[HN Gopher] Gene therapy allows an 11-year-old boy to hear
___________________________________________________________________
Gene therapy allows an 11-year-old boy to hear
Author : mikhael
Score : 445 points
Date : 2024-01-23 17:26 UTC (1 days 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:
| Repairing our own bodies/preventing damage initially has
| significant advantages over something like Neuralink; Not
| fighting the bodies' own self repair mechanisms being a huge
| one.
| starttoaster wrote:
| This is incredible news to me, as someone with horrible
| tinnitus, I'm choosing to take this to mean that some day we
| may even have a treatment. If I ever could hear the sound of
| absolute silence again, I think it would reduce me to tears for
| a while. Hearing a ringing sound everywhere you go isn't
| literal torture, but it's maybe just below it, and I've been
| living with it for the past decade thanks to poor choices in
| the military.
|
| I recognize that they solved a completely different issue, by
| the way. But the fact that it's possible to do this means to me
| that a different treatment for tinnitus may some day also be.
| adventured wrote:
| What are the modern approaches used to try to lessen
| tinnitus?
|
| I've seen stories about therapy targeting white or brown
| style noise at it, adjusting frequency until it has an
| affect. And that over time it can, for some people, reduce
| the tinnitus.
| starttoaster wrote:
| I tried one of those frequency adjustment videos where it
| just loops through a bunch of different frequencies to try
| to find the one that cancels out the ring of your tinnitus.
| I haven't found one that worked for me, really. But I
| should give that more time. Other than that, I'm not aware
| of any modern treatments for tinnitus.
| lIIllIIllIIllII wrote:
| note - maybe for others suffering with it given your
| background but idk - I've had low-level tinnitus for ages, at
| some point it suddenly got really bad (hearing it over street
| traffic), turns out my ears were totally filled with wax
| which muffled sound and destroyed my SNR, therefore tinnitus
|
| back to normal slight tinnitus once removed. very easy
| process.
| CommanderData wrote:
| This could lead a way to fix Tinnitus, there's many causes of
| Tinnitus but it's almost always thought to be trauma to the
| Cochlea organ / structure.
|
| There's many cells involved in sensing and relaying signals
| to the brain, and are damaged by a growing list of things
| (Antibiotics, viral, Osteoporosis, lack of blood flow,
| Acoustic trauma, protein loss - long lived proteins in the
| Cochlea). Even our own immune system has the ability to
| damage the Cochlea.
|
| There is actually limited recovery of the OHC's at least but
| lack of regeneration potential. If there's a path to
| regenerate, it could one day fix the underlying cause and
| with this news it seems we're closer than we ever have been.
| 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.
| SnazzyJeff wrote:
| > It's fascinating how our brains are wired in such a way to
| enable read-only mode at an certain age in development.
|
| You're responding to a quote that is trivially false with a
| quick google. Ok.
| 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, but could hear. 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
| mkl wrote:
| They said "languages" for a reason. There are quite a few:
| https://en.wikipedia.org/wiki/Click_consonant#Languages_wit
| h...
| lucubratory wrote:
| Thank you!
| dghughes wrote:
| But those are not the only language they know though it's
| just a different language. The parts of a person's brain
| where language and speech exist are already developed. It's a
| mix of several areas for comprehension, speech, flow of
| speech - it's quite complex and not a single spot in a
| person's brain.
| dghughes wrote:
| Then again ruining the point of my own comment what about
| cochlear implants?
| 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".
| spywaregorilla wrote:
| Moreso comprehend it. It seems impossible to suggest that
| a person could learn to vocalize language but not to
| understand those vocalizations. It may be impossible in
| spite of what it seems.
| 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.
| makeitdouble wrote:
| > Aren't there people who learn to speak, albeit with an
| accent, even though they have never been able to hear?
|
| Yes, some people go through "speech therapy" and train to
| emit the right sounds while not hearing the output (but I
| think they rely on the inner vibrations ?).
|
| Understandably that requires a ton of training on top of
| existing skills and not everyone ends up with something
| workable.
|
| Part of the existing skills is the the ability to vocalize
| the sounds in the first place, and if a kid never
| intentionally vocalized for 11 years, I wonder if their vocal
| cords could ever develop to a point they can make the range
| of sounds needed.
| smeej wrote:
| It does seem like the difficulty, then, must be with the
| comprehension of spoken language, then, not strictly the
| speaking of it.
|
| I had wondered about this for awhile, how when you see
| adults have their cochlear implants turned on for the first
| time, sometimes they respond as though they do understand
| what people are saying to them. I had wondered how they
| could possibly know how to interpret the sounds as specific
| words, even if they knew the words, but this makes it seem
| like that's not what's happening. They're probably still
| reading lips to understand the words themselves.
| bb88 wrote:
| There's a youtube video of Helen Keller who was both blind
| and deaf. She learned to speak in her adult life.
|
| I don't have the link handy but it's entitled: "Helen Keller
| Speaks."
|
| If you look at the video it seems she even appeared to pick
| up the accent of her teacher.
| jimbob45 wrote:
| Was she not profoundly deaf?
| bb88 wrote:
| Well she was deaf deaf and blind blind. Not partially
| deaf or blind. The vocalizations and mouth movements
| could have been learned by touch.
|
| Tongue movements would have been harder to learn which
| explains why her vocalizations are hard to understand.
| tedd4u wrote:
| Came here to say I hope this can someday lead to a tinnitus
| cure :'|
| iopq wrote:
| Funnily enough, just listening to high pitched noises reduces
| my tinnitus:
|
| https://www.youtube.com/watch?v=xUZOSg3a1rk
|
| it turns out it's a "phantom limb" problem of hearing - when
| your high pitch hearing ability decreases, you start to have
| phantom sounds "fill in the blanks" at the frequency it got
| worse at
|
| you can test it yourself by generating sine waves and seeing
| when your hearing becomes worse
|
| https://www.szynalski.com/tone-generator/
|
| mine drops off at 12.5KHz and goes almost completely silent
| above 16KHz
|
| the tinnitus frequency is about 13Khz!
|
| Trying to listen to quiet noises between 12Khz and 16Khz
| trained me to be more sensitive to those sounds and to
| generate less tinnitus
| magicalhippo wrote:
| Ah interesting. I had noticed my tinnitus would get worse
| when I had been sitting in silence for a while, especially
| with over-ear headphones to further dampen ambient sounds
| (ie album ran out and I was so preoccupied with coding I
| didn't put another on).
|
| Fortunately mine is just at a mild annoyance level so far,
| but will try your trick.
| ebiester wrote:
| I am confused at this: the window that people speak about is
| largely one of having any language. Aissam seems to have a
| language, albeit an idiomatic one that was developed to
| communicate with parents. If so, he has developed the speech
| pathways, even if any given language will be one of second
| language acquisition.
|
| Now there may be another reason, but the article is either
| missing context or the question was not expressed in a way
| where the doctor answered in a way that follows the science
| around the critical language period, as I understand it (at
| least)
| SamBam wrote:
| Yeah, this seems confusing. Obviously his brain has a solid
| understanding of language, and he even recently learned a new
| language (Spanish Sign Language) so learning another language
| should be possible.
|
| It sounds like the researchers are saying there's something
| special about learning spoken language. But it seems to me
| that there can't have been many cases similar to his.
| mlyle wrote:
| > It sounds like the researchers are saying there's
| something special about learning spoken language. But it
| seems to me that there can't have been many cases similar
| to his.
|
| We've learned a lot from people who have received cochlear
| implants at different ages. Earlier implantation is
| strongly associated with functional spoken language use and
| fluent speech. There's a big benefit before age 5; a large
| proportion of those implanted before 24 months basically
| have normal language skills, while few after age 5 ever
| fully "catch up."
|
| edit: Here's a study of prelingually deafened adult
| outcomes with CIs
| https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5720870/ All
| of those studied had acquired spoken language before
| implantation and had some degree of effective hearing
| earlier in life, so were not fully deafened before the
| language acquisition window.
|
| The implants provide an improvement of quality of life but
| do not allow most of even this population to e.g.
| understand spoken language on TV without subtitles.
| Retric wrote:
| Listening to TV conversations is a high bar. In
| conversations people can ask for clarification or to slow
| down etc.
