II. Pathophysiology

  1. Benign squamous metaplasia of middle ear epithelium
    1. TM epithelium retracts into mastoid air cells
    2. Mass gradually expands and keratinizes
    3. Forms epithelial inclusion cyst
    4. Contains Cholesterol crystals and keratinous debris
  2. Results in destruction of middle Ear Ossicles
    1. Conductive Hearing Loss

III. Cause

  1. Congenital Cholesteatoma
  2. Acquired Cholesteatoma
    1. Chronic or recurrent Otitis Media
    2. Tympanic Membrane Rupture

IV. Symptoms

  1. Otalgia or Ear fullness
    1. Ear Pain is a late finding
  2. Headache
  3. Hearing Loss
    1. Gradual onset
  4. Otorrhea
    1. Chronic suppurative discharge from middle ear
    2. Via perforated Tympanic Membrane

V. Signs

  1. Middle ear Deafness
  2. Otoscope Exam
    1. Pearly gray-white middle ear mass of debris behind the Tympanic Membrane

VI. Imaging

  1. Non-contrast CT of Temporal Bone
    1. Bony erosion and enlargement of middle ear
    2. Mastoid process with air cell opacification

VII. Management

  1. Surgical excision of Cholesteatoma
    1. Often with mastoidectomy, Ossicular Chain reconstruction
  2. Second look procedure may be scheduled at 6 months

VIII. Complications

  1. Conductive Hearing Loss
  2. Mastoiditis (most common cause)

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Ontology: Cholesteatoma (C0008373)

Definition (NCI) A pathologic process characterized by the proliferation of keratinizing squamous epithelium resulting in the accumulation of keratin and cells in the middle ear and/or mastoid. It may be congenital or acquired. If left untreated, it may increase in size and destroy adjacent structures.
Definition (CSP) non-neoplastic keratinizing mass with stratified squamous epithelium, frequently occurring in the meninges, central nervous system, bones of the skull, and most commonly in the middle ear and mastoid region.
Definition (MSH) A non-neoplastic mass of keratin-producing squamous EPITHELIUM, frequently occurring in the MENINGES; bones of the skull, and most commonly in the MIDDLE EAR and MASTOID region. Cholesteatoma can be congenital or acquired. Cholesteatoma is not a tumor nor is it associated with high CHOLESTEROL.
Concepts Disease or Syndrome (T047)
MSH D002781
ICD9 385.30
ICD10 H71.9 , H71.90
SnomedCT 194338004, 155244001, 267766007, 575006, 363668000
English Cholesteatomas, Cholesteatoma, unspecified, cholesteatoma (diagnosis), cholesteatoma, Cholesteatoma NOS, Unspecified cholesteatoma, Unspecified cholesteatoma, unspecified ear, Cholesteatoma [Disease/Finding], cholesteatomas, Cholesteatoma, Unspecified cholesteatoma (morphologic abnormality), Epidermoid cholesteatoma, Cholesteatoma (disorder), Cholesteatoma (morphologic abnormality), Cholesteatoma, NOS
Dutch cholesteatoom, niet-gespecificeerd, cholesteatoom, Cholesteatoom
French Cholestéatome, non précisé, Cholestéatome
German Cholestatom, unspezifisch, Cholestatom, Cholesteatom
Italian Colesteatoma, non specificato, Colesteatoma
Portuguese Colesteatoma NE, Colesteatoma
Spanish Colesteatoma no especificado, colesteatoma (anomalía morfológica), colesteatoma (trastorno), colesteatoma epidermoide, colesteatoma, Colesteatoma
Japanese 真珠腫, 詳細不明の真珠腫, ショウサイフメイノシンジュシュ, シンジュシュ
Swedish Kolesteatom
Czech cholesteatom, Cholesteatom, Cholesteatom, blíže neurčený
Finnish Kolesteatooma
Polish Perlak
Hungarian Cholesteatoma, Nem meghatározott cholesteatoma
Norwegian Kolesteatom, Perlemorsvulst