Congenital Profound Hearing Loss: Management of Hypoplastic and Aplastic Vestibulocochlear Nerves

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Cochlear implantation has become an established intervention for selected children with severe to profound deafness. As implant programmes have grown, an increasing number of children with congenital deafness have been identified with aplastic or hypoplastic cochlear nerves (Figure 1). These children pose a significant management challenge. Options for management include a cochlear implant, auditory brainstem implant, or no surgical intervention (Table 1).