Purpose: To describe Okihiro syndrome, a syndrome consisting of Duane's syndrome, radial ray anomaly, and sensorineural deafness. Methods: The incidence of anomalies in 41 individuals from 9 affected families was assessed by literature review and patient examination, and the frequency of the characteristics occurring together was determined. Results: Of all the individuals with at least one of the above characteristics, only 12% had all three characteristics. However, 44% had both Duane's syndrome and radial ray anomaly, whereas sensorineural deafness alone occurred in only 17% of patients. Conclusion: Okihiro syndrome is consistent with autosomal dominance with variable penetrance, manifesting primarily with Duane's syndrome and radial ray anomaly, and less frequently, sensorineural deafness. Journal of Pediatric Ophthalmology and Strabismus 2001;38:235-239.

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