• Journal of neurosurgery · Dec 2017

    Review

    Superior semicircular canal dehiscence syndrome.

    • Wenya Linda Bi, Ryan Brewster, Dennis Poe, David Vernick, Daniel J Lee, Eduardo CorralesCC3Department of Otolaryngology, Harvard Medical School.5Division of Otolaryngology, Brigham and Women's Hospital, Boston, Massachusetts., and Ian F Dunn.
    • 1Center for Skull Base and Pituitary Surgery, Department of Neurosurgery, Brigham and Women's Hospital.
    • J. Neurosurg. 2017 Dec 1; 127 (6): 1268-1276.

    AbstractSuperior 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.

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