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Enlarged vestibular aqueduct as a cause of postneonatal deafness 前庭导水管扩大是导致新生儿耳聋的原因之一。
Pub Date : 2024-05-01 DOI: 10.1016/j.otoeng.2023.10.004
Carmen Fernández-Cedrón , Paula Sánchez-Fernández , Maite Guntín-García , Isabel Sandoval-Menéndez , Justo Gómez-Martínez , Jose Luis Llorente-Pendás , Faustino Núñez-Batalla

Introduction

The enlarged vestibular aqueduct (EVA) is the most frequent malformation of the inner ear associated with sensorineural hearing loss (5–15%). It exists when the diameter in imaging tests is greater than 1.5 mm at its midpoint. The association between hearing loss and EVA has been described in a syndromic and non-syndromic manner. It can appear as a familial or isolated form and the audiological profile is highly variable. The gene responsible for sensorineural hearing loss associated with EVA is located in the same region described for Pendred syndrome, where the SCL26A4 gene is located.

Objective

To describe a series of children diagnosed with EVA in order to study their clinical and audiological characteristics, as well as the associated genetic and vestibular alterations.

Method

Retrospective study of data collection of children diagnosed with EVA, from April 2014 to February 2023.

Results

Of the 17 cases, 12 were male and 5 were female. 5 of them were unilateral and 12 bilateral. In 5 cases, a cranial traumatism triggered the hearing loss. Genetic alterations were detected in 3 cases: 2 mutations in the SCL26A4 gene and 1 mutation in the MCT1 gene. 13 patients (76.5%) were rehabilitated with hearing aids and 9 of them required cochlear implantation.

Discussion

The clinical importance of AVD lies in the fact that it is a frequent finding in the context of postneonatal hearing loss. It is convenient to have a high suspicion to diagnose it with imaging tests, to monitor its evolution, and to rehabilitate early.

引言前庭导水管扩大(EVA)是与感音神经性听力损失相关的最常见的内耳畸形(5%-15%)。当造影检查发现其中点直径大于 1.5 毫米时,即为 EVA。听力损失与 EVA 之间的关系有综合征和非综合征之分。听力损失可表现为家族性或孤立性,听力学特征变化很大。与 EVA 相关的感音神经性听力损失的致病基因位于与 Pendred 综合征相同的区域,即 SCL26A4 基因所在区域:描述一系列被诊断为 EVA 的儿童,以研究他们的临床和听力特征,以及相关的遗传和前庭改变:方法:对2014年4月至2023年2月期间确诊为EVA的儿童进行数据收集的回顾性研究:结果:17 例病例中,12 例为男性,5 例为女性。其中 5 例为单侧,12 例为双侧。在 5 例病例中,颅脑创伤引发了听力损失。3例患者的基因发生了改变:2例是SCL26A4基因突变,1例是MCT1基因突变。13名患者(76.5%)使用助听器进行了康复治疗,其中9人需要植入人工耳蜗:讨论:AVD 的临床重要性在于它是新生儿出生后听力损失的常见病。高度怀疑并通过影像学检查进行诊断、监测其发展并及早进行康复治疗是非常方便的。
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引用次数: 0
Current opinion on laryngeal electromyography 喉肌电图研究现状。
Pub Date : 2024-05-01 DOI: 10.1016/j.otoeng.2023.07.001
Rosa-Delia Ramírez-Ruiz , Mariam Quintillá , Marta Sandoval , Lucía León , Jose Miguel Costa , Miquel Quer

Purpose

This study evaluates expert opinion on laryngeal electromyography (LEMG).

Methods

A cross-sectional design was used to conduct an online survey of LEMG experts in 2021. They were questioned about the number LEMG performed annually, type of electrodes used, sector worked in, pain during the test, placement of the needle electrodes, interpretation of electrical muscle parameters, diagnosis of neuromuscular injury, prognostic sensitivity in vocal fold paralysis (VFP), laryngeal dystonia, tremor and synkinesis and quantifying LEMG.

