[Susac syndrome or microangiopathy of the cochlea, brain and retina].
OBJECTIVE
Susac syndrome, also called SICRET syndrome (small infarction of cochlear, retinal, and encephalic tissue) is a rare condition difficult to diagnose. Sudden deafness may be the inaugural sign.


METHODS
A female patient developed subacute encephalopathy, bilateral sensorineural hearing loss, and ischemic retinopathy. The patient was given cyclophosphamid and methylprednisolone for six months, followed by prednisone for eight months.


RESULTS
Signs of encephalopathy had totally regressed by month 14 and retinal arteries were free of obstruction. Deafness remained unchanged.


CONCLUSIONS
Diagnosis of this microangiopathy involving the inner ear, the brain, and the retina is suggested by the clinical triad and established on the basis of tonal audiometry, fundus examination, fluorescein angiography, examination of the cerebrospinal fluid, magnetic resonance imaging. Multiple sclerosis is the main differential diagnosis. The pathogenesis remains unknown. We observed transient-evoked otoacoustic emissions. There is no consensus concerning treatment. Many advocate combining corticosteroids and immunosuppressors. Otolaryngologists should be aware that an ophthalmological examination is required for patients with central or visual disorders associated with hearing loss.
Clément
Ph
P
Service d'Oto-Rhino-Laryngologie et de Chirurgie de la Face et du Cou, Hôpital d'Instruction des Armées Percy, 101, avenue Henri-Barbusse, 92140 Clamart. orl.percy@free.fr
Conessa
C
C
Dot
C
C
Kossowski
M
M
Flocard
F
F
Poncet
J L
JL
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001157:Arterial Occlusive Diseases / Q000150:complications / Q000188:drug therapy / Q000473:pathology; D001301:Audiometry, Pure-Tone; D001928:Brain Diseases, Metabolic / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D002533:Cerebral Angiography; D003051:Cochlea / Q000503:physiopathology*; D003937:Diagnosis, Differential; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology*; D006801:Humans; D007511:Ischemia / Q000150:complications* / Q000188:drug therapy / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008775:Methylprednisolone / Q000627:therapeutic use; D011239:Prednisolone / Q000627:therapeutic use; D012161:Retinal Artery / Q000473:pathology*
D000893:Anti-Inflammatory Agents; D011239:Prednisolone; D008775:Methylprednisolone
Syndrome de Susac ou microangiopathie cochléo-encéphalo-rétinienne.