PMC6482645_01
(a) T2 sagittal MRI brain showing high signal foci in the corpus callosum (white vertical arrows). Similar changes were also found in the subcortical white matter of the cerebral and cerebellar hemispheres bilaterally. (b) Right ear audiogram showing pan-frequency severe sensorineural hearing loss (circles) with relative sparing of bone conduction hearing (open triangles). (c) Fluorescein angiography showing filling defects in occluded branch retinal arteries (black horizontal arrows). There was evidence of multifocal fluorescence (not shown). (d) Retinal photography showing occluded branch retinal artery (black vertical arrows).
10.1177_2048004019844687-fig1.jpg
PMC6482645_01_10.1177_2048004019844687-fig1.jpg
fig1-2048004019844687
A magnetic resonance imaging (MRI) brain scan (12 days from symptom onset) revealed 'snowball' white matter lesions in both cerebral hemispheres mostly centred on the splenium of the corpus callosum (Figure 1(a)). Audiometry revealed severe bilateral sensorineural hearing loss, especially on the right (Figure 1(b)), but responses were inconsistent. Retinal imaging and retinal fluorescein angiography (RFA) revealed a branch retinal artery occlusion in the right eye (Figure 1(c) and (d)), thus fulfilling the diagnostic criteria for Susac syndrome proposed by Kleffner et al.