Diagnosis and outcomes of middle cranial fossa repair for patients with superior semicircular canal dehiscence syndrome.

The aim of this study was to retrospectively review the clinical presentation, diagnostic features, in particular cervical vestibular evoked myogenic potentials (cVEMPs), and the outcomes of surgical repair for superior semicircular canal dehiscence syndrome (SSCDS). SSCDS is a well-described syndrome of auditory and vestibular symptoms due to a bony dehiscence of the superior semicircular canal in the middle cranial fossa. A series of six procedures on five patients with SSCDS who underwent surgical repair via a middle fossa craniotomy were retrospectively reviewed. Preoperative and postoperative audiometric and vestibular symptoms as well as investigation findings were reviewed. Auditory and vestibular symptoms improved and hearing was preserved in all patients. The low frequency pseudo-conductive loss was corrected in four out of five patients, and the lowered preoperative cVEMP thresholds normalised following successful middle cranial fossa repair. In this series, middle fossa repair of SSCD was safe and effective with excellent sensorineural hearing preservation.