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Middle-ear osteolytic transthyretin amyloidosis: A CARE case report. 中耳溶骨性转甲状腺素淀粉样变性病:CARE病例报告。
IF 1.9 4区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-09-17 DOI: 10.1016/j.anorl.2024.09.002
C Maquet, A Willemet, A Francois, F Crampon, S Deneuve

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.

简介野生型转甲状腺素淀粉样变性(ATTRwt)是一种罕见但严重的疾病,由于无症状进展而被低估。心脏沉积物会使预后恶化,这突出了早期检测对预防性治疗的重要性:病例报告:一名老年患者出现中耳溶骨性病变。病理诊断为胆脂瘤相关的淀粉样转甲状腺素沉积:讨论:由于心脏淀粉样变性的预后较差,因此确定可靠的标志物来筛查心脏淀粉样变性的风险非常重要。最近的研究发现,ATTRwt 患者听力损失的发生率高于普通人群。本病例确定了中耳是 ATTR 的靶点,这有助于加深我们对病理生理学的理解。
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引用次数: 0
Sensory room: Naturalistic assessment of auditory and visual perception in developing children. 感官室:对发育中儿童的听觉和视觉感知进行自然评估。
IF 1.9 4区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-09-10 DOI: 10.1016/j.anorl.2024.08.002
A Lasfargues-Delannoy, A Berland, N Cochard, H Husson, M-N Calmels, J Tardieu, P Gaillard, P Barone, C Farrer, O Deguine

Introduction: Hearing is essential for language acquisition and understanding the environment. Understanding how children react to auditory and visual information is essential for appropriate management in case of hearing loss. Objective and subjective assessments can diagnose hearing loss, but do not measure natural perception in children. We developed a "sensory room" for complementary assessment of children's perceptions so as to assess behavioral responses to meaningful natural sounds and visual stimuli in an ecologic environment suited to children.

Material and methods: Sixteen normal-hearing children and 10 with congenital hearing loss before cochlear implantation, aged 13 to 32months, were included in this feasibility study. They perceived 18 environmental sounds and 9 visual stimuli, and their behavioral responses were coded accordingly as: stopping, looking, moving, pointing, language or emotional reactions.

Result: All children completed the task, demonstrating its feasibility in children. Percentage responses to auditory versus visual stimuli did not differ in normal-hearing children; those with congenital hearing loss responded like normal-hearing children to visual stimuli, but did not react to auditory stimuli. Progression in normal-hearing children's behavioral responses corresponded to cognitive and linguistic development according to age.

Conclusion: The "sensory room" quantified children's responses to various auditory and visual stimuli, providing clinicians with measurable insight into the children's sensory perception and processing.

导言听觉对于语言学习和理解环境至关重要。了解儿童对听觉和视觉信息的反应对于听力损失的适当管理至关重要。客观和主观评估可以诊断听力损失,但无法测量儿童的自然感知能力。我们开发了一个 "感官室",用于对儿童的感知进行补充评估,以便在适合儿童的生态环境中评估他们对有意义的自然声音和视觉刺激的行为反应:16名听力正常的儿童和10名植入人工耳蜗前患有先天性听力损失的儿童参加了这项可行性研究,他们的年龄在13至32个月之间。他们感知了 18 种环境声音和 9 种视觉刺激,其行为反应被编码为:停止、看、移动、指向、语言或情绪反应:结果:所有儿童都完成了任务,证明了这项任务在儿童中的可行性。听力正常儿童对听觉刺激和视觉刺激的反应百分比没有差异;先天性听力损失儿童对视觉刺激的反应与听力正常儿童相同,但对听觉刺激没有反应。正常听力儿童行为反应的进展与认知和语言发展的年龄相符:感官室 "可以量化儿童对各种听觉和视觉刺激的反应,让临床医生对儿童的感知和处理过程有了可测量的了解。
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引用次数: 0
IgG4-related disease with primary otologic manifestations. 具有原发性耳科表现的 IgG4 相关疾病。
IF 1.9 4区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-09-04 DOI: 10.1016/j.anorl.2024.07.004
S Lu, J Peng, L Cui, Y Li
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引用次数: 0
Systematic MRI in post-COVID-19 anosmia might not be necessary 可能没有必要对 COVID-19 后的无嗅症进行系统的 MRI 检查。
IF 1.9 4区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-09-01 DOI: 10.1016/j.anorl.2024.02.005
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引用次数: 0
Cervical approach for prestyloid parapharyngeal pleomorphic adenoma resection 咽旁多形性腺瘤前淀粉样变性切除术的颈部入路。
IF 1.9 4区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-09-01 DOI: 10.1016/j.anorl.2024.03.005

Objective

To describe the key points of cervical resection for prestyloid parapharyngeal pleomorphic adenoma and to discuss the role of modern imaging.

