title: Treating hearing loss in patients with infantile Bartter syndrome.
abstract: OBJECTIVE
  Most existing studies about infantile Bartter syndrome (BS) have focused on renal function, and deafness has not been closely studied. Our objective was to evaluate the treatment of hearing impairment in children with infantile BS and analyze relevant, unexplored issues.
  
  
  METHODS
  Retrospective chart review.
  
  
  METHODS
  The present study was conducted in a tertiary referral center over a 20-year period involving children with infantile BS. Demographic factors, general health status, genetic information, features of hearing loss, and the outcome of cochlear implantation as determined mainly by the categories of auditory performance (CAP), as well as imaging of the temporal bones, were evaluated.
  
  
  RESULTS
  Six children with infantile BS were identified, four girls and two boys. One child had terminal renal insufficiency and one had undergone kidney transplantation; all children had several hospital admissions due to renal dysfunction. Sensorineural hearing loss was congenital, bilateral, and profound in all children. Five patients were treated with cochlear implants resulting in improved speech perception and development without any exceptional performance (CAP scores, 4-6), mainly because of the delayed treatment and the comorbidities. Anatomic ear anomalies were not observed in any case.
  
  
  CONCLUSIONS
  Hearing loss in children with infantile BS is congenital and profound but not related to inner ear malformations. Although cochlear implantation results in certain benefits, general health status and delayed referral to cochlear implant centers have a negative impact on speech perception and development.
authors.affiliation: Department of Otolaryngology, Hanover Medical School, Hanover, Germany. gkontorinis@gmail.com
authors.forename: Georgios
authors.initials: G
authors.lastname: Kontorinis
authors.forename: Anja M
authors.initials: AM
authors.lastname: Giesemann
authors.forename: Zoe
authors.initials: Z
authors.lastname: Iliodromiti
authors.forename: Juergen
authors.initials: J
authors.lastname: Weidemann
authors.forename: Talal
authors.initials: T
authors.lastname: Aljeraisi
authors.forename: Burkard
authors.initials: B
authors.lastname: Schwab
mesh_terms: D001477:Bartter Syndrome / Q000150:complications*; D003054:Cochlear Implants; D005260:Female; D034381:Hearing Loss / Q000209:etiology* / Q000473:pathology / Q000628:therapy*; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome