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Clinical characteristics of delayed facial palsy after middle ear surgery- a descriptive study of 40 cases. 中耳手术后迟发性面瘫40例临床特点分析
IF 2.2 3区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2025-09-01 Epub Date: 2025-04-12 DOI: 10.1007/s00405-025-09384-y
Attila Óvári, Max Bielenberg, Bruno Neuner, Jens Eduard Meyer

Purpose: To identify probable triggers of delayed facial palsy (DFP) after middle ear surgery.

Methods: Retrospective single-center chart review of DFP cases between 2010 and 2021.

Results: Forty patients with DFP could be identified after 3,508 middle ear surgeries (1.14%). The occurrence of DFP was after tympanoplasty type 1 1.60%, after tympanoplasty type 3 without mastoidectomy 1.32%, after tympanoplasty type 3 with mastoidectomy 0.36%, and after all tympanomastoid surgery cases 0.35%. The anti-HSV-1 IgM was positive in three cases and borderline in two patients. The anti-VZV IgM was positive in three cases. Beside herpes virus reactivation, facial canal dehiscence (n = 8), bacterial infection, direct nerve microtrauma and intratympanal use of collagenous or oxidized cellulose sponges are presumed pathogenetic factors in our study.

Conclusion: The etiology of DFP is still not conclusively clarified, it has probably a heterogeneous pathogenesis and therefore requires further scientific research. Mastoidectomy may have a protecting effect against DFP. The risk may be reduced by avoiding possible promoting factors but this adverse event cannot be substantially eliminated yet. Nonetheless, we summarize practical considerations for the prevention, diagnosis, and therapy of DFP in the future.

目的:探讨中耳手术后迟发性面瘫(DFP)的可能诱因。方法:对2010 - 2021年DFP病例进行回顾性单中心图表分析。结果:3508例中耳手术后可确诊DFP患者40例(1.14%)。1型鼓室成形术后DFP发生率为1.60%,3型鼓室成形术后不切除乳突1.32%,3型鼓室成形术后切除乳突0.36%,所有鼓室乳突手术后DFP发生率为0.35%。抗hsv -1 IgM阳性3例,临界2例。抗vzv IgM阳性3例。在我们的研究中,除了疱疹病毒再激活外,面神经管开裂(n = 8)、细菌感染、直接神经微损伤和鼓室内使用胶原或氧化纤维素海绵被认为是致病因素。结论:DFP的病因尚不明确,其发病机制可能具有异质性,需要进一步的科学研究。乳突切除术可能对DFP有保护作用。通过避免可能的促进因素可以降低风险,但这一不良事件仍不能完全消除。尽管如此,我们总结了未来预防、诊断和治疗DFP的实际考虑。
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引用次数: 0
Volume rendering technique and high-resolution microCT: 3D exploration of the cochlear anatomy. 体绘制技术和高分辨率微ct:耳蜗解剖的三维探索。
IF 2.2 3区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2025-09-01 Epub Date: 2025-04-03 DOI: 10.1007/s00405-025-09360-6
Gabriela O'Toole Bom Braga, Robert Zboray, Annapaola Parrilli, Franca Wagner

Purpose: Given its unique anatomical position and the amalgamation of bony and soft tissues within the cochlea, exploring its intricacies poses persistent challenges. Histopathology remains the gold standard in research, but given its inherent limitations, there is a clear need for innovative alternatives. The integration of microCT technology with advanced volume rendering techniques emerges as a promising approach for overcoming the hurdles associated with anatomical investigations of the cochlea.

Methods: We seamlessly integrated high-resolution microCT cochlear images with medical imaging analysis software to create detailed 3D anatomical images of the human cochlea without the need of sample processing.

Results: Volume rendering allowed a multiplanar, non-destructive, detailed anatomical evaluation of the human cochlea, including its capillary system, as well as soft tissue visualization at single-micron resolution in 3D.

Conclusion: The use of volume rendering in cochlear anatomical studies is underexplored despite the prevalence of 3D reconstruction. This technique presents a promising avenue for scientific investigation, providing researchers with unprecedented insights that can potentially benefit patients with hearing disorders.