|
| Which explains: _Before implantation, 7% of the patients
| were able to have telephone conversations._ vs _After
| implantation, 60% of patients are able to have telephone
| conversations._
|
| Also, the technology dramatically improved over time so
| we don't have long term data on high quality implants.
| mlyle wrote:
| Still, pay attention to context. This was people who were
| successfully using hearing aids and oral language before
| implantation. Even in this subpopulation, they did not do
| _nearly_ as well as children do, even though this
| subpopulation was less deafened than most deaf children.
|
| > Also, the technology dramatically improved over time so
| we don't have long term data on high quality implants.
|
| We do have enough series to know that 5 year olds
| receiving treatment have (on average) significantly worse
| outcomes than 18-24mos.
| Retric wrote:
| For the timescales we have tested there's significant
| differences, and in terms of quality of life it's clear
| early intervention is a significant benefit.
|
| However, slower adaptation isn't zero adaptation. The
| limits for people implanted at 5 when they are 50 is
| still an open and IMO interesting question.
| mlyle wrote:
| > The limits for people implanted at 5 when they are 50
| is still an open and IMO interesting question.
|
| Slower development usually means a lower plateau, and I
| think we pretty much have to assume as such (and can be
| prepared to be pleasantly surprised).
|
| Else, we get to wishful thinking: older people on older
| devices developed more slowly and plateaued at a lesser
| value of hearing. Now, we have implanted older people on
| newer devices, and they're developing more slowly, but
| hey, maybe they'll _eventually_ develop fully normal
| hearing.
| Retric wrote:
| Do you have a source for that plateau? I've read that
| early success predicts future success on age adjusted
| tests. But the children were still improving in absolute
| terms over a decade post surgery.
| mlyle wrote:
| I was arguing mostly from the standpoint of delays in
| development are almost always correlated with lower
| ultimate attainment, no matter what measure you're
| looking at.
|
| But if you want cochlear implant specific data, here--
| https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10760633/
|
| You're right that time narrows the gap between early
| implantation and later implantation, but the slope of
| that narrowing is pretty small by the 20 year mark (and
| barely statistically significant in this moderately-sized
| study) and the gap is relatively big.
|
| The difference of time of implantation between the two
| groups was relatively small (mean implantation at 45
| months vs. 34 months) and produces a gap that's durable
| for decades. >130 months is way, way, out from 45 months.
| vladms wrote:
| Very interesting paper!
|
| Still, lots of work to do, to quote "However, it was not
| possible to control other factors, such as the socio-
| economic environment of the participants.".
|
| In my view this could affect the study quite a lot (or
| not, but unknown for now). They mention the initial
| intervention was 3 months on-site, but after "the patient
| returned to his area of residence, where he/she would
| have speech therapy and special education".
| Retric wrote:
| Thank you, interesting read.
|
| Looks like scores for both groups are still improving at
| 25 vs 20 years so gap isn't closing. I was expecting
| people to max out what the hardware is capable of or
| reach normal levels, but that doesn't seem to be what's
| happening.
| mlyle wrote:
| I was surprised at the amount of continuing improvement,
| too.
| nsxwolf wrote:
| Never fully catching up sounds a lot different than
| "window for learning spoken language is permanently
| shut". Am I missing something?
| mlyle wrote:
| "Permanently shut" for everyone is probably an
| exaggeration.
|
| A better description "very, very few of those [with
| hearing restored] after age 5, who had never had any
| hearing before, develop anything close to normal spoken
| language skills."
| graphe wrote:
| I recall that they trained a part of an insect's head like
| the nasal passage and it was able to be used for language
| better than the model at that time chatGPT2. So there's
| something innate in nature that can learn human languages.
| SamBam wrote:
| Can you find that study?
| graphe wrote:
| Took me a few hours but it was fascinating. I couldn't
| find it again easily.
|
| https://www.discovermagazine.com/the-sciences/fruit-fly-
| brai...
|
| https://oadoi.org/10.1371/journal.pgen.1007430
| SamBam wrote:
| Cool, and thanks for search!
|
| I think you may have slightly over-sold the study, though
| -- or at least what you remembered from it.
|
| My reading of the first study was that they took a
| simulated version of a relatively large (2000 node)
| neutral network that makes up part of a fruitfly brain,
| and were able to do standard neutral network training on
| it to do some language prediction.
|
| I'm not sure that this says anything about fruitfly noses
| being wired for language though. I expect that they could
| have taken that same simulated architecture and trained
| it to do anything that regular neutral networks could do
| -- detect faces, make stock market predictions, play a
| (poor) game of Go, or learn a homeowner's thermostat
| patterns.
|
| I think it's just more a statement about the power of
| neutral networks in general.
| graphe wrote:
| I did misremember, it seems they used a network of
| algorithms based on how flies use 2000 neurons and the
| original article I read it from a while ago may have
| oversold it. The fact it was able to predict well is them
| looking for inspiration in insects, and that they'll be
| using more insect inspired behavior.
| dbcurtis wrote:
| Language development starts earlier than most people think.
| Babies babble with all the sounds the human vocal tract can
| make, but by 6 months they are only babbling with the sounds
| of the language in their household. Up to age 12 or so you
| can learn a second language accent-free, but at that age an
| important developmental milestone cements phonemes in place.
|
| About 45 years ago I heard Chomsky speak on the idea that the
| human brain is wired to learn a grammar as much as a bird is
| wired for birdsong. So learning _some_ grammar is innate, but
| the particulars are up to environment.
|
| Source: 1/2 a century ago I was a bit of a developmental
| linguistics nerd. Disclaimer: But many memories have faded.
| omeid2 wrote:
| > Up to age 12 or so you can learn a second language
| accent-free, but at that age an important developmental
| milestone cements phonemes in place.
|
| This is only true if the second language has sounds that
| you don't have in your first language.
| dbcurtis wrote:
| Can you name a language pair where that works? I can't
| think of one.
| iopq wrote:
| If your native language is Ukrainian, when you learn
| Russian you don't get more phonemes, unless you count
| slightly different mouth positions of vowels.
| dataflow wrote:
| https://linguistics.stackexchange.com/a/30633
| Tor3 wrote:
| Oh, right. I forgot all those Dutch speed skaters who
| used to train in Norway a couple of generations back. Ard
| Schenk.. Kees Verkerk and many others. The majority of
| them learned Norwegian completely accent-free (you really
| had to listen to figure out they weren't native), despite
| not learning the language as kids. I figured that must
| have been because Dutch has such a variety of native
| sounds that it basically covers Norwegian language
| sounds.
| dataflow wrote:
| > Up to age 12 or so you can learn a second language
| accent-free, but at that age an important developmental
| milestone cements phonemes in place.
|
| Seems... dubious? What about people who immigrate later
| (like in high school) and actually pick up the sounds and
| accents flawlessly? I've seen folks like that and I'm
| pretty sure they weren't in high school at age 12.
| iopq wrote:
| Anecdotally, I know a Serbian who went to study to the US
| for a year in high school and has a perfect Californian
| accent.
| dataflow wrote:
| There you go!
| criddell wrote:
| > perfect Californian accent
|
| Oh man, I hope they sound like this:
|
| https://www.youtube.com/watch?v=dCer2e0t8r8
| Tor3 wrote:
| It's very very rare. Though it's not a cut-off at age 12,
| it just gets more and more rare (and takes longer) from
| around ten and older. Not sure if it's so much about the
| vocal tract, it's more about the brain's ability to hear
| sounds (my wife can't hear the difference between a
| number of sounds despite having lived in my country for
| many years and speaking the language well).
|
| In all my life I've only met two 100% accent-free
| speakers who learned my language as adults, and a third
| one who was almost there. Everyone else has _something_ I
| can detect. But children.. a five-year old Japanese girl
| could repeat everything I said with perfect pronunciation
| and intonation, first try. Slightly exceptional girl
| perhaps, she learned the language in a very short time.
| dataflow wrote:
| > Though it's not a cut-off at age 12, it just gets more
| and more rare (and takes longer) from around ten and
| older.
|
| I mean, nobody is disputing "it gets harder as you become
| older". I totally believe that. Lots of things gradually
| become harder as you grow older, and language doesn't
| seem particularly different in that regard. The question
| is whether that's because your body "cements phonemes in
| place" around age 12, or whether there's something else
| at play that's likely gradual and not such a sharp
| boundary. The fact that it's rare might be just due to
| the (a) effort required to learn something new being
| higher _in general_ , or the (b) perceived RoI being
| lower, or a ton of other factors that don't boil down to
| "your phonemes are cemented in place"... right?
