Superior semicircular canal dehiscence syndrome: Successful treatment with repair of the middle fossa floor: Technical case report

OBJECTIVE: Superior semicircular canal dehiscence syndrome has recently been reported as a cause of pressure-or sound-induced oscillopsia (Tullio phenomenon). We report the presentation and successful treatment of 3 patients with superior semicircular dehiscence syndrome by a joint neurosurgical/neuro- otology team. CLINICAL PRESENTATION: Patient 1 is a 37-year-old man who presented with complaints of disequilibrium, fullness in the left ear, hearing loss, and oscillopsia when pressure was applied to the left external auditory canal. Patient 2 is a 46-year-old man who presented with complaints of disequilibrium, fullness in the left ear, and blurred vision associated with heavy lifting or straining. On examination, pneumatic otoscopy produced a sense of motion. Patient 3 is a 29-year-old woman who presented with chronic disequilibrium that resulted in frequent falls. She had a positive fistula test on the left, and vertical nystagmus was elicited when pressure was applied to the left ear. In each patient, high-resolution computed tomographic scanning through the temporal bone revealed dehiscence of the superior semicircular canal on the symptomatic side. INTERVENTION: In all 3 cases, a subtemporal, extradural approach was performed with repair of the middle fossa floor using calcium phosphate BoneSource (Howmedica Leibinger, Inc., Dallas, TX). All patients recovered well, with resolution of their symptoms. CONCLUSION: Superior semicircular canal dehiscence syndrome is a cause of disequilibrium associated with sound or pressure stimuli. The workup includes a detailed history, electronystagmography including Valsalva maneuvers, and a high-resolution computed tomographic scan though the temporal bone. An extradural repair of the middle fossa floor with BoneSource can successfully treat this condition.