Bilateral cochlear implantation in a child with Johanson Blizzard Syndrome

Int J Pediatr Otorhinolaryngol. 2017 Apr:95:69-71. doi: 10.1016/j.ijporl.2017.02.001. Epub 2017 Feb 6.

Abstract

Sensorineural hearing loss (SNHL) occurs in more than 80% of cases of Johanson Blizzard Syndrome (JBS). However, limited knowledge exists in medical literature of cochlear implantation (CI) outcomes in children with JBS. We report the case of a 5 year-old male with JBS and bilateral CI. While minimal progress in spoken language scores was noted after 4 years of bilateral CI use, substantial improvements in discrimination of speech sounds and audibility of spoken language and environmental sounds were documented. Cochlear implantation is an available treatment option of profound SNHL in children with JBS even if spoken language outcomes are marginal.

Keywords: Cochlear implantation; Johanson Blizzard Syndrome; Sensorineural hearing loss; Speech outcomes.

Publication types

  • Case Reports

MeSH terms

  • Anus, Imperforate / complications
  • Anus, Imperforate / surgery*
  • Child, Preschool
  • Cochlear Implantation / methods*
  • Cochlear Implants
  • Ectodermal Dysplasia / complications
  • Ectodermal Dysplasia / surgery*
  • Growth Disorders / complications
  • Growth Disorders / surgery*
  • Hearing Loss, Sensorineural / complications
  • Hearing Loss, Sensorineural / etiology
  • Hearing Loss, Sensorineural / surgery*
  • Humans
  • Hypothyroidism / complications
  • Hypothyroidism / surgery*
  • Intellectual Disability / complications
  • Intellectual Disability / surgery*
  • Male
  • Nose / abnormalities*
  • Nose / surgery
  • Pancreatic Diseases / complications
  • Pancreatic Diseases / surgery*
  • Speech Perception
  • Treatment Outcome

Supplementary concepts

  • Johanson Blizzard syndrome