Observation

Retrospective case series of 10 patients (4 women and 6 men, age 29–63 years) with prestyloid parapharyngeal pleomorphic adenoma with 2 to 8 cm largest diameter on MRI, consecutively resected via a cervical approach between 2000 and 2020 in a French tertiary university referral care center. Seven patients had a minimum 10 years’ follow-up, and one was lost to follow-up before the fifth postoperative year. Peri- and postoperative complications comprised great auricular nerve transection without subsequent symptomatic neuroma (2 patients), associated transoral approach to free the upper pole of the adenoma (2 patients), capsule effraction (3 patients), and hematoma (1 patient). There were no cases of facial paresis or palsy, other cranial nerve impairment, trismus, auriculotemporal or first-bite syndrome. One of the three patients with capsule effraction showed local recurrence at month 17.

Conclusion

In agreement with previous reports, the present case series confirmed the role of the cervical approach to resect prestyloid parapharyngeal pleomorphic adenoma, and hence the need to continue teaching it.
目的:描述咽旁多形性腺瘤颈部切除术的要点,并讨论现代影像学的作用:描述咽旁前叶多形性腺瘤颈部切除术的要点,并讨论现代影像学的作用:回顾性病例系列:10 例患者(4 名女性和 6 名男性,年龄 29-63 岁)均患有前淀粉样咽旁多形性腺瘤,磁共振成像显示其最大直径为 2-8 厘米,2000 年至 2020 年期间在一家法国三级大学转诊护理中心连续通过颈部入路进行了切除。七名患者接受了至少十年的随访,一名患者在术后第五年前失去了随访机会。围手术期和术后并发症包括大耳廓神经横断但随后没有症状的神经瘤(2 名患者)、相关经口方法游离腺瘤上极(2 名患者)、囊肿脱出(3 名患者)和血肿(1 名患者)。没有出现面瘫或麻痹、其他颅神经损伤、三叉神经痛、耳颞综合征或第一咬合综合征的病例。三名囊肿脱出患者中有一人在第 17 个月时出现局部复发:与之前的报道一致,本病例系列证实了颈部入路切除咽旁多形性腺瘤的作用,因此有必要继续开展相关教学。
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引用次数: 0
Systemic therapies for salivary gland cancer: Adenoid cystic carcinoma. REFCOR recommendations by the formal consensus method 唾液腺癌的系统疗法:腺样囊性癌。REFCOR通过正式共识法提出的建议
IF 1.9 4区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-09-01 DOI: 10.1016/j.anorl.2023.11.009

Objective

To determine the therapeutic indications for systemic medical treatment in the management of adenoid cystic carcinoma (ACC) according to the clinical situation.

Materials and methods

The French Network of Rare Head and Neck Tumors (REFCOR) formed a steering group, which drafted a narrative review of the literature published on Medline and proposed recommendations. The level of adherence to the recommendations was then assessed by a rating group, according to the formal consensus method.

Results

ACCs are rare tumors and there is currently insufficient evidence to indicate chemotherapy at the localized stage. At the metastatic stage, progression is often slow. In case of oligometastatic ACC, local treatment should be discussed. The most often indolent nature of polymetastatic ACC can allow management by active surveillance. Molecular screening is recommended, for abnormalities potentially accessible to targeted therapy.

Conclusion

ACCs are rare tumors for which there are currently few effective medical treatments. It is therefore recommended to include patients in clinical trials.
材料与方法法国罕见头颈部肿瘤网络(REFCOR)成立了一个指导小组,该小组对 Medline 上发表的文献进行了叙述性综述,并提出了建议。结果ACCs是一种罕见肿瘤,目前还没有足够的证据表明可以在局部阶段进行化疗。在转移阶段,病情发展通常比较缓慢。对于少转移性 ACC,应讨论局部治疗。多发转移性 ACC 通常病情较轻,可以通过积极的监测进行治疗。建议进行分子筛查,以确定是否存在可用于靶向治疗的异常。因此,建议将患者纳入临床试验。
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引用次数: 0
A century ago, the birth of the decibel 一个世纪前,分贝诞生。
IF 1.9 4区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-09-01 DOI: 10.1016/j.anorl.2024.06.002
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引用次数: 0
An unusual case of cervical cellulitis 一个不寻常的宫颈蜂窝组织炎病例。
IF 1.9 4区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-09-01 DOI: 10.1016/j.anorl.2024.05.001
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引用次数: 0
Sustainable ENT: international collaboration is key 可持续的环境技术:国际合作是关键
IF 1.9 4区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-09-01 DOI: 10.1016/j.anorl.2024.02.013
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引用次数: 0
Systemic therapies for salivary gland carcinoma (excluding adenoid cystic carcinoma): REFCOR recommendations by the formal consensus method 唾液腺癌(不包括腺样囊性癌)的全身治疗:REFCOR通过正式共识方法推荐。
IF 1.9 4区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-09-01 DOI: 10.1016/j.anorl.2023.11.004

