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Cochlear implantation in Children with CHARGE syndrome: therapeutic decisions and outcomes.
Laryngoscope. 2007 Jul; 117(7):1260-6.L

Abstract

OBJECTIVES

Ear anomalies and deafness are associated with CHARGE syndrome, which also presents with a cluster of features including coloboma of the eye, heart defects, atresia of the choanae, developmental retardation, and genitourinary abnormalities. The aim of this study is to explore the viability of cochlear implantation in children with CHARGE syndrome and to assess the outcome.

STUDY DESIGN

Retrospective chart review.

METHODS

Eleven children presenting with severe to profound sensorineural hearing loss associated with CHARGE syndrome were the subjects of this study. Routine audiometric measurements and the Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS) were performed pre- and postoperatively. In addition, the degree of the subjects' cochlear deformity were measured and correlated to outcome.

RESULTS

All patients had varying degrees of ear anomalies, seven patients suffered from coloboma of the eyes, two had heart defects, five exhibited choanal atresia, eleven showed developmental retardation, and six had genitourinary abnormalities. Ten of the children underwent cochlear implantation with complete insertion of the electrode array without complication and were followed over a 3-month to a 7-year period. The eleventh child was not implanted because of severe retardation. All of the implanted children showed varying, but limited degrees, of auditory benefit as measured by routine audiometry and the IT-MAIS.

CONCLUSIONS

Careful treatment planning for children with sensorineural hearing loss and CHARGE syndrome can lead to varying, but limited degrees, of auditory benefit with no increase in surgical complications. Although the implant enhanced the children's 'connectivity' to the environment, it did not promote the development of oral language skills in this population.

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Authors+Show Affiliations

Lanson BG
Department of Otolaryngology, NYU School of Medicine, New York, NY 10016, USA.
Green JE
No affiliation info available
Roland JT
No affiliation info available
Lalwani AK
No affiliation info available
Waltzman SB
No affiliation info available

MeSH

Abnormalities, MultipleChild, PreschoolChoanal AtresiaCochlear ImplantationColobomaDevelopmental DisabilitiesEarFemaleGenitaliaHearing Loss, SensorineuralHeart Defects, CongenitalHumansInfantMalePostoperative ComplicationsRetrospective StudiesSyndromeTreatment Outcome

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

17507827

Citation

Lanson, Biana G., et al. "Cochlear Implantation in Children With CHARGE Syndrome: Therapeutic Decisions and Outcomes." The Laryngoscope, vol. 117, no. 7, 2007, pp. 1260-6.
Lanson BG, Green JE, Roland JT, et al. Cochlear implantation in Children with CHARGE syndrome: therapeutic decisions and outcomes. Laryngoscope. 2007;117(7):1260-6.
Lanson, B. G., Green, J. E., Roland, J. T., Lalwani, A. K., & Waltzman, S. B. (2007). Cochlear implantation in Children with CHARGE syndrome: therapeutic decisions and outcomes. The Laryngoscope, 117(7), 1260-6.
Lanson BG, et al. Cochlear Implantation in Children With CHARGE Syndrome: Therapeutic Decisions and Outcomes. Laryngoscope. 2007;117(7):1260-6. PubMed PMID: 17507827.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cochlear implantation in Children with CHARGE syndrome: therapeutic decisions and outcomes. AU - Lanson,Biana G, AU - Green,Janet E, AU - Roland,J Thomas,Jr AU - Lalwani,Anil K, AU - Waltzman,Susan B, PY - 2007/5/18/pubmed PY - 2007/8/3/medline PY - 2007/5/18/entrez SP - 1260 EP - 6 JF - The Laryngoscope JO - Laryngoscope VL - 117 IS - 7 N2 - OBJECTIVES: Ear anomalies and deafness are associated with CHARGE syndrome, which also presents with a cluster of features including coloboma of the eye, heart defects, atresia of the choanae, developmental retardation, and genitourinary abnormalities. The aim of this study is to explore the viability of cochlear implantation in children with CHARGE syndrome and to assess the outcome. STUDY DESIGN: Retrospective chart review. METHODS: Eleven children presenting with severe to profound sensorineural hearing loss associated with CHARGE syndrome were the subjects of this study. Routine audiometric measurements and the Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS) were performed pre- and postoperatively. In addition, the degree of the subjects' cochlear deformity were measured and correlated to outcome. RESULTS: All patients had varying degrees of ear anomalies, seven patients suffered from coloboma of the eyes, two had heart defects, five exhibited choanal atresia, eleven showed developmental retardation, and six had genitourinary abnormalities. Ten of the children underwent cochlear implantation with complete insertion of the electrode array without complication and were followed over a 3-month to a 7-year period. The eleventh child was not implanted because of severe retardation. All of the implanted children showed varying, but limited degrees, of auditory benefit as measured by routine audiometry and the IT-MAIS. CONCLUSIONS: Careful treatment planning for children with sensorineural hearing loss and CHARGE syndrome can lead to varying, but limited degrees, of auditory benefit with no increase in surgical complications. Although the implant enhanced the children's 'connectivity' to the environment, it did not promote the development of oral language skills in this population. SN - 0023-852X UR - https://www.unboundmedicine.com/medline/citation/17507827/Cochlear_implantation_in_Children_with_CHARGE_syndrome:_therapeutic_decisions_and_outcomes_ L2 - https://doi.org/10.1097/MLG.0b013e31806009c9 DB - PRIME DP - Unbound Medicine ER -