I Can Hear You Whisper Page 9

  All of this matters when you’re learning to talk because you have to know how to form the sounds you want to make. Most of us can go our whole lives blissfully ignorant of all the unvoiced labial plosives we produce in a day. But we had to learn, too. As newborns, we had a vocal tract that was not yet capable of the fine motor skills necessary for speech. An infant’s larynx is too high in the throat, and the tongue fills the mouth. But the system matures quickly, and usually by four months, babies begin playing around, babbling, with their teeth and tongues. By trial and error, most get there naturally, though some sounds are harder to master than others. Our son Matthew had trouble with “r’s” and “l’s” until he was six. Words like “girl” and “earth” were almost unintelligible, which wasn’t all that uncommon. “R” and “l” tend to be among the last phonetic sounds that children master.

  Without hearing aids or a cochlear implant, someone who is profoundly deaf can not only not hear others, he also cannot hear himself. The only way for him to form spoken words is to memorize where exactly to put his tongue, how to form his lips, and what it feels like if he touches his larynx for each separate sound.

  For Alex, on the other hand, his new hearing aids were now bringing him information about sound he hadn’t had access to before. Relying on residual hearing, hearing aids amplify sound to bring as much as possible into the range necessary for understanding speech. They had come a very long way since Alfred duPont’s desk-size boardroom set. Today, they are digital; audiologists can program them very precisely to an individual’s hearing loss. It was as if we had pushed the reset button on learning language, but with a lot of ground to make up.

  • • •

  Perched on a wooden stool in the hallway of the Clarke School on a Monday morning in April, just after Alex’s second birthday, I watched through an observation window as he worked with a speech language pathologist named Alison for the first time. A headset allowed me to listen as well.

  “Choo choo,” Alison said as she rolled a wooden engine along a piece of track.

  “Push,” she said, showing him how she moved the train. “Push.”

  She blew a bubble. “Pop!”

  She held her hand over his and touched his chest. “Me,” she said, tapping his chest with his own little hand, “me.”

  Alex, watchful and shy, blew some bubbles, but he didn’t say “pop” or “me.” Instead, he pointed to a toy farm on her shelf that had caught his eye. When Alison held the cow to her face and said “moo,” Alex didn’t imitate her. But he laughed and pointed to the next animal.

  Though he enjoyed the animals, Alex was anxious and tearful in his first few weeks at Clarke. Each week, his teachers of the deaf and therapists sent home notes on his progress.

  “Limited verbal output today; still a bit fearful during transitions,” his teacher noted in the second week.

  “Alex cried upon separation from his mother … ,” the next day’s note read. “He repeated ‘pat-pat-pat’ and ‘rooooll’ during play with Play-Doh; when he saw his mother through the window/door, he fell to the floor and cried.”

  Note to self: Stay well back from the window.

  The next week was better: He “actively stomped his feet” for “If You’re Happy and You Know It” and “enjoyed pasting pictures to the construction paper.” On the other hand, he “monopolized the glue sticks.”

  Play never sounded so un-fun. But if the clinical detail effaced the joy, I knew there was a point. For these children, no word could be taken for granted. Everything had to be introduced and repeated. The notes came with lesson plans for every week, listing vocabulary to be targeted and all the themed activities that would be used to teach the new words. As for all children, one of the goals of preschool was to learn how to get along with others. A few of Alex’s new classmates had issues beyond hearing: behavioral problems, or motor skill delays. Glue sticks notwithstanding, I was relieved that there was no sign in Alex of some of the disruptive or asocial behavior I saw in some of the other children.

  Rather, Alex seemed to be the kind of kid who needed to learn to stick up for himself. He had to be taught to say “me” or “no” if a classmate took the truck he was using or tried to steal his Goldfish.

  Beyond his behavior, the daily reports teemed with approximations, verbalizations, modeling, mouthing, gesturing, cuing, and so on. It took me a little studying to learn how to decode the notes.