目的:鉴于耳蜗独特的解剖位置以及耳蜗内骨组织和软组织的混合,探索耳蜗的复杂性一直是个挑战。组织病理学仍是研究的黄金标准,但鉴于其固有的局限性,显然需要创新的替代方法。将显微 CT 技术与先进的容积渲染技术相结合,是克服耳蜗解剖研究相关障碍的一种可行方法:我们将高分辨率显微CT耳蜗图像与医学成像分析软件无缝整合,无需样本处理即可创建详细的人体耳蜗三维解剖图像:结果:体渲染技术可对人类耳蜗(包括其毛细血管系统)进行多平面、非破坏性的详细解剖评估,并以单微米分辨率在三维空间对软组织进行可视化:结论:尽管三维重建技术已经非常普及,但在耳蜗解剖研究中使用体绘制技术的探索还很不够。这项技术为科学研究提供了一个前景广阔的途径,为研究人员提供了前所未有的见解,有可能造福听力障碍患者。
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引用次数: 0
Variants of rhinitis medicamentosa treatment: a systematic review. 变异型鼻炎药物治疗:系统综述。
IF 2.2 3区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2025-09-01 Epub Date: 2025-04-02 DOI: 10.1007/s00405-025-09344-6
Xi Yang, Kseniya Eremeeva, Valeriy Svistushkin, Daria Lisenkova, Elena Smolyarchuk, Andrey Nedorubov

Purpose: Nasal breathing dysfunction resulting from uncontrolled decongestant use is an extremely urgent public health problem. This condition is referred to as rhinitis medicamentosa. Despite the high incidence of patients with this diagnosis, there is still no consensus on treatment tactics. The purpose of this study was to review the available literature on rhinitis medicamentosa treatment and summarize the findings reported in different approaches.

Methods and materials: We conducted a systematic review of PubMed (MEDLINE), The Cochrane Library, and Clinicaltrials.gov databases to identify studies that describe conservative and surgical treatments for rhinitis medicamentosa.

Results: Twelve studies, including 373 patients, met the search criteria. Out of these, seven studies used topical intranasal steroids like budesonide or fluticasone propionate sprays as a conservative treatment. One study used dexamethasone nasal drops. Five studies involved surgical treatment for patients; three of these studies used radiofrequency ablation to reduce the inferior turbinates, while the other two studies used diode laser and kinetic stimulation, respectively.

Conclusion: All studies included in this systematic review demonstrate the high efficacy of the separately presented treatment methods. However, the different design and evaluation methods do not allow us to systematize the data and develop a unified algorithm for treating rhinitis medicamentosa. We see the potential for conducting comparative evidence-based studies on a larger sample, along with the evaluation of long-term treatment results.

目的:无节制使用减充血剂导致的鼻呼吸功能障碍是一个极为紧迫的公共卫生问题。这种情况被称为药物性鼻炎。尽管这种病症的发病率很高,但在治疗策略上仍未达成共识。本研究的目的是回顾有关药物性鼻炎治疗的现有文献,并总结不同方法的研究结果:我们对 PubMed (MEDLINE)、The Cochrane Library 和 Clinicaltrials.gov 数据库进行了系统性回顾,以确定描述药物性鼻炎保守疗法和手术疗法的研究:有 12 项研究(包括 373 名患者)符合搜索标准。其中,7 项研究使用布地奈德或丙酸氟替卡松喷雾剂等局部鼻内类固醇作为保守疗法。一项研究使用地塞米松滴鼻剂。五项研究涉及对患者进行手术治疗;其中三项研究使用射频消融术缩小下鼻甲,另外两项研究分别使用二极管激光和动能刺激:结论:本系统性综述中包含的所有研究都证明了分别介绍的治疗方法具有很高的疗效。然而,由于设计和评估方法的不同,我们无法对数据进行系统化处理,也无法制定出治疗药物性鼻炎的统一算法。我们认为有可能对更大样本进行循证比较研究,并对长期治疗效果进行评估。
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引用次数: 0
The impact of bilateral vestibulopathy on quality of life: data from the Antwerp University Hospital registry. 双侧前庭病变对生活质量的影响:来自安特卫普大学医院注册的数据。
IF 2.2 3区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2025-09-01 Epub Date: 2025-04-16 DOI: 10.1007/s00405-025-09369-x
Vincent Van Rompaey, Julie Moyaert, Paul Van de Heyning, Bieke Dobbels, Raymond van de Berg, Nils Guinand, Angelica Perez-Fornos, Griet Mertens

Objective: Bilateral vestibulopathy (BVP) is a rare condition which causes significant impairments in balance, spatial orientation, and gaze stability. The goal of this study was to assess the impact of BVP on several outcome domains related to daily life.

