August 31, 2004
A quiet revolution
From: Portland Tribune, OR - Aug 31, 2004
22-month-old Aliyah Huget has never clearly heard the sound of her mother's voice.
Developing technology may be about to change that
By JOSEPH GALLIVAN Issue date: Tue, Aug 31, 2004
Of all the amazing things they do up there on Pill Hill (tinkering with stem cells, splicing mouse DNA, popping open the hippocampus), nothing beats making a deaf person hear.
On July 13, a toddler named Aliyah Huget is up at the Hearing & Speech Institute, for just another game of "find the cow" with her infant-family specialist. Behind the one-way mirror in a consulting room full of toys, Norene Kennedy Broyles gets down on her knees and enters Aliyah's muffled world. It's Broyles' job to monitor the 22-month-old's sign language, and her incipient speech. Such as it is; Aliyah can say some vowels — "aah," "oo," "ee" — but nothing that would get her very far in a sandbox of her peers.
Aliyah's parents have decided that they want her to talk and hear, and they've arranged their lives around her weekly visits to the institute. Today she's come with her father, Brian, who hears fine. Her mother, Tracy, arrives with a knock at the door, which Aliyah may or may not have picked up on her hearing aid. Tracy was born deaf, but she speaks, lip-reads and uses some signs.
Whichever way the child eventually communicates, one of her parents will be at a disadvantage. They've chosen to get her a cochlear implant so she can hear more and, hopefully, learn to speak. As they play together on the carpet, only Aliyah doesn't know that today is the last day with the therapist before she goes for her operation.
Even the newest hearing aids, like the ones Aliyah and her mother use, only amplify sounds in the hope that a damaged ear can pick them up. A cochlear implant is far more bionic. The human ear contains a bony whorl — the cochlea — lined with about 30,000 hairlike cells that pick up air vibrations — sounds — and translate them into electrical signals for the brain. If these cells have been clear-cut by some disaster or defect, a hearing aid may not help.
An implant has a rubbery strip that unfurls inside the cochlea. Stuck to it is an array of 24 electrodes. These, an external microphone and a speech processor do a crude version of the job of the hair cells: converting sounds into electrical signals that the brain can understand. (This is what saved Rush Limbaugh's hearing in December 2001.)
"Right now Aliyah can hear sounds in the range of about 500 hertz," Brian explains. "That's like the sound of bouncing a tennis ball on a wooden floor." Tracy elaborates: "When this is done she could be able to hear up to 4,000 hertz, and 25 decibels of sound."
This should be enough to hear human speech, most of which falls into the 4,000 to 6,000 hertz range. Never mind car horns and symphonies, this is what counts if this little girl is to be socialized.
Part of audiologist Marsha Owen's job is to know what deaf people can hear.
"Once the implant is in, she'll be able to detect sound at fairly soft levels," she says. "Speech has all sorts of fine-tuned differences — timing, intensity, pitch, etc. — that tell the brain things like the difference between an 'Sss' and a 'Zzz.' Hearing sounds is one thing, but making use of that information and attaching meaning is the next part."
She adds that stimulating the brain electrically doesn't create the same sound. "Some adults have told us that sometimes it sounds like a robot speaking. I've heard one person say, 'Your voice sounds like Donald Duck,' " she says.
Aliyah's parents are a little uneasy. They've gone over it dozens of times. How the surgeon will carve out a piece of their child's skull the size of a dime behind her ear and implant the electrode array. Magnets keep this in contact with the coil on the outside. The child can disconnect it whenever she wants. A processor is worn around the waist.
Brian and Tracy live in Washington County. He's a home inspector, she's a systems analyst for the Internal Revenue Service. Insurance covers most of Aliyah's implant. The hardware costs about $6,000. There are three or four main manufacturers of cochlear implants, one of them a corporation called Cochlear. They went with the Cochlear Nucleus 24.
