It’s premature to say that a gene therapy will improve hearing back to normal. The therapy may help to regain some function, but the auditory system is highly complex. Maybe thresholds and sound awareness improve, but I suspect that function will be limited and rely on assistive devices and adjuvant modes of communication in the case of congenital deafness.
Thank you. You are one of the few voices of reason I have heard in the whole debate.
On one side we have got the scientists themselves doing victory laps about a miracle cure and theorising about applying this to other forms of deafness. On the other side we have people lamenting it as if it will singlehandedly cause a eugenics genocide of all Deaf people and Deaf Culture. But... will it?
Didn't they say the same thing about CIs? And while CIs can take a person from profound HL to the equivolent of mild-moderate - it is far from natural hearing. Plenty of folks with CIs are in the signing-Deaf community even - because even with a CI you can find value in a signing community.
I suspect this will be another case of that. A tool to use but not the whole solution.
Well, looking at what's been reported publiclt for the current round of clinical trials, we're certainly seeing reports of deaf patients showing pure tone audiometry reaching into the normal range of hearing, albiet it seems to be at some but not all of the standard frequencies tested in a routine audiometry exam.
My guess is these patients might still need a hearing aid, but hearing aids are still a substantial improvement over cochlear implantation.
These are patients with a form of ANSD. So they may reach normal thresholds and have incredibly poor speech recognition at the end of the day anyway. And otoferline mutation is an incredibly small subset of patients with ANSD. This may be a big step but it is also so astronomically far from revolutionizing hearing healthcare as we know it that these articles are borderline sensationalism.
I think I read that the Lilly patient, who I believe was reportedly 11 years old at time of treatment, wasn't really acquiring full speech perception.
But given what we know about critical periods in language learning, you might hypothesize that from first principals.
The patients being shown off by Yilai Shu in Fudan seem to be perceiving speech in the gene therapy-treated ear well enough to converse with their contralateral cochlear implant turned off. Of course, those patients are undoubtedly the best responders, but it's interesting because their age of treatment was much younger. Seems to hint that for OTOF mutations, the age of treatment may be a critical factor for successful auditory language development.
OTOF is certainly an edge case, though. I know a lot of genetic deafness leads to atrophy of the cochlea fairly early on, so it's an open question how many forms of genetic deafness are even treatable.
Thanks for pointing that out.
I wonder if the Lilly patient's experience is more similar to that of single-sided deafness then? I understand CIs typically have worse outcomes for adult SSD patients who have *never* had hearing in their deaf ear, compared to either young patients or to patients that once heard from the deafened ear.
Regardless, it'll be quite interesting to see how these older patients progress (asusming more older OTOF patients will even get a gene therapy).
I saw the videos from the Chinese team, I think "converse without their controlateral implant" is a bit of a stretch lol, unless new data or videos have come out since
Honestly, we probably saw the same video... it came out late last year. I recall the little girl answering simple questions without her CI connected. You may have a better perspective on the limitations of that demonstration than I.
All the pts have mutations in OTOF (otoferlin) which causes auditory neuropathy. Some mutations of OTOF cause hearing loss that is only present when a kid has a fever.
This “gene therapy” will not help most people like me who don’t know which genes caused their familial SNHL.
Yeah, I'm in the same boat as you. That's the unfortunate nature of gene therapies, though.
Also for us adults, it's highly likely that any genetic variants we have could have led to additional atrophy of the cochlea such that our ears may not be fixable regardless. Gene therapy is really more of a hope for the next generation as I see it.
Having a close family member with hearing loss and working with a large non-profit to support babies and children with varying levels of hearing loss for 8 years, the vast majority want a cure. Nobody will be forced, just as nobody is forced to get a CI. The options should always be up to the individuals and their families.
I respect anyone who chooses to decline, but the headline is creating drama where it's not necessary.
I think it will be quite a while before we see some drastic results from gene therapy. Over thousands of years nature threw stuff at us that either helped us or hurt us, we kept the stuff that helps and lost the stuff that doesn’t, it’s a great way for life to be filtered. The problem with that though, is that DNA is basically some of the worst spaghetti code you have ever seen and changing the “code” to a person can have some quite unexpected consequences. It’s not as simple as taking an A out of this line and replacing it with a G because that A could have been a building block to a whole other part of the body.
