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Leia's auditory brainstem implant

Photo: Leia's story

Four-year-old Leia is a bright bundle of energy and is just starting to talk. Parents Bob and Alison use a combination of speech and sign to communicate with her as her speech begins to develop, but it’s been an emotional rollercoaster to get to this point. 

After Leia was diagnosed profoundly deaf, they investigated cochlear implants. “We were devastated, initially,” says Alison. “We felt confused and on our own.”

They were even more shocked when an MRI scan revealed Leia had inner ear abnormalities with no visible auditory nerves. As a cochlear implant works when sound is converted into electrical signals which stimulate the nerves in the cochlea and then travel along the auditory nerve to the brain, without these nerves the implants wouldn’t work.

“That was worse than being told she was deaf,” says Alison. “We’d been led to believe she could have them – she’d have hearing and she’d do really well.”

However, the audiologist suggested a new option that only a handful of children born without a cochlea or auditory nerves in the UK had tried: an auditory brainstem implant (ABI). “It was highly invasive,” says Bob. “There were only a few children in the world with one. Most had been done in Italy.”

“There was no guarantee something might not go wrong or when they switched the implant on that it would work."

As there was little data on ABI outcomes, it was hard to predict how much sound it could give Leia access to. Alison and Bob faced a difficult decision. “We familiarised ourselves with the process, the prognosis and the risks,” says Alison.

“It was invasive brain surgery, which is very different to a cochlear implant, and there was no guarantee something might not go wrong or when they switched the implant on that it would work.

"There could be side effects, like numbness in the throat and pins and needles in the arms and legs from the current that goes through to the brain. We thought long and hard, but we decided it was worth it to give Leia an opportunity to have a spoken language and some sort of hearing.”

Having made their decision, they contacted Italy, and were also referred to Professor Saeed at a London hospital. “Within minutes of meeting him we knew we wanted to stay in London,” says Bob.

“He put us at ease. It was then about funding, which was difficult, but we argued that the cost of an ABI is basically the same as cochlear implant surgery.” Fortunately, the family had a lot of support from their local MP.

“We weren’t sure what to expect.”

Leia was 22 months old when she had 12 electrodes implanted into her brainstem. “It was an emotional time because you’re aware of the risks,” says Alison.

“But she had an excellent team of experts, including people from the company that make the ABI. It was hard, but we felt we did the right thing.” They were pleased to be told the surgery was a complete success.

Six weeks later, the day before switch-on, Leia’s implant was tested but no response was received from it. “We were thinking ‘what have we done?’” says Bob. “That was probably the worst 24 hours of my life.”

“The switch-on was strange,” remembers Alison. “We weren’t sure what to expect because an ABI is never going to be as good as a cochlear implant.

They tried clapping and all sorts of things with Leia. She didn’t look like she was responding, but they said you could see it in her eyes and she was blinking. They were quite pleased, but we felt not much happened.

But on the journey home she responded to the tube doors, turning to look when previously she hadn’t. A couple of weeks later we noticed her turning for loud noises and that was the start of it.”

“At a party, when the music starts, she starts dancing."

Two years on, Leia is doing very well with all 12 electrodes fully functioning and no real side-effects. “She responds very well to high frequency sounds, so if you call her name from another room she comes. We’re starting down the road of spoken language."

Also now she shows interest in TV and music. “At a party, when the music starts, she starts dancing,” smiles Bob. “And she repeats sounds she’s getting from the TV.”

Leia’s hearing is now into the range expected by a cochlear implant user. “The experts didn’t think that would happen this soon, so they’re very happy with her progress,” says Bob.

But because so few very young children have had the procedure in the UK it’s difficult to judge. “There’s nothing to benchmark her against,” says Alison. “They don’t really know what Leia’s sound ‘sounds’ like to her.”

Bob and Alison advise other parents of children with no auditory nerves to be open to all options. “It feels quite daunting to talk about brainstem implants, and it isn’t for every family, but go and listen to what the experts have to say before you make any decisions,” says Alison.

“I like to think that Leia will have the same opportunities as any other child, whether she has a spoken language or not. We’d like to think we’ve given her the best possible start to achieve that.”