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YOUR HEALTH: How mice are changing the lives of some deaf children

Researchers are using a cutting-edge way to treat a hereditary form of deafness linked to Beethoven

CAMBRIDGE, Mass. — 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.

"These children are born fairly normal, but then over ten or 20 years, they lose their hearing," explained David Corey, a neurobiologist at Harvard Medical School.

Now some 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 may have caused Beethoven's deafness.

"Our genome is composed of about three billion letters of DNA that together make up 20,000 genes," said Corey.  

"For the disease we're studying, one mistake in the DNA in one of the genes causes deafness."

Researchers identified that hearing gene called TMC-1.  It's a gene that comes in pairs. 

Using a newly-refined gene editing system, they disabled the defective copy of the TMC-1 gene, leaving the good gene in place.

"By eliminating just the bad copy, that would be sufficient to preserve hearing," Corey explained.

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," said otolaryngologist Jeffrey Holt said.

Researchers say the mice were able to hear sounds as low as 45 decibels, the level of a quiet conversation.

"This could be life-changing," said Holt.

The scientists say this research paves the way for using the new editing system to treat as many as 3,500 other genetic diseases that are caused by one defective copy of a gene. 

If this story has impacted your life or prompted you or someone you know to seek or change treatments, please let us know by contacting Jim Mertens at jim.mertens@wqad.com or Marjorie Bekaert Thomas at mthomas@ivanhoe.com.

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