• Int. J. Pediatr. Otorhinolaryngol. · Apr 2017

    Case Reports

    Bilateral cochlear implantation in a child with Johanson Blizzard Syndrome.

    • Meredith A Holcomb, Habib G Rizk, Nevitte S Morris, and Ted A Meyer.
    • Department of Otolaryngology-Head and Neck Surgery, Medical University of South Carolina, United States. Electronic address: holcombm@musc.edu.
    • Int. J. Pediatr. Otorhinolaryngol. 2017 Apr 1; 95: 69-71.

    AbstractSensorineural 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.Copyright © 2017 Elsevier B.V. All rights reserved.

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