Inner ear malformations and neurological involvement: a review

Inner ear malformations are associated with different degrees of hearing loss. Some of these anomalies require cochlear implantation because of the occurrence of profound hearing loss. The radiological imaging necessary for the evaluation of the congenital hearing loss might also reveal the concomitant presence of important collateral findings such as the central nervous system and cranial nerves malformations. These neurological features might be extremely important in the surgical planning of the cochlear implantation and also have a role in the hearing and speech development of the affected children. In addition, anomalies affecting specific structures of the inner ear should be considered predisposing factors for otogenic meningitis and they should be identified as early as possible. The present review aims to summarize the available literature about this specific topic crucial for the patients’ neuropsychological and auditory development.