Study design: Cross-sectional survey.

Setting: Tertiary referral center for otology and neurotology.

Patients: Sixty-one patients treated for BVP at the Antwerp University Hospital.

Intervention: Questionnaire with items covering several outcome domains.

Main outcome measures: Impact of BVP on employment, mobility, activities of daily living, health outcomes, and finances.

Results: A total of 61 patients with BVP completed the questionnaire. Of these, 47.5% were retired, 26.2% were actively employed and 23% were unable to work due to their symptoms. 4.9% had to change work in the past, and 6.6% had to adjust their work situation. 8.1% had been absent from work for more than 4 weeks in the last year. Of those who had driven in the last year, 42.6% reported that their balance problems had affected their driving ability. 54.1% reported having fallen as a result of their BVP symptoms and 39.4% of these required a visit to their general practitioner or a hospital due to a fall. 14.8% reported that they had visited the emergency room and 21.3% reported that they had been admitted to the hospital in the last year. 13.1% reported that they had borne extra financial costs in the last year due to their condition, with a median burden of €1,000.

Conclusions: BVP has a significant impact on daily life as measured across several domains. These findings indicate that there is a significant disease burden associated with BVP. New treatment or management modalities are needed to reduce this burden.

目的:双侧前庭病变(BVP)是一种罕见的疾病,它会导致平衡、空间定向和凝视稳定性的严重损害。本研究的目的是评估BVP对与日常生活相关的几个结果领域的影响。研究设计:横断面调查。单位:耳科和神经科三级转诊中心。患者:在安特卫普大学医院接受BVP治疗的61例患者。干预:问卷调查项目涵盖几个结果领域。主要结果测量:BVP对就业、流动性、日常生活活动、健康结果和财务的影响。结果:61例BVP患者完成了问卷调查。其中,47.5%退休,26.2%在职,23%因症状无法工作。4.9%的人曾经换过工作,6.6%的人曾经调整过工作状况。8.1%的人去年旷工超过4周。在去年驾车的受访者中,42.6%的人表示他们的平衡问题影响了他们的驾驶能力。54.1%的人报告因BVP症状而跌倒,其中39.4%的人因跌倒而需要去全科医生或医院就诊。14.8%的人报告说他们去过急诊室,21.3%的人报告说他们在去年住过医院。13.1%的受访者表示,他们在过去一年中因身体状况而承担了额外的财务费用,平均负担为1,000欧元。结论:通过多个领域的测量,BVP对日常生活有显著影响。这些发现表明,存在与BVP相关的重大疾病负担。需要新的治疗或管理方式来减轻这一负担。
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引用次数: 0
A novel approach to the follow up of children with otitis media with effusion: wideband absorbance findings. 一种新的方法来跟踪儿童中耳炎积液:宽带吸收发现。
IF 2.2 3区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2025-09-01 Epub Date: 2025-04-12 DOI: 10.1007/s00405-025-09355-3
Melis Keskin Yıldız, Gamze Atay, Esra Kutsal Mergen, Songül Aksoy, Bilgehan Böke

Purpose: Otitis media with effusion (OME) is one of the most common childhood diseases. It is recommended to use tympanometry in addition to otoscopy and/or pneumatic otoscopy for the diagnosis and follow-up of OME. Clinitians are using Wideband absorbance (WBA), which is one of the methods of evaluating the middle ear in the diagnosis of OME, more widely.

Methods: The relationship between the changes in the examination findings obtained by otoscopy and pneumatic otoscopy and the findings of WBA in the monthly follow-ups performed during the three-month period of children diagnosed with OME, was examined. In the study, 48 ears of 26 individuals aged 24-71 month who were diagnosed with OME were evaluated. Otoscopy, pneumatic otoscopy, 226 Hz tympanometry and WBA measurements were performed at the initial, first, second and third month examinations. The relationship between the difference between consecutive measurements was examined because it was thought that consecutive measurements might be more significant in the relationship between measurements. The Spearman test was used to determine whether there was a relationship between WBA and otoscopic and pneumatic otoscopic examination results. For assessing parameters where there was a significant difference between the groups compared, the variables were assessed using Kruskal-Wallis analysis of variance. The Dunn test was used for post hoc testing.

