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Toddler born deaf is able to hear thanks to pioneering treatment

Opal Sandy

A baby girl who was born with profound deafness has been able to hear for the first time after undergoing pioneering therapy.

Eighteen-month-old Opal Sandy had severe hearing loss up until a few weeks ago, but a new form of treatment has changed her life.

Her older sister, Nora, who has the same genetic condition, is able to hear wearing cochlear implants - but the hearing devices could, one day, become a thing of the past.

The girls’ parents Jo Sandy,33, and husband James, also 33, from Oxfordshire were “gobsmacked” when their youngest daughter was able to hear without using a device despite being born deaf through CHORD gene therapy.

Mrs Sandy told PA news agency: “Although Nora and Opal passed the newborn hearing screening, which picks up the majority of deafness, when Opal was a newborn she went for the additional testing and we found out she was deaf when she was four days old,” she said.

“Nora had her cochlear implants on both sides fitted at 15 months old and following that comes quite an intense rehabilitation process of speech and language therapy and audiologist appointments.

 “She’s done really well learning to speak and has managed to close the language gap with her peers.

“So, hearing that Opal was deaf – of course there was a grieving process that we went through the same as when we found out that Nora was as well – but Nora had set the bar really high and we knew what was possible with lots of hard work and support from lots of people.”

The family heard about CHORD therapy from an ear, nose and throat surgeon at the John Radcliffe Hospital in Oxford who referred them on to Professor Manohar Bance, who was running a trial from biotech firm Regeneron at Addenbrooke’s Hospital in Cambridge.

“We were quite nervous to go down a different path to one that we knew had already worked so well for our eldest daughter. But it also sounded like a really unique opportunity,” Mrs Sandy explained.

“We sort of had a relatively reliable safety net that, even if it didn’t work, Opal would still be eligible to have a cochlear implant in her other ear six months down the line.

“So that was like a really good sort of safety blanket that we could fall back on if we needed to.”

Opal underwent the groundbreaking therapy last September, she was fitted with a cochlear implant in her left ear to ensure she could hear.

Mrs Sandy said: “It was about three weeks post-surgery, which was about a week after her implant had been turned on.

“So we were sort of in the routine of testing quite loud sounds like banging, clapping, wooden spoons on saucepans, that kind of really intermittent loud noise.

“I was testing that with her implant on and hadn’t realised that her implant had actually come off, and she turned to pretty loud clapping. When she first turned, I couldn’t believe it.

“I thought it was a fluke or like a change in light or something that had caught her eye, but I repeated it a few times.

“I picked my phone up and texted James, and said ‘I think it’s working’. I was absolutely gobsmacked. I thought it was a fluke.”

The family were initially told they could notice a change within the first six weeks - so it was a complete shock when Opal started to hear within three.

“I couldn’t really believe it,” Mrs Sandy said. “It was … bonkers, there is no way in a million years I thought that Opal would be able to turn to sound without wearing an implant”.

This February, six months after treatment, Opal could hear quiet sounds, such as a whisper.

“The audiologist played back some of the sounds that she was responding to and they were ridiculously quiet sort of sounds that in the real world wouldn’t catch your attention during a conversation,” Mrs Sandy said.

 “Without an implant, she can understand basically the same things that she can understand when it’s on, so ‘Opal, where’s your nose? ‘Where’s daddy?’ ‘Who’s at the door?’ ‘Bye bye’ …sort of basic language acquisition, that she can understand just as well with her implant on and off.”

Mr Sandy, who noticed a “massive” improvement between 18 to 24 weeks after surgery, said the “big moment” came when he realised his daughter could hear almost perfectly after six months.

Mrs Sandy continued: “Certainly since February, we’ve noticed her sister waking her up in the morning because she’s running around on the landing, or someone rings on the door so her nap’s cut short.

“She’s definitely responding more to sort of what we would call functional sounds rather than just sounds that we use to test her.

“We were told she had near normal hearing last time – I think they got responses at sort of 25 to 30 decibels.

“I think normal hearing is classed at 20 decibels, so she’s not far off. Before, she had no hearing whatsoever.”

Opal’s next milestone was when she started speaking within the past six months.

Mrs Sandy added: “I always said I’d never get annoyed with them making noise and I do get annoyed with them making noise.

“So, Opal loves playing with her little musical instrument set … playing the drums, playing her little piano, tapping some of her wooden blocks and things like that.

“She’s started becoming more interested in books, so like lots of lift the flap books, ‘where’s Spot?’…those kinds of really interactive books, she really likes.

“She loves slamming her cutlery on the table asking us where her dinner is.

“Nora got into music quite recently and (Opal) likes to put her arms up and does little dances in the kitchen.

“So they like dancing together. Nora likes reading to her, they like fighting, they like jumping off the sofa.”

[ Opal’s favourite words to say are ‘daddy’, ‘uh oh’ and ‘bye bye’. ]

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