Jan 2020 Ivanhoe Newswire

Researchers at Harvard Medical School and Boston Children’s Hospital have found a potential treatment for hereditary deafness, the same condition thought to have caused Beethoven to lose his hearing. The scientists are using a new gene-editing approach that they say could someday prevent profound hearing loss. Beethoven’s Fifth Symphony is a cornerstone of classical music. Hard to believe the composer was almost completely deaf from a genetic condition when he finished it.

David Corey, Ph.D., professor of neurobiology at Harvard Medical School, told Ivanhoe, “These children are born fairly normal, but then over ten or 20 years, they lose their hearing.”

Now, these aptly named Beethoven mice might hold the key to a potential cure. Scientists believe the animals have a defect in the same gene that most believe may have caused Beethoven’s deafness. “Our genome is composed of about 3 billion letters of DNA that together make up 20,000 genes. For the disease we’re studying, one mistake in the DNA in one of the genes causes deafness,” Corey said.

Researchers identified that a hearing gene called TMC one. It’s a gene that comes in pairs. Using a newly-refined gene-editing system, they disabled the defective copy of the TMC one gene, leaving the good gene in place. “By eliminating just the bad copy, that would be sufficient to preserve hearing,” Corey explained. The scientists then delivered the edited DNA back into the cells of the mice and tested their hearing. “We put little scalp electrodes on the back of the head, play sounds into the ear, and can measure the brain activity in response,” Jeffery Holt, Ph.D., professor of otolaryngology at Boston Children’s Hospital, said. Researchers said the mice were able to hear sounds as low as 45 decibels, the level of a quiet conversation. Corey told Ivanhoe, “This could be life-changing.”

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Here is a link to Deafblindness support and information.
They are based in Western Australia and supported by Senses Australia.

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