X-linked hearing loss

mixed conductive and sensorineural hearing loss, occurring through an X-linked mode of inheritance. A distinctive inner ear deformity has been described, consisting of a wide bulbous internal auditory canal and deficient or absent bone between the lateral end of the internal auditory canal and the basal turn of the cochlea. This results in an abnormal communication between the subarachnoid space and the perilymph in the cochlea, leading to perilymphatic hydrops and a gusher if stapedectomy is attempted.

RH