Fig. 1

The patient presented with hearing loss, decreased vision, optic atrophy and neurogenic bladder. a Audiograms show left sensorineural hearing loss. b Audiograms show right sensorineural hearing loss. c Fundus image shows atrophic optic discs with temporal pallor. d Axial brain MRI shows bilateral optic nerve atrophy on T1WI (left) and T2WI (right). e MRU shows marked dilation of bilateral renal pelvis, calyces and ureter