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August 6, 2005

From a silent world to the miracle of sound

Until May, 31/2-year-old Peyton Jones had lived much of his life in a world without the sound of his parents' voices or his own, where the monster trucks he loves to watch were powered by mute engines and his favorite cartoon characters never uttered an audible word.

"Peyton lost his hearing around age 2," said Dr. David Cameron, a Westerly ear, nose and throat specialist and Peyton's physician. "We don't know why."

His parents, Bob and Angela Jones of Westerly, recalled their moment of stark recognition of how little their son could hear when, several months ago, the three attended a monster truck show. While the rest of the crowd was shrinking back in their seats from the mighty roar, Peyton acted as though the auditorium was silent.

"When I looked over at him and saw him not responding to the sound at all, it just blew my mind," his father recalled as Peyton played with a few of his many toy trucks in the living room of the family's home.

At that point, the couple had taken their son through a series of hearing tests, eventually learning that the nerve endings in his ears were receiving almost no impulses and that he could not hear sound below 100 decibels. They had ruled out hearing aids and had decided that the only way to bring their son back into the world of sound would be to have a bionic device surgically implanted in his brain.

The incident at the truck show, his father recalled, came during the weeks they were waiting for the surgery. It was, for the couple, a concrete confirmation that they had made the right decision for their son.

That decision led them to Cameron and The Westerly Hospital, where Peyton was outfitted in his left ear with a cochlear implant — an electronic device that recreates the sensation of hearing within the brain for those who are totally or near deaf. Since then, he has been gradually re-entering the world of sounds and learning how to use his voice again.

"At first we were told his problem was speech,” said Bob Jones. “Juice was his first word, and then he wouldn't say it anymore. He would just point to it. Where we are now is that we're trying to get him talking again."

Already he's added "soda," "doggie" and "digger" — his name for a bulldozer or backhoe — to his vocabulary, reassuring his parents that predictions that he'll be able to be in a regular classroom by the time he's in kindergarten will come true.

"We've met a lot of people with implants lately, and you'd never know it," Bob Jones said. "They talk normally."

•••

The procedure represented not only a breakthrough for Peyton and his parents, but also for The Westerly Hospital. According to hospital officials, Peyton's was the first time implant surgery has been done on a child at any hospital in Rhode Island. Previously, patients would have to go to a hospital in New York or Boston. Before Cameron arrived a year ago, The Westerly Hospital didn't have a physician on staff who could do cochlear implant surgery on either adults or children. Cameron estimates he's done at least 20 implants on children over his career. Each one costs about $27,000 and lasts a lifetime.

"Kids are the best candidates for these," he said. "Children as young as nine months old are getting these. There's a lot of interest in getting them in as fast as possible so they can take advantage of the best time for early language acquisition."

The implant, Cameron explained, has four parts. Tiny electrodes surgically placed in the auditory area of the brain receive electrical impulses from a transmitter. A speech processor and a microphone fit around the outer ear. The devices have been perfected over the 20 years since they've become available, and now allow about 13,000 adults and 10,000 children in the United States to hear, according to the National Institutes of Health.

Peyton's parents, pleased with the results thus far, are weighing the possibility of eventually giving their son a second implant to enable him to locate sounds more easily.

"He's much more observant now," his mother said. "Where we notice it most is in the store. Before, he was in his own little world. Now he's looking around, trying to figure out where different sounds are coming from."

The sound the brain receives with an implant is different from normal hearing. According to Cameron, adults and teenagers who become deaf and then have the implants say voices have an artificial sound, “like a robot version of Donald Duck talking under water.” Still, compared to life without sound, cochlear implants are a godsend, he added.

"Many studies have shown that people who are deaf score well below people who are totally blind when they rate their quality of life," Cameron said. "Deafness tends to be much more isolating. That's the reason implants are so exciting for people who are deaf."

•••The surgery was just the beginning of the process. Seconds after the implant was placed in the brain and tested, Cameron turned it off. Immediately flooding Peyton with auditory stimulation would have been too confusing, especially while the surgery was healing and the boy was getting used to wearing the device around his ear. It wasn't until several weeks later that the implant was reactivated, when the Joneses took their son to the New England Center for Hearing Rehabilitation, in Hampton.

There, Peyton began twice-a-week sessions with speech and hearing therapists to learn how to talk and interpret sound.

"We're helping him attach meaning to what he hears," said Alicia Ayles, rehabilitative audiologist at the center. "Those of us with hearing never had to be taught how to listen. There's so much more beyond the surgery. This is where the work begins."

Ayles and another therapist at the center, Jennifer Cox, use interactive computer programs, puzzles, toys and their own sound effects to help Peyton learn that the "vroom" sound comes from a car, that's there's a difference between the "m" sound and the "n" sound, and that a train goes "whooo-whooo." Some of their work involves remotely reprogramming the computer inside the implant to emphasize certain sounds if they discover Peyton's having trouble discerning one from another.

He's also learning to use his own voice, sometimes babbling like an infant just testing out the sounds he can make and sometimes forming a new word, like "boat."

"Right now, he's just trying to listen to himself, getting used to hearing his own voice," said Ayles. “He'll move rapidly through those early stages, to where we can channel more of that babbling into real words. He's definitely learning to trust himself and his hearing more, and becoming more confident as a listener.

"It really is a miracle to go from no sound to hearing again."

By Judy Benson

Posted by 4HL on August 6, 2005 4:23 PM


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