Sounds of Silence
February 6, 2012 9:03 PM
State Journal Register
Ellie Tjelmeland had never heard her mother's voice.
At 18 months of age, she couldn't say mamma or daddy or speak her own name. Born profoundly deaf, Ellie had never learned to speak.
She wore the most powerful hearing aids available, one in each ear, but she could barely hear anything, say her parents, Joel and Michelle Tjelmeland (the name, Norwegian, is pronounced Chum-land). She could form sounds but wasn't learning to talk. Infants learn speech by listening to it, but Ellie could not hear the voices of her mother and father.
Now, thanks to her parents' determination and the skill of a team of doctors and speech specialists, Ellie can hear sounds, and there's an excellent chance she will learn to speak. The key to that transformation is a tiny device called a cochlear implant.
Hearing bad news
Exactly why Ellie is deaf isn't known, but her doctors suspect that the cause is congenital.
Her mother, Michelle, 25, also suffers from deafness that began in childhood. Michelle Tjelmeland wears a hearing aid in both ears, and her speech has a slurred, slightly buzzing quality, probably the result of poor hearing when she was learning to speak.
A former teacher at Franklin Middle School, Michelle gave up teaching after Ellie's birth. She and husband Joel, 32, suspected from the beginning that something was wrong with Ellie's hearing.
"I wanted her hearing tested when she was born, but for some reason that wasn't followed up on," Michelle remembers.
Figuring out how well an infant hears is always tricky even for professionals, but Michelle and Joel kept picking up cues that something was amiss with Ellie's hearing.
"She was so impervious to noise that it began to worry me," recalls Michelle. "Loud noises didn't seem to bother her at all. Nothing scared her."
Concerned, the Tjelmelands took their daughter, then 2 months old, for a hearing test. The results should have been reassuring. "They told us there was nothing wrong," Michelle says. A later test was interpreted as showing a mild hearing loss.
Despite the scientific testing, the sophisticated equipment, the assurances of doctors, the Tjelmelands weren't convinced. Why wasn't their daughter responding to their voices?
One day, Michelle recalls, she decided to do her own test of her daughter's hearing.
Not very scientific, perhaps, but very convincing.
From then on, Michelle and Joel were on a crusade - find out what was wrong and what could be done to fix it.
Hearing bad news
About four months ago, they took Ellie to Louisiana State University for hearing tests. These confirmed the Tjelmeland's worst suspicions. Ellie was profoundly deaf.
"They said she had essentially no hearing," Michelle recalls.
Specialists at Southern Illinois University School of Medicine fitted her for the most powerful hearing aids on the market. They took measurements, made molds of her ears and generally did everything they could to give her the best technology.
But getting an infant to wear hearing aids isn't easy. And as Ellie grew, they had to be refitted to her ears constantly. "It was very hard to fit her because she was growing so fast," Michelle says. "Every time she needed new shoes, she needed a new fitting."
Michelle Tjelmeland wanted her daughter to learn to speak normally, and with Ellie's level of hearing impairment, she feared that wouldn't happen.
"Even with the best hearing aids, she would never learn to speak," says Michelle. She and Joel kept searching for an alternative. Enter Dr. Michael Novak.
A Carle Clinic otolaryngologist - a surgeon who specializes in the ears, nose and throat - Novak is a short, slender man of about 45. His neat, trim appearance, small, well-manicured hands and controlled, measured way of speaking all seem to fit the mold of a surgeon.
Trained at Baylor College of Medicine in Texas, he moved to Urbana and joined Carle about 18 years ago. A few years later, he began working with a new, and then revolutionary technology for aiding the deaf, called the cochlear (coke-lee-er) implant.
The cochlea is a snail-shell-shaped organ buried deep in the ear. Inside it are tiny canals filled with fluid and lined with microscopic structures called hair cells. These are one of the keys to human hearing, says Novak, and they are the reason why the cochlear implant helps kids like Ellie to hear.
Vibrations transmitted from the eardrum pass through the bones of the ear into the cochlea. The vibrations stimulate the hair cells and they in turn stimulate the auditory nerve, explains Novak. Anything that interferes with that progression diminishes hearing.
"Ellie's hearing loss is probably congenital," Novak speculates. "Nearly one-half of hearing loss in young children is inherited."
Conventional hearing aids work by amplifying sounds. From the old ear trumpet to the latest digital models, they're basically an attempt to power sound past the blockage.
