Bilateral cochlear implantation in a child with Johanson Blizzard Syndrome. (April 2017)

• Record Type: Journal Article
• Authors: Holcomb, Meredith A.; Rizk, Habib G.; Morris, Nevitte S.; Meyer, Ted A.
• Is Part Of: International journal of pediatric otorhinolaryngology. Volume 95(2017:Apr.)
• Page Start: 69
• Page End: 71
• Publication Date: 2017-04
• Subjects: Johanson Blizzard Syndrome -- Cochlear implantation -- Speech outcomes -- Sensorineural hearing loss; Otolaryngology -- Periodicals; Pediatrics -- Periodicals; Otolaryngology -- Periodicals; Pediatrics -- Periodicals; Oto-rhino-laryngologie -- Périodiques; Pédiatrie -- Périodiques; 618.9209751
• DOI: 10.1016/j.ijporl.2017.02.001
• Languages: English
• ISSNs: 0165-5876

Abstract

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.