Sensorineural hearing loss in children

Sensorineural hearing loss in children, either congenital or acquired, has an incidence of 2-4 per million. Molecular diagnosis of early childhood deafness became available for some types of syndromal and non-syndromal forms and will offer different treatment modalities in the future. Severe to profound sensorineural hearing loss can be effectively treated with cochlear implants. There is evidence of cerebral auditory plasticity under electrical stimulation of the auditory nerve with better performance in early implanted children. Other predicting factors are related to the type of schooling, family support and residual hearing. In the long-term, prelingually deafened children will develop considerable speech perception and production. Children with marginal benefit from hearing aid amplification show significant improvements in speech perception following implantation. Implantation is also possible in cases of cochlear malformation. However, special attention has to be given to the facial nerve, a possible CSF leak and electrode misplacement. Apart from hearing improvement cochlear implants have a positive impact on the family situation, schooling and personal well-being.