The cochlear implant, by contrast, works by stimulating the hair cells in the cochlea directly.
The implant itself is a slender thread of wire not much bigger around than a strand of spaghetti. Along its length are electrodes, which can transmit signals at different wavelengths, explains Novak.
The implant is connected to a central microprocessor - a computer brain - which picks up incoming sound waves, converts them to digital impulses and directs them to the implant's electrodes. The electrodes fire, stimulating the cochlear hair cells, which pass the impulses up the auditory nerve to the area of the brain that interprets sound.
But there's a daunting element to this technology. Ordinary hearing aids just sit behind the ear. A cochlear implant requires a surgeon to open the skull, uncover the cochlea and thread the implant into place. Novak has done the procedure hundreds of times; but the Tjelmelands have only one child. And she's only 18 months old.
Braving the fix
It is 6 a.m. Tuesday, Aug. 24. Michelle, Joel and Ellie Tjelmeland arrive at Carle Hospital in Urbana. They seem to have brought the whole family with them. Both sets of grandparents are in attendance as are Michelle's sister, Melissa and one of Melissa's college chums. Movie and still cameras are in evidence.
Michelle and Joel seem cheerful, but beneath the anticipation, both are tense. This is the first step toward a new life for Ellie, a life in which she will have both feet firmly in the hearing world. But the step is a big one. The only one who doesn't appear apprehensive is Ellie herself. Despite the strange and somewhat menacing atmosphere of the hospital, she's playful.
They take Ellie to a waiting room for same day surgery. Amazingly, if all goes well, the Tjelmelands will take Ellie home that afternoon. A nurse takes Ellie's vital signs, then squirts a dose of preoperative medication into her mouth. The Tjelmelands are keeping their own record of the day. They have a video camera and a digital still camera, and they take turns recording what's happening to their daughter. Every time the camera points her way, Ellie responds with a grin.
"With all this taping she's going to think it's Christmas morning," cracks one of the nurses.
"Well, it is," answers Michelle. "It's going to change the rest of her life."
Michelle and Joel get Ellie into a special gown that resembles a pair of pajamas. Novak's nurse, Shirley Sharp, breezes in to check on Ellie and gives her a tiny stuffed cougar with "Especially for Ellie, your Clarion cougar" written on it. Ellie plays with it delightedly.
Sharp has a quick reassuring word with both parents, then hustles off to scrub for the coming operation. Michelle and Joel take Ellie to a playroom while they wait for the medication to take effect.
After a few minutes, Novak makes an appearance. The surgery will take about an hour, he tells them. And don't worry, he says. "Just assume I'm doing fine," he tells them. "As soon as I'm done, I'll come out and find you guys."
Michelle muses on what it must be like to know how to do what Novak does. "How fun it would be to be able to change someone's life like that," she says.
Ellie crawls around the playroom, seemingly determined to touch everything at least once. Michelle and Joel never take their eyes off her. Michelle can't seem to touch her enough. Ellie looks at her aunt, Melissa, and points toward her mouth, sign language for "I'm hungry." Melissa shakes her head, "No." This close to surgery, Ellie can't have anything to eat or drink.
Ellie points to her mouth again and nods her head up and down vigorously.
She wants food.
Dianne Hammes, a speech therapist who has worked with Ellie for the past ine months and will be with her in surgery, talks with Michelle and Joel and via sign language, with Ellie. The Tjelmelands feel close to Hammes, who has a deaf brother.
"She somewhat understands what we were going through as parents trying to evaluate all the options," says Michelle.
At around 8:15, Shirley Sharp pokes her head around the playroom door: "We're ready," she says. Hammes takes Ellie in her arms and walks toward the operating room. Ellie is subdued; she's finally figured out that something is going on.
In the operating room, a frightened Ellie begins to cry. But the nurses and the anesthetist have done this many times. They place a mask over Ellie's face and tell her to breathe in and out. Within seconds, her cries have quieted down; she's unconscious. A tube in her windpipe connects her to a machine that will keep her anesthetized during surgery and provide her with oxygen.
By 8:30, Ellie is positioned for surgery and covered with a special blanket to keep her body temperature steady. In the background, the songs of Sheryl Crow's "Globe Sessions" form a melodic counterpoint to the mechanical beeping and hissing made by the medical machines.
Novak shaves Ellie's head; then using a marking pen, he plots a series of blue points in the skin behind Ellie's right ear - the arc of the incision he'll make.
