What is otosclerosis?

The term otosclerosis is derived from the Greek words for “hard” (scler-o) and “ear” (oto). It is a disease in which an abnormal bone growth around the stapes bone, one of the tiny bones of the middle ear, prevents the ear bones from vibrating normally and leads to conductive hearing loss.

It is estimated that ten percent of the adult Caucasian population is affected by otosclerosis. The condition is less common in people of Japanese and South American decent and is rare in African Americans. Overall, Caucasian, middle-aged women are most at risk. It can begin anytime between the ages of 15 and 45, but it usually starts in the early 20s. The disease can develop in both women and men, but is particularly troublesome for pregnant women who, for unknown reasons, can experience a rapid decrease in hearing ability.

Symptoms

Gradual hearing loss is the most frequent symptom of otosclerosis. Often, individuals with otosclerosis will first notice that they cannot hear low-pitched sounds or whispers. Other symptoms of the disorder can include dizziness, balance problems, or a sensation of ringing, roaring, buzzing, or hissing in the ears or head known as tinnitus.

Common Symptoms:

Hearing loss (slow at first, but worsens over time)
Ringing in the ears (tinnitus)
Vertigo or dizziness

Causes

The exact cause of otosclerosis is unknown but approximately 60 percent of otosclerosis cases have a genetic predisposition. On average, a person who has one parent with otosclerosis has a 25 percent chance of developing the disorder. If both parents have otosclerosis, the risk goes up to 50 percent.

Diagnosis

To help determine the severity of hearing loss, a hearing test (audiometry/audiology) is conducted

Examination
Because many of the symptoms typical of otosclerosis can be caused by other medical conditions, it is important to be examined by an otolaryngologist (an ear, nose and throat doctor) to eliminate these other causes.

Testing
After an examination, a hearing test is usually conducted to help determine the severity of hearing loss. The typical finding on the hearing test is a conductive hearing loss in the low frequency tones. This means that the loss of hearing is due to an inability of the sound vibrations to get transferred into the inner ear. A special imaging test of the head called a temporal-bone CT may be used to look for other causes of hearing loss.

Treatment

Hearing Aids
If the hearing loss is mild, hearing aids can be used to treat the hearing loss by amplifying the sound reaching the ear drum.

Cochlear Implantation
Cochlear implants are used successfully in patients with otosclerosis. Patients with the best preoperative hearing levels are most likely to benefit.

Surgical Treatment
A stapedectomy is an outpatient surgical procedure done under local or general anesthesia in which the surgeon uses an operating microscope to remove part or all of the immobilized stapes bone and replaces it with a tiny synthetic prosthesic device. The prosthetic device allows the bones of the middle ear to resume movement, which stimulates fluid in the inner ear and improves or restores hearing.

Josiane DeK., 63

“I was diagnosed at age 19 with a genetic condition where the bones of my inner ear grow together. Over the years I suffered continuing loss of hearing. Later I learned of a brand new operation that would fix the problem. When I had the right ear operated on, it was a nightmare. It was so bad, I never had the left ear done. I’m now 63, and a lady I work with had the same problem with her ears. After learning about Dr. Shohet, she decided to have him conduct the procedure, a stapedectomy, and I thought, ‘I’ll see how she does.’ She was so impressed with her results–and with Dr. Shohet–she went back 6 months later and had her second ear done. I decided then to make an appointment with him myself.

I told Dr. Shohet how scared I was because of my first experience, and how bad my condition was. There is a difference between listening and really hearing. I felt he really heard me. He showed such compassion and yet, he had a sense of humor. He assured me that the procedure had improved over 30 years. I decided to have the surgery. When I came out of the anesthesia, I could hear. It was like a miracle! I had no dizziness, and no nausea. He showed his concern for me by calling the following morning, a Saturday, to see how I was doing. It took 30 years for me to make the decision, but I am so glad I did. I am so happy with my result, I want to fly my daughter out—she has the same genetic condition–to have her procedure done with Dr. Shohet. I have complete confidence in his ability to help her too. I am so grateful to Dr Shohet!”

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