Bilateral cochlear implantation in a child with Johanson Blizzard Syndrome

Meredith A. Holcomb, Habib G. Rizk, Nevitte S. Morris, Ted A. Meyer

Research output: Contribution to journalArticlepeer-review

1 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)69-71
Number of pages3
JournalInternational Journal of Pediatric Otorhinolaryngology
StatePublished - Apr 1 2017
Externally publishedYes


  • Cochlear implantation
  • Johanson Blizzard Syndrome
  • Sensorineural hearing loss
  • Speech outcomes

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Otorhinolaryngology


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