Joy as 18-month-old Opal is able to hear (and dance with her sister) for the first time after being...

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Opal Sandy, aged 18 months, from Oxfordshire has had her hearing restored after becoming the first person in the world to take part in a groundbreaking new gene therapy trial.

The 18 month old received an infusion with the working gene to her right ear. READ MORE:A British girl has had her hearing restored after becoming the first person in the world to take part in a groundbreaking gene therapy trial.

Professor Manohar Bance, an ear surgeon at the trust and chief investigator for the trial, said the results were 'better than I hoped or expected' and may cure patients with this type of deafness. To overcome the fault, the 'new era' gene therapy – from biotech firm Regeneron – delivers a working copy of the gene to the ear.

Opal now loves slamming her cutlery on the table to make a noise and enjoys playing with toy drums, a piano and wooden blocks. Opal now loves slamming her cutlery on the table to make a noise and enjoys playing with toy drums, a piano and wooden blocks Auditory neuropathy can be caused by a fault in the OTOF gene, which is responsible for making a protein called otoferlin. This enables cells in the ear to communicate with the hearing nerve.

The Sandy family, from Oxfordshire, have an older daughter Nora, aged five, who has the same genetic form of auditory neuropathy as Opal and wears cochlear implants

 

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Oxfordshire toddler first in world to have hearing restored on new gene therapy trialEighteen-month-old Opal Sandy was born completely deaf due to the condition auditory neuropathy. Now her hearing is almost normal.
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