Results

Thirty-seven professionals answered (23 Spanish and 14 from other countries), with a response rate of 21.56%. All physicians used LEMG. 91.9% had one- or two-years’ experience and 56.8% performed 10–40 LEMG per year. 70.3% were otolaryngologists and 27%, neurologists. In 89.1% of cases, a team of electrodiagnostic physician and otolaryngologist performed LEMG. 91.3% of Spanish respondents worked in Public Health, 7.14% of other nationalities; 37.8% in a university department. Bipolar concentric needles electrodes were used by 45.9% and monopolar concentric by 40.5%. 57% professionals considered good patients’ tolerance to the test. LEMG sensitivity was regarded as strong, median and interquartile range were 80.0 [60.0;90.0] to diagnose peripheral nerve injuries, less for other levels of lesions, and strong to evaluate prognosis, 70.0 [50.0;80.0]. Respondents believe locate the thyroarytenoid and the cricothyroid muscles with the needle, 80.0 [70.0;90.0], as opposed to 20.0 [0.00;60.0] the posterior cricoarytenoid. The interpretation of the electrical parts of the LEMG was strong, 80.0 [60.0;90.0]. LEMG identify movements disorders, 60.0 [20.0;80.0], and synkinesis, 70.0 [30.0;80.0]. The professionals prefer quantitative LEMG, 90.0 [60.0;90.0].

Conclusions

The experts surveyed consider LEMG that is well tolerated by patients. The insertional and spontaneous activity, recruitment and waveform morphology can be assessed easily. LEMG is mainly useful in the study of peripheral nerve injuries, and its value in VFP prognosis is considered strong.

目的:本研究评估专家对喉肌电图(LEMG)的意见。方法:采用横断面设计对2021年LEMG专家进行在线调查。他们被问及每年进行LEMG的次数、使用的电极类型、工作部位、测试期间的疼痛、针电极的放置、肌肉电参数的解释、神经肌肉损伤的诊断、声带麻痹(VFP)的预后敏感性、喉肌张力障碍,结果:37名专业人员(23名西班牙语和14名其他国家的专业人员)回答了问题,回答率为21.56%。所有医生都使用了LEMG。91.9%的患者有一到两年的经验,56.8%的患者每年进行10-40次LEMG。70.3%为耳鼻喉科医生,27%为神经科医生。在89.1%的病例中,一个由电诊断医生和耳鼻喉科医生组成的团队进行了LEMG。91.3%的西班牙受访者在公共卫生部门工作,7.14%的其他国籍受访者;37.8%在大学系。45.9%的患者使用双极同心针电极,40.5%的患者使用单极同心针电极。57%的专业人员认为患者对测试的耐受性良好。LEMG的敏感性很强,诊断周围神经损伤的中位和四分位间距为80.0[60.00;90.0],其他级别的病变较少,评估预后的敏感性很高,为70.0[50.00;80.0]。受访者认为用针定位甲状腺杓和环甲肌,为80.0[70.00;90.0],而环杓后肌为20.0[0.00;60.0]。对LEMG的电气部分的解释很强,为80.0[60.00;90.0]。LEMG识别运动障碍,为60.0[20.00;80.0],识别联合运动,为70.0[30.0;80.0]。专业人士更喜欢定量LEMG,为90.0[60.00;90.0]。结论:接受调查的专家认为LEMG患者耐受性良好。插入和自发活动、募集和波形形态可以很容易地评估。LEMG主要用于外周神经损伤的研究,其在VFP预后中的价值被认为是很高的。
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引用次数: 0
Results of surgical treatment of lymph node metastases in patients with cutaneous squamous cell carcinoma of the head and neck 头颈部皮肤鳞状细胞癌患者淋巴结转移的手术治疗效果。
Pub Date : 2024-05-01 DOI: 10.1016/j.otoeng.2023.07.005
Xavier León , Susana López , María Pérez , Cristina Valero , Anna Holgado , Miquel Quer , Carmen Vega

Introduction

Out of all cutaneous squamous cell carcinomas originating in the head and neck (HNCSCC), 2–4% are associated with parotid or cervical lymph node metastasis. The aim of this study is to analyse the prognostic factors of patients with HNCSCC with lymph node involvement treated surgically. Additionally, we aim to compare the prognostic capacity of the classification of these patients according to the 8th edition of the TNM, and an alternative classification proposed by O'Brien et al.

Patients and methods

Retrospective review of 65 patients with HNCSCC with lymph node metastasis treated surgically during the period 2000–2020.

Results

During the study period we carried out 13 neck dissections and 52 parotidectomies + neck dissection in patients with lymph node metastases from a HNCSCC. The great majority of patients (89.2%) received post-operative radiotherapy. The 5 year disease-specific survival was 69.9%, and the overall survival it was 42.8%. The classification proposed by O'Brien et al., based on the parotid or cervical location of the lymph node metastases, and the size and number of the metastatic lymph nodes, had a better prognostic capacity than the TNM classification.

