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August 27, 2004

Making a world of difference

From: The News & Advance, Lynchburg,VA - Aug 27, 2004

By Cynthia T. Pegram
Lynchburg News & Advance
Friday, August 27, 2004

Jason Quakenbush loves to talk to his younger brother Sam.

Although they are years apart, the boys share a special bond. They learned to talk at about the same time.

Profoundly hearing impaired, Jason was 3 when he had a cochlear implant; his little brother was 6 months old at the time.

The decision to go ahead with the surgery and implant was an agonizing one for the family - they researched, talked to experts, and got a second opinion at Johns Hopkins in Baltimore before they made the decision.

Jason, born in March 1999 with a life-threatening breathing disorder called pulmonary hypertension, was transferred to the University of Virginia Medical Center, where he was placed on a ventilator for five weeks and treated with nitric oxide, antibiotics, and other medications.

"You just thank God, that he came out of it - whatever they had to give him, that was fine," said Jenny Quakenbush, their mother.

Although he passed early hearing tests, "now that I have Sam, who has normal hearing, I know that Jason wasn't babbling enough sounds, but I didn't know that then. Jason would look out the window and say 'Buh, buh,' and we'd say 'Look - he's trying to say 'bird.' "

But at 15 months, she took him to a musical event where lots of other children were present - and they were hopping around and dancing. Jason wasn't reacting at all.

By the time he was 21 months old, his hearing loss was showing up as a profound loss," said Quakenbush. Hearing aids were prescribed, along with auditory-verbal therapy.

At first he improved, but it didn't continue. Ultimately they found out that his hearing loss was unusual.

Hair cells in his cochlea - snail-shaped structures in the inner ears that contain the organ of hearing - were fine, but they misfired.

"He had auditory neuropathy," said Quakenbush. "The message either doesn't get across or the hair cells fire the wrong message."

That is an unusual form of hearing loss, said Anne De Michele, senior audiologist and member of the UVa cochlear implant team. Hearing aids won't help - the problem is with the interpretation of sound.

"He could put three or four words together," said his mother. "No one could understand him except for me. And I could only understand him half the time. He would say 'I Ower' and that meant daddy is in the shower. He called himself 'Ah E' It was like a code ? you had to take it from the context and fill in the gaps."

In preschool, hearing children surged ahead in communication and understanding.

"You could tell he was having a rough time," his mother said.

Deciding on the cochlear implant was difficult.

"God gives an opportunity for a reason," said Quakenbush, "and that opportunity was sitting right there. We had to take it."

He did well with the November 2002 surgery. And a month later, when the cochlear implant was activated, "He cried," said his mother. "He didn't know sounds. I was so relieved because it was a reaction."

But his fear didn't last long.

"Jason just GOT it - he was so amazing. I think his adjustment period was three days."

With every sound, they would say, "Do you hear that? It's a dog barking. Do you hear that? It's the microwave. The door bell."

At Christmas, he was sitting on the floor, "and he said 'car' with a 'c' - I was totally not expecting that. I thought it would take three to six months."

His multi-channel implant was the newest technology of the time, but was briefly recalled when patients using it were found to have a higher rate of meningitis - giving the family a scare, and Jason a vaccination.

His teacher uses an FM system that broadcasts directly into Jason's cochlear implant. He wears a speech processor around his waist.

He's trained and wired to listen, said his mother. He goes to UVa once a week for special training and will be in kindergarten - mainstreamed. He undergoes auditory-verbal therapy training in addition to the implant.

"The processor is not a natural stimulation of the nerve," said De Michele. "We can't match that, but we're getting closer every day."

Some older patients don't like the implant, said De Michele, "and put it in a drawer."

But for children, said De Michele, "the younger you get an implant, the greater it can impact speech development."

When Jason got the implant, said his mother, "He wasn't really talking at that point, just speaking here and there," but his brother Sam had started talking a little early because of the family's emphasis on speech.

She loved what happened.

"It was so neat - here was the deaf child teaching the hearing child how to talk."

Cochlear implant changes teenager's life for the good

When Christi Krohn first heard words as others hear them, they were a lot longer than she'd thought.

Now, she said, "I hear more sounds."

Born nearly deaf, Christi was fluent in sign language. An excellent lip reader, her skills were boosted by hearing aids that gave her the sense of sound, but not the fullness of its texture.

