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Cochlear implants (CIs) are the treatment of choice for hearing rehabilitation in children with congenital or acquired profound hearing loss or deafness in order to ensure appropriate speech development and avoid social deprivation. However, in the case of a radiologically detectable malformation of the inner ear structures and potentially associated hypo- or aplasia of the vestibulocochlear nerve, application of a CI is either not possible, or the functional outcome may be of limited predictability. In addition, the risk of surgical complications is also increased in these patients. Counseling parents and developing an appropriate individual therapeutic decision can therefore be a major challenge for the medical team. The current paper is intended to provide support in this regard. It presents criteria for various inner ear malformations and discusses possible treatment options., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)