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

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 Overview

• Status: Recruiting
• Conditions: Hearing Loss, Sensorineural; DFNA9; Radiology; Bilateral Vestibular Deficiency
• Intervention / Treatment: Diagnostic test: vestibular test 1; Diagnostic test: vestibular test 2; Diagnostic test: vestibular evoked myogenic potentials; Diagnostic test: audiometry; Diagnostic test: CT and MR imaging of temporal bone; Genetic: p.P51S mutation

• Study Type: Observational
• Enrollment (Anticipated): 40

Contacts and Locations

• Study Contact: Name: sebastien P JanssensdeVarebeke, MD; Phone Number: 011337420; Email: drsjanssens@gmail.com

Study Locations

• Belgium
  • Limburg
    • Hasselt, Limburg, Belgium, 3500
      • Recruiting
      • Jessa Hospital
      • Contact: sebastien P Janssens, MD; Phone Number: 011 337420; Email: drsjanssens@gmail.com
      • Principal Investigator: sebastien PF Janssens, MD

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 90 years (ADULT, OLDER_ADULT)
• Accepts Healthy Volunteers: N/A
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

all patients presenting at our Department of ENT with vestibular or balance disorders, in whom videonystagmography, video head impulse test and VEMP tests meet the Barany criteria for bilateral vestibulopathy.

Description

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

Study Plan

How is the study designed?

Number of groups / cohorts

2

Cohorts and Interventions

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

What is the study measuring?

Primary Outcome Measures

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

Secondary Outcome Measures

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

Collaborators and Investigators

• Sponsor: Jessa Hospital

Study record dates

Study Major Dates

• Study Start (ACTUAL): September 1, 2019
• Primary Completion (ANTICIPATED): October 30, 2026
• Study Completion (ANTICIPATED): October 30, 2026

Study Registration Dates

• First Submitted: August 21, 2019
• First Submitted That Met QC Criteria: August 26, 2019
• First Posted (ACTUAL): August 28, 2019

Study Record Updates

• Last Update Posted (ACTUAL): August 25, 2021
• Last Update Submitted That Met QC Criteria: August 24, 2021
• Last Verified: August 1, 2021

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Nervous System Diseases; Neurologic Manifestations; Otorhinolaryngologic Diseases; Labyrinth Diseases; Ear Diseases; Vestibular Diseases; Sensation Disorders; Hearing Disorders; Hearing Loss; Hearing Loss, Sensorineural; Bilateral Vestibulopathy
• Other Study ID Numbers: JessaH-4

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No