Congenital Cholesteatoma of the Sphenoid, Occipital, and Temporal Bones: 54-Year Follow Up.

To provide long-term follow up of a unique patient with history of massive petrous apex congenital cholesteatoma. 75-year-old man who presented at age 18 with left Gradenigo-like syndrome. Staged left radical mastoidectomy and open transsphenoidal marsupialization. Followed with routine in-office cholesteatoma debridement. Bony erosion on computed tomography (CT), monitoring for new symptoms. Interval development of sensorineural component of hearing loss due to cochlear fistulization. Very slow skull base bony erosion continues without neck destabilization. No new or recurrent cranial neuropathies have developed. Marsupialized cholesteatoma may be followed with routine debridement and interval imaging for monitoring over many decades.