Primary Mastoid Cholesteatoma: A Case Report and Review of the Literature.

Congenital cholesteatomas are defined as a collection of epithelium in the absence of prior surgery or pathologic retraction. They are most commonly found in the middle ear and are thought to arise from a residual epithelial rest present since birth; however, a small number of cases present with disease isolated to the mastoid bone. One such case and a review of prior reports are presented. A 29-year-old male with a 2-year history of headaches and no otologic surgery was found to have a destructive mass centered in the right mastoid bone, invading the jugular foramen and posterior fossa without middle ear disease. He underwent a mastoidectomy with complete resection of the cholesteatoma. A literature review identified 21 prior cases of isolated mastoid congenital cholesteatoma. Congenital cholesteatoma should be considered in the differential diagnosis when patients present with postauricular pain or headaches. Mastoidectomy is considered the treatment of choice.