7 ms·

Gene therapy restored hearing in deaf patients

355 points by justacrow 1y ago

jonathanlb 1y ago
This is great news. Hopefully this will be expanded to other forms deafness like those caused by ototoxic medication, ear infections, and general sensorineural hearing loss.
- im3w1l 1y ago
  This particular study looks like it's dealing with a pretty narrow condition and solution (protein missing - add gene for protein). I don't think this particular research can be extended the way you hope.
  - jonathanlb 1y ago
    I agree. The causes of deafness I mentioned are diverse and fall outside the specific focus of this current study. Ideally, future research will address deafness caused by these and other other factors.
  - janeerie 1y ago
    Yes, I would really like to see research targeting Connexin 26, since it's the most common cause of hearing loss, but it seems it's much more difficult to "cure."
vonnik 1y ago
adjacent, same trend: https://news.ufl.edu/2024/09/blindness-gene-therapy/ https://news.ufl.edu/2024/09/blindness-gene-therapy/
- agumonkey 1y ago
  somehow it seems the singularity is here .. i hope it's well distributed
  - fao_ 1y ago
    I don't see how this represents the singularity. This is all humans doing work and building knowledge, much like it has always been. Can you point to a specific section of the article that elicits that assumption? Thanks
    - agumonkey 1y ago
      The general acceleration in research and outcomes, we're now fixing deep issues more and more often. And hardware / software improvements keep coming too, even outside of computing. Sorry if that sounded pompous but there's a slight feeling that we're not plateauing, quite the contrary.
    - perlgeek 1y ago
      I'm not super familiar with this, but I think many of the gene therapy advances did involve some form of machine learning to find relevant genes. Some might also use protein folding simulations, and the best ways to do that involve narrow AI. I'd love to hear from somebody in the medical research field how much AI/ML actually speeds up their progress.
- perlgeek 1y ago
  ... and then there was this Phase-1 trial in Japan about re-growing missing teeth. We're really making astonishing progress in many different areas in medicine these days. They are mostly pretty narrow, but also so awesome that ~20 years ago, many doctors would have called it science fiction.
wetpaws 1y ago
[dead]
d0100 1y ago
Anyone from the deaf community that can tell us how these kind of news are received? I wonder how this compares to animosity towards coclear implants by a subsection of the community
- guerrilla 1y ago
  That was my first thought too. I wonder how many people would choose to stay deaf just to stay in the community.
- retrac 1y ago
  Controversial in the same way cochlear implants are. Many deaf/Deaf parents want children who hear. And I think absent the cultural consideration, almost all would want children who hear. But you can't ignore the cultural consideration. If you are deaf, and have a deaf child, curing that child's deafness means they will move away from you later in life. It's a kind of alienation even when the child remains bicultural, they usually end up almost entirely in the hearing world. That said, most deaf people who have children have hearing children anyway. Hereditary deafness like that is relatively rare like that. But for people from such families, and who live in a culturally deaf world -- they are not disabled. The cultural environment they live in is ... one in which deafness is not disabling. And it's going to be a very high hill to climb to convince them that they are missing something. They certainly don't feel it. This is particularly true in the United States which has such a proud tradition of deaf culture and education -- you can go all the way to doctorate level studies in ASL, work in ASL, the hearing world being a strange foreign culture you only rarely wade into -- only rarely need to. I'd cure it for myself, and my child if I had one. No question. But I'm not culturally deaf. I feel isolated by it in the same way most hearing people anticipate deafness to be as an experience. But again -- people who live in the deaf cultural world -- they do not feel that, and they don't feel disabled because, in their context, they aren't. It's hard to communicate this to most hearing people. The usual response is dismissive, and unfortunately I think a lot of that ultimately goes back to very old metaphysical attitudes towards language and intelligence. A lot of hearing people still don't believe, deep down, that sign languages are equivalent to spoken languages, in particular. It's just gesture. You're lacking something essential to the human condition without spoken language. Etc. But for the culturally deaf, nothing is missing from their lives, except the perception of sound.