"The nice thing about Cochlear," Brian says, brightening after all this talk about hertz and decibels, "what sold us on it, was the support you get through the company — it's kind of like Dell computer, they send you a new product in 24 hours, you don't have to wait" for a replacement.
Waiting for a sign
By Aug. 17, the incision behind her ear from the July 24 implant operation has healed enough that Aliyah can wear the unit. In the waiting room she plays bubbles and Slinky and peekaboo, effortlessly charming the staff. Although they've practiced putting on the harness that holds the processor, Brian isn't sure what to expect at the moment when his daughter will be able to hear. Brian and Tracy look to the experts for whatever cues they can.
Their surgeon, Dr. Sean McMenomey of Oregon Health & Science University's department of otolaryngology, has done about 500 of these implants. "He's a real serious guy," Brian says, adding that they had a tense four-hour wait before the surgery even began. "But when he came out after the operation, he was grinning from ear to ear."
The Hearing & Speech Institute is a nonprofit organization, independent of OHSU. It's been up on Marquam Hill since 1927. For surgery though, patients are sent across the campus to OHSU Hospital.
Today, another audiologist, Donald Plapinger, is switching on the processor and tuning it. He sits at a computer running a Windows program that checks how the 22 active electrodes and two grounding electrodes are working. He's already done this once before with Aliyah, when she was unconscious in the operating room.
"It took three minutes, but if we had to do the whole thing now it would take three hours, getting her to sit still." Plapinger is intense and matter of fact. He warns the parents not to expect too much. He has to balance the needs of the child and her parents with getting to his next appointment.
He starts implant recipients off at a very low sound level, so as not to scare them. "My idea of a good first day is when they come in wearing (the processor) the second day," he says.
Aliyah whimpers a little when it goes on, but soon she focusses on eating animal crackers and playing with a plush koala bear. It's wearing a Cochlear Corp. T-shirt and a Velcro patch behind its ear for a toy implant. Aliyah largely ignores the adults around her, who play, but with one eye on the monitor.
After a while she gets bored and pulls off the earpiece. Plapinger lets her keep it off while he shows the parents how to turn up the volume.
"Always start at zero and turn it up slowly," he cautions.
Implants aren't for all
Plapinger and Broyles confirm that the implant business is booming. OHSU does 60 a year now, and 60 percent of the hearing-impaired kids Broyles works with have them. Today babies can be diagnosed with hearing loss at 10 days, and as soon as they can keep them on, they get hearing aids and therapy. Tracy was 2 1/2 before her deafness was diagnosed.
Plapinger explains that many deaf people look down on implants.
"It's not so much now, but this was very, very controversial in the deaf community," he says. "The thought was implants would kill the deaf culture. The deaf culture is a very powerful culture, it is based on a commonality of American Sign Language, so if you can talk, you're not going to use ASL. If you're an oral deaf adult, like Tracy, you'd not be considered to be part of the deaf culture."
As an insight into the fractious world of the deaf, Plapinger cites the documentary "The Sound and the Fury" about familial strife when deaf and hearing adults marry.
"I see Aliyah as someone who was born to hear," he adds, definitively.
At one point Brian goes to put the unit back on the girl's head, forgetting to turn the level back to zero. It's the equivalent of jamming in the iPod headphones in the middle of a song, albeit at about volume level 2. Aliyah jolts slightly, then carries on playing.
The adults in the room immediately start analyzing: Did it hurt? Can she hear? Will she reject it? The father learns his lesson about checking the volume levels. They play for a few more minutes, then their time is up.
Afterward, Brian is noticeably relieved.
"The fact that she was leaving it alone is good. … I don't know, maybe deep down inside I thought maybe she was going to start talking right away, but it doesn't quite happen that way, does it?"
The next few weeks are crucial as her parents attend therapy sessions and try to teach her the meaning of the new sounds she is hearing.
Thanks to medical science, maybe one day they'll be able to talk about it.
© 2004 THE PORTLAND TRIBUNE