Facial nerve paralysis as presenting sign of congenital cholesteatoma in an adult

Abstract

Congenital cholesteatomas are epidermoid cysts that develop from epithelial remnants within the temporal bone. While commonly diagnosed in children, adult presentations are rare and can pose diagnostic challenges. We present a 53-year-old man with a nine-month history of right-sided facial paralysis, hearing loss, and headaches. Examination revealed House-Brackmann grade VI facial nerve dysfunction. Imaging showed an expansile lesion involving the mastoid, eroding the otic capsule, facial nerve canal, and tegmen with extension into the middle cranial fossa. The patient underwent a middle fossa craniotomy for complete resection of the lesion, which was confirmed to be a congenital cholesteatoma. Despite gross-total removal, facial nerve function did not recover, likely due to the long-standing paralysis. This case highlights the importance of considering congenital cholesteatoma in the differential diagnosis of facial paralysis in adults. Prompt recognition and surgical intervention are critical to prevent disease progression and optimize outcomes.