congenital nerve deafness

Congenital nerve deafness is a hearing impairment present at birth or acquired shortly after. It results from a defect in the auditory nerve within the cochlea or the vestibulocochlear nerve itself. This condition is typically severe and affects both ears, often preventing speech acquisition without specialized training.12

Causes can include maternal infections like rubella during pregnancy, or complications during birth such as anoxia or kernicterus. In some instances, it can be inherited. While medical interventions are generally ineffective for congenital nerve deafness, hearing aids can assist, and cochlear implants offer an option for profound hearing loss. The development of a rubella vaccine has significantly reduced cases in developed countries.2