In recent years, it was reported that some manifestations of this disease were correlated with the specific genotype (SLC4A1, ATP6V1B1, or ATP6V0A4 mutations).[14] Patients with dRTA usually suffer from SNHL,[15,16] but the correlation between the mutation and the development of SNHL remains unknown. Here, SLC4A1 is linked to distal renal tubular acidosis.