GJB2 and sensorineural hearing loss disorder: Of over 150 genes identified to be associated with human SNHL, mutations in genes encoding ion channels (e.g., KCNQ1, KCNQ4, TMC1, P2RX2, KCNJ10) [86,87,88,89], connexin hemichannels (e.g., GJB2, GJB6) [90,91] or synaptic proteins (OTOF) [92] have also been associated with hereditary forms of SNHL.