Hearing Loss Clinical Trial
— CARESSOfficial title:
Epidemiological, Clinical and Etiological Features of SUSAC's Syndrome (RETINOCOCHLEOCEREBRAL Vasculopathy)
SUSAC's Syndrome (SS) is characterized by the clinical triad of encephalopathy, hearing loss,
and retinal artery branch occlusions. Since the first description of SS in 1979, hundreds of
patients with SS, mostly young women, have been reported. However, comprehensive
epidemiological, clinical and etiological features of SS have never been specifically
addressed so far.
The objective of this study is to characterize the epidemiological, clinical, and etiological
features of SUSAC's Syndrome. In this aim, the investigators will constitute a national
clinical-based cohort including all SS cases retrospectively reported in France since the
last 20 years and all new cases prospectively observed. French Society of Neurology,
Ophthalmology and Internal Medicine will be asked to collaborate. Every case will be reviewed
by an expert comity of internists, neurologists and neuroradiologists to validate the
diagnosis. The exhaustive and systematic analysis of each case will help to better define
different aspects of the disease such as the incidence and prevalence, the clinical
presentation, the diagnostic modalities and the impact of treatments. Diffusion tensor
magnetic resonance imaging of the brain will be obtained to more carefully study the cerebral
microvasculopathy of the disease. Serum, cerebrospinal fluid, and DNA samples from each
patient will also be collected to study potential autoimmune, thrombotic and infectious
markers.
Status | Recruiting |
Enrollment | 100 |
Est. completion date | May 2021 |
Est. primary completion date | May 2020 |
Accepts healthy volunteers | No |
Gender | All |
Age group | 18 Years and older |
Eligibility |
INCLUSION CRITERIA: - Age older than 18 - Two clinical features of the triad present: encephalopathy, sensorineural hearing loss assessed by audiogram, retinal artery occlusion assessed by fundoscopy or fluorescein retinal angiography. - Written informed consent provided. In case of subjects unable to give a written informed consent because of encephalopathy associated with the disease, a written statement of non-opposition should be signed by a relative. This non-opposition statement should be then confirmed by the subject as soon as possible. - Realization of a medical examination beforehand EXCLUSION CRITERIA: - Alternative diagnosis: multiple sclerosis, mitochondriopathy, Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL), primary brain tumor, Lyme disease. - In case of associated disease (autoimmune disease, tumor, metabolical disease,…), inclusion will need further analysis by the expert comity. - Not membership in a national insurance scheme |
Country | Name | City | State |
---|---|---|---|
France | Hospital BICHAT | Paris |
Lead Sponsor | Collaborator |
---|---|
Assistance Publique - Hôpitaux de Paris |
France,
Type | Measure | Description | Time frame | Safety issue |
---|---|---|---|---|
Primary | To characterize the epidemiological, clinical, and etiological of SUSAC's Syndrome | To characterize the epidemiological, clinical, and etiological of SUSAC's Syndrome | 1 year | |
Secondary | diffusion MRI | diffusion MRI results | 12 months | |
Secondary | serum sample | all samples from each patient will be collected to study potential autoimmune, thrombotic and infectious markers | day 1 | |
Secondary | cerebrospinal fluid sample | all samples from each patient will be collected to study potential autoimmune, thrombotic and infectious markers | day 1 | |
Secondary | DNA sample | all samples from each patient will be collected to study potential autoimmune, thrombotic and infectious markers | day 1 | |
Secondary | RNA sample | all samples from each patient will be collected to study potential autoimmune, thrombotic and infectious markers | day 1 | |
Secondary | To characterize the epidemiological, clinical, and etiological of SUSAC's Syndrome | To characterize the epidemiological, clinical, and etiological of SUSAC's Syndrome | 5 years | |
Secondary | Peripheral Blood Mononuclear Cell | all samples from each patient will be collected to study potential autoimmune, thrombotic and infectious markers | 1 day |
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