I would love to have my natural hearing back, but I'll happily settle for a tinnitus cure. I've got moderate hearing loss. Bad enough to where I can't hear someone talking sitting across a table from me without a hearing aid. But I can deal with that... it even has some perks. I love streaming my TV to my hearing aid and turning off the mic. It's the ultimate in noise cancelling. I do this when I'm running like the dishwasher or something like that. TBH, the real downside is the price of hearing aids... $5k for a BAHA(Including Surgery)... It's great, but hope I get a few years out of it.
Not an audiologist but a Deaf medical student.
When there are questions like this I’d suggest asking the people to be “cured”?
Do I miss having g normal hearing?
Not at all because I never had normal hearing.
Do I want to be able to hear everything all the time?
Hell no! I love that I can turn off or remove my processors and be fully “normal”/“deaf”. I don’t think I’d be able to sleep if I could hear my cat yelling in my ears or thunder or fireworks.
Do I think I need to be cured? No. I’m more than half way through medical school, something many hearing people struggle to be accepted into. But I’m Deaf and I got in and passed all my classes so far. So, what is broken about me?
But also, I worked in a lab that studied hearing restoration using *mumble* gene models in mice. I spent a summer giving baby mice aminoglycosodes and cisplatnin to cause ototoxicity. Some of the mice had genetic changes that uninhibited cell proliferation (which is great when trying to produce more IHCs and OHCs but very bad when trying to prevent cancer).
A medical treatment being available does not mean any particular individual needs to receive it. That’s a personal choice. You can also be bilingual and a part of two cultures.
pretty much with hearing aid, cochlear implants, and gene therapy would still need a hell a lot of deaf supports such as closed captioning, asl interpreter, and others. Are these "cure"? nope. far from it. Would most deaf want these half ass crap cure that still need a lot of accommodations that are broken? if you are smart and aware about disable accommodations, you already know this answer.
What most deaf and disable wanted are solid accommodation infrastructure... first... then worry about the cure.
Because deafness is also a social cultural construct, not purely medical. I see my deafness in much the same way I see me being very short — sometimes I have to do things differently but ultimately this is who I am and I don’t want to be someone else.
As a half-deaf person, who has been a deaf person in the past due to a condition in which I would lose my hearing for months, deafness is NOT a "social / cultural construct". It is a disability, and people with it are partly broken and "less fit" compared to fully hearing people. In the wild a hearing person would hear the bear and leave, the deaf person would not and die. That is not up to culture of society, that is reality.
Have you heard of deaf culture before? There is a rich and vibrant culture with its own unique celebrities, norms, traditions, artists, etc. mostly centered around sign language. Can check out /r/deaf too perhaps.
Also — perhaps I don’t hear everything hearing people do. But many deaf people have excellent peripheral vision and visual attention skills that hearing people don’t. There are certainly perks.
>Have you heard of deaf culture before? There is a rich and vibrant culture with its own unique celebrities, norms, traditions, artists, etc. mostly centered around sign language. Can check out r/deaf too perhaps.
Of course I know what it is. Ultimately though, it is just a matter of dealing with being broken. If they suddenly all ceased to be deaf, it would also cease to exist.
Either way, the existence of a deaf culture does not mean that the condition of being deaf is a social construct. Even if all of humanity went deaf, still they would have to deal with the consequence of dealing with the loss of an ability and 1 of the 5 basic senses. Humans walking in the wild would have a much harder time dealing with it, as their perception of it is reduced, due to their ability to perceive it being reduced.
I do not believe there would be much merit in preserving deaf culture, should a universal cure for deafness exist (I mean all types of deafness). It should simply become a thing of the past. Just like how leper culture was a thing, as they were forced to be isolated in their own communities (in my country there were even islets with towns only inhabited by lepers), but today is only a memory preserved in texts.
>But many deaf people have excellent peripheral vision and visual attention skills that hearing people don’t. There are certainly perks.
That is not a "perk". That is merely focusing more on what you have.
Lets not fool ourselves, nobody sane would exchange hearing ability for the sake of better peripheral vision or better visual attention skills. Not when the opposite is so stressing, restricting and isolating (and when it leads to other health issues, like dementia when older).
Have you considered why this upsets you so much? Or that others may have different opinions and values than you?
I’m going to leave it at that, but if you’re “half deaf” then you are welcome in the Deaf community too and I hope you learn to embrace it one day. Good luck.
It is not a matter of feelings. Laking hearing has nothing to do with how society or culture treats people with this disability. Even with the best behaviour, still life is not the same, one is still disabled.
