Ophthalmologic correlates of disease severity in children and adolescents with Wolfram syndrome

J Hoekel, SA Chisholm, A Al-Lozi, T Hershey… - Journal of American …, 2014 - Elsevier
Purpose To describe an ophthalmic phenotype in children at relatively early stages of
Wolfram syndrome. Methods Quantitative ophthalmic testing of visual acuity, color vision,
automated visual field sensitivity, optic nerve pallor and cupping, and retinal nerve fiber
layer (RNFL) thickness assessed by optical coherence tomography (OCT) was performed in
18 subjects 5-25 years of age. Subjects were also examined for presence or absence of
afferent pupillary defects, cataracts, nystagmus, and strabismus. Results Subnormal visual …