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Video: Watch this boy born deaf hear music for the first time

Scientific ResearchNational Institutes of Health
This little boy was born deaf. Watch him react to disco music

A 3-year-old boy who was born deaf has been caught reacting to music for the first time — and it looks like he’s ready to bust a move.

In the home video, Auguste Majkowski listens with a smile on his face to a 1977 tune called “Ma Baker.” When the music cuts off, he makes a tilted “T” with his hands – the sign for ‘again!’ – and he jumps to his feet when the speaker turns back on.

“It was amazing,” said Auguste’s mother, Sophie Gareau. Caught off-guard, the adults in the room took a few beats to realize they should probably capture the moment on camera, she added. “We were so awestruck.”

Auguste can now hear thanks to a device called an auditory brainstem implant. The youngster from Montreal had an electrode array surgically implanted on his brainstem in May and he’s been using it regularly since mid-June, when researchers with USC and Children's Hospital Los Angeles first turned the device on. [Great Reads: A Gamble for Auguste]

But since returning home, Auguste’s reactions have come in fits and starts. A beating drum or a barking dog might make his head turn, but not every time. 

That’s to be expected, said USC audiologist Laurie Eisenberg, one of the lead scientists. It’s going to take a while for Auguste to be able to fully process sound – and to figure out which noises are important. 

At first, these precious moments were so sporadic that even when Auguste reacted dramatically to a new noise, Sophie said, her husband Christophe Majkowski seemed to just miss them.

Christophe has been cautious in expressing his hopes for Auguste post-surgery, Sophie said. So for him, dancing to “Ma Baker” with Auguste marked a turning point.

“That was the moment where he said, ‘OK, this thing is working,’” Sophie said.

The results offer encouragement to Auguste’s parents, who flew to Los Angeles from Canada to give their son a chance to hear. And the progress is promising for the team of scientists whose NIH-funded clinical trial, if successful, could help set a clear precedent for the safety and efficacy of this procedure in the U.S.

The ABI device uses an external earpiece that can be worn rather like a bluetooth headset and an electrode array embedded in the brainstem. The earpiece processes the sound outside the ear and sends it to the electrode inside the skull, which stimulates a region of the brainstem related to hearing.

The implant doesn’t bring hearing up to the level of biological hearing. Sophie, who constantly chats with Auguste in Quebec Sign Language, says that all she really wanted for Auguste was reference sounds, such as the honk of an oncoming car to help keep him safe, and enough sound to help him interpret spoken words while lip-reading.

But she did admit that she held out a little hope that he might be able to appreciate music – which can be much more difficult for the brain to pick up than speech.

“If he can hear music, this is a miracle,” she said in an earlier interview before the surgery in May. “Music is so fantastic in life.”

The brain is geared to understand language, so surprisingly little sound information is needed to process it, Eisenberg said. Music, on the other hand, can require listeners to pick up incredibly precise sound information, she said – so its subtleties may be lost on children with ABIs, unless it’s something primarily rhythmic, like drums or rap.

Either way, it will be a long time until Auguste’s brain adjusts and can fully interpret the signal coming from the electrode array embedded in his head, said Eisenberg said.

“Think of him like a 3-month-old,” Eisenberg said – his sense of hearing was essentially "born" in May, which means there’s a long way to go before he can understand words, and perhaps more fully appreciate music.

Eisenberg spoke soon after learning that two other children who underwent the surgery after Auguste also performed extremely well when they were tested, she said. A total of 10 children will undergo the trial.

Follow @aminawrite for more fascinating science news. 

Copyright © 2014, Los Angeles Times
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