Results: The change observed with both otoscopy and pneumatic otoscopy between the initial and first month control could not be adequately determined by 226 Hz tympanometry, but the change in the amount absorbance at 2520 Hz and 3175 Hz was moderately correlated with the examination findings. However, it was observed that the change between the first and the second follow-up examinations was significant at the frequency range of 226-630 Hz in WBA and the change between the second and third months was significant at the frequency range of 226-4000 Hz in WBA.

Conclusions: The findings obtained in the study show that, 226 Hz tympanometry may be insufficient to reflect the change in examination findings while WBA can provide more detailed information to support the examination findings during the follow-up period as well as the diagnosis of OME.

目的:分泌性中耳炎(OME)是儿童最常见的疾病之一。建议在耳镜检查和/或气动耳镜检查的基础上使用鼓室测量法进行OME的诊断和随访。宽频带吸光度(WBA)作为评价中耳的方法之一,在OME诊断中的应用越来越广泛。方法:对诊断为OME的患儿进行为期三个月的月度随访,分析耳镜和气动耳镜检查结果的变化与WBA的关系。在这项研究中,对26名年龄在24-71个月之间被诊断患有OME的患者的48只耳朵进行了评估。在第一次、第一次、第二次和第三次检查时进行耳镜检查、气动耳镜检查、226 Hz鼓室测量和WBA测量。研究了连续测量之间的差异之间的关系,因为人们认为连续测量可能在测量之间的关系中更为重要。使用Spearman检验来确定WBA与耳镜和气动耳镜检查结果之间是否存在关系。为了评估组间有显著差异的参数,使用Kruskal-Wallis方差分析评估变量。事后检验采用Dunn检验。结果:耳镜和气动耳镜观察到的第一个月对照和第一个月对照之间的变化不能用226 Hz鼓室测量法充分确定,但2520 Hz和3175 Hz吸光度的变化与检查结果有一定的相关性。然而,观察到第一次和第二次随访检查的变化在226-630 Hz的WBA频率范围内是显著的,第二和第三个月的变化在226-4000 Hz的WBA频率范围内是显著的。结论:本研究结果表明,226 Hz鼓室测量可能不足以反映检查结果的变化,而WBA可以提供更详细的信息来支持随访期间的检查结果以及OME的诊断。
{"title":"A novel approach to the follow up of children with otitis media with effusion: wideband absorbance findings.","authors":"Melis Keskin Yıldız, Gamze Atay, Esra Kutsal Mergen, Songül Aksoy, Bilgehan Böke","doi":"10.1007/s00405-025-09355-3","DOIUrl":"10.1007/s00405-025-09355-3","url":null,"abstract":"<p><strong>Purpose: </strong>Otitis media with effusion (OME) is one of the most common childhood diseases. It is recommended to use tympanometry in addition to otoscopy and/or pneumatic otoscopy for the diagnosis and follow-up of OME. Clinitians are using Wideband absorbance (WBA), which is one of the methods of evaluating the middle ear in the diagnosis of OME, more widely.</p><p><strong>Methods: </strong>The relationship between the changes in the examination findings obtained by otoscopy and pneumatic otoscopy and the findings of WBA in the monthly follow-ups performed during the three-month period of children diagnosed with OME, was examined. In the study, 48 ears of 26 individuals aged 24-71 month who were diagnosed with OME were evaluated. Otoscopy, pneumatic otoscopy, 226 Hz tympanometry and WBA measurements were performed at the initial, first, second and third month examinations. The relationship between the difference between consecutive measurements was examined because it was thought that consecutive measurements might be more significant in the relationship between measurements. The Spearman test was used to determine whether there was a relationship between WBA and otoscopic and pneumatic otoscopic examination results. For assessing parameters where there was a significant difference between the groups compared, the variables were assessed using Kruskal-Wallis analysis of variance. The Dunn test was used for post hoc testing.</p><p><strong>Results: </strong>The change observed with both otoscopy and pneumatic otoscopy between the initial and first month control could not be adequately determined by 226 Hz tympanometry, but the change in the amount absorbance at 2520 Hz and 3175 Hz was moderately correlated with the examination findings. However, it was observed that the change between the first and the second follow-up examinations was significant at the frequency range of 226-630 Hz in WBA and the change between the second and third months was significant at the frequency range of 226-4000 Hz in WBA.</p><p><strong>Conclusions: </strong>The findings obtained in the study show that, 226 Hz tympanometry may be insufficient to reflect the change in examination findings while WBA can provide more detailed information to support the examination findings during the follow-up period as well as the diagnosis of OME.</p>","PeriodicalId":11952,"journal":{"name":"European Archives of Oto-Rhino-Laryngology","volume":" ","pages":"4487-4496"},"PeriodicalIF":2.2,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12423127/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143978794","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Translation, cultural adaptation and validation of the Stapesplasty Outcome Test 25 (SPOT-25) for measurement of disease-specific health-related quality of life in Dutch otosclerosis patients: a prospective study. 荷兰耳硬化患者疾病特异性健康相关生活质量的Stapesplasty结果测试25 (SPOT-25)的翻译、文化适应和验证:一项前瞻性研究
IF 2.2 3区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2025-09-01 Epub Date: 2025-05-08 DOI: 10.1007/s00405-025-09353-5
Esther E Blijleven, Maaike Jellema, Joeri Buwalda, Raphael J B Hemler, Huib F van Waegeningh, Robert J Stokroos, Hans G X M Thomeer, Inge Wegner

