Hearing is the ability of a person to recognize sound in the surroundings and it makes communication possible. Ear is the human organ serving as a transducer that perceives signals from the environment and converts it into detectable forms for interpretation by the brain. The auditory system is among one of the most highly studied systems. Researchers have described the physiological function of the system in detail but due to its complexity, the genetic mechanisms and genes implicated in auditory function are still being revealed. Numerous studies on the genetics of hearing indicate hearing loss as one of the most common and prevalent disorders as it affects approximately five million people worldwide. Besides hearing loss, there are several other pathologies of auditory system which are common and have an established genetic basis. In this chapter, we will introduce the genetics of some common auditory pathologies including syndromic and non-syndromic hearing loss, auditory neuropathy, age-related hearing loss, and tinnitus. These understandings will 1 day lead to better diagnosis, management, and cures.