Christi, now 18, entered the hearing world in 2002 when she received a cochlear implant, one of about 50,000 in use in the United States.

"It literally is a 'Bionic ear,'" said her mother, Frieda Krohn.

Cochlear implants were developed in the 1970s, but by the mid 1980s the technology began to flourish and lead to the systems now available. In simplest terms, the device is made up of an internal and an external component. Sounds are picked up by a microphone and sent to a speech processor, which turns the sounds into codes. The coded signals are sent to the internal implant, which converts the code to electrical signals, which stimulate the nerve fibers. Those signals, ultimately, are recognized as sounds by the brain, and produce a hearing sensation.

"It is a real technical process," said her mother. The processor is re-programmed periodically because her hearing is constantly changing as her brain accepts the onslaught of new data.

"She's hearing things she never heard before, and so her brain has to determine what those sounds are. And as that happens, she's picking up more and more. And it has to be changed so that she can accept more sound and have all that translated."

Christi, now a Virginia Tech freshman, was a senior at Jefferson Forest High School when she got the transplant. Her audiologist encouraged her to take the step. And now there is no turning back; once the electrode goes in, the old way is gone.

"I began thinking about it when I was a sophomore in high school," said Christi. "But I wasn't sure. I just kept reading. There were insurance troubles - it took quite a while."

She went to the University of Virginia for tests.

"Before they gave her the major test," said her mother, "she sat down with the surgeon, and they were kind of giving her the worst case scenario: You're older, you do OK with your hearing aid, you read lips really well. Mechanically it's a lot of work. What do you want to get from it?"

"I really want to talk on the phone," she said.

She had the outpatient surgery at UVa - it took about an hour and a half to implant the electrodes in the cochlea.

Anne De Michele, senior audiologist and member of the UVa Cochlear Implant Team, says implants can be done in children about 12 months old - about half in use are implanted in children - "straight through to older folks" in their 70s and 80s.

Not all people succeed with cochlear implants.

"If they've been using a hearing aid, and have hearing-acquired language, it is an easier transition," said De Michele.

Christi, despite her deafness, was a good student, a hard worker and an achiever in a hearing world. And she had a supportive family. (Her dad, mom and older siblings learned sign language and used it at home. "We don't use that anymore," said Christi. "Sometimes my mom gives a sign, and I'll say, 'Mom, I can hear you fine - no need to sign.")

Christi "was a tremendously good hearing-aid user," said De Michele, and was extremely successful. She was also motivated and wanted to use an implant - and it takes work and commitment to use it well, said De Michele.

"She has had a wonderful transition, and does wonderfully well with it."

Four weeks after the surgery, when healing was complete, Christi returned to Charlottesville to turn it on.

"It was awful at first," said Christi. "I was scared, because I didn't know what was going on."

They had been warned it might take a while to get used to, and sounds were high-pitched and sharp.

"It was hurting my ears," said Christi. "I didn't know what was going on around me."

"It was very scary," said her mother. "For the first 15 minutes we weren't sure this was a right decision ? we were in a daze - then Christi started picking up on sounds. Our ride home from Charlottesville was so much fun. ? A car drove buy, its tires squeaking. 'She said, what is that?'"

She heard her shoes as her foot moved.

"My shoes make a sound," she said to her mother. "Everything around her was opening up. She was getting excited, which was very helpful to us. We were realizing 'Yes, she is hearing everything.' In the car, she started pushing buttons, opening windows, slamming the door. We were laughing and talking, she was telling us all the different sounds she was hearing."

At school, other kids wanted to see her scar and were curious about the implant. And it was a different world for her.

She heard things others didn't - they'd learned to tune out things like the whoosh of air conditioning or the tinkling of ice in a glass of tea, the amazing noise of a football game, the sounds of people clapping.

"Everything had a sound. I couldn't believe it," said Christi.

Always a verbal person, even though deaf, the spoken word changed for her.

"Everyone sounded like ducks to me, quacking. It was because I never heard a voice really clear before," she said. "Now everything sounds normal."

Not too long ago, Christi called her mother from college. They had a 20-minute conversation, "and she understood it all."

And yes, now Christi does have her own cell phone.

Contact Cynthia Pegram at or (434) 385-5541.

© 2004 Media General Inc.