The first eletrocnic Journal of Otolaryngology in the world
ISSN: 1809-9777

E-ISSN: 1809-4864

 
1831 

Year: 2013  Vol. 17   Num. 4  - Oct/Dec - (18º)
DOI: 10.1055/s-0033-1352500
Section: Case Report
 
Cochlear Implants in Children Diagnosed with CHARGE Syndrome
Author(s):
Carolina Costa Cardoso, Michelle Sales de Meneses, Isabella Monteiro de Castro Silva, Angela Maria Vaccaro Silva Alves
Key words:
charge syndrome - cochlear implants - hearing - language
Abstract:

Introduction: The CHARGE association (coloboma of the eyes; heart disease; atresia of the choanae; retarded growth and development; genital hypoplasia/genitourinary anomalies; ear anomalies and/or hearing loss) was first described in 1979 by Hall, and among its main features is hearing loss. This study presents a case aiming to establish relationships between performance on Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS) and Meaningful Use of Speech Scales (MUSS) tests and the analysis of hearing and language categories of a patient diagnosed with CHARGE syndrome, before and after cochlear implant (CI) surgery.

Case Report: A 7-year-old girl was diagnosed with CHARGE. She had severe sensorineural hearing loss and was a prelingual unilateral CI user. We analyzed data from the patient's medical records regarding therapies and video recordings.

Results: The patient showed positive results in all evaluations after CI. IT-MAIS rose from 5 to 90% following the use of CI. MUSS also rose, from 75 to 72.5%, after use of CI. Classification of Auditory Skills changed from category 1 before use of CI to category 6 after use of CI. Classification of Language Skills changed from category 1 before use of CI to category 3 after use of CI. The CI is an aid but there are many factors in the therapeutic process, and great heterogeneity in individuals diagnosed with CHARGE should be investigated.

Conclusion: The development of listening and language skills after CI use was demonstrated by IT-MAIS and MUSS tests, and categorization of speech and hearing in this child with a diagnosis of CHARGE syndrome shows that CI can be an effective technological resource to provide information on hearing as one source for language construction.

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