Observation concerning the age of onset and the nature of optic atrophy in Wolfram's syndrome (DIDMOADS).

A 31/2-year-old boy, who was diagnosed to have diabetes 6 months earlier, was referred for routine fundus check-up. No family history of diabetes was known. Ophthalmologic findings were: slight hyperopic astigmatism, visual acuity right = left = 1/5, examination of the visual field not yet possible, optic atrophy, no diabetic retinopathy, very narrow vessels. ERG: distinctly reduced potentials. Audiogram: loss of high tones in the inner ear. Pediatric examination ruled out other endocrine disorders, no symptoms of diabetes insipidus were found. These results perhaps reveal the nature of the optic atrophy: a retinal one. The authors found no other ERGs in the literature at such an early stage.