Cholesteatoma

Cholesteatoma
Cholesteatoma
Classification and external resources

Cholesteatoma
ICD-10 H71
ICD-9 385.32
DiseasesDB 2553
eMedicine ped/384 ent/220
MeSH D002781

Cholesteatoma is a destructive and expanding growth consisting of keratinizing squamous epithelium in the middle ear and/or mastoid process.

Contents

Signs and symptoms

The patient may have a recurrent ear discharge. Granulation tissue and a discharge (through a marginal perforation of the ear drum) may be seen on examination. A cholesteatoma cyst consists of desquamating (peeling) layers of scaly or keratinised (horny) layers of epithelium, which may also contain cholesterol crystals. Often the debris is infected with Pseudomonas aeruginosa or other bacteria or organisms.

If untreated, a cholesteatoma can eat into the three small bones located in the middle ear (the malleus, incus and stapes, collectively called ossicles), which can result in nerve deterioration, deafness, imbalance and vertigo. It can also affect and erode, through the enzymes it produces, the thin bone structure that isolates the top of the ear from the brain, as well as lay the covering of the brain open to infection with serious complications (rarely even death due to causing a brain abscess and septicemia).

Both the acquired as well as the congenital types of the disease can affect the facial nerve that extends from the brain to the face and passes through the inner and middle ear and leaves at the anterior tip of the mastoid bone, and then rises to the front of the ear and extends into the upper and lower face.

A history of ear infection or flooding of the ear during swimming should be taken seriously and investigated as cholesteatoma should be considered a possible outcome.

Common symptoms of cholesteatoma may include: Hearing loss, mucopurulent discharge from the ear (usually brown/yellow) with a strong odor, bleeding from the ear, dizziness, vertigo, balance disruption, ear ache, headaches or tinnitus. There can also be facial nerve weakness.

Causes

There are two types: congenital and acquired. Acquired cholesteatomas, which are more common, can be caused by a tear or retraction of the ear drum.

Cholesteatoma is an epidermoid cyst of the middle ear and/or mastoid. It developed as a consequence of eustachian tube dysfunction and chronic otitis media, secondary to retraction of squameous elements of the tympanic membrane into the middle ear space. Those squamous elements cause bone destruction secondary to its expanding nature and through enzymatic destruction.[1]

Less commonly the disease may be congenital, when it grows from birth behind the eardrum. Congenital cholesteatomas are more often found in the anterior aspect of the ear drum, in contrast to acquired cholesteatomas that usually arise from the pars flaccida region of the ear drum in the posterior-superior aspect of the ear drum.

Pathophysiology

The status of cholesteatomas as tumors is currently unresolved. There is some evidence to support the hypothesis that cholesteatomas are low-grade tumors.[2] However, recent studies have failed to show consistent DNA instability in cholesteatomas.[3]

Treatment

Surgery is performed to remove the sac of squamous debris and a mastoidectomy is performed. Cholesteatomas of the middle ear may be congenital and in some cases can be removed through the ear canal. The majority of cholesteatomas require that an incision be made behind the ear to expose the affected area adequately.

Prognosis

Even after careful microscopic surgical removal, 10% to 20% of cholesteatomas may recur, which then require follow-up checks and/or treatment.

References

  1. ^ Schwartz's principles of surgery: self assessment and board review, 8th edition, page 123 question 1
  2. ^ Bollmann R, Knopp U, Tolsdorff P (1991). "[DNA cytometric studies of cholesteatoma of the middle ear]". HNO 39 (8): 313–4. PMID 1938497. 
  3. ^ Desloge RB, Carew JF, Finstad CL, Steiner MG, Sassoon J, Levenson MJ, Staiano-Coico L, Parisier SC, Albino AP (1997). "DNA analysis of human cholesteatomas". Am J Otol 18 (2): 155–9. PMID 9093669. 

External links

v · d · eDiseases of the ear and mastoid process (H60–H99, 380–389)
Outer ear
Middle ear and mastoid
Inner ear and
central pathways
Common pathway
Excessive response
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Acquired auditory processing disorder  · Spatial hearing loss

M: EAR

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  • cholesteatoma — 1. A mass of keratinized squamous cell epithelium and cholesterol in the middle ear, usually resulting from chronic otitis media, with squamous metaplasia or …   Medical dictionary

  • cholesteatoma — n. a skin lined sac containing debris from dead skin cells that grows from the eardrum into the mastoid bone, eroding normal structures in its path. Left untreated, it can carry infection to the brain, causing meningitis or a cerebral abscess.… …   The new mediacal dictionary

  • cholesteatoma tympani — a type of cholesteatoma usually associated with chronic infection of the middle ear, formed of the outer desquamating layers of stratified squamous epithelium that have extended inward and upward to line the tympanum, epitympanum, and antrum …   Medical dictionary

  • cholesteatoma — noun A destructive and expanding keratinizing squamous epithelium in the middle ear and/or mastoid process …   Wiktionary

  • cholesteatoma — cho·les·te·a·to·ma …   English syllables

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