artursapek 1y ago
[flagged]
- deleted 1y ago
  [deleted]
- deleted 1y ago
  [deleted]
- pugworthy 1y ago
  If this is a joke it's in extremely bad taste
smath 1y ago
Regeneron had announced positive results in its gene therapy drug for deafness in Feb 2025: https://investor.regeneron.com/news-releases/news-release-details/latest-db-oto-results-demonstrate-clinically-meaningful-hearing https://investor.regeneron.com/news-releases/news-release-de...
thomassmith65 1y ago
It's good that the article title doesn't use "congenital deafness", but it would be even better if it were simply "Deafness Cured!" /s
- thomassmith65 1y ago
  Without sarcasm: Please rename this to "Gene therapy restored hearing in patients with autosomal recessive deafness" so as not to raise and then dash hopes of deaf HN readers
max_ 1y ago
I see alot of advances powered by genetics now days. Is there a specific field in genetics pushing this? I used to hear buzz about CRISPER/CAS9 is it what is underlying most of these advancements? How come alot of gene editing stocks have taken a serious beating if the tech is so good. Many, many gene editing stocks have lost more than 90% of thier value since IPO.
- searine 1y ago
  The tech behind this is not new or difficult. The issues are related to safety and regulation. Early efforts in gene therapy had disastrous results and current treatments are not trying to repeat past mistakes. There is tremendous potential for gene therapy to cure disease, however it needs (and so far has had) strict regulation, particularly if the changes can be inherited.
  - beambot 1y ago
    > Early efforts in gene therapy had disastrous results Can you share examples...? Just curious as an outsider looking in.
    - ortusdux 1y ago
      https://pharmaceutical-journal.com/article/feature/gene-therapy-from-catastrophe-to-cure-in-20-years https://pharmaceutical-journal.com/article/feature/gene-ther... "The most notable obstacle faced in the gene therapy field was that of Gelsinger in 1999, who is understood to have died after his body overreacted to the adenovirus vector. Gelsinger had a rare disorder in which the liver lacks a functional copy of the ornithine transcarbamylase (OTC) gene and, consequently, the body is unable to eliminate ammonia, a toxic breakdown product of protein metabolism." "A gene therapy for children with severe combined immunodeficiency (SCID) was delivered [to two independent groups in London and Paris] and was incredibly successful,” explains Griesenbach. “But, [in 2008; between three and six years later], a small proportion of children [in Paris] developed leukaemia induced by the vector, which had inserted itself into a gene that controls cell division [4] .”
S4H 1y ago
How challenging is it for a person who has been deaf for let's say 20 years to suddenly regain hearing?
- jallmann 1y ago
  I can't say anything about the specifics of this treatment, but in terms of their ability to fully benefit from hearing, it would depend on when they became deaf, and the severity of their deafness. If they were born deaf, or lost hearing as a young child during the language development stage, then it would probably be a long adjustment. Things would just be noise and it would take a lot of training to distinguish sounds, speech, etc. And unlike a cochlear implant, you couldn't just take it off to give your brain a rest. If they had hearing loss later in life, or some residual hearing, then they probably have a better chance of re-adjusting to hearing.
- ordu 1y ago
  Really challenging. In some aspects it can be worse than to regain vision. If you are not accustomed to sounds, they can be annoying, and may make you feel tired. The same can happen with vision, it is just too much, but you can close your eyes, and shut out vision stimuli. You can't do that with hearing. At least if you regain hearing with normal sensitivity, you can be overwhelmed by sounds of your body. It is easier with implants, which can be shut off.
- dd82 1y ago
  If they've been deaf from infancy, basically the entire hearing center of the brain is non-existent. So they'd be hearing sound, but processing it into meaningful content would not happen, if at all. So basically, its like having a cacophany of sound that you can't filter and process... As for others, one thing hearing people, particularly monolingual hearing people, don't understand very well is that hearing != understanding. Just because you hear a sound doesn't automatically equate to it having meaning. The default for many people is to just SPEAK LOUDER and slower, which does not help in the vast majority of encounters
squigz 1y ago
