The hearing loss may be a sign of a rare disease. Babies with Mondini dysplasia, for example, are born with one and a half coils in the cochlea instead of the standard two, in either one or both ears. Most children with this condition have profound hearing loss. They may need a surgical repair, as well as a cochlear implant, but some can benefit from hearing aids. Babies with KID syndrome , Donnai-Barrow syndrome , and Wildervanck syndrome —among other rare diseases—may have hearing loss.