Manchaiah, VKC, Zhao, F., Danesh, A.A. & Duprey, R.
International Journal of Pediatric Otorhinolaryngology, 75, 151-158.
Publication year: 2011


Auditory neuropathy is a hearing disorder where outer hair cellfunction within the cochlea is normal, but inner hair cell and/or the auditory nerve function is disrupted. It is a heterogeneous disorder which can have either congenital or acquired causes. Furthermore, the aetiology of auditory neuropathy is vast, which may include prematurity, hyperbilirubinaemia, anoxia, hypoxia, congenital brain anomalies, ototoxic drug exposure, and genetic factors. It is estimated that approximately 40% of cases have an underlying genetic basis, which can be inherited in both syndromic and non syndromic conditions. This review paper provides an overview of the genetic conditions associated with auditory neuropathy spectrum disorders(ANSDs) and highlights some of the defective genes that have been found to be linked to the pathological auditory changes.