title: Non-classical presentation of congenital cholesteatoma as cerebrospinal fluid rhinorrhea - Case report and systematic review of the literature.
abstract: OBJECTIVE
  Congenital cholesteatoma (CC) becomes clinically apparent as a cholesteatoma usually during childhood. Nontraumatic otogenic cerebrospinal fluid (CSF) rhinorrhea with an intact tympanic membrane is a very rare symptom.
  
  
  METHODS
  The review of recent literature and case report of the 60-year old patient - a trumpeter presented with nontraumatic otogenic CSF rhinorrhea, intact tympanic membrane on microotoscopy, and besides colorless fluid in right nasal cavity, normal finding on nasal endoscopy examination.
  
  
  RESULTS
  CSF rhinorrhea was caused by CC in the petrous bone apex. Early diagnosis was facilitated by computed tomography scanning. Complete cholesteatoma removal was accomplished using a middle fossa craniotomy and an open non-radical antromastoidectomy.
  
  
  CONCLUSIONS
  The diagnosis of otogenic cerebrospinal fluid (CSF) rhinorrhea is challenging and it can easily be misdiagnosed. Congenital cholesteatoma is a rare entity. We present a non-classical presentation of CC in an adult male, with a previously unreported symptom of CSF rhinorrhea. Symptomatic improvement occurred after surgical treatment of the disease.
authors.affiliation: Department of Otolaryngology, Division of Dentistry, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Karolina
authors.initials: K
authors.lastname: Dzaman
authors.affiliation: Department of Otolaryngology, Division of Dentistry, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Malgorzata
authors.initials: M
authors.lastname: Tomaszewska
authors.affiliation: Department of Otolaryngology, Division of Dentistry, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Antoni
authors.initials: A
authors.lastname: Krzeski
authors.affiliation: Department of Otolaryngology, Division of Dentistry, Medical University of Warsaw, Warsaw, Poland. Electronic address: popkom@interia.pl.
authors.forename: Mariola
authors.initials: M
authors.lastname: Zagor
mesh_terms: D002559:Cerebrospinal Fluid Rhinorrhea / Q000175:diagnosis* / Q000209:etiology; D002781:Cholesteatoma / Q000150:complications / Q000151:congenital* / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome
keywords: Cerebrospinal fluid leak; Congenital cholesteatoma; Otogenic rhinorrhea; Otorhinorrhea