Superior Canal Dehiscence Syndrome Associated With the Superior Petrosal Sinus in Pediatric and Adult Patients

Abstract

To determine whether pediatric and adult patients with superior canal dehiscence (SCD) at the superior petrosal sinus (SPS) develop superior canal dehiscence syndrome (SCDS). Retrospective review. Tertiary care academic medical center. Pediatric and adult patients with SPS-associated SCD were identified from a database of 131 patients with SCD based on high-resolution temporal bone computed tomography. One pediatric patient experienced incapacitating exercise-induced vertigo, and this patient's superior semicircular canal defect was plugged via a transmastoid approach. The 11 remaining patients were managed by observation. Clinical symptoms and signs, audiologic testing, vestibular evoked myogenic potentials, and radiologic data. Twelve patients, aged 15 to 84 years, with SCD caused by the SPS contacting the superior semicircular canal were identified. The most characteristic clinical feature in this population (5/12) was dizziness related to exercise and exertion. Bilateral SCD was observed in 3 patients. Eleven patients did not have severe symptoms and were managed conservatively. One patient, aged 15, required surgical intervention for incapacitating vertigo and experienced relief of symptoms with reversal of diagnostic indicators postoperatively. This is the first reported surgical repair of SCDS in a pediatric patient. This is the first series of patients who have SCDS due to contact of the SPS with the superior semicircular canal. Exercise and exertion-related symptoms are common in patients who have SCD owing to this cause. Transmastoid superior canal plugging is feasible and successful in treating SCDS in the pediatric patient.