You know, whenever treatment for autism comes up, we get a lot of comments heavily suggesting curing autism is basically eugenics. Why is it that some things are seen as a disability we should try to fix in our children, and others - which are in many ways just as debilitating - seen as some kind of beautiful part of humanity?
- joefourier 1y ago
  Autism is a spectrum disorder and I don’t think it should be controversial to cure low functioning autism. However, high functioning autists can be argued to be more of a personality variant than a disability, with different strengths and weaknesses compared to neurotypical people. Society benefits greatly from supporting high functioning autistic people in say, technical fields where hyperfocus, narrow obsessions and systemising thinking are an advantage. Meanwhile, having a genetic condition like haemophilia doesn’t give you any conceivable advantage.
  - squigz 1y ago
    > Society benefits greatly from supporting high functioning autistic people in say, technical fields where hyperfocus, narrow obsessions and systemising thinking are an advantage. At the expense of those people having to live with all the unmentioned negative aspects of autism. (To say nothing of whether those are actually positives or not. Personally, I don't see how hyperfixating on something for a few weeks at a time at the expense of all my other responsibilities is a superpower, but hey)
  - iteria 1y ago
    I don't even think there should be a conversation around high functioning autistics. My kid suffers from autistic catatonia. She's also extremely high functioning. I'm sorry, there is no world where I'm going to say no thanks to a cure for my daughter's body suddenly locking into place for an unknown period of time or losing the ability to speak or function randomly or hell just understand human expression without intense intervention. We can argue about their special brain powers or whatever, but all I'm seeing is that high functioning autistic have a much higher rate of self-harm and suicide. It can't be that great.
jhaddow 1y ago
How would one find out if they have this type of hearing loss? I have moderate to severe hearing loss in both ears since birth and there’s never been an attempt that I’m aware of to diagnose the cause beyond a standard inner ear examination.
- apt-apt-apt-apt 1y ago
  Asking your ear doctor seems like a good idea for this, rather than random people on HN..
  - jhaddow 1y ago
    Do you want to be my ENT?
- codytruscott 1y ago
  Whole Genome Sequencing is affordable now. I’d suggest a 20x hifi long read from broad clinical labs for $1200 or so. Use opencravet to dig into the results. They just posted a webinar for personal analysis https://wse.zoom.us/webinar/register/WN_-VvYJ8FKRcGaKCQtLFrUpQ?_x_zm_rtaid=jJ-eSZOuR7C63JL6AWUgDw.1749849096416.125c494ceb92f310ba2b3c244ccaadf5&_x_zm_rhtaid=831#/registration https://wse.zoom.us/webinar/register/WN_-VvYJ8FKRcGaKCQtLFrU... Franklin by genoox is a slicker and possibly more approachable product depending on your interface preferences. Genetic research — due to the number and subtly of variants — is ripe for citizen science in my opinion.
  - gavinray 1y ago
    As a follow-on to this: If you have partial genome data from 23andMe, Ancestry, etc, you can use what's called "genomic imputation" to do a sort of probabilistic gap-filling in your genome. It's a bit tricky to do yourself, but there are paid services that will run the imputation for you and share the results. I paid $15 for mine at https://dnagenics.com https://dnagenics.com --- @codytruscott I signed up for that webinar, I hadn't heard of this tool before, thanks! Got any other useful links/tools to share by chance?
coolKid721 1y ago
okay can we cure tinnitus please
froggertoaster 1y ago
What immediately sprung to mind is how the deaf community has seen things like this as a personal and existential threat. To me it's an obvious disability, and deaf people SHOULD want to be cured, but tribalism wins that argument all too often.
UltraSane 1y ago
This is interesting because the quack who created Chiropractic wrongly thought he cured deafness with spinal manipulation. Just shows how powerful the real scientific method is.
deleted 1y ago
[deleted]
FranckDernoncou 1y ago
The research study is behind a paywall: https://doi.org/10.1038/s41591-025-03773-w https://doi.org/10.1038/s41591-025-03773-w I'm always amazed that some people still choose to publish in paywalled journals.
Saul1998zx 1y ago
[dead]
Elaris 1y ago
I have always felt that hearing problems are difficult to truly "cure" and that most people can only use hearing aids. But this study is really different. If the key protein can be repaired, those conditions that seemed unchangeable in the past may now have a chance of turning around. Both children and adults can benefit from it, and this medical progress is really impressive.
tomfucksdan 1y ago
[flagged]

