An 8-year-old white girl with a history of
vertigo,
nausea, and
vomiting developed a progressive
hearing loss, bilateral retinal arteriolar narrowing in each eye, vasoproliferation, and subsequent intravitreal
hemorrhage. An attempt at peripheral
retinal ablation with
cryotherapy in the left eye resulted in
retinal detachment. Spontaneous
retinal detachment occurred in the right eye and was successfully repaired. Repeated intermittent
hemorrhages occurred despite intraocular
diathermy. Three years after onset, visual acuity was R.E.: 6/21 (20/66) and L.E.: light perception. She remains totally deaf. A 20-year-old white woman developed severe bilateral
sensorineural hearing loss with poorly functioning labyrinths, followed by midperipheral
retinal arteriolar occlusions and vasoproliferation on the optic nerve head. Progressive
retinal neovascularization was followed by rubeosis iridis and repeated episodes of intravitreal
bleeding. Six years after onset, visula acuity was R.E.: hand motions, and L.E.: 6/3 (20/100). She remains totally deaf. Both patients were of normal gestation, development, and mentality, without evidence of other systemic disease. The cause of this disease was not found.