Vital Signs: Sounds of Silence

An accident threatened Mr. Sinclair's poor hearing. Could microsurgery save his fragile world?

By Norman T. Berlinger|Wednesday, April 01, 1998
RELATED TAGS: SENSES
"You must be a good kisser," said Steve Sinclair, my next patient. He was talking to Pam, our hospital’s surgical check-in clerk. She had asked him if he’d brought in copies of his physical exam and blood work, and his answer clearly caught her off guard. Mr. Sinclair, a handsome man with an imposing T-shaped physique, sensed his blunder immediately and tried to repair the damage.

"I mean, your lips move well, and it’s not too hard to read them." He headed for his briefcase and gathered up the papers she had asked for.

A little ruffled, Pam nonetheless managed to smile back sympathetically. I knew she was used to this behavior—just another case of the pre-op jitters. A seasoned nurse, Pam figured out by the time he finished the word kisser that Mr. Sinclair had been born with poor hearing. It sounded more like kishuh. His words lacked the crisply articulated consonants, like T and K, that he could barely hear and therefore never quite imitate. And most of his vowels were too guttural and long.

But Pam couldn’t have known that his glib remarks, delivered with a mischievous smile, weren’t a defense against fear but a well-practiced camouflage for his rage over his handicap. Mr. Sinclair was afraid, all right, but not of the impending surgery. He had, in fact, sought it out, embraced it, and rushed to schedule it as if it were an emergency. What he feared was the uncertainty of the outcome. A lot was riding on this.

The month was unusually wintry, and a few days earlier Mr. Sinclair had tumbled down a set of icy steps, striking his head hard enough to require stitches. Within a day of his fall, the hearing in his better ear had worsened, going from terrible to almost nothing. And of all things, his hearing aid was now making him dizzy and sick to his stomach.

I had met Mr. Sinclair and his father in the examining room shortly after the fall. When I came in, he stared at my face and, without diverting his eyes, deftly switched on the black box in his breast pocket. Jacked into the device was a pair of clumsy, molded earpieces with crinkled wires.

Even before he introduced himself, he said with a note of panic, "You’ve got to help me with this hearing aid. It makes me throw up." The device is called a body aid because the battery pack and amplifier are worn on the body, not behind the ear or inconspicuously in the ear canal. Wearing the amplifier on the body eliminates problems with receiver feedback.

Then the graying gentleman accompanying Mr. Sinclair spoke up: "I’m Steve’s father. I’m the one responsible for his hearing problem." After talking with them, though, I knew the hearing loss wasn’t hereditary but congenital. Mr. Sinclair had somehow suffered an insult in utero that impaired his hearing. Doctors didn’t know what caused it. His mother hadn’t contracted rubella while pregnant with him. Nor had he entered the world as an oxygen-starved blue baby. Since the doctors couldn’t identify what had caused the deafness, his father had decided to blame himself.

Mr. Sinclair quickly added, "We may not have a lot of time to talk, because I might be upchucking soon. Let me tell you what’s happened." Over the past few years, his hearing had grown progressively worse, and he now relied on the bulky body aid to help him read lips. The trouble he faced, he added, was that sounds like M and B looked pretty much the same. F and V were problems as well, but the body aid conveyed a little hissing or buzzing sound here and there to help him distinguish them. These shards of sound also gave him a sense of the rhythm and intonation of speech. And that, too, made reading lips a little easier. But since Mr. Sinclair’s fall down the icy stairs, the body aid had betrayed him.

The cochlea of the inner ear is a fluid-filled chamber that resembles a lilliputian snail shell. Sounds travel up the coiled chamber as tiny liquid waves before being translated into the impulses that are sent on to the brain. Normally, the body aid helped amplify sound by sending larger than normal waves up into the cochlea. Now, after his fall, Mr. Sinclair was using the highest settings on the aid and so was sending miniature tidal waves through the cochlea. These waves were apparently disturbing a neighboring region of the inner ear that controls balance. Just listening to speech made him dizzy. Sometimes, he said, the dizziness became a nauseating, whirling vertigo, causing him to yank out the aid’s earpieces and stagger away to vomit.

Mr. Sinclair cupped his hands over the body aid’s earpieces and began to plead.

"I can’t give up the body aid. It helps me read lips, and it’s the only real link I have with the world of the hearing—your world," he added with a hint of resentment. "Without it, I’ll be pretty much limiting conversation to people who sign. There aren’t many of them around. "

I think I know what’s wrong, I said. He stared at my lips, puzzled, then immediately looked to his father, who repeated what I’d said. I apologized for perhaps speaking too rapidly.

"No. It’s just that I am so used to my father, he said. I can usually interpret speech pretty well."

The rest of our conversation progressed in this way, with me looking at Mr. Sinclair, and Mr. Sinclair mostly looking at his father.

It is the strange wisdom of evolution to house the organ of balance and the organ of hearing side by side within the same bones on each side of the skull. The two neighboring organs, both similar in design, are connected by a closed labyrinthine system of fluid-filled ducts and sacs. Their proximity helps explain why diseases that adversely affect hearing can also disturb balance.