| Anecdotally I know in at least one particular case that I
| observed and inquired about, that person (who's also very
| smart and hard-working in general) told me they made a
| very deliberate effort over a handful of years to improve
| their accent after immigrating, and that's how they
| sounded like a native now. I totally believe that many
| people are just unwilling to invest the effort required
| (which certainly increases with age). I'm just finding it
| hard to believe there's some biological force preventing
| you from doing it past age 12, given I've seen otherwise.
|
| > it's more about the brain's ability to hear sounds (my
| wife can't hear the difference between a number of sounds
| despite having lived in my country for many years and
| speaking the language well).
|
| That might be true for _some_ sounds for _some_ people,
| but I also have a hard time believing it 's such a
| general thing to the extent you're painting it here. It
| seems more likely to me the explanation is something
| else, like maybe nobody has managed to give her a good
| enough explanation as to how they're different sounds.
| (Maybe not the best example, but I had a hard time
| distinguishing ch and s in German until someone explained
| to me how they're each pronounced. Now I can hear them
| much better, and pronounce them not-too-awfully too.)
| Tor3 wrote:
| The sounds my wife can't separate are sounds which aren't
| separate in her native language - the mapping is trivial.
| It's the same with others from her country, it just
| varies between individuals (but strongly correlated with
| age, and to what exposure they've had to other languages
| when younger). And believe me, it's not about putting in
| the effort. But to distinguish certain sounds my wife has
| to watch my lips - this is particularly noticeable if I
| dictate and she writes. As for training - she received
| two years of training (30 hours a week) with expert
| teachers who knew a lot of tricks for how to hear and
| (not the least) pronounce sounds. Tricks that I didn't
| know about.
|
| But I've also seen this with American and some English
| adults trying to learn Norwegian - a great many of them
| can't hear the difference between vowels which, to me,
| are totally different. Can't hear the difference between
| the words "har" and "her", for example (NB: Norwegian
| sounds. Not English vowels). It seems to take a couple of
| years of daily ear training (or rather, brain training).
| As always, there are exceptions. But those exceptions are
| truly standing out.
|
| (Added: As soon as there's context or visibility the
| problem is much reduced - but it's still there, as soon
| as there's only audio and their language level isn't good
| enough to "select" the right words from context).
| lucioperca wrote:
| https://www.youtube.com/watch?v=qXxV2C1ri2k
| eythian wrote:
| I've noticed this within accents of the same language. My
| own English accent has a very mild separation between the
| _i_ and _e_ vowels in, e.g., _bitter_ and _better_. To my
| ear they sound wildly different, but many native speakers
| from other places and more English-as-a-second-language
| speakers can really struggle to tell apart what I 'm
| saying, to the point that it's usually easier for me to
| switch languages to the local one to distinguish the
| words if the context is ambiguous. Oddly, despite
| noticing shifts in my accent having lived abroad for some
| time now, this is something that hasn't budged.
|
| Similarly in my (learnt as an adult) second language,
| there are a couple of vowel sounds that aren't in English
| and I usually have to really focus to hear them, and to
| pronounce them correctly.
| LocalH wrote:
| The classic American example for me was always "ten/tin",
| "pen/pin", which in my Southern neck of the woods are
| typically homophones. My girlfriend is Midwestern, and
| definitely pronounces the "e" more in those variants.
| LastNevadan wrote:
| My favorite example in English is this sentence: "I
| peered at a pair of pears on the pier."
|
| As a native speaker, the sounds in peer/pair/pear/pier
| are slightly but detectable different. But non-native
| speakers can almost never say or hear these differences.
| Thorrez wrote:
| As a native English speaker (American) I think "peer" and
| "pier" are pronounced the same, and that "pair" and
| "pear" are pronounced the same, but that the 2 groups are
| pronounced quite differently.
| bobmaxup wrote:
| To be fair, they did qualify the statement with "or so".
| dataflow wrote:
| This isn't 12 vs. 12.5 I'm disputing, it's like 12 vs. >=
| 16.
| resonious wrote:
| To take your point farther, I've met plenty of people
| who've learned their second language as an adult and can
| speak nearly accent free. As far as I can tell, the only
| difference between those with and without an accent is
| conscious effort.
|
| The whole idea that there's a window that closes when
| you're a kid has seemed a little weird to me. Adults
| learn new vocab and grammar all the time. Learning
| another language is the same, just a little more extreme.
|
| I wonder if there's some actual scientific evidence for
| the language learning window, and not just some
| developmental psychology observations.
| mariuolo wrote:
| Perhaps their native languages were phoneme-rich and
| could map those to their L2?
| otikik wrote:
| > people who've learned their second language as an adult
| and can speak nearly accent free
|
| Perhaps it's just a capacity that some people have,
| similar to perfect pitch hearing. Possible, but rare.
| Jorge1o1 wrote:
| Funny that you mention perfect-pitch because in a similar
| vein it seems like one of those things that can be taught
| but the window closes very early in childhood.
|
| https://journals.sagepub.com/doi/abs/10.1177/030573561246
| 394...
| tough wrote:
| I was gonna bring this relation of also being a skill
| needing to be developed in early age, but you beat me to
| it.
|
| good thing there's already a drug for that
| https://www.npr.org/2014/01/04/259552442/want-perfect-
| pitch-...
|
| science is never fixed is it
| yodsanklai wrote:
| > To take your point farther, I've met plenty of people
| who've learned their second language as an adult and can
| speak nearly accent free.
|
| I've seen some instances of this, for instance Russians
| learning French.
|
| My guess is that it's possible if the native language
| phonemes are a superset of the second language.
| nlpparty wrote:
| Russian doesn't have the guttural 'r' of French.
| megablast wrote:
| It's not accent free, you learn the accent when you learn
| the language.
| poulsbohemian wrote:
| >As far as I can tell, the only difference between those
| with and without an accent is conscious effort.
|
| In my completely unscientific sampling, I'm going to
| agree with you. This holds true too for native speakers
| who make no effort to improve their grammar and
| vocabulary. If one wants to be a better speaker, writer,
| and communicator, they will be by effort.
| radicalbyte wrote:
| It's bullshit. I can speak Dutch without an English
| accent and I learned the language in my 20s, having 0
| exposure to it in my youth.
|
| There are to main sounds which are in Dutch but not
| English ([ui] and the hard [G]) and one which isn't in
| some English dialects (the rolling [R]). However you can
| absolutely learn them as an adult. It just requires
| serious training (years of hard practice, same as with
| sports). You literally have to build up the facial
| muscles.
|
| One thing I will admit: I choose not to pronounce the
| [ui] sound properly due to a combination of being lazy,
| identifying as an Dutch-as-second-language speaker and
| because for some absolutely irrational it sounds really
| childish to my years. That latter point played a
| surprising role in my lack of ability with the French
| language. It feels theatrical in the way that certain
| queer people choose to project their speak and I do not
| want to project as being something I am not (or be
| confused as someone making crude n-phobic caricatures
| which would be 1000x worse because it could make someone
| else feel insulted). Honestly it's probably a tick I have
| from being raised to be a polite British gentleman :)
| darkerside wrote:
| I've noticed that some people speak foreign languages
| with what I consider to be quite poor accents, but they
| enunciate strongly. If they are smiling, it almost comes
| off to me like they are making fun of the language or
| just pretending they can speak it well. To the point I've
| laughed along with them before realizing my mistake and
| cringing.
|
| And then I wonder which of us is more fluent? The one who
| with the better accent or the one who can more
| confidently project their thoughts in that language
| regardless of "skill"? (Probably the latter)
| 4gotunameagain wrote:
| Yea but you are talking about two languages that as you
| correctly pointed out only a few phonemes are different,
| with very similar phonology, stress, etc. They are very
| closely related.
