{"doi":"10.3389/fnmol.2021.670013","title":"Cochlin Deficiency Protects Against Noise-Induced Hearing Loss","abstract":"Cochlin is the most abundant protein in the inner ear. To study its function in response to noise trauma, we exposed adolescent wild-type ( Coch +/+ ) and cochlin knock-out ( Coch –/– ) mice to noise (8–16 kHz, 103 dB SPL, 2 h) that causes a permanent threshold shift and hair cell loss. Two weeks after noise exposure, Coch –/– mice had substantially less elevation in noise-induced auditory thresholds and hair cell loss than Coch + / + mice, consistent with cochlin deficiency providing protection from noise trauma. Comparison of pre-noise exposure thresholds of auditory brain stem responses (ABRs) and distortion product otoacoustic emissions (DPOAEs) in Coch –/– mice and Coch + / + littermates revealed a small and significant elevation in thresholds of Coch –/– mice, overall consistent with a small conductive hearing loss in Coch –/– mice. We show quantitatively that the pro-inflammatory component of cochlin, LCCL, is upregulated after noise exposure in perilymph of wild-type mice compared to unexposed mice, as is the enzyme catalyzing LCCL release, aggrecanase1, encoded by Adamts4 . We further show that upregulation of pro-inflammatory cytokines in perilymph and cochlear soft-tissue after noise exposure is lower in cochlin knock-out than wild-type mice. Taken together, our data demonstrate for the first time that cochlin deficiency results in conductive hearing loss that protects against physiologic and molecular effects of noise trauma.","journal":"Frontiers in Molecular Neuroscience","year":2021,"id":182046,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":15,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9571,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2021-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":481039,"name":"Lukas D. Landegger","orcid":"0000-0002-9660-2625","position":1,"is_corresponding":false},{"id":23873,"name":"Nahid G. Robertson","orcid":null,"position":2,"is_corresponding":false},{"id":317936,"name":"Saša Vasilijić","orcid":"0000-0002-3923-3448","position":3,"is_corresponding":false},{"id":23893,"name":"Cynthia C. Morton","orcid":"0000-0003-2198-6756","position":4,"is_corresponding":false},{"id":317938,"name":"Konstantina M. Stanković","orcid":"0000-0003-0233-279X","position":5,"is_corresponding":false},{"id":368591,"name":"Richard Seist","orcid":"0000-0003-1895-6513","position":0,"is_corresponding":true}],"reference_count":40,"raw_metadata":null,"created_at":"2026-07-18T23:48:09.223560Z","pmid":"34108864","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}