Purpose: To translate and culturally adapt the SPOT-25 to the Dutch language and validate the Dutch SPOT-25 in a Dutch population of otosclerosis patients undergoing primary stapes surgery.

Methods: A multicenter prospective validation study was performed between November 2018 and May 2024. The translation into Dutch and validation process of the SPOT-25 was performed according to the COSMIN guidelines. Patients were asked to complete the SPOT-25 and Glasgow Health Status Questionnaire (GHSQ) preoperatively, the SPOT-25, GHSQ and Glasgow Benefit Inventory six to eight weeks postoperatively and the SPOT-25 eight to ten weeks postoperatively. Healthy controls were asked to complete the translated SPOT-25 once. Preoperative and postoperative audiometric results were also obtained. The evaluated measurement properties included construct validity, measurement invariance, discriminative validity, reliability and responsiveness of the translated SPOT-25.

Results: Hundred and fifteen patients and 50 healthy controls were analyzed. Analyses of the translated SPOT-25 showed adequate construct validity, discriminative validity, reliability and responsiveness. The SPOT-25 scores were strongly correlated with the GHSQ score. The internal consistency and test-retest reliability were good as Cronbach's alpha and intraclass correlation coefficients were higher than 0.70. The four-factor model fitted best in our population of otosclerosis patients; however the results indicated a mediocre fit between the model and the data.

Conclusion: The Dutch SPOT-25 showed good validity, reliability and responsiveness and can be implemented as an additional outcome measure to improve otosclerosis research and clinical practice.

目的:将SPOT-25翻译成荷兰语,并在荷兰接受初级镫骨手术的耳硬化症患者中验证荷兰语SPOT-25。方法:于2018年11月至2024年5月进行多中心前瞻性验证研究。SPOT-25的荷兰语翻译和验证过程是根据COSMIN指南进行的。患者被要求在术前完成SPOT-25和格拉斯哥健康状况问卷(GHSQ),在术后6 - 8周完成SPOT-25、GHSQ和格拉斯哥福利问卷,在术后8 - 10周完成SPOT-25问卷。健康对照者被要求完成翻译的SPOT-25一次。术前和术后的听力测量结果也得到了。评估的测量性质包括:构念效度、测量不变性、判别效度、信度和反应性。结果:对115例患者和50例健康对照进行了分析。对翻译后的SPOT-25进行分析,结果显示具有足够的构念效度、判别效度、信度和反应性。SPOT-25分数与GHSQ分数密切相关。内部一致性和重测信度较好,Cronbach’s alpha和类内相关系数均大于0.70。四因素模型最适合我国耳硬化患者群体;然而,结果表明模型和数据之间的拟合一般。结论:荷兰SPOT-25量表具有良好的效度、信度和反应性,可作为耳硬化研究和临床实践的附加指标。
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引用次数: 0
Optimization of surgical interventions in auditory rehabilitation for chronic otitis media: comparative between passive middle ear implants, bone conduction implants, and active middle ear systems. 慢性中耳炎听觉康复手术干预的优化:被动中耳植入物、骨传导植入物和主动中耳系统的比较
IF 2.2 3区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2025-09-01 Epub Date: 2025-04-13 DOI: 10.1007/s00405-025-09365-1
Joan Lorente-Piera, Raquel Manrique-Huarte, Sebastián Picciafuoco, Janaina P Lima, Diego Calavia, Valeria Serra, Manuel Manrique