| vik0 wrote:
| I feel like we're so deep in the thread - where after
| people have time and time again disproven the original
| comments message about how being 12 years old is some
| magic cut-off point to language learning - there are now
| people who just keep adding different requirements,
| leading this conversation nowhere and making the new
| point unfalsifiable and - by extension - unscientific;
| whereas the original comment had an allure of having a
| modicum of scientific rigor behind it (which, in the end,
| was disproven)
| radicalbyte wrote:
| I think that the original comment has some anecdotal
| truth - because the vast majority of people do not spend
| the effort required to speak their second/third languages
| without an accent. You have to do silly exercises and
| stuff for very little gain in the end. You're better off
| learning a 3rd language.
|
| Professional Actors do it all the time, they spend a lot
| of time practicing (and I assume money) being trained by
| Speech Therapists to different perfectly.
| darkwater wrote:
| My anecdotal example: Italian living in Catalonia (for a
| few years now, but moved as an adult), I speak both
| Catalan and Spanish with no Italian accent and what's
| even "worse", I speak Italian with Spanish accent ^^;
|
| I know these languages are all very similar, so that
| helped a lot for sure, but the 12 years rule is
| definitely not absolute.
| linuxftw wrote:
| Indeed. Sounds like another 'replication crisis' paper in
| action.
| hiAndrewQuinn wrote:
| Oh, rad, I didn't know accents worked like that! So if I
| speak to my kids in English, and my wife speaks to them in
| Finnish, they'll get to grow up with a disconcerting mix of
| newscaster English and northern Finnish drawl.
|
| I wonder if getting exposed to a bunch of languages as a
| kid is why I have a (relatively) mild accent in Finnish
| now, despite only starting to learn at 26 or so.
| vintermann wrote:
| For the two-language trick to work, both languages need
| to be equally useful for the kids. My sister and her
| Canadian husband tried having her speak to them in
| Norwegian, and him in English, but as soon as they
| understood that she could speak and understand English,
| but he could not speak or understand Norwegian, the kids
| switched to English. They still understand Norwegian, but
| they speak little of it and with a heavy English accent.
| compsciphd wrote:
| https://www.scientificamerican.com/article/evidence-
| rebuts-c...
| _flat20 wrote:
| https://blogs.scientificamerican.com/cross-check/is-
| chomskys...
| compsciphd wrote:
| that's not much of a defense
|
| "Another problem with the claim that Chomsky's theory of
| language "is being overturned" (as if it had ever been
| accepted, which is not true), is that it's not clear what
| "Chomsky's theory of language" refers to. He has proposed
| a succession of technical theories in syntax, and at the
| same time has made decades of informal remarks about
| language being innate, which have changed over the
| decades, and have never been precise enough to confirm or
| disconfirm."
| foobarqux wrote:
| There are a bunch of ideas that are more core and
| strongly supported (language is innate) which you use to
| explore more tenuous ideas about what the implications
| are and how they specifically manifest. Linguistics is an
| extremely nascent field compared to other sciences,
| Chomsky calls our stage of understanding "pre-Galilean",
| no one has claimed to have solved the basic questions yet
| so it isn't surprising that anything other than the core
| ideas are in constant flux. I haven't seen a good counter
| argument to the core ideas of universal grammar (or the
| minimalist program) and to refute an idea you need to
| actually present a counter-argument not simply say some
| sub-hypothesis has been refuted in the past so every
| fundamental idea has been refuted.
|
| See also https://ling.auf.net/lingbuzz/007363
|
| The SciAM article you linked doesn't understand the
| arguments Chomsky makes when "refuting" them (e.g. they
| erroneously say that superficial differences between
| languages show that there is no universal grammar).
| compsciphd wrote:
| I thought I read an article one that some very untouched
| tribes (ala amazon?) have fundamental different ways of
| communicating that undercut Chomsky's notions of
| universal grammer. Which the SciAm article glances on,
| but doesn't really go into any depth.
| Anotheroneagain wrote:
| It's because you lose the ability to form new abstractions
| once your neocortex shuts down. The sensory areas begin
| shutting down quite early (around five) for most. You can
| only hear which category the sound belongs to + the error
| ("accent" in this case) and lack the ability to perceive
| finer nuances after it happens.
| runlaszlorun wrote:
| My personal experience actually maps to this exactly and,
| when I've encountered it, it's been a far more predictable
| rule than I would have thought. This is anecdotal I know,
| and I've even looked up the science on this and it seems
| like isn't as black and white as my experience would
| indicate.
|
| But by now I've actually asked this of prob 20-30 people.
| All of them who came to the US before 12 (or attended
| American international schools overseas) had no accent. And
| all of them except two who had come here at 14 or later had
| at least a hint of an accent. There are the few like the
| one Hungarian I met who had no English other than spending
| 3 months in the US and whose English was so spot on I
| actually thought he was American.
|
| In my experience it does seem that there's something about
| the brain's plasticity that changes around 13ish. For
| example, I started programming young and also had took
| physics early at my local college and seem to internalize
| those much better than, for example, the follow-on physics
| course I took later on.
|
| But if anyone knows the science better feel free to correct
| me! A neuroscientist I am not...
| i2shar wrote:
| This is well known and well studied. I highly recommend
| Sapolksy's lecture on this:
| https://youtu.be/SIOQgY1tqrU?feature=shared
|
| Start at 12:14 for the relevant topic, but the entire
| lecture is a good watch.
| js2 wrote:
| Relevant _This American Life_ episode:
|
| > Jiayang Fan has this theory that because she's spent so
| much time thinking about her own accent when she speaks
| English, she believes that when she hears other Chinese-
| Americans speak, she can tell how old they were when they
| immigrated to the U.S. (7 minutes)
|
| > We test Jiayang Fan's self-proclaimed special skill by
| having her listen to three Chinese-Americans speak, and
| then guess when they came to the U.S. (20 minutes)
|
| https://www.thisamericanlife.org/786/its-a-game-show
| foobiekr wrote:
| Audio processing is a bit light sight processing - once you
| miss the critical development period, which is not directly
| related to the "critical period" for language learning, you
| will never actually develop them. People can develop
| something, for example people who had oxygen-destroyed
| corneas causing blindness who later got corneal transplants,
| but it will never be vision as you know it.
| Tor3 wrote:
| Search "kitten vertical lines experiment" on the Famous
| Search Engine. Kittens not allowed to see horizontal lines
| for the first few months would never be able to see
| horizontal lines, ever.
|
| It's at least somewhat like that with humans too. Ever
| wondered why some kids are wearing a patch over one eye? If
| the child needs glasses but (in particular) when they
| didn't get proper correction early on then they may have
| double vision, and what the brain does is to block one eye.
| That eye, despite "seeing", will lose the paths in the
| brain necessary for seeing well. The patch forces the brain
| to start using the eye again.
|
| This happened to me - the doctor told my father "no need to
| check this regularly", and after some years one of my eyes
| had indeed lost resolution. It's still like that. One eye
| can see very well, the other at much lower resolution.
| Though I found that even at middle age it was possible to
| improve that to some extent - not the actual resolution,
| but the brain's ability to actively use the eye could be
| improved a little. I would read books with only one eye.
| Could only read half a page at the beginning. But it's
| impossible to recover the vision I lost as a child, which
| was caused by the brain ignoring the eye.
| wildylion wrote:
| I have the same however my other eye is fine with regard
| to resolution - I just need glasses or a contact lens.
|
| But yes, the binocular vision is permanently shot (though
| I get some improvement at times).
|
| That's what you get for being an insufferably stubborn
| kid.
| branko_d wrote:
| I have the same situation.
|
| The annoying part is that my "untrained" eye is not near-
| sighted, but my "trained" eye is. I suspect it was
| different in the childhood (untrained: far-sighted,
| trained: not far-sighted) and then shifted in the
| direction of near-sightedness over time.
| hoseja wrote:
| The ad-hoc language (generically called "kitchensign"
| apparently?) might be too primitive.
| lofaszvanitt wrote:
| Maybe the brain's plasticity. I read somewhere, a long time
| ago, that brain nerve cells wonder around and do their thing
| based on incoming stimuli when the child develops. During
| very early age a lot of things can be corrected by the brain
| if something goes sideways, thanks to the plasticity of these
| nerve cells, but after a while, when the nerve cells "settle"
| and "conquer their role", they cannot change anymore.
| chriskanan wrote:
| Sensory and motor systems have critical or sensitive periods
| early in life and then the neural network is pruned and the
| critical period closes. This is why second language
| acquisition is much easier at younger ages. There are some
| drugs that may reopen the critical period although not much
| can be done about the pruning. So basically all the early
| neural circuits for interpreting sounds are not organized
| correctly and it's unclear if they can be post critical
| period.
