BY HOLLY STRAWBRIDGE

Some evenings after Sherra (B.S.E. ’85, M.S.E. ’92) and Todd (A.B. ’84, J.D. ’89) Payne tuck in their eight-year-old son and turn out the lights, they spend a few minutes talking about things important to little boys. They treasure this intimate time, not only because they love their son, but because they are thrilled that Austin can hear their voices in the dark. The happy, talkative second-grader was born profoundly deaf. But because of a world-renowned cochlear implant program at the University of Miami Ear Institute, Department of Otolaryngology, Austin now can hear.

Cochlear implants enable thousands of men, women, boys, and girls with profound nerve deafness to function as hearing members of society. Unlike hearing aids, which merely amplify sound, cochlear implants take over the function of the cochlea, the portion of the inner ear that translates sound waves into electrochemical signals that can be decoded by the brain, where hearing actually takes place. Since the devices were approved for adults in 1978 and children in 1990, advances in design and surgical technique have reduced complications and expanded the conditions of deafness that succumb to artificial nerve stimulation. As a result, many adults, teenagers, and children born profoundly deaf are able to understand spoken language and even enjoy music with a cochlear implant.

The earliest devices were reserved for adults who had lost their hearing and did not benefit from a hearing aid. They utilized a single electrode that enabled the wearer to hear sounds, but not necessarily distinguish them. They bear little resemblance to the computerized implants used today. “Multichannel cochlear implants are the most sophisticated neuroprostheses ever developed. They enable two-thirds of patients to understand speech without visual clues,” says Austin’s surgeon, Thomas J. Balkany (M.D. ’72), director of the University of Miami Ear Institute and chairman of the Department of Otolaryngology at the University of Miami School of Medicine.

“Just three decades ago, on the basis of experience with the cochlear implants available then, the possibility that a patient would someday be able to understand words without lipreading was nearly unthinkable,” adds Balkany, who is the school’s Hotchkiss Professor and Chairman of Otolaryngology and professor of neurological surgery and pediatrics.

Such rapid development in a relatively short time is the direct result of research done at the University of Miami and other sites where scientists, educators, and bioengineers, working in tandem with manufacturers, have created effective computerized devices that transduce incoming sound waves into coded electrical signals and deliver them to the cochlear nerve for transmission to the brain.

The University saw the potential of cochlear implants early and recruited Balkany, already a noted cochlear implant surgeon and researcher, from Colorado in 1990 to build a clinical and research program. And build it he did. Today, the cochlear implant program at the University of Miami Ear Institute is one of the busiest and most comprehensive in the nation, having restored hearing and provided language therapy to more than 400 deaf children and adults. The program is comprised of three surgeons, four audiologists, and an auditory-verbal therapist who work closely with psychologists and teachers of the deaf from the Debbie School at the University of Miami’s Mailman Center for Child Development.

The extensive staff is necessary to evaluate, treat, and teach pediatric patients, most of whom have never heard a single sound. “Speech performance and language acquisition are primary benefits of the cochlear implant in children. Children who receive implants at a younger age speak more accurately than those who wear hearing aids or use vibrotactile devices,” says Annelle Hodges, director of the Division of Audiology at the School of Medicine. “After implantation, speech intelligibility continues to improve over time.”

The benefits are enormous, but so are the obstacles. Simply determining a child’s degree of hearing loss or response to hearing aids is a difficult task. After the implant is installed, finding its optimal sensitivity and comfort level are equally challenging. In order to benefit from an implant, a child who never has heard requires intensive long-term training in listening and speaking skills. Additionally, parents and teachers must be educated in order to assist in the acclimation process.

“Parents must be fully cognizant that repeat visits to the implant center for programming and evaluation, months (even years) of therapy, and ongoing efforts to obtain adequate services from the school are necessary commitments assumed by the whole family,” says Balkany.

Adult patients are much easier to treat, because the implants are restoring a lost sense rather than creating a new one. In order to qualify for a cochlear implant at the University, adults must have had some degree of hearing before age three and worn hearing aids most of their life. Adults and teenagers who were born deaf generally derive less benefit from the device unless they have learned to rely on communication through lipreading and speech, rather than signing.

Nevertheless, a cochlear implant can make a tremendous difference in an adult’s ability to earn an income, enjoy the company of hearing people, or raise a family (see sidebar). “Psychological studies have shown decline in loneliness, reduction in depression, increase in self-esteem and independence, reduced isolation, and improved job opportunities,” says Hodges. “In other words, a better quality of life.”

Balkany and his team are highly sought-after by technology manufacturers from the United States, Europe, and Australia who are eager to develop new devices. “The University has been involved in clinical trials of virtually all cochlear implants tried, usually as one of several centers nationwide,” he explains.

Through the years, implants have evolved from a single channel to devices that handle eight to 22 channels—plenty to accommodate the majority of sounds in our everyday world. More discreet stimulation has resulted in better hearing and rendered the electrodes less damaging to the cochlea.

In addition to technology development, the emphasis at the University includes outcomes research, the study of how well an implant works on different types of patients. At any given time, a handful of trials are ongoing. One is evaluating the results of implantation in patients whose cochleas have developed incorrectly or become filled with bone. Another is comparing the development of babies who receive an implant at 12 months with those implanted at age two, the current norm.

On another tack, vocal opposition to cochlear implants by the Deaf community in the 1990s led Balkany to conduct extensive research into the ethics of transplantation. His research, now concluded, was instrumental in calming many of the fears and helping many families, like the Paynes, make an informed choice.

