Table 3.

Major clinical features observed in DOA+ patients

Clinical featuresa	n	%	95% CI
			Lower	Upper
Optic atrophy	89/104	85.6	77.5	91.2
Deafness	65/104	62.5	52.9	71.2
Ataxia	31/104	29.8	21.8	39.2
Neuropathy	31/104	29.8	21.8	39.2
Myopathy	37/104	35.6	27.0	45.2
Progressive external ophthalmoplegia	48/104	46.2	36.9	55.7

CI = confidence interval.

^aMeta-analysis of 104 OPA1 mutation carriers from 45 DOA+ families, which includes previously published data on 44 individuals from 18 DOA+ families.