London, Thanasis Gavos

Doctors in Britain succeeded with a pioneering gene therapy to restore the hearing of a little girl who had been born whole deaf.

THE 18 months old Opal Sandy was born with auditory neuropathy which disrupted the ear’s neurological connection to the brain, preventing any sound intake.

It was, however, first patient worldwide which was submitted to a new type operationlasting only 16 minutes, which almost completely restored her hearing in the right ear.

It is believed that further improvement is possible.

The operation took place at Addenbrooke’s Hospital, Cambridge, with the chief attending physician, Professor Manohar Bantse, stating that things went better than he had expected. Rate the results «spectacularly».

Auditory neuropathy is caused by a defect in the OTOF gene, which produces the protein otoferrin which in turn allows cells in the ear to communicate with the nerve that carries sounds to the brain.

The operation, performed last September, simply consists of injecting a functional gene into the patient’s inner ear (cochlea) via a catheter. This is a treatment developed by the biotechnology company Regeneron.

Little Opal’s parents noticed positive signs at four weeks, with the improvement in their daughter’s hearing clearly noticeable 24 weeks after the operation.

Jo and James Sandy, 33, described her surprise and the emotion them when their daughter responded to sound tests without the aid of an external headset.

Now, they say, Opal enjoys banging cutlery on the table and her toys and enjoying the sound of them.

A second child in Britain with the same problem has already undergone the same treatment and is also showing positive results.

The clinical stage of the treatment involves operating on a total of 18 patients in Britain, the US and Spain. These patients will be monitored for five years, in order to fully establish the effectiveness and safety of the treatment.