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 salivary choristomata and other anomalies of the middle and inner ears.
Peron DL, Schuknecht HF. Congenital Cholesteatomata With Other Anomalies. Arch Otolaryngol. 1975;101(8):498–505. doi:10.1001/archotol.1975.00780370040015
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