Results: Among the total 41 ears (from 30 infants) with profound HL, 13 ears (7 (36.8%) of 19 ears with unilateral HL and 6 (27.3%) of 22 ears associated with bilateral HL) showed AN characteristics (37.1%),and 21 ears showed cochlear nerve deficiency (CND) (51.2%). AN characteristics was detected about two times more frequently in cases with CND (38.1%) than with anatomically normal cochlear nerve (20.0%), the difference not reaching a statistical significance probably due to a small sample size. Detection of AN characteristics did not differ between unilateral and bilateral profound HL, even though presence of CND was more frequently detected in cases with unilateral profound HL than with bilateral cases. There were at least five types of etiologies related to AN characteristics in 13 ears (from 10 infants) in our series depending on the laterality of deafness and presence of CND.Conclusions: This study demonstrates that there were diverse etiologies related to AN characteristics from infants with unilateral or bilateral profound HL. Association between CND and AN characteristics is suggestive but not solid at this moment and AN characteristics is not a fully penetrant feature of CND.