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

NCT04070937 | Recruiting

• 1 other identifier: JessaH-4
• Study Type: observational
• Target: 40
• Locations: 1 country
• Sites: 1

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.

Conditions

Hearing Loss, Sensorineural; Bilateral Vestibular Deficiency; DFNA9; Radiology

Outcome Measures

Primary Outcomes (2)

• 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 Outcomes (4)

• vestibular test 1

  at time of recruitment

• vestibular test 2

  at time of recruitment

• vestibular test 3

  at time of recruitment

• audiometry

  at time of recruitment

Study Arms (2)

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; 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

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; 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

Interventions

vestibular test 1 DIAGNOSTIC_TEST

videonystagmography

control group; study group

vestibular test 2 DIAGNOSTIC_TEST

video head impulse test

control group; study group

vestibular evoked myogenic potentials DIAGNOSTIC_TEST

vestibular evoked myogenic potentials to measure the function of otolith organs

control group; study group

audiometry DIAGNOSTIC_TEST

tonal liminar audiometric thresholds

control group; study group

CT and MR imaging of temporal bone DIAGNOSTIC_TEST

Ct and MR imaging of temporal bone

control group; study group

p.P51S mutation GENETIC

analysis of presence of p.P51S mutation in COCH

control group; study group

Eligibility Criteria

• Age: 18 Years - 90 Years
• Sex: all
• Age Groups: Adult (18-64), Older Adult (65+)
• 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.

You may qualify if:

• 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

You may not qualify if:

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

Sponsors & Collaborators

• Jessa Hospital: lead

Study Sites (1)

Jessa Hospital

Hasselt, Limburg, 3500, Belgium

RECRUITING

Biospecimen

Retention: SAMPLES WITH DNA

from all patients presenting bilateral vestibulopathy samples will be taken to investigate presence of p.P51S mutation in COCH

MeSH Terms

Conditions

Hearing Loss, Sensorineural; Bilateral Vestibulopathy

Interventions

Vestibular Evoked Myogenic Potentials; Audiometry

Condition Hierarchy (Ancestors)

Hearing Loss; Hearing Disorders; Ear Diseases; Otorhinolaryngologic Diseases; Sensation Disorders; Neurologic Manifestations; Nervous System Diseases; Signs and Symptoms; Pathological Conditions, Signs and Symptoms; Vestibular Diseases; Labyrinth Diseases

Intervention Hierarchy (Ancestors)

Evoked Potentials, Motor; Evoked Potentials; Cortical Excitability; Electrophysiological Phenomena; Physiological Phenomena; Hearing Tests; Diagnostic Techniques, Otological; Diagnostic Techniques and Procedures; Diagnosis

Central Study Contacts

sebastien p JanssensdeVarebeke, MD

CONTACT

011337420; drsjanssens@gmail.com

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: PROSPECTIVE
• Target Duration: 2 Years
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: principal investigator

Study Record Dates

• First Submitted: August 21, 2019
• First Posted: August 28, 2019
• Study Start: September 1, 2019
• Primary Completion (Estimated): October 30, 2026
• Study Completion (Estimated): October 30, 2026
• Last Updated: August 25, 2021

Record last verified: 2021-08

Data Sharing

• IPD Sharing: Will not share

Locations

BE (1)