Dominant optic atrophy
(DOA) is an inherited optic nerve disorder
characterized by degeneration of the optic nerves.
It typically starts during the first decade of life. Affected people usually develop moderate visual loss and color vision defects. The severity varies and visual acuity can range from normal to legal blindness. About 20% of people with DOA have non-ocular features, such as sensorineural hearing loss
; peripheral neuropathy
; multiple sclerosis-like illness; and spastic paraplegia (impaired function of the legs).
These cases may be referred to as 'DOA plus.'
DOA is inherited in an autosomal dominant
manner and may be caused by a mutation
in any of several genes, some of which have not been identified. There is currently no way to prevent or cure DOA, but affected people may benefit from low vision aids.
Last updated: 12/27/2015