USH2A and hearing loss disorder: However, later onset hearing loss in patients with USH2A variants who have initially presented with NS-ARRP was first reported by Rivolta et al., who found that this was significantly associated with the c.2299delG, p.(Glu767SerfsTer21) variant in their cohort and proposed that subjective late-onset hearing loss can occur in NS-ARRP secondary to USH2A variants [11].