Superior canal dehiscence syndrome
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Superior canal dehiscence syndrome (SCDS) is a relatively rare neurotological disorder that is characterized by a heterogeneous clinical picture. Recently, vestibular evoked myogenic potential (VEMP) measurement was established for the diagnosis of SCDS. In the present study, a case series of patients with SCDS were analyzed, with a focus on VEMP.
Four patients with SCDS were prospectively examined with ocular VEMP (oVEMP) and cervical VEMP (cVEMP). The clinical features and the standard audiovestibular test battery results are summarized and analyzed. The diagnostic accuracy of VEMP testing is evaluated.
The increased oVEMP amplitudes had a specificity of 100% in this patient population. All patients had normal caloric function and head impulse testing. The Tullio sign was observed in two patients. Three patients had autophony. The air-bone gap was not greater than 10 dB in any of the patients. Two patients had marked fremitus nystagmus. All patients had a bony dehiscence of the superior semicircular canal on computed tomography imaging.
The subjective and clinical features in this case series of SCDS patients were heterogeneous. However, objective oVEMP testing had the highest diagnostic value. Furthermore, we describe a new diagnostic clinical sign: fremitus nystagmus.
KeywordsInner ear disease Vestibular evoked myogenic potentials Vestibulum auris Vertigo Dizziness
Dehiszenzsyndrom des oberen Bogengangs
We are grateful to David Zee, Lloyd Minor, Paul Carey, Brian Ward for valuable scientific discussions and to Beatrix Lentge and Andrea Roßkopf for excellent technical assistance.
Compliance with ethical guidelines
Conflict of interest
R. Gürkov has received research grants from the BMBF, VolkswagenStiftung, outside the submitted work, and consulting fees in the past from Otonomy Inc. C. Jerin, W. Flatz, and R. Maxwell declare that they have no competing interests.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Informed consent was obtained from all individual participants included in the study.
The supplement containing this article is not sponsored by industry.
Video: Recording of the eye movements under Frenzel goggles, while the patient with left-sided superior canal dehiscence syndrome produces a humming sound (“hmmmmm”). The fast phase of this fremitus nystagmus has two components: a vertical–downward component and a torsional–counterclockwise component. © R. Gürkov, all rights reserved.
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