International journal of pediatric otorhinolaryngology
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Int. J. Pediatr. Otorhinolaryngol. · Apr 2017
Case ReportsBilateral cochlear implantation in a child with Johanson Blizzard Syndrome.
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. ⋯ 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.