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.
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.
Tinnitis and instances of progressive hearing deficit run in my family, I am hoping this is successful.
Anthony, like others I would gladly make a donation to help you out. When I was at university one of the lecturers said that he thought that in evolutionary terms, hearing was more important than sight because there are more areas of the brain dealing with this sensory input compared to sight.
Pamela Grey how are you? I was in Newcastle city centre a couple of Saturdays ago and walked through a nineteenth century arcade, that I don’t normally go to, there was Hutchinsons bookshop, established in 1881 and still very much in the pre Internet age. This is why I couldn’t find it. I have taken a photo of it with my phone, so if you are on Facebook, I will upload it for you!
Having had tinnitis since I was in grammar school, and because it is increasingly a problem, this is very interesting news. After reading Anthony’s news I did a little checking and found this:
http://www.ncbi.nlm.nih.gov/pubmed/14763322
So, for us with tinnitis there is some hope in this as well.
WOW!
I have total loss in my left ear (damaged auditory nerve caused by mumps at age 10). My daughter also has total loss in her left ear (damaged auditory nerve caused by a bad reaction to the mumps shot at age 6). My father-in-law has 75% hearing loss (NIHL), my mom etc., etc. etc…..this hits close to home for everyone.
What a blessing this treatment could be! I had given up any idea of there ever being a cure. I’ll start paying more attention. If there is ever an opportunity to support this kind of work, please let us know where to send the checks.
Probably, like many discoveries in medicine we will never hear about this again. The cynic in me says that this is more than likely a way to push up a stock price. We have seen these new breakthrough stories before.
That sounds interesting, I could do with some of that myself.
In my case, it was running up racing motorcycles in garages, sitting too close to big speaker stacks and shooting.
Exciting news. Hope you can get in on the trial or shortly thereafter when the treatment proves successful and becomes available to the public. All the best.
Hmm, I don’t suppose this would help the alarmists who will hear no dissent?
OK, I know, theirs is an entirely different malfunctioning organ.:)
I remember reading about this gene therapy approach in Nature Medicine in 2005. I told my wife because her dad’s hearing was so bad from the time he was 30 he had lost over 70% of his hearing. It has taken 8+ years to get the technology human-study ready and FDA regulatory approvals with Novartis as the lead big pharma biotech.
The original experiments were done in guinea pigs. The researchers were amazed at how quickly and well the treated animals recovered their hearing compared to zero in the controls.
If you have a Nature Med subscription (or institutional access like me), you can read the groundbreaking research article here:
http://www.nature.com/nm/journal/v11/n3/full/nm1193.html
or the reader’s digest Views summary article (better for non-scientists) here:
http://www.nature.com/nm/journal/v11/n3/full/nm0305-249.html
O.T. but this great few sentences here:
“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”
…are a classic example of why I lost my faith (as indoctrinated into me at school) in the religion of Darwinism (as did Darwin, apparently). There’s no way I can see something as incredibly complex as an ear could ever have evolved through a process of natural selection. It’s just too much of a stretch to believe that no intelligence played a hand. And that’s not even counting the fact that evolution doesn’t explain how the very first simple life-form appeared in the first place. I’m not remotely religious; there are too many religions in the world to know which one, if any, to subscribe to. And yet due to the many inexplicable wonders of our world I have an easier time with a Creationist theory than an evolutionary one.
Anyhoo, carry on!
Anthony, hope you are a candidate and this works. I have 85% lost in Rt. ear, but thankfully a powerful hearing aide helps when 1 to 1. My Lt. ear is a 95% loss and totally out of the hearing game. Best, best if luck on this endeavor.
Fingers crossed that it works. Good luck, Anthony.
That means you are an Agnostic
Gene therapy is rapidly advancing. I remember the excitement of seeing the
first human gene therapy trial in the U.S.A. In 1994, a young Canadian woman
was treated at Children’s Hospital of Philadelphia. She had familial hypercholesterolemia.
Her cholesterol was over 600; 2 members of her family had died in their 20s
from heart attack. A portion of her liver was extracted, genetically modified, and
reintroduced into her; it implanted and her problems were much reduced. These
results were announced some two years after she had the procedure.
Anthony, I sincerely hope that you are able to participate in a trail soon and
that the results are even better than you hope.
ghl says:
April 23, 2014 at 5:44 pm
“I’m going to inject the top of my head.”
