Today, after 50 years of being deaf, Sigrid Cerf is going to hear again.
At age 3, Cerf contracted meningitis, which rendered her severely deaf. (Without a hearing aid, she could only begin to faintly hear sounds in the 85-100 decibel range, roughly the volume of a rock concert.) Though she could no longer hear much, she had already learned how to talk. With her ability to lip-read and the help of hearing aids, she managed to converse for many years with a barely perceptible difference in her speech. Then, at age 30, she woke up one terrifying morning unable to hear anything at all. What she calls "a freak ear infection" had left her unable to hear anything softer than 95-100 DB (about the sound of a fired up jet engine). Overnight she went from being severely to profoundly deaf.
Years later, after investigational trials and FDA approval, she became a candidate for a cochlear implant, a device developed in the 1970s independently in California and Australia, that helps the profoundly deaf receive auditory information and stimulus by bypassing the defective hair cells in the ear and directly triggering the auditory nerve.
The implant consists of eight electrodes and a decoder. A surgeon implants the electrodes into the cochlea of one ear and the decoder in the bone behind the ear. On the ear a patient wears a tiny microphone that fits along the upper rim, to which the portable speech processor is attached by wire. The microphone is also wired to a transmitter, worn externally, that is magnetically attached to the implant. Three to four weeks after surgery, audiologists activate the implant in a process that involves programming the maximum and minimum volume settings for each of the electrodes.
On this May morning, Hopkins audiologist Karin Young adjusts the volume on the electrodes that have been implanted in the cochlea of Cerf's left ear. It is a trial-and-error process that uses a pulsed stimulus to determine how much current/amplitude is enough for the patient to hear. For now, Cerf is receiving stimuli only from a computer to which she is linked by wire through her speech processor. The pulse on the first electrode is getting stronger.
"I feel it, I feel it," she says. "It's like there's a little gerbil, crawling around up there."
Cerf's microphone has yet to be turned on, so she's yet to hear sound from people or things around her, but she's intent on trying to relay what she is detecting from Young's electronic stimuli: "Imagine a migraine without a headache. It's like the ringing from a blow to your head." She fiddles with the magnetic transmitter that keeps falling off her head.
After Young sets the volumes on all the electrodes, she activates the microphone. Cerf is all smiles as she tries to come up with another metaphor so that all the hearing people around her can understand what she's experiencing: "It's like when, at 2 a.m., a TV channel is out of service." Young turns up the volume on the microphone while Cerf is speaking. Suddenly she cries out, "I heard my own voice! Oh, it sounds so much more feminine than I thought it would."
Those in the room share Cerf's excitement as she adjusts the transmitter some more and exclaims, "That's amazing!"
She characterizes the sound she's receiving as having a "Donald Duck quality," then turns to her friend who has traveled with her from Virginia. "I'm dying to know what John's voice sounds like," she says.
A few words and few moist eyes (though not Cerf's) later, she grins "Boy, this is going to keep me busy for a while."
Surgeons have been implanting adults with cochlear implants since 1984, when the the procedure gained approval from the FDA, and children since 1990, when the agency approved the procedure in children over 2 years of age.
Niparko says it quickly became apparent at Hopkins that newly implanted patients, particularly children, who had little to no experience with past hearing, needed follow-up rehabilitation after surgery to make the transition to the hearing world. Thus the Department of Otolaryngology last year launched its Listening Center for pediatric patients (who in all cases are either congenitally deaf or have lost their hearing by age 1). There are currently 52 children in the center's rehab program, and two to three new children are implanted each month, says Niparko, who does all the implants on both adults and children. To date, insurance companies have been covering the costs of surgery and rehabilitation, which at Hopkins are upward of $15,000.
According to Young, follow-up rehabilitation is crucial, and something too often overlooked: "Centers that don't have counseling and rehabilitation hurt the implant's image because they don't teach [children] who get the implant how to use it."
When children are involved, Hopkins audiologists and physicians say it is particularly important to spend time with families to help them decide whether or not to implant, and to make sure that their expectations are realistic. Children are initially tested to see if they make good candidates, in terms of hearing loss and familial support, and there are follow-up consultations and meetings with their teachers and speech pathologists to help them understand the nature of the implant.
"To just activate the implant is a gross injustice," says Niparko. "It does not acknowledge the need to allow a period of training for the children." For some children the new perception of sound can be quite traumatic, even scary. After activation, all need time to develop a new foundation for hearing, and to attach meaning to the auditory information that they are being fed.
The rehabilitation process at the center starts soon after activation and consists of a weekly two-hour session with an audiologist.
"The first thing we always work on in these sessions is sound detection," explains Mary Koch, coordinator of cochlear implant rehabilitation. She is taking a break from working with Melissa, a 4-year-old whose implant was activated about four weeks earlier. Koch has been working with Melissa for about 20 minutes, playing a game in which she, or Melissa's mother, alternately cover their own mouths (so Melissa can't lip-read) and make a sound. If Melissa can hear the sound, she drops a ball down a chute. Parents are always involved in these sessions. "They are a cornerstone in the rehabilitation, so educating the parent is essential," says Koch.
