Are cochlear implants useful for children with auditory nerve aplasia?

A common cause of congenital hearing loss is malformation of the ears and possibly also of the downstream neural structures such as the auditory nerves, brain stem and their synapses. There are children with severe hearing loss in whom no auditory nerves can be detected radiologically, although nerve fibers may still be present. For these children, acoustic hearing systems are not sufficient to understand language and learn to speak. This study investigated whether cochlear implants can still enable spoken language in these cases, even if no auditory nerve is visible in the MRI.