ROFL!!! I’ll buy that for a dollar.
Prayers also whether you want them or not. (If “not” then just consider them “best wishes”.)
I would suggest that if you are able to be a part of the first round of human testing that they make clear to you the possible side effects as far as they can know at this point.
Maybe give what they say a little extra weight since your desire for the positive results may tip the scale in the “yes” direction.
PS I’d only consider it “selfish” if you lied or cheated in some way to be a part of it. Wanting something that could meet a genuine need is not selfish. How would your family benefit if it works? So don’t let feeling “selfish” about it be a deterrent.
Good luck Anthony . When I was about 15 I was prescribed Streptomycin for pneumonia which is ototoxic.
I have suffered frequency dependent hearing loss for a long time now with the loss in the speech spectrum. I know what it is like to feel isolated in environments where everone else can participate in conversation -when all I can do is try to lip read – and usually make some comment which , having heard the conversation incorrectly , people think you are mentally disabled !
When is it available in the UK?
This is great news Anthony! As it happens, we just learned today that my 20-year-old daughter has been approved for a cochlear implant. She has Large Vestibular Aqueduct Syndrome and is almost totally deaf in her right ear (she can hear OK in her left with a hearing aid). I know this is a different procedure but anything that promises improved hearing is well worth it. Best of luck to Anthony and all who struggle with hearing deficits.
Good luck Anthony for everything you have done you certainly deserve an opportunity to check it out. But a word of warning to all. I have hearing loss. So 3 years ago I went ahead and spend a lot of money on top of the line hearing aids. At first they were absolutely astounding, I could hear birds, leafs rustling in the wind etc etc it just blew me away!!! Wow conversations between people (at times in places I was glad I could control the sound level). But within a few months I started getting blinding headaches. Over time the doctors and ear specialists figured out the fact I struggle with chronic pain started to overload some parts of my brain in a ways they are still trying to sort through. I had to stop wearing the aids, so before anyone tries this in the future be careful side effects maybe unexpected! Especially if the repairs are permanent!
( One Dr proposed that my brain slowly confused pain signals with sound input but who knows? I wear a cheap set only a few hrs a day if that).
I lost my hearing a child for about two weeks due to a viral infection (back in the early 60’s). Several children died from it and others remained deaf. I can remember the day my hearing came back and remember vividly the mouths of people moving but not being able to hear what they were saying. It was so traumatic that I can still remember it clearly so many years later, On the downside I have pretty serious tinnitus that I have learned to live with since then and like so many others my hearing is getting worse. For me, it is especially embarrassing to be in a relatively noisy atmosphere such as a restaurant with friends and family, remain silent and occasionally nod my head looking for visual clues that I may be spoken to. I never start a conversation because of this and have now (because it is bad enough), explained to them that my hearing is not quite up to par. I have been pretty crafty at selecting restaurants and other venue’s that are reasonable, but with grandchildren, the noisier places are where we go and obviously I am over-ruled (grin). At least they know now and speak up when they talk to Papa.
I hope that this works out because if it does, they have a customer.
Anthony, I can only sympathize with your condition metaphorically, but I wanted to let you know (along with others here) that I do sympathize.
I do hope that an appropriate treatment comes soon for you.
Let’s hope this therapy really works and will be on the market ASAP so you can be treated.
I wish you the best of luck restoring your hearing.
Progressive self-inflicted tinnitus from earbuds, chainsaws, and heavy equipment leaves me often saying, “say again”… so I can really empathize with all who suffer faultlessly like Anthony, who compensates well as a prolific writer. Best wishes to all with hearing loss; hopefully the “science isn’t settled” on that one either.
Yes, good luck with this, Anthony. I hope it does the trick!
My knowledge of biology is almost no-existent but it strikes me that the technique of using these viral ‘nano-devices’ ought to be generalisable. If this is a success—and I very much hope it is—then perhaps it will spur further research with the result that all manner of ailments will become curable. Big smiles! 🙂
Like so many above, I offer my sincerest wishes for your wish to be realized . . and would happily contribute to any fund which helps with the journey.
I am fortunate to have reasonably good hearing and I can appreciate how significant this development is to those less fortunate. I hope this is the success story you and others have been waiting for, Mr. W. and that it speeds it’s way through without delay. Best of luck with it.
Eamon.