Causative variants in more than 150 genes have been identified so far, including GJB2 (OMIM 220290), SLC26A4 (OMIM 605646), OTOF (OMIM 603681), CDH23 (OMIM 601067), and STRC (OMIM 606440).6 Understanding the causative genes for a child’s HL can provide important prognostic information; many genes and variants have characteristic clinical phenotypes and can be broadly categorized into those principally associated with nonsyndromic stable sensorineural HL (SNHL), nonsyndromic progressive SNHL, and syndromic SNHL. This evidence concerns the gene CDH23 and sensorineural hearing loss disorder.