Superior semicircular canal dehiscence (SSCD) syndrome is an increasingly recognized cause of vestibular and/or auditory symptoms in both adults and children. These symptoms are believed to result from the presence of a pathological mobile "third window" into the labyrinth due to deficiency in the osseous shell, leading to inadvertent hydroacoustic transmissions through the cochlea and labyrinth. The most common bony defect of the superior canal is found over the arcuate eminence, with rare cases involving the posteromedial limb of the superior canal associated with the superior petrosal sinus. Operative intervention is indicated for intractable or debilitating symptoms that persist despite conservative management and vestibular sedation. Surgical repair can be accomplished by reconstruction or plugging of the bony defect or reinforcement of the round window through a variety of operative approaches. The authors review the etiology, pathophysiology, presentation, diagnosis, surgical options, and outcomes in the treatment of this entity, with a focus on potential pitfalls that may be encountered during clinical management.
Keywords: ECoG = electrocochleography; HRCT = high-resolution CT; ICP = intracranial pressure; SSC = superior semicircular canal; SSCD = superior semicircular canal dehiscence; VEMP = vestibular-evoked myogenic potential; dizziness; hearing loss; skull base; superior semicircular canal; superior semicircular canal dehiscence syndrome; transmastoid approach; transtemporal repair.