Download 13th International Conference on Cochlear Implants and Other

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Cochlear implantation in children with CHARGE syndrome
Kobayashi T. , Doi K. , MIyashita M. , Sato M. , Saito K. , Kohama K. , Kinoshita T. , Tobayashi S.
Kinki University Graduate School of Medicine, Otolaryngology & HNS, Osaka-Sayama, Japan
CHARGE syndrome represented a cluster of features including coloboma of the eye , congenital heart defects
(H), atresia or stenosis of the nasal choanae (A), retardation of growth or development and/or central nervous
system anomalies , genital hypoplasia (G), and anomalies of the ear and/or deafness (E). The "E" refers to
hearing loss (HL) and malformations of the ear of these patients and they can present with mixed HL,
sensorineural deafness, cochlear defects, and vestibular problems. When severe sensorineural HL or deafness
is present, cochlear implantation can be a therapeutic option. According to the literatures review, children with
CHARGE syndrome in whom the neurologic deficits are extremely severe might not benefit from cochlear
implantation, and careful therapeutic decision making and parental counseling must take place. In considering
cochlear implantation for patients with CHARGE syndrome, attention must be given to the varied temporal bone
anomalies that may be suggested by pre-operative CT/MRI test and be encountered during the surgery. In our
institute, six children with CHARGE syndrome underwent cochlear implantation. The radiologic and audiologic
records and surgical techniques used should be reviewed.