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Keywords:

  • Semicircular canal dehiscence;
  • pediatric hearing loss;
  • vestibular dysfunction;
  • VEMP testing;
  • Level of Evidence: 4

Abstract

Objectives/Hypothesis:

To identify clinical characteristics of pediatric superior semicircular canal dehiscence (SSCD) and explore suitable options of management.

Study Design:

Retrospective review.

Methods:

The study comprised 10 patients with auditory and/or vestibular symptoms suspicious for SSCD. One patient pursued care at another institution, and two did not return for follow-up. Subsequently, seven patients (11 ears, 6 females and 1 male, aged 5–11 years) were included. Patients were evaluated using high-resolution temporal bone computed tomography. Those suspected of having SSCD underwent vestibular evoked myogenic potential testing for confirmation in addition to routine audiologic tests.

Results:

All seven patients had auditory and/or vestibular impairment. Auditory symptoms included autophony, tinnitus, and conductive or mixed hearing loss. Bone conduction responses were occasionally better than 0 dB HL. Vestibular dysfunction included vertigo, often in response to loud noises, and chronic disequilibrium. One patient underwent surgical repair for disabling vestibular symptoms with dramatic improvement in both auditory and vestibular symptoms postoperatively. The remaining six were closely monitored with routine exams.

Conclusions:

In contrast to adults, children with SSCD usually present with auditory symptoms first, although they share some similarities with adults in clinical manifestations of SSCD. Our study shows that SSCD syndrome, a well-accepted clinical entity, exists in the pediatric population. Conservative management is preferred for children with SSCD; nevertheless, surgical intervention is necessary for those with disabling vestibular symptoms. To date, this is the first clinical case series of symptomatic pediatric patients with SSCD.