Table 2.

Demographics by USH Subtype, Genetic Resolution Rate, and Genotype

	Total	USH1	USH2	USH3	USH4
n = (%)	145	35 (24.1%)	107 (73.8%)	2 (1.4%)	1 (0.7%)
Pedigrees	117	32	82	2	1
Male	74 (51.0%)	16 (50.0%)	57 (53.8%)	0	1 (100%)
Age in years at diagnosis ± SD	26.6 ± 12.0	20.9 ± 9.3	27.6 ± 11.5	48	64
Age at most recent examination (yr) ±SD	43.31 ± 15.76	42.74 ± 15.1	43.0 ± 15.7	48	84
Genetically resolved	119 (82.1%)	27 (77.1%)	89 (83.2%)	2 (100%)	1 (100%)
Genotype	Biallelic 119 (82.1%), SA 6 (4.1%), Unresolved 20 (13.8%)	MYO7A 22 (62.9%), CDH23 4 (11.4%), USH1C 1 (2.9%), SA 3 (8.5%), Unresolved 5 (14.3%)	USH2A 74 (69.2%), ADGRV1 15 (14.0%), SA 3 (2.8%) Unresolved 15 (14.0%),	CLRN1 2 (100%)	ARSG 1 (100%)

SA, single allele; SD, standard deviation.