Histopathology of the ears, eyes, and brain in Norrie's disease (oculoacousticocerebral degeneration)
JB Nadol Jr, RD Eavey, RM Liberfarb… - American journal of …, 1990 - Elsevier
Norrie's disease is an x-linked recessive disorder characterized by progressive
oculoacousticocerebral degeneration. The light and electron microscopic changes in the
temporal bones, eyes, and brain of an affected 77-year-old man who suffered from bilateral
profound sensorineural hearing loss, blindness, and mental retardation are described. The
inner ears showed marked atrophy of the stria vascularis, severe degeneration of hair cells
and cochlear neurons, and connective tissue proliferation in the spiral ganglion, osseous …
oculoacousticocerebral degeneration. The light and electron microscopic changes in the
temporal bones, eyes, and brain of an affected 77-year-old man who suffered from bilateral
profound sensorineural hearing loss, blindness, and mental retardation are described. The
inner ears showed marked atrophy of the stria vascularis, severe degeneration of hair cells
and cochlear neurons, and connective tissue proliferation in the spiral ganglion, osseous …