Deafness or hearing loss is a major issue in human health. Inner ear hair cells 
are the main sensory receptors responsible for hearing. Defects in hair cells 
are one of the major causes of deafness. A combination of induced pluripotent 
stem cell (iPSC) technology with genome-editing technology may provide an 
attractive cell-based strategy to regenerate hair cells and treat hereditary 
deafness in humans. Here, we report the generation of iPSCs from members of a 
Chinese family carrying MYO15A c.4642G>A and c.8374G>A mutations and the 
induction of hair cell-like cells from those iPSCs. The compound heterozygous 
MYO15A mutations resulted in abnormal morphology and dysfunction of the derived 
hair cell-like cells. We used a CRISPR/Cas9 approach to genetically correct the 
MYO15A mutation in the iPSCs and rescued the morphology and function of the 
derived hair cell-like cells. Our data demonstrate the feasibility of generating 
inner ear hair cells from human iPSCs and the functional rescue of gene 
mutation-based deafness by using genetic correction.