Pediatric superior semicircular canal dehiscence: illustrative case and systematic review

J Neurosurg Pediatr. 2017 Aug;20(2):196-203. doi: 10.3171/2017.3.PEDS1734. Epub 2017 May 26.


OBJECTIVE The purpose of this study is to present an illustrative case of pediatric superior semicircular canal dehiscence (SSCD) and to systematically review the current published literature in the pediatric population. METHODS An electronic search of the Scopus, Web of Science, PsycINFO, Cochrane, and Embase databases was performed by 2 independent authors through January 2017. Search term combinations included "pediatrics," "children," "canal," and "dehiscence." Inclusion criteria were as follows: English, full-text clinical studies, case reports, and case series describing pediatric patient(s) (younger than 18 years) with CT evidence of SSCD. Baseline patient demographic characteristics, clinical presentations, dehiscence characteristics, management strategies, and outcome data were extracted. RESULTS A total of 14 studies involving 122 patients were included in the quantitative synthesis. The patients' mean age was 7.22 years. Male predominance was observed (approximate male-to-female ratio of 1.65:1). Neurodevelopmental disorders were common (n = 14, 11.5%). Auditory signs and symptoms were more common than vestibular signs and symptoms. Hearing loss (n = 62, 50.8%) was the most common auditory symptom and an indicator for imaging evaluation. Vertigo was the most common vestibular symptom (n = 8, 6.6%). Hearing aids were recommended in 8 cases (6.6%), and surgical repair was performed in 1 case (0.8%). Symptom outcomes and follow-up durations were infrequently reported. CONCLUSIONS The authors' data suggest that in pediatric SSCD, males are more commonly affected than females. This is different than the adult population in which females are predominantly affected. A history of otologic and/or neurodevelopmental abnormalities was common. There was a preponderance of auditory symptoms in this age group. Conservative management was favored in the majority.

Keywords: ASD = autism spectrum disorder; CP = cerebral palsy; GH = growth hormone; HRCT = high-resolution computed tomography; IGF = insulin-like growth factor; SSCD = superior semicircular canal dehiscence; dehiscence; pediatrics; superior semicircular canal.

Publication types

  • Case Reports
  • Review
  • Systematic Review

MeSH terms

  • Child, Preschool
  • Hearing Loss, Sensorineural / etiology
  • Humans
  • Labyrinth Diseases / complications
  • Labyrinth Diseases / diagnosis*
  • Labyrinth Diseases / epidemiology
  • Labyrinth Diseases / physiopathology
  • Male
  • Semicircular Canals* / physiopathology
  • Skull Base / abnormalities*
  • Syndrome