Endolymphatic Sac Enlargement in a Girl with a Novel Mutation for Distal Renal Tubular Acidosis and Severe Deafness

Figure 1

(a) Results of bicarbonate loading test with 1 mmol/kg NaHCO₃ infusion. (b) Ultrasound of the left and right kidney with evidence of nephrocalcinosis. (c) Axial FIESTA cerebral MRI: bilateral enlargement of the endolymphatic sac (arrows), markedly larger on the right. (d) Schematic image of the inner ear. (e) Results of free field measurement and inserted right ear audiogram.