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Genes In Human Inner Ear Cells Restored (6/17/2007)
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| Human hair cells. (Credit: Image courtesy of University of Virginia Health System) |
Researchers at the University of Virginia Health System have discovered a way to transfer genes, which they hope will restore hearing, into diseased tissue of the human inner ear. This important step brings scientists closer to curing genetic or acquired hearing loss.
Dr. Jeffrey Holt, associate professor of neuroscience and otolaryngology at UVa, and his research team, including Dr. Bradley Kesser, an assistant professor of otolaryngology, targeted a gene known as KCNQ4, which causes genetic hearing loss in humans when mutated. They engineered a correct form of the gene and created a gene therapy delivery system that successfully transferred the KCNQ4 gene into human hair cells harvested from the inner ears of patients with hearing loss.
"Our results show that gene therapy reagents are effective in human inner ear tissue. Taken together with the results from another group of scientists who showed that similar gene therapy compounds can produce new hair cells and restore hearing function in guinea pigs suggest that the future of gene therapy in the human inner ear is sound," Holt said.
Hair cells have hair-like projections that line the cochlea. In people with normal hearing, hair cells convert sound into electrical signals, which are ultimately transmitted to the brain. People with hearing loss suffer from too few, damaged or missing hair cells. Holt's past research uncovered the speed at which hair cells develop in mouse embryos, a finding necessary to help researchers learn how to regenerate hair cells. With this current development, Holt and his team could one day restore the hearing process in damaged hair cells.
"This is a critically important step forward. We hope this breakthrough will propel the field of hearing and deafness research toward our collective goal of curing genetic and acquired deafness," Holt said.
This discovery will appear Thursday, June 14, in the online issue of Gene Therapy.
Note: This story has been adapted from a news release issued by the University of Virginia Health System
Comments:
| 1. |
Dave |
6/19/2007 11:21:08 AM MST |
This is fantastic news! I'm deaf in my left ear since birth, and the thought of being able to use it makes me very happy.
I hope I am able to recieve the fruits of this labor someday.
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| 2. |
Darren |
6/22/2007 3:40:36 PM MST |
Wow. I have this exact problem in both ears (too few cilia in the Cochlea). My mother had Rubella during her pregnancy and this was thought to cause the birth defect. I've worn hearing aids for 35 years and consider myself a poster child for hearing aid advancement. This sounds like a wonderful development that perhaps someday can benefit many people. |
| 3. |
Dan |
6/24/2007 5:38:25 PM MST |
I think that it is the most dumbest thing because I am born deaf and I am damn proud of it. Being Deaf is who I am and I am so tired of people trying to change us. Leave us alone! What is it with you people accepting blind people, mentally challenged and so on. But you do not accept deaf people? That is so messed up. I am happy to be deaf and most people that I know are happy to be deaf. You people and medical people need to learn to leave us alone! Case closed! It is YOUR problem for not accepting Deaf people. Those deaf people who have "fix me" attitude, need help because it is obvious that they do not accept themselves. You all need a life period! I am proud to be genetically Deaf! Thanks for your time |
| 4. |
Ryan |
6/25/2007 7:49:22 PM MST |
I don't understand Dan. Are you angry because some people who are deaf or whose hearing may be failing look forward to being able to hear in the future? There is nothing wrong with being deaf, but is the a problem with making whether you hear at least an option? |
| 5. |
Dan |
6/25/2007 8:36:59 PM MST |
Not angry... just annoyed because like you said there is nothing wrong with being deaf, but WHY you want to change that? If I have deaf kids, I will be thrilled! The way I see it is that heaing people (society) tend to force deaf people to be hearing. Let me play devil advocate... I am deaf and I am married to deaf woman; we gave a birth to a hearing child, then we decided not to accept that this child is hearing... I decided to have the hearing taken out. How would hearing people feel about that? If you say angry or upset, that is how I feel about people trying to change us deaf people. We just wanted to be left alone AND accepted by society.
If you know deaf history you will understand more about the feelings that I am feeling.
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| 6. |
Branden |
6/25/2007 9:07:30 PM MST |
Hi Dan,
It seems to me that people are looking for options, read diversity. When has diversity been a problem?