Conclusions

The surgical treatment of lymph node metastases in patients with HNCSCC achieved a high disease control. The classification based on the location, size and number of lymph node metastases proposed by O'Brien et al had better prognostic capacity than the TNM classification.

导言:在所有起源于头颈部的皮肤鳞状细胞癌(HNCSCC)中,2%-4%伴有腮腺或颈淋巴结转移。本研究旨在分析接受手术治疗的淋巴结受累的 HNCSCC 患者的预后因素。此外,我们还旨在比较根据第 8 版 TNM 和 O'Brien 等人提出的替代分类法对这些患者进行分类的预后能力。 患者和方法:回顾性研究 2000-2020 年间接受手术治疗的 65 例淋巴结转移的 HNCSCC 患者:在研究期间,我们为 HNCSCC 淋巴结转移患者实施了 13 例颈部切除术和 52 例腮腺切除术 + 颈部切除术。绝大多数患者(89.2%)接受了术后放疗。5年疾病特异性生存率为69.9%,总生存率为42.8%。O'Brien等人根据淋巴结转移的腮腺或颈部位置、转移淋巴结的大小和数量提出的分类方法,其预后能力优于TNM分类方法:结论:对HNCSCC患者淋巴结转移的手术治疗可达到较高的疾病控制率。O'Brien等人提出的基于淋巴结转移位置、大小和数量的分类方法比TNM分类方法具有更好的预后能力。
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引用次数: 0
Diagnostic yield of genetic testing in adults with sensorineural hearing loss 感音神经性听力损失成人基因检测的诊断率。
Pub Date : 2024-05-01 DOI: 10.1016/j.otoeng.2023.10.007
Sara Reda del Barrio , Joaquín de Vergas Gutiérrez , Juan Francisco Quesada-Espinosa , María Teresa Sánchez-Calvín , Irene Gómez-Manjón , Olalla Sierra-Tomillo , Alexandra Juárez-Rufián , Alfredo García Fernández

Introduction

The contribution of genetic causes to sensorineural hearing loss (SNHL) in adults is less clear than in children, and genetic diagnosis is still not standardized in adults. In this study we present the genetic results obtained in a cohort of adult patients with SNHL.

Materials and methods

We included 63 adults with SNHL that received genetic testing between 2019 and 2022. Whole exome sequencing was performed and variants in genes related to hearing loss (virtual panel with 244 genes) were prioritised and analysed.

Results

24% (15/63) of patients were genetically diagnosed: 87% (13/15) of patients had non-syndromic hearing loss and 13% (2/15) had syndromic hearing loss. We identified pathogenic and likely pathogenic variants in 11 different genes.

Conclusions

Our results show that a significant proportion of adults with SNHL have a genetic origin, and that implementation of genetic testing improves diagnostic accuracy and allows personalized management of these patients.

