Cochlear implants and IUSM researchers help the deaf
journey from silence into the world of sound.

Playing It By Ear

The family's poodle -- G iGi - bounces into the living room, yapping at some chirping phantom near the back-yard playset. Grant Phillips leaps from his tiny rocking chair, rolls across the floor and throws a loving headlock around the dog. "You're noisy! Quit barking!" he commands with a giggle.

Not an unusual scene, a boy and his dog, both on edge to scramble outside and play. What makes it unusual is that the boy can hear all these sounds and is able to respond with normal speech. Grant, the son of Richard and Amy Phillips of Clermont, Ind., was born profoundly deaf. He is one of about two million Americans with profound deafness from all different causes, according to the National Institute of Deafness and Other Communication Disorders.

"We suspected Grant was deaf when he was as young as three months old," says Richard Phillips, a captain with the Speedway (Ind.) Police Department. "He responded to visual cues, smiled and laughed. But when we deliberately made loud sounds directly behind him, noises that would drive any child or adult to the ceiling, he didn't even flinch. When the diagnosis finally was made, our worst fears were confirmed. We weren't exactly sure how we would be able to help our son."

While physicians could not offer an exact cause for Grant's condition (neither of his parents has a family history of deafness nor of hearing loss), they knew the source of the problems lay within the inner ear and the fluid filled, snail-shaped cochlea, with its 16,000 hair cells. Normal functioning hair cells move in response to pressure changes in the fluid around them, creating an electrical current that is transmitted through the auditory nerve to the brain. When these cells are damaged or missing, sensorineural hearing loss occurs and, in extreme cases like Grant's, profound deafness results.

The Phillips were committed to finding help for Grant, who amazingly was teaching himself how to lip-read. The hearing aid he got when he was a year old was of little benefit. Then a chance conversation led the Phillips to the family of another boy who had been born deaf but whose hearing was significantly restored by a cochlear implant performed at Indiana University School of Medicine. The Phillips learned Grant might be a candidate too.

"We believe God intervened and showed us there was another path Grant's life could take, and it didn't have to be deafness," says Amy Phillips, an airport services coordinator with American Trans Air in Indianapolis. "That path led us to the IU medical campus."

There they met Richard T. Miyamoto, MD, chairman of the Department of Otolaryngology-Head and Neck Surgery. Dr. Miyamoto heads a team of otolaryngologists, audiologists and researchers who were among the first in the nation to perform cochlear implant surgery. The implants were approved for general use by the U.S. Food and Drug Administration in the mid-1980s.

Since 1979, Dr. Miyamoto and colleagues have performed more than 300 cochlear implants at IU Medical Center and James Whitcomb Riley Hospital for Children. Principal funding from the Indiana District of Lions Clubs International, Psi Xi Iota National Sorority, James Whitcomb Riley Memorial Association and Virgil T. DeVault, MD, a long-time friend of Indiana University, helped get their research off the ground.

Uncharted Territory

"We were venturing into uncharted territory but we were confident the procedure would work," Dr. Miyamoto says. "However, we knew there would be a range of performance and varying degrees of success with each patient."

When Grant was cleared for the implant, his parents were armed with the latest research and felt confident that surgical risks were minimal. "There was no reason for us to hold back, nothing to lose and everything to gain," says Amy Phillips, adding that she and her spouse were gratified and surprised to learn their health insurance provider would cover the cost of the procedure and the device. The average cost of a cochlear implant ranges from $35,000 to $40,000.

In 1995, at the age of sixteen months, Grant became the youngest cochlear implant user in Indiana and among the youngest in the U.S. at the time. (Since then, the FDA has lowered its recommendation from twenty four to eighteen months, but some patients have been younger.) Six weeks after the surgery, the cochlear implant device implanted in Grant's right ear was activated by audiologists at IUSM's DeVault Otologic Research Laboratory. Gradually, he began to process sounds and, with the help of auditory specialists and therapists, interpret their various meanings.

When Grant was two years old, his parents enrolled him in preschool so he could begin acclimating to the hearing world with children his own age. Last August, he entered kindergarten, where the only special accommodation made for him was to equip his teachers with a special microphone (that filters out much of the peripheral environmental sound) and speakers.

"The sooner prelingually deaf children can perceive sound, the better," says Karen Iler Kirk, PhD, director and coordinator of the DeVault lab's Cochlear Implant Program. "A child's first five years are critical to language and communication skills," says Dr. Kirk. "Our data show that if children receive an implant and rehabilitation at an early enough age, they should be able to develop language skills at the same rate as a child with normal hearing."

Wired For Sound

Though much of the focus of IUSM's cochlear implant research and clinical work is on children, cochlear implantation offers benefits for adults as well. Elizabeth Shiffman suffered profound hearing loss as a result of Meniere's Disease, which causes dysfunction of the mechanism that regulates production of inner ear fluids. Shiffman's hearing deteriorated over a decade, and hearing aids were not much help. Ironically, the loss forced the West Lafayette resident to give up her position as a speech-language pathology instructor at Purdue University.

"The first time I sought an implant, the doctors said I was not ready to benefit from it," she recalls. She continued to rely on her visual and auditory "memory" of speech and other sounds to help her communicate. By 1995, her hearing loss had become more profound and cochlear implant technology had improved. She was given the green light for implantation.

Like other patients seeking an implant, Shiffman first was taught what to expect. A cochlear implant is not a superamplified hearing aid and does not route sounds through usual hearing channels. Nor does it restore normal hearing. But with proper training, wearers can learn to perceive speech and environmental sounds.

The devices often lead to positive psychological and social benefits, particularly among the postlingually deaf, such as Elizabeth Shiffman. Prelingually deaf adults who have had a prolonged period of auditory deprivation tend to be more limited in speech perception and speech production post-implantation.

