목적: Auditory neuropathy (AN) is easy to miss these patients during the newborn hearing screening and misdiagnose them as sensorineural hearing loss or sudden hearing loss. The aim of this investigation is to describe a group of infants who exhibited electrophysiologic responses characteristic of AN, but later were subsequently identified as having an anatomical etiology. 방법:We retrospectively reviewed the imaging and functional tests on infants under 24 months of age between May, 2005 and Dec, 2014 and recruited only subjects with mainly unilateral profound hearing loss, available imaging studies and otoacoustic emission results (or cochlear microphonics data). Imaging studies were reviewed for the recruited patients to identify the anatomical abnormality. In total 27 subjects have been recruited. Unilateral profound hearing loss was found in 18 patients of these, 56% occurred on the left side. Nine had a bilaterally profound hearing loss three with cochlear hypoplasia. Syndromic patients were excluded. 결과:Of the 36 ears with profound hearing loss, twenty three were boys and thirteen were girls. Average age at time of the final diagnosis of the imaging and functional test was 0.8 years. In total, twenty cases (55.6%) had narrow bony cochlear nerve canal (nBCNC) as documented by TBCT or cochlear nerve hypoplasia as documented by MRI of these, seven cases (35.0%) was diagnosed as having auditory neuropathy characteristics. Interestingly, nBCNC or cochlear nerve hypoplasia was detected in all subjects with this characteristic. In contrast, none of the subjects without anatomical abnormality in imaging studies showed the AN characteristics. This association was statistically significant (p=0.011). 결론:This study demonstrates that most infants with AN characteristics and profound hearing loss unilaterally or bilaterally need to be evaluated with radiologic test to reach the final diagnosis.