Cochlear implantation in children with Bartter syndrome: A case report

Bartter syndrome (BS) is an autosomal recessive disorder characterized by polyhydramnios, premature birth, polyuria, renal salt-wasting, hypokalemic metabolic alkalosis, normal blood pressure with increased levels of renin and aldosterone, and the presence of hearing loss. Mutations in BSND, CLCNKA, and CLCNKB cause the disorder. We present a 3-year-old girl with BS type 4 and sensorineural hearing loss who received cochlear implantation (CI) after failing a hearing screening. The patient had a successful left-side implantation but later developed a flap infection, initially treated with antibiotics and surgical repair using a vascularized flap, which failed. During the same repair procedure, the right ear was implanted successfully. A third surgery was required to remove the left implant and close the wound with a temporoparietal flap, performed by plastic surgery. This is the earliest report on managing device extrusion in BS. The literature review highlights that the outcomes of CI in patients with BS vary significantly within each syndrome group and that the presence of additional disabilities accentuates this diversity. Our case illustrates that CI can improve the quality of life for patients with BS, but a multidisciplinary team is crucial for managing these cases. We stress the need for further studies to evaluate realistic outcomes and complication rates, as this will guide future treatment strategies.