title: Superior canal dehiscence length and location influences clinical presentation and audiometric and cervical vestibular-evoked myogenic potential testing.
abstract: Superior canal dehiscence (SCD) is caused by an absence of bony covering of the arcuate eminence or posteromedial aspect of the superior semicircular canal. However, the clinical presentation of SCD syndrome varies considerably, as some SCD patients are asymptomatic and others have auditory and/or vestibular complaints. In order to determine the basis for these observations, we examined the association between SCD length and location with: (1) auditory and vestibular signs and symptoms; (2) air conduction (AC) loss and air-bone gap (ABG) measured by pure-tone audiometric testing, and (3) cervical vestibular-evoked myogenic potential (cVEMP) thresholds. 104 patients (147 ears) underwent SCD length and location measurements using a novel method of measuring bone density along 0.2-mm radial CT sections. We found that patients with auditory symptoms have a larger dehiscence (median length: 4.5 vs. 2.7 mm) with a beginning closer to the ampulla (median location: 4.8 vs. 6.4 mm from ampulla) than patients with no auditory symptoms (only vestibular symptoms). An increase in AC threshold was found as the SCD length increased at 250 Hz (95% CI: 1.7-4.7), 500 Hz (95% CI: 0.7-3.5) and 1,000 Hz (95% CI: 0.0-2.5), and an increase in ABG as the SCD length increased at 250 Hz (95% CI: 2.0-5.3), 500 Hz (95% CI: 1.6-4.6) and 1,000 Hz (95% CI: 1.3-3.3) was also seen. Finally, a larger dehiscence was associated with lowered cVEMP thresholds at 250 Hz (95% CI: -4.4 to -0.3), 500 Hz (95% CI: -4.1 to -1.0), 750 Hz (95% CI: -4.2 to -0.7) and 1,000 Hz (95% CI: -3.6 to -0.5) and a starting location closer to the ampulla at 250 Hz (95% CI: 1.3-5.1), 750 Hz (95% CI: 0.2-3.3) and 1,000 Hz (95% CI: 0.6-3.5). These findings may help to explain the variation of signs and symptoms seen in patients with SCD syndrome.
authors.affiliation: Department of Otorhinolaryngology - Head and Neck Surgery, University Medical Center, Utrecht, The Netherlands.
authors.forename: Marlien E F
authors.initials: ME
authors.lastname: Niesten
authors.forename: Leena M
authors.initials: LM
authors.lastname: Hamberg
authors.forename: Joshua B
authors.initials: JB
authors.lastname: Silverman
authors.forename: Kristina V
authors.initials: KV
authors.lastname: Lou
authors.forename: Andrew A
authors.initials: AA
authors.lastname: McCall
authors.forename: Alanna
authors.initials: A
authors.lastname: Windsor
authors.forename: Hugh D
authors.initials: HD
authors.lastname: Curtin
authors.forename: Barbara S
authors.initials: BS
authors.lastname: Herrmann
authors.forename: Wilko
authors.initials: W
authors.lastname: Grolman
authors.forename: Hideko H
authors.initials: HH
authors.lastname: Nakajima
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Lee
mesh_terms: D000161:Acoustic Stimulation; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001301:Audiometry, Pure-Tone; D004427:Ear Diseases / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012665:Semicircular Canals / Q000473:pathology* / Q000503:physiopathology; D058585:Vestibular Evoked Myogenic Potentials / Q000502:physiology*; D014724:Vestibular Function Tests; D055815:Young Adult