[HN Gopher] Gene therapy allows an 11-year-old boy to hear
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Gene therapy allows an 11-year-old boy to hear
Author : mikhael
Score : 60 points
Date : 2024-01-23 17:26 UTC (5 hours ago)
(HTM) web link (www.nytimes.com)
(TXT) w3m dump (www.nytimes.com)
| CommanderData wrote:
| If true this is ground breaking news especially in the Audiology
| community.
|
| There is zero treatment besides hearing aids / cochlear implants
| for sensory hearing loss in Human history until now.
| ijhuygft776 wrote:
| while this is great, couldn't something like Neuralink help
| too?
| ceejayoz wrote:
| Neuralink has a long way to go to demonstrate that
| capability.
| GauntletWizard wrote:
| [delayed]
| neonate wrote:
| https://archive.ph/3FmSZ
| twostorytower wrote:
| _But no matter how well the gene therapy works, the researchers
| recognize that Aissam may never be able to understand or speak a
| language, Dr. Germiller said. The brain has a narrow window for
| learning to speak beginning around ages 2 to 3, he explained.
| After age 5, the window for learning spoken language is
| permanently shut._
|
| Wow that's incredibly sad, but I am glad that this will
| eventually get into the ears of thousands of deaf newborns.
| Incredible medical advancement. Gives me hope that one day my
| tinnitus may have a cure.
| doublerabbit wrote:
| > After age 5, the window for learning spoken language is
| permanently shut.
|
| It's fascinating how our brains are wired in such a way to
| enable read-only mode at an certain age in development.
| s1artibartfast wrote:
| I think it is more like telling the marketing team that you
| can't add an HDMI port to the computuer because it has
| already finished the production run.
|
| That is to say, it is as much of a hardware issue as a
| software issue.
| shermantanktop wrote:
| Get ready to repeat yourself, because the marketing team
| really _wants_ that port. The CMO just said "We should
| really add an HDMI port in a code patch because it would
| help OEM sales a lot." A sales engineer has agreed and is
| scheduling a brainstorm session.
| ricardobeat wrote:
| > the window for learning spoken language is permanently shut
|
| People still learn languages with completely different sounds
| when they are much older? Japanese, the african click-sound
| languages... is it some lower-level abstraction that goes
| missing?
| teaearlgraycold wrote:
| I think it's the ability to understand any language through
| sound. Presumably other languages learned lean heavily on
| what you already know from other languages.
| GauntletWizard wrote:
| I think the true answer is not impossibility, but
| significant, near insurmountable difficulty. The sound
| processing is not hooked up to cognition in the way it would
| be in a brain that had always had sensory input from the
| ears. Aissam would first need to learn to differentiate
| tones, voices, mouth-sounds, consonants vs vowels, etc.
| That's a lot to ask of a brain that had no understanding of
| that form of input at all.
|
| But all of this may turn out to be untrue! Our understanding
| of language acquisition comes from Feral Children[1], who had
| no language understanding at all. Aissam has language skills,
| though developed late - The article mentions he started
| learning Spanish Sign Language at 8 years old. That's already
| a remarkable feat. This might overturn our views of language
| acquisition, which were mostly formed in the 1800's; Pedagogy
| has come a long way since then.
|
| [1] https://en.wikipedia.org/wiki/Language_acquisition#As_a_t
| ypi...
| lucubratory wrote:
| For reference, the language you're thinking of is Xhosa
| lawlessone wrote:
| Even still there's plenty of situations where this could save
| his life.
| spywaregorilla wrote:
| How are born deaf people able to learn to speak if this is the
| case?
| stank345 wrote:
| The sensory medium is separate from one's capacity to learn
| and use language. Sign languages have grammar, vocabulary,
| "accents" etc just like spoken languages.
| zamadatix wrote:
| I think they mean how can a person born deaf learn to make
| speech if the above quote says this individual will not be
| able to speak a language. I think the answer to that is
| it's more "they won't be able to make speech like a person
| born with hearing would do by listening and naturally
| learning" rather than "they won't be able to try to make
| sounds with their voice they are not able to process
| auditorily".
| janeerie wrote:
| Deaf children can be taught to speak by very explicitly
| demonstrating tongue/throat position. It's a pretty arduous
| process and has fallen out of favor, so most deaf people who
| don't get a cochlear implant will use sign language only.
|
| However, with early implantation language acquisition is
| relatively easy (thought it varies per child).
| smeej wrote:
| Is it the capacity to learn to _speak_ a language, or the
| capacity to learn to _understand_ spoken language that shuts?
| Or both? It 's not quite clear from the way it's phrased here
| if maybe the inability to speak is only a consequence of the
| inability to understand, or if it's theoretically separable.
| Aren't there people who learn to speak, albeit with an accent,
| even though they have never been able to hear? So they might
| learn to read lips and speak, even though they wouldn't be able
| to understand a spoken language if they gained the ability to
| hear?
|
| I ask because I'm interested to know which parts of brain
| research might eventually try to prop that door open. Granted,
| most people born with this genetic condition would probably
| just be treated shortly after birth and learn spoken language
| during the normal time frame, not go through some special other
| treatment just to prop that mental door open, but I'd still be
| interested to understand what's actually going on in the brain
| better.
| coderintherye wrote:
| Looking up Otoferlin (the gene in question) led to this
| accessible and understandable paper:
| https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5283607/ (2016)
| somethoughts wrote:
| Also a slide deck/presentation that seems related by Akouos -
| the original biotech company that Eli Lilly acquired.
|
| https://akouos.com/wp-content/uploads/2021/05/2021_0503_ASGC...
| chewmieser wrote:
| Gift article link:
| https://www.nytimes.com/2024/01/23/health/deaf-gene-therapy....
| karaterobot wrote:
| > Some Deaf parents, he added, celebrate when their newborn
| baby's hearing test indicates that the baby is deaf too and so
| can be part of their community.
|
| Tough one. You want to respect the wishes of the parents, but you
| also want the kid to have the option to hear (and understand
| spoken language) when they are an adult and can make their own
| decisions. You may not be able to have both, given that this kind
| of deafness is progressive, and even with gene therapy you
| evidently need to treat it when young to give the child any hope
| of hearing. What if it turns out the kid wants to be able to
| hear, but by the time they are of age, it's too late and their
| inner ear's hair cells are all dead?
| throwuxiytayq wrote:
| Moronic tribalism at its worst. This is why we can't have nice
| things.
| Loughla wrote:
| Deaf culture (with a capital D) is a fascinating study in what
| it means to have a disability.
|
| The definition of disability is impairing one or more major
| life function. Capital D says that's not them. They just
| communicate differently.
|
| So. If they have that culture, is it bad for them to celebrate
| that they can share in it with their children?
|
| For reference, I think it's bad. But I can see the logic.
| rafaelero wrote:
| > Some Deaf parents, he added, celebrate when their newborn
| baby's hearing test indicates that the baby is deaf too and so
| can be part of their community.
|
| Omg, not only some people are deaf but also blind.
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