Bilateral Vestibular Deficiency, DFNA9, Hearing Loss, Sensorineural, Radiology
Conditions
Brief summary
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.
Interventions
DIAGNOSTIC_TESTvideo nystagmography
routine test battery applied to all cochlear implant candidates
Sponsors
Jessa Hospital
Study design
Observational model
CASE_CONTROL
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to 90 Years
Healthy volunteers
No
Inclusion criteria
* must be eligible for cochlear implantation * must be 18 years of age or older
Exclusion criteria
* \<18 years of age * not eligible to undergo vestibular tests, especially videonystagmography * contra-indication for MR or CT
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| CT/MR of SCC | at time of recruitment | lesions to one or more semicircular canals on CT and/or MR of temporal bone |
| p.P51S | at time of recruitment | carriers hip of p.P51S mutation in COCH |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| audiometry | at time of recruitment | tonale liminar audiometry thresholds |
| vestibular function 1 | at time of recruitment | video nystagmography |
| vestibular function 2 | at time of recruitment | video head impulse test |
| vestibular function 3 | at time of recruitment | vestibular evoked myogenic potentials |
Countries
Belgium
Contacts
Primary Contactsebastien P JanssensdeVarebeke, MD
Outcome results
None listed