Introduction: In otology consultations, patients with chronic otitis media (COM) often present as candidates for various hearing rehabilitation options. Selecting the most suitable approach requires careful consideration of patient preferences and expectations, the risk of disease progression, and the integrity of the bone conduction pathway. This study aims to evaluate and compare postoperative hearing outcomes in COM patients undergoing tympanoplasty (with or without passive middle ear implants), bone conduction systems (BCI), or active middle ear implants (AMEI). The objective is to assess the effectiveness of each surgical approach in hearing rehabilitation, considering the type and severity of hearing loss as well as the duration of the disease.

Methods: Retrospective data analysis in a tertiary referral center studying average PTA across six different frequencies, speech perception at 65 dB, influence of Eustachian tube dysfunction, reintervention rate and adverse effects, and the influence of disease duration on functional outcomes via linear regression analysis.

Results: 116 patients underwent surgery due to COM between 1998 and 2024. With a slight female predominance (54.31%). AMEIs and bone conduction devices provided the highest amplification in terms of PTA and speech discrimination, with a lower reintervention rate when comparing both groups with passive middle ear implants, OR in BCI group of 0.30 (0.10; 0.89, p = 0.030), OR in VSB group of 0.15 (0.04; 0.56, p = 0.005). It was also observed that a longer evolution time could be associated with greater auditory gain, with a p-value = 0.033.

Conclusions: The selection of each treatment option primarily depends on bone conduction thresholds, along with surgical risk, patient preferences, and MRI compatibility. In our study, AMEIs demonstrated the highest functional gain in terms of speech discrimination and frequency-specific amplification, followed by BCI. These findings support the use of implantable hearing solutions as effective alternatives for auditory rehabilitation in COM patients.

在耳科咨询中,慢性中耳炎(COM)患者经常作为各种听力康复选择的候选人。选择最合适的方法需要仔细考虑患者的偏好和期望、疾病进展的风险以及骨传导途径的完整性。本研究旨在评估和比较接受鼓室成形术(带或不带被动中耳植入物)、骨传导系统(BCI)或主动中耳植入物(AMEI)的COM患者术后听力结果。考虑到听力损失的类型和严重程度以及疾病的持续时间,目的是评估每种手术方法在听力康复中的有效性。方法:回顾性分析某三级转诊中心6个不同频率的平均PTA、65 dB语音感知、耳咽管功能障碍的影响、再干预率和不良反应、疾病持续时间对功能结局的影响,采用线性回归分析。结果:1998年至2024年间,116例患者因COM接受手术治疗。女性略占优势(54.31%)。与被动中耳植入物相比,ami和骨传导装置在PTA和言语辨别方面的放大效果最高,再干预率较低,BCI组OR为0.30 (0.10;0.89, p = 0.030), VSB组OR为0.15 (0.04;0.56, p = 0.005)。进化时间越长,听觉增益越大,p值= 0.033。结论:每种治疗方案的选择主要取决于骨传导阈值、手术风险、患者偏好和MRI兼容性。在我们的研究中,amei在语音识别和特定频率放大方面表现出最高的功能增益,其次是脑机接口。这些发现支持使用植入式听力解决方案作为COM患者听觉康复的有效选择。
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引用次数: 0
Hearing loss in Chinese osteogenesis imperfecta patients. 中国成骨不全患者的听力损失。
IF 2.2 3区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2025-09-01 Epub Date: 2025-04-12 DOI: 10.1007/s00405-025-09390-0
Yuan Tian, Yanxuan Shao, Yazhao Mei, Yunyi Jiang, Zhenlin Zhang, Hao Zhang

Propose: Osteogenesis imperfecta (OI) is a genetic connective tissue disorder primarily characterized by bone fragility. Hearing loss is an extra-skeletal manifestation of OI. This study aims to characterize the audiological findings of a sample of patients with OI in China.

Methods: A total of 76 patients (aged 4-77 years) were recruited and evaluated using audiometric evaluations, including pure tone audiometry (PTA), acoustic admittance measurements, and distortion-product otoacoustic emissions (DPOAEs). Patients were categorized into 2 age groups: adults (more than 18 years) and children (under 18 years), and classified by hearing type: normal hearing, conductive, sensorineural, or mixed hearing loss.

