Learning Objectives: Both congenital and acquired cholesteatomas are found in the petrous temporal bone, with the latter being more common. Congenital cholesteatomas arising in the apex erode from the medial aspect involving the facial nerve more frequently in its IAC and labyrinthine segments. Congenital middle ear cholesteatomas tend to present earlier with a conductive hearing loss and if left untreated can erode along the facial nerve superior to the cochlea involving the geniculate ganglion and labyrinthine segments. Acquired cholesteatomas involving the apex in contrast all arise in the tympanomastoid region and extend medially. These extensive acquired cholesteatomas can involve multiple segments of the intratemporal nerve from IAC to stylomastoid foramen. In the authors experience 50% of cases of apical cholesteatoma had facial nerve involvement at presentation.

The management of apical disease remains contentious. While the aim of surgery should be complete excision of disease, this should be balanced against achieving a good neurological outcome for the patient. Complete excision is often complicated by limitations in access and the tight adherence of cholesteatoma matrix to key structures such as the facial nerve, internal carotid artery and dura. The author would typically not sacrifice a functioning facial nerve in order to improve the chance of complete excision of disease. Using this approach long-term disease control and good facial motor function can be achieved in both congenital and acquired apical cholesteatoma.