Congenital aural atresia (CAA) is usually accompanied by an anomalous course of facial nerves, which may run over the round window niche toward the promontory in some cases of CAA. Very rarely, cochlear malformation might coexist at the same ear which has CAA, in which case cochlear implantation can be difficult due to anomalous facial nerve. Herein we present a case of cochlear implantation performed in a child who had CAA along with cochlear hypoplasia and cochlear nerve aplasia. The mastoid segment of facial nerve was displaced anteriorly, running over the round window niche. The surgery was done successfully using the round window approach without facial nerve injury.
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