Congenital Cholesteatoma of the Sphenoid, Occipital, and Temporal Bones: 54-Year Follow Up. Issue 5 (June 2020)

• Record Type: Journal Article
• Authors: Maxwell, Anne K.; Muelleman, Thomas; Brackmann, Derald E.
• Is Part Of: Otology & neurotology. Volume 41:Issue 5(2020)
• Publication Date: 2020-06
• Subjects: Cochlea fistula -- Congenital cholesteatoma -- Long-term follow up -- Otology -- Skull base erosion; Otology -- Periodicals; Ear -- Diseases -- Periodicals; Skull base -- Surgery -- Periodicals; 617.8005
• DOI: 10.1097/MAO.0000000000002601
• Languages: English
• ISSNs: 1531-7129

Abstract

Abstract : Objective: To provide long-term follow up of a unique patient with history of massive petrous apex congenital cholesteatoma. Patient: 75-year-old man who presented at age 18 with left Gradenigo-like syndrome. Intervention: Staged left radical mastoidectomy and open transsphenoidal marsupialization. Followed with routine in-office cholesteatoma debridement. Main Outcome Measures: Bony erosion on computed tomography (CT), monitoring for new symptoms. Results: 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. Conclusions: Marsupialized cholesteatoma may be followed with routine debridement and interval imaging for monitoring over many decades. Abstract : Supplemental Digital Content is available in the text