Get access

Clinical experience in diagnosis and management of superior semicircular canal dehiscence in children

Authors

  • Gi Soo Lee MD, EdM,

    Corresponding author
    1. Department of Otolaryngology and Communication Enhancement, Children's Hospital Boston, Boston, Massachusetts, U.S.A
    2. Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, U.S.A
    • Department of Otolaryngology and Communication Enhancement, 300 Longwood Ave, LO-367, Boston, MA 02115
    Search for more papers by this author
  • Guangwei Zhou MD, ScD,

    1. Department of Otolaryngology and Communication Enhancement, Children's Hospital Boston, Boston, Massachusetts, U.S.A
    Search for more papers by this author
  • Dennis Poe MD,

    1. Department of Otolaryngology and Communication Enhancement, Children's Hospital Boston, Boston, Massachusetts, U.S.A
    2. Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, U.S.A
    Search for more papers by this author
  • Margaret Kenna MD, MPH,

    1. Department of Otolaryngology and Communication Enhancement, Children's Hospital Boston, Boston, Massachusetts, U.S.A
    2. Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, U.S.A
    Search for more papers by this author
  • Manali Amin MD,

    1. Department of Otolaryngology and Communication Enhancement, Children's Hospital Boston, Boston, Massachusetts, U.S.A
    2. Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, U.S.A
    Search for more papers by this author
  • Laurie Ohlms MD,

    1. Department of Otolaryngology and Communication Enhancement, Children's Hospital Boston, Boston, Massachusetts, U.S.A
    2. Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, U.S.A
    Search for more papers by this author
  • Quinton Gopen MD

    1. Division of Head and Neck, David Geffen School of Medicine at UCLA, Los Angeles, California, U.S.A
    Search for more papers by this author

  • The authors have no funding, financial relationships, or conflicts of interest to disclose.

  • This study was presented at the Combined Otolaryngological Spring Meeting, American Otological Society, Las Vegas, Nevada, April 28 – May 2, 2010.

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.

Ancillary