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posted by janrinok on Friday May 10, @08:43PM   Printer-friendly

Gene Therapy Restored Hearing in Deaf Child: [Paywalled in some regions]

Regeneron's gene therapy is being tested in a clinical trial.

An experimental gene therapy gave a deaf child the ability to hear, her family and investigators for a clinical trial say.

Opal Sandy, the girl, received an injection of DB-OTO, Regeneron's gene therapy, in her ear when she was 11 months old. Her hearing was assessed as normal within six months.

"When Opal could first hear us clapping unaided it was mind-blowing," Jo Sandy, the girl's mother, said in a statement released by the UK's National Health Service (NHS).

[...] "Providing the full complexity and spectrum of sound in children born with profound genetic deafness is a phenomenon I did not expect to see in my lifetime," Dr. Lawrence Lustig, chair of Columbia University's Department of Otolaryngology and one of the trial investigators, said in a statement.

Opal was born with genetic deafness from mutations of the otoferlin gene. About one in every 500 children are born in the United States with little or no ability to hear, and genetic changes like variants of the otoferlin gene are one cause.

Variants in the gene inhibit the production of a protein necessary for communication between the inner ear and the auditory nerve. The problem can be partially rectified with hearing aids or cochlear implants.

"It was our ultimate goal for Opal to hear all the speech sounds. It's already making a difference to our day-to-day lives, like at bath-time or swimming, when Opal can't wear her cochlear implant," James Sandy, Opal's father, said.

The DB-OTO is aimed at restoring the full spectrum of sound to people with mutated otoferlin gene. It's given as an intracochlear injection into one ear, or administered directly to the inner ear. A phase 1/2 clinical trial testing the gene therapy started in 2023, enrolling children in the United States, the United Kingdom, and Spain.


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  • (Score: 4, Insightful) by corey on Saturday May 11, @11:23AM

    by corey (2202) on Saturday May 11, @11:23AM (#1356538)

    I didn’t read TFA but i also saw this in my news reading earlier. But it’s a nice bit of good news in a sea of bad news.

    It must be pretty insane to hear for the first time ever. Like i imagine that the kid didn’t even have a sensation or perception of hearing or not being able to hear. Then someone does something and you get this new sense. I guess this is similar to Fred Hollows’ work in developing countries.

  • (Score: 1) by BenCollver on Saturday May 11, @07:08PM (2 children)

    by BenCollver (25395) on Saturday May 11, @07:08PM (#1356571) Homepage

    "Many Deaf people have the fear of seeing their language & culture eventually being wiped out. Most (abled) people see this as odd as deafness is a disability, and it’s better to not to be disabled....right? It’s not that simple. Many Deaf people love being the way they are."

    "This celebration of our identity is an act of resistance as many people believe it’s not something we should celebrate."

    "Often, I notice a pattern where people who are not Deaf making assumptions about how it'd be better to be hearing. This sort of thinking is what encourages a lot of hearing people who have deaf babies to do anything possible to make their babies 'hearing'. ... People have it so ingrained that it's better to be as 'normal' as possible and will choose to cure their deaf babies."

    Here is a link to a mock documentary on this subject titled The End and it is a bit of a thought experiment or at least food for thought.

    https://www.bslzone.co.uk/watch/zoom-focus-end [bslzone.co.uk]

    • (Score: 1, Insightful) by Anonymous Coward on Sunday May 12, @01:19AM

      by Anonymous Coward on Sunday May 12, @01:19AM (#1356603)
      The "cured babies" can make themselves deaf later if they really think being deaf is better.

      IMO thinking deaf is better is just copium.

      Now maybe if you have an "on/off" deafness on a timer or something, so you can sleep/code when it's noisy, then that might be better than the "norm".

      Yes I know some people drown out the noise with more noise, but that often makes your hearing worse in the long run.
    • (Score: 3, Insightful) by Spamalope on Sunday May 12, @01:53PM

      by Spamalope (5233) on Sunday May 12, @01:53PM (#1356656) Homepage

      Belonging to a subculture should be voluntary. The folks who love belonging to that community don't have to leave because they can hear, though the 'community' will have to be one that folks who aren't forced would like to be in. (also - this is treating all deaf folks as part of the same group/thinking of themselves like that - there will be "we're in the same boat" comradery but not a cultural monolith)

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