| jv22222 wrote:
| You probably know about all about meniere's. If not, have you
| tried going off salt?
| Etheryte wrote:
| Have you tried going off pseudoscience?
| holdsleeper wrote:
| What part of their comment was "pseudoscience"?
| JumpCrisscross wrote:
| > _What part of their comment was "pseudoscience"?_
|
| Is hypernatremia a cause of Meniere's?
| MathMonkeyMan wrote:
| Reducing salt might help with symptoms[1].
|
| [1]: https://www.mountsinai.org/health-library/special-
| topic/m-ni...
| fouc wrote:
| I suspect this is a reference to the neural mapping around
| auditory signals, but given that the brain is still capable of
| changing at any point in life I disagree it's "permanently
| shut"
| blitz_skull wrote:
| I find this incredibly hard to believe with all of the research
| on neuroplasticity. Not to mention there's a VERY famous case
| that proves this is not a hard and fast rule: Helen Keller.
| bradrn wrote:
| Helen Keller had already learnt a 'home sign' system, which
| was presumably language-like enough to allow her to learn
| English later.
| avarun wrote:
| And this kid knows sign language too.
| hmcq6 wrote:
| I just can't imagine we have that much hard data on the
| topic. Unless there have been massive breakthroughs in
| hearing aid tech that I'm unaware of.
|
| Cochlear implants are amazing but my understanding is they're
| not 100% restorative. To make a bad metaphorical comparison
| with blindness, they're like glasses that restore your vision
| but if the only shape produced were shutter shades.
|
| (pic for reference: http://lh6.ggpht.com/nML2bdK30Z0OS3cHBINn
| LcXCv6XVI8dWpLvMu8m...)
| phire wrote:
| Worth keeping in mind that Helen Keller didn't loose her
| hearing (and sight) until an illness at 19 months old.
|
| At this age, a child's brain has already locked in the sounds
| for their native language and lost the ability to learn non-
| native sounds (hell, research suggests that unborn infants
| can recognise the difference between their mothers native
| language and foreign languages before they even leave the
| womb). The typical child will have been using single word
| sentences for months and just starting to move onto two word
| sentences.
|
| Keller might have regressed to zero language abilities after
| her illness, but she didn't need to start completely from
| scratch when she learned how to speak.
| SnazzyJeff wrote:
| > Worth keeping in mind that Helen Keller didn't loose her
| hearing (and sight) until an illness at 19 months old.
|
| While this is in fact an important sample, this doesn't
| imply much about how humans develop after 19 months, much
| less how they develop before 19 months.
|
| > At this age, a child's brain has already locked in the
| sounds for their native language and lost the ability to
| learn non-native sounds (hell, research suggests that
| unborn infants can recognise the difference between their
| mothers native language and foreign languages before they
| even leave the womb).
|
| We have nearly zero clue _how_ the child 's brain
| recognizes their "native language". We know they react
| differently at different stages of their development to the
| same stimulus, which is occasionally linguistic. We have
| nearly zero clue what the mechanism is that corresponds
| input to measurable output. This is a very disingenuous
| characterization of the data.
|
| It's also worth mentioning that the root of this question
| is trivially false--people obviously learn language after
| the age of five. Such haphazard presentation (at best)
| should not be taken seriously.
| SnazzyJeff wrote:
| Hellen Keller never developed the skill to listen to spoken
| language.
|
| I agree with you fwiw, but your argument needs to acknowledge
| the above statement.
| blitz_skull wrote:
| Hellen Keller was deaf. How could she _develop_ the ability
| to listen to a spoke language?
| SnazzyJeff wrote:
| if only this site could manage something more complicated
| than the dialectic of "not retarded enough for y-combinator"
| and "too retarded for y-ycombinator"
|
| one day, y-combinator will give a shit about disability.
| There is not enough money in the game for the powers to be to
| care yet.
| heyoni wrote:
| Is this one in deaf children or others with receptive language
| but no ability to speak?
| SnazzyJeff wrote:
| > Gives me hope that one day my tinnitus may have a cure.
|
| We are all chained to reality. We must all accept reality or
| kill ourselves trying to.
| SnazzyJeff 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.
|
| This is trivially false. How are you acting like this person
| can be taken seriously? At best, they're wildly hyperbolic in
| their statements. At worst, they're funded to push a polemic.
| MathMonkeyMan wrote:
| How is it trivially false? I know nothing about it.
| judge2020 wrote:
| Maybe the quote is about the critical period hypothesis[0],
| which is not universally accepted.
|
| 0: https://en.wikipedia.org/wiki/Critical_period_hypothesis
| SnazzyJeff wrote:
| Well, the part that was claimed. "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." The person seems to mistake
| the term "speech" for the phrase "language comprehension"--
| the field moved past that decades ago.
| iteria wrote:
| I was extremely confused by this statement because
| well... I exist. I didn't start speaking until around 5
| because of various health issues and I wouldn't say I was
| reasonable at it until I was a preteen, but I definitely
| acquired language, just extremely slowly.
| Tor3 wrote:
| I too have to ask why this is "trivially false". We hardly
| have even anecdotal references to people who never heard
| language until after five (except for stories about children
| raised by wolves and couldn't learn to speak - not exactly
| stories we can trust).
|
| Of course that's goes the other way too - which studies are
| Dr. Germiller referencing? But again - if it was "trivially
| false" this would mean that it's something generally known
| because it's observable. And it isn't, as far as I'm aware.
| TomK32 wrote:
| The brain is a surprising organ, it's only been four month
| since the kid can hear anything and maybe the brain is powerful
| enough to use this first-time influx of new impulses as the
| same start when learning language as a baby. After all, the
| brain needs 25 years to fully form.
| mannyv wrote:
| The problem with these kinds of statements is they're
| impossible to test. They depend on 'found' examples, like kids
| raised by wild animals.
|
| But those kids were different.
| anonymousnotme wrote:
| It is my understanding that psychedelics can open up that
| learning window. I wonder if that could be used to benefit in
| this case. (I imagine a lot of people might be opposed to
| having a minor take psychedelics; but if it works, does it
| matter?)
| joedevon wrote:
| Look up neosensory. They are a company by David Eagleman who
| has some amazing TED Talks. They have a product that treats
| tinnitus.
| 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.
| BurningFrog wrote:
| We 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.
| oefnak wrote:
| You don't need to be deaf to communicate with deaf people,
| right? You could learn sign language either way.
| actionfromafar wrote:
| What does it mean to _be_ a bat? (Not just be able to talk
| to one.)
| throwuxiytayq wrote:
| Are deaf people a different species now?
| malnourish wrote:
| They're referencing a philosophical essay on the nature
| of conscious experience
| themaninthedark wrote:
| I can't understand it.
|
| Someone who is deaf has a large number of obstacles to
| overcome and it is amazing that they are able to do so.
|
| Neuro-Atypical people could make the same argument, they just
| think and process things differently.
|
| But why wish that your children or anyone else has to
| overcome the same obstacles?
| cortesoft wrote:
| > Neuro-Atypical people could make the same argument, they
| just think and process things differently.
|
| Many have. There is a major movement in the community to
| treat neurodivergence as something other than a disability.
| overstay8930 wrote:
| I'm surprised it's taken so long for academia to catch
| up, militaries have figured out decades ago that these
| "disabilities" are just people who are good at different
| things and are assigned tasks that they can do much
| better than the average person.
|
| It is no coincidence that people with ADHD are drawn to
| certain fields, for example.
| rocqua wrote:
| Do you have any such examples? Only thing I know of in a
| military context is colorblind people being better at
| defeating camouflage, and I believe that isn't actually
| used in practice.
| overstay8930 wrote:
| Israel Defense Forces is probably the best example (they
| were the first to put it in practice publicly):
| https://www.businessinsider.com/army-autistic-people-
| israel-...
|
| You can see hits of it through Five Eyes, cybersecurity
| is hot if you are autistic as well:
|
| https://www.independent.co.uk/news/uk/home-news/gchq-
| jobs-re...
|
| https://hbr.org/2017/12/why-the-australian-defence-
| organizat...