导言:与儿童相比,遗传因素对成人感音神经性听力损失(SNHL)的影响并不明显,而且成人的遗传诊断仍未标准化。在本研究中,我们介绍了一组成年 SNHL 患者的基因检测结果:我们纳入了在 2019 年至 2022 年期间接受基因检测的 63 名 SNHL 成人患者。进行了全外显子组测序,并优先分析了与听力损失相关的基因变异(包含 244 个基因的虚拟面板).结果:24%(15/63)的患者得到了基因诊断:结果:24%(15/63)的患者得到了基因诊断:87%(13/15)的患者患有非综合征性听力损失,13%(2/15)的患者患有综合征性听力损失。我们在 11 个不同的基因中发现了致病变体和可能致病的变体:我们的研究结果表明,相当一部分成年 SNHL 患者有遗传病史,实施基因检测可提高诊断准确性,并对这些患者进行个性化管理。
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引用次数: 0
Regarding the process of adaptation and validation into Spanish of the specific questionnaire on quality of life in patients with tracheostomy (TQOL) 关于气管造口术患者生活质量(TQOL)专项调查问卷的西班牙文改编和验证过程。
Pub Date : 2024-05-01 DOI: 10.1016/j.otoeng.2023.10.009
Ángel Roco-Videla , Valeska Torres-Bustos
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引用次数: 0
Comment on “Skin and soft tissue complications of bone-anchored hearing aids: Introducing a new classification system” 关于 "骨固定助听器的皮肤和软组织并发症:引入新的分类系统"
Pub Date : 2024-03-01 DOI: 10.1016/j.otoeng.2023.09.005
Tjerk Winus Aukema , Ivo Joachim Kruyt , Myrthe Karianne Sophie Hol
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引用次数: 0
Comments on “Otolaryngological manifestations in monkeypox” 关于 "猴痘中的耳鼻喉科疾病 "的评论。
Pub Date : 2024-03-01 DOI: 10.1016/j.otoeng.2023.10.006
Aida Veiga-Alonso , Trinidad Dierssen-Sotos , Carmelo Morales-Angulo
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引用次数: 0
Clinical practice guideline on the management of vestibular schwannoma 前庭分裂瘤治疗临床实践指南。
Pub Date : 2024-03-01 DOI: 10.1016/j.otoeng.2023.10.005
Luis Lassaletta , Leticia Acle Cervera , Xabier Altuna , Emilio Amilibia Cabeza , Miguel Arístegui Ruiz , Ángel Batuecas Caletrio , Jesús Benítez del Rosario , Rubén Cabanillas Farpón , María Costales Marcos , Pedro Escada , Juan Manuel Espinosa-Sánchez , Roberto García Leal , Javier Gavilán , Justo Gómez Martínez , Rocío González-Aguado , Victor Martinez-Glez , Gloria Guerra Jiménez , Alejandro Harguindey Antolí-Candela , Borja J. Hernández García , Cesar Orús Dotú , José Manuel Morales Puebla

Introduction

Vestibular schwannoma (VS) is the most common tumour of the cerebellopontine angle. The greater accessibility to radiological tests has increased its diagnosis. Taking into account the characteristics of the tumour, the symptoms and the age of the patient, three therapeutic strategies have been proposed: observation, surgery or radiotherapy. Choosing the most appropriate for each patient is a frequent source of controversy.

Material and methods

This paper includes an exhaustive literature review of issues related to VS that can serve as a clinical guide in the management of patients with these lesions. The presentation has been oriented in the form of questions that the clinician usually asks himself and the answers have been written and/or reviewed by a panel of national and international experts consulted by the Otology Commission of the SEORL-CCC.

Results

A list has been compiled containing the 13 most controversial thematic blocks on the management of VS in the form of 50 questions, and answers to all of them have been sought through a systematic literature review (articles published on PubMed and Cochrane Library between 1992 and 2023 related to each thematic area). Thirty-three experts, led by the Otology Committee of SEORL-CCC, have analyzed and discussed all the answers. In Annex 1, 14 additional questions divided into 4 thematic areas can be found.

Conclusions

This clinical practice guideline on the management of VS offers agreed answers to the most common questions that are asked about this tumour. The absence of sufficient prospective studies means that the levels of evidence on the subject are generally medium or low. This fact increases the interest of this type of clinical practice guidelines prepared by experts.

简介前庭分裂瘤(VS)是小脑视角最常见的肿瘤。放射学检查的普及提高了对该肿瘤的诊断率。根据肿瘤的特征、症状和患者的年龄,提出了三种治疗策略:观察、手术或放疗。材料和方法:本文对与 VS 相关的问题进行了详尽的文献综述,可作为此类病变患者的临床治疗指南。本文以临床医生通常会提出的问题为导向,由 SEORL-CCC 耳科委员会咨询的国内外专家小组撰写和/或审阅了答案:通过系统的文献回顾(1992 年至 2023 年期间在 PubMed 和 Cochrane 图书馆上发表的与每个专题领域相关的文章),为所有这些问题寻找答案。由 SEORL-CCC 耳科委员会领导的 33 位专家对所有答案进行了分析和讨论。在附件 1 中,还列出了 14 个分为 4 个专题领域的附加问题:本临床实践指南为 VS 的治疗提供了关于该肿瘤最常见问题的一致答案。由于缺乏足够的前瞻性研究,因此该主题的证据水平一般为中等或较低。这一事实增加了人们对这类由专家编写的临床实践指南的兴趣。
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引用次数: 0
The role of allergic and nonallergic rhinitis on success of type 1 cartilage tympanoplasty 过敏性和非过敏性鼻炎对1型软骨鼓室成形术成功的影响。
Pub Date : 2024-03-01 DOI: 10.1016/j.otoeng.2023.10.001
Elif Ersoy Çallıoğlu , Kazım Bozdemir , Şadan Soyyiğit , Saliha Atalay , Bengi Arslan

Objective

To investigate the role of allergic rhinitis (AR) and non-allergic rhinitis (NAR) on success of type 1 cartilage tympanoplasty.

