Table 4.

Modifiable Clinical Features of USH Phenotypes and Genotypes (Cataract, Refraction, Cystoid Macular Lesions)

	Total	USH1	MYO7A	CDH23	USH1C	USH2	USH2A	ADGRV1	USH3/CLRN1	USH4/ARSG
Number	145	36	23	7	1	106	75	16	2	1
Age (yr), mean ± SD	43.31 ± 15.76	42.74 ± 15.1	42.0 ± 15.7	44.5 ± 15.7	48	43.0 ± 15.7	43.0 ± 15.6	46.3 ± 11.8	48	84
Len status
Eyes	270 (93.1%)	70 (97.2)	46 (100%)	12 (85.7)	2 (100%)	196 (92.5)	138 (92.0%)	32 (100%)	2 (100%)	2 (100%)
Cataract eyes	120 (44.4%)	29 (41.4%)	21 (45.7%)	4 (33.3%)	—	89 (45.4%)	65 (47.1%)	16 (50.0%)	—	2 (100%)
IOL eyes	89 (33.0%)	23 (32.9%)	17 (37.0%)	0	2 (100%)	64 (32.7%)	50 (36.2%)	6 (18.8%)	2 (100%)	—
Clear lens eyes	61 (22.6%)	18 (25.7%)	8 (17.4%)	8 (66.7%)	—	43 (21.9%)	23 (16.7%)	10 (31.3%)	—	—
≥1 eye with cataract	104 (77.0%)	27 (77.1%)	20 (87.0%)	2 (33.3%)	1 (100%)	75 (76.5%)	56 (81.2%)	11 (68.8%)	1 (100%)	1 (100%)
Bilateral cataract	101 (74.8%)	25 (71.4%)	18 (78.3%)	2 (33.3%)	1 (100%)	74 (75.5%)	55 (79.7%)	11 (68.8%)	1 (100%)	1 (100%)
Refraction
Phakic eyes	124 (42.8%)	38 (52.8%)	26 (56.5%)	8 (57.1%)	0	84 (39.6%)	50 (33.3%)	20 (62.5%)	0	2 (100%)
SE ametropia DS, mean ± SE	−1.27 ± 6.02	−1.53 ± 6.07	0.16 ± 2.44	−0.91 ± 2.14	—	−2.56 ± 3.07	−3 ± 2.39	−0.66 ± 1.13	—	3.63 ± 0.88
Cylinder DC, mean ± SE	1.47 ± 1.07	1.54 ± 1.06	1.57 ± 0.78	2.19 ± 1.37	—	1.45 ± 0.95	1 ± 0.90	1.07 ± 0.99	—	0.25 ± 0.35
CML
CML	208 (71.7%)	66 (91.7%	46 (100%)	8 (57.1)	2 (100%)	138 (65.1%)	104 (69.3%)	22 (68.8%)	2 (100%)	2 (100%)
CML eyes	56 (26.9%)	14 (21.2%)	10 (21.7%)	4 (50.0%)	0	42 (30.4%)	34 (32.7%)	5 (22.7%)	—	0
Bilateral CML (patients)	22 (21.2%)	6 (18.2%)	4 (17.4%)	2 (50.0%)	0	16 (23.2%)	13 (25.0%)	2 (18.2%)	—	0

CML, cystoid macular lesions; IOL, intraocular lens; SD, standard deviation; SE, spherical equivalent.

Percent affected were calculated by assessing patients/eyes with the relevant available clinical data.