-Branden
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| 7. |
Dan |
6/26/2007 9:57:01 PM MST |
I never have problem with diversity. Why don't we leave diversity alone? This crazy invention is a way of reducing divrsity. I see this invention as a way of reducing diversity because I believe Deaf people is a group of diversity and these people are trying to get rid of us. I think this invention needs to be stopped. What's wrong with hearing aids? What's wrong with American Sign Language (ASL)? I love ASL makes me feel more connected with me. I know many deafies feel that way. It seems that hearing people are trying to abolish us. These people still since 1880 in Milan Covention. So, enough with trying to prevent deaf genes. Being deaf is who we are. I understand if hearing people lose their hearing and they want it back, I am fine with it. However, leave those people including me who are born deaf alone. Why try change us? Enough is enough. |
| 8. |
Branden |
6/28/2007 9:39:32 PM MST |
Hi Dan,
I read your reply many times, and realized you may be talking about genetic diversity, which I understand and agree with. Deafness has been around a long time, and would have been selected out of our genome if it didn't imbue some advantage.
Not being deaf, and not knowing anyone deaf, I don't have first hand experience. However, I've read that deaf people have better spacial understanding and orientation than people dependent (or addicted) to vision alone.
I'd like to hear your experience in this area.
Best Regards,
Branden
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| 9. |
Dan |
6/29/2007 8:24:40 AM MST |
Hello Branden;
I am Deaf and I grew up with doctors trying to fix me by giving my hearing aids, but they do not work for me at all. Then my parents decided to give me Cochlear Implant... It is surgery procedure to "help" you "hear". I did not understand what was going on. Once I figured out what was really going on (that's after the surgery). I was really pissed off because I never wanted it at the first place. I left my family when I was only 13 years old and I lived with Deaf family for three years. I moved back home because I decided to forgive my family and move on. I am now a student of Gallaudet University and I am very happy. I am majoring in education, ASL and Deaf Studies. My goal is to educate hearing people about Deaf people and Deaf Culture. I am annoyed by doctors and professionals trying to fix us. Deafness is not life threatening. I think professionals do not want to help us... they just want to take advantage of us because they see us as their money makers. I hope I explained to you clearly.
Dan
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| 10. |
Suzanne |
7/2/2007 7:13:45 AM MST |
When I think of my mom who was born hearing and began losing her hearing at age 32, and then read Dan's entries, I know he does not understand the devastation caused by a progressive hearing loss on the life of someone who grew up hearing and then became deaf after she was and adult. It is one thing if you have developed you life in one manner (deaf), but if you develop your life hearing it's completely different. All her friends and family are hearing. The work she does requires her to have hearing, her entire life is in the hearing world and at age 55, you know she would not, could not re-define who she is and how she interacts in life. So, I see Dan's points about his being deaf and what a great full life he has. Do you think he will ever understand it from a hearing person's point of view who lost her whole way of interacting with the world? She has enough hearing to barely get by, but many sounds hurt her ears so that hearing aids are a problem in many situations, and even when she hears, speech is not clear. She was a music teacher. Need I say more? Mostly, she is cut off from many activities. She is neither hearing or deaf. Just miserable. I wonder if Dan can understand her situation? |
| 11. |
Dan |
7/2/2007 1:51:21 PM MST |
Hello Suzanne;
As I said on June 26th, "I understand if hearing people lose their hearing and they want it back, I am fine with it"
If a HEARING person wanted to get their hearing back... I am fine. I can understand your mom situation 100% because I try to think about my life if I suddenly lost my sight, I will be miserable. However, your mom seems to be afraid to accept her deafness. I can give you information about late deafened (meaning people become deaf later in life). I have known many late deafened people and they told me that once they learned about opportunities for late-deafened and they learned how to meet deaf people and jobs that helps deaf people. I know many deaf people plays music things like that.
I am sorry about your mother being devasting about her losing her hearing. My family is ALL hearing and I have friends who are hearing AND deaf friends also. I am not trying to top you... I am just trying to say being deaf doesn't mean that it is end of the world. She can learn sign language and get a video phone (www.sorensonvrs.com) and make phone calls and VCO (voice carry over) is provided. I make my own calls all the time and I love it because no more phone bills!! So, maybe your mom needs time to research about deaf people and how to meet people. All your mother needs are supports from her family and friends and self-acceptance. I know easier said than done.. But in time, maybe she will not be miserable. What do you mean by cut off from many activities? care to explain?
Sorry for long message. Hope it helps.
Dan
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| 12. |
Michelle |
7/3/2007 2:22:24 PM MST |
Dan I would appreciate your advise on something if you dont mind. I'm a hearing 25 year old woman, who is in love with a deaf man. We met about 5 years ago and just came into contact again; these feelings were not there before, perhaps because I have matured. He lost his hearing when he was two from an illness and does not sign, since at that time being deaf as mind boggling. He does however communicate very well and reads lips, but needless to say, there are communication barriers. Will a life with me make his more difficult? What other challnges should I expect? Please offer your opinions. |
| 13. |
Dan |
7/5/2007 7:59:11 PM MST |
Hello Michelle-
as you asked about your relationship with a deaf man. I know many deaf friends of mine dates with hearing people. A life with you will NOT make his life difficult, only if you accept his deafness and he accepts you as a hearing person. Both of you need to sit down and try to understand where both of you are coming from. I dated a hearing woman and my biggest mistake was not trying to understand where she is coming from.
