The study evaluated the prevalence and underlying diseases of hearing loss (HL) in patients with childhood-onset chronic kidney disease (CKD).   In this retrospective study in single center, pediatric patients with CKD stage 2-5 were recruited. Audiometric examinations were performed using pure tone audiometry (PTA) or automated brainstem response test (ABRT). Genetic or syndromic disease in addition to HL and CKD was defined as syndromic HL. A total of 421 patients (279 males, 142 females) were recruited. Audiometric examinations were performed in 178. The mean onset age of renal symptom was 4.1 ± 4.8 years and the mean follow-up period was 9.4 ± 6.2 years. The patients were grouped according to the causes of CKD: congenital anomalies of the kidney and urinary tract (CAKUT, n=184), glomerulopathy (GP, n=105), cystic kidney diseases (CYST, n=39), perinatal problem (PP, n=29), and others (n=64). HL was detected in 82, including 51 with sensorineural hearing loss (SNHL), 30 with conductive hearing loss (CHL), and 1 with mixed HL.  The prevalence rates of HL were as follows: 16.8% (SNHL:CHL:mixed HL 7.6:8.7: 0.5) in the CAKUT, 28.6% (22.9:5.7:0) in the GP, 12.8% (5.1:7.7:0) in the CYST, 24.1% (20.7:3.4:0) in the PP, and 14.1% (7.8:6.3:0) in the others group. The prevalence of HL increased as the CKD stage increased (P=0.002), and the prevalence of HL and SNHL were higher in GP (P=0.007 and 0.021, respectively). Of a total 82 with HL, 41 had syndromic HL (SNHL:CHL:mixed HL 30:10:1). Among 51 with SNHL, 30 patients were syndromic HL. Otitis media with effusion was present in 27 of 30 patients with CHL. The overall prevalence of HL were 19.5%, including 12.1% of SNHL and 7.1% of CHL. SNHL was a manifestation of genetic or syndromic disease in more than half, while CHL was associated with otitis media with effusion mostly.