ABSTRACT
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.
Subject(s)
Abnormalities, Multiple/diagnosis , Duane Retraction Syndrome/diagnosis , Forearm/abnormalities , Hearing Loss, Sensorineural/diagnosis , Adult , Child, Preschool , Duane Retraction Syndrome/genetics , Female , Hearing Loss, Sensorineural/genetics , Humans , PedigreeABSTRACT
This report describes two unusual cases of parasellar arachnoid cyst with different neuro-ophthalmologic manifestations and clinical courses: a 33-year-old woman with parasellar arachnoid cyst, manifested by incongruous homonymous hemianopia, and a 64-year-old man with a presumed parasellar arachnoid cyst and bitemporal hemianopia that subsequently decompressed spontaneously. Parasellar arachnoid cyst is uncommon, and the clinical course has been incompletely described in the literature. Optimal treatment of patients with these cysts necessitates better understanding of their signs, symptoms, and clinical course.