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Wildervanck's syndrome—unilateral Mondini dysplasia identified by computed tomography

Published online by Cambridge University Press:  29 June 2007

P. D. B. West ,
A. Gholkar  and
R. T. Ramsden
P. D. B. West*
Affiliation:
Department of Otolaryngology, Manchester Royal Infirmary.
A. Gholkar
Affiliation:
Department of Diagnostic Radiology, University of Manchester.
R. T. Ramsden
Affiliation:
Department of Otolaryngology, Manchester Royal Infirmary.
*
P. D. B. West, F.R.C.S., 13 Gildridge Road, Chorlton, Manchester M16 8PS.
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Abstract

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.

Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 103 , Issue 4 , April 1989 , pp. 408 - 411
Copyright
Copyright © JLO (1984) Limited 1989

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