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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Anthony, you say “I have just enough function left to be assisted by hearing aids, but the aids themselves do damage to the remaining hair cells.”
My husband is recovering from Pneumoccocal Meningitis (14 weeks in) and he has lost all hearing in his right ear, and has recenctly got a hearing aid for the profound loss in his left, both due to cilia damage/destruction. Are you saying that the aid will damage the remaining cilla in the left ear?
Shouldn’t we be spending this money on something useful like saving the planet from CO2? We have enough humans on the planet already, now they have to be able to hear too?!?!?!
(sarc)
Anthony, all the best and prayers for you and your hearing (and the trials going on – although it’s probably better to wait for “SP2” or “SP3” as it were….).
My son had a massive injection of antibiotics shortly after birth for Strep-B which damaged his inner ear, but it was a life-or-death decision, so no real alternative was probably available…fortunately he didn’t lose everything, but had middle ear issues putting him in the
severe-to-profound category which so far Widex Fusions (forerunner of Dreams) are helping out OK. That said, a gene-splice therapy would be wonderful.
He uses FM transmitter/receivers in school, but there are lots of situations where that isn’t enough (e.g. noisy class, teacher cooperation, etc.). But it’s a wonder the things that are available today.
Looking at what he’s had to go through, I can only imagine the h-ll it must have been for you. Folks often misinterpret being hearing-impaired as being slow or downright dumb, when in point of fact it’s often “did I hear that right” or “was that a ‘d’, a ‘b’, or a ‘p’ ?”. And the crazy thing is, even with almost perfect correction, the lag is still there…
Congratulations to you for getting through all of it so well, and for having such a great blog, and for your work in spreading the truth about weather and climate.
A few hearing links if folks are interested (sorry if not):
Dr. Staecker, who’s running the study
http://experts.kumc.edu/en/people/565-Hinrich_Staecker
A paper on promising (umbilical) stem-cell treatment methods:
http://kuscholarworks.ku.edu/dspace/bitstream/1808/8056/1/Devarajan_ku_0099M_11665_DATA_1.pdf
There are others pointed to from Dr. Staecker’s site, but unfortunately some of them are behind
paywalls, even though the public has already paid for them….$89.00 to download a pdf or other
file is a bit much….
as in w$w$w.ncbi.nlm.nih.gov/pubmed/23560526 which is the one I really wanted to
read (remove the $ signs)…..sigh….
Finally, a couple more for folks with hearing issues or interests:
http://www.hearinglosshelp.com (Dr. Neil Bauman)
He has a LOT of information on ototoxic drugs. Even for folks with NO hearing issues, it’s a good idea to check out this info, as ototoxicity is a potential problem for everyone, Lasix users in particular (had that with my son, too).
And a good “meeting place”: http://www.hearingaidforums.com/ Lots of helpful info there (look for Doc Audio and Um bongo)…
Folks interested in the difficulties of deafness or hearing impairment might want to watch (or read about) “Sue Thomas, F.B.Eye”… Some interesting episodes there about “big D versus little d deafness”, which new parents might find interesting/helpful.
Again, major RESPECT for folks who have to pull through with this or other difficulties…
“David, UK says:
April 24, 2014 at 12:48 pm”
Have a look at Werner Gitt, “If Animals Could Talk”. ISBN-10: 0890514607,
ISBN-13: 978-0890514603. Really interesting info about eyes, ears, etc. I have the same viewpoint, which is, sadly, rare over here.
My son has middle ear as well as inner ear issues (anvil malformed). I’ve heard that multiple ear infections and antibiotics can cause that…not sure. They don’t want to do a Stapedotomy, as even with that there’s still loss, and if it doesn’t work, then it’s CI time….so I’m hoping and praying
this test will work out and the therapy helps people.
Some good news is that there are now CI’s (Cochlear Implants) that don’t risk the residual hearing, but if a (relatively) non-invasive treatment becomes available, that would be great.
“Janice says:
April 23, 2014 at 5:55 pm
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.”
Just think, though, if they make a sequel to Avatar….
(Just kidding….)
Here’s hoping for a huge success. Hearing loss that can be restored, with nature’s own materials.
One of the most widely publicized cases of severe hearing loss is the case of Rush Limbaugh, who had a relatively sudden, almost total loss of hearing; a major handicap for a prominent radio and TV figure (nothing to do with his political positions).
So Rush underwent some significant remedial procedure, which was not simply installing megawatt Bose stereo hearing aids. Apparently the result is an almost total return to normal hearing.
