목적: The differential natural course of hearing, depending on the etiology of prelingual auditory neuropathy spectrum disorder (ANSD), renders a dilemma determine appropriated rehabilitation. We, herein, provide an etiologic portfolio associated with prelingual ANSD and classify this condition in reference to etiology and audiological characteristics. 방법:We retrospectively reviewed the prelingual ANSD database. Imaging and genetic testing were analyzed, and the etiology of prelingual ANSD were classified accordingly. For patients who underwent ASSR first and pure tone audiometry (PTA) later, correlation analyses between the two tests were performed depending on etiology. 결과:A total of 73 patients were enrolled. Specifically, in non-syndromic bilateral cases evidenced by definite etiology (N=23), genetic variants or cochlear nerve deficiency were predominant, comprising 60.9% (N=14), followed by kernicterus (N=4, 17.4%). While the most frequent causative etiology was genetic variants (91.7%) in syndromic bilateral cases, most unilateral ANSD cases had cochlear nerve deficiency (64.3%). The causative genes associated with prelingual ANSD were OTOF, PLP1, CNTNAP1, MELAS, ECHS1, F213I/F213I, ATP1A3, and PINK1. Positive significant correlations between ASSR and PTA was noted in cases with genetic variants and cochlear nerve deficiency. Conversely, audiogram in cases with kernicterus or hypoxic ischemic encephalopathy (HIE) improved over time, indicating no significant correlation between ASSR and PTA. 결론:We present a portfolio of etiology and audiological characteristics associated with prelingual ANSD. A preponderance of genetic variants was observed exclusively among syndromic- and non-syndromic bilateral ANSD cases, whereas cochlear nerve deficiency is predominant in unilateral ANSD cases. Depending on the causative etiology, the differential correlation of ASSR and pure tone audiogram can be highlighted, which justifies the implication of etiology-based tailored rehabilitation in prelingual ANSD.