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Related Experiment Video

Updated: Dec 6, 2025

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Audiovestibular Quantification in Rare Third Window Disorders in Children.

Soumit Dasgupta1, Sudhira Ratnayake1, Rosa Crunkhorn1

  • 1Department of Paediatric Audiology and Audiovestibular Medicine, Alder Hey Children's NHS Foundation Trust, Liverpool, United Kingdom.

Frontiers in Neurology
|October 12, 2020
PubMed
Summary

Rare third window disorders, structural ear abnormalities, are uncommon in children. This study found they cause mixed/conductive hearing loss and disequilibrium, often without typical symptoms.

Keywords:
HRCTX linked gusheraudiovestibularcVEMPchildrensemicircular canal dehiscencethird windowvHIT

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Area of Science:

  • Pediatric Otolaryngology
  • Neurotology
  • Vestibular Science

Background:

  • Third window disorders involve structural abnormalities of the bony otic capsule, connecting the middle/inner ear or inner ear/cranial cavity.
  • While extensively studied in adults, pediatric third window disorders remain under-investigated.
  • This study focuses on rare third window disorders in children, aiming to characterize their audiovestibular function.

Purpose of the Study:

  • To investigate the audiovestibular function in children diagnosed with rare third window disorders.
  • To identify the prevalence and specific types of rare third window disorders in a pediatric population.
  • To describe the clinical presentation and audiological/vestibular findings associated with these rare pediatric conditions.

Main Methods:

  • Retrospective analysis of children (aged 5-17 years) diagnosed with rare third window disorders at a UK tertiary pediatric vestibular unit.
  • High-resolution computed tomography (CT) scans of the temporal bones for definitive diagnosis.
  • Audiovestibular assessments including audiometry, tympanometry, otoacoustic emissions, video head impulse testing (vHIT), and vestibular evoked myogenic potentials (VEMPs).

Main Results:

  • Rare third window disorders were identified in 8 out of 920 children (<1%), including posterior semicircular canal dehiscence/thinning, X-linked gusher, and complex malformations.
  • The majority (87.5%) presented with mixed/conductive hearing loss and an air-bone gap, with normal tympanometry.
  • Disequilibrium was observed in 75%, with abnormal vestibular function tests (vHIT, cVEMP) in 50% and all tested children, respectively. Two new rare pediatric phenotypes were identified.

Conclusions:

  • Rare third window disorders are an important, albeit infrequent, cause of hearing loss and disequilibrium in children.
  • Pediatric third window disorders may present atypically, with variable audiovestibular findings and potential cochlear involvement.
  • These findings highlight the need to consider rare third window disorders in the differential diagnosis of pediatric hearing loss and vestibular dysfunction.