Pediatric superior semicircular canal dehiscence: illustrative case and systematic review

Abstract

<jats:sec><jats:title>OBJECTIVE</jats:title><jats:p>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.</jats:p></jats:sec><jats:sec><jats:title>METHODS</jats:title><jats:p>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.</jats:p></jats:sec><jats:sec><jats:title>RESULTS</jats:title><jats:p>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.</jats:p></jats:sec><jats:sec><jats:title>CONCLUSIONS</jats:title><jats:p>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.</jats:p></jats:sec>

Functional and Objective Audiovestibular Evaluation of Children With Apparent Semicircular Canal Dehiscence–A Case Series in a Pediatric Vestibular Center

“…As discussed earlier, a cochlear hearing loss can be a feature of semicircular dehiscences in children and this hearing loss can serve as a phenotype for the condition. The extensive study by Lagman et al (37) analyzing data in existing literature pertaining to SSCD in children concluded that hearing loss was the commonest indication for performing a high resolution CT scan that showed SSCD; however, it must be noted that about 25% children in this analysis had other causes explaining the hearing loss. The current series is the first to exclude this comorbidity group by performing extensive investigation for hearing loss and also employed other features for example third window symptoms and disequilibrium and vestibular function tests as indicators for further imaging.…”

Superior semicircular canal dehiscence syndrome: Diagnostic criteria consensus document of the committee for the classification of vestibular disorders of the Bárány Society

“…Younger adults and those over age 65 tend to present with similar symptoms, as do men and women [40]. Cases of SCDS in children have been identified [20,29]. Children tend to report more auditory symptoms and may report hyperacusis or generalized dizziness [29].…”

“…Cases of SCDS in children have been identified [20,29]. Children tend to report more auditory symptoms and may report hyperacusis or generalized dizziness [29]. Differences in presenting symptoms between children and adults may be attributed to younger children having difficulty explaining their symptoms to adults.…”

Audiovestibular Quantification in Rare Third Window Disorders in Children

“…The children with mixed losses above 30 dBHL in the series returned absent TEOAEs. Sensorineural hearing loss has been reported in pediatric SCDS ( 5 , 66 – 68 ). There was 1 child with normal hearing that has also been reported in third window disorders ( 5 , 68 ).…”