Cervico-oculo-acusticus syndrome with pseudopapilloedema.

The triad of the Klippel-Feil anomaly, Duane's retraction syndrome, and deaf-mutism was described by Wildervanck (1960) as the cervico-oculoacusticus syndrome. The Klippel-Feil anomaly essentially comprises a variety of bony deformities of the cervical spine, usually involving fusion, which appear clinically as a short neck with a limited range of movements of the head and neck and a low posterior hairline. The retraction syndrome itself comprises retraction movements of the eyeball, with narrowing of the palpebral fissure on adduction and, in the presence of an apparent lateral rectus palsy, forward movement of the globe, with widening of the palpebral fissure on attempted abduction. Duane (1905) points out that there is often limitation of adduction as well as the usually more marked limitation of abduction. In addition, there is often an upshoot or a downshoot of the globe on adduction. The term profound childhood deafness is to be preferred to that of deaf-mutism; profound deafness in infancy is now regarded as a socio-educational problem (Fraser, 1964). Duane's syndrome has usually been considered an isolated phenomenon. Danis (1948) reported on 229 cases from the literature, and did not record a single instance of deafness or cervical spine anomaly. Similarly, most of the published cases of the Klippel-Feil anomaly, reviewed by Gray, Romaine, and Skandalakis (1964), have not been associated with deafness or squint. However, isolated cases of Duane's syndrome with the Klippel-Feil anomaly have been reported by Magnus (1944) and Waardenburg (1953). In her series of 77 patients of Duane's syndrome, Mein (1968) recorded the Klippel-Feil anomaly in 3 and deafness in 2 cases. Evidence is accumulating that there is a genetic relationship between Duane's syndrome, perceptive