You might try contacting him Anthony, to find out exactly what he had done. But in the meantime, this gene trickery sounds exciting.
I heard within the last week of a similar viral messenger sent to switch a SINGLE BASE PAIR in some aberrant gene, that will completely cure some malady, in this patient, which currently, I can’t recall.
This is not like having a clone of yourself in the freezer, to raid for spare parts as things crap out.
This little viral bugger goes about switching bases in the adult patient, and replicating itself to take over new cells and switch them before they replicate; so over time, your body with viral turbo boost simply replaces every cell in your body (nearly) over time, with government approved ones, as the old ones wear out.
It’s magic, I tell ya!
Keep on it Anthony, and nyet with the selfishness. The first law of altruism, is that you have to survive to be able to do good; and you ARE doing good’s work.
G
It’s almost too clever to believe; but it is happening.
Congratulations and godspeed, A.
I hope this works for you, Anthony.
Can kinda relate. I wish there was therapy for a crushed heel. A truly crushed heel will never, ever heal in a lifetime. Every day ends the same, swollen and sore.
Polish idea of cancer.
Nanoparticles , microcapsules or slightly smaller than the virus , are used in human medicine for several years. Then the transport of drugs. But they have two drawbacks . Firstly, tend to be unstable – quickly disintegrate , as if by themselves .
Secondly . – In the body quickly overlap plasma proteins, which alerts the immune system , which sends phagocytic cells and within minutes the nanoparticles are removed – said prof . Ciach.
His nanoparticles do not have these disadvantages – they are durable, easy to manufacture ( ” formed almost the same” ) and to this kind of untouchable . The result can be very long to circulate the blood , looking for cancer cells. Why do you owe ? Polysaccharide coating that is highly hydrated and sweet. It first makes them invisible to the immune system. The latter – attractive for cancer cells .
Here we have to go back 90 years , when German biochemist Otto Wartburg , examining their metabolism, detected a fundamental difference : instead of oxygen cancer cells ” fed up ” glucose. Consume it up to 200 times more than normal. This is because cancer cells undergo anaerobic glucose changes , which are much less efficient than aerobic . For this so-called . Wartburg effect its discoverer seven years later received a Nobel Prize .
Weakness of tumor cells to glucose is used in the diagnosis of cancer (e.g., administered to a patient labeled deoxyglucose fluorine -18 and tested its concentration technique of positron emission tomography – PET- CT) . But not in the treatment of cancer.
Prof. Ciach precursor here is : firstly surrounded their sugar polysaccharide nanoparticles , then put into the doxorubicin , and finally let them into the bloodstream. When the concentration of the drug investigated in cancer cells , we found that there was gone by 20 percent , as in the standard targeted therapy , but as much as 500 percent .
http://wyborcza.pl/piatekekstra/1,137924,15850377,Polski_pomysl_na_raka.html
The science blogosphere is rooting for you, mate. Few of us have met you, but your selfless work means a lot. So do you.
The very best of luck with this Anthony, fingers crossed. After exhausting the latest titanium fix (neat but unsuitable for my mess) now waiting for bio epoxy. 27 years and counting, never give up hope!
Anthony I sure hope something comes along and it works,
BUT
theres quite a lot of fake n fraud science..just like the climate scene In Big Med.
see this. as folks above have warned be wary, and wait a bit.
=====================================
http://retractionwatch.com/2014/04/25/former-mount-sinai-postdoc-faked-gene-therapy-data-ori/
Former Mount Sinai postdoc faked gene therapy data: ORI
by ivanoransky
A former postdoc at the Mount Sinai School of Medicine in New York faked data in four published papers, one submitted manuscript, and four NIH grant applications, according to new findings by the Office of Research Integrity. We reported on six retractions from Savio Woo’s Mount Sinai lab in 2010, from the Proceedings of the […]
Read more of this post
Anthony,
I can only wish you the best. My wife is loosing her hearing and can understand how this impacts her life quality and also for those around us.
Rush Limbaugh recently had a second implant and in his normal way describes the problem and the procedure in comprehensive detail. I suspect that you are already aware of the specifics, but it also is an informative read. He still has issues but is thankful since his career would end without hearing ability.
