More About Cochlear Implantation in Children with Charge Association

Abstract

CHARGE is the acronym for Coloboma, Heart malformation, Choanal Atresia, Retardation of growth and/or development, Genital and Ear anomalies. It is a rare genetic syndrome, usually with no family history. From an otological point of view, it is characterized by semicircular canal agenesia, Mondini-type cochlear hypoplasia, frequent vestibular dysfunction, and hearing impairment (ranging from transient OME to profound deafness) (Abadie et al., 2000).Speech and language delay in this syndrome is due not only to hearing problems, but also to mental retardation, facial palsy, oro-lingual motor deficit, and swallowing disorders, which often require enteral feeding or gastrostomy. Moreover, anatomical cochlear or facial nerve anomalies have been described.Due to these aggravating factors, few children affected by CHARGE have undergone cochlear implantation. However, several cochlear implantation centres, including ours, consider that children affected by this syndrome and profound hearing impairment might obtain benefit from a cochlear implant (CI), at least to improve communication, although not necessarily to develop spoken language (Bauer et al., 2002; McArdle et al., 2002; Lanson et al., 2007). This point of view was supported by a previous work about four children with CHARGE and sensorineural hearing loss: communication mode and language reception and expression progressed in a similar way in the two implanted profoundly deaf children as in the two of them with severe hearing impairment and hearing aids (Lina-Granade et al., 2006). Hence, we undertook this case study with more children and a longer follow-up, in order to have a more accurate description of the surgical particularities, fitting, and outcomes of implantation in children with CHARGE.