Bilateral cochlear implantation in infantile Bartter's syndrome

Bartter's syndrome (BS) is a rare group of hereditary salt losing tubulopathies due to impairment of renal transport mechanism. Herein, we report a boy in early childhood who was diagnosed to have infantile Bartter's syndrome. Laboratory investigations revealed hypokalaemia and metabolic alkalosis which was managed with oral potassium chloride supplementation. The inner ear showed no anatomical abnormalities. Sequential cochlear implantation was performed 4 months apart. Postoperative electrical stimulation yielded good response and a symmetric mapping. The patient was on regular audio-verbal therapy. Cognitive impairment improved over time. Bilateral cochlear implantation showed excellent auditory outcomes.