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Posted by justacrow 3 days ago

Gene therapy restored hearing in deaf patients(news.ki.se)
352 points | 89 comments
jonathanlb 3 days 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 3 days 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.
janeerie 3 days 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."
jonathanlb 3 days 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.
vonnik 3 days ago||
adjacent, same trend: https://news.ufl.edu/2024/09/blindness-gene-therapy/
perlgeek 3 days 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.

agumonkey 3 days ago||
somehow it seems the singularity is here .. i hope it's well distributed
fao_ 3 days 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
perlgeek 2 days 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.

agumonkey 3 days 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.
jhaddow 3 days 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.
codytruscott 3 days 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_-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 3 days 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

---

@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?

codytruscott 3 days ago||
Rather than 23andMe, Ancestry ($50-$100) etc => imputation, broad clinical labs offers exome + genome blended for $120.

https://usegalaxy.org is pretty remarkable and provides access to a ton of open source bioinformatics tools + compute to process the files.

I really think the $1200 20x pacbio from broad is worth it if you are going to make it serious hobby.

beaugunderson 3 days ago||||
the site i maintain is a bit out of date, but i accept PRs if you have time to add the broad clinical labs data!

https://whichgenome.com/

edwardog 2 days ago|||
Thank you for this! My wife recently got a pathology report back reporting a rare variant of a rare cancer and I’m trying to get back into genomics (an almost masters degree) now to see if there’s anything my computering can do to aid. I’ve contacted Broad Clinical Labs.
2dvisio 2 days ago|||
In the UK, Newborn hearing screening (https://www.nhs.uk/baby/newborn-screening/hearing-test/) is a mandatory test that happens in the first weeks of life.

Genomics-driven diagnosis of several (treatable) conditions is not science fiction anymore, but requires support from governments and national health systems. The technology is there, and can be scaled up.

With studies like this: https://www.genomicsengland.co.uk/initiatives/newborns

and initiatives like this: https://www.bbc.co.uk/news/articles/c1ljg7v0vmpo#:~:text=Eve...

apt-apt-apt-apt 3 days ago|||
Asking your ear doctor seems like a good idea for this, rather than random people on HN..
jhaddow 3 days ago||
Do you want to be my ENT?
invalidOrTaken 3 days ago||
[flagged]
squigz 3 days 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 3 days 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.

iteria 3 days 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.
squigz 3 days 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)

guerrilla 3 days ago|||
> Meanwhile, having a genetic condition like haemophilia doesn’t give you any conceivable advantage.

Sickle-cell anemia does though. I wonder if some day there could be a survival advantage for haemophilia. What if we erase the genetic code that ends up saving us from some alien virus, you know?

I'm not saying this is a good argument, just something interesting to think about.

pabs3 18 hours ago|||
Some part of the deaf community would see the treatment in this article similarly to eugenics; erasing deaf culture.
jeroenhd 2 days ago||
While I'm probably considered high-functioning autistic, I have seen the devastating effects autism can have on people less lucky than me. I don't know if I'd go for an autism cure myself, but if autism can be corrected for in the womb or right after birth, I would definitely be in favour. How necessary such a cure would be, depends on how extreme someone's autism is, and if the cure can be administered before their disability helps form and solidify their personality.

However, I think mental disorders like autism and physical ailments like deafness don't have the same ethical impact. One changes who a person is, the other changes what a person is capable of. It also depends on how bad the disability is; in this case, the kids showing most promise could already hear, though badly, and the treatment let them hear much better. I'm not even deaf but I'd happily take a treatment to fix whatever hearing damage I've collected over the years.

And for what it's worth, eugenics is already with us and that's actually not so evil. People carrying certain genetic diseases choose not to have (biological) kids all the time. Others still choose to risk it. As long as there isn't some large conspiracy about perfecting the human race behind it, eugenics can be helpful.

max_ 3 days 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 3 days 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 3 days ago||
> Early efforts in gene therapy had disastrous results

Can you share examples...? Just curious as an outsider looking in.

ortusdux 3 days ago||
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 3 days ago||
How challenging is it for a person who has been deaf for let's say 20 years to suddenly regain hearing?
jallmann 3 days 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.

dd82 2 days 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

ordu 3 days 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.

Elaris 3 days 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.
smath 3 days ago||
Regeneron had announced positive results in its gene therapy drug for deafness in Feb 2025: https://investor.regeneron.com/news-releases/news-release-de...
froggertoaster 3 days 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 3 days 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.
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