Auto sclerosis

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Overview

Otosclerosis is a term derived from oto, meaning “of the ear,” and sclerosis, meaning “abnormal hardening of body tissue.” The condition is caused by abnormal bone remodeling in the middle ear. Bone remodeling is a lifelong process in which bone tissue renews itself by replacing old tissue with new. In otosclerosis, abnormal remodeling disrupts the ability of sound to travel from the middle ear to the inner ear. Otosclerosis affects more than three million Americans. Many cases of otosclerosis are thought to be inherited. White, middle-aged women are most at risk.

Symptoms

Many people with otosclerosis first notice that they are unable to hear low-pitched sounds or can't hear a whisper. Some people may also experience dizziness, balance problems, or tinnitus. Tinnitus is a ringing, roaring, buzzing, or hissing in the ears or head that sometimes occurs with hearing loss.

Causes

Otosclerosis is most often caused when one of the bones in the middle ear, the stapes, becomes stuck in place. When this bone is unable to vibrate, sound is unable to travel through the ear and hearing becomes impaired (see illustration).


The outer ear includes the pinna, temporal bone, and ear canal. The middle ear includes the eardrum, malleus, incus, and stapes. The inner ear includes semicircular canals, eustachian tube, cochlea, and vestibule and auditory nerves.


Why this happens is still unclear, but scientists think it could be related to a previous measles infection, stress fractures to the bony tissue surrounding the inner ear, or immune disorders. Otosclerosis also tends to run in families.


It may also have to do with the interaction among three different immune-system cells known as cytokines. Researchers believe that the proper balance of these three substances is necessary for healthy bone remodeling and that an imbalance in their levels could cause the kind of abnormal remodeling that occurs in otosclerosis.

Risk factors

Otosclerosis affects more than 3 million Americans. Experts aren't sure exactly what causes it. But they do know these risk factors may make you more likely to get it:

  • Age: It usually starts when you're young. You can develop otosclerosis between the ages of 10 and 45, but you're most likely to get it during your 20s. Symptoms usually are at their worst in your 30s.
  • Genetics:  It often runs in families. About half of all people with otosclerosis have a gene that's linked to the condition. But even if you have the gene, you won't necessarily get it.
  • Gender:  Both men and women get otosclerosis. Women, though, have a higher risk. Experts aren't sure why, but if you're a woman and develop otosclerosis during pregnancy, you're likely to lose your hearing faster than if you were a man or you weren't pregnant.
  • Race and Ethnicity: Caucasians are most likely to get it. About 10% develop otosclerosis. It's less common in other groups and rare for African Americans.
  • Medical History: Certain medical problems can raise your chances of otosclerosis. For example, if you had measles at any time, your risk may go up. Stress fractures to the bony tissue around your inner ear also might make it more likely to happen. And immune disorders, in which your immune system mistakenly attacks parts of your body, also can be linked to the condition

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Complications

Complete deafness.

Funny taste in the mouth or loss of taste to part of the tongue, temporary or permanent.

Infection, dizziness, pain, or a blood clot in the ear after surgery.

Nerve damage.

Prevention

 There are no preventable risk factors for otosclerosis (such as exposure to loud noises). Some people simply have a genetic predisposition for the condition. As a result, there's no way to prevent otosclerosis from developing