Initially, Melissa was reluctant to be the one who listens first in the game, insisting it was her mother's or Koch's turn to listen. She seemed playful yet frustrated, crying intermittently.
Koch explained later, "You can't always deal just with the hearing loss. There are so many peripheral issues such as control, attention span, or moodiness."
For the next round, Melissa must repeat the sound Koch is making as the audiologist covers her mouth again. Koch begins with a "meow," hoping Melissa will imitate the syllable. But instead the little girl jumps ahead and signs "cat."
Melissa has the advantage of a vocabulary with which to work-- sign language. This will make assigning meaning to sounds an easier task, Koch says. Some of the center's children come without any language ability, which makes work more difficult for Koch and her team.
Later that afternoon, Nancy Mellon brings in her 4-year-old son, Will. He has had an implant for two years now, and Koch is excited to have him demonstrate his abilities. Standing 20 feet away from him in the hallway, Koch covers her mouth and tells him to jump if he hears anything. In a voice barely above a whisper, she makes a series of sounds. Will jumps every time, holding on to his mother's long blond hair as she squats next to him. With each jump, he rattles her earrings.
As Will moves into the rehabilitation room with Koch, his mother, who also happens to be the center's program coordinator, tells how her son hates to be without his hearing. He even sleeps with the processor on, taking it off only when he's in water. "Will was a loner before the implant," says Mellon. "Now he's more tuned in and connected."
Though the cochlear implant may sound like a miracle cure for deafness, it's not. The implant is not for all deaf people. It is recommended for those who are profoundly deaf, deafness so severe that a hearing aid does not help. Since 1993, that standard has loosened a bit, however, and surgeons at Hopkins and elsewhere have begun implanting some severely deaf adults and a handful of severely deaf children.
Among adults, the implant is only used with those who have an auditory memory--people who, like Cerf, had hearing and then lost it, and thus are capable of processing sound into language. Adults born deaf would not benefit from the technology because the brain's cognitive processes are less adaptable later in life; neural connections used in acquiring language are lost to disuse. Niparko estimates that in the United States, 20 million people are hearing impaired. Of those, about 10 percent are profoundly deaf, the majority of whom are adults who were born deaf or have been deaf since age 1, and so have missed the critical period for language development. That leaves about 200,000-500,000 potential candidates for the implant.
Adult patients initially find the sounds they hear with the implant to be mechanical, Young says. After about two months, they say things sound the way they remember. Over time, the sound becomes increasingly more natural as the brain begins to compensate.
For children the experience is quite different, Young says. They've never experienced hearing, so they have to learn how to integrate listening--environmental sounds, phonemes--into daily life. They have to practice listening without it being work. It's an ongoing process. She says, "I haven't seen kids plateau, though. They continue to progress."
Implanting deaf children with cochlear devices, however, has met with controversy from an increasingly vocal "deaf culture" that resists the idea that deafness is a defect that needs to be fixed or cured. For them, parents who implant their children are making the decision for them not to be a part of the deaf culture.
Though officials at the National Association for the Deaf (NAD) declined to be quoted directly, they provided position papers that contend that the cochlear implant can raise parents' hopes of a "miracle cure," and of "normalizing" their child. This delays acceptance of the child's deafness, the NAD argues, and thereby delays early educational and communicative intervention processes, such as learning sign language.
While research is necessary and needs to be supported, according to the NAD, it is equally important not to prematurely employ surgery that, the NAD contends, may turn out to be irrevocable.
Like the NAD, Hopkins's Moise Goldstein, emeritus professor of electrical and computer engineering, opposes implanting deaf children. "If I thought the cochlear implant was giving input that a deaf child could use, without extensive training, so that they could naturally use it like a hearing child uses his/her ears, I would be supportive," says Goldstein, who served as a consultant to the FDA panels that investigated using the implants in adults and children. He says the data given to support the implant showed encouraging scores of implanted children on tests that asked them to distinguish a male voice from a female one, or one consonant sound from another. But, according to Goldstein, the data failed to distinguish between those children who were congenitally deaf and those with an auditory memory who had lost their hearing much later.
Goldstein believes that deaf children should learn sign language (ASL) as their first language, and English as their second. He says his approach is based on neurology. "Deaf children have a visual channel that is fine, but an auditory one that is not. One approach is to accept them as different, then use their visual channel to the brain to allow them to achieve language [ASL] at the same pace as other children."
He continues, "The seed of language is in the brain, and there's great similarity in the cognitive processes of someone who knows ASL as a first language and someone who knows spoken English. Neurally, you get language with deaf children who sign--they babble with their hands, their first word around age 1--all the normal benchmarks you have in hearing children. Then you work to add English as a second language.