  “Alex assumed articulatory posture for w sound without sound emission… .” Translation: He mouthed the “wah, wah, wah” of the babies crying in the “Wheels on the Bus” song.

  “Alex approximated production of ‘cut cut cut’ and ‘knock knock knock’ imitating clinician’s productions with accuracy in number of repetitions and syllable length.” He said (probably) “cu, cu, cu” and “na, na, na.”

  By June, he was combining “actions with labels”: “wash baby,” “come doggie,” “go car.”

  By August, according to a list I kept, he had close to a hundred words, although most were approximations. He called himself “Ala.” He could handle Mama, Dada, and Matty, but he had trouble with “j” and “s,” so big brother Jake and his babysitters, Jacky and Sean, who took turns taking him to school, all got “d” at the front of their names: Dake, Dacky, and Dawn. Other new words and phrases were painstakingly added:

  Eyes, nose, hat, milk …

  Up, down, shoes, juice …

  Water, boat, bike, away …

  Uh-oh, more, all done …

  Stop it. No touch. I love you.

  Because he couldn’t hear high frequencies, he left out all “k,” “t,” and “s” sounds. He said “ow” for “cow” and “um” for “come.”

  He was still very quiet and observant. Mostly he was compliant, and if he was frustrated, he didn’t show it. So it came as a surprise one day when he threw his hearing aids into the street as he rolled along in his stroller. They were nearly run over by a bus.

  “I’m relieved to hear it,” said one of his teachers when I told her.

  “What?!”

  “It’s developmentally appropriate,” she said.

  He was two after all.

  • • •

  Alex had an audiogram. He had hearing aids. He was in a specialized school. He was making progress, and the flurry of activity had apparently slowed into routine. One question remained: Why had this happened?

  Neither Mark nor I knew of anyone in either of our families who had been deaf or hard of hearing as a child. Nothing dramatic had happened during my pregnancy: no infections that we knew of, no trauma, no worrisome blood tests or ultrasounds. The only drama had come at the end, when Alex arrived four weeks earlier than expected, which doctors classified as preterm but not premature. I couldn’t help but wonder if I was responsible somehow, even though the rational part of my brain knew that was unlikely and that such thinking was counterproductive.

  “We will probably never know why this happened,” Dr. Dolitsky told me. “You could spend a lot of time and money trying to figure it out, but I don’t think that’s worth it.” Many cases of congenital hearing loss, meaning a loss present at birth, are thought to be hereditary, but that didn’t seem likely in our case. Nonhereditary causes included infections during pregnancy. For example, an outbreak of rubella (German measles) in the 1960s affected many pregnant women and their fetuses and led to a higher incidence of deaf and hard-of-hearing babies, the so-called Rubella Bulge. Maternal diabetes, prematurity, toxemia, lack of oxygen, or complications with the Rh factor in blood could all cause hearing loss. Or a young child could have acquired hearing loss after birth from ear infections, meningitis, ototoxic drugs (medicine that damaged the auditory system), measles, encephalitis, chicken pox, mumps, flu, or head injury. Babies could even lose hearing from noise exposure, though that is far more common in adults.

  There are, however, a few causes of hearing loss that are linked to other medical issues. Those, said our doctor, are things you would want to know
about. His recommendation was a medical workup that included only tests that were either easy (like a blood test) or that allowed us to rule out complications. I went home with a stack of prescriptions: EKG, ultrasound, CT scan, and so forth.

