A patient with Turner's syndrome presented with a rare anomaly of absent oval window, inferiorly placed facial nerve, and abnormal stapes. To our knowledge, this is the first report of this combination of malformations.
We report an extremely rare case of duplication of the internal auditory canal associated with dysfunction of both the facial and vestibulocochlear nerves. We also review the literature regarding the integrity of the facial and vestibulocochlear nerves in such cases.