There's sureness in Novak's movements that bespeak experience. He's done more than 200 of these operations. Kids as young as Ellie are unusual but not unheard of, he says. In fact, Novak has operated on a youngster only 9 months old.
"Carle is one of the big national centers for doing cochlear implants," he says. "We've been doing them for about as long as anyone."
Satisfied that he has marked the incision area precisely, Novak goes off to scrub for surgery. Sharp scrubs with disinfectant the area marked on Ellie's scalp, leaving a bright orange-brown swath along the right side of the child's head.
Novak returns and pulls a stool up to Ellie's head. At eight minutes before 9 a.m., his foot tapping to the beat of Sheryl Crow, he makes the first incision. A monitor relays the reassuringly steady rhythm of Ellie's heart at 134 beats per minute.
Novak's operating room technician, Mike Hammer, passes a steady stream of instruments to the surgeon. Novak and Hammer have done more than 150 of these operations together. Each is attuned to the other's rhythm.
Cutting through skin and subcutaneous tissue, Novak exposes the mastoid bone. A special cauterizing scalpel uses heat to seal off tiny blood vessels, minimizing bleeding and filling the surgery with the acrid smell of burned flesh. The mastoid bone looks honeycombed inside because it's filled with air sacs, Novak explains.
Using a special device that sounds remarkably like a dental drill, Novak shaves off bits of bone to get to the cochlea. Once it's uncovered, he drills a hole in it. Taking the implant, he threads it with a special tool into the inner labyrinth of the organ.
The device has 16 electrodes in it, he says. Electrodes will go into different regions of the cochlea and stimulate hair cells there in a variety of combinations, creating a range of sound perceptions, he says.
Adults who've had implants say speech at first sounds tinny and mechanical, like a robot speaking, says Novak. How a child Ellie's age will perceive the brave new world of sounds is anyone's guess.
Ellie will need a month of recovery before returning to Carle to get her implant hooked up to its computer brain, says Novak.
Novak shaves a portion of the mastoid bone into the shape of the receiver end of the implant, which will rest on that spot, just underneath Ellie's scalp. He also shaves a groove to carry the implant on its course down into the brain. On the outside of the scalp will sit a headpiece with a microphone in it. Magnets in both will hold the microphone in place. From the microphone, a wire will run to a microprocessor that Ellie will wear in a special harness.
The sound processor picks up environmental sounds such as speech and transmits them to the microphone, which passes them to the receiver below Ellie's scalp. It sends the signals along to the electrodes, which fire in sequence and stimulate cochlear hair cells. The result will be sounds that Ellie eventually will learn to interpret.
Novak takes a flap of tissue from the incision on Ellie's scalp and sews it in place over the hole he made over the cochlea. Then, at 9:40 a.m., Novak begins sewing the implant in place. At just before 10, he tests the device to make sure its electrodes respond. Everything checks out.
By a little past 10, Ellie is all sewn up and beginning to stir. Even as Novak closes the incision in Ellie's head, the anesthetist has been changing the mix of gases that she breathes, easing her return to consciousness.
The final touch is a bit of stuff called dermabond, sort of medical Super Glue. "It helps hold the incision together," says Novak. "As soon as her hair grows back a little, you'll hardly know we were there."
By 10:30, Ellie, her head swathed in gauze, is in the recovery room, with an oxygen mask clamped on her face. Novak walks to the waiting area where the Tjelmelands and family have waited nervously for a little more than an hour. "She's in the recovery room," he tells them. "She's waking up and doing fine."
The relief is almost palpable, and everyone begins talking at once, jostling to shake Novak's hand. There are tears in more than a few eyes.
"The implant is working just like it should," Novak continues. "Everything went just like it's supposed to. Now, it's just a matter of letting it heal."
Joel and Michelle follow Novak to recovery, where Michelle sits in a chair. A nurse places the still sedated Ellie in her arms. Michelle cries silently. Nurses bustle in and out, sometimes taking the child's temperature or blood pressure. Whenever anyone disturbs her, Ellie lifts her head and begins to cry. As soon as they leave, she burrows back down into her mother's arms.
She's very sleepy.
Nurse Amy Matthews takes out Ellie's intravenous line and gives Michelle and Joel some final instructions. "She may not be hungry," Matthews tells them, "and she may be a bit crabby. That's to be expected."