| 331c8c71 wrote:
| It gets even funnier. Some autistics claim that it's the
| "normies" who are messed up with all their emotions,
| irrationality and social status games. Lots of that on
| reddit.
|
| It's an immense rabbit hole btw trying to understand how
| autistics experience life and interactions. I was totally
| unaware of all this until I met someone who interacted in
| a very unusual way (to put it mildly)...
| IAmNotACellist wrote:
| They have obstacles to overcome, but those obstacles are
| mostly imposed on them by the wider culture, as I've come
| to understand it. Having spent 4 years learning ASL and
| being close friends with a lot of deaf people now, I'm
| starting to conclude that ASL and especially technology
| makes it clear it doesn't have to be a disability at all,
| at least for people who were born deaf. Losing a sense is
| still quite a disability.
| rocqua wrote:
| I believe people with down-syndrome have a similar
| community with similar worries. The worries are more about
| genetic screening shrinking their community than about
| children, but I feel like the sentiment of wanting to
| protect and share their community is the same.
| mbil wrote:
| A little off topic, but the 2019 movie Sound of Metal
| explores some of deaf culture and is a pretty excellent film
| for anybody interested.
| at_a_remove wrote:
| I'm curious to know if their stance and logic extends to
| taking government money, registering as disabled for any kind
| of benefit, and so forth. If it isn't a disability, don't
| take the money.
| graphe wrote:
| If you can't get aid for it, I don't think it's classified
| a disability. What if they get rid of it later? I
| registered for adhd in my old school and I don't even
| mention it anymore since I didn't need the extra time for
| exams.
| coffeemug wrote:
| This argument is a classic case of "Yeah, sure, I mean, if
| you spend all day shuffling words around, you can make
| anything sound bad, Morty."
| kelnos wrote:
| I see the logic, but it feels rooted in a sort of state of
| extreme denial, based on a false starting premise. Deafness,
| in our world, is a disability. Not being able to hear does
| actually exclude you from a lot of things that us hearing
| folks take for granted for the most part. I think it's
| amazing that so many deaf people are able to function in the
| world as well as they can. They _should_ be proud of what
| they 've accomplished.
|
| But... man, no no no no. And it's not just communication,
| either. Like... deliberately denying a child the opportunity
| to hear birdsong, raindrops landing on a roof, the crashing
| of ocean waves, their cat purring and meowing at them. Hell,
| being able to listen to human-made music, more than just
| feeling the vibrations if it's loud enough and the speakers
| are on the floor. That's criminally abusive.
|
| If parents had a child with normal hearing, and deliberately
| damaged it to make the child deaf, we'd call that abuse. Why
| is refusing a treatment to restore hearing not at least in
| the same ballpark?
| RoyalHenOil wrote:
| I guess the question for me is, if they have a hearing child,
| what's stopping that child from being a part of their
| parents' culture?
|
| If a Deaf couple had two children, one with hearing and one
| without, would the hearing child be excluded from the
| community and only the non-hearing child welcomed?
|
| Or is the worry that the hearing child will leave the Deaf
| community and move on to greener pastures once they grow up,
| while the non-hearing child will have no choice but to stay?
|
| Either way, it paints a pretty grim picture.
| PlunderBunny wrote:
| Wouldn't a child that can hear (and therefore speak) born to
| deaf parents also learn sign language as a matter of course?
| I.e. they would be able to communicate with their parents in
| the parent's preferred medium either way.
| akoboldfrying wrote:
| Yes, they could also learn to sign, just as children growing
| up in bilingual families learn both languages without issues.
| This puts to bed any question over whether the child can
| participate in the parents' community.
|
| I firmly agree with another commenter that any deliberate
| effort to restrict a child's sense experience is child abuse.
| I'll add that I think it's about the most selfish thing I can
| imagine, and that I put it in the same category as female
| genital mutilation.
| lacrimacida wrote:
| I dont think this is happening too often and I also have a
| hard time understanding how this could be put into practice
| unless the community is isolated and cultish. Hearing abled
| children will develop more or less their hearing naturally.
| If they're not completely isolated they will learn to
| communicate with others even without their parents help.
| Modified3019 wrote:
| Denying their child an entire sense because the idea of the
| child having a full human experience makes the parent feel
| lonely, is straight up child abuse. Good parents want what's
| best for their child, not themselves.
| richbell wrote:
| A popular belief in the Deaf community is that having hearing
| children is undesirable. Some people argue that things like
| cochlear implants are "genocide."
| KingMob wrote:
| Reminds me of the rabbis accusing Jewish people of marrying
| non-Jews as participating in a "Silent Holocaust". Way to
| drive people away, my dude.
|
| In some ways, it's a moot point, since iiuc, most deaf
| children are born to hearing adults, and not within the
| Deaf community. Genetic treatments will almost certainly be
| preferred by those parents, and the Deaf community will
| slowly age up and die out in a few generations for lack of
| replenishment.
| kelnos wrote:
| Well, a few generations after _all_ types of congenital
| deafness are treatable. The gene therapy in the article
| so far only treats one, fairly uncommon, genetic cause.
| IAmNotACellist wrote:
| >makes the parent feel lonely
|
| You misunderstood. It says some of them celebrate that their
| child will have the opportunity to be a part of their
| community and culture. From what they've experienced, they
| can see it'd be a profound shame if their child isn't able to
| participate in something they've had so much positive
| experience from. Though that's not universally true. Also a
| child growing up hearing with deaf parents will have a whole
| set of problems that they would find challenging to meet, and
| could fear not being able to help.
|
| On top of that, cochlear implants are not miracle devices,
| and as I understand it, deaf children who get it will still
| have significant hearing and speech issues and may end up
| isolated from both sides.
| kelnos wrote:
| Regardless of the deaf community potentially being a place
| of rich, positive culture, deafness is still a disadvantage
| and a burden in everyday life.
|
| I get that people need to accept things like deafness or
| blindness, and adopting a community and sharing the support
| that provides is a big part of that. But denying your child
| treatment that would allow them to have all their senses,
| because you want them to be a part of your community and
| culture, is a selfish act, full stop. If parents are
| expected to try to give their child the best life possible,
| a treatment to restore a deaf infant's hearing is a no-
| brainer. It's table stakes. I agree that denying a child
| that is abuse.
| Tor3 wrote:
| But many children grow up with deaf parents - one or
| sometimes both of them. And they learn sign language to
| perfection, as any child can learn languages to perfection.
| It's hard to see that the ability to also hear will make
| them not able to participate in anything.
| didntcheck wrote:
| Yep. If the "community" is excluding them for being able
| to hear, that sounds like their problem
| IAmNotACellist wrote:
| The hearing community excluding deaf people is about a
| million times more common
| IAmNotACellist wrote:
| >It's hard to see that the ability to also hear will make
| them not able to participate in anything.
|
| If they're hearing they won't understand the deaf
| community, nor need it, and will miss out on what their
| parents and other community members valued so much
| scrps wrote:
| I think some religious parents with gay kids have tried
| that line before.
|
| Deafness is a medical issue depriving a human being of a
| naturally evolved sense that is critical to survival even
| in a stable civilized world. Denying them that sense
| because of identity politics is every type of wrong.
| IAmNotACellist wrote:
| 1. Hearing isn't critical to survival in our modern
| world, at least in the West
|
| 2. What you mean to say is denying people the ability to
| regain their sense of hearing, and the medical
| interventions you're alluding to don't do that to the
| extent you're imagining. Our best approaches, cochlear
| implants: don't restore hearing in the way that removing
| earplugs would, have a large learning curve; only work on
| some people; and the affected person's brain usually
| can't be fully capable of understanding and reproducing
| spoken language the way a born-hearing person would.
| People who receive them also still rely on some amount of
| lip-reading, sign, and apps.
|
| 3. People who are adept with sign and familiar with the
| deaf community often communicate far, far easier with
| sign language than a hearing person communicating with a
| deaf/HoH person who has a cochlear implant
|
| To what extent do you actually have knowledge and
| experience on the topics you're discussing?
| scrps wrote:
| A friend of mine was stabbed to death during a robbery,
| he was from a low income neighborhood very much in a
| wealthy western city, he was stabbed to death simply to
| avoid him fighting back because he was a big guy so they
| ran up on him from behind, something he would have heard
| had he not been deaf but thank you for the lecture.
