type of treatment revive pancreas that are not working well too and cure diabetes? Parkinson’s disease? Nerve regeneration at the spinal cord level?
I wonder with a bunch of new cells how the brain will react to the new signals. Often new hearing aid users like myself along with the hearing loss develop ringing in the ears which often is the brains reaction to the loss of signal. Often getting hearing aids will stop the ringing after a time as the brain gets used to a return of the missing signal levels. What if you end up with more receptors than you were born with? Thankfully the brain is more plastic than what used to be thought so I’m hopeful the side effects of this treatment can be ‘tuned’ out of the brain and real normal hearing returned.
This may be the first practical application of gene therapy I’ve read. This could be the ushering in of a new age in medicine!

Margaret

I wonder if this might help people with Meniere’s.

David Ramsay Steele

Mr. Watts, Have you given a thought to cochlear implants, as described in the book Rebuilt by Michael Chorost?

ChipMonk

Janice, I have had Tinnitus for many years. I would gladly take the “risk” of turning blue, losing hair, etc.. if I could just “hear” silence. It literally brings tears to my eyes when I consider how many other people like Anthony, you, at. al. are “missing out” on life’s simple things. Hey we could become… The 2-Blue Group… oh wait, someone did something like that… blue bald people… hmmmm

Jim G

Anthony,
Thank you and please keep us informed as to progress in this endeavor. I will be one of those in line behind you. I have, however, been happily married for 35 years and am somewhat concerned about the totallity of the results if I were able to hear more clearly everything my wife has to say. Just a thought.

Doug Jones

My hearing losses are in the 60-80 dB range (to simulate it, insert earplugs AND put a headset on), and man o man do I hope this will become available soon!

AntonyIndia

We (= millions across the globe) heard you loud and clear. I hope you can hear clearly too soon.

That’s fantastic! I hope you get a good video of the first time you’re able to hear at full volume again– it would be great to see your response!

Cold in Wisconsin

Ototoxic drugs are not uncommon these days, and many times represent the drug of last resort due to antibiotic resistance to other safer drugs. I wonder what the prevalence of this type of hearing loss is. Another cause of deafness, German measles during pregnancy, is almost completely preventable with universal immunization. I knew a family with a deaf sister who suffered total hearing loss via this mechanism.
I too have some mild hearing loss, most likely due to loud noise and lack of hearing protection. I hope this treatment might be a help for me. Some old age hearing loss might be due to arthritis in the tiny bones of the inner ear so might not be amenable to this cure, but it could still be helpful to improve the other structures.
Good luck to the researchers and the patients in this trial.

george e. smith

Well I hope you get a chance to experience, and benefit from such a treatment, Anthony. I’d be devastated if I lost my hearing, and couldn’t listen to the great and inspiring music we have. I’d also be happy to be rid of what I presume is Tinnitis. For me it never stops.
The loss of basic senses (that we got used to having) is a heavy burden. But there are many who are far worse impaired, and we shouldn’t complain, with lesser impairments. I’m getting used to seeing unsharp images with one eye; but it still sees very well. Thank technology for camera autofocus.
Having recently dealt with loss of speech by a family member, I’m increasingly sensitized to such issues.
Hope it happens for you soon Anthony.
G

Aphan

My hopes and prayers are with you Anthony!!! If there is anything your readers can do to help with costs or anything else, just say the word. What a great thing!!!