C Maquet, A Willemet, A Francois, F Crampon, S Deneuve
{"title":"Middle-ear osteolytic transthyretin amyloidosis: A CARE case report.","authors":"C Maquet, A Willemet, A Francois, F Crampon, S Deneuve","doi":"10.1016/j.anorl.2024.09.002","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>Wild-type transthyretin amyloidosis (ATTRwt) is a rare but serious disease that is underestimated due to asymptomatic progression. Cardiac deposits worsen prognosis, highlighting the importance of early detection for preventive treatment.</p><p><strong>Case report: </strong>An elderly patient presented with an osteolytic lesion of the middle ear. Pathology diagnosed amyloid transthyretin deposits associated with cholesteatoma.</p><p><strong>Discussion: </strong>Identifying reliable markers to screen for risk of cardiac amyloidosis is important, due to poor prognosis. Recent studies found higher prevalence of hearing loss in ATTRwt than in the general population. The present case identified the middle ear as a target of ATTR, which could improve our understanding of the pathophysiology.</p>","PeriodicalId":48834,"journal":{"name":"European Annals of Otorhinolaryngology-Head and Neck Diseases","volume":" ","pages":""},"PeriodicalIF":1.9000,"publicationDate":"2024-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"European Annals of Otorhinolaryngology-Head and Neck Diseases","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1016/j.anorl.2024.09.002","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"OTORHINOLARYNGOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Introduction: Wild-type transthyretin amyloidosis (ATTRwt) is a rare but serious disease that is underestimated due to asymptomatic progression. Cardiac deposits worsen prognosis, highlighting the importance of early detection for preventive treatment.
Case report: An elderly patient presented with an osteolytic lesion of the middle ear. Pathology diagnosed amyloid transthyretin deposits associated with cholesteatoma.
Discussion: Identifying reliable markers to screen for risk of cardiac amyloidosis is important, due to poor prognosis. Recent studies found higher prevalence of hearing loss in ATTRwt than in the general population. The present case identified the middle ear as a target of ATTR, which could improve our understanding of the pathophysiology.
期刊介绍:
European Annals of Oto-rhino-laryngology, Head and Neck diseases heir of one of the oldest otorhinolaryngology journals in Europe is the official organ of the French Society of Otorhinolaryngology (SFORL) and the the International Francophone Society of Otorhinolaryngology (SIFORL). Today six annual issues provide original peer reviewed clinical and research articles, epidemiological studies, new methodological clinical approaches and review articles giving most up-to-date insights in all areas of otology, laryngology rhinology, head and neck surgery. The European Annals also publish the SFORL guidelines and recommendations.The journal is a unique two-armed publication: the European Annals (ANORL) is an English language well referenced online journal (e-only) whereas the Annales Françaises d’ORL (AFORL), mail-order paper and online edition in French language are aimed at the French-speaking community. French language teams must submit their articles in French to the AFORL site.
Federating journal in its field, the European Annals has an Editorial board of experts with international reputation that allow to make an important contribution to communication on new research data and clinical practice by publishing high-quality articles.