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Superior semicircular canal dehiscence simulating otosclerosis

Published online by Cambridge University Press:  08 March 2006

G. Michael Halmagyi
Affiliation:
Neurology Department, Royal Prince Alfred Hospital, Sydney, and the Otolaryngology Department, St Vincent’s Hospital, Sydney, Australia.
Swee T. Aw
Affiliation:
Neurology Department, Royal Prince Alfred Hospital, Sydney, and the Otolaryngology Department, St Vincent’s Hospital, Sydney, Australia.
Leigh A. McGarvie
Affiliation:
Neurology Department, Royal Prince Alfred Hospital, Sydney, and the Otolaryngology Department, St Vincent’s Hospital, Sydney, Australia.
Michael J. Todd
Affiliation:
Neurology Department, Royal Prince Alfred Hospital, Sydney, and the Otolaryngology Department, St Vincent’s Hospital, Sydney, Australia.
Andrew Bradshaw
Affiliation:
Neurology Department, Royal Prince Alfred Hospital, Sydney, and the Otolaryngology Department, St Vincent’s Hospital, Sydney, Australia.
Robyn A. Yavor
Affiliation:
Neurology Department, Royal Prince Alfred Hospital, Sydney, and the Otolaryngology Department, St Vincent’s Hospital, Sydney, Australia.
Paul A. Fagan
Affiliation:
Neurology Department, Royal Prince Alfred Hospital, Sydney, and the Otolaryngology Department, St Vincent’s Hospital, Sydney, Australia.

Abstract

This is a report of a patient with an air-bone gap, thought 10 years ago to be a conductive hearing loss due to otosclerosis and treated with a stapedectomy. It now transpires that the patient actually had a conductive hearing gain due to superior semicircular canal dehiscence. In retrospect for as long as he could remember the patient had experienced cochlear hypersensitivity to bone-conducted sounds so that he could hear his own heart beat and joints move, as well as a tuning fork placed at his ankle. He also had vestibular hypersensitivity to air-conducted sounds with sound-induced eye movements (Tullio phenomenon), pressure-induced nystagmus and low-threshold, high-amplitude vestibular-evoked myogenic potentials. Furthermore some of his acoustic reflexes were preserved even after stapedectomy and two revisions. This case shows that if acoustic reflexes are preserved in a patient with an air-bone gap then the patient needs to be checked for sound- and pressure-induced nystagmus and needs to have vestibular-evoked myogenic potential testing. If there is sound- or pressure-induced nystagmus and if the vestibular-evoked myogenic potentials are also preserved, the problem is most likely in the floor of the middle fossa and not in the middle ear, and the patient needs a high-resolution spiral computed tomography (CT) of the temporal bones to show this.

Type
Research Article
Copyright
© Royal Society of Medicine Press Limited 2003

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