Again wish you restoration of your hearing.
http://www.rushlimbaugh.com/daily/2014/04/24/where_i_ve_been_the_last_week
“ozspeaksup says:
April 25, 2014 at 5:17 am ”
Fellow was probably suffering under “publish or perish” – that and political/commercial pressure are producing bad science and tempting good scientists to be unethical…sad…
Good points (always good to be skeptical). There are other applications for gene therapy as well, where there are proven results. A number of causes of blindness are genetic in nature (Usher Syndrome, LHON, etc.) see https://www.fightingblindness.ie/cure/genes-and-gene-therapy/
for some examples, infos, and links, and one fellow was cured of LCA:
” Professor Robin Ali pioneered the first ever successful trial of gene therapy for a form of blindness called Leber’s Congenital Amaurosis.
The research conducted by Professor Robin Ali at University College London and Moorfields Eye Hospital in London brings hope to millions affected by eye diseases as a result of revolutionary gene therapy treatment.
Prof Ali treated a patient who has a rare genetic eye disease called Leber’s Congenital Amaurosis (LCA). Steven Howarth, then 18, from Bolton, England, had been left with extremely poor vision and completely unable to see in the dark.
Following the treatment, his sight improved sufficiently to be able to navigate a ‘maze’ in conditions similar to street lighting at night.
Prof Ali managed to replace the faulty ‘RPE65′ gene causing the condition with a normal gene. The therapy was delivered in a harmless virus or ‘vector’ which was injected into the back of the eye and spread to the cells.” (quoted from fightingblindness site)
The related study is here:
https://www.ucl.ac.uk/ioo/genetics/gene-and-cell-therapy/our-research-programme/current-gene-cell-therapy-clinical-trials/accordion01/RPE65/
On the hearingaidsforum that I mentioned above, there are a number of threads on gene therapy and related studies under the “Sensorineural Hearing Loss & Stem Cell Research” tab.
So even with all the “noise” out there, there’s a lot of hope as well…
This is the most amazing and very very good news…I pray a lot for this to be successful and bring hearing back to normal for many many people.
One of the test report shows that my daughter is having severe to profound Sensorineural hearing loss because of non functional hair cells. MRI and CT scan results came negative. Doctor came to a conclusion that my daughter is having problem with inner ear hair cells.
God bless all those involved in this process.
RK
For a brief moment, I wondered how my own words could be reflected on your page!! I began to lose my hearing at seven, wore hearing aids at ten, have wonderful speech ability, and read lips beyond belief. I’ve been waiting for entire life for something like this which would restore my hearing. Cochlear implants are NOT appealing to me — not true hearing. I want hearing to be restored naturally by transplant or regrowth, and suddenly this article is sent to me by my brother who knows how I feel. Wonderful, wonderful, wonderful that medical fields are at last turning to more natural ways of restoring hearing. Would love to be part of the 45 who might take place in the testing, but I’m 75, and beyond the age range. But the hope and joy this idea gives me is wonderful!! I’ve lived a full and happy life in the world of deafness, married, mother, retired from a great career, and currently work in community things — always expanding, and struggling in the hearing world which never slows down for the hearing impaired. To know that future generations might be able to have hearing restored naturally
is a ray of sunshine to me!!!! Oh, wonderful, wonderful, wonderful!!!
Konrad says:
April 23, 2014 at 5:41 pm
It might not work to treat one ear at a time. A virus would trigger an immune reaction, and once that happens, the virus would no longer be effective if the process of DNA transformation requires the virus to pass through spaces where antibodies could reach them. I suppose you could have a second independent virus deliver the same treatment to the other ear, if the immune system’s response to the first virus had no effect on the second virus.
David (UK) says
…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.
Henry says
You are correct that Darwin did accept that there is a God at the end of his life.
Problem is: either everything is coincidence or everything is a plan.
You cannot have it both ways.
The other day we had a blind preacher on the Hour of Power and I was amazed.
I would not know how to get through one day if I were blind.
Yet, in the final analysis, the truth is that nobody is absolutely perfect. The truth is that God reveals Himself to us there in our weakness(es), when we are down. Religion is not a stairway to heaven, as if when you keep to enough rules, you will make it. True religion is God coming down to you and meeting you in your need, which you had just asked for….
http://blogs.24.com/henryp/2011/07/23/why-do-i-believe-in-god/
Have a great weekend you all.
Stem cells from their own blood are good. Even when you download blood stem cells are activated and treat the affected place in the body. They as if their own intelligence.
My hearing loss is from noise exposure over a lifetime. At 64, I won’t hold my breath in anticipation of a cure trickling down to me. If it does, what a wonderful thing that would be.