> A baby girl born deaf can hear unaided for the first time, after receiving ground-breaking gene therapy when she was eleven months old at Addenbrooke’s Hospital in Cambridge.
> Opal was born completely 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 and at 24 weeks confirmed close to normal hearing levels for soft sounds, such as whispering, in her treated ear.
> Auditory neuropathy can be due to a variation in a single gene, known as the OTOF gene. The gene produces a protein called otoferlin, needed to allow the inner hair cells in the ear to communicate with the hearing nerve. Approximately 20,000 people across the UK, Germany, France, Spain and Italy are deaf due to a mutation in the OTOF gene.
Very exciting progress being made to give the gift of hearing to people.
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> A baby girl born deaf can hear unaided for the first time, after receiving ground-breaking gene therapy when she was eleven months old at Addenbrooke’s Hospital in Cambridge.
> Opal was born completely 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 and at 24 weeks confirmed close to normal hearing levels for soft sounds, such as whispering, in her treated ear.
> Auditory neuropathy can be due to a variation in a single gene, known as the OTOF gene. The gene produces a protein called otoferlin, needed to allow the inner hair cells in the ear to communicate with the hearing nerve. Approximately 20,000 people across the UK, Germany, France, Spain and Italy are deaf due to a mutation in the OTOF gene.
Very exciting progress being made to give the gift of hearing to people.
This genetic condition [affects about one to two babies in every 10,000 born and occurs in about 1 in 10 children with sensorineural deafness](https://www.ndcs.org.uk/information-and-support/childhood-deafness/causes-of-deafness/auditory-neuropathy-spectrum-disorder-ansd/). This means that it’s going to be impacting 10% of people born deaf!
This makes me very happy 🙂 some rays of sunshine in a dark period