British toddler born deaf can hear after gene therapy treatment | Good news

One-year-old Opal has never been able to hear because she was born with auditory neuropathy, a condition that disrupts the nerve impulses that travel from the inner ear to the brain. This can be caused by a defective OTOF gene.

Opal’s older sister Nora (5) has the same form of deafness and uses a hearing prosthesis. It does not amplify the sound, as a hearing aid does, but sends speech and environmental sounds directly to the auditory nerve via electrical stimuli.

The toddler from Oxfordshire was chosen, along with seventeen other deaf children from the United Kingdom, the United States and Spain, for a Cambridge University study. As part of the study, Opal received treatment just before her first birthday.

In an operation that lasted just sixteen minutes, Opal received a working variant of the OTOF gene through an IV in her right ear. This was done under anesthesia. She received the same prosthesis in her left ear as her sister.

Just a few weeks after treatment, Opal heard loud noises such as blows in her right ear. After six months, doctors at Cambridge confirmed that her right ear had near-normal hearing for soft sounds, even very soft whispers.

Her parents were “stunned” when they realized Opal could hear again, they say The Guardian. “I really couldn’t believe it,” her mother says. “It was crazy.”

“It’s great to see Opal react to sound,” says one of the researchers BBC News. The research team hopes that the successful treatment can also be used for other types of hearing loss.