Superior canal dehiscence syndrome: Diagnosis with vestibular evoked myogenic potentials and fremitus nystagmus

Background 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 serie...

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Bibliographic Details
Published in:HNO 2018, Vol.66 (Suppl 1), p.28-33
Main Authors: Gürkov, R., Jerin, C., Flatz, W., Maxwell, R.
Format: Article
Language:English
Subjects:
Adult
Allergology
Female
Head and Neck Surgery
Humans
Labyrinth Diseases - diagnosis
Male
Medicine
Medicine & Public Health
Middle Aged
Original Articles
Otorhinolaryngology
Plastic Surgery
Semicircular Canals
Vestibular Diseases - diagnosis
Vestibular Evoked Myogenic Potentials
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Summary:Background 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. Methods 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. Results 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. Conclusion 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.
ISSN:0017-6192
1433-0458
DOI:10.1007/s00106-017-0441-x