The Prevalence of Superior Semicircular Canal Dehiscence in Patients With Mastoid Encephalocele or Cerebrospinal Fluid Otorrhea.

OBJECTIVE: To evaluate the association between mastoid encephalocele or cerebrospinal fluid (CSF) otorrhea and concurrent superior semicircular canal dehiscence (SSCD). STUDY DESIGN: Retrospective case-control study with chart and imaging review. SETTING: University-affiliated tertiary referral center. PATIENTS: A chart review was conducted of all patients greater than 18 years of age who had primary surgery for CSF otorrhea or encephalocele at our university-affiliated tertiary center from 2000 to 2016. Eighty-three patients matched inclusion criteria for case subjects, and 100 patients without CSF otorrhea or encephalocele were included for controls. MAIN OUTCOME MEASURE: High-resolution computed tomography (CT) scans were reviewed to assess superior semicircular canal roof integrity. Student's t test was used to determine significance of continuous variables. Odds ratio (OR) and χ test was used to determine the association between SSCD and concurrent mastoid encephalocele or CSF otorrhea compared with the control population. RESULTS: The mean age of the 83 case subjects was 54 years, and 73% were women. In patients with confirmed encephalocele and CSF otorrhea, the prevalence of SSCD was 35%, which was significantly greater than controls (2%) (OR = 26.1, p < 0.001). In patients with only CSF otorrhea, 21% had concurrent SSCD (OR = 10.3, p = 0.001). In patients with SSCD, 46% had bilateral canal dehiscence. CONCLUSIONS: This is the largest study to evaluate the prevalence of SSCD in patients with concurrent mastoid encephalocele. This study showed a significant association between SSCD and the presence of both mastoid encephalocele and CSF otorrhea. This data suggests that surgeons should have a high suspicion for SSCD in patients presenting with encephalocele or CSF otorrhea.