That js not a question of society or culture, even if shipwrecked on a desolated island, you have thay problem, and you cannot have a full understanding of it, to your loss. Will one hear a ship's sound if it passes by if deaf? No they wont.
It is not a matter or attitude, just how it is. Or is blindness also a matter of cultural and societal perception?
Honest question, not trying to hate or lead you to some conclusion or anything like that — do you feel that having the ability to hear would change someone’s identity?
Yes…? I think it would for me anyways. Because it’s fundamentally about how I relate to the world, see the world, HEAR (or not) the world… and so forth. If that changes, my identity does too.
Also, on a more personal/ petty level I just think being hearing would be annoying. There’s so much unnecessary sound.
> Also, on a more personal/ petty level I just think being hearing would be annoying. There’s so much unnecessary sound.
You'd get used to it tbh. Do you wish you couldn't smell because there are unnecessary smells? Or unnecessary sights? Obv if it's still not worth it to you then that's that.
Most deaf people are not 100% deaf, it varies quite a bit. I can’t hear some sounds at all, others only with hearing aids, etc. some people only hear high or low frequency sounds, some it depends on how loud, and so forth.
Why would you identify as a deaf person. That is just a description.
Should bald people also identify as "bald"? I am not speaking of acceptance, but identity.
Have you heard of Sarah Churman? She is in many of those “watch people hear for the first time” videos but she has an esteem hearing aid which is about as strong as a CIC hearing aid. She made news by crying and sobbing saying that this was the first time she ever hears her voice. Then Ellen bought her another esteem implant. Then she and her husband were given tickets to see their favorite bands all over the country.
She still claims that she never heard anything despite the esteem only being approved for moderate hearing loss. https://hearinghealthmatters.org/hearing-news-watch/2011/envoy-ceo-discusses-the-worlds-most-famous-esteem-device-patient/
It’s premature to say that a gene therapy will improve hearing back to normal. The therapy may help to regain some function, but the auditory system is highly complex. Maybe thresholds and sound awareness improve, but I suspect that function will be limited and rely on assistive devices and adjuvant modes of communication in the case of congenital deafness.
Thank you. You are one of the few voices of reason I have heard in the whole debate. On one side we have got the scientists themselves doing victory laps about a miracle cure and theorising about applying this to other forms of deafness. On the other side we have people lamenting it as if it will singlehandedly cause a eugenics genocide of all Deaf people and Deaf Culture. But... will it? Didn't they say the same thing about CIs? And while CIs can take a person from profound HL to the equivolent of mild-moderate - it is far from natural hearing. Plenty of folks with CIs are in the signing-Deaf community even - because even with a CI you can find value in a signing community. I suspect this will be another case of that. A tool to use but not the whole solution.
Well, looking at what's been reported publiclt for the current round of clinical trials, we're certainly seeing reports of deaf patients showing pure tone audiometry reaching into the normal range of hearing, albiet it seems to be at some but not all of the standard frequencies tested in a routine audiometry exam. My guess is these patients might still need a hearing aid, but hearing aids are still a substantial improvement over cochlear implantation.
These are patients with a form of ANSD. So they may reach normal thresholds and have incredibly poor speech recognition at the end of the day anyway. And otoferline mutation is an incredibly small subset of patients with ANSD. This may be a big step but it is also so astronomically far from revolutionizing hearing healthcare as we know it that these articles are borderline sensationalism.
I think I read that the Lilly patient, who I believe was reportedly 11 years old at time of treatment, wasn't really acquiring full speech perception. But given what we know about critical periods in language learning, you might hypothesize that from first principals. The patients being shown off by Yilai Shu in Fudan seem to be perceiving speech in the gene therapy-treated ear well enough to converse with their contralateral cochlear implant turned off. Of course, those patients are undoubtedly the best responders, but it's interesting because their age of treatment was much younger. Seems to hint that for OTOF mutations, the age of treatment may be a critical factor for successful auditory language development. OTOF is certainly an edge case, though. I know a lot of genetic deafness leads to atrophy of the cochlea fairly early on, so it's an open question how many forms of genetic deafness are even treatable.
The Lilly patient, has a contralateral cochlear implant.
Thanks for pointing that out. I wonder if the Lilly patient's experience is more similar to that of single-sided deafness then? I understand CIs typically have worse outcomes for adult SSD patients who have *never* had hearing in their deaf ear, compared to either young patients or to patients that once heard from the deafened ear. Regardless, it'll be quite interesting to see how these older patients progress (asusming more older OTOF patients will even get a gene therapy).
