Gene Selection
The DVD v.8.1 includes 152 genes and microRNAs known to cause hearing loss-related phenotypes including NSHL, NSHL mimics such as Usher, Perrault, and Pendred syndromes (PDS [MIM: 274600]), and common forms of syndromic hearing loss like Alström (ALMS [MIM: 203800]), branchio-oto-renal (BOR1 [MIM: 113650], BOR2 [MIM: 610896]), Jervell and Lange-Nielsen (JLNS1 [MIM: 220400], JLNS1 [MIM: 612347]), and Wolfram (WFS1 [MIM: 222300], WFS2 [MIM: 604928]) syndromes (Table S1). The genes are curated from the Hereditary Hearing Loss Homepage and published literature after careful review of the supporting evidence including the strength of the genetic data (linkage data, allele frequency and deletriousness of the candidate variant, segregation analysis) and functional data (gene expression in inner ear, in vivo experiments, animal models). The gene list is regularly updated by adding or removing genes based on newly published data.