The syndrome of distal renal tubular acidosis and nerve deafness is a distinct nosological entity that is inherited as an autosomal recessive trait. Features associated with distal renal tubular acidosis include impaired urine acidification leading to severe hyperchloremic hypokalemic metabolic acidosis, hypercalciuria, hypocitraturia, potassium depletion, nephrocalcinosis (prominent renal tract calcification) and nephrolithiasis. Nephrocalcinosis is thought to result from the hypercalciuria and hypocitraturia.