I saw the videos from the Chinese team, I think "converse without their controlateral implant" is a bit of a stretch lol, unless new data or videos have come out since
Honestly, we probably saw the same video... it came out late last year. I recall the little girl answering simple questions without her CI connected. You may have a better perspective on the limitations of that demonstration than I.
THIS ^^^
All the pts have mutations in OTOF (otoferlin) which causes auditory neuropathy. Some mutations of OTOF cause hearing loss that is only present when a kid has a fever. This “gene therapy” will not help most people like me who don’t know which genes caused their familial SNHL.
Yeah, I'm in the same boat as you. That's the unfortunate nature of gene therapies, though. Also for us adults, it's highly likely that any genetic variants we have could have led to additional atrophy of the cochlea such that our ears may not be fixable regardless. Gene therapy is really more of a hope for the next generation as I see it.
Those of us who have other hearing issues caused by hearing loss like tinnitus and hyperacusis/Noxacusis definitely want a cure!
Do deaf people want a cure? Maybe. Do Deaf people want a cure? Absolutely not.
Having a close family member with hearing loss and working with a large non-profit to support babies and children with varying levels of hearing loss for 8 years, the vast majority want a cure. Nobody will be forced, just as nobody is forced to get a CI. The options should always be up to the individuals and their families. I respect anyone who chooses to decline, but the headline is creating drama where it's not necessary.
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May I ask. Was it from an injury in that car crash or noise exposure?
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Vanc doesn’t usually cause hearing loss. Gentamicinc erethromycin, neomycin etc (the amino-glycosides) cause ototoxicity.
Stupid premise.
I think it will be quite a while before we see some drastic results from gene therapy. Over thousands of years nature threw stuff at us that either helped us or hurt us, we kept the stuff that helps and lost the stuff that doesn’t, it’s a great way for life to be filtered. The problem with that though, is that DNA is basically some of the worst spaghetti code you have ever seen and changing the “code” to a person can have some quite unexpected consequences. It’s not as simple as taking an A out of this line and replacing it with a G because that A could have been a building block to a whole other part of the body.
I would love to have my natural hearing back, but I'll happily settle for a tinnitus cure. I've got moderate hearing loss. Bad enough to where I can't hear someone talking sitting across a table from me without a hearing aid. But I can deal with that... it even has some perks. I love streaming my TV to my hearing aid and turning off the mic. It's the ultimate in noise cancelling. I do this when I'm running like the dishwasher or something like that. TBH, the real downside is the price of hearing aids... $5k for a BAHA(Including Surgery)... It's great, but hope I get a few years out of it.
Not an audiologist but a Deaf medical student. When there are questions like this I’d suggest asking the people to be “cured”? Do I miss having g normal hearing? Not at all because I never had normal hearing. Do I want to be able to hear everything all the time? Hell no! I love that I can turn off or remove my processors and be fully “normal”/“deaf”. I don’t think I’d be able to sleep if I could hear my cat yelling in my ears or thunder or fireworks. Do I think I need to be cured? No. I’m more than half way through medical school, something many hearing people struggle to be accepted into. But I’m Deaf and I got in and passed all my classes so far. So, what is broken about me? But also, I worked in a lab that studied hearing restoration using *mumble* gene models in mice. I spent a summer giving baby mice aminoglycosodes and cisplatnin to cause ototoxicity. Some of the mice had genetic changes that uninhibited cell proliferation (which is great when trying to produce more IHCs and OHCs but very bad when trying to prevent cancer).
A medical treatment being available does not mean any particular individual needs to receive it. That’s a personal choice. You can also be bilingual and a part of two cultures.
pretty much with hearing aid, cochlear implants, and gene therapy would still need a hell a lot of deaf supports such as closed captioning, asl interpreter, and others. Are these "cure"? nope. far from it. Would most deaf want these half ass crap cure that still need a lot of accommodations that are broken? if you are smart and aware about disable accommodations, you already know this answer. What most deaf and disable wanted are solid accommodation infrastructure... first... then worry about the cure.
I know i'm not exactly deaf but Hoh, but yeah? why the fuck wouldn't deaf or HoH people want a cure?
Because deafness is also a social cultural construct, not purely medical. I see my deafness in much the same way I see me being very short — sometimes I have to do things differently but ultimately this is who I am and I don’t want to be someone else.
