Deaf Baby Turns Toward Loud Sounds After Treatment — A Remarkable Breakthrough in the UK

Opal Sandy, a British baby born with hearing loss caused by auditory neuropathy—a condition in which neural signals carrying sound to the brain are disrupted—has regained her hearing following gene therapy.
Image captured from The Independent’s YouTube channel

A British baby born profoundly deaf has reportedly regained her hearing through gene therapy.

According to reports from the BBC and other British media outlets on the 9th, 18-month-old Opal Sandy, who lives in Oxfordshire, England, was born with congenital hearing loss and was unable to hear any sound.

After participating in a clinical trial using gene therapy to treat hereditary deafness, however, she has reportedly recovered near-normal hearing.

British newspaper The Times reported that Opal is believed to be the first child in the world to have her hearing restored through this type of gene therapy.

Opal was born with auditory neuropathy, a condition in which the transmission of sound signals from the ear to the brain is disrupted.

The condition is associated with mutations in the OTOF gene, which produces a protein required for cells in the inner ear to communicate properly with the auditory nerve. An estimated 20,000 people across the United States, the United Kingdom, Germany, France, Spain, and Italy are believed to have this form of auditory neuropathy.

Opal’s parents enrolled her in a clinical trial led by Cambridge University Hospitals NHS Foundation Trust. The trial uses an advanced gene therapy approach that delivers a functional copy of the defective gene into cells of the inner ear, with the goal of restoring their ability to transmit sound signals.

In September last year, doctors surgically administered the gene therapy to Opal’s right ear, using a functional gene copy developed by U.S. biotechnology company Regeneron.

Opal Sandy, a British baby born with hearing loss caused by auditory neuropathy—a condition that disrupts the transmission of sound signals to the brain—has regained her hearing following gene therapy. Opal is pictured with her parents.
Image captured from The Independent’s YouTube channel

Remarkably, Opal began responding to loud sounds just three weeks after the procedure.

Her mother, Jo Sandy, 33, said, “When she turned her head toward a loud sound for the first time, I couldn’t believe it.” She later texted her husband, saying, “I’m so shocked. I thought it might have been a coincidence. I really can’t believe it.”

At a hearing assessment conducted in February, 24 weeks after the procedure, Opal’s hearing had improved to the point where she could detect even whispered sounds. She was also reported to have started speaking within the previous six weeks.

Manohar Bance, the ear surgeon and principal investigator of the clinical trial, described the outcome as “dramatic.” He noted that the treatment is designed as a one-time therapy, with the aim of allowing patients to receive treatment and then return to normal daily life.

Opal was the first patient enrolled in the trial, which is expected to include up to 18 young participants across the United Kingdom, Spain, and the United States.

Bance said, “This trial is only the beginning of gene therapy.”

[JoongAng Ilbo, Reporter Ha Soo-young]
Email: ha.suyoung@joongang.co.kr