Otosclerosis

Alternative names
Otospongiosis

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

Causes, incidence, and risk factors
Otosclerosis is an inherited disorder involving the growth of abnormal spongy bone in the middle ear. This growth prevents the stapes (stirrup bone) from vibrating in response to sound waves, thus leading to progressive (worsening over time) hearing loss.

Otosclerosis is the most frequent cause of middle ear hearing loss in young adults, affecting about 10% of the population of the US. Otosclerosis usually affects both ears, and is most commonly seen in women aged 15-30.

Risks include pregnancy (which may trigger onset) and a family history of hearing loss. Caucasians are more susceptible than others to otosclerosis.

Symptoms

     
  • hearing loss       o slow, progressive       o hearing may be better in noisy environments than quiet areas.  
  • ringing in the ears (tinnitus)

Signs and tests
An examination of the ears may rule out other causes of hearing loss.

     
  • A hearing test (audiometry/audiology) may determine the extent of hearing loss.  
  • A temporal-bone CT scan may be used to distinguish otosclerosis from other causes of hearing loss.

Treatment

Otosclerosis may be slowly progressive, and the condition may not require treatment until the extent of hearing loss is significant.

Medications such as oral fluoride, calcium or Vitamin D may help to stabilize the hearing loss, but their benefit has not yet been proved.

A hearing aid may be used to treat the hearing loss. This will not cure or prevent the progression of the disease, but may help alleviate the symptoms of hearing loss.

Surgery to remove the stapes and replace it with a prosthesis is curative. This may be total replacement (stapedectomy) or a laser may be used to make a hole in the stapes (stapedotomy) to allow placement of the prosthesis.

To reduce the risk of complications after surgery:

     
  • Nose blowing is discouraged for 1 week after surgery.  
  • Avoid people with respiratory or other infections.  
  • Protect the ears against cold.  
  • Avoid bending, lifting, straining (may cause dizziness).  
  • Avoid loud noises or sudden pressure changes (scuba diving, flying, driving in the mountains) for 6 months or until healed.

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

Expectations (prognosis)
Otosclerosis is progressive without treatment; however, surgery may restore at least partial hearing. Most complications of surgery correct themselves within a few weeks.

Complications

     
  • total deafness  
  • infection, dizziness, pain, 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.

Johns Hopkins patient information

Last revised: December 3, 2012
by Martin A. Harms, M.D.

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