title: Indispensable Role of Ion Channels and Transporters in the Auditory System.
abstract: Ear is a complex system where appropriate ionic composition is essential for maintaining the tissue homeostasis and hearing function. Ion transporters and channels present in the auditory system plays a crucial role in maintaining proper ionic composition in the ear. The extracellular fluid, called endolymph, found in the cochlea of the mammalian inner ear is particularly unique due to its electrochemical properties. At an endocochlear potential of about +80 mV, signaling initiated by acoustic stimuli at the level of the hair cells is dependent on the unusually high potassium (K+) concentration of endolymph. There are ion channels and transporters that exists in the ear to ensure that K+is continually being cycled into the stria media endolymph. This review is focused on the discussion of the molecular and genetic basis of previously and newly recognized ion channels and transporters that support sensory hair cell excitation based on recent knock-in and knock-out studies of these channels. This article also addresses the molecular and genetic defects and the pathophysiology behind Meniere's disease as well as how the dysregulation of these ion transporters can result in severe defects in hearing or even deafness. Understanding the role of ion channels and transporters in the auditory system will facilitate in designing effective treatment modalities against ear disorders including Meniere's disease and hearing loss. J. Cell. Physiol. 232: 743-758, 2017. © 2016 Wiley Periodicals, Inc.
authors.affiliation: Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, Florida.
authors.forename: Rahul
authors.initials: R
authors.lastname: Mittal
authors.affiliation: Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, Florida.
authors.forename: Mayank
authors.initials: M
authors.lastname: Aranke
authors.affiliation: Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, Florida.
authors.forename: Luca H
authors.initials: LH
authors.lastname: Debs
authors.affiliation: Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, Florida.
authors.forename: Desiree
authors.initials: D
authors.lastname: Nguyen
authors.affiliation: Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, Florida.
authors.forename: Amit P
authors.initials: AP
authors.lastname: Patel
authors.affiliation: Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, Florida.
authors.forename: M'hamed
authors.initials: M
authors.lastname: Grati
authors.affiliation: Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, Florida.
authors.forename: Jeenu
authors.initials: J
authors.lastname: Mittal
authors.affiliation: Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, Florida.
authors.forename: Denise
authors.initials: D
authors.lastname: Yan
authors.affiliation: Department of Physics, Florida International University, Miami, Florida.|Biomolecular Science Institute, Florida International University, Miami, Florida.
authors.forename: Prem
authors.initials: P
authors.lastname: Chapagain
authors.affiliation: Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, Florida.
authors.forename: Adrien A
authors.initials: AA
authors.lastname: Eshraghi
authors.affiliation: Department of Otolaryngology, University of Miami Miller School of Medicine, Miami, Florida.
authors.forename: Xue Zhong
authors.initials: XZ
authors.lastname: Liu
mesh_terms: D000818:Animals; D001306:Auditory Pathways / Q000378:metabolism*; D006801:Humans; D007473:Ion Channels / Q000378:metabolism*; D026901:Membrane Transport Proteins / Q000378:metabolism*; D008954:Models, Biological; D008958:Models, Molecular; D009154:Mutation / Q000235:genetics
chemical_list: D007473:Ion Channels; D026901:Membrane Transport Proteins