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Genetic and Rare Diseases Information Center (GARD)

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Cholesteatoma


Other Names for this Disease

  • Congenital cholesteatoma (type)
  • Primary acquired cholesteatoma (type)
  • Secondary acquired cholesteatoma (type)
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

I have been diagnosed with cholesteatoma. I am looking for general information about this condition, including the chances of being affected.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is cholesteatoma?

Cholesteatoma is a type of skin cyst located in the middle ear. It can be congenital (present from birth), but it more commonly occurs as a complication of chronic ear infection.[1] The hallmark symptom is a painless discharge from the ear.[2] Hearing loss, dizziness, and facial muscle paralysis are rare but can result from continued cholesteatoma growth.[3] Surgery can stop infections and prevent complications.[4]  
Last updated: 12/30/2009

What causes cholesteatoma?

A cholesteatoma usually occurs because of poor eustachian tube function in conjunction with infection in the middle ear.[1][3] Negative pressure within the middle ear pulls a part of the eardrum the wrong way, creating a sac or cyst that fills with old skin cells and other waste material. As the cyst gets bigger, some of the middle ear bones break down, affecting hearing.[1] A rare congenital form of cholesteatoma (one present at birth) can occur in the middle ear and elsewhere, such as in the nearby skull bones.[3]
Last updated: 12/30/2009

What symptoms are associated with cholesteatoma?

Early symptoms may include drainage from the ear, sometimes with a foul odor.[1][3][4] As the cholesteatoma cyst or sac enlarges, it can lead to a full feeling or pressure in the ear, hearing loss, dizziness and pain, numbness or muscle weakness on one side of the face.[1][3] On examination, the ear drum (tympanic membrane) appears abnormal. In rare cases, a cholesteatoma may erode through the tegmen, allowing an epidural abscess to form which could lead to a more serious brain infection.[4]
Last updated: 12/30/2009

What are the chances of getting a cholesteatoma?

The incidence of cholesteatomas is unknown, but it is a relatively common reason for otologic surgery (approximately weekly in tertiary otologic practices or children's hospitals).[2][5] The overall chances of being affected by a cholesteatomas are based on factors such as frequency of ear infections. It occurs most often in individuals who have recurrent ear infections.[4] Congenital cases are rare.[3]
Last updated: 12/30/2009

How might cholesteatoma be treated?

An examination by an otolaryngologist - a doctor who specializes in head and neck conditions -  can confirm the presence of a cholesteatoma. Initial treatment may consist of a careful cleaning of the ear, antibiotics, and eardrops. Therapy aims to stop drainage in the ear by controlling the infection.[3] Large or complicated cholesteatomas may require surgical treatment to protect the patient from serious complications.[1][3]
Last updated: 12/30/2009

What is the prognosis for individuals with cholesteatoma?

Cholesteatomas usually continue to grow if not removed. Surgery is usually successful, but occasional follow-up ear cleaning by a health care provider may be necessary. Additional surgery may be needed if the cholesteatoma comes back.[1]

In rare cases, complications may arise. These include:[1]

  • Brain abscess
  • Deafness in one ear
  • Dizziness (vertigo)
  • Erosion into the facial nerve (causing facial paralysis)
  • Labyrinthitis
  • Meningitis
  • Persistent ear drainage
  • Spread of the cyst into the brain


  • Last updated: 12/30/2009

    References
    Other Names for this Disease
    • Congenital cholesteatoma (type)
    • Primary acquired cholesteatoma (type)
    • Secondary acquired cholesteatoma (type)
    See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.