Congenital (primary) cholesteatomata may arise in the petrous apex, mastoid, middle ear, or external auditory canal. Histological studies of the temporal bones of a 20-year old man with bilateral combined deafness show bilateral congenital cholesteatomata of the middle ears associated with dalivary choristomata and other anomalies of the middle and inner ears.
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Peron et al. (1975) studied this question.
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