The c.631A>G (p.Ser211Gly) variant in MYO7A was present in 0.009% (6/64570) of non-Finnish European chromosomes in gnomAD, which is a low enough frequency to apply PM2_Supporting based on the thresholds defined by the ClinGen Hearing Loss Expert Panel for autosomal recessive hearing loss (PM2_Supporting). This variant has been observed in at least 3 probands with Usher syndrome who carried other pathogenic or likely pathogenic variants in MYO7A; in two cases the other variant was confirmed to be in trans (PM3_Strong; PMID: 25472526; Laboratory for Molecular Medicine internal data, SCV000059882.6). All three of these probands displayed hearing loss and retinitis pigmentosa, features highly specific for MYO7A and Usher syndrome (PP4). The REVEL computational prediction analysis tool produced a score of 0.96, 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 (PM2_Supporting, PM3_Strong, PP3, PP4).