Congenital Cholesteatoma of Petrous Apex – case report and review of literature

Abstract

Objective: Congenital cholesteatomas of the petrous apex are rare lesions that develop insidiously, which delays diagnosis and requires a high index of suspicion. A case report of supralabyrinthine congenital petrous bone cholesteatoma and review of recent literature are presented. Methods: A 27-year-old woman presented with progressive facial palsy. Otomicroscopy did not reveal any abnormalities, and hearing was normal. Although there were no complaints of vertigo or instability, vestibular examinations indicated a non-compensated peripheral vestibular lesion. Radiological examinations revealed a lesion in the petrous apex and epitympanum that had features of a cholesteatoma. Results: To preserve normal hearing, the middle fossa approach was chosen for surgery. A damaged part of the facial nerve was reconstructed with cable graft. Conclusion: Advances in radiological imaging facilitate surgical planning, and improvements in the techniques of lateral skull base surgery enable safe and radical removal of petrous bone cholesteatomas with minimal morbidity.