Int Arch Otorhinolaryngol 2013; 17(04): 424-428
DOI: 10.1055/s-0033-1352500
Case Report
Thieme Publicações Ltda Rio de Janeiro, Brazil

Cochlear Implants in Children Diagnosed with CHARGE Syndrome

Carolina Costa Cardoso
1  Audiologist; Speech Therapy, University Center Federal District (UNIPLAN), Brasília/DF, Brazil
,
Michelle Sales de Meneses
1  Audiologist; Speech Therapy, University Center Federal District (UNIPLAN), Brasília/DF, Brazil
,
Isabella Monteiro de Castro Silva
2  Audiologist; Speech Therapy, University of Brasília (UnB), Brasília/DF, Brazil
,
Angela Maria Vaccaro Silva Alves
3  Audiologist; Speech Therapy, Pontifical Catholic University (PUCSP), São Paulo/SP, Brazil
› Author Affiliations
Further Information

Publication History

23 November 2011

18 March 2012

Publication Date:
13 September 2013 (online)

  

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.