Clinical and electrophysiological features of tilted disc syndrome

Nine patients with tilted disc syndrome were examined psychophysically and electrophysiologically. Visual field defects did not correspond to fundus abnormalities in half of the eyes. Visual evoked responses were more reduced than electroretinogram responses. These results indicate that associated visual field defects and impairment of visual acuity were related not only to a localized staphylomatous ectasia of the posterior globe but also to a lesion of the inner retinal layer, optic nerve, or more proximal visual pathway. This suggests that a developmental abnormality in the embryonic retina may result in hypoplasia of the retinal pigment epithelium and neural elements as well as an anomaly of the optic disc. Investigation of visual function in patients with tilted disc syndrome may help to identify possible concomitant retinal and optic nerve dysfunction.