Objective:To explore the HRCT features of internal auditory canal(IAC)stenosis and abnormality of cochlear nerve canal(CNC)in children with congenital sensorineural hearing loss(SNHL)and the correlation between IAC stenosis and CNC abnormality.Methods:The images of 67 children with congenital SNHL were analyzed retrospectively,all children had IAC stenosis and/or CNC abnormality diagnosed by HRCT.Results:67 cases(75 ears)had CNC abnormality,8 cases were with bilateral abnormalities and 59 cases with unilateral abnormalities(30 left ears,29 right ears),and among the 75 ears,30 ears complicated with IAC stenosis and 16 ears with other internal ear abnormality.Of the 28 cases(30 ears)with IAC stenosis,2 cases were bilateral stenosis and 26 cases unilateral(11 left ears,15 right ears),8 ears complicated with abnormality of semicircular canal,vestibule or cochlea.IAC stenosis and CNC abnormality were mainly unilateral,and both left and right ears could develop,with no statistically significant differences(both P>0.05).Conclusions:In SNHL children,CNC abnormality is more common than IAC stenosis and has more clinical significance.However,CNC abnormality is easy to be misdiagnosed,which should be highly valued.It is suggested that the CNC width should be routinely measured in the internal ear HRCT.