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Hearing in the mouse of Usher

Nature Biotechnology volume 35, pages 216–218 (2017)Cite this article

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Hearing and balance are rescued by a synthetic virus that delivers a transgene to the inner ear of neonatal mice.

Global health data reveal this sobering reality: either you have hearing loss, or chances are you care about someone who does. An estimated 32 million children and 328 million adults worldwide are affected by hearing loss that profoundly interferes with learning, interpersonal communication, and the ability to work productively1. Around 100 genes that cause non-syndromic hearing loss are known2, but efforts to develop gene-based therapies have struggled owing to a lack of suitable delivery vectors. In this issue, two companion studies report important progress in research on gene therapy for the inner ear. Landegger et al.3 first characterize a synthetic adeno-associated viral vector (AAV) that highly efficiently transduces auditory and vestibular sensory hair cells in the mouse inner ear in vitro and in vivo, and the human vestibular sensory epithelium in vitro. Pan et al.4 then apply this vector to rescue hearing and balance in a mouse model of a human deaf-blindness disease called Usher syndrome type 1c.

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Figure 1: Structure, function, and viral transduction of the inner ear.

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Acknowledgements

Karen Thiebes, Ph.D., created the artwork for Figure 1. Work in the author's laboratory is funded by the Hearing Health Foundation's Hearing Restoration Project and by the National Institute on Deafness and Other Communication Disorders (NIDCD).

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  1. Department of Otolaryngology, John V. Brigande is at the Oregon Hearing Research Center, Oregon Health & Science University, Portland, Oregon, USA.

    John V Brigande

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  1. John V Brigande
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Correspondence to John V Brigande.

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Brigande, J. Hearing in the mouse of Usher. Nat Biotechnol 35, 216–218 (2017). https://doi.org/10.1038/nbt.3815

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