There are two types of sensory receptor cell in the mammalian hearing organ (the cochlea), the so-called inner and outer hair cells. Inner hair cells convey most auditory information to the brain, whereas the role of the outer hair cells may be to amplify the input to the inner hair cells. Our work is designed to describe the operation and the role in hearing of these two cell types. Intracellular recordings are made from these cells in anesthetised guinea pigs. Our techniques allow recordings from both cell types and from different locations along the cochlea. This is important because different sound frequencies are processed at different cochlear locations. There is some evidence that the mode of operation of outer hair observations, however, are based on different techniques and, consequently, are not incontrovertible. We intend to use identical techniques in recording from all cochlear locations and thus to ascertain whether or not there are longitudinal differences in outer hair cell function. Without inner hair cells there is no hearing. Without hearing hair cells, hearing is severely deteriorated. There are over 20 million hearing impaired individuals in the U.S. The majority of them suffer their handicap due to hair cell, particularly outer hair cell, damage. An understanding of the neurobiology of hair cells is a key to prevention and eventual remediation of hearing loss. Our work has been and is designed to provide such fundamental understanding.

Agency
National Institute of Health (NIH)
Institute
National Institute on Deafness and Other Communication Disorders (NIDCD)
Type
Research Project (R01)
Project #
5R01DC000089-25
Application #
2124508
Study Section
Hearing Research Study Section (HAR)
Project Start
1991-01-01
Project End
1996-03-31
Budget Start
1995-01-01
Budget End
1996-03-31
Support Year
25
Fiscal Year
1995
Total Cost
Indirect Cost
Name
Northwestern University at Chicago
Department
Other Health Professions
Type
Schools of Arts and Sciences
DUNS #
City
Evanston
State
IL
Country
United States
Zip Code
60201
Wiwatpanit, Teerawat; Lorenzen, Sarah M; CantĂș, Jorge A et al. (2018) Trans-differentiation of outer hair cells into inner hair cells in the absence of INSM1. Nature 563:691-695
Takahashi, Satoe; Sun, Willy; Zhou, Yingjie et al. (2018) Prestin Contributes to Membrane Compartmentalization and Is Required for Normal Innervation of Outer Hair Cells. Front Cell Neurosci 12:211
Wiwatpanit, Teerawat; Remis, Natalie N; Ahmad, Aisha et al. (2018) Codeficiency of Lysosomal Mucolipins 3 and 1 in Cochlear Hair Cells Diminishes Outer Hair Cell Longevity and Accelerates Age-Related Hearing Loss. J Neurosci 38:3177-3189
Xu, Yingyue; Cheatham, Mary Ann; Siegel, Jonathan H (2017) Identifying the Origin of Effects of Contralateral Noise on Transient Evoked Otoacoustic Emissions in Unanesthetized Mice. J Assoc Res Otolaryngol 18:543-553
Takahashi, Satoe; Cheatham, Mary Ann; Zheng, Jing et al. (2016) The R130S mutation significantly affects the function of prestin, the outer hair cell motor protein. J Mol Med (Berl) 94:1053-62
Cheatham, Mary Ann; Ahmad, Aisha; Zhou, Yingjie et al. (2016) Increased Spontaneous Otoacoustic Emissions in Mice with a Detached Tectorial Membrane. J Assoc Res Otolaryngol 17:81-8
Takahashi, Satoe; Homma, Kazuaki; Zhou, Yingjie et al. (2016) Susceptibility of outer hair cells to cholesterol chelator 2-hydroxypropyl-?-cyclodextrine is prestin-dependent. Sci Rep 6:21973
Cheatham, Mary Ann; Edge, Roxanne M; Homma, Kazuaki et al. (2015) Prestin-Dependence of Outer Hair Cell Survival and Partial Rescue of Outer Hair Cell Loss in PrestinV499G/Y501H Knockin Mice. PLoS One 10:e0145428
Keller, Jacob Pearson; Homma, Kazuaki; Duan, Chongwen et al. (2014) Functional regulation of the SLC26-family protein prestin by calcium/calmodulin. J Neurosci 34:1325-32
Homma, Kazuaki; Duan, Chongwen; Zheng, Jing et al. (2013) The V499G/Y501H mutation impairs fast motor kinetics of prestin and has significance for defining functional independence of individual prestin subunits. J Biol Chem 288:2452-63

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