Bilateral Cochlear Implantation in a Child with Johanson Blizzard Syndrome

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. Whi...

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Bibliographic Details
Published in:International journal of pediatric otorhinolaryngology 2017-04, Vol.95, p.69-71
Main Authors: Holcomb, Meredith A, Rizk, Habib G, Morris, Nevitte S, Meyer, Ted A
Format: Article
Language:English
Subjects:
Anus, Imperforate - complications
Anus, Imperforate - surgery
Child, Preschool
Cochlear implantation
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
Johanson Blizzard Syndrome
Male
Nose - abnormalities
Nose - surgery
Otolaryngology
Pancreatic Diseases - complications
Pancreatic Diseases - surgery
Pediatrics
Sensorineural hearing loss
Speech outcomes
Speech Perception
Treatment Outcome
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Description
Summary: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.
ISSN:0165-5876
1872-8464
DOI:10.1016/j.ijporl.2017.02.001