Author(s): Sabarish A
Auditory neuropathy Spectrum Disorder (ANSD) is a unique hearing disorder where outer hair cell status is found to be normal, but inner hair cell and/or the synaptic connections to the auditory nerve is disrupted. It is a diverse group of hearing disorder which can be manifested either congenitally or can be late in onset. However, there are various etiologies of auditory neuropathy which is represented in the literature, which includes birth related risk factors for hearing loss like prematurity, bilirubin synthesis issues, lack of oxygen supply to the baby during or after delivery, and genetic actors. It is estimated that approximately 40% of cases have an underlying causal factor as genetic origin, which can be inherited in either syndromic or non-syndromic way. The below case report provides an extra support for the fundamental genetic trait in ANSD. The study presents two congenital ANSD cases where, both children were diagnosed as congenital ANSD.
View PDF