ARSG and Atrophy: In 2018, a homozygous missense variant (Supplemental Table 1) in the arylsulfatase G (ARSG) gene (OMIM 610008) was associated with an atypical USH phenotype characterized by late-onset RP with a distinctive retinal phenotype of ring-shaped atrophy along the arcades, late-onset progressive SNHL, and no vestibular involvement (Khateb et al. 2018).