My question for you is: Are you willing to help your boyfriend to communicate with people that he is having hard time understanding for the rest of your life? If the answer is yes, then you will have no problem.
Other question: Is he willing to try to understand where you are coming from since he may do not remember what it is like to hear? If he says yes, then you have nothing to worry.
The challenges would be the number of people trying to have conversations and your boyfriend might want to try keep up and you and the company may have to slow down to make sure that he keeps up. It would be tough to keep him included 100% of the time (Hearing people will exclude him unconsciously).
Hope this helps.
Dan
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| 14. |
Ali |
12/11/2007 11:45:14 AM MST |
I agree with Dan!
I know a Deaf guy who was born with no hearing at all to hearing parents who knew nothing about deafness. But instead of going cochlear, they (and his older brother) went and learned ASL. He goes to a Deaf school and he and his family seem perfectly happy.
BUT, like Dan said, if they grew up hearing, losing it can suck, in which case it would be nice to fix.
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| 15. |
James |
12/23/2007 6:21:10 PM MST |
To be honest I think ironically that Dan is a little close minded, this news is very good news, nobody is trying to force Dan to have surgery in the future he is welcome to remain deaf, however has Dan ever considered how expensive it is to raise a deaf child? How much more time they require? How this time cuts into a job? How even thought he is ok with his disability some people may not be? I do apologizes for being blunt but it is how I feel. |
| 16. |
Thomas |
1/9/2008 12:11:25 PM MST |
Dan,Do you have any deaf children? |
| 17. |
sebastian |
1/25/2008 2:37:01 PM MST |
Hi i think this could be an incredible advance if applied in humans, like it could also regenerate other cells like hair cells for bold people, if it happened it would be great. |
| 18. |
Slip |
4/29/2008 3:33:04 PM MST |
Dan,
1. Since you had a cpchlear implant, gene therapy wouldn't work for you because of the damage the implant caused.
2. Gene therapy would be a choice, either of the individual or their parents.
3. Deafness is amedical condition, not a normal condition.
If I can regain my hearing, I don't care what you say to try and deny me or anyone else that right, you bigot. Being deaf does not make you special. It just makes you DEAF!
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| 19. |
Laura |
8/3/2008 12:06:56 PM MST |
I myself, am not deaf but I've been dating a deaf man for almost five years. He was born deaf and he didn't have a problem with it. It was all he knew, so why should he? His family learned BSL (as we're from Britain and not America) and when I first met him, I decided to learn it too. We instantly became friends at a young age - and I wanted to be able to understand him and him me. When we reached our late teens, our feelings grew and we've been a couple ever since.
Recently he proposed, and we're planning to marry next year. He's being taught to say his vows out loud because he wants the world to hear. He's researched so many different cures of deafness, desperate to find something to rid him of it. At one point he considered offering himself as a volunteer for a new treatment idea which I was upset about. I couldn't understand why'd he'd risk his life to hear when I was content with him being him. I love him and I don't see his deafness as a disability. It took him a while to explain why he wanted treatment. He said he wanted to hear me laugh, to hear me say his name and to hear me tell him I loved him. He said that through signing, we could talk but there were still those few things missing. He said that it hurt not having ever heard the woman he loved laugh, moan or speak. Is that so wrong? That he wants to be able to hear those things? Although he can talk out loud, it's hard to decipher anything he says. He can say my name, I love you and our marriage vows. But I hear his laughter, and if I couldn't hear it I'd be pretty distraught myself. His laughter brightens my day, so I understand why he wants the same.
I understand you might not want to be able to hear, but everyone has a different view. I do agree however, much of society almost...shuns the deaf AND blind. They're not disabled, and if anything they're nicer people. They're far more perceptive, observant and usually intelligent for the most part. However, it's unfair that scientists who want to make a small group of people happy should be critised.
Everyone has their choices.
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| 20. |
rajendra |
2/5/2009 6:37:11 PM MST |
hii; this is a great and wonderful achievement; i also do have a problem.
and m frustruated and i am eager for these trails; at the earliest possible.
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| 21. |
Stephanie |
5/18/2009 6:50:14 PM MST |
I have been praying for this, and I hope it happens soon. My daughter is hearing impaired, and she could lose more hearing anytme. I would like her to have this OPTION. It would be a choice, a gift if she wants it. She has a wonderful future either way, but to lose more hearing after living in a hearing world, well, it just breaks my heart. |
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