Learning Objectives: Cholesteatoma can exist for many years with little or no symptoms before presentation with life-threatening complications. Patients with persisting ear discharge should undergo CT scanning of the temporal bones.

Introduction: Cholesteatoma is usually acquired. Congenital cholesteatoma is rare and occurs at three important sites: the middle ear, the Petrous apex, and CPA. For the diagnosis to be made the following three conditions should normally apply: there should be a mass medial to the tympanic membrane; the tympanic membrane should be normal and intact and there should be no previous history of ear discharge, perforation or ear surgery. Presentation of congenital middle ear cholesteatoma is normally as a conductive hearing loss in childhood. Petrous apex and cerobellopotine cholesteatomas may present with CPA symptoms or be picked up as in incidental radiological finding in early adult life.

Method: A 54 year old man presented with a short history of hearing loss and ear discharge. He was treated for otitis externa and wax. Microsuction was performed several times before a CT scan of the temporal bones was requested which showed a massive erosive lesion consistent with a giant cholesteatoma (images). As he was leaving the consultation he asked for further micro suction. This provoked a profuse CSF leak. Urgent tertiary referral was made and the patient underwent craniotomy and petrosectomy(operative photographs).

Results: The patient recovered well with no cranial nerve deficits or other complications and is managing well a CROS hearing aid. Four years on he remains well with no sign of recurrence on two diffusion weighted MRI scans(images). He remains under lifelong surveillance.

Conclusion: Congenital cholesteatoma can remain silent for many years presenting late in life as a giant cholesteatoma with bony erosion and extension into the cranial cavity. CT and diffusion weighted MRI imaging can help in diagnosis and pre-operative planning.