Congenital sensorineural hearing loss (SNHL) is caused by genetic defects in about 50% to 60% of cases, which is often referred to as hereditary deafness. Although progress has been made in treating hereditary deafness, effective prevention and control have not yet been achieved. Induced pluripotent stem cells (iPSCs) are reprogrammed from somatic cells into pluripotent stem cells, which can then differentiate into hair cells, spiral ganglion neurons, and other inner ear sensory epithelial cells or organoids through induction methods of two-dimensional or three-dimensional in vitro. Therefore, it has received widespread attention in the field of regenerative medicine. With the continuous advancement of iPSCs technology, its application in the field of hereditary deafness research is becoming increasingly widespread. This paper reviews the the application of iPSCs in hereditary deafness research, focusing on their advantages, induction strategies, and application methods.