 | THE VESTIBULAR LABYRINTH IN HEALTH AND DISEASE
Copyright © 2001 by the New York Academy of Sciences
description
Annals of the New York Academy of Sciences 942:259-273 (2001)
© 2001 New York Academy of Sciences
Symptoms and Signs in Superior Canal Dehiscence Syndrome
LLOYD B. MINORa,
PHILLIP D. CREMERa,b,
JOHN P. CAREYa,
CHARLES C. DELLA SANTINAa,
SVEN-OLRIK STREUBELa AND
NOAH WEGc
aDepartment of Otolaryngology—Head & Neck Surgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA
bEye and Ear Research Unit, Institute of Clinical Neurosciences, Royal Prince Alfred Hospital, Sydney, Australia
cDr. Noah Weg and Associates, 4 South Airmont Road, Suffern, New York 10901, USA
Address for correspondence: Dr. Lloyd B. Minor, Department of Otolaryngology—Head & Neck Surgery, Johns Hopkins Outpatient Center, Rm 6253, 601 N. Caroline St., Baltimore, MD 21287-0910. Voice: (410) 955-3403; fax: (410) 955-0035.
lminor{at}jhmi.edu
Patients with superior canal dehiscence (SCD) syndrome experience vertigo and oscillopsia in response to loud sounds and to stimuli that result in changes in middle ear or intracranial pressure. They may also experience hyperacusis to bone-conducted sounds. The evoked eye movements in this syndrome align with the plane of the dehiscent superior canal. The symptoms and signs can be understood in terms of the effect of the dehiscence in creation of a third mobile window into the inner ear. The SCD syndrome has been diagnosed in 28 patients who were examined in the neuro-otology clinics at the Johns Hopkins Medical Institutions from May 1995 through January 2001. The diagnosis is best established based upon the symptoms that are characteristic for the syndrome, the vertical-torsional eye movements evoked by sound or pressure stimuli noted on examination performed with Frenzel goggles, the lowered thresholds for responses to vestibular-evoked myogenic potentials, and CT imaging of the temporal bones.
Key Words: Vertigo • Oscillopsia • Nystagmus • Semicircular canal • Vestibulo-ocular reflex • Temporal bone
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