Results: Tympanometry (152 ears) showed 71.7% Type A tympanograms. There was a significant association between tympanogram type and hearing loss (p < 0.01). DPOAEs were predominantly abnormal in ears with sensorineural and mixed hearing loss (p < 0.01). PTA (146 ears) revealed hearing loss in 39.0%: 16.4% conductive, 15.1% sensorineural, and 7.5% mixed. Age correlated significantly with hearing loss type (p < 0.01). Adults' group exhibited higher pure-tone averages (11.3 dB vs. 8.8 dB, p < 0.01), average air-bone gap (2.5 dB vs.2.5 dB, p < 0.05), and bone conduction thresholds than children's group.

Conclusions: Hearing loss is common and progressive in Chinese OI patients, with a significant age-related increase in both the prevalence and severity of hearing loss. Establishing a hearing baseline and regular follow-up is essential for early intervention.

建议:成骨不全症(Osteogenesis imperfecta, OI)是一种以骨脆性为主要特征的遗传性结缔组织疾病。听力损失是成骨不全的骨骼外表现。本研究旨在描述中国成骨不全患者样本的听力学结果。方法:共招募76例患者(年龄4-77岁),采用纯音听力学(PTA)、声导纳测量和失真积耳声发射(dpoae)等听力测量方法进行评估。患者分为成人(18岁以上)和儿童(18岁以下)2个年龄组,按听力类型分为正常听力、传导性听力、感音神经性听力和混合性听力损失。结果:A型鼓室图占71.7%(152耳)。结论:听力损失在中国成骨不全患者中是常见且进行性的,听力损失的患病率和严重程度都与年龄相关。建立听力基线和定期随访对早期干预至关重要。
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引用次数: 0
Using T7 endonuclease I to detect SLC26A4 mutations in children with large vestibular aqueduct syndrome, with or without Mondini malformation and assess cochlear implant outcomes. 应用T7核酸内切酶I检测儿童大前庭导尿管综合征伴或不伴蒙迪尼畸形的SLC26A4突变并评估人工耳蜗的预后。
IF 2.2 3区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2025-09-01 Epub Date: 2025-04-07 DOI: 10.1007/s00405-025-09378-w
Yan Ding, Yong Zhang, Cheng Wen, Hua-Ping Xie, Bing-Lin Xie, Lihua Li, Weijing Wu, Ding-Hua Xie, Ruosha Lai

Objective: To analyze SLC26A4 gene mutations in children with large vestibular aqueduct syndrome (LVAS) with or without Mondini malformation, and to compare their hearing phenotypes, rehabilitation outcomes, and learning performance after cochlear implantation.

Methods: We used T7 Endonuclease I enzyme digestion to detect SLC26A4 mutations in 48 children with LVAS without Mondini malformation (EVA group), 29 children with LVAS and Mondini malformation (EVA + MD group). Negative results were confirmed by Sanger sequencing. Auditory performance (CAP) and speech intelligibility (SIR) scores assessed hearing and speech rehabilitation outcomes, while academic performance evaluated learning post-cochlear implantation.

Results: Electrophoresis showed that the positive detection rates of SLC26A4 mutations were 89.58% in the EVA group, 89.66% in the EVA + MD group, and 0% in the control group. In the EVA group, the most common mutations were in exons 7 + 8 (52.08%), 11 + 12 (22.92%), and 19 (18.75%). In the EVA + MD group, the predominant mutations were in exons 11 + 12 (51.72%), 4 (34.48%), 7 + 8 (27.59%), and 19 (24.14%). The EVA + MD group had higher detection rates for two-site (37.93%) and three-site compound heterozygous mutations (13.79%) compared to the EVA group (22.92% and 10.42%, respectively). The median diagnosis time for profound hearing loss was 6.62 months in the EVA + MD group versus 10.56 months in the EVA group. There were no significant differences in CAP and SIR scores between the groups, but the EVA group showed better learning performance.

Conclusion: This study reports, for the first time, multiple cases exhibiting a three-site compound heterozygous mutation in the SLC26A4 gene. The hotspot exons of the SLC26A4 gene differ between children with simple LVAS and those with LVAS accompanied by Mondini malformation. Children with both conditions show earlier onset of profound hearing loss and poorer learning performance compared to those with only LVAS.

