A Rare, Inherited Condition Was Causing Me to Go Deaf

Television had sounded muffled for about a month, and I began to wonder why everybody was mumbling. Like an elderly person without a hearing aid, I urged people to speak up. But I was only 38. I didn't attribute these problems to hearing loss.

Then one day when I was walking, I noticed something strange. An airplane flying low overhead was eerily silent. Not even the distant drone of an engine pierced the quiet of a sunny summer afternoon.

I still didn't suspect that I was going deaf. I was busy taking care of young children, who were loud enough and time-consuming enough to camouflage my hearing loss. While my husband watched television at night, I read books, so it was no big deal if he didn't turn the volume up.

The truth hit me suddenly when I was on the phone with a friend. For a couple of weeks, I had complained about low phone volume. My friend's voice sounded faint and faraway, as if she were speaking through a tunnel.

As usual, I was multitasking; making brownies and talking on the phone at the same time. Being left handed, I held the phone to my left ear. But in the middle of our conversation, I switched it to my right ear so I could stir batter with my left hand. After the switch, I was startled to hear my friend clearly. The tunnel effect was gone.

Fascinated, I switched the phone back and forth a few more times. There was no doubt about it. The problem wasn't the phone. The problem was my left ear.

It was only then that I remembered something my father had said a decade earlier. I had scarcely listened at the time, but now I recalled his words. "If you ever start losing your hearing, have it checked out. There's a condition in our family that causes deafness and it's hereditary. It usually begins when people are in their thirties."

Other memories flooded back. My father had undergone surgery that restored his hearing but destroyed his sense of taste. He had eventually gone deaf in his other ear, yet opted not to have surgery a second time.

Bursting with questions, I drove to my parent's house to get answers. What was the inherited condition? How effective was the surgery? Was losing my sense of taste the price of restoring my hearing?

They told me the disease was called otosclerosis, and that surgery was highly effective. The reason my father had lost his sense of taste was because a nerve close to the ear had accidently been severed during surgery, but his sense of taste had gradually returned to normal.

The Doctor Was Skeptical

My next step was to visit a doctor and tell him I thought I had otosclerosis. The doctor was skeptical. "It's very rare. I've never seen a case of it," he said. "Sometimes people experience a slight loss of hearing when they listen to music that's too loud or when they don't clean the wax out of their ears. Have you been listening to loud music?"

A hearing test changed the doctor's attitude. The auditory loss in my left ear was too significant to be attributed to loud music or ear wax, so he referred me to surgeon.

The surgeon, who confirmed a diagnosis of otosclerosis, explained that the condition is rare, with fewer than 200,000 U.S. cases a year. It occurs when a small bone in the middle ear, usually the stapes, becomes frozen in place because of an overgrowth in bone tissue. The stapes bone has to vibrate for us to hear well. If it can't move, sound can't travel from the middle to the inner ear.

Genetic predisposition and measles infections are contributing factors. Some women with otosclerosis develop hearing loss during pregnancy. The disease usually begins when people are in their twenties or thirties.

Through a surgery called stapedectomy, the surgeon removes all or part of the original stapes bone and replaces it with an artificial device that allows sound vibrations to reach the inner ear.

Opting for surgery wasn't a difficult decision. I was worried, though. What if surgery didn't work? What if I lost my hearing completely, or never regained my sense of taste? These were real risks, but I decided the odds were in my favor and scheduled the stapedectomy.

I remember being on the surgical table. The surgeon asked me several times which ear was affected and if I could hear him speaking. Once when I turned my head, he said, "It's very important that you don't move! Stay perfectly still!"

The reason a local anesthesia and numbing medication are used instead of general anesthesia during a stapedectomy is so the surgeon can check the patient's hearing to make sure the operation is successful.

Successful Surgery With Only One Side Effect

My surgery was a success. Following a one-night stay in the hospital, I was able to return home. The only side effect was that I can't always tell where sounds are coming from. Someone can speak to me from across a room, and I don’t know if they are on my right or left, behind or in front of me. In 80 percent of cases, otosclerosis affects both ears, but almost 30 years post-surgery, the hearing in my right ear remains normal.

A few days after surgery, I was outside when an airplane passed low overhead. This time, I heard the engine's drone as the plane dipped low, then disappeared beyond the trees. I also heard birds singing and the rustle of leaves stirred by an autumn breeze.

It was a wonderful feeling, to hear sounds I didn't know I was missing.