Susac syndrome or microangiopathy of the cochlea, brain and retina
Clément, P.; Conessa, C.; Dot, C.; Kossowski, M.; Flocard, F.; Poncet, J.L.
Annales d'Oto-Laryngologie et de Chirurgie Cervico Faciale Bulletin de la Societe d'Oto-Laryngologie des Hopitaux de Paris 120(1): 49-53
2003
ISSN/ISBN: 0003-438X PMID: 12717318 Document Number: 554603
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. 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. Signs of encephalopathy had totally regressed by month 14 and retinal arteries were free of obstruction. Deafness remained unchanged. 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.