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

Jessa Hospital

Status: Enrolling

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

Treatments: Diagnostic Test: CT and MR imaging of temporal bone; Diagnostic Test: vestibular test 2; Diagnostic Test: vestibular evoked myogenic potentials; Diagnostic Test: audiometry; Diagnostic Test: vestibular test 1; Genetic: p.P51S mutation

Study type: Observational

Funder types: Other

Identifiers

NCT04070937

JessaH-4

Details and patient eligibility

About

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.

Enrollment: 40 estimated patients

Sex: All

Ages: 18 to 90 years old

Volunteers: No Healthy Volunteers

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

Trial design

40 participants in 2 patient groups

study group

Description:

all patients at least 18 years of age who present bilateral vestibulopathy on vestibular investigations according to the Barany criteria

Treatment:

Genetic: p.P51S mutation

Diagnostic Test: vestibular test 1

Diagnostic Test: CT and MR imaging of temporal bone

Diagnostic Test: vestibular evoked myogenic potentials

Diagnostic Test: audiometry

Diagnostic Test: vestibular test 2

control group

Description:

DFNA9 patients carrying the p.P51S mutation in COCH gene presenting bilateral vestibulopathy according to the Barany criteria

Treatment:

Genetic: p.P51S mutation

Diagnostic Test: vestibular test 1

Diagnostic Test: CT and MR imaging of temporal bone

Diagnostic Test: vestibular evoked myogenic potentials

Diagnostic Test: audiometry

Diagnostic Test: vestibular test 2