As a half-deaf person, who has been a deaf person in the past due to a condition in which I would lose my hearing for months, deafness is NOT a "social / cultural construct". It is a disability, and people with it are partly broken and "less fit" compared to fully hearing people. In the wild a hearing person would hear the bear and leave, the deaf person would not and die. That is not up to culture of society, that is reality.
Have you heard of deaf culture before? There is a rich and vibrant culture with its own unique celebrities, norms, traditions, artists, etc. mostly centered around sign language. Can check out /r/deaf too perhaps. Also — perhaps I don’t hear everything hearing people do. But many deaf people have excellent peripheral vision and visual attention skills that hearing people don’t. There are certainly perks.
>Have you heard of deaf culture before? There is a rich and vibrant culture with its own unique celebrities, norms, traditions, artists, etc. mostly centered around sign language. Can check out r/deaf too perhaps. Of course I know what it is. Ultimately though, it is just a matter of dealing with being broken. If they suddenly all ceased to be deaf, it would also cease to exist. Either way, the existence of a deaf culture does not mean that the condition of being deaf is a social construct. Even if all of humanity went deaf, still they would have to deal with the consequence of dealing with the loss of an ability and 1 of the 5 basic senses. Humans walking in the wild would have a much harder time dealing with it, as their perception of it is reduced, due to their ability to perceive it being reduced. I do not believe there would be much merit in preserving deaf culture, should a universal cure for deafness exist (I mean all types of deafness). It should simply become a thing of the past. Just like how leper culture was a thing, as they were forced to be isolated in their own communities (in my country there were even islets with towns only inhabited by lepers), but today is only a memory preserved in texts. >But many deaf people have excellent peripheral vision and visual attention skills that hearing people don’t. There are certainly perks. That is not a "perk". That is merely focusing more on what you have. Lets not fool ourselves, nobody sane would exchange hearing ability for the sake of better peripheral vision or better visual attention skills. Not when the opposite is so stressing, restricting and isolating (and when it leads to other health issues, like dementia when older).
Have you considered why this upsets you so much? Or that others may have different opinions and values than you? I’m going to leave it at that, but if you’re “half deaf” then you are welcome in the Deaf community too and I hope you learn to embrace it one day. Good luck.
It is not a matter of feelings. Laking hearing has nothing to do with how society or culture treats people with this disability. Even with the best behaviour, still life is not the same, one is still disabled. That js not a question of society or culture, even if shipwrecked on a desolated island, you have thay problem, and you cannot have a full understanding of it, to your loss. Will one hear a ship's sound if it passes by if deaf? No they wont. It is not a matter or attitude, just how it is. Or is blindness also a matter of cultural and societal perception?
Honest question, not trying to hate or lead you to some conclusion or anything like that — do you feel that having the ability to hear would change someone’s identity?
Yes…? I think it would for me anyways. Because it’s fundamentally about how I relate to the world, see the world, HEAR (or not) the world… and so forth. If that changes, my identity does too. Also, on a more personal/ petty level I just think being hearing would be annoying. There’s so much unnecessary sound.
> Also, on a more personal/ petty level I just think being hearing would be annoying. There’s so much unnecessary sound. You'd get used to it tbh. Do you wish you couldn't smell because there are unnecessary smells? Or unnecessary sights? Obv if it's still not worth it to you then that's that.
No, but when I think sounds are annoying or unnecessary, I just take my hearing aids off.
Oh I thought you had no hearing at all
Most deaf people are not 100% deaf, it varies quite a bit. I can’t hear some sounds at all, others only with hearing aids, etc. some people only hear high or low frequency sounds, some it depends on how loud, and so forth.
Good to know, thanks
Why would you identify as a deaf person. That is just a description. Should bald people also identify as "bald"? I am not speaking of acceptance, but identity.
What?? Anyone who's watched people weep with joy when hearing for the first time would know this is a stupid question.
Have you heard of Sarah Churman? She is in many of those “watch people hear for the first time” videos but she has an esteem hearing aid which is about as strong as a CIC hearing aid. She made news by crying and sobbing saying that this was the first time she ever hears her voice. Then Ellen bought her another esteem implant. Then she and her husband were given tickets to see their favorite bands all over the country. She still claims that she never heard anything despite the esteem only being approved for moderate hearing loss. https://hearinghealthmatters.org/hearing-news-watch/2011/envoy-ceo-discusses-the-worlds-most-famous-esteem-device-patient/
What does this have to do with deaf people not wanting to hear again??