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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Dominant optic atrophy


Other Names for this Disease
  • Autosomal dominant optic atrophy
  • DOA
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Treatment


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How might dominant optic atrophy be treated?

There is currently no cure for dominant optic atrophy (DOA). Management generally consists of regular eye exams, including measurement of visual acuity, color vision, visual fields and optical coherence tomography (OCT). Currently there is no specific treatment, but low-vision aids in individuals with severely decreased visual acuity can be helpful.[1]

A preliminary study published in February 2013 found that several individuals with specific OPA1 mutations who underwent idebenone therapy (which has been used to treat some cases of Leber hereditary optic neuropathy) experienced some improvement of visual function. However, more thorough research is necessary to confirm these findings.[2] Acupuncture is also being studied as a potential treatment.

Avoiding tobacco and alcohol intake and certain medications (antibiotics, antivirals), which can interfere with mitochondrial metabolism, may help to slow the progression. Cochlear implants have been shown to markedly improve hearing in individuals with sensorineural hearing loss.[1]
Last updated: 11/13/2013

References
  1. Guy Lenaers et al. Dominant optic atrophy. Orphanet Journal of Rare Diseases. July 9, 2012; 7(46):http://www.ojrd.com/content/7/1/46. Accessed 11/13/2013.
  2. Barboni P. et al. Idebenone treatment in patients with OPA1-mutant dominant optic atrophy. Brain. 2013; 136(Pt 2):e231:Accessed 11/13/2013.


Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Dominant optic atrophy. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.