355 points by justacrow 1y ago

This is great news. Hopefully this will be expanded to other forms deafness like those caused by ototoxic medication, ear infections, and general sensorineural hearing loss.

im3w1l 1y ago
This particular study looks like it's dealing with a pretty narrow condition and solution (protein missing - add gene for protein). I don't think this particular research can be extended the way you hope.
- jonathanlb 1y ago
  I agree. The causes of deafness I mentioned are diverse and fall outside the specific focus of this current study. Ideally, future research will address deafness caused by these and other other factors.
- janeerie 1y ago
  Yes, I would really like to see research targeting Connexin 26, since it's the most common cause of hearing loss, but it seems it's much more difficult to "cure."

vonnik 1y ago

adjacent, same trend: https://news.ufl.edu/2024/09/blindness-gene-therapy/ https://news.ufl.edu/2024/09/blindness-gene-therapy/

agumonkey 1y ago
somehow it seems the singularity is here .. i hope it's well distributed
- fao_ 1y ago
  I don't see how this represents the singularity. This is all humans doing work and building knowledge, much like it has always been. Can you point to a specific section of the article that elicits that assumption? Thanks
  - agumonkey 1y ago
    The general acceleration in research and outcomes, we're now fixing deep issues more and more often. And hardware / software improvements keep coming too, even outside of computing. Sorry if that sounded pompous but there's a slight feeling that we're not plateauing, quite the contrary.
  - perlgeek 1y ago
    I'm not super familiar with this, but I think many of the gene therapy advances did involve some form of machine learning to find relevant genes. Some might also use protein folding simulations, and the best ways to do that involve narrow AI. I'd love to hear from somebody in the medical research field how much AI/ML actually speeds up their progress.
perlgeek 1y ago
... and then there was this Phase-1 trial in Japan about re-growing missing teeth. We're really making astonishing progress in many different areas in medicine these days. They are mostly pretty narrow, but also so awesome that ~20 years ago, many doctors would have called it science fiction.

wetpaws 1y ago

[dead]

d0100 1y ago

Anyone from the deaf community that can tell us how these kind of news are received? I wonder how this compares to animosity towards coclear implants by a subsection of the community

guerrilla 1y ago
That was my first thought too. I wonder how many people would choose to stay deaf just to stay in the community.
retrac 1y ago
Controversial in the same way cochlear implants are. Many deaf/Deaf parents want children who hear. And I think absent the cultural consideration, almost all would want children who hear. But you can't ignore the cultural consideration. If you are deaf, and have a deaf child, curing that child's deafness means they will move away from you later in life. It's a kind of alienation even when the child remains bicultural, they usually end up almost entirely in the hearing world. That said, most deaf people who have children have hearing children anyway. Hereditary deafness like that is relatively rare like that. But for people from such families, and who live in a culturally deaf world -- they are not disabled. The cultural environment they live in is ... one in which deafness is not disabling. And it's going to be a very high hill to climb to convince them that they are missing something. They certainly don't feel it. This is particularly true in the United States which has such a proud tradition of deaf culture and education -- you can go all the way to doctorate level studies in ASL, work in ASL, the hearing world being a strange foreign culture you only rarely wade into -- only rarely need to. I'd cure it for myself, and my child if I had one. No question. But I'm not culturally deaf. I feel isolated by it in the same way most hearing people anticipate deafness to be as an experience. But again -- people who live in the deaf cultural world -- they do not feel that, and they don't feel disabled because, in their context, they aren't. It's hard to communicate this to most hearing people. The usual response is dismissive, and unfortunately I think a lot of that ultimately goes back to very old metaphysical attitudes towards language and intelligence. A lot of hearing people still don't believe, deep down, that sign languages are equivalent to spoken languages, in particular. It's just gesture. You're lacking something essential to the human condition without spoken language. Etc. But for the culturally deaf, nothing is missing from their lives, except the perception of sound.

