Renal tubular acidosis with nerve deafness.

Renal tubular acidosis (RTA) is a non -uraemic clinical syndrome of disordered renal acidification.1 In the classic distal RTA (Type 1), subjects have hyperchloraemic acidosis, hyponatraemia and hypokalaemia. The urinary pH remains high (pH > 6.2), even in the presence of systemic acidosis. Although the defect is permanent, the prognosis is good when the diagnosis, with correct management, is established early in order to prevent nephrocalcinosis and secondary renal damage.2 Inheritance of the classic distal RTA (Type 1) is autosomal dominant. Several subtypes of distal RTA have been described such as incomplete distal RTA, distal RTA with bicarbonate wasting, transient distal RTA in infants and distal RTA with nerve deafness. We describe a patient, to our knowledge the first case from Ireland, with distal renal tubular acidosis and bilateral sensorineural deafness.