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Year : 2020  |  Volume : 26  |  Issue : 2  |  Page : 106-108

Classical bartter's syndrome (Type III) with deafness: A very rare case management

1 King Abdullah Ear Specialist Center, College of Medicine, King Saud University, Riyadh, Saudi Arabia
2 College of Medicine and Research Center, King Saud University, Riyadh, Saudi Arabia

Correspondence Address:
Dr. Farid A Alzhrani
King Abdullah Ear Specialist Center, College of Medicine, King Saud University, PO Box 245, Riyadh 11411
Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/indianjotol.INDIANJOTOL_97_19

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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.

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