In 2014 radiological lesions at one or more semicircular canals (SCC) were described using CT \& MR imaging in subjects presenting advanced hearing and vestibular deterioration caused by the p.P51S mutation in COCH. Similar lesions were also described in other non-genetic advanced hearing and vestibular deterioration as well. With this prospective observational study it is the purpose to inventory imaging results of candidates for cochlear implantation which are routinely performed during the preoperative work up, since these patient present severe hearing impairment at both ears. A considerable part of them might present vestibular deterioration as well. It is the purpose to detect possible presence of these SCC lesions on CT and MR in this population and the prevalence of these lesions compared to DFNA9 patients.
Study Type
OBSERVATIONAL
Enrollment
40
routine test battery applied to all cochlear implant candidates
Jessa Hospital
Hasselt, Limburg, Belgium
RECRUITINGCT/MR of SCC
lesions to one or more semicircular canals on CT and/or MR of temporal bone
Time frame: at time of recruitment
p.P51S
carriers hip of p.P51S mutation in COCH
Time frame: at time of recruitment
audiometry
tonale liminar audiometry thresholds
Time frame: at time of recruitment
vestibular function 1
video nystagmography
Time frame: at time of recruitment
vestibular function 2
video head impulse test
Time frame: at time of recruitment
vestibular function 3
vestibular evoked myogenic potentials
Time frame: at time of recruitment
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