Neurotrophin therapy and cochlear implantation: translating animal models to human therapy

Cochlear implantation is a highly successful intervention for the treatment of deafness that depends on electrical stimulation of the inner ear's surviving spiral ganglion neurons. It is thought that some of the variability in hearing outcomes that is seen in patients receiving implants may be a reflection of the number or health of surviving neurons. A variety of studies have demonstrated a relationship between hair cell loss and degeneration of the spiral ganglion. This has been attributed to the loss of neurotrophin production with destruction of the spiral ganglion's target, the hair cell. Delivery of neurotrophins either through a device or through gene therapy has been shown to improve spiral ganglion survival after hair cell loss and additionally improves the function of cochlear implants in animal models. Translation of these observations to human therapy will require a clear understanding of the relationship between human spiral ganglion health and cochlear implant outcomes as well as the development of novel pre- and post-implantation outcomes measures.