목적: CHARGE syndrome is a common congenital anomaly. Hearing loss affects 60-90% of these children. With the advent of more sophisticated temporal bone computed tomography, more abnormalities of the middle and inner ear are found. We aim to describe the detailed CT findings and the correlation with audiograms in CHARGE. 방법:We performed a retrospective review of 12 patients with CHARGE syndrome, identified between 1990 to 2010 who underwent temporal bone computed tomography for evaluation of their hearing loss at Princess Margaret Hospital for Children, Western Australia. 결과:We present our findings of the twenty four ears in terms of the cochlear, SCC, middle ear, facial nerve, external auditory canal, venous and jugular anomalies.The internal auditory canal (IAC) was normal in 83.3% (n=20) of ears. There were three (12.5%) ears with an enlarged basal turn four (13.3%) each with hypoplastic & incompletely partitioned apical turn. Majority (n=13, 56.5%) of the vestibules were dysplastic. Up to 70.8% had abnormalities of the SCC. The ME cavity was normal in 55% (n=11) and up to two third had some abnormality of the ossicles and up to 70% had an abnormality of the Facial nerve (CN7) segments, especially in the labyrinthine segment. We also found that the CT findings did not correlate with the audiogram. 결론:The management of children with CHARGE syndrome is complex requiring early evaluation and close attention of the multidisciplinary team. Early identification of hearing deficits is vital for the childs linguistic development.