In autosomal recessive distal renal tubular acidosis (dRTA), progressive bilateral sensorineural hearing loss is common association. This coexistence is due to the mutations of a gene expressed both in the kidney and in the cochlea. The aim of this study was to assess the correlation between hearing loss and dRTA. In this study, 26 patients diagnosed with renal tubular acidosis were evaluated. Diagnosis of dRTA was based on clinical manifestations and detection of normal anion gap metabolic acidosis, urine pH higher than 5.5. Tympanometry was performed to all subjects but audiometry or ABR was performed in patients with DRTA according to patient age and cooperation. The median age of the studied patients was 4.7 years, 57.7% were males, and 42.3% were females. Twelve patients (46.2%) had bilateral sensorineural hearing loss, consisting of 6 of 15 boys (40%) and 6 of 11 girls (54.5%). There is no statistically significant difference between patients of dRTA with SNHL and others with CHL or normal hearing in serum pH, Ca and urinary pH. This study indicated that a significant percentage of the children with dRTA had sensorineural hearing loss. It is recommended to investigate hearing impairment in all children with dRTA and to study the genotype phenotype relation in our population.