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. 2021 Mar;1(3):e76.
doi: 10.1002/cpz1.76.

Congenital Deafness and Recent Advances Towards Restoring Hearing Loss

Justine M Renauld  1 , Martin L Basch  1   2   3   4
Affiliations

Congenital Deafness and Recent Advances Towards Restoring Hearing Loss

Justine M Renauld et al. Curr Protoc. 2021 Mar.

Abstract

Congenital hearing loss is the most common birth defect, estimated to affect 2-3 in every 1000 births. Currently there is no cure for hearing loss. Treatment options are limited to hearing aids for mild and moderate cases, and cochlear implants for severe and profound hearing loss. Here we provide a literature overview of the environmental and genetic causes of congenital hearing loss, common animal models and methods used for hearing research, as well as recent advances towards developing therapies to treat congenital deafness. © 2021 The Authors.

Keywords: animal models; congenital deafness; gene therapy; hearing loss.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Anatomy of the ear. (A) The outer ear transmits sound vibrations to the middle ear through the external auditory canal. The bones in the middle ear transmit the pressure of the sound vibrations into the perilymph‐filled scala vestibuli of the inner ear through the oval window. (B) The inner ear houses the sensory organs of the vestibular system responsible for detecting motion and acceleration, and the cochlea, which houses the auditory organ of Corti. (C) Section through the turns of the cochlea. (D) Magnification of one turn of the cochlea showing supporting cells, inner and outer hair cells of the organ of Corti, synapses of inner hair cells with the neurons of the VIIIth nerve (cochleovestibular ganglion), and marginal, intermediate, and basal cells of the Stria Vascularis.
See this image and copyright information in PMC

References

LITERATURE CITED

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INTERNET RESOURCES
    1. https://www.asha.org/public/hearing/Degree‐of‐Hearing‐Loss/
    1. American Speech‐Language‐Hearing Association (ASHA) . https://hereditaryhearingloss.org

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