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.
![29662401[1]](http://wattsupwiththat.files.wordpress.com/2014/04/296624011.jpg?quality=83)
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I do hope that the trials are successful and that Anthony will soon have his full hearing back. He is a lesson to all of us in that, notwithstanding his hearing loss, he has achieved so much, not least with this outpost of science and reason. I am still chuckling over how WattsUpWithThat, on an averagely slow Sunday, gets almost twice the audience of a heavily over-promoted, $20 million schlockumentary series in which once-big names make asses of themselves telling us we’re a’ doomed. The F. of D. had better watch out once Anthony’s hearing is 100% again.
This is just another step into the right direction.
On the long term, what I see is that we simply breed spare organs, based on the patient’s DNA. You lost your arm? Okay, we’ll just breed a new one. Heart? Eyes? Liver? Kidneys? No problem.
And the best, since it’s the patient’s cells already, there won’t be any rejection.
Well, godspeed to the Kansas researchers.
Are human genes will be the patented?
I became profoundly deaf in my mid-30s (exposure to military jet aircraft noise) and have had bilateral cochlear implants for some years now. While not a universal panacea, they work very well for me. If you haven’t already explored the implant option, Anthony, I’d strongly recommend that you do so as an alternative solution. If you are a suitable candidate, it offers proven technology and a very satisfactory result that is available now.
Edohiguma says:
April 24, 2014 at 12:42 am
“On the long term, what I see is that we simply breed spare organs, based on the patient’s DNA. You lost your arm? Okay, we’ll just breed a new one. Heart? Eyes? Liver? Kidneys? No problem.”
It looks like that it’s the scar tissue that overgrows a wound that stops the regeneration of limbs in mammals. Fingertips in humans BTW do regenerate to a certain extent.
There have been experiments with gene knockout mice that were able to regenerate limbs after the scarring mechanism was disabled.
It’s a few years since I read about that, I don’t know how far this research advanced.
I have no hearing in my left ear only tinnitus to all people with this problem good news
“There are still many things we don’t know about how the ear works. ” No kidding? Yet we know for a “fact” what the earth’s temperature will be in 2100!
My sympathies & even greater respect for you Anthony. Let’s hope this is successful! I sing with a girl (well she’s a girl to me in her 30s) & she developed a hearing impairment a few years ago requiring her to need hearing assistance, pretty cool things that she can adjust the volume, tone, etc, & even some pretty fancy electronic trickery, yet she puts many of us at choir to shame with her musicality, pitch, & timing!!! That’s why I thank the big fella up stairs each day for my health, eyesight, & hearing, & huge respect for those of us who have to deal with & overcome a disability on a daily basis. Aren’t Human beings amazing? Well to me they are, I appreciate there are those out there who consider us vermin & a blight upon Gaia! Bon chance, Anthony!
“Edohiguma says:
April 24, 2014 at 12:42 am”
I believe, eventually, we’ll be able to use 3D printers to literally print off body parts. I understand this has alreday been tried for some organs.
I too suffer ear problems and have done since birth. Tinnitus was bad when younger so too were ear aches which had me in bed for weeks sometimes. No-one can really appreciate how ear problems affect people. One of my friends recently had a severe ear ache and now appreciates my tales of problems past with ears.
And research like this requires lots of energy!
Really good news for anyone this treatment can help. My grandmother was totally deaf by the time see was in her teens. A wonderful lady who made the best of things and never complained about her lot in life. I hope this treatment enables many grandparents and grandchildren get closer together (parents and children for that matter).
I have had a cochlear implant since 1991, having been totally deaf for the preceding 18 years following a climbing accident (falling off, that is).
It is marvellous and life-chnaging yet at the same time limited.
The problems are mosty down to microphones – you get background noise and cannot distinguish a voice in a noisy environment. Beyond about 6ft the signal just falls away. Unless you have two implants which recently some people do, you have no sense of directionality so no idea which person is speaking and by the time you detect this they stop and someone else starts talking.
In the end for me the implant allows 1-1 conversations in quiet enviroments. That is so much better than the alternative. Peoples’ experience does vary – the key parameter being period of deafness before fitting. But I think I am fairly typical.
Fitting implants to deaf babies has the greatest benefit. They learn to speak, for example.
Implants do massively reduce tinnitus. In some cases that is the greatest benfit.
Good luch with the gene therapy – it is the way of the future no doubt.
“peter2108 says:
April 24, 2014 at 2:09 am
The problems are mosty down to microphones – you get background noise and cannot distinguish a voice in a noisy environment.”
I don’t have inplants but I suffer from this now. And badly too with mobile phone calls when there is a lot of background noise. Interestingly enough, the mobile handset I have actually allows you to “tune” it to your ears, especially with the hands free ear set. At first I thought it was a gimmick, but it actually works and calls are much clearer. And talking of gene therapy and genetics I have been diagnosed with haemochromatosis. Most poeple have enough iron in their bodies to make a nail. I have enough to make a bag full. My friends tell me to stay away from large magnets!
Hope you can get to benefit from this groundbreaking therapy, Anthony. How amazing would that be for you! Good luck
Nice. It would be interesting to see if it works on me with near 100% hearing loss due to meningitis.
However, it is best for everybody that pregnant/lactating mothers get enough vitamin D3 supplement (at least 4000 IU a day for pregnant and 6000 IU a day for lactating mothers) to minimize the risk of being damaged by anything. Also babies/toddlers/kids should get enough vitamin D3 (1000 for every 25lbs of bodyweight) to minimize the severity of symptoms from disease that would lead to damages such as hearing loss. Vitamin D deficiency is a major problem that’s not addressed enough.
Thanks for bringing this important news to our attention. And thank you for sharing some of your personal story regarding this.
A really strange situation. My cousin’s husband taught music at a school that was stressful for many reasons. As he neared retirement his hearing started to go. Eventually he retired and I asked him how his hearing was, he said it was coming back. Stress is a strange beast!
Is the wife very vocal, you might get out of the frying pan into the fire, with a hair all over the place too. Blessings come in many guises.
Only joking!
Interesting news. Hope it works out.
I’m reasonably certain this does not embryonic stem cells but gene splicing. That may be important to some.
The truly profound miracle here is that the NS actually published something worth reading rather than their ACGW drivel. This may be a sign. Superficial as usual, but at least not their usual fare.
That’s great news although it would mean my father would have to start paying attention at family gatherings.
I saw a documentary where the scientist was trying to understand the evolution of the ear. She was studying some form of rodent that gives birth to very premature babies that live on the underbelly of the mother and develop from there. She would take a baby at different stages of development and clean them with worms (which would eat the flesh clean from the carcass) and then scan the developing bones of the ear to make a time elapsed model. Turns out this animal started out with three main bones that also show up in ancient rodents (millions of years ago) in a less developed form but that fuse together to form a very complex shape. The largest bone of the ear breaks away from the jaw early on to fuse with the other bones. It looked like the ear developed as a primitive ear first, and then “evolved” into a more complex design while the baby was still growing in its very early stages. Interesting and humbling at the same time. I can’t remember the rodent’s name.
is this technigue the same as the technigue applied here?
I suffer myself, cant wait for this to be available 🙂
Konrad says:
April 23, 2014 at 5:41 pm
This is amazing news.
Do you know how soon after the trial this would be available?
______
Assuming the trials are successful (there will have to be several) this is a question about the regulatory process. A decade would be a plausible estimate.
I hope it works and I hope it benefits Anthony.
This is great news! If anyone is interested in crying some tears of joy, serarch youtube for “cochlear implants turned on”. HenryP has the right idea!
HenryP, nope that is an electronic inplant.