Objective

To determine the therapeutic indications for systemic medical treatment in the management of salivary gland carcinoma (excluding adenoid cystic carcinoma) according to the clinical situation.

Materials and methods

The French Network of Rare Head and Neck Tumors (REFCOR) formed a steering group who drafted a narrative review of the literature published on Medline and proposed recommendations. The level of adherence to the recommendations was then assessed by a rating group, according to the formal consensus method.

Results

Salivary gland carcinoma is rare and there is currently insufficient evidence to indicate chemotherapy at the localized stage. At the metastatic stage, initial management can be based on a phase of monitoring for indolent disease. Some histological subtypes (salivary duct carcinoma and adenocarcinoma) are more aggressive and require systemic treatment from the outset. To guide systemic treatment, it is recommended to perform immunohistochemistry and molecular biology analyses (overexpression of HER2 and androgen receptors, NTRK fusion, next-generation sequencing).

Conclusion

Salivary gland carcinoma is a rare tumor for which there are currently few effective medical treatments. It is therefore recommended to include patients in clinical trials.
目的:根据临床情况,确定涎腺癌(不包括腺样囊性癌)全身性药物治疗的指征。材料和方法:法国罕见头颈部肿瘤网络(REFCOR)成立了一个指导小组,起草了Medline上发表的文献的叙述性综述,并提出了建议。然后,根据正式的共识方法,由一个评级小组评估对建议的遵守程度。结果:唾液腺癌是罕见的,目前没有足够的证据表明在局部阶段化疗。在转移期,最初的管理可以基于对惰性疾病的监测阶段。一些组织学亚型(唾液管癌和腺癌)更具侵袭性,需要从一开始就进行全身治疗。为了指导全身治疗,建议进行免疫组织化学和分子生物学分析(HER2和雄激素受体的过表达,NTRK融合,下一代测序)。结论:唾液腺癌是一种罕见的肿瘤,目前有效的治疗方法很少。因此,建议将患者纳入临床试验。
{"title":"Systemic therapies for salivary gland carcinoma (excluding adenoid cystic carcinoma): REFCOR recommendations by the formal consensus method","authors":"","doi":"10.1016/j.anorl.2023.11.004","DOIUrl":"10.1016/j.anorl.2023.11.004","url":null,"abstract":"<div><h3>Objective</h3><div>To determine the therapeutic indications for systemic medical treatment in the management of salivary gland carcinoma (excluding adenoid cystic carcinoma) according to the clinical situation.</div></div><div><h3>Materials and methods</h3><div>The French Network of Rare Head and Neck Tumors (REFCOR) formed a steering group who drafted a narrative review of the literature published on Medline and proposed recommendations. The level of adherence to the recommendations was then assessed by a rating group, according to the formal consensus method.</div></div><div><h3>Results</h3><div>Salivary gland carcinoma is rare and there is currently insufficient evidence to indicate chemotherapy at the localized stage. At the metastatic stage, initial management can be based on a phase of monitoring for indolent disease. Some histological subtypes (salivary duct carcinoma and adenocarcinoma) are more aggressive and require systemic treatment from the outset. To guide systemic treatment, it is recommended to perform immunohistochemistry and molecular biology analyses (overexpression of HER2 and androgen receptors, NTRK fusion, next-generation sequencing).</div></div><div><h3>Conclusion</h3><div>Salivary gland carcinoma is a rare tumor for which there are currently few effective medical treatments. It is therefore recommended to include patients in clinical trials.</div></div>","PeriodicalId":48834,"journal":{"name":"European Annals of Otorhinolaryngology-Head and Neck Diseases","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138471081","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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European Annals of Otorhinolaryngology-Head and Neck Diseases
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