Electrocochleographic findings in superior canal dehiscence syndrome

Abstract

This study evaluated the electrocochleographic findings of patients with superior canal dehiscence (SCD) syndrome and determined their diagnostic values and relationships with audiometric parameters. Thirteen symptomatic SCD patients (1 bilateral) confirmed by temporal bone computed tomography (TBCT) and cervical vestibular evoked myogenic potentials (cVEMP) were recruited. SCD sizes were measured on reformatted images in the plane of the superior canal (SC). Results of audiologic tests (audiometry, cVEMP, electrocochleography (ECoG)) for 14 affected and 12 contralateral unaffected ears were evaluated. Relationships between summating potential (SP) to action potential (AP) ratios, as measured by ECoG, and other audiometric parameters were evaluated. Sensitivity analysis of SP/AP ratios was performed by plotting receiver operating characteristic (ROC) curves for SCD syndrome patients and 19 age-matched healthy controls. Mean SP/AP ratio of SCD ears was significantly higher than that of unaffected ears (0.52 versus 0.25, p < 0.001) and SPs were significantly elevated in affected ears (p = 0.011), whereas APs were similar for affected and unaffected ears. SP/AP ratio showed a sensitivity of 92.3% and a specificity of 94.0% for distinguishing SCD syndrome patients given the inclusion criteria applied (symptoms, TBCT, cVEMP threshold) at a cutoff value of 0.34 (p < 0.001). SP/AP ratio was not correlated with SCD size or cVEMP threshold in affected ears. Negative absolute values of bone conduction at low frequency tended to increase with SP/AP ratio. Five out of 13 patients underwent surgical repair experienced symptomatic improvement with normalization of SP/AP ratios. ECoG appears to be a valuable diagnostic adjunct for functional demonstration of the third window in the otic capsule with high sensitivity and specificity, and thus, can support a clinical diagnosis of SCD when used in conjunction with clinical and radiological findings.