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

Recruiting

• Conditions: Hearing Loss, Sensorineural; Radiology; Bilateral Vestibular Deficiency; DFNA9
• Interventions: 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

• Registration Number: NCT04070937
• Lead Sponsor: Jessa Hospital

Brief Summary

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.

Detailed Description

Not available

Study Timeline

• Study First Submitted: 2019-08-21
• Study First Submitted QC: 2019-08-26
• Study First Posted: 2019-08-28
• Study Start: 2019-09-01
• Status Verified: 2021-08
• Last Update Submitted: 2021-08-24
• Last Update Posted: 2021-08-25
• Primary Completion: 2026-10-30
• Study Completion: 2026-10-30

Recruitment & Eligibility

• Status: RECRUITING
• Sex: All
• Target Recruitment: 40

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 & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
study group	vestibular test 2	all patients at least 18 years of age who present bilateral vestibulopathy on vestibular investigations according to the Barany criteria
study group	vestibular test 1	all patients at least 18 years of age who present bilateral vestibulopathy on vestibular investigations according to the Barany criteria
study group	vestibular evoked myogenic potentials	all patients at least 18 years of age who present bilateral vestibulopathy on vestibular investigations according to the Barany criteria
study group	audiometry	all patients at least 18 years of age who present bilateral vestibulopathy on vestibular investigations according to the Barany criteria
study group	CT and MR imaging of temporal bone	all patients at least 18 years of age who present bilateral vestibulopathy on vestibular investigations according to the Barany criteria
study group	p.P51S mutation	all patients at least 18 years of age who present bilateral vestibulopathy on vestibular investigations according to the Barany criteria
control group	vestibular test 1	DFNA9 patients carrying the p.P51S mutation in COCH gene presenting bilateral vestibulopathy according to the Barany criteria
control group	vestibular test 2	DFNA9 patients carrying the p.P51S mutation in COCH gene presenting bilateral vestibulopathy according to the Barany criteria
control group	vestibular evoked myogenic potentials	DFNA9 patients carrying the p.P51S mutation in COCH gene presenting bilateral vestibulopathy according to the Barany criteria
control group	CT and MR imaging of temporal bone	DFNA9 patients carrying the p.P51S mutation in COCH gene presenting bilateral vestibulopathy according to the Barany criteria
control group	audiometry	DFNA9 patients carrying the p.P51S mutation in COCH gene presenting bilateral vestibulopathy according to the Barany criteria
control group	p.P51S mutation	DFNA9 patients carrying the p.P51S mutation in COCH gene presenting bilateral vestibulopathy according to the Barany criteria

Primary Outcome Measures

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

Secondary Outcome Measures

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

Trial Locations

Locations (1)

Jessa Hospital; 🇧🇪 Hasselt, Limburg, Belgium