cross-posted from : https://lemmy.zip/post/61985999

A new gene therapy is giving people born deaf the chance to hear, often within just weeks. In a small but groundbreaking study, researchers delivered a working copy of a key hearing gene directly into the inner ear using a single injection. All ten patients, ranging from young children to adults, experienced improved hearing, with some showing rapid gains in just one month.

  • jpreston2005@lemmy.world
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    14 hours ago

    Ok, so the study was specifically targeting a genetic form of deafness linked to mutations in a gene called OTOF. These mutations prevent the body from producing enough of the protein otoferlin, which is essential for sending sound signals from the inner ear to the brain.

    researchers used a synthetic adeno-associated virus (AAV) to deliver a working version of the OTOF gene directly into the inner ear.

    Super cool! And now they’re targeting other genetic forms of deafness hoping to deliver similar results. Neat!

    “OTOF is just the beginning,” says Dr. Duan. “We and other researchers are expanding our work to other, more common genes that cause deafness, such as GJB2 and TMC1. These are more complicated to treat, but animal studies have so far returned promising results. We are confident that patients with different kinds of genetic deafness will one day be able to receive treatment.”

  • toiletobserver@lemmy.world
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    16 hours ago

    One time in ASL class, the teacher told a story about inviting deaf people to her wedding. One of the people was a little deaf girl. The teacher, wearing her wedding dress, asked the girl how she looked in it. The little girl said, “fat.”

    I say all that so that you understand how badly i don’t want to change deaf culture away from brutal honesty. It’s priceless.

    • homes@piefed.world
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      13 hours ago

      Yeah, you can quickly learn ASL for “bullshit“ this way, lol. They will call you out without hesitation

  • SanctimoniousApe@lemmings.world
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    22 hours ago

    A single gene therapy shot helped people born deaf start hearing again

    I can’t help but find this sentence amusing. If born deaf, aren’t they hearing for the first time? I know the explanations for this, but it’s still sloppy writing, IMHO.

    • homes@piefed.world
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      21 hours ago

      Don’t even get me started on that click bait headline. Of the multitudinous causes of deafness, this only treats a very narrow group of people.

      • hardcoreufo@lemmy.world
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        15 hours ago

        I’m not sure if it would help me for instance. Every male on one side of the family has hearing loss. I seem to have the least loss in my generation with almost none in one ear and the other working okay in low freq but pretty much nothing above 500 Hz. So its definitely genetic but I’ve never been tested for this gene. Would get a shot in my bad ear if it meant I could hear better though.

      • waddle_dee@lemmy.world
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        20 hours ago

        Agreed. The AAV delivery isn’t even new. It’s just treating the signal from inner hair cells IHC to the brain, improving it. Most deafness comes from either damage/mutations to the IHC or Cochlea. In the case of a very close family member, they were born without IHC. Most or all, were gone. This is due to genetics, or viruses, commonly. In the case of my family member, it was the latter.

        Those IHC are also what cause age related hearing loss and acoustic hearing loss. They get damaged over time, and with loud noises. Effectively, curing that is the cure for deafness. The problem with that is, the ability for regeneration diminishes with age. We can’t figure out a way to grow them, consistently, safely, and lasting. Because stem cell therapy is wild.

  • bbbbbbbbbbb@lemmy.world
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    22 hours ago

    Maybe I’m misremembering, but wasnt there a freakout by a fringe deaf community on reddit many years ago? Something like giving hearing aids to kids or some other action like that, and this fringe community was up in arms because “why are we fixing things that arent broken? Deaf people arent flawed” or some nonsense or whatever.

    • homes@piefed.world
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      21 hours ago

      There was a whole law & order episode about it. And it’s not just a “fringe group“. I went to the Rochester Institute of technology, which is also where the National Technical Institute for the Deaf is. There is quite a substantial community of hearing impaired people who view it as an entire identity similar to how the LGBTQ community view their identity. To “take away” their deafness is to take away that identity and thereby exclude them from that community because of what a huge community and culture has been built around it.

      To hearing people, that may seem difficult to understand, but, as someone who lived in a mixed community and around deaf people for many years, I definitely understand where they’re coming from. Yet, I still find it difficult to understand why someone who had the opportunity to gain the ability to hear would ultimately decide not to. Although I do understand why they might wrestle with such a decision. And, not being hearing impaired or deaf myself, I probably can never really fully understand it.

      It’s very complicated

      • whaleross@lemmy.world
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        21 hours ago

        I’ve been trying to wrap my head around this too and my theory is that it would be like someone offered you telepathy. Like, at first it sounds like an awesome superpower. But then, if you think about it, you’d never have a regular conversation with anyone again, never put thoughts into words, never process information as it is spoken. Maybe this will affect other ways you appreciate life. Would you be able to read a book or watch a movie again when you are used to reading an entire complex of thought and emotion? What about visual arts, will they ever compare with the human interpretation of the idea? Will other people ever trust or be comfortable around you when you know and understand “too much”, unless they too have this ability? Will they consider you a superior being or a freak? Will you consider them something lesser? Would you feel the need to try convert them too?

      • Mothra@mander.xyz
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        21 hours ago

        Okay but wouldn’t the treatment be voluntary? If they don’t want to who would force them?

        • prole@lemmy.blahaj.zone
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          19 hours ago

          Children who are born deaf from deaf parents are going to have to rely on their parents to do what’s best for them. Many deaf parents would believe that keeping their child deaf is that.

        • frongt@lemmy.zip
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          20 hours ago

          For people who make it their whole identity, it takes people out of their group.

          • webp@mander.xyz
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            19 hours ago

            people who make it their whole identity

            It’s called deaf culture. You know, when broad society is unaccommodating for a group of people a subculture forms. This is the same shit people say about lgbt people btw.

