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Superior semicircular canal dehiscence (SSCD) is a rare defect of the arcuate eminence that causes an abnormal connection between the superior semicircular canal and middle cranial fossa. Patients often present with a variety of auditory and vestibular symptoms. Trigger avoidance is the initial strategy, but surgery may be necessary in debilitating cases. We retrospectively reviewed SSCD patients undergoing repair via a middle fossa craniotomy between March 2011 and September 2015. Forty-nine patients undergoing 58 surgeries were identified. Autophony was the most common symptom at presentation ( n = 44; 90%). Mean follow-up was 10.9 months, with 100% of patients reporting resolution of at least one symptom. Aural fullness was the most commonly resolved symptom following surgical repair ( n = 19/22; 86%). Hearing loss ( n = 11/25; 44%) and tinnitus ( n = 11/38; 29%) were the most common symptoms to persist following surgery. The most common symptom to develop after surgery was disequilibrium ( n = 4/18; 22%). Upon comparing the overall pre-operative and post-operative groups, the number of patients with autophony ( p < 0.0001), aural fullness ( p = 0.0006), hearing loss ( p = 0.0119), disequilibrium ( p = 0.0002), sound- and pressure-induced vertigo ( p < 0.0001), and tinnitus ( p < 0.0001) were significantly different. Improved clinical outcomes were demonstrated in patients undergoing SSCD repair through a middle cranial fossa approach. The most common presenting symptom (autophony) was also most likely to resolve after surgery. Hearing loss is less amenable to surgical correction. Disequilibrium developed in a small number of patients after repair. [ABSTRACT FROM AUTHOR]