Emerging therapies for human hearing loss

Abstract

Introduction: More than 5% of the world's population have a disabling hearing loss which can be managed by hearing aids or implanted electrical devices. However, outcomes are highly variable, and the sound perceived by recipients is far from perfect. Sparked by the discovery of progenitor cells in the cochlea and rapid progress in drug delivery to the cochlea, biological and pharmaceutical therapies are currently in development to improve the function of the cochlear implant or eliminate the need for it altogether.

Areas covered: This review highlights progress in emerging regenerative strategies to restore hearing and adjunct therapies to augment the cochlear implant. Novel approaches include the reprogramming of progenitor cells to restore the sensory hair cell population in the cochlea, gene therapy, and gene editing to treat hereditary and acquired hearing loss. A detailed review of optogenetics is also presented as a technique that could enable optical stimulation of the spiral ganglion neurons, replacing or complementing electrical stimulation.

Expert opinion: Increasing evidence of substantial reversal of hearing loss in animal models, alongside rapid advances in delivery strategies to the cochlea and learnings from clinical trials will amalgamate into a biological or pharmaceutical therapy to replace or complement the cochlear implant.

Keywords: Cell-based therapy; cochlear implant; gene therapy; hair cell; hearing loss; optogenetics; regeneration; spiral ganglion neuron.