The c.11713C>T (p.Arg3905Cys) variant in USH2A was present in 0.03281% (1/3048) of South Asian chromosomes in gnomAD v3, which is a low enough frequency to apply PM2_Supporting based on the thresholds defined for autosomal recessive hearing loss by the ClinGen Hearing Loss Expert Panel (PM2_Supporting; gnomad.broadinstitute.org). This variant has been reported in 3 individuals with Usher syndrome and a second pathogenic or likely pathogenic variant identified in USH2A, one of which was confirmed to be in trans with the p.Arg3905Cys variant (PM3_Strong; PMID: 28559085, 27208204, 31877679). At least one of these probands was confirmed to have both hearing loss and retinitis pigmentosa, features highly specific for Usher syndrome (PP4). The REVEL computational prediction tool produced a score of 0.8, which is above the threshold necessary to apply PP3. In summary, this variant meets criteria to be classified as likely pathogenic for autosomal recessive Usher syndrome based on ACMG/AMP criteria applied as specified by the Hearing Loss Expert Panel: PM3_Strong, PM2_Supporting, PP3, PP4.