title: Congenital cholesteatoma of the mastoid temporal bone.
abstract: OBJECTIVE
  Congenital mastoid cholesteatomas are rare lesions of the temporal bone. The clinical presentation of these lesions is variable, making them difficult to identify preoperatively. We evaluated our series of mastoid congenital cholesteatomas (CCs) in an effort to better define the clinical presentation, imaging characteristics, and surgical challenges specific to this lesion.
  
  
  METHODS
  Retrospective chart and radiologic study review.
  
  
  METHODS
  The medical records of patients with the diagnosis of mastoid CC on radiologic imaging over a 15-year period were reviewed. All had surgical and pathologic confirmation. Eight patients underwent preoperative computed tomography (CT). Six also underwent magnetic resonance (MR) scanning. Demographic information, clinical presentation, imaging results, and operative findings were recorded.
  
  
  RESULTS
  Nine patients with the diagnosis of mastoid CC satisfying the inclusion criteria were found. Clinical findings were variable, with the most common presentation being an incidental finding. Imaging findings were more uniform. All CT scans demonstrated an expansile, well-circumscribed mass centered within the mastoid portion of the temporal bone. All MR scans showed a well-circumscribed mass with high intensity on T2-weighted images with precontrasted T1 sequences showing the lesion to be isointense or slightly hyperintense to cerebrospinal fluid (CSF). Operative findings included lateral mastoid cortex erosion, sigmoid sinus exposure, ossicular destruction, facial nerve exposure, and associated postauricular abscess. Management of these lesions is reviewed.
  
  
  CONCLUSIONS
  Congenital mastoid cholesteatomas have a variable and nonspecific clinical presentation. Surgical challenges arise from the indolent nature of this clinical entity, which belies the extent of otologic involvement. Imaging with CT and magnetic resonance imaging are diagnostic, defines the extent of these lesions, and facilitates preoperative surgical planning.
authors.affiliation: Division of Otolaryngology, University of Utah, Salt Lake City, Utah, USA. frank.warren@earthlink.net
authors.forename: Frank M
authors.initials: FM
authors.lastname: Warren
authors.forename: Marc L
authors.initials: ML
authors.lastname: Bennett
authors.forename: Richard H
authors.initials: RH
authors.lastname: Wiggins
authors.forename: Karen L
authors.initials: KL
authors.lastname: Saltzman
authors.forename: Katherine S
authors.initials: KS
authors.lastname: Blevins
authors.forename: Clough
authors.initials: C
authors.lastname: Shelton
authors.forename: H Ric
authors.initials: HR
authors.lastname: Harnsberger
mesh_terms: D000293:Adolescent; D000328:Adult; D001301:Audiometry, Pure-Tone; D002648:Child; D002675:Child, Preschool; D002781:Cholesteatoma / Q000151:congenital / Q000175:diagnosis / Q000601:surgery; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D008416:Mastoid; D012189:Retrospective Studies; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed