Otosclerosis

Definition

Otosclerosis is an abnormal bone growth in the middle ear that causes hearing loss.

Alternative Names

Otospongiosis; Stapedectomy

Causes, incidence, and risk factors

The cause of otosclerosis is unknown. However, there is some evidence that it may be passed down through families.

People who have otosclerosis have an abnormal sponge-like bone growing in the middle ear. This growth prevents the ear from vibrating in response to sound waves. Such vibrations are needed in order for you to hear.

Otosclerosis is the most frequent cause of middle ear hearing loss in young adults. It typically begins in early to mid-adulthood. It is more common in women than in men. The condition usually affects both ears.

Risks for this condition include pregnancy and a family history of hearing loss. Caucasians are more likely to develop this condition than others.

Symptoms

• Hearing loss may occur slowly at first but continue to get worse.
• You may hear better in noisy environments that quiet ones.
• Ringing in the ears (tinnitus) may also occur.

Signs and tests

A hearing test (audiometry /audiology) may help determine the severity of hearing loss.

A special imaging test of the head called a temporal-bone CT may be used to rule out other causes of hearing loss.

Treatment

Otosclerosis may slowly get worse. The condition may not require treatment until you having severe hearing problems.

Medications such as fluoride, calcium, or vitamin D may help to slow the hearing loss, but the benefits have not yet been proved.

A hearing aid may be used to treat the hearing loss. This will not cure or prevent hearing loss from getting worse, but may help relieve some of the symptoms.

Surgery to remove part of the ear (stapes) and replace it with a prosthesis can cure conductive hearing loss. A total replacement is called a stapedectomy. Sometimes a laser is used to make a hole in the stapes to allow placement of the prosthetic device. This is called a stapedotomy.

Support Groups

Expectations (prognosis)

Otosclerosis gets worse without treatment, but surgery may restore at least some hearing. Pain and dizziness related to surgery usually goes away within a few weeks.

To reduce the risk of complications after surgery:

• Do not blow your nose for 1 week after surgery.
• Avoid people with respiratory or other infections.
• Avoid bending, lifting, or straining, which may cause dizziness.
• Avoid loud noises or sudden pressure changes such as scuba diving, flying, or driving in the mountains until healed.

If surgery is unsuccessful, total hearing loss may occur. Treatment then involves developing skills to cope with deafness, including use of hearing aids and visual cues.

Complications

• Complete deafness
• Nerve damage
• Infection, dizziness, pain, or blood clot in the ear after surgery

Calling your health care provider

Call your health care provider if hearing loss occurs.

Call your health care provider if fever, ear pain, dizziness, or other symptoms develop after surgery.

Prevention

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References

House JW, Cunningham CD III. Otosclerosis. In: Cummings CW, Flint PW, Haughey BH, et al, eds. Otolaryngology: Head & Neck Surgery. 5th ed. Philadelphia, Pa: Mosby Elsevier; 2010:chap 144.