artursapek 1y ago

[flagged]

deleted 1y ago
[deleted]
deleted 1y ago
[deleted]
pugworthy 1y ago
If this is a joke it's in extremely bad taste

smath 1y ago

Regeneron had announced positive results in its gene therapy drug for deafness in Feb 2025: https://investor.regeneron.com/news-releases/news-release-details/latest-db-oto-results-demonstrate-clinically-meaningful-hearing https://investor.regeneron.com/news-releases/news-release-de...

thomassmith65 1y ago

It's good that the article title doesn't use "congenital deafness", but it would be even better if it were simply "Deafness Cured!" /s

thomassmith65 1y ago
Without sarcasm: Please rename this to "Gene therapy restored hearing in patients with autosomal recessive deafness" so as not to raise and then dash hopes of deaf HN readers

max_ 1y ago

I see alot of advances powered by genetics now days. Is there a specific field in genetics pushing this? I used to hear buzz about CRISPER/CAS9 is it what is underlying most of these advancements? How come alot of gene editing stocks have taken a serious beating if the tech is so good. Many, many gene editing stocks have lost more than 90% of thier value since IPO.

searine 1y ago
The tech behind this is not new or difficult. The issues are related to safety and regulation. Early efforts in gene therapy had disastrous results and current treatments are not trying to repeat past mistakes. There is tremendous potential for gene therapy to cure disease, however it needs (and so far has had) strict regulation, particularly if the changes can be inherited.
- beambot 1y ago
  > Early efforts in gene therapy had disastrous results Can you share examples...? Just curious as an outsider looking in.
  - ortusdux 1y ago
    https://pharmaceutical-journal.com/article/feature/gene-therapy-from-catastrophe-to-cure-in-20-years https://pharmaceutical-journal.com/article/feature/gene-ther... "The most notable obstacle faced in the gene therapy field was that of Gelsinger in 1999, who is understood to have died after his body overreacted to the adenovirus vector. Gelsinger had a rare disorder in which the liver lacks a functional copy of the ornithine transcarbamylase (OTC) gene and, consequently, the body is unable to eliminate ammonia, a toxic breakdown product of protein metabolism." "A gene therapy for children with severe combined immunodeficiency (SCID) was delivered [to two independent groups in London and Paris] and was incredibly successful,” explains Griesenbach. “But, [in 2008; between three and six years later], a small proportion of children [in Paris] developed leukaemia induced by the vector, which had inserted itself into a gene that controls cell division [4] .”

S4H 1y ago

How challenging is it for a person who has been deaf for let's say 20 years to suddenly regain hearing?

jallmann 1y ago
I can't say anything about the specifics of this treatment, but in terms of their ability to fully benefit from hearing, it would depend on when they became deaf, and the severity of their deafness. If they were born deaf, or lost hearing as a young child during the language development stage, then it would probably be a long adjustment. Things would just be noise and it would take a lot of training to distinguish sounds, speech, etc. And unlike a cochlear implant, you couldn't just take it off to give your brain a rest. If they had hearing loss later in life, or some residual hearing, then they probably have a better chance of re-adjusting to hearing.
ordu 1y ago
Really challenging. In some aspects it can be worse than to regain vision. If you are not accustomed to sounds, they can be annoying, and may make you feel tired. The same can happen with vision, it is just too much, but you can close your eyes, and shut out vision stimuli. You can't do that with hearing. At least if you regain hearing with normal sensitivity, you can be overwhelmed by sounds of your body. It is easier with implants, which can be shut off.
dd82 1y ago
If they've been deaf from infancy, basically the entire hearing center of the brain is non-existent. So they'd be hearing sound, but processing it into meaningful content would not happen, if at all. So basically, its like having a cacophany of sound that you can't filter and process... As for others, one thing hearing people, particularly monolingual hearing people, don't understand very well is that hearing != understanding. Just because you hear a sound doesn't automatically equate to it having meaning. The default for many people is to just SPEAK LOUDER and slower, which does not help in the vast majority of encounters

squigz 1y ago

You know, whenever treatment for autism comes up, we get a lot of comments heavily suggesting curing autism is basically eugenics. Why is it that some things are seen as a disability we should try to fix in our children, and others - which are in many ways just as debilitating - seen as some kind of beautiful part of humanity?

