Giant Congenital Cholesteatoma of the Petrous Bone Personal Experience and Literature Review

Congenital cholesteatoma of the petrous bone, though originating in childhood, has often a delayed diagnosis due to its poor symptoms and can grow achieving great extension. When asymptomatic or pauci-symptomatic, the diagnosis is incidental and purely radiological. Leaving the lesion untreated implies an inexorable progression leading to extensive erosion of the petrous bone and cranial nerves involvement. First choice treatment is radical surgical excision. An aggressive treatment is recommended, a combination of approaches to the petrous bone and skull base may be used to obtain complete removal. Surgical morbidity is a balance between the choice of the approach and the goal of radical excision. Prevention of relapse is essential not to expose the patient to further morbidity some years later. Long term follow-up is essential.