title: Transient auditory nerve demyelination as a new mechanism for hidden hearing loss.
abstract: Hidden hearing loss (HHL) is a recently described auditory neuropathy believed to contribute to speech discrimination and intelligibility deficits in people with normal audiological tests. Animals and humans with HHL have normal auditory thresholds but defective cochlear neurotransmission, that is, reduced suprathreshold amplitude of the sound-evoked auditory nerve compound action potential. Currently, the only cellular mechanism known for HHL is loss of inner hair cell synapses (synaptopathy). Here we report that transient loss of cochlear Schwann cells results in permanent auditory deficits characteristic of HHL. This auditory neuropathy is not associated with synaptic loss, but rather with disruption of the first heminodes at the auditory nerve peripheral terminal. Thus, this study identifies a new mechanism for HHL, highlights the long-term consequences of transient Schwann cell loss on hearing and might provide insights into the causes of the auditory deficits reported in patients that recover from acute demyelinating diseases such as Guillain-Barré syndrome.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Kresge Hearing Research Institute, University of Michigan, 1150 West Medical Center Drive, Ann Arbor, Michigan 48109, USA.|MOE Key Laboratory of Model Animals for Disease Study, Model Animal Research Center of Nanjing University, Nanjing, Jiangsu Province, China.
authors.forename: Guoqiang
authors.initials: G
authors.lastname: Wan
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Kresge Hearing Research Institute, University of Michigan, 1150 West Medical Center Drive, Ann Arbor, Michigan 48109, USA.
authors.forename: Gabriel
authors.initials: G
authors.lastname: Corfas
mesh_terms: D000200:Action Potentials; D000818:Animals; D001369:Axons / Q000473:pathology; D003051:Cochlea / Q000294:innervation; D003056:Cochlear Nerve / Q000473:pathology* / Q000503:physiopathology; D003711:Demyelinating Diseases / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D016057:Evoked Potentials, Auditory, Brain Stem; D034381:Hearing Loss / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D008810:Mice, Inbred C57BL; D018991:Myelin Proteolipid Protein / Q000378:metabolism; D009622:Noise; D011901:Ranvier's Nodes / Q000473:pathology; D012038:Regeneration; D012583:Schwann Cells / Q000378:metabolism / Q000473:pathology; D013569:Synapses / Q000473:pathology
chemical_list: D018991:Myelin Proteolipid Protein; C047516:Plp1 protein, mouse