Methods

This prospective study was conducted on 60 patients who had type 1 cartilage tympanoplasty. The patients were divided into three groups as no-rhinitis (n = 28), NAR (n = 18) and AR (n = 14) groups, based on their symptoms, skin prick tests and/or serum specific IgE levels. AR and NAR groups were treated for their rhinitis symptoms both pre- and postoperatively. The patients were followed up for a minimum of 6 months and compared for graft success rates and audiological outcomes.

Results

Three study groups were similar for age, gender distributions and preoperative air-bone gaps (p = 0.780, p = 0.167 and p = 0.676, respectively). Postoperative graft perforation rate was 0% in no-rhinitis and AR groups while it was 16.7% in NAR group, with a significant difference among three groups (p = 0.034). The comparison of three study groups for change in the postoperative air bone gaps in comparison with preoperative air bone gaps did not yield any statistically significant result (p = 0.729).

Conclusion

Although AR does not result in failure of type 1 cartilage tympanoplasty in patients treated for rhinitis compared to the control group, NAR does. Pre- and postoperative treatment of patients for rhinitis and employment of cartilage graft may be the key factors for success of surgery in patients with AR. Further studies with a larger sample size are needed.

目的:探讨过敏性鼻炎和非过敏性鼻炎对1型软骨鼓室成形术成功率的影响。方法:对60例1型软骨鼓室成形术患者进行前瞻性研究。将患者分为无鼻炎组(n = 28),NAR(n = 18) 和AR(n = 14) 根据症状、皮肤点刺试验和/或血清特异性IgE水平。AR组和NAR组在术前和术后均对其鼻炎症状进行治疗。对患者进行了至少6个月的随访,并比较了移植物成功率和听力学结果。结果:三个研究组在年龄、性别分布和术前气骨间隙方面相似(p = 0.780,p = 0.167和p = 0.676)。无鼻炎组和AR组术后移植物穿孔率为0%,NAR组为16.7%,三组间差异有统计学意义(p = 0.034)。三个研究组的术后气骨间隙与术前气骨间隙的变化比较没有产生任何统计学上显著的结果(p = 0.729)。结论:尽管与对照组相比,AR不会导致鼻炎患者1型软骨鼓室成形术失败,但NAR确实会。鼻炎患者的术前和术后治疗以及软骨移植可能是AR患者手术成功的关键因素。需要进行更大样本量的进一步研究。
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引用次数: 0
Perichondritis and auricular cellulitis related to piercings as first manifestation of monkeypox 与穿孔有关的软骨周炎和耳廓蜂窝织炎是猴痘的首发症状。
Pub Date : 2024-03-01 DOI: 10.1016/j.otoeng.2024.01.001
Encarnación Antúnez-Estudillo , Laura Riera Tur , Andrés Caballero García

Introduction

Pinna infections are usually due to Staphylococcus aureus infection. It is common for the patient to have had an earring in the area of infection. Monkeypox infection has gone from being an endemic infection to a worldwide health emergency.

Case summary

In this article we present five cases of monkeypox earring infection of the pinna and what common features we have seen that differentiate them from Staphylococcus aureus infection.

Discussion

Symptoms of monkeypox include general malaise, fever with uni- or bilateral lymphadenopathy, and then the appearance within one or two days of skin lesions, we want to alert he otolaryngologist and the medical society to the possibility the diagnostic possibility of monkeypox in patients with an auricular perichondritis.

简介耳廓感染通常是由于金黄色葡萄球菌感染所致。患者通常在感染部位戴过耳环。病例摘要:本文介绍了五例猴痘耳环感染耳廓的病例,以及我们所见的区别于金黄色葡萄球菌感染的共同特征:猴痘的症状包括全身不适、发热并伴有单侧或双侧淋巴结肿大,然后在一到两天内出现皮损,我们希望提醒耳鼻喉科医生和医学会注意耳廓周软骨炎患者诊断猴痘的可能性。
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引用次数: 0
期刊
Acta otorrinolaringologica espanola
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