We present the clinical and pathologic findings in an atypical case of idio
pathic intracranial hypertension. A 51-year-old man had headaches, visual d
eterioration, papilloedema, and deafness, Neuroimaging was normal, and cere
brospinal fluid pressure monitoring confirmed increased intracranial pressu
re. The patient was treated with a ventriculo-peritoneal shunt. Histopathol
ogy revealed grossly atrophic optic nerves with almost complete axonal loss
. The prelaminar portion of the optic nerves was thickened by gliosis and h
yalinized capillaries, which have not been described previously.