title: Variations in the cochlear implant experience in children with enlarged vestibular aqueduct.
abstract: OBJECTIVE
  To describe the clinical experience and characterize the outcomes of cochlear implantation (CI) in children with isolated enlarged vestibular aqueduct (IEVA) as compared to children with enlarged vestibular aqueduct (EVA) associated with other bony labyrinth abnormalities.
  
  
  METHODS
  Single, tertiary care, institutional retrospective review over 2 decades.
  
  
  METHODS
  The clinical course and outcomes of 55 children with EVA undergoing CI between 1991 and 2013 were reviewed. Test measures included open and closed set speech perception tests, and various speech and language measures.
  
  
  RESULTS
  In 18 children (32.7%), IEVA was the only defect present. In 33 children (60%), EVA occurred concomitantly with incomplete partition type 2 (IP 2) bilaterally, and three children with incomplete partition type 1 bilaterally. Ninety-two percent (51 of 55) occurred bilaterally and had matching bony defects. Mean age of CI was 73.4 months. A statistically significant defect-related and linguistic-status pattern was noted, impacting the timing of implantation: IEVA = 112.8 months, IP 2 = 58.4 months (P < .001), prelingual deafness = 53.8 months, postlingual deafness = 110.8 months (P < .001). Controlling for implant age and hearing loss severity, IEVA children demonstrated superior performance on speech perception tests (8.2 to 20.3 point differences), though statistical significance was inconsistent (P = .01-.40. Performance was also superior in speech and language tests, though statistical significance was never reached (2.9-13.9 point differences; P = .14-.69).
  
  
  CONCLUSIONS
  Children with hearing loss secondary to EVA respond meaningfully to cochlear implantation. However, the severity of temporal bone anomalies in these children has clinical relevance.
  
  
  METHODS
  4.
authors: Department of Otolaryngology-Head and Neck Surgery, Northwestern University, Chicago, Illinois.
authors: Cedric
authors: C
authors: Pritchett
authors: Division of Otology-Neurotology, Department of Otolaryngology-Head and Neck Surgery, University of Michigan Health System, Ann Arbor, Michigan.
authors: Teresa
authors: T
authors: Zwolan
authors: Department of Otolaryngology-Head and Neck Surgery, Henry Ford Health System, Detroit, Michigan.
authors: Farhan
authors: F
authors: Huq
authors: University of Michigan Medical School, Ann Arbor, Michigan.
authors: Amanda
authors: A
authors: Phillips
authors: Hemant
authors: H
authors: Parmar
authors: Mohannad
authors: M
authors: Ibrahim
authors: Department of Radiology, University of Michigan Health System, Ann Arbor, Michigan.|Division of Pediatric Otolaryngology, Department of Otolaryngology-Head and Neck Surgery, University of Michigan Health System, Ann Arbor, Michigan, U.S.A.
authors: Marc
authors: M
authors: Thorne
authors: Division of Otology-Neurotology, Department of Otolaryngology-Head and Neck Surgery, University of Michigan Health System, Ann Arbor, Michigan.
authors: Steven
authors: S
authors: Telian
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003054:Cochlear Implants; D005260:Female; D005500:Follow-Up Studies; D006319:Hearing Loss, Sensorineural / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D013067:Speech Perception / Q000502:physiology*; D014723:Vestibular Aqueduct / Q000002:abnormalities* / Q000503:physiopathology / Q000601:surgery
keywords: Cochlear implant; congenital anomalies; language development; pediatric