"You could achieve a language bilingualism, if you make the one they can learn at the brain's pace the one without the deficit," says Goldstein.
Niparko, who is also the Listening Center's director, takes issue with Goldstein's contention that the implant is not widely effective. "At Hopkins, over 60 percent of our adults are phone users," says Niparko, "and the average sentence understanding score is over 80 percent, which means that in four out of five sentences said, our adults are able to recite them back. Virtually all of our patients can understand phrases without looking at the speaker. Most can hear a whisper from six to eight feet."
What's more, Niparko believes that deaf children can use the implant to build the foundation for spoken language; the Listening Center endorses early intervention for profoundly deaf children, he notes, because in these early developmental stages the brain is developing the neural pathways for speech perception.
Doctors and audiologists at the center point out that by not implanting children early, any potential benefits that might be gained are lost, and in effect, the decision is being made for these children to remain solely in the deaf community.
"To really integrate, you need to speak the language of the culture," says Niparko. "To effectively move in any society, you need the common currency of language. With the cochlear implant you're taking potentially successful members of the deaf world and giving them access to the hearing world."
Mellon concurs. "For us there was no controversy in deciding to implant Will. We want to give him as much hearing as we can. To deny a child the opportunity to get the implant is choosing for them."
A month after Cerf's activation and after we exchange several e- mail messages, she calls me on the phone. A normal, non-TTY phone. I'm surprised to hear from her this way, even more surprised that she can hear me.
We chat for 10 minutes, a fluid, uninterrupted conversation about restaurants in Fells Point, without having to repeat words and sentences. We make a lunch date for the following day.
"Music sounds better now," she tells me in the car the next day, as I lower the volume on the radio. "Everything sounds better now. Things are improving every day."
Driving down Caroline Street in East Baltimore, from the Outpatient Clinic to Fells Point, Cerf remarks how fascinated she is by the city. She's been here several times over the past eight months to consult with Niparko and then to go through the business of getting the implant.
Cerf was living on the West Coast during the 1970s when the cochlear technology was being developed there. At that time she was told that her hearing was too good for her to be a candidate. After the ear infection that cost her what little hearing she had left, however, she was deemed "deaf enough."
"I had been trying since last June  to find out about the cochlear implant program at Hopkins," she says. "I couldn't get anyone's attention." Finally, she e-mailed a friend overseas who in turn e-mailed someone at Hopkins. Nine months after she had started, Cerf connected with Niparko.
According to Cerf, at her evaluation, Niparko did not know if she would make a good candidate because it had been 50 years since she had lost her hearing. The Kansas native had fared well during those years, graduating from Kansas State University College with a degree in art and architectural illustrating (she sat up front during lectures and read professors' lips), then moving to Los Angeles, where she met her husband, Vint Cerf, and later had two children. Recently, she had been active with hearing impaired people in Northern Virginia, working with the Smithsonian Institution on accessibility issues for the deaf and hearing impaired.
Cerf says she tried to separate herself emotionally from the surgery. She didn't allow her husband to come with her. Instead she invited a small group of neighbors, fellow members of an ice cream club. Having them there, she says, kept her mind off surgery and focused on dessert. She was activated on May 14. As we speak, she's been living with sound for a month.
"The cochlear implant has radically changed my life. I was introverted before. Now I am extroverted," Cerf says. "It's opened a new world of possibilities. Communication is everything.
"I'm catching up on lost time," she explains. "My son was home for a week and we got to know each other. We had had inhibited conversations before. This time we were lying on my bed in the dark just talking. But he was testing me. He mentioned our dog, Sally, and said 'salad' and I caught it." She smiles.
At lunch, she says while perusing the wine list,"I'm taken by the whole metamorphosis. They say it gets even better. I can't believe that." Switching gears she says, "I can't [understand] the opponents who say it's a crime to invade someone's skull."
Cerf concedes that the controversy may never go away. She even concedes some validity to the deaf community's objections. But she insists, "I would no more want my child growing up knowing only Japanese in America. The cochlear implant gives someone the option of more than one language, the difference between saying you can do everything instead of saying you can only do one thing."
For Cerf, this is a "glorious time to be deaf, " a time in which she is borrowing taped books from the library so she can practice her hearing, a time in which her pronunciation of Italian (a language she had been studying even before the implant) can be corrected because she can hear the difference between what sounds like bru-shet-ta and what is bru-sket-ta.
She declares, "This has been the happiest year of my life."
Cerf looks forward to taking a car trip all day with the radio on. "I want to listen to everything, even the commercials. They're so clear!" And if she's not listening to the radio, she's going to the movies--most recently to see Mission Impossible. "Tom Cruise is such a hunk," she says.
Before we part back at the Outpatient Clinic, she says, "I was listening to Beethoven's Ninth Symphony on my first day with hearing. It was so symbolic, I said, 'Gee, do you think Beethoven could have written any better with a cochlear implant?'"
Student intern Alia Malek '96 graduated from Hopkins in May with a degree in international studies.
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