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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| study group | candidates for cochlear implantation 18 years or older, who are eligible for implantation for the indication of bilateral severe hearing loss or single sided deafness. clinical audiometric and vestibular investigations, CT and MR imaging of petrous bone |
| |
| control group | Symptomatic DFNA9 patients carrying the p.P51S mutation in COCH, presenting the radiologic semicircular canal lesion(s) on CT and/or MR. clinical audiometric and vestibular investigations, CT and MR imaging of petrous bone |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| video nystagmography | Diagnostic Test | routine test battery applied to all cochlear implant candidates |
|
| Measure | Description | Time Frame |
|---|---|---|
| CT/MR of SCC | lesions to one or more semicircular canals on CT and/or MR of temporal bone | at time of recruitment |
| p.P51S | carriers hip of p.P51S mutation in COCH | at time of recruitment |
| Measure | Description | Time Frame |
|---|---|---|
| audiometry | tonale liminar audiometry thresholds | at time of recruitment |
| vestibular function 1 | video nystagmography | at time of recruitment |
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Inclusion Criteria:
Exclusion Criteria:
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-all patients with severe hearing loss who are eligible for cochlear implantation who do not carry the p.P51S mutation in COCH and are at least 18 years of age
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| sebastien P JanssensdeVarebeke, MD | Contact | 011337420 | drsjanssens@gmail.com |
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Jessa Hospital | Recruiting | Hasselt | Limburg | 3500 | Belgium |
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| ID | Term |
|---|---|
| D006319 | Hearing Loss, Sensorineural |
| D000071699 | Bilateral Vestibulopathy |
| ID | Term |
|---|---|
| D034381 | Hearing Loss |
| D006311 | Hearing Disorders |
| D004427 | Ear Diseases |
| D010038 | Otorhinolaryngologic Diseases |
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| ID | Term |
|---|---|
| D058585 | Vestibular Evoked Myogenic Potentials |
| ID | Term |
|---|---|
| D019054 | Evoked Potentials, Motor |
| D005071 | Evoked Potentials |
| D000071080 | Cortical Excitability |
| D055724 | Electrophysiological Phenomena |
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| vestibular function 2 | video head impulse test | at time of recruitment |
| vestibular function 3 | vestibular evoked myogenic potentials | at time of recruitment |
| D012678 |
| Sensation Disorders |
| D009461 | Neurologic Manifestations |
| D009422 | Nervous System Diseases |
| D012816 | Signs and Symptoms |
| D013568 | Pathological Conditions, Signs and Symptoms |
| D015837 | Vestibular Diseases |
| D007759 | Labyrinth Diseases |
| D010829 | Physiological Phenomena |