A pair of male siblings with dominant progressive optic atrophy and hearing loss Kayoko Ide 1 , Torao Sugiura 1 , Hiroyuki Miyazawa 1 1Dept of Ophthalmol, Kobe Sch of Med pp.1739-1743
Published Date 1990/10/15
DOI https://doi.org/10.11477/mf.1410908267
  • Abstract
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A pair of male siblings, aged 34 and 28 years each, presented with progressive diffuse optic atro-phy and neural hearing loss. No other systemic abnormalities were present. Corrected visual acuity was 0.2 each in the elder brother and 0.4 each in the younger. Both cases manifested acquired blue-yel-low color deficiency. There were 5 members over 2 generations in the pedigree with similar symptoms. No other causes for hearing and visual loss could be identified except heredity. This pedigree of progres-sive optic atrophy and neural hearing loss of autosomal dominant inheritance should be distin-guished from monosymptomatic dominant optic atrophy.

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