By Anne Dooley
Special to The Washington Post
Tuesday, January 6, 2009
If anyone had suggested to me just a few years ago that my deaf daughter should undergo intricate operations on both ears to receive cochlear implants, I would have recoiled. Believe me, I wanted Ruthie to be able to hear -- and speak. Her progressive hearing loss had been identified when she was 2, by which point she was profoundly deaf and almost entirely lacking in language. My husband, Aaron, and I felt a decided sense of urgency to get her communicating. We arranged for Ruthie to have surgery on her left ear when she was nearly 3.
Not that it was an easy decision. Like any other procedure, cochlear implant surgery carries risks. What's more, it destroys all residual hearing in the ear, eliminating the possibility that some yet-to-be-invented technology would one day give our little girl less-invasive access to hearing. So one surgery, one implant seemed enough, thank you very much. We'd leave her second ear alone.
My views shifted in the intervening years, and two months ago Ruthie, who is now 8, underwent surgery again, this time on her right ear. A few weeks later her new implant was turned on, and she began the laborious and sometimes funny process of learning to hear all over again.
How did we get here? When Ruthie's hearing loss was diagnosed, Aaron and I felt we had to consider every option. Ruthie attended preschool classes at the Maryland School for the Deaf in Columbia and picked up American Sign Language (ASL) quickly, as did Aaron and I. We believed then -- as we still do -- that she would always have a place in the ASL-based deaf community. Coming to terms with her deafness meant having long discussions with adults who had made rich lives for themselves without hearing or speech; it also meant understanding that nothing would ever "cure" it; Ruthie will always be deaf.
Despite the disapproval we were warned we might face from some members of the deaf community who do not see deafness as something that needs to be fixed, our family and friends were supportive. The near-miraculous promise of the cochlear implant seemed impossible to deny. The implant, Aaron and I were repeatedly told, would give Ruthie access to speech, something she would never achieve with even the most high-powered hearing aids. So it was that six years ago, after several hours of surgery at the Johns Hopkins Medical Center, Ruthie emerged from anesthesia with an electrode array wrapped around her auditory nerve and a magnet, which would later hold a microphone to the outside of her head, nestled into her skull. A few weeks later, her implant was activated, and her brain began decoding the new electrical stimuli it was now able to receive.
Ruthie did well. She continued to use ASL with deaf friends, and at school used "cued speech," a visual system invented at Gallaudet University in the 1960s that eliminates ambiguity from lip reading by representing speech sounds with different hand shapes. At home, we signed, cued and spoke. Ruthie's vocabulary and reading skills grew by leaps and bounds.
But she was exhausted. As Aaron and I watched Ruthie drag home day after day, worn out from the demands of listening in school, we began to investigate ways to ease her difficulty. We found that medical research and anecdotal reports of other families pointed toward a second surgery.
Getting a second implant made a certain logical sense. If people are advised to use two hearing aids when both ears are damaged, why wouldn't the same be true of cochlear implants?
Our brains are designed to process sound from two ears. Studies of bilaterally implanted children and adults have shown that their hearing improves in three ways: First, the head itself acts as a barrier, eliminating certain sounds and allowing the listener to tune into speech using the ear closest to its source. Second, the brain is better able to zero in on the important speech signals, even in a noisy environment. And third, by using both ears a listener can more accurately sense where a sound is coming from. A friend told me about a recent conference of cochlear implant users: Waiting in the hotel lobby, all the implanted participants snapped to attention when a bell signaled the arrival of an elevator. But the bilaterally implanted individuals were the ones who immediately moved toward the elevator whose bell had sounded.
Ruthie's former babysitter, Laura Cunningham, received a second implant last year -- 18 years after her first, at age 3. Laura was able to talk on the phone after her first implant and has held such hearing-dependent jobs as receptionist and waitress. She still uses ASL with deaf friends who prefer that mode of communication and says the majority of her deaf friends support her decision to get a second implant. The only negative comments she has heard, Laura says, come from "some cochlear implant users who think that they should save their [second] ear" for a future technology.
For a while, that was the conventional wisdom. And for individuals with sufficient hearing to benefit from a hearing aid in the unimplanted ear, bilateral implants may not be the right choice. But for others, every year that the unimplanted ear is not used reduces the brain's ability to adjust to an additional source of hearing. Children are now regularly being bilaterally implanted as young as 1 year old. If we believed a second implant could help Ruthie, there was really no point in waiting.
As John Niparko, director of the Listening Center at Johns Hopkins, put it, "If a child is implanted on only one side, the parts of the brain that would have been stimulated by the non-implanted ear will not develop, and eventually plasticity will be greatly diminished."
What does that mean for a child? Ruthie's friend Mia received a second implant 17 months ago, at age 8. "Now," her mother, Carolyn Jeppsen, says, "Mia is able to participate in group conversations with much more ease than before, making her less reluctant to do so. She is able to appreciate music, which she did not do at all with one implant. The binaural hearing has strengthened her balance and coordination. Mia's life is much easier with two implants."
Ruthie's road to bilateral implantation hit a snag when our insurer denied the surgery with the rationale that bilateral implantation is "experimental and untested." We turned to the Let Them Hear Foundation, whose pro bono lawyer prepared an appeal several inches thick containing letters from Ruthie's teacher, audiologist, surgeon and speech pathologist, along with reams of research. Our insurance company eventually agreed to pay for Ruthie's surgery.
Our appeal was bolstered by a study released last May by Indiana University; it found that the benefits of a second implant far outweigh the cost. (Estimates range from $40,000 to $100,000 when the costs of rehabilitation and maintaining the device are factored in.)
"We didn't know that cognitive skills and emotional issues would so significantly improve with the implantation of a second cochlear device," said the study's senior author, Richard Miyamoto, chairman of the university's Department of Otolaryngology -- Head and Neck Surgery. "Our hope is that with these findings more health insurance companies will cover the cost of bilateral implants and bring a superior quality of life to a large number of individuals."
The day after her second implant was activated, Ruthie turned off the left, older implant during dinner so that she could practice making sense of the sounds around her with her right ear alone. As Aaron asked Hannah, our older daughter, to pass the pepper, Ruthie began to smile. When I offered her grated cheese, she laughed and said, "Beep! You are all saying 'beep, beep, beep!' "
By the next morning, she was able to identify three of the six basic speech sounds with her new implant.