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Table 1 The grading system for the cystic macular lesions severity and selected tomograms examples in Usher syndrome patients. A-B Mild: the right eye of 26 year male with mutation in USH2A:c.7595-3C > G+ c.7595-2144A > G, having less than 10 small size cystic lesions and central macula thickness (CMT) within 200 μm (A), the right eye of 38 year male with mutation in USH2A: E767fs, having less than 10 medium size cystic lesions (B); C-D Moderate: the right eye of 14 year female with mutation in MYO7A: L2186P *2, having more than 10 medium size cystic lesions and CMT up to 300 μm (C), the right eye of 31 year male with mutation in USH2A:T4337M + C717G, having up to 20 small and up to 10 medium size cystic lesions and one large diameter cyst (D); E-F severe: the right eye of 35 year female with mutation in USH2A:V218E + G660R, having more than 10 medium size cystic lesions, more than 5 large and several extra large (E), the right eye of 24 year male with mutation in MYO7A:N1182K*2, having several extra large diameter cystic lesion (F). * The presence of epiretinal membrane

From: Novel grading system for quantification of cystic macular lesions in Usher syndrome