Purpose: Patients with Williams syndrome have been found to have a hig
h prevalence of strabismus. This may be due to a primary sensory abnor
mality. The purpose of this study was to determine the prevalence of s
ubnormal binocular vision in patients with Williams syndrome. Methods:
Patients being followed in an interdisciplinary Williams syndrome cli
nic were prospectively evaluated to determine their binocular status.
Results: Eleven patients with Williams syndrome underwent an ophthalmo
logic evaluation. Twenty-seven percent of patients had strabismus (3/1
1). Eight patients demonstrated no measurable strabismus. Six of these
patients were found to have monofixation syndrome. Conclusions: There
is a high prevalence of subnormal binocular vision in Williams syndro
me. This subnormal binocular vision may explain the high prevalence of
strabismus in this syndrome.