ObjectiveTo compare treatment response from the middle cranial fossa repair of superior canal dehiscence (SCD) between cases with and cases without low‐lying tegmen (LLT).Study DesignCohort study.SettingSingle tertiary care institution.MethodsTwo investigators independently reviewed preoperative high‐resolution temporal bone computed tomography images and classified the ipsilateral tegmen as either “low‐lying” or “control.” Patients completed a symptom questionnaire and underwent audiometric testing pre‐ and post‐operatively. Multivariable regression models assessed for symptomatic resolution and audiometric improvement following surgery with tegmen status as the primary predictor. Models controlled for patient age, sex, bilateral SCD disease, dehiscence location, prior ear surgery status, surgery duration, and follow‐up duration.ResultsAmong a total of 410 cases included, we identified 121 (29.5%) LLT cases. Accounting for all control measures, patients with LLT were significantly less likely to experience overall symptom improvement (adjusted odds ratio: 0.32, 95% confidence interval [CI]: 0.18–0.57, p < .001) and reported a significantly lower proportion of preoperative symptoms that resolved following surgery (adjusted β: −25.6%, 95% CI: −37.0% to −14.3%, p < .001). However, audiometric outcomes following surgery did not differ significantly between patients with and patients without LLT.ConclusionThis is the first investigation on the relationship between LLT and surgical outcomes following the middle fossa repair of SCD. Patients with LLT reported less favorable symptomatic response but exhibited a similar degree of audiometric improvement.