Correlation of Radiological Lesions With Vestibular Function in Patients With Bilateral Vestibulopathy

Overview

In 2014 radiologic lesions were detected at one or more semicircular canals using CT and MR imaging of temporal bone in subjects carrying the p.P51S mutation in COCH. These lesions are believed to present at more advanced stages of the hearing and vestibular deterioration. Since then, other authors have described similar lesions in advanced non-genetic hearing and vestibular impairment as well. The purpose of this study is therefore to assess the radiologic investigation using CT and MR imaging of temporal bone to all subjects presenting with bilateral vestibulopathy, using the Barany criteria, compared to the p.P51S population.

Study Type

  • Study Type: Observational [Patient Registry]
  • Study Design
    • Time Perspective: Prospective
  • Study Primary Completion Date: October 30, 2026

Interventions

  • Diagnostic Test: vestibular test 1
    • videonystagmography
  • Diagnostic Test: vestibular test 2
    • video head impulse test
  • Diagnostic Test: vestibular evoked myogenic potentials
    • vestibular evoked myogenic potentials to measure the function of otolith organs
  • Diagnostic Test: audiometry
    • tonal liminar audiometric thresholds
  • Diagnostic Test: CT and MR imaging of temporal bone
    • Ct and MR imaging of temporal bone
  • Genetic: p.P51S mutation
    • analysis of presence of p.P51S mutation in COCH

Arms, Groups and Cohorts

  • study group
    • all patients at least 18 years of age who present bilateral vestibulopathy on vestibular investigations according to the Barany criteria
  • control group
    • DFNA9 patients carrying the p.P51S mutation in COCH gene presenting bilateral vestibulopathy according to the Barany criteria

Clinical Trial Outcome Measures

Primary Measures

  • CT and MR findings
    • Time Frame: at time of recruitment
    • presence of focal sclerosis or signal loss on CT & MR at one or more semicircular canals
  • genetic test
    • Time Frame: at time of recruitment
    • p.P51S mutation in COCH gene

Secondary Measures

  • vestibular test 1
    • Time Frame: at time of recruitment
    • video nystagmography using caloric tests ; caloric tests: °/s
  • vestibular test 2
    • Time Frame: at time of recruitment
    • video head impulse test; gain for each SCC in °/s
  • vestibular test 3
    • Time Frame: at time of recruitment
    • vestibular evoked myogenic potentials: threshold in decibel normal hearing loss (dB nHL)
  • audiometry
    • Time Frame: at time of recruitment
    • tonal liminar audiometry using calibrated audiometer, threshold in dB HL

Participating in This Clinical Trial

Inclusion Criteria

  • must meet the Barany criteria for bilateral vestibulopathy: bilaterally pathological horizontal angu- lar VOR gain <0.6, measured by the video-HIT5 or scleral-coil technique and/or – reduced caloric response6 (sum of bither- mal max. peak SPV on each side <6◦/sec7) and/or – reduced horizontal angular VOR gain <0.1 upon sinusoidal stimulation on a rota- tory chair (0.1 Hz, Vmax = 50◦ /sec) and a phase lead >68 degrees (time constant <5 sec). – must be 18 or older Exclusion Criteria:

  • < 18 y – does not meet Barany criteria for BV – contraindication for CT and MR imaging

Gender Eligibility: All

Minimum Age: 18 Years

Maximum Age: 90 Years

Investigator Details

  • Lead Sponsor
    • Jessa Hospital
  • Provider of Information About this Clinical Study
    • Principal Investigator: Sebastien Janssens de Varebeke, principal investigator – Jessa Hospital
  • Overall Contact(s)
    • sebastien p JanssensdeVarebeke, MD, 011337420, drsjanssens@gmail.com

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