Research Paper Volume 8, Issue 3 pp 427—439

Expression of amyloid-β in mouse cochlear hair cells causes an early-onset auditory defect in high-frequency sound perception

Yasuhiro Omata1,4, , Suganya Tharasegaran1, , Young-Mi Lim1, , Yasutoyo Yamasaki1, , Yasuhito Ishigaki2, , Takanori Tatsuno2, , Mitsuo Maruyama3, , Leo Tsuda1, ,

  • 1 Center for Development of Advanced Medicine for Dementia (CAMD), National Center for Geriatrics and Gerontology (NCGG), Obu, Aichi, Japan
  • 2 Division of Molecular and Cellular Biology, Kanazawa Medical University, Kanazawa, Ishikawa, Japan
  • 3 Department of Molecular Aging, National Center for Geriatrics and Gerontology, Obu, Aichi, Japan
  • 4 Presend address: Department of Occupational and Environmental Health, Graduate School of Medicine, Nagoya University, Nagoya, Aichi, Japan

Received: November 18, 2015       Accepted: January 27, 2016       Published: March 7, 2016
How to Cite

Copyright: © 2016 Omata et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.


Increasing evidence indicates that defects in the sensory system are highly correlated with age-related neurodegenerative diseases, including Alzheimer's disease (AD). This raises the possibility that sensory cells possess some commonalities with neurons and may provide a tool for studying AD. The sensory system, especially the auditory system, has the advantage that depression in function over time can easily be measured with electrophysiological methods. To establish a new mouse AD model that takes advantage of this benefit, we produced transgenic mice expressing amyloid-β (Aβ), a causative element for AD, in their auditory hair cells. Electrophysiological assessment indicated that these mice had hearing impairment, specifically in high-frequency sound perception (>32 kHz), at 4 months after birth. Furthermore, loss of hair cells in the basal region of the cochlea, which is known to be associated with age-related hearing loss, appeared to be involved in this hearing defect. Interestingly, overexpression of human microtubule-associated protein tau, another factor in AD development, synergistically enhanced the Aβ-induced hearing defects. These results suggest that our new system reflects some, if not all, aspects of AD progression and, therefore, could complement the traditional AD mouse model to monitor Aβ-induced neuronal dysfunction quantitatively over time.


ABR: auditory brainstem response; AD: Alzheimer's disease; Aβ: amyloid-β; APP: amyloid precursor protein; ARHL: age-related hearing loss.