The Paynes followed the cautious path many parents take before deciding whether a cochlear implant is right for their child. When tests performed on Austin at the age of seven months suggested he was deaf, he was referred to the University of Miami School of Medicine. There, more sophisticated auditory brain-stem tests confirmed he had been born without the ability to hear. The baby was then fitted for hearing aids. “He was eight months old and wearing the same aids as an adult. They dropped off, or he pulled them out. Eventually, we went to smaller aids, which were more tolerable,” Sherra Payne recalls.

For three years, the Paynes took Austin three days a week for auditory verbal therapy. Discouraging results led Balkany to suggest that Austin might benefit from a cochlear implant at age two. The couple hesitated. “We weren’t sure an implant was the best answer. We wanted him to have it only if he wasn’t successful with hearing aids,” she says.

Time passed. The Paynes knew their son was bright, but he was unable to reach his potential as he struggled to use spoken communication. “We were encouraging him to be auditory, so we needed to give him the tools to help him hear,” she says. “However, the time frame was not right for our family.”

Then at age three, Austin broke his leg. For eight weeks, his mom sat with the captive toddler playing language games. At the end of the period, he had learned little. The Paynes realized their son had gone as far as he could with hearing aids. Intensive schooling, sign language, or cochlear implants remained their only options. The Paynes chose to proceed with implantation.

In May of 1996, Balkany performed the procedure. Three weeks later, after Austin had healed, the external radiotransmitter was hooked up to its antenna and programmed. For the first time in his life, Austin heard sound. “This was a difficult day,” Sherra Payne recalls. “Austin was scared. We realized he had not heard much, if anything, before the surgery.”

For two days Austin was unwilling to wear the implant. Gradually, he got used to wearing the device on low volume. Its sensitivity was increased a little bit every day until it reached optimal sound a week later.

It did not take long for the inquisitive little boy to realize that sounds meant something. Only a day or so later, Sherra Payne was in the back of the house when Austin’s younger brother began to wail in his high chair in another room. “Austin started telling me the baby was crying by pointing to his ear and crying with gestures,” she says. “Then he went to see if his brother was okay.”

Six months later, Austin was making sounds that resembled words. “He grew by leaps and bounds,” she says. “We could hear things change in his voice, or he would learn a new word. Eventually, he told us what he wanted through his words, not by gesturing or taking us places.”

Today, Austin’s speech is essentially like that of any eight-year-old child. He is at the top of his class in a mainstream school, has plenty of friends, and enjoys all the benefits of being a carefree second-grader. “There have been times of progress and times of plateau, but we have no doubt we did the right thing,” says Sherra Payne. “He’s a normal little boy.”

Because of its research—and positive outcomes like Austin’s—the University’s cochlear implant team has earned a reputation as one of the most outstanding programs in the world. As a result, the team receives many of the most challenging cases, and surgeons flock to the University to receive training.

Despite the success of cochlear implants, research at the University may eventually render them unnecessary. Balkany’s team is working on regeneration of the tiny hair cells found in the inner ear. Because sensorineural deafness is caused primarily by the loss of these cells, which are the neural receptors of sound, the ability to replace damaged cells would mean an end to deafness. “In the next 20 years we hope to be able to restore some degree of natural hearing,” says Balkany.

Until that time comes, cochlear implants remain an excellent option.

Holly Strawbridge is a frequent contributor to the University’s alumni magazines. Photography by John Zillioux.

Cochlear Implants: Hearing Both Sides

erhaps no device aimed at correcting a defect has engendered as much debate as the cochlear implant. Some members of the Deaf community have viewed the devices as miracles of modern medicine, while others have seen them as demeaning or threatening to Deaf culture.

Thomas J. Balkany, chairman of the Department of Otolaryngology at the School of Medicine, recognizes logical arguments for both sides of the issue and has published extensively on the ethics of cochlear implantation. He feels strongly that the decision should be made by the parents alone after careful consideration of their alternatives. This means both sides must disseminate accurate, truthful information that can be weighed with the child’s—not the Deaf society’s—best interests in mind.

“The American Deaf community sees their lives as emotionally fulfilling, promising, and independent. Raising children with sign language is a rational option,” he says. “The Deaf culture is rich and diverse. Deaf people have lives much like hearing people: they attend parties, date, marry, have families, and raise children.”

On the other hand, the arguments for giving a deaf child the ability to hear are powerful. A survey conducted in California and published in 1993 showed that deafness was associated with the lowest educational level, lowest family income, lowest percentage employed, lowest percentage holding professional or technical jobs, and the poorest feelings of self-worth among people with all kinds of disabilities.

The facts are that the average culturally deaf high school graduate reads at a third- or fourth-grade level. Seventy-five percent of deaf or hard-of-hearing people cannot read a newspaper when they finish high school, which occurs at the average age of 21. Because of these educational limitations, a large percentage of deaf adults have difficulty finding adequate employment and are forced to rely on Social Security disability payments. Deafness also is a major obstacle to communication, even when a deaf person is fluent in Amer-ican Sign Language (ASL). Deaf people tend to be isolated from hearing society, since 99.8 percent of hearing adults do not know ASL. This may prevent a deaf person from communicating with shopkeepers, bus drivers, doctors, neighbors, and many family members. Deafness also eliminates the enjoyment of pleasant sounds, such as music and birds, and creates a variety of safety issues.

Perhaps the most interesting argument in favor of the cochlear implant is that it is merely a device that bridges two worlds. “Children with cochlear implants are still deaf, but are able to use the devices to communicate,” Balkany explains. “We ask the Deaf community to accept these children into their community, just as they are accepted into mainstream society.”

 

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