title: Cochlear implantation in Pendred syndrome.
abstract: OBJECTIVE
  The objective of this study was to examine the anatomical, audiological, and surgical factors that can affect the hearing outcome in Pendred syndrome cochlear implant recipients. Patients with Pendred syndrome represent challenging cochlear implant candidates, combining goiter, severe-to-profound hearing loss, and inner-ear dysplasias. The hearing outcome and further relevant parameters in such patients have not been adequately evaluated yet.
  
  
  METHODS
  Retrospective study in a tertiary referral cochlear implant centre. Data of patients with Pendred syndrome, who had undergone cochlear implantation in the 10-year period 1999-2008, were evaluated.
  
  
  RESULTS
  Five cochlear implant recipients with Pendred syndrome were identified, four unilateral and one bilateral. The average postoperative follow-up duration for these patients was 4.8 years. Enlarged vestibular aqueduct and Mondini dysplasia were the most common anatomical findings. Although these anatomical variances did not affect the electrode insertion, they prolonged the duration of the surgery. Cerebrospinal fluid gusher was the most common intraoperative complication. All patients enjoyed satisfactory hearing rehabilitation.
  
  
  CONCLUSIONS
  Cochlear implantation is the proper method for optimal hearing rehabilitation in patients with Pendred syndrome. Although the precise pathogenetic mechanism and the genetic background of deafness in Pendred syndrome have not been completely understood, the preexisting hearing experience represents a positive factor for satisfying hearing outcome. The inner-ear malformations can cause mild surgical difficulties and extended surgery duration. The cochlear implant surgeon should be aware of these difficulties to avoid complications.
authors.affiliation: Otorhinolaryngology Department, Hanover Medical University, Hanover, Germany. Kontorinis.Georgios@mh-hannover.de
authors.forename: Georgios
authors.initials: G
authors.lastname: Kontorinis
authors.forename: Thomas
authors.initials: T
authors.lastname: Lenarz
authors.forename: Anke
authors.initials: A
authors.lastname: Lesinski-Schiedat
authors.forename: Juergen
authors.initials: J
authors.lastname: Neuburger
mesh_terms: D065634:Cerebrospinal Fluid Leak; D002559:Cerebrospinal Fluid Rhinorrhea / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation / Q000534:rehabilitation*; D005260:Female; D005500:Follow-Up Studies; D006044:Goiter, Nodular / Q000473:pathology / Q000534:rehabilitation / Q000601:surgery; D006319:Hearing Loss, Sensorineural / Q000473:pathology / Q000534:rehabilitation / Q000601:surgery; D006801:Humans; D007223:Infant; D007431:Intraoperative Complications / Q000517:prevention & control; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000517:prevention & control; D012189:Retrospective Studies; D012720:Severity of Illness Index; D014723:Vestibular Aqueduct / Q000002:abnormalities* / Q000601:surgery; D055815:Young Adult