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Frequency and clinical features of hearing loss caused by STRC deletions \uff08STRC\u907a\u4f1d\u5b50\u6b20\u5931\u306b\u3088\u308b\u96e3\u8074\u306e\u983b\u5ea6\u3068\u81e8\u5e8a\u50cf\u306e\u691c\u8a0e\uff09. \u4fe1\u5dde\u5927\u5b66, 2019, \u535a\u58eb\u8ad6\u6587.", "subitem_description_type": "Other"}]}, "item_14_dissertation_number_7": {"attribute_name": "\u5b66\u4f4d\u6388\u4e0e\u756a\u53f7", "attribute_value_mlt": [{"subitem_dissertationnumber": "13601\u7532\u7b2c1181\u53f7"}]}, "item_14_link_74": {"attribute_name": "WoS", "attribute_value_mlt": [{"subitem_link_text": "Web of Science", "subitem_link_url": "http://gateway.isiknowledge.com/gateway/Gateway.cgi?\u0026GWVersion=2\u0026SrcAuth=ShinshuUniv\u0026SrcApp=ShinshuUniv\u0026DestLinkType=FullRecord\u0026DestApp=WOS\u0026KeyUT=000461019400004"}]}, "item_14_publisher_4": {"attribute_name": "\u51fa\u7248\u8005", "attribute_value_mlt": [{"subitem_publisher": "\u4fe1\u5dde\u5927\u5b66"}]}, "item_14_relation_54": {"attribute_name": "PubMed", "attribute_value_mlt": [{"subitem_relation_name": [{"subitem_relation_name_text": "30867468"}], "subitem_relation_type_id": {"subitem_relation_type_id_text": "http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?CMD=search\u0026DB=pubmed\u0026term=30867468", "subitem_relation_type_select": "PMID"}}]}, "item_14_relation_55": {"attribute_name": "DOI", "attribute_value_mlt": [{"subitem_relation_name": [{"subitem_relation_name_text": "10.1038/s41598-019-40586-7"}], "subitem_relation_type_id": {"subitem_relation_type_id_text": "https://doi.org/10.1038/s41598-019-40586-7", "subitem_relation_type_select": "DOI"}}]}, "item_14_rights_69": {"attribute_name": "\u6a29\u5229", "attribute_value_mlt": [{"subitem_rights": "\u00a9 The Author(s) 2019 / This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article\u2019s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article\u2019s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/."}]}, "item_14_select_71": {"attribute_name": "\u8457\u8005\u7248\u30d5\u30e9\u30b0", "attribute_value_mlt": [{"subitem_select_item": "ETD"}]}, "item_14_text_11": {"attribute_name": "\u5b66\u4f4d\u306e\u533a\u5206", "attribute_value_mlt": [{"subitem_text_value": "Doctoral"}]}, "item_14_text_12": {"attribute_name": "\u5b66\u4f4d\u306e\u5206\u91ce", "attribute_value_mlt": [{"subitem_text_value": "\u533b\u5b66"}]}, "item_14_text_13": {"attribute_name": "\u5b66\u4f4d\u306e\u5831\u544a\u756a\u53f7", "attribute_value_mlt": [{"subitem_text_value": "\u7532\u7b2c1181\u53f7"}]}, "item_14_text_77": {"attribute_name": "wosonly keywords", "attribute_value_mlt": [{"subitem_text_value": "GENE; DEAFNESS; MUTATIONS"}]}, "item_14_textarea_75": {"attribute_name": "wosonly abstract", "attribute_value_mlt": [{"subitem_textarea_value": "Sensorineural hearing loss is a common deficit and mainly occurs due to genetic factors. Recently, copy number variants (CNVs) in the STRC gene have also been recognized as a major cause of genetic hearing loss. We investigated the frequency of STRC deletions in the Japanese population and the characteristics of associated hearing loss. For CNV analysis, we employed a specialized method of Ion AmpliSeg (TM) sequencing, and confirmed the CNV results via custom array comparative genomic hybridization. We identified 17 probands with STRC homozygous deletions. The prevalence of STRC homozygous deletions was 1.7% in the hearing loss population overall, and 4.3% among mild-to-moderate hearing loss patients. A 2.63% carrier deletion rate was identified in both the hearing loss and the control population with normal hearing. In conclusion, our results show that STRC deletions are the second most common cause of mild-to-moderate hearing loss after the GJB2 gene, which accounts for the majority of genetic hearing loss. The phenotype of hearing loss is congenital and appears to be moderate, and is most likely to be stable without deterioration even after the age of 50. The present study highlights the importance of the STRC gene as a major cause of mild-to-moderate hearing loss."}]}, "item_creator": {"attribute_name": "\u8457\u8005", "attribute_type": "creator", "attribute_value_mlt": [{"creatorNames": [{"creatorName": "\u6a2a\u7530, \u967d"}], "nameIdentifiers": [{"nameIdentifier": "109518", "nameIdentifierScheme": "WEKO"}]}]}, "item_files": {"attribute_name": "\u30d5\u30a1\u30a4\u30eb\u60c5\u5831", "attribute_type": "file", "attribute_value_mlt": [{"accessrole": "open_date", "date": [{"dateType": "Available", "dateValue": "2020-06-12"}], "displaytype": "detail", "download_preview_message": "", "file_order": 0, "filename": "15MH041G_yoshi.pdf", "filesize": [{"value": "149.1 kB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensetype": "license_free", "mimetype": "application/pdf", "size": 149100.0, "url": {"label": "\u5185\u5bb9\u306e\u8981\u65e8", "url": "https://soar-ir.repo.nii.ac.jp/record/21391/files/15MH041G_yoshi.pdf"}, "version_id": "8a0482a3-cec1-46fe-be40-568d64b217ea"}, {"accessrole": "open_date", "date": [{"dateType": "Available", "dateValue": "2020-06-12"}], "displaytype": "detail", "download_preview_message": "", "file_order": 1, "filename": "15MH041G_shinsa.pdf", "filesize": [{"value": "164.7 kB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensetype": "license_free", "mimetype": "application/pdf", "size": 164700.0, "url": {"label": "\u5be9\u67fb\u7d50\u679c\u306e\u8981\u65e8", "url": "https://soar-ir.repo.nii.ac.jp/record/21391/files/15MH041G_shinsa.pdf"}, "version_id": "bca599c0-7ca4-46e8-9bc2-6bef712e096c"}, {"accessrole": "open_date", "date": [{"dateType": "Available", "dateValue": "2020-06-12"}], "displaytype": "detail", "download_preview_message": "", "file_order": 2, "filename": "15MH041G_ronbun.pdf", "filesize": [{"value": "1.0 MB"}], "format": "application/pdf", "future_date_message": "", "is_thumbnail": false, "licensefree": "\u00a9 The Author(s) 2019 / This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article\u2019s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article\u2019s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.", "licensetype": "license_free", "mimetype": "application/pdf", "size": 1000000.0, "url": {"label": "\u535a\u58eb\u8ad6\u6587\u306e\u5168\u6587", "url": "https://soar-ir.repo.nii.ac.jp/record/21391/files/15MH041G_ronbun.pdf"}, "version_id": "b4917223-2f12-49ce-a9fb-17b07c8abb63"}]}, "item_language": {"attribute_name": "\u8a00\u8a9e", "attribute_value_mlt": [{"subitem_language": "eng"}]}, "item_resource_type": {"attribute_name": "\u8cc7\u6e90\u30bf\u30a4\u30d7", "attribute_value_mlt": [{"resourcetype": "thesis", "resourceuri": "http://purl.org/coar/resource_type/c_46ec"}]}, "item_title": "Frequency and clinical features of hearing loss caused by STRC deletions \uff08STRC\u907a\u4f1d\u5b50\u6b20\u5931\u306b\u3088\u308b\u96e3\u8074\u306e\u983b\u5ea6\u3068\u81e8\u5e8a\u50cf\u306e\u691c\u8a0e\uff09", "item_titles": {"attribute_name": "\u30bf\u30a4\u30c8\u30eb", "attribute_value_mlt": [{"subitem_title": "Frequency and clinical features of hearing loss caused by STRC deletions \uff08STRC\u907a\u4f1d\u5b50\u6b20\u5931\u306b\u3088\u308b\u96e3\u8074\u306e\u983b\u5ea6\u3068\u81e8\u5e8a\u50cf\u306e\u691c\u8a0e\uff09"}]}, "item_type_id": "14", "owner": "1", "path": ["461/848/849"], "permalink_uri": "http://hdl.handle.net/10091/00022148", "pubdate": {"attribute_name": "\u516c\u958b\u65e5", "attribute_name_i18n": "\u516c\u958b\u65e5", "attribute_value": "2020-06-15"}, "publish_date": "2020-06-15", "publish_status": "0", "recid": "21391", "relation": {}, "relation_version_is_last": true, "title": ["Frequency and clinical features of hearing loss caused by STRC deletions \uff08STRC\u907a\u4f1d\u5b50\u6b20\u5931\u306b\u3088\u308b\u96e3\u8074\u306e\u983b\u5ea6\u3068\u81e8\u5e8a\u50cf\u306e\u691c\u8a0e\uff09"], "weko_shared_id": null}