Superior semicircular canal dehiscence (SCD) syndrome is a recognized condition associated with varying degrees of vestibular and auditory dysfunction. The authors present a case study of disabling SCD syndrome in a 37-year-old man who was successfully treated with a complete round window niche occlusion via a transcanal approach. This case represents the first report of a transcanal complete round window niche occlusion for the treatment of SCD syndrome. A brief literature review and discussion of surgical techniques follow.