Table 1.

Clinical Subtypes of Usher Syndrome: Phenotype, Onset, and Associated Genes/Loci

Subtype	RP Onset	SNHL	Vestibular Dysfunction	Associated Genes
USH1	First decade	Profound congenital, unintelligible speech	Present, congenital	MYO7A, USH1C, CDH23, PCDH15, USH1G, 21q21, 15q22-15q23, 10p11.21-10q21.1
USH2	Second decade	moderate, intelligible speech	None	USH2A, ADGRV1, WHRN
USH3	First decade	Progressive	Variable	CLRN1, HARS
USH4	Fifth decade	Late onset, progressive, moderate/severe	None	ASRG