Bilateral congenital cholesteatoma of the temporal bone in Crouzon syndrome.

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 con- genital 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.