Dataset Information

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

ABSTRACT: 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-? (A?), 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 A?-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 A?-induced neuronal dysfunction quantitatively over time.

SUBMITTER: Omata Y

PROVIDER: S-EPMC4833138 | biostudies-literature | 2016 Mar

REPOSITORIES: biostudies-literature

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

Omata Yasuhiro Y Tharasegaran Suganya S Lim Young-Mi YM Yamasaki Yasutoyo Y Ishigaki Yasuhito Y Tatsuno Takanori T Maruyama Mitsuo M Tsuda Leo L

Aging 20160301 3

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 ...[more]

PMID: 26959388

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Project description:Spatial hearing skills are essential for children as they grow, learn and play. These skills provide critical cues for determining the locations of sources in the environment, and enable segregation of important sounds, such as speech, from background maskers or interferers. Spatial hearing depends on availability of monaural cues and binaural cues. The latter result from integration of inputs arriving at the two ears from sounds that vary in location. The binaural system has exquisite mechanisms for capturing differences between the ears in both time of arrival and intensity. The major cues that are thus referred to as being vital for binaural hearing are: interaural differences in time (ITDs) and interaural differences in levels (ILDs). In children with normal hearing (NH), spatial hearing abilities are fairly well developed by age 4-5 years. In contrast, most children who are deaf and hear through cochlear implants (CIs) do not have an opportunity to experience normal, binaural acoustic hearing early in life. These children may function by having to utilize auditory cues that are degraded with regard to numerous stimulus features. In recent years there has been a notable increase in the number of children receiving bilateral CIs, and evidence suggests that while having two CIs helps them function better than when listening through a single CI, these children generally perform worse than their NH peers. This paper reviews some of the recent work on bilaterally implanted children. The focus is on measures of spatial hearing, including sound localization, release from masking for speech understanding in noise and binaural sensitivity using research processors. Data from behavioral and electrophysiological studies are included, with a focus on the recent work of the authors and their collaborators. The effects of auditory plasticity and deprivation on the emergence of binaural and spatial hearing are discussed along with evidence for reorganized processing from both behavioral and electrophysiological studies. The consequences of both unilateral and bilateral auditory deprivation during development suggest that the relevant set of issues is highly complex with regard to successes and the limitations experienced by children receiving bilateral cochlear implants. This article is part of a Special Issue entitled <Annual Reviews 2016>.

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Dataset Information

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

Publications

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

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