Abstract： A 12-year-old boy presented with bilateral visual loss for 3 years and progressed in the past half a year.The patient was diagnosed with bilateral profound sensorineural hearing loss 5 years ago and then a cochlear was implanted.He was also diagnosed with recurrent uveitis in the right eye for 3 years.His best corrected visual acuity was CF/40cm in the right eye(OD)and 0.05 in the left(OS).The intraocular pressure was 19 mmHg(OD)and 13 mmHg(OS).Slit lamp examination of the right eye revealed small to medium-sized dendritic keratic precipitates(KPs).The right pupil was irregular and displaced superonasally.Ectropion uvea,superonasal anterior synechia,multiple iris nodules and RAPD were present in OD.The left anterior segment was unremarkable.The funduscopic examination showed bilateral pale optic discs with blurred disc margin nasally in OS.Orbital CT demonstrated significant enlargement of the intraorbital and intracranial segments of the right optic nerve and slight enlargement of the left optic nerve.High-resolution transbulbar ultrasonography revealed asymmetrically enlarged subarachnoid space(SAS)of the optic nerve in OD and evenly enlarged in OS.The subarachnoid echo of the right eye also revealed a moderately solid echogenic pattern.Bilateral facial nerve paralysis occurred successively during the period of follow-ups.Brain CT showed widened labyrinthine segment and the geniculate fossa of the right facial nerve canal,thickened tentorium cerebelli involving bilateral paracavernous sinus meninges.Cerebrospinal fluid examination revealed a large number of immature lymphocytes presenting uniform morphology.Cervical MRI showed obviously thickened dura mater with spinal compression,but tumor cells were not detected in biopsy tissue.Primary diagnosis:multiple cranial neuropathies,meningeal lesions and uveitis(OD).According to patient's clinical manifestations and associated auxiliary tests,lymphoma or inflammatory pathogenesis was highly suspected.However,further examinations screening for systemic disorders were needed to obtain a finial diagnosis.