Wildervanck's syndrome--unilateral Mondini dysplasia identified by computed tomography.
We present a case of the Wildervanck (cervico-oculo-acoustic) syndrome exhibiting congenital deafness. Klippel-Feil anomaly and lateral rectus palsy with enophthalmos. Audiometry indicated a predominantly conductive loss which, because of masking difficulties, was assumed to be bilateral: an erroneous assumption supported by results of conventional petrous bone tomography which failed to demonstrate any abnormality of the inner ears. Computed tomography (CT), however, revealed a severe Mondini dysplasia of one ear, a condition which must be assumed to be associated with severe sensorineural hearing loss. Reconstructive middle ear surgery for the conductive loss on the other side was therefore contraindicated.
West
P D
PD
Department of Otolaryngology, Manchester Royal Infirmary.
Gholkar
A
A
Ramsden
R T
RT
D002675:Child, Preschool; D003051:Cochlea / Q000002:abnormalities* / Q000000981:diagnostic imaging; D003638:Deafness / Q000151:congenital*; D004370:Duane Retraction Syndrome / Q000150:complications*; D006801:Humans; D007714:Klippel-Feil Syndrome / Q000150:complications*; D008297:Male; D009886:Ophthalmoplegia / Q000150:complications*; D013577:Syndrome; D014057:Tomography, X-Ray Computed