Background: The nephrotic syndrome (NS) is a glomerular disease, in which the glomerular capillary wall becomes no longer impermeable to proteins. The organs of the inner ear bear a physiological similarity to the kidney, both being concerned with maintaining the electrolyte concentration gradient. There is an association between renal and inner ear disorders, either genetically determined as in Alport syndrome and branchio-oto-renal syndrome or acquired as in acute kidney injury and chronic kidney disease. Objective: To evaluate hearing status in children with idiopathic nephrotic syndrome and to identify the underlying etiology. Subjects and methods: This was a case controlled study which included 40 patients with idiopathic nephrotic syndrome. Patients were selected according to a certain inclusion and exclusion criteria from those attending the pediatric nephrology clinic, Al-Hussein University Hospital. Those patients have been classified into 2 equal groups: Group 1: Steroid sensitive nephrotic syndrome (SSNS) and Group 2: Steroid resistant nephrotic syndrome (SRNS). Twenty apparent healthy age and gender matched children were included as a control group (group3).      All patients and control subjects were subjected to detailed history, full clinical examination and laboratory assessment including CBC, renal function, serum electrolytes (Na, K, Ca), investigations specific for nephrotic syndrome and audiometric evaluation, and repeated after remission for those who were in relapse. Results: Sensorineural hearing loss (SNHL) was detected in 8 of the 40 patients (20%). Six out of the 20 patients with SRNS (30%) had SNHL (4 mild, 2 moderate) and 2 out of 20 patients with SSNS (10%) had mild sensorineural loss. Conclusion: Hypocalcemia, hypercholesterolemia, and hyponatremia seemed to affect hearing and should be followed up in childhood nephrotic syndrome. Disease flare and steroids using for a long period may also be risk factors for SNHL in children with NS.