Bilateral Congenital Cholesteatoma of the Temporal Bone in Crouzon Syndrome

Abstract

Introduction Crouzon syndrome is an autosomal dominant genetic disease characterized by bicoronal craniosynostosis, exorbitism with hypertelorism, and maxillary hypoplasia with mandibular prognathism.

Case Outline We present the first reported case of Crouzon syndrome associated with a bilateral congenital cholesteatoma of the temporal bone and discuss about the potential pathogenesis.

Conclusion Early diagnosis and management are crucial to prevent complications and an otologist should be an integral part of the multidisciplinary team.

Keywords: Crouzon syndrome; bilateral congenital cholesteatoma; hearing loss