Section Editor: Sandy Cheng-Yu Chen, M.D.
Taipei Medical University Hospital, Taipei, Taiwan
Congenital X-linked mixed hearing loss, or stapes gusher, is a form of congenital mixed hearing loss caused by a fistulous connection between the basal turn of the cochlea and the internal auditory canal (arrows) due to deficiency of the laminae cribosa (A, open arrow). The coronal image also demonstrates the fistulous connection (B, open arrow). A cochlear implant is also present (asterisks). Attempted stapedectomy can result in a perilymph gusher, as perforating the footplate of the stapes allows the freely communicating perilymph of the cochlea and CSF of the internal auditory canal to fill the middle ear cavity.