Embryonic stem cells (ESCs) are pluripotent stem cells that are derived from early embryos. Induced pluripotent stem cells (iPSCs) are somatic cell-derived reprogrammed cells that have equivalent characteristics to ESCs. Disease-specific iPSCs have started to be used for the investigation of and development of the treatment for several diseases. These cells have been intensively used for the regeneration of inner ear. In this chapter, we summarize the background of ESCs and iPSCs, introduce recent advances in disease-specific iPSCs, and discuss the difference between ESCs and iPSCs in terms of clinical application. Then, the major achievement for the induction of inner ear sensory cells and the transplantation of ESC- and iPSC-derived neural precursor cells are overviewed.