Abstract
Bartter syndrome (BS) represents a group of autosomal recessive salt-losing nephropathy, characterized by hypokalemic metabolic alkalosis with normal or low blood pressure. Hearing defect is a feature of BS Type IV and is typically absent in BS I, II, or III. We report the case of a 2-year-old boy with severe to profound sensory neural deafness who diagnosed with BS Type III in the neonatal period. His deafness was noted at 9 months. He used hearing aids regularly for 1 year with very limited benefit, an aided visual reinforcement audiometry test resulted in a reading of only 55 dB. Cochlear implantation was performed at the age of 3 years with excellent postoperative audiometric results and improvement in speech performance. To the best of our knowledge, this is the second-documented case of BS Type III with bilateral sensory neural deafness and the first-reported case of cochlear implantation in such a patient.