A baby girl born completely deaf can hear for the first time after receiving ground-breaking gene therapy. Opal Sandy from Oxfordshire was eleven months old when she was treated at Addenbrooke’s Hospital in Cambridge. She is the first British patient and the youngest child to receive this type of treatment.
Opal was born deaf because of a rare genetic condition, auditory neuropathy, caused by the disruption of nerve impulses travelling from the inner ear to the brain. Within four weeks of having the gene therapy infusion to her right ear, Opal responded to sound, even with the cochlear implant in her left ear switched off. Clinicians noticed continuous improvement in Opal’s hearing in the weeks afterwards.
At 24 weeks, they confirmed Opal had close to normal hearing levels for soft sounds, such as whispering, in her treated ear. Now 18 months old, Opal can respond to her parents’ voices and can communicate words such as “Dada” and “bye-bye.” Opal’s mother, Jo Sandy, said: “When Opal could first hear us clapping unaided it was mind-blowing.
We were so happy when the clinical team confirmed at 24 weeks that her hearing was also picking up softer sounds and speech. "The phrase ‘near normal’ hearing was used and everyone was so excited such amazing results had been achieved.”
ATR COMMENT: We know there will be mutterings this is a one-off success over a rare deaf gene, but ATR welcomes the brilliant work of those dedicated professionals attacking this pandemic of deafness and hearing loss. We know certain deaf areas oppose intervention, but today nobody is going to listen to them. Today one gene is addressed tomorrow maybe more, at least something positive is beginning to emerge, after years and years of 'breakthrough's' that never manages to.
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