“When Opal could hear us clap our hands, it was incredible”. Jo Sandy, mother of little Opal, can’t believe it. This little British girl, born deaf 18 months ago in Oxfordshire (north-west of London), can now hear the surrounding world thanks to gene therapy which is in its first trials.
As recalled byAFP, this operation, carried out by the NHS, the British public health service, aimed to repair a genetic anomaly from which the little girl suffered: auditory neuropathy, caused by a disruption of nerve signals from the inner ear to the brain. This operation consisted of an injection into the cochlea (part of the inner ear) under general anesthesia.
” So happy “
Within four weeks of surgery, Opal was already responding to sounds. Then, after 24 weeks, his hearing had become almost normal for soft sounds, such as whispers. Now 18 months old, the little girl responds to her parents’ voices and can say words like ” dad “ Or ” Goodbye “.
“We were so happy when the clinical team confirmed at 24 weeks that his hearing was also picking up softer sounds and speech. »she added.
Spectacular results
“These results are spectacular and better than I expected”explained Professor Manohar Bance, of Cambridge University Hospital, the chief investigator of the CHORD trial, which began in May 2023. “This is, we hope, the start of a new era for gene therapies for the inner ear and many types of hearing loss.”he added.
As reported byAFParound 20,000 people in the UK, Germany, France, Spain and Italy are deaf due to a defect in a gene that produces otoferin, a protein needed by hair cells in the inner ear to communicate with the auditory nerve.
Precedents
Operations of the type that restored Opal’s hearing are planned or have already been successfully carried out around the world. In early 2024, the Children’s Hospital of Philadelphia announced that an 11-year-old boy “born profoundly deaf” heard “for the first time in his life” after gene therapy and four months after the operation, the child only has mild to moderate hearing loss.
