It has previously been described that missense variants in ADGRV1 can be associated with isolated hearing loss,63 with currently 55% of reported missense variants associated with isolated hearing loss, vs 45% with USH2 (https://my.qiagendigitalinsights.com/bbp/view/hgmd/.php, reviewed in March 2023), without clear clusters or hot spots. This evidence concerns the gene USH2A and hearing loss disorder.