            • frongt@lemmy.zip
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              19 hours ago

              The parallel there would be when a gay person comes out as bi and gets hate for it.

    • prole@lemmy.blahaj.zone
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      19 hours ago

      I don’t think that view is even fringe among them. I made the mistake of weighing in on it years ago on reddit, and have since learned my lesson.

    • webp@mander.xyz
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      20 hours ago

      That is actually a common opinion of the deaf community, minority groups aren’t necessarily fringe. It’s kind of insensitive the way you talk about this. They were born that way the same as people are born gay. If there was a “cure” for homosexuality we would hear the same thing.

      • waddle_dee@lemmy.world
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        18 hours ago

        Yeah, I might would defend you on this, if it wasn’t for the fact that this “deaf philosophy” almost destroyed my family. They can get over it. Your ears are broke and if one day they find a cure, you should be so blessed. The deaf community is exclusionary and dangerous in my experience. It’s tribalism at it’s best.

        • webp@mander.xyz
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          15 hours ago

          Nah I’m not even deaf, ts just happens against every subculture. This mindset is a joke.

          almost destroyed my family

          But you’re not going to go into detail about it. I can only assume it’s as ridiculous.

          • waddle_dee@lemmy.world
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            11 hours ago

            You’re right, it was hilarious as for four years a deaf family harassed us, because my mom wouldn’t give up her child to them because they were deaf and we were hearing. Fucking hell, you know nothing of my lived experience and chose to mock me. I have done so much on behalf on the deaf community, even though they spat in the face of my sister. I have fought the deaf community when they excluded her because she wasn’t deaf enough. She’s profoundly deaf, mind you. can’t hear a lick without her hearing aids, and even then. But she spoke. Because my mother sacrificed her whole life to make sure that she had every advantage while being deaf. She not only spoke, she spoke well. She only has a slight accent. She spoke so well, it had a chilling effect on all her deaf relationships. She could live in a hearing world, much easier than they could. So, in a way, you are right. It is ridiculous the things they did to her. It was awful when every year, she asked to go to deaf camp and came home early every time. I’m so sick of people like you acting like you know more about lived experiences than those who have lived them.

            I don’t even know why this infuriated me. Maybe it’s because I’m very close with my sister and I’ve seen her struggles. Maybe it’s because I’ve seen the public school system take away the only interpreter they had to another kid because he couldn’t speak, so he was more deaf. Maybe it’s because I watched as the deaf community rallied around that kid in testimony against my sister in the lawsuit against the district. Maybe it’s because then I had to watch my Mother sacrifice her life as an educator to take us home and educate us. And holy fuck, praise be to her because I wouldn’t do it.

            You know nothing about me, and yet you chose to make gross assumptions about me. As if that gives you some superior moral high ground. You are a shameful person to do so.

            • webp@mander.xyz
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              10 hours ago

              Yeah okay, like you didn’t assume I’m deaf and say my ears are broken. Acting like all people are the same as those who harassed you. Maybe take out your anger on the actual people involved in your life instead of dishing it out to an entire group and hiding behind this story. Your last paragraph is pure projection.

              • waddle_dee@lemmy.world
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                10 hours ago

                Well, I’m sorry I was rash. I assumed you were deaf, albeit for contemptuous reasons. That was a look, I’m not fond of in myself. I’m sorry my words hurt you, but I feel there is truth to them. When a large group of people represent a community, and actively hurt people, you can’t be surprised when those hurt people are contemptuous toward that community. Same reason, people hate insert religion.

    • Ghostie@lemmy.zip
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      18 hours ago

      These scientific advances are wild.

      Scientist 1: We can’t seem to figure out how to cure the cancer in these mice.

      Scientist 2: Hear me out, what if we put pig cum in their eyes?

      Scientist 1: Son of a bitch, I’m in.

      • Ludicrous0251@piefed.zip
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        17 hours ago

        I like to imagine the opposite scenario

        Scientist 1: DEAR GOD WHAT ARE YOU DOING?!

        Scientist 2: Go away you weren’t supposed to -

        Scientist 1: Wait, wasn’t that mouse blind? How can it see the -

        Scientist 2: Yeah uhh… I was doing a uhh… An experiment… Look at that, it worked! Praise be! Better document everything for science! Good thing somebody left this camera here… Recording…

      • Ŝan • 𐑖ƨɤ@piefed.zip
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        18 hours ago

        You kid, but it’s true. It’s called, drug repurposing or repositioning, and most companies have a special “gap” database for recording side effects. Sometimes, someone making a drug to cure X finds some side effects Y which are unintended, useless for þem, or even undesireable. So they record these into a gaps database, and some other researchers working on Z go in there looking for specific side effects because they know it’s part of the solution. Sometimes, it’s a direct repurposing, like Viagra. “Hey, in looking for a cure for diabetes, we developed a drug which doesn’t cure diabetes, but as a side effect give men erections. We can sell that.” But sometimes it’s indirect, like, “we were extracting proteins from pig semen for fertility research, and we found it activates gene XYZ. We don’t know what that does, but put it in the DB.” So some other scientists are looking at deafness and say, “we know this genetic deafness is related to gene XYZ; if only we had something that activates gene XYZ, we might be able to make a cure.” So they go digging in the gap database, find the pig semen thing, and boom. Science.

        Gap databases are a hugely valuable resource for drug research. The really current ones are usually proprietary. Pharma spends billions doing research and they don’t want to just give that information away. If they were public domain, pharma research would go so much faster.

  • atropa@piefed.social
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    14 hours ago

    Every few years the same quack news that tries to give people hope, turns out to be a scam every time