The aim of this study was to provide more information on the causes of sensorineural hearing loss (SNHL) in children cochlear implant candidates in Croatia. The retrospective study included 270 children candidates for cochlear implantation between January 1997 and January 2005 at our institution. The medical assessment of the candidates included the history, physical examination, radiologic evaluation of the temporal bone and audiologic assessment. A family history of SNHL had 82 (30.4%) candidates. The prematurity and/or complicated perinatal course was found in 35 (12.9%) of candidates. Computerized tomography (CT) scan analysis identified 44 (16.3%) candidates presenting with an inner ear malformation. Overall, a definite or probable cause of SNHL was identified in 58.9% of candidates and 41.1% had no obvious cause. The results of the study might give us better insight into the potential causes of SNHL and allow more timely intervention, allowing children with SNHL to reach their potential.