Reversible electrocochleographic abnormalities in superior canal dehiscence

OBJECTIVE: Electrocochleography (ECoG) is often used in the evaluation of episodic vertigo, and abnormal findings are commonly thought to be due to endolymphatic hydrops. We have observed that a number of patients with abnormal ECoG were ultimately found to have superior semicircular canal dehiscence (SSCD). Therefore, we examined the electrocochleographic findings in a series of patients with documented SSCD. STUDY DESIGN: Retrospective case series. SETTING: Academic medical center. PATIENTS: Seven adult patients with unilateral SSCD and 4 patients with bilateral SSCD who underwent tympanic ECoG as part of a diagnostic or preoperative evaluation that also included vestibular-evoked myogenic potentials (VEMPs). INTERVENTIONS: Patients underwent audiometric testing, ECoG, VEMP, and high-resolution temporal bone computed tomography reformatted to optimally view the superior semicircular canal. Five patients underwent superior canal obliteration. Postoperative VEMP and ECoG were performed in 4 of these patients. Intraoperative continuous ECoG was performed in 1 patient. MAIN OUTCOME MEASURES: Summating potential to action potential (SP/AP) ratio on ECoG. RESULTS: Fourteen of 15 ears confirmed to have SSCD on computed tomographic imaging were found to have an elevated SP/AP ratio (defined as >0.40). In one patient with bilateral SSCD, the ear with the radiographically less severe dehiscence had an SP/AP ratio of 0.40, at the upper limit of normal, and a normal VEMP threshold. In all 4 patients who underwent obliteration of the dehiscent canal, and for whom postoperative test results were available, the SP/AP ratio normalized in the operated ear. In the 1 patient who underwent intraoperative ECoG, the SP/AP ratio normalized immediately after canal occlusion. CONCLUSION: An elevated SP/AP ratio seems to be a consistent finding in SSCD syndrome and, like the other abnormal audiometric and electrophysiologic findings associated with the syndrome, normalizes after surgical correction. Elevation of the SP/AP ratio has historically been associated with endolymphatic hydrops. The present findings expand the differential diagnosis of an abnormal ECoG and may shed light on the origin of an elevated summating potential.