British Opal gets her hearing back with gene therapy

18-month-old Opal Sandy has regained her hearing after a unique ‘gene therapy’ operation. The girl is the first in the world to undergo the procedure.

Opal Sandy was born deaf as a result of auditory neuropathy, a condition that disrupts nerve impulses from the inner ear to the brain and can be caused by a faulty gene. Opal’s five-year-old sister, Nora, has the same type of deafness, but is helped by a cochlear implant that sends electrical signals to the auditory nerve, bypassing the defective parts of the ear.

It was a big decision for parents Jo and James, from Owfordshire, to have their daughter operated on.

– It was really scary, but I think we’d been given this unique opportunity, her mother said in an interview with the British state broadcaster BBC.

During the operation, the girl was given an infusion containing a copy of her missing gene. After the 16-minute operation, the girl was able to hear.

– I couldn’t really believe it, Opal’s mother Jo Sandy told The Guardian. It was … bonkers.

The operation is the first of its kind and was performed at Addenbrooke’s Hospital, part of the Cambridge University Hospitals NHS Foundation Trust. Other deaf children are now being recruited for the operation and will be followed for five years.

Ear surgeon Professor Manohar Bance says these early results from the operation are “better than I hoped or expected”.

– We have results from [Opal] which are very spectacular – so close to normal hearing restoration. So we do hope it could be a potential cure, he says, adding:

– There’s been so much work, decades of work.

A second child recently underwent a similar operation at Cambridge University with good results.

It is now hoped that this type of gene therapy could be used for other types of deafness, as half of all hearing loss in children has a genetic cause. Today, most people, like Opal’s sister Nora, receive a cochlear implant, a device that needs to be replaced several times. But it is important to have the surgery as early as possible, before the age of three, when the brain is more adaptable.

Now that Opal’s hearing has been restored, her parents have other problems to deal with. Opal has a new hobby: banging cutlery on the table as hard as she can to make as much noise as possible.