Wildervanck's syndrome--unilateral Mondini dysplasia identified by computed tomography.

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.

• Authors: P D West, A Gholkar, R T Ramsden
• Journal: The Journal of laryngology and otology (J Laryngol Otol) Vol. 103 Issue 4 Pg. 408-11 (Apr 1989) ISSN: 0022-2151 [Print] England
• PMID: 2715696 (Publication Type: Case Reports, Journal Article)

Topics

• Child, Preschool
• Cochlea (abnormalities, diagnostic imaging)
• Deafness (congenital)
• Duane Retraction Syndrome (complications)
• Humans
• Klippel-Feil Syndrome (complications)
• Male
• Ophthalmoplegia (complications)
• Syndrome
• Tomography, X-Ray Computed