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
OBSERVATIONAL
Enrollment
40
videonystagmography
video head impulse test
vestibular evoked myogenic potentials to measure the function of otolith organs
tonal liminar audiometric thresholds
Ct and MR imaging of temporal bone
analysis of presence of p.P51S mutation in COCH
Jessa Hospital
Hasselt, Limburg, Belgium
RECRUITINGCT and MR findings
presence of focal sclerosis or signal loss on CT \& MR at one or more semicircular canals
Time frame: at time of recruitment
genetic test
p.P51S mutation in COCH gene
Time frame: at time of recruitment
vestibular test 1
video nystagmography using caloric tests ; caloric tests: °/s
Time frame: at time of recruitment
vestibular test 2
video head impulse test; gain for each SCC in °/s
Time frame: at time of recruitment
vestibular test 3
vestibular evoked myogenic potentials: threshold in decibel normal hearing loss (dB nHL)
Time frame: at time of recruitment
audiometry
tonal liminar audiometry using calibrated audiometer, threshold in dB HL
Time frame: at time of recruitment
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