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OBJECTIVE: We report an extremely rare case of congenital cholesteatoma affecting the occipital bone. METHODS: We present a case report, plus a review of the world literature on similar lesions. RESULTS: This case report describes the presentation and treatment of a congenital cholesteatoma arising in an apparently unique location within the occipital bone, with no effect on middle-ear structure or function. The different imaging characteristics of this lesion are described and illustrated. The discussion centres on the differentiation of this lesion from intradiploic epidermoid cysts, more commonly described in the neurosurgical literature. The possible methods of pathogenesis are discussed, along with treatment suggestions. CONCLUSION: Congenital cholesteatomas and intradiploic epidermoid cysts are indistinguishable both histologically and radiologically, and would appear to be the same disease.

Original publication




Journal article


J Laryngol Otol

Publication Date





673 - 675


Bone Diseases, Cholesteatoma, Epidermal Cyst, Female, Humans, Magnetic Resonance Imaging, Middle Aged, Occipital Bone, Tomography, X-Ray Computed