Cochlear implantation in children with labyrinthine anomalies and cochlear nerve deficiency: Implications for auditory brainstem implantation

Objectives/Hypothesis: Compare outcomes among children with inner ear malformations and/or cochlear nerve deficiency (CND) who have received a cochlear implant (CI). Study Design: Individual retrospective cohort study from 1993 to 2010. Methods: A select cohort of 76 children was identified. Imaging characteristics, operative findings, complications, mapping parameters, and performance were assessed. Comparisons among the different groups were undertaken. Results: Surgery was mostly uncomplicated. Nearly all children demonstrated behavioral responses to CI stimulation irrespective of inner ear morphology or the presence of CND. Children with CND had higher pure tone averages (PTAs) and required greater charge for stimulation than other malformation types. Open-set speech perception was achieved in 100% of children with incomplete partition-enlarged vestibular aqueduct (IP-EVA), 50% of those with hypoplastic malformations, and 19% of CND cases. Robust responses on eighth nerve compound action potential (ECAP) testing through the implant was associated with higher levels of speech perception. Manually supplemented communication strategies were more common among children with hypoplastic malformations (69%) and CND (95%) than those with IP-EVA (18%). Conclusions: Children with IP-EVA malformations have an excellent prognosis for developing open-set speech perception and using oral communication modes following CI. On the contrary, children with severe malformations or CND may have elevated charge requirements for attaining sound detection alone. These children's prognosis for obtaining open-set speech understanding, using exclusive oral communication, and participating in mainstream education is more limited. These findings have important implications for considering alternative forms of intervention such as auditory brainstem implantation and/or supplementation with visually based communication strategies.