Characteristics of Okihiro syndrome

Purpose: To describe Okihiro syndrome, a syndrome consisting of Duane's syn drome, radial ray anomaly, and sensorineural deafness. Methods: The incidence of anomalies in 41 individuals from 9 affected famil ies was assessed by literature review and patient examination, and the freq uency of the characteristics occurring together was determined. Results: Of all the individuals with at least one of the above characterist ics, only 12% had all three characteristics. However, 44% had both Duane's syndrome and radial ray anomaly, whereas sensorineural deafness alone occur red in only 17% of patients. Conclusion: Okihiro syndrome is consistent with autosomal dominance with va riable penetrance, manifesting primarily with Duane's syndrome and radial r ay anomaly, and less frequently, sensorineural deafness.