doiSerbia

Bilateral congenital cholesteatoma of the temporal bone in Crouzon syndrome

Đerić Dragoslava (School of Medicine, Belgrade + Clinical Center of Serbia, Clinic of Otorhinolaryngology and Maxillofacial Surgery, Belgrade)
Čvorović Ljiljana (School of Medicine, Belgrade + Clinical Center of Serbia, Clinic of Otorhinolaryngology and Maxillofacial Surgery, Belgrade)
Blažić Srbislav (Clinical Center of Serbia, Clinic of Otorhinolaryngology and Maxillofacial Surgery, Belgrade)

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