title: Bilateral congenital cholesteatoma of the temporal bone in Crouzon syndrome.
abstract: BACKGROUND
  Crouzon syndrome is an autosomal dominant genetic disease characterized by bicoronal craniosynostosis, exorbitism with hypertelorism, and maxillary hypoplasia with mandibular prognathism.
  
  
  METHODS
  We present the first reported case of Crouzon syndrome associated with a bilateral con- genital cholesteatoma of the temporal bone and discuss about the potential pathogenesis.
  
  
  CONCLUSIONS
  Early diagnosis and management are crucial to prevent complications and an otologist should be an integral part of the multidisciplinary team.
authors.forename: Dragoslava
authors.initials: D
authors.lastname: Djerić
authors.forename: Ljiljana
authors.initials: L
authors.lastname: Čvorović
authors.forename: Srbislav
authors.initials: S
authors.lastname: Blažić
mesh_terms: D002781:Cholesteatoma / Q000473:pathology*; D003394:Craniofacial Dysostosis / Q000473:pathology*; D003398:Craniosynostoses; D006801:Humans; D013701:Temporal Bone / Q000473:pathology