  First, we looked at genetics, which, now that medicine has succeeded in reducing infectious disease, accounts for about half of all hearing loss in newborns. In recent years, scientists have begun to isolate genes related to hearing loss. In three-quarters of inherited cases, the cause is an “autosomal recessive” gene called connexin 26, which can be passed on if each parent carries it. Alex’s blood test was negative for connexin 26. Then we tested his heart, as there is evidence of a correlation between hearing loss and cardiovascular disease. Doctors don’t yet know why that should be but hypothesize that the link might be impaired blood flow that damages the sensitive inner ear and can also damage the heart. Alex’s EKG was normal. Next, we looked at his kidneys. Since both the ears and the kidneys form in utero around seven weeks, it’s possible that if the fetus suffered some sort of trauma that affected the development of the ears, it might also have damaged the kidneys. Alex’s kidneys were fine. Nonetheless, for a time, I replayed the seventh week of pregnancy in my head. We had been on a vacation in Italy with my extended family, a trip planned before I’d known I was pregnant. Because I was in Italy and because it was my third pregnancy, I allowed myself a few glasses of wine and some caffeine to battle the jet lag. Was that what did it? I knew better. There would be a far higher incidence of hearing loss in French and Italian babies if a glass of wine could cause it, but I wished I had never left Brooklyn.

  The final test was a CT scan, which would give us a look at the inside of Alex’s head. It required him to be completely still. Because that was an impossibility for a two-year-old, he had to be sedated again. Out came a big needle, and I held his hand. He whimpered but quickly fell asleep. Once he was asleep, the technicians laid him onto the scanner bed, and I moved to the control room, where I could watch through the window and see the computer screens.

  The CT scanner consisted of an adult-size white bed that moved through a circular opening in a big white machine. It looked like a portal on a spaceship. CT stands for “computed tomography.” Tomos is Greek for “slice,” and that’s what the machine captures: slices. It uses X-rays to make two-dimensional images of multiple layers of a three-dimensional object—in this case, Alex’s skull. Together, those slices would be compiled to create a detailed picture of his anatomy.

  There was my small son, dwarfed by the bed, his soft arms and legs peeking out of his orange shorts and blue-and-orange striped shirt, unconscious on the other side of the glass. Suddenly, overwhelmed by his vulnerability, I had to turn away and lean against the wall. Images were collecting on the screen, and the technician began to describe what he was doing.

  “Uh-huh,” I managed to respond, my voice cracking a little. “Okay.”

  When it was all done, I cradled Alex in my arms and carried him out of the hospital and into the sunlight of a bright June day, unwilling to let go.

  A few days later, the phone rang. It was Dr. Dolitsky. “We found it,” he said. The CT scan had revealed that Alex had a congenital deformity of the inner ear similar to something known as Mondini dysplasia or Mondini deformity. It was rare, affecting fewer than 200,000 people in the United States, and it meant that his cochlea had failed to form completely. It seemed unlikely, then, that he had ever had normal hearing, despite the early hearing test that said otherwise.

  In addition, he had a second condition that often accompanies Mondini dysplasia: enlarged vestibular aqueduct (EVA), also known as large vestibular aqueduct syndrome (LVAS). Vestibular aqueducts are circular bony canals that look like soda-can pull-top rings and sit just above the inner ear. They help us balance. In Alex and others with EVA, one or more of the vestibular aqueducts is larger than normal, meaning it’s more than one millimeter in diameter, roughly the size of the head of a pin. That makes it susceptible to injury. A bump on the head or a change in pressure could result in a rupture in the sac of endolymphatic fluid that is attached to the aqueduct. When that happens, the fluid inside drips down onto the inner ear, with which it is not chemically compatible. The result is further damage to the hair cells of the inner ear. Nearly every child with EVA develops some hearing loss, and according to the National Institute on Deafness and Other Communication Disorders, 5 to 15 percent of children with sensorineural hearing loss have EVA.

  “The usual recommendation is no contact sports,” Dr. Dolitsky told me over the phone, explaining that strategy as a way of eliminating one category of risk. “And nothing that would involve a big change in pressure.” He ran down the list: karate, soccer, football, scuba diving … I scribbled notes on the paper that was at hand, a bright blue-and-green pad for making grocery lists that seemed a bit too lighthearted for the occasion. “You will have to make your own decisions,” he said. “Unfortunately, in some instances, a drop can be caused by an airplane flight or even a big sneeze.”

  We finished our conversation and I hung up, a little stunned. I was glad to have an explanation, but now the situation could change again at any moment.

  The boys were playing nearby in the living room, and I turned in time to see Alex follow the lead of his big brothers and leap off the couch. All three then rolled around wrestling on the floor. Scuba diving was not going to be my problem.

  8

  THE HUB

  My rental car bumped down a long dirt road flanked by fields of crops. I glanced at my scribbled instructions and kept to the right. In the distance, I finally spotted a tall, thin, elderly man standing in front of a one-story frame house, part of a small group of buildings at the end of the road. He waved as I pulled up in front of him.

  “You found me,” he said with a smile.

  “At last,” I answered.

  I had been driving for hours to reach this small town in northwestern Oregon between Eugene and Portland, but that wasn’t what I meant. Dr. William House had been on my mind for some time.

  As a surgeon in Los Angeles in the late 1950s, House had been the first American to seize on the idea of electrically stimulating the ear. The device he ultimately created, which was marketed by 3M, became the first to win FDA approval in 1984. Adoring patients called him Dr. Bill and considered him a hero. Ear surgeons today describe him as a “creative genius.” “Without him, we might not have a cochlear implant,” says Dr. Marc Eisen, a Connecticut otolaryngologist who has written about the history of cochlear implants.

  Yet House has also been roundly criticized over the years. Early on, while he was developing his implant, he was practically shouted down at scientific meetings. Establishment researchers—most of them on the East Coast—thought his idea would never work. “Otology needs a new surgery; this isn’t it,” said Harold Schuknecht, Harvard Medical School professor and chief of otolaryngology at Massachusetts Eye and Ear Infirmary, at one conference. “If I tell you that a lead balloon will not fly, and you go out and build a lead balloon and it does not fly, what have you learned?” demanded another prestigious scientist. Even if it did work, they didn’t like the way House was going about it.

  Nonetheless, House built a cochlear implant that worked—his lead balloon flew, for a time anyway. It has since been replaced by more sophisticated devices, a development he resented and resisted. When I brought up House’s name with basic scientists rather than doctors, some dismissed him as a kook or a “crazy surgeon.” I wondered what the truth of it all was, and I wasn’t sure House would want to talk to me.

  Then one day my phone rang. The man on the other end was cheery and welcoming even if he sounded every one of his eighty-seven years.

  “I got your letter,” he said. “I’d be happy to have you visit.”

  • • •

  The idea of using electricity to treat deafness would seem to require a futuristic faith in the possibilities of science, but it dates to the late 1700s. Electricity was a rela
tively new source of fascination then, and curious scientists everywhere were working to understand its principles. The Italian physician and physicist Luigi Galvani kept an electrostatic machine in his laboratory. One day, just as the machine was generating sparks, an assistant happened to touch the sciatic nerve of a dissected frog with a scalpel. The frog’s leg muscle twitched. Intrigued, Galvani set up a series of experiments and succeeded in making the frog’s muscle twitch under a variety of conditions. He had discovered bioelectricity, the fact that our nerves use electricity to send signals, though he didn’t quite understand what he was seeing. The force Galvani called “electric fluid” or “animal electricity” looked to him like an innate, unique form of energy. The 1791 publication of his finding stirred excitement for its potential in treating medical conditions.

  Galvani’s compatriot Alessandro Volta, a professor of physics at the University of Pavia, was paying close attention. Unlike most of their colleagues, Volta didn’t believe in Galvani’s “animal electricity” theory. Instead, Volta guessed it was contact between two dissimilar metals touching the frog’s leg that caused the stimulation—the frog was simply a conductor. Galvani answered by inducing the same response with two pieces of the same metal. Back and forth the two scientists went, trading competing theories. The disagreement was cordial (it was Volta who coined the term “galvanism” in honor of his friend), but it was persistent and public. Today, we know Galvani was correct in recognizing that the electricity occurred naturally in the animal tissue, and Volta was right that this was not “animal electricity.”