Clinical manifestations of superior semicircular canal dehiscence

Objectives/Hypotheses: To determine the symptoms, signs, and findings on diagnostic tests in patients with clinical manifestations of superior canal dehiscence. To investigate hypotheses about the effects of superior canal dehiscence. To analyze the outcomes in patients who underwent surgical repair of the dehiscence. Study Design: Review and analysis of clinical data obtained as a part of the diagnosis and treatment of patients with superior canal dehiscence at a tertiary care referral center. Methods: Clinical manifestations of superior semicircular canal dehiscence were studied in patients identified with this abnormality over the time period of May 1995 to July 2004. Criteria for inclusion in this series were identification of the dehiscence of bone overlying the superior canal confirmed with a high-resolution temporal bone computed tomography and the presence of at least one sign on physiologic testing indicative of superior canal dehiscence. There were 65 patients who qualified for inclusion in this study on the basis of these criteria. Vestibular manifestations were present in 60 and exclusively auditory manifestations without vestibular symptoms or signs were noted in 5 patients. Results: For the 60 patients with vestibular manifestations, symptoms induced by loud sounds were noted in 54 patients and pressure-induced symptoms (coughing, sneezing, straining) were present in 44. An air-bone on audiometry in these patients with vestibular manifestations measured (mean +/- SD) 19 +/- dB at 250 Hz; 15 +/- 11 dB at 500 Hz; 11 +/- 9 dB at 1,000 Hz; and 4 +/- 6 dB at 2,000 Hz. An air-bone gap 10 dB or greater was present in 70% of ears with superior canal dehiscence tested at 250 Hz, 68% at 500 Hz, 64% at 1,000 Hz, and 21% at 2,000 Hz. Similar audiometric findings were noted in the five patients with exclusively auditory manifestations of dehiscence. The threshold for eliciting vestibular-evoked myogenic potentials from affected ears was (mean +/- SD) 81 +/- 9 dB normal hearing level. The threshold for unaffected ears was 99 +/- 7 dB, and the threshold for control ears was 98 +/- 4 dB. The thresholds in the affected ear were significantly different from both the unaffected ear and normal control thresholds (P < .001 for both comparisons). There was no difference between thresholds in the unaffected ear and normal control (P = .2). There were 20 patients who were debilitated by their symptoms and underwent surgical repair of superior canal dehiscence through a middle cranial fossa approach. Canal plugging was performed in 9 and resurfacing of the canal without plugging of the lumen in 11 patients. Complete resolution of vestibular symptoms and signs was achieved in 8 of the 9 patients after canal plugging and in 7 of the 11 patients after resurfacing. Conclusions: Superior canal dehiscence causes vestibular and auditory symptoms and signs as a consequence of the third mobile window in the inner ear created by the dehiscence. Surgical repair of the dehiscence can achieve control of the symptoms and signs. Canal plugging achieves long-term control more often than does resurfacing.