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Gene therapy restores hearing to children with inherited deafness

cosmosmagazine.com

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Re: Gene therapy restores hearing to children with inherited deafness

#41

Post by narrator »

dyauspitr wrote: post #12
gota wrote: post #2

This stuff seems miraculous. > "Through minimally invasive surgery, Shu injected adeno-associated virus (AAV) engineered to carry and deliver functioning copies of the human OTOF transgene, into the children’s inner ears. " Can't read that and not get curious, excited, for the future. Congratulations to all those involved in the research and thanks to all who contributed to it - including all taxpayers if public fund…

I can’t wait for a geneCAD where we for example, design wings for a human body and then it works out the rest and generates a CRISPR based AAV that you can inject yourself with. True race and sex changes too. Hopefully in the next 50 years but that’s wishful thinking.

No doubt you'll see an Android/Apple split and the Apple people won't be able to date the Android people because of incompatible augmented pheromones that smell funny if you're not on the same platform.

Re: Gene therapy restores hearing to children with inherited deafness

#42

Post by dyauspitr »

akoboldfrying wrote: post #39
dyauspitr wrote: post #12

Earlier quoted context omitted.

I can’t wait for a geneCAD where we for example, design wings for a human body and then it works out the rest and generates a CRISPR based AAV that you can inject yourself with. True race and sex changes too. Hopefully in the next 50 years but that’s wishful thinking.

Let's try to fix the world's vast number of actual, boring health problems before trying to add the equivalent of spoilers and chrome-plated hubcaps to perfectly healthy human bodies.

You can do both things at the same time.

Re: Gene therapy restores hearing to children with inherited deafness

#45

Post by zac256 »

speedylight wrote: post #19

How hard is it to introduce permanent mutations to DNA in adults, across every cell in the body? That seems like the holy grail of gene editing to me!

This is an area of active research, and there are many issues to solve. A technology that distributes a gene editor throughout the body is called a delivery vector, and it is typically a modified virus.

After the patient is two years old, many large molecules do not pass through the blood brain barrier. Typically most of the treatment ends up in the liver. Viruses and the gene editor can be toxic. Combining the two previous points the correct dose for most of the body can be highly toxic to the liver. The delivery vector may support genes up to a specific size (in kilobases) which may be smaller than the gene you are trying to fit. Sometimes you don't want to target organs that the delivery vector hits. Sometimes you want to target organs that the delivery vector misses. And more.

There is rapid progress being made here and all kinds of caveats and nuance to the above.

Re: Gene therapy restores hearing to children with inherited deafness

#46

Post by akoboldfrying »

sircastor wrote: post #21

I’m curious about the effect on the deaf community. I understand there’s resistance to “fixing” deafness as some consider it a component of their culture and community, not unlike queer people and the attempts to “fix” them. https://en.wikipedia.org/wiki/Cochlear_implant#Criticism_and...

>It is important to consider the sociocultural context, particularly in regards to the deaf community, which has its own unique language and culture

Frankly, I disagree. I think a deaf person should be entitled to decline a low-risk treatment that would cure or mitigate their own deafness -- but to make that decision for a child is altogether different. Deliberately restricting a human being's sense experience throughout their lives should be considered child abuse, in the same basket as female genital mutilation. It's also about the most selfish thing I can imagine -- especially given that there's no reason I can see why a hearing child of deaf parents can't grow up "bilingual", learning both spoken and sign languages.

Re: Gene therapy restores hearing to children with inherited deafness

#47

Post by akoboldfrying »

dyauspitr wrote: post #42
akoboldfrying wrote: post #39

Earlier quoted context omitted.

Let's try to fix the world's vast number of actual, boring health problems before trying to add the equivalent of spoilers and chrome-plated hubcaps to perfectly healthy human bodies.

You can do both things at the same time.

That's true if we assume unlimited resources and equal awareness/perceived social value of the two applications. But I think the former doesn't hold, and the latter is greatly influenced by sites like HN, where I'm disappointed to consistently (if not exclusively) see breathless proposals for ways to design shinier hubcaps whenever some promising new technology appears.

Yes, they can and likely will both happen to some extent, but I think they aren't independent, so I feel justified in trying to nudge the public conversation back towards the issues that I think matter more.

Re: Gene therapy restores hearing to children with inherited deafness

#48

Post by gugagore »

lazyasciiart wrote: post #34
devilbunny wrote: post #28

Earlier quoted context omitted.

There is a fascinating documentary about this, but I cannot recall its name. One segment talks about a form of sign that represents sound, so that the child learns their family’s language (rather than sign language, which is another language entirely). It is very unpopular in the deaf community despite its advantages for daily life.

Sounds like Signed Exact English, which is disliked both for the context around it and for being more awkward than a natural sign language (e.g I think it doesn’t take advantage of using positions in space for grammar, it only uses the hand shapes and movements?) I don’t know of any language agnostic phonetic sign used as a first language for children: that sounds about as hard as making them learn through writing in…

They are likely talking about "cued speech".

Re: Gene therapy restores hearing to children with inherited deafness

#50

Post by SJC_Hacker »

arjie wrote: post #33

Through a mutual acquaintance, I met someone who did a little work for on the OTOF gene therapy and it really is remarkable stuff. My wife and I had our embryos screened by Orchid Health since we have a related genetic condition: a pathogenic mutation in GJB-2. Amazingly, Decibel Therapeutics has has one of these in R&D and the OTOF success gives us hope. We have a few embryos that are affected and the more that can…

Screening can also "repair" inherited deafness through selection - you simply don't produce offspring to those that have the defect.
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