While the Novartis trial was going on, Lustig and his colleagues were working on ways to restore hearing to people with genetic hearing loss. In 2019, Lustig and his collaborators showed that: Otoff Gene therapy could potentially be used to Restoring hearing in mice lacking otoferlin protein.Another group in Germany Similar survey results to be announced in 2021we show that the results are reproducible.
“For gene therapy to be effective, the target cells must be alive and not dead,” says Jeffrey Holt, professor of otorhinolaryngology and neurology at Harvard Medical School. He is researching gene therapy for hearing loss, but is not involved in the current clinical trial. . Many genetic mutations associated with hearing loss cause the death of hair cells in the inner ear; Otoff The mutation leaves these cells intact. “That bodes well for this strategy,” he says.
The trial is open to participants up to the age of 18, but Holt said it might be better to receive gene therapy earlier in life. “The auditory system goes through a maturation process, but if you provide this in the absence of sound at a stage after the system has already matured, it doesn’t really know how to process that new information input. “Hmm,” he says. . This is why adults have a harder time learning new languages than children. Children’s brains are highly plastic, allowing them to easily form new connections and learn new things.
Doctors recommend cochlear implant surgery by age 3 to help children learn sounds when they are still in the developmental stages of language, but older adults can also benefit from the device. .
Although the current trials are targeting a rare type of hearing loss, researchers from both companies believe that other genetic mutations that cause hearing loss could also be addressed with gene therapy.
However, not everyone believes that hearing loss requires medical intervention. Gene therapy is an extension of the debate that began in the 1990s over cochlear implants, said Jaipreet Virdi, a hearing-impaired historian of medicine, technology and disability at the University of Delaware. It states that it considers cochlear implants to be a threat to humanity. Deaf community. They argue that implanting deaf children before they have learned language denies them autonomy and access to deaf culture. “It’s problematic to eliminate the option before the individual, rather than the parent, is presented with the option,” Virdi said.
Wyatte Hall, a psychologist and public health researcher at the University of Rochester who studies the role of language acquisition in the health of deaf people and who is also deaf, is a hearing expert who has a deaf child. Some parents say they may consider medical intervention or technology as a way to help their child. Fits into the world they know. But Hall says deaf people contribute to the enrichment of society. “For as long as deaf people have existed on the planet, people have always tried to correct us,” he says. “The fact that we are still here suggests that there is still some kind of innate evolutionary value within us, and that our differences are important to us all. We are contributing to the world in which we live.”
While he’s not necessarily against cochlear implants or gene therapy, he doesn’t think parents should limit access to sign language in favor of a medical-only approach. When working with families of deaf children, he emphasizes a “both-hand” approach to using technology. and sign language. “If gene therapy or technology doesn’t work, sign language is a way to protect development,” Hall says.
