Abstract
Sensorineural hearing impairment is the most frequent form of hearing impairment affecting 1–2 in 1000 newborns and another 1 in 1000 adolescents. More than 50% of congenital hearing impairment is of genetic origin and some forms of monogenic deafness are likely targets for future gene therapy. Good progress has been made in clinical phenotyping, genetic diagnostics, and counselling. Disease modelling, e.g. in transgenic mice, has helped elucidate disease mechanisms underlying genetic hearing impairment and informed clinical phenotyping in recent years. Clinical management of paediatric hearing impairment involves hearing aids, cochlear or brainstem implants, signal-to-noise improvement in educational settings, speech therapy, and sign language. Cochlear implants, for example, have much improved the situation of profoundly hearing impaired and deaf children. Nonetheless there remains a major unmet clinical need for improving hearing restoration. Preclinical studies promise that we will witness clinical trials on gene therapy and a next generation of cochlear implants during the coming decade. Moreover, progress in generating sensory hair cells and neurons from stem cells spurs disease modelling, drug screening, and regenerative approaches. This review briefly summarizes the pathophysiology of paediatric hearing impairment and provides an update on the current preclinical development of innovative approaches toward improved hearing restoration.
Highlights
Appropriate sensory input and normal sensory processing are required for full establishment of neural function
Future gene therapy approaches ideally will build upon late preclinical work in non-human primate models of human monogenic hearing impairment (HI)
Safe and efficient transduction of hair cells is another important development for future gene therapy
Summary
Appropriate sensory input and normal sensory processing are required for full establishment of neural function. Efforts are being undertaken to bring electrodes and SGNs closer together for reducing the current thresholds and potentially increasing the number of separate channels, e.g. by penetrating the auditory nerve with a multielectrode array for direct contact of electrodes and nerve [16,17] Another approach is to attract SGN neurites to nearby electrode-arrays using neurotrophic factors Major efforts to improve hearing rehabilitation of children with hearing aids and cochlear implants are well justified by the fact that we do not yet have other efficient means of restoring hearing and these devices represent a “one suits all” solution, as long as there is a functional auditory nerve. Cerebrospinal fluid space into the cochlea via an enlarged vestibular aqueduct that is typically found in Pendred syndrome
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