Auditory neuropathy spectrum disorder (ANSD) is one of the many hearing impairments, where sound may enter the inner ear, but does not have a clear path from the ear to the brain. Since the indicators show up in different ways it is often hard to diagnose and treat. This objective of this research is to better understand the genetic origins of ANSD and study if genetic testing could help spot where the hearing problem comes from by following a literature review in PubMed database. In a clinical trial, researchers studied 23 people who had ANSD who had no blood relations and collected information from their medical records. Specimens were collected from each patient, and their DNA were deconstructed using advanced genetic technique, which included whole-exome and next-generation sequencing, with results confirmed by additional testing. According to the clinical findings, the patients were organized into three separate groups: non-syndromic ANSD, which is when hearing loss occurred alone; syndromic with peripheral neuropathy, where ANSD occurred with nerve problems outside the brain and spinal cord; and syndromic plus central nervous system involvement with hearing disorder and brain / spinal cord problems. The researchers established numerous genetic variants in genes such as Otoferlin (OTOF), Pejvakin (PJVK), and Diaphanous-related formin 3 (DIAPH3). The severity of hearing loss among patients ranged between mild and profound. In general, the study demonstrated that ANSD can be caused by mutations in various genes which makes genetic testing a valuable tool. It can help differentiate ANSD from other related conditions, determine whether it is part of a larger disorder, and provide useful information for predicting the outcomes and guiding treatment discussions, options and decisions.