Approximately 60 % of hearing impairment is caused by genetic defects, known as hereditary hearing loss. Although gene therapy for hereditary hearing loss has made progress, effective prevention and control have not yet been achieved. Hereditary hearing loss is usually caused by damage to the inner ear cells.Induced pluripotent stem cells (iPSCs) can be derived from adult cells and induced to differentiate into pluripotent stem cells. Subsequently, through two-dimensional or three-dimensional induction, they can be further induced in vitro into inner ear sensory epithelial cells like cells, such as hair cells, spiral ganglion neurons and organoids. In recent years, with the development of iPSC technology, its application in the research of hereditary hearing loss has become increasingly prominent. This review will provide an overview of the progress in the application of iPSCs in the research of hereditary hearing loss, covering aspects such as the advantages of iPSCs, induction strategies, and application methods.