The six-week wait between surgery and the day her implant was activated was an emotional roller-coaster, says the British-born Shiffman. The first sound she heard was her own voice. She recalls that she and the medical staff burst into laughter when she blurted out, "Oh, my God. . . I have an English accent!"

Her perception of everyday sounds began to return, sometimes with a bit of confusion. Shortly after the implant was activated, she was walking in the rain. She heard a pattern of static, which she soon discerned was the sound of rain slapping against her umbrella. "At first, conversations were difficult," she remembers. "People's voices sounded like drunken frogs. It was a challenge to readjust, but voices now sound quite normal."

She still relies on her lip-reading skills and, in one-on-oneconversations, she often uses a hand-held microphone similar to the one used by Grant's teachers. In time she too, like Grant, mastered the use of the telephone, a rarity among implant users. "People who lose their hearing later in life and are fitted with an implant should remember it takes great patience to relearn to hear," Shiffman suggests. "Like a lot of things in life, progress comes slowly - one small step at a time."

The Sound And The Fury

Not everyone regards cochlear implantation as progress. The most vocal opponents suggest that implants are an invasion of the unique communications (signing and lip-reading) within the deaf culture. Some demand that implant procedures should be an individual choice of deaf individuals when they reach legal age.

The National Association for the Deaf (NAD) holds that it should have been consulted by the FDA when the agency first investigated implant safety and efficacy. NAD claims that, given the chance, it would have described the cultural advantages of remaining deaf. At a convention in 1996, the group voted to renew efforts to repeal the FDA ruling that approved implants for children. The following year, when reviewing clinical results in children using a new cochlear implant system, the FDA did allow for NAD arguments but subsequently approved use of the new system.

"I empathize with the concerns of many in the deaf community who are skeptical or opposed to the implants," says Shiffman, "but I don't think they have the right to exert their influence over parents, who ultimately must decide whether an implant is right for their children."

That same sentiment is echoed by the Phillips. "On one hand, I can understand why some deaf parents are opposed to implants if their child also is deaf, because that is a part of the environment in which the child will live," says Amy Phillips. "On the other hand, what I always ask hearing parents is why they wouldn't want an implant done on their child if it improves the quality of their life? In our case, we didn't want Grant to come back to us as a teenager and ask why we chose against an implant when it was an option early in his life."

An estimated ninety percent of deaf children are born into families where the parents have normal hearing. "I personally don't think there's going to be a great meeting of the minds on the issue of implanting children," says Dr. Miyamoto, who has served as a member of the advisory boards of the Indiana School for the Deaf in Indianapolis, Alexander Graham Bell Association for the Deaf, and on the advisory council of the National Institute on Deafness and Other Communication Disorders. "The real test perhaps is years away when these children become articulate adults and speak out on how cochlear implants affected their lives. My guess is that most of their opinions will be positive"

Important to grasp in any discussion about cochlear implants is that not all users realize the same level of success experienced by Grant Phillips and Elizabeth Shiffman. IUSM scientists are working on ways to improve results for all patients. Research related to implants, and language/ communication studies, have brought IUSM more than $9 million in federal, public and private grants.

The Hear Frontier

Mario A. Svirsky, PhD, associate professor of otolaryngology, is spearheading several studies to help the profoundly deaf. Chief among them is a study funded by a $900,000 National Institutes of Health grant to probe how implant users translate noise into understandable sound such as speech, even though they receive electrical stimulation directly to the auditory nerve instead of receiving an acoustic signal through their ears.

The other main goal of the study is to investigate the brain's ability to adapt to sensory distortion. The implanted electrodes cover only two-thirds of the cochlea and so do not stimulate all the neurons in the patient's ear. "This is the auditory equivalent of wearing glasses that distort the image of a viewer," Dr. Svirsky notes.

A biomedical engineer internationally recognized for his work with implants and neural prostheses, Dr. Svirsky has worked with Mohamed El Sharkawy, PhD, a Purdue University professor of electrical engineering, at Indiana University-Purdue University at Indianapolis and other researchers to develop a "real time" speech processor for cochlear implant users and auditory patients.

Another of Dr. Svirsky's collaborators is Ted A. Meyer, MD, PhD. Dr. Meyer is principal investigator on two projects to model speech perception and word recognition among implant users. The findings may shed light on why some implant users fare better than others.

IUSM physicians and researchers were the first in Indiana to implant electrodes directly into the brainstem. Still in its infancy, the procedure is for people whose auditory nerves and hair cells have been destroyed by tumors.

Together with research innovations from other institutions, these advances will provide continued improvement for the hearing-impaired. "Our research findings are integrated with procedures we use on a daily basis in clinical applications," Dr. Kirk says. "But our primary commitment is to provide top-notch service and timely care for the patients we serve."

What Counts Most

It's the little things in life that count the most. Now that she has regained her sense of hearing, Elizabeth Shiffman relaxes to the sounds of chamber and orchestral music. She travels extensively and recently returned from a six-week sojourn to Canada, where she learned to speak French. To her, the best sounds of all are the rippling and gurgling of a stream, the roar of the ocean.

Cochlear implant users also have a key advantage over hearing folks, muses Shiffman. "I can turn it off any time I like and shut out the outside world."

As for Grant Phillips, the sound of a passing train, thunder and falling rain top his hit parade of sounds. Kids, being the curious creatures they are, often ask Grant about the earpiece and wires attached to his headset. "I tell them it helps me hear them talk and to listen to music," he says, lightly touching the device in his right ear.

One thing, though: Will someone please turn down the volume switch on GiGi? She's yapping again, and there's a regular, little guy itching to get outside and climb the rope to his playset.