In another trial at Addenbrooke’s NHS hospital, (Cambridge), Opal Sandy (18months) from Oxfordshire,  a girl completely deaf, after she was diagnosed with auditory neuropathy caused by disruption of nerve impulses going from her inner ear to the brain,  can now hear after having world-leading gene therapy.  

A working copy of the fault gene that caused it  was delivered by infusion through a tube into her right ear during surgery last September. Within three months, her mum Joy 33, realised Opal could hear clapping. “ I thought it was a fluke, or something caught her eye, but I repeated it a few times, I was absolutely gobsmacked”. She texted her husband James (33)  “ I think it’s working . Opal had a traditional cochlear implant in her other ear, so she would get some hearing if the gene op failed, but it was turned off”, Joy explained.

Now Opal enjoys hearing bedtime stories, with sister Nora, five,  who has two implants. Surgeon and trial leader Prof Manohar Bance called Opal’s results very spectacular, better than hoped  or expected, so close to normal hearing restoration”. The trial is by biotech firm Regeneron.

Cochlear implants  (electronic devices with a cable) have issues filtering out noise as multiple people speak and require intensive therapy to understand sounds.  Prof Bance said “ the Gene Operation is a one and done, therapy. So you have it and go back to your life”.