| janeerie wrote:
| Cochlear implants actually are kind of miracle devices.
| With early implementation, deaf children can have perfect
| speech and pretty good hearing (not perfect). We are at the
| point where denying your child speech and hearing is a
| choice.
|
| My son is deaf, with a CI in one ear and hearing aid in the
| other. If you couldn't see them, you wouldn't think there
| was anything different about him.
| collegeburner wrote:
| no actually, we don't. not any more than we want to allow
| parents to circumsise their children, or feed them garbage
| until they have a BMI of 35
| kelnos wrote:
| My first reaction to that line in the article is that it's
| incredibly selfish of a parent to feel this way, and then to
| potentially deny the kid treatment that could restore their
| kid's hearing.
|
| But I agree with a sibling poster that this is actually
| straight-up child abuse. I'm generally skeptical of people who
| invoke child protective services for all sorts of imagined
| things, but I think this qualifies. If a child is born deaf,
| that deafness is treatable, and the parents refuse to treat
| their child, that child should be removed from the care of
| those parents, treated, and placed with a family that doesn't
| put their own selfish needs over the health of their child.
| suslik wrote:
| But why? What if an average deaf child is happier than an
| average hearing child because of their community support,
| culture, and the sense of identity? I don't know anything
| about the lives of deaf people - but it definitely looks like
| there's something to it; what I don't get is why is it so
| obvious to everyone else.
| mchusma wrote:
| Most people would consider deliberately damaging a child's
| hearing permanently so they can be a part of the deaf
| community to be child abuse. Similar to paralyzing a child
| to be a part of that community.
|
| It's great that there are communities for the disabled,
| trauma victims, etc. But those communities should hope that
| someday those communities are no longer needed.
| kelnos wrote:
| You won't know about the level of happiness the child
| experiences until it's too late to reverse the damage. Or
| if it's still possible to reverse the damage, it may be
| difficult or impossible to acquire full spoken language
| skills, as the article notes is the case for this 11-year-
| old boy.
|
| So the plan is to leave the child without their hearing, in
| the _hope_ that they just _might_ end up being happier than
| if they could hear? And we can 't run this experiment both
| ways! There's no way to know definitively which way the
| child will be happier. So it makes sense to me to give the
| child the ability to go through life without having to bear
| the burden of the disadvantages of deafness. And as much as
| some people in the deaf community will try to tell you that
| they have no disability, and that they just communicate
| differently... well, that sounds like a denial-based coping
| strategy to me. If that works for them to make themselves
| happy and get through life, then seriously, genuinely, I am
| glad they have that. But it feels abusive to force their
| child to have to go through the same thing.
|
| Consider a different case: parents are deaf, child is born
| with normal hearing. What would we think if the parents
| then deliberately damaged or destroyed their child's
| hearing -- because they believe their child could be a
| happy member of the deaf community and deaf culture -- even
| if it could be done surgically, without causing the child
| immediate pain or distress? No doctor would ever perform
| that procedure, and we'd absolutely call that child abuse.
| While I don't think that's _exactly_ the same thing as
| denying a deaf child treatment, it 's IMO close enough for
| the conclusion to be the same.
|
| (And before anyone thinks to bring up religious practices
| that forbid certain kinds of medical treatments: IMO those
| people are child abusers too, if they deny a child a live-
| saving or even life-improving treatment on religious
| grounds.)
| avnigo wrote:
| CODA (2021) is a great film I'd recommend that touches on that
| (stands for Children of Deaf Adults).
|
| https://en.wikipedia.org/wiki/CODA_(2021_film)
| mgl wrote:
| Not particularly connected but for anyone interested in analyzing
| their DNA:
|
| You may get your somehow accurate (not: medical grade accurate)
| raw DNA sequence from Ancestry DNA kit. Why from Ancestry and not
| other similar services like 23andme? Because they probably have
| the best accuracy for the money.
|
| You may then submit your DNA code to https://promethease.com/
| that builds a personal DNA report based on connecting a file of
| DNA genotypes to the scientific findings cited in SNPedia.
|
| You may learn a few things about yourself and your kids, which
| may also include severe conditions which could unfold in the
| future.
|
| Sample report:
|
| https://files.snpedia.com/reports/promethease_data/promethea...
|
| Disclaimer: sharing your DNA is always risky
| jkingsman wrote:
| Without commenting on the risks vs. rewards of sharing your
| genetic material, services like Nebula Genomics have reasonably
| priced (USD$249 30x, USD$899 100x) sequencing that's extremely
| high quality and suitable for getting into learning
| bioinformatics, if you're willing to wait for a few months and
| that's your jam (i.e. the data is sufficiently deep and full
| coverage that you can get meaningful results from it as opposed
| to the limited view of SNP analysis like 23andme or ulta-low-
| depth sequencing).
|
| The last frontier for consumer/prosumer genomics is hifi
| sequencing for correctly getting at your hard-to-read areas
| that are full of long repeated runs. Dante Labs offers
| sequencing that targets this for about USD$1900, but it's an
| evolving area in terms of bang for your buck.
| derefr wrote:
| Maybe an odd question, but -- given modern technology, if you
| were a bio lab tech and bioinformaticist yourself, would it
| be _practical_ to just order some used equipment off eBay and
| build yourself a home DNA sequencing setup?
|
| And if so, would it then just be a matter of _time and
| effort_ (rather than equipment and materials cost) to do a
| more thorough sequencing of your own DNA than any lab would
| ever be willing to do for you?
| femto wrote:
| https://www.linkedin.com/pulse/art-possible-biohacking-
| diy-d...
| cjbgkagh wrote:
| AFAIK the cost of consumables are amortized by doing a few
| people's DNA sequencing in quick succession. Low cost
| suppliers like Dante Labs will run specials where you agree
| to wait longer and they'll fit you in with a batch with
| other people who will pay extra for faster results. Oxford
| Nano-pore will sell a kit that I think is $1K but the
| training on how to use it is a lot more. Hopefully
| technology will keep getting better.
| new299 wrote:
| I've done this:
|
| https://aseq.substack.com/p/bringing-up-an-old-ebay-miseq
|
| Your issues are that you will still need to purchase
| reagents from the sequencing instrument vendor. They will
| try and push you toward a service contract.
|
| Each kit will cost ~$600 (cheapest kit) an old Illumina
| sequencer which you can still buy reagents for will cost at
| least $5000.
|
| Doing a whole genome this way would be expensive... I'd
| guess $10K to $20K perhaps? You'd need a lot of kits... or
| one of the high spec sequencers (NextSeq 550 etc).
|
| Alternatively you could look at getting a nanopore
| sequencer. This will be cheap but the data quality is
| different (and may not be comparable/require high coverage
| for certain applications). I'd guess you could do a (30x)
| whole genome for <$10K all inc here?
| darkerside wrote:
| What makes nanopore screening quality worse? Aren't these
| long read sequencers that are supposed to read more of
| the DNA strand?
| jhbadger wrote:
| The problem with Nanopore (which I've used for some
| projects) is that the per-base accuracy is still quite
| low. This can be helped to a degree by either high
| coverage (basically sequencing the same area over and
| over again with the hope that the errors are stochastic
| and will be corrected if you take the "average" base at
| each position) or combining it with shorter read but
| higher quality data from Illumina.
| tjpnz wrote:
| Will Nebula Genomics share my genetic information with othrr
| corporations or law enforcement? In what country is the
| sequencing done?
| 4gotunameagain wrote:
| > Will Nebula Genomics share my genetic information with
| othrr corporations or law enforcement?
|
| yes, as it is stated in their website they have to comply
| with U.S. laws.
| judge2020 wrote:
| Complying with subpenas sure, but AFAIK 23andme et al.
| voluntarily sell access to searching DNA data to law
| enforcement agencies. Does Nebula do this?
| graphe wrote:
| Dante doesn't and these companies use labs everywhere.
| Dante may even lose your sample and may give you the wrong
| data, it used labs in Italy so your data is (not) safe in
| the "best" way possible. Dante takes longer as well. My
| friend used them, I don't think you can choose the lab.
| aloer wrote:
| What is the expectation here for the coming years, does it
| make sense to wait?
|
| I assume this tech moves like most tech and it will only get
| cheaper like you say but also better. Are we still in the
| early adopter phase?
