The authors enrolled 95 patients with keratoconus and 96 matched controls in a cross-sectional study to determine if mitral valve prolapse and hypermobile joints occur with greater frequency in individuals with keratoconus than in individuals without keratoconus. The hypothesis that keratoconus may not be a distinct eye disease, but a nonspecific sign representing a more generalized systemic disorder, possibly a mild collagen tissue abnormality, was considered. M-mode and two dimensional echocardiography and cardiac auscultation detected no statistically significant difference in the prevalence of mitral valve prolapse in patients with keratoconus compared with controls. Formal, systematic examination of five joints also failed to detect a statistically significant difference in the prevalence of hypermobile joints in keratoconus patients and controls. However, as an ancillary finding, a significantly higher proportion of patients with keratoconus was found to have a history of hay fever than was the case with controls.