The cells inside the cochlea that capture sound are fittingly called hair cells because they are tufted with microscopic cowlicks. When waves travel up the cochlea, their passage nudges the cowlicks ever so gently. Instantly the hair cells send signals to the brain, which identifies the incoming sound—music, crickets, or a slamming door.

The balance portion of the inner ear, or vestibular apparatus, also contains hair cells. But these cells do not fire in response to sounds. They fire when a roller coaster is diving or a pirouette is too long or too brisk. And if the signals are strong enough, they can make a person feel dizzy and miserable.

But sound alone can sometimes stimulate hair cells in the vestibular apparatus. I recalled the relevant passage from my medical training: Loud noises can cause dizziness but usually only in pathological states such as syphilis, Ménière’s disease, or a fistula. Mr. Sinclair had a fistula. A fistula is a tear in tissue, and his ear had probably sprung a leak in the tiny membrane that seals off the fluid-filled inner ear. It was a small leak, not enough to make a decent-size drop. Still, it was making him sick by disrupting the intricate fluid dynamics within the ear.

We depend on a tiny bone called the stapes (the stirrup) to magnify and transmit sound into the cochlea. The job of this bone—so tiny that 13 would fit around a penny—is to make waves. When vibrations in the air, or sound, strike the eardrum, they cause the stapes to vibrate. That vibration creates waves that travel through the fluid-filled canals of the inner ear.

Mr. Sinclair’s fistula had changed the way the fluid was being distributed. And his body aid, by making larger waves in the cochlea, magnified the leakage. The loss of that fluid disturbed the way hair cells that sense movement worked. When these cells are no longer properly stimulated, a person can feel nauseated.

During Mr. Sinclair’s first visit, I explained that the techniques for microscopic surgery have been refined over the past decade. Puny but crucial nerves and vessels can be rejoined after being accidentally severed, and misbehaving organs, no matter how small, can sometimes be reengineered. I thought I could seal the tear in his ear and prevent the nausea-inducing vertigo.

He made an appointment for the surgery the day we met. Now the day of the appointment had arrived. Mr. Sinclair was ready for surgery, but he would have to undergo it without the assistance of either his body aid or his father.

Once in the operating room, all the doctors and nurses put on their surgical masks. You’re going to feel a needle stick now, the anesthesiologist announced as he started the iv in Mr. Sinclair’s left arm. He then realized his mistake and pulled down his mask to reveal his lips. But the needle had already slid in and Mr. Sinclair was wincing. Within moments, the intravenous anesthetic was taking effect.

In preparation for surgery, I had skipped coffee that morning. Under the operating microscope, an ordinarily imperceptible caffeine tremor is magnified 25 times; it is suddenly a big deal. I avoid over-the-counter cold remedies for the same reason. The circulating nurse turned down the ceiling lights for the stilling effect of dimness. In front of the scrub nurse stood a rack lined with specialized instruments. To the naked eye, they looked like identical needle-tipped tools with pencil-like handles, each bearing a number. Under the microscope, however, one could see that craftsmen had fashioned them into miniature scalpels, pushers, and curettes.

With bantam scalpels shaped like a sickle and a hoe, I made a complex incision through the delicate skin lining Mr. Sinclair’s narrow ear canal. Some segments of the cut were as much as eight or nine millimeters long. Starting at the cut edge, I rolled up the tiny carpet of canal skin toward the eardrum and burrowed under it, using my lighted instrument to peer into the middle ear.

A leak never looks like a microscopic Old Faithful; it looks more like dampness in the basement. A fistula can be as small as .1 millimeter; it may leak only one or two microliters of fluid at a time. Mr. Sinclair’s leak was not even a puddle—merely a glistening area near the buttery yellow bone of the stapes. I had prepared the tissue beforehand with an injection that constricted blood vessels. One or two drops of blood in the operative field can drown the stapes from view, and a portion of a drop can obscure a leak.

I would need fascia, the sturdy tissue that encases muscle, to seal the leak. (Fascia is what you’d call gristle on a steak.) I harvested some fascia from underneath his scalp and slid six or seven minced pieces of it no bigger than the letter o on this page around the end of the stapes. That completed the operation. As Mr. Sinclair’s ear healed over the next few weeks, the pieces of this tissue necklace would coalesce, sealing the tear.

Mr. Sinclair returned to my office for several post-op visits. He wasn’t smiling as easily anymore. He said he was feeling much better, though, because the dizziness was gone. But the invaluable bit of hearing he’d lost when he fell that icy winter day never returned. And it never would. The fluid within the ear is not only the medium that carries sound waves within the cochlea; it is also a nutrient for the hair cells. We can replenish this supply, but if too much oozes out, hair cells can die. After the fall, Mr. Sinclair had leaked out sustenance for the few remaining hair cells he had that hadn’t been damaged at birth. Without them, he could no longer travel easily within the culture of the spoken word.

I never saw Mr. Sinclair again, but I heard that he had left his job as a computer programmer. I hoped that he was learning new skills, skills that would allow him to join the deaf community. This world is different from the culture of the hearing but no less rich. I had done as much as modern medicine could for Mr. Sinclair. Sadly, it wasn’t enough. The rest was up to him.
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