Otological findings in cervico-oculo-auditory dysplasia

Publisher Website
Google Scholar
Abstract
A patient with congenital ear dysplasia, combined with cleft palate and anomalies of the cervical spine and eyes, is described. The case belongs to the group of cervico-ocular-auditory displasia (Klippel-Feil syndrome). A strange feature in this case was the coexistence of conductive hypakousia in the right ear, due to congenital fixation of the stapes footplate, with perceptive deafness in the left ear, due to dysplasia of the cochlea and internal auditory meatus. Conductive hypakousia in the right ear was successfully treated by stapedectomy. In some Klippel-Feil syndrome patients conductive deafness is unexplained; in others deafness is due to dysplasia of the ossicular chain or to a combination of dysplasias of the labyrinth and middle ear in the same ear. The coexistence of middle ear dysplasia in 1 ear with contralateral dysplasia of the inner ear and internal auditory meatus is rate.