The major achievements in disease modeling using a hiPSC-derived inner ear for genetic SNHL include the genes MYO7A, MYO15, and MERRF in HCs [70,89,90], and GJB2 and SLC26A4 (Pendred syndrome) in SCs [106,120,121,122,123,124] (Table 2). This evidence concerns the gene GJB2 and sensorineural hearing loss disorder.