| graphe wrote:
| Your info is woefully outdated. Promethese was sold and doesn't
| have good data, last I checked it had only old papers and
| outdated GWAS. Neither of them are the best accuracy for the
| money. Dante labs and nebula labs have real 100% 30x not the
| crap 5% ancestry and 23andme have.
| mfld wrote:
| Thanks for the update, didn't know they were acquired by
| MyHeritage in 2019.
| graphe wrote:
| 23 and me also sold data. GSK also owns the data now.
| https://cglife.com/blog/23andme-sold-your-genetic-data-to-
| gs...
|
| Hacked too
| https://www.theverge.com/2023/10/7/23907330/23andme-leak-
| hac...
| iamthejuan wrote:
| I used Nebula Genomics with lifetime membership to see any new
| medical research that affects my genes.
| brcmthrowaway wrote:
| Referral link?
| mfld wrote:
| Yes, but the type of GWAS-based reports they offer not very
| well suited to uncover medical conditions like this.
| 331c8c71 wrote:
| One needs to remember about incentives here. Nebula or
| similar benefits from having you as a client. Most likely
| than not they would overplay the relevance and the
| actionability of the variants they report.
| enjaydee wrote:
| I've got a startup in this field in Australia. We're still
| working on the retail pipeline but we're up and running for
| bespoke cases. We deal mostly with IVF, and then stuff like
| CVD, and rare diseases. WGS, Methylation...
|
| https://www.23strands.com/
| 911e wrote:
| Amazing news
| enjaydee wrote:
| I've got a startup in this field in Australia. We're still
| working on the retail pipeline but we're up and running for
| bespoke cases. We deal mostly with IVF, and then stuff like
| CVD, and rare diseases. WGS, Methylation...
| bratwurst3000 wrote:
| Is this relevant to tinnitus? Sounds like it could help but I am
| no expert
| CommanderData wrote:
| Not directly, but breaking it down - they've successfully
| delivered viral code to inner hair cells.
|
| I have been following Tinnitus for quite a while now and it's
| assumed it's caused by some form of trauma to the Cochlear,
| either auditory nerve, IHC/OHC or SGN cells. Even with
| patient's with no record to prior trauma (potentially immune
| system dysfunction or compromised blood labyrinth barrier /
| BLB).
|
| Now if this is continues to show success we could start seeing
| more therapies targeting the Cochlear by way of gene therapy
| and it potentially helping people with Tinnitus by treating
| it's underlying cause. I.e. Regenerating lost cells in the
| Cochlear.
| ikesau wrote:
| Does this go both ways? can we use gene therapy to make 11-year
| old boys deaf?
| copperx wrote:
| That's the dream of many deaf parents.
| aquafox wrote:
| > His is an extremely rare form, caused by a mutation in a single
| gene, otoferlin
|
| No. Otoferlin is the _protein_ encoded by the OTOF gene. I wish
| science journalists would be more science literal.
| CreRecombinase wrote:
| No. OTOF is the gene symbol. Referring to the gene as otoferlin
| is perfectly legitimate
| JR1427 wrote:
| Exactly. Also, even if the gene and protein names were
| different, real scientists wouldn't get hung up on minor
| technical slip-ups like this, because everyone knows what is
| meant. (Former cell biologist)
| zooi wrote:
| Did you mean "literate"?
| DoingIsLearning wrote:
| Question for the Bio people, from what I've seen gene therapies
| so far target very narrow scope sometimes rare diseases. Is there
| any 'broad spectrum' type gene therapy currently in the pipeline
| of any company out there? (not specific to audiology)
|
| And is the narrow scope because it is easy to control in these
| early days or is that we simple don't know enough to make more
| complex gene therapeutics without understanding collateral
| damage, side-effects, for example?
| kozinc wrote:
| Being a layman too, as I understand it, it's because the
| deafness is caused by a single (mutated) gene which makes it
| (comparatively) easy to change. In this case, because the
| altered gene causes a necessary protein to be faulty or
| missing, gene transfer therapy can introduce a normal copy of
| the gene to recover the function of the protein.
|
| For other diseases caused like this you're mostly right on the
| money - sometimes because while a single gene might be the
| culprit, the mechanism is unknown, sometimes mutations in some
| 'single-gene disorders' may not be in a single-gene at all, not
| to even mention other possible interactions or the risks
| inherent to gene therapy.
| salubrioustoxin wrote:
| Yes. Also, even within rare disease this application has
| desirable features. Obvious bio marker (hearing), relatively
| isolated organ system with obvious delivery method (inject into
| cochlea). main downside is that there is an existing
| alternative (implant) so risk/benefit question is less obvious
| compared to eg neurodegenerative diseases.
| DoingIsLearning wrote:
| Is delivery critical or just get it close enough to tissue in
| ROI?
|
| Are we aiming to modify specific cells/layers? And what
| happens to adjacent tissue that is 'accidentally' modified?
|
| Do you have any literature recommendations on the state of
| the art in Gene Therapy 'delivery' mechanisms/apparatus?
| huytersd wrote:
| EBT-101 is an experimental procedure where they try to use
| viruses to excise the HIV retrovirus DNA from every cell in
| your body.
| smallhands wrote:
| language he invented and had no schooling.
|
| Last year, after moving to Spain, his family took him to a
| hearing specialist, who made a surprising suggestion: Aissam
| might be eligible for a clinical trial using gene therapy.
|
| Exploitation. I wonder how much this doctor get paid for this !
| formvoltron wrote:
| Was this via gene transfer?
|
| Any idea how far off we are from overwriting entire genes with
| known perfect copies?
|
| And if we can do that, then how much longer before we can
| iteratively overwrite segments until we've overwritten the entire
| genome in every cell with a perfect copy (derived by merging the
| sequences of many of our mutated cells)?
| verisimi wrote:
| I don't get this gene treatment at all. We are told:
|
| > The gene therapy consists of a harmless virus carrying new
| otoferlin genes in two drops of liquid that are delicately
| injected down the length of the cochlea, delivering the genes to
| each hair cell.
|
| You just squirt some medicine near the cells, and then the cells
| are sorted? This is no explanation at all.
|
| You could say, 'the cells absorb the protein and as the completes
| the bio-circuit, it is now activated correctly and the patient
| can hear'. Obviously I just made that up! But how does the thing
| work?
|
| Eg, if this was cancer and you operated to take the cancer out,
| fine - I get the principle. It if it's chemo, you insert some
| chemicals typically and it kills the cancerous growth, I get that
| too.
|
| But what is the gene therapy doing, at an engineering level? It's
| a mystery to me.
|
| Frankly, while I can understand a gene therapy working when
| treating a fetus, I don't get how it can work once the cells are
| developed and in place. I'm sure I'm dumb, but there it is.
|
| The whole article reads like a gene therapy promo, with no
| details that help understanding what the treatment is. You might
| as well say 'magic'.
| immersible wrote:
| It is funny that this supposed hereditary deafness is caused by
| what they call a "nonsense mutation".
|
| "Yasunaga S. et al. (1999) showed that the affected individuals
| in this family were homozygous for a nonsense mutation in the
| OTOF gene. "
|
| (retrieved in 2024/jan/24 from
| https://www.sciencedirect.com/topics/biochemistry-
| genetics-a...)
| tsol wrote:
| It's kind of amazing. The issue is that the cells have a
| dysfunctional copy of the OTOH gene, which leads to production
| of a dysfunctional otoferlin protein that does not work. There
| are virii that inject their DNA into cells. They modify one of
| these to make them inject a working copy of the OTOH gene into
| the cell. Cells have a mechanism where if there is a gene
| floating around nearby it will sometimes pick it up and
| integrate it. This is the process that is exploited in gene
| therapy to change individual cells to produce the correct
| protein. If enough cells produce the correct protein, there
| will be enough to have the desired effects. It's really
| unbelievably cool. I wish there were more appreciation for how
| these things work.
| verisimi wrote:
| Thanks for the explanation. That is amazing. What happens to
| the dysfunctional copy of the gene, I wonder? And what
| happens when the cell dies?
| HankB99 wrote:
| I am listening to a podcast on StarTalk Radio (Neil deGrassse
| Tyson) on gene editing. "Unlocking Gene Therapy with Gaurav
| Shah." If the subject interests, it might be worth a listen.
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