joefourier 1y ago
Autism is a spectrum disorder and I don’t think it should be controversial to cure low functioning autism. However, high functioning autists can be argued to be more of a personality variant than a disability, with different strengths and weaknesses compared to neurotypical people. Society benefits greatly from supporting high functioning autistic people in say, technical fields where hyperfocus, narrow obsessions and systemising thinking are an advantage. Meanwhile, having a genetic condition like haemophilia doesn’t give you any conceivable advantage.
- squigz 1y ago
  > Society benefits greatly from supporting high functioning autistic people in say, technical fields where hyperfocus, narrow obsessions and systemising thinking are an advantage. At the expense of those people having to live with all the unmentioned negative aspects of autism. (To say nothing of whether those are actually positives or not. Personally, I don't see how hyperfixating on something for a few weeks at a time at the expense of all my other responsibilities is a superpower, but hey)
- iteria 1y ago
  I don't even think there should be a conversation around high functioning autistics. My kid suffers from autistic catatonia. She's also extremely high functioning. I'm sorry, there is no world where I'm going to say no thanks to a cure for my daughter's body suddenly locking into place for an unknown period of time or losing the ability to speak or function randomly or hell just understand human expression without intense intervention. We can argue about their special brain powers or whatever, but all I'm seeing is that high functioning autistic have a much higher rate of self-harm and suicide. It can't be that great.

jhaddow 1y ago

How would one find out if they have this type of hearing loss? I have moderate to severe hearing loss in both ears since birth and there’s never been an attempt that I’m aware of to diagnose the cause beyond a standard inner ear examination.

apt-apt-apt-apt 1y ago
Asking your ear doctor seems like a good idea for this, rather than random people on HN..
- jhaddow 1y ago
  Do you want to be my ENT?
codytruscott 1y ago
Whole Genome Sequencing is affordable now. I’d suggest a 20x hifi long read from broad clinical labs for $1200 or so. Use opencravet to dig into the results. They just posted a webinar for personal analysis https://wse.zoom.us/webinar/register/WN_-VvYJ8FKRcGaKCQtLFrUpQ?_x_zm_rtaid=jJ-eSZOuR7C63JL6AWUgDw.1749849096416.125c494ceb92f310ba2b3c244ccaadf5&_x_zm_rhtaid=831#/registration https://wse.zoom.us/webinar/register/WN_-VvYJ8FKRcGaKCQtLFrU... Franklin by genoox is a slicker and possibly more approachable product depending on your interface preferences. Genetic research — due to the number and subtly of variants — is ripe for citizen science in my opinion.
- gavinray 1y ago
  As a follow-on to this: If you have partial genome data from 23andMe, Ancestry, etc, you can use what's called "genomic imputation" to do a sort of probabilistic gap-filling in your genome. It's a bit tricky to do yourself, but there are paid services that will run the imputation for you and share the results. I paid $15 for mine at https://dnagenics.com https://dnagenics.com --- @codytruscott I signed up for that webinar, I hadn't heard of this tool before, thanks! Got any other useful links/tools to share by chance?

coolKid721 1y ago

okay can we cure tinnitus please

froggertoaster 1y ago

What immediately sprung to mind is how the deaf community has seen things like this as a personal and existential threat. To me it's an obvious disability, and deaf people SHOULD want to be cured, but tribalism wins that argument all too often.

UltraSane 1y ago

This is interesting because the quack who created Chiropractic wrongly thought he cured deafness with spinal manipulation. Just shows how powerful the real scientific method is.

deleted 1y ago

[deleted]

FranckDernoncou 1y ago

The research study is behind a paywall: https://doi.org/10.1038/s41591-025-03773-w https://doi.org/10.1038/s41591-025-03773-w I'm always amazed that some people still choose to publish in paywalled journals.

Saul1998zx 1y ago

[dead]

Elaris 1y ago

I have always felt that hearing problems are difficult to truly "cure" and that most people can only use hearing aids. But this study is really different. If the key protein can be repaired, those conditions that seemed unchangeable in the past may now have a chance of turning around. Both children and adults can benefit from it, and this medical progress is really impressive.

tomfucksdan 1y ago

[flagged]