At 12:40 p.m., Joel Tjelmeland carries his sleeping daughter out to the family car. He and his wife return to Springfield. Phase one is complete.
In a month, they'll be back for phase two - when Ellie hears her first sounds through the implant.
A moment of truth
It's 9:30 a.m. on Thursday, Sept. 23. Carried in her father's arms, Ellie Tjelmeland enters Carle Hearing Services. Once again, the entire family is on hand.
If the operating suite is Novak's natural habitat, these offices with their soundproofed rooms and testing devices are home base for the audiologists.
Hammes works here, and she comes out to greet them and lead the way to the testing area. Ellie, her mom and dad, Hammes and another audiologist, Lee Ann Rotz, are crammed into a small room. Rotz works at a laptop computer, which she'll use to stimulate the electrodes in Ellie's implant. It's a test, to see if they're working and to observe Ellie's responses.
Novak is also on hand. This is a charged moment for him, too, the culmination of the work begun a month ago.
The atmosphere is loaded with high expectations. Ellie's grandparents and her aunt and a few friends cram into an observation room adjacent to the test area. Speaking with one of Ellie's grandparents, Novak does his best to tone down expectations a bit. "Some kids seem to respond right away, and some don't," he says gently.
Ellie has had some sound through her hearing aids, but chances are what she hears through the implant won't have any meaning, he says. It's going to be a learning process for all concerned.
Rotz plugs one end of a wire into the back of the laptop. The other end goes into the microprocessor.
With everything hooked up, Rotz pushes a key on the laptop to generate a pulse that causes an electrode in Ellie's implant to fire off. Instantly, Ellie looks up.
"Yes, that's good, beep, beep, beep," says Rotz, while signing to Ellie. The child returns to her playing. Each time Rotz stimulates one of the electrodes in the implant, Ellie freezes and looks directly at Rotz, who is watching her intently for signs that she's hearing. For close to an hour, she generates a series of pulses, stimulating electrodes at different frequencies and recording the results.
For all the technology, it's a subtle process, one that relies heavily on the observations and skills of Hammes and Rotz. Both watch Ellie carefully to see if she's hearing anything, all the while not trying not to give away too many cues as to what reaction they're looking for. Together, they've hooked up more than 130 children to cochlear implants. They've seen all kinds of initial reactions to the sounds, says Rotz. Some kids don't respond at all; sound scares some others, she says.
Ellie is doing well.
"She's having an excellent response," Rotz tells Michelle and Joel.
Once the initial testing is done, Rotz hooks up Ellie's implant to the microprocessor. No more computer-generated tones. The sounds Ellie hears from this point on will be the sounds of life going on around her, the sounds of her parents' voices.
Hammes turns the volume on the microprocessor way down. If it's too loud at first, it might well scare her. It does and Ellie starts to cry. Hammes quickly distracts her with a game and they lower the volume on the processor further.
They try again. Michelle calls her daughter's name, but Ellie doesn't even look up.
"I don't think we've ever had a kid turn to her name," says Rotz, trying to reassure her.
"I don't think she even knows her name," Michelle replies.
Gradually, Ellie adjusts to the sounds of the outside world. Over the coming weeks, her parents will adjust the volume upward, until she becomes acclimated to hearing.
Michelle and Joel know there is a lot of work to do. Ellie is way behind her age mates in learning spoken language, although she already knows more than 30 words in sign language. Ellie will keep coming to Carle in the coming year to learn to speak.
Ellie wears the microprocessor in a special harness that fits over her shirt. On it is a pouch with a Velcro flap to hold the calculator-sized
computer brain in place. A microphone, connected by wire to the microprocessor, sits tucked behind Ellie's right ear on a spot directly over the head of the cochlear implant. Magnets in both hold the microphone in place.
Michelle Tjelmeland faces some decisions about her own hearing, which is continuing to deteriorate. She has a hard time hearing the telephone. She says she's thinking seriously about having a cochlear implant herself in the future.
But for now, the focus is on Ellie. Initially, at least, they'll go back to Carle weekly for so called "mapping sessions" in which a computer programs the implant to Ellie's specific level of hearing loss.
For example, Ellie has no hearing at all in the high-pitched sounds, so the device is programmed to make up for that in different environments. It could take a year before the implant is fully adjusted.
It's worth the effort, Michelle says. "I want the first voice she hears to be mine saying, `I love you.'`
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