Massachusetts Health & Hospital Association

MEMBER MOMENTS: Children’s Noted for Gene Therapy for Deaf Children

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Boston Children’s Hospital was featured in the Boston Globe this week for its first-in-the-nation gene therapy treatment that is aimed at restoring a child’s genetic hearing loss by “bringing a working copy of [the patient’s] defective gene, OTOF, into the sound-processing part of the inner ear. A modified, non-infectious virus carries the gene into the inner ear cells, where it instructs the cells to make a missing protein needed to transmit sound signals.”

The article by reporter Brianna Abbott tracks the treatment, which requires surgery to deliver, in 16-month-old Everett Tynes.

“While a cochlear implant circumvents the parts of the ear that aren’t working, this one-time treatment restores the natural hearing process,” the Globe noted, quoting Dr. A. Eliot Shearer, a pediatric ear, nose, and throat surgeon at Boston Children’s who treated Everett. He was also an investigator on the clinical trial that led to the treatment’s approval.

“The weeks and months after we do the gene therapy, it’s like the sound level is just being slowly turned on,” Shearer said. “When I see these kids now, and they’re hearing, and they’re not wearing cochlear implants, even that is just amazing.”