Novel insights into inner ear development and regeneration for targeted hearing loss therapies. 2020

Marta Roccio, and Pascal Senn, and Stefan Heller
Laboratory of Inner Ear Research, Department of Biomedical Research, University of Bern, Switzerland; Department of Otorhinolaryngology, Head & Neck Surgery, University Hospital Zurich, Switzerland. Electronic address: marta.roccio@usz.ch.

Sensorineural hearing loss is the most common sensory deficit in humans. Despite the global scale of the problem, only limited treatment options are available today. The mammalian inner ear is a highly specialized postmitotic organ, which lacks proliferative or regenerative capacity. Since the discovery of hair cell regeneration in non-mammalian species however, much attention has been placed on identifying possible strategies to reactivate similar responses in humans. The development of successful regenerative approaches for hearing loss strongly depends on a detailed understanding of the mechanisms that control human inner ear cellular specification, differentiation and function, as well as on the development of robust in vitro cellular assays, based on human inner ear cells, to study these processes and optimize therapeutic interventions. We summarize here some aspects of inner ear development and strategies to induce regeneration that have been investigated in rodents. Moreover, we discuss recent findings in human inner ear development and compare the results with findings from animal models. Finally, we provide an overview of strategies for in vitro generation of human sensory cells from pluripotent and somatic progenitors that may provide a platform for drug development and validation of therapeutic strategies in vitro.

UI MeSH Term Description Entries
D007758 Ear, Inner The essential part of the hearing organ consists of two labyrinthine compartments: the bony labyrinthine and the membranous labyrinth. The bony labyrinth is a complex of three interconnecting cavities or spaces (COCHLEA; VESTIBULAR LABYRINTH; and SEMICIRCULAR CANALS) in the TEMPORAL BONE. Within the bony labyrinth lies the membranous labyrinth which is a complex of sacs and tubules (COCHLEAR DUCT; SACCULE AND UTRICLE; and SEMICIRCULAR DUCTS) forming a continuous space enclosed by EPITHELIUM and connective tissue. These spaces are filled with LABYRINTHINE FLUIDS of various compositions. Labyrinth,Bony Labyrinth,Ear, Internal,Inner Ear,Membranous Labyrinth,Bony Labyrinths,Ears, Inner,Ears, Internal,Inner Ears,Internal Ear,Internal Ears,Labyrinth, Bony,Labyrinth, Membranous,Labyrinths,Labyrinths, Bony,Labyrinths, Membranous,Membranous Labyrinths
D012038 Regeneration The physiological renewal, repair, or replacement of tissue. Endogenous Regeneration,Regeneration, Endogenous,Regenerations
D003638 Deafness A general term for the complete loss of the ability to hear from both ears. Deafness Permanent,Hearing Loss Permanent,Prelingual Deafness,Deaf Mutism,Deaf-Mutism,Deafness, Acquired,Hearing Loss, Complete,Hearing Loss, Extreme,Acquired Deafness,Complete Hearing Loss,Deafness, Prelingual,Extreme Hearing Loss,Permanent, Deafness,Permanent, Hearing Loss,Permanents, Deafness
D006198 Hair Cells, Auditory Sensory cells in the organ of Corti, characterized by their apical stereocilia (hair-like projections). The inner and outer hair cells, as defined by their proximity to the core of spongy bone (the modiolus), change morphologically along the COCHLEA. Towards the cochlear apex, the length of hair cell bodies and their apical STEREOCILIA increase, allowing differential responses to various frequencies of sound. Auditory Hair Cells,Cochlear Hair Cells,Auditory Hair Cell,Cell, Cochlear Hair,Cells, Cochlear Hair,Cochlear Hair Cell,Hair Cell, Auditory,Hair Cell, Cochlear,Hair Cells, Cochlear
D000818 Animals Unicellular or multicellular, heterotrophic organisms, that have sensation and the power of voluntary movement. Under the older five kingdom paradigm, Animalia was one of the kingdoms. Under the modern three domain model, Animalia represents one of the many groups in the domain EUKARYOTA. Animal,Metazoa,Animalia
D034381 Hearing Loss A general term for the complete or partial loss of the ability to hear from one or both ears. Deafness, Transitory,Hearing Impairment,Hearing Loss, Transitory,Hypoacusis,Transitory Hearing Loss,Deafnesses, Transitory,Hypoacuses,Loss, Hearing,Loss, Transitory Hearing,Transitory Deafness,Transitory Deafnesses,Transitory Hearing Losses

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