目的:分析伴有或不伴有蒙迪尼畸形的大前庭导尿管综合征(LVAS)患儿SLC26A4基因突变,比较其人工耳蜗植入后的听力表型、康复效果和学习表现。方法:采用T7酶切I酶切法检测48例LVAS无Mondini畸形患儿(EVA组)和29例LVAS合并Mondini畸形患儿(EVA + MD组)的SLC26A4基因突变。Sanger测序证实阴性结果。听力表现(CAP)和言语清晰度(SIR)评分评估听力和语言康复效果,而学习成绩评估人工耳蜗植入后的学习效果。结果:电泳结果显示,EVA组SLC26A4突变阳性检出率为89.58%,EVA + MD组为89.66%,对照组为0%。EVA组最常见的突变位点为外显子7 + 8(52.08%)、11 + 12(22.92%)和19(18.75%)。在EVA + MD组中,显性突变位于外显子11 + 12(51.72%)、4(34.48%)、7 + 8(27.59%)和19(24.14%)。EVA + MD组两位点杂合突变检出率(37.93%)和三位点复合杂合突变检出率(13.79%)高于EVA组(22.92%和10.42%)。EVA + MD组深度听力损失的中位诊断时间为6.62个月,而EVA组为10.56个月。两组间CAP和SIR得分无显著差异,但EVA组表现出更好的学习表现。结论:本研究首次报道了SLC26A4基因出现三位点复合杂合突变的多例病例。SLC26A4基因的热点外显子在单纯LVAS患儿和LVAS合并Mondini畸形患儿中存在差异。与只有LVAS的儿童相比,患有这两种疾病的儿童表现出更早的重度听力损失和更差的学习表现。
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引用次数: 0
Ten years of stapes surgery in France (2013-2022). About 35368 procedures. 法国十年镫骨手术(2013-2022)。大约35368个程序。
IF 2.2 3区 医学 Q2 OTORHINOLARYNGOLOGY Pub Date : 2025-09-01 Epub Date: 2025-05-02 DOI: 10.1007/s00405-025-09399-5
Rémi Hervochon, Diane Picard, Yann Nguyen, Ghizlene Lahlou, Georges Lamas, Frédéric Tankere

Introduction: The incidence of otosclerosis appears to have been declining since the late 1970s. We conducted a retrospective nationwide study to assess the evolution of the annual number of stapes surgeries and to analyze the changes in technical practices, specifically comparing laser techniques to cold instruments.

Methods: We examined the French national dataset from the "Programme de Médicalisation des Systèmes d'Information" (PMSI). Within this national database, we collected data on the number of stapes surgical procedures performed using cold instruments and laser technology from 2013 to 2022 in France.

Results: The total number of stapes surgeries has decreased each year, dropping from 4,268 procedures in 2013 to 3,007 procedures in 2022, which represents a decline of 29.5% over a decade. On average, 162 fewer procedures were performed each year compared to the previous year. The use of laser technology increased from 42% of cases in 2013 to 61% of cases in 2022. Laser techniques were more commonly employed in university hospitals and private non-profit clinics than in general hospitals and private clinics.

Conclusion: The number of stapes surgeries is decreasing linearly and consistently, raising concerns regarding the training of young surgeons. Furthermore, laser techniques are gradually replacing cold instruments in clinical practice.

导读:自20世纪70年代末以来,耳硬化的发病率似乎一直在下降。我们进行了一项全国性的回顾性研究,以评估每年镫骨手术数量的演变,并分析技术实践的变化,特别是将激光技术与冷器械进行比较。方法:我们检查了法国国家数据集,这些数据集来自于“信息系统管理计划”(PMSI)。在这个国家数据库中,我们收集了2013年至2022年在法国使用冷仪器和激光技术进行镫骨手术的数量数据。结果:镫骨手术总数逐年下降,从2013年的4268例下降到2022年的3007例,十年间下降了29.5%。与前一年相比,每年平均减少162例手术。激光技术的使用从2013年的42%增加到2022年的61%。激光技术在大学医院和私人非营利性诊所比在综合医院和私人诊所更常用。结论:镫骨手术数量呈线性持续下降趋势,青年外科医生的培训问题日益突出。此外,在临床实践中,激光技术正逐渐取代冷仪器。
{"title":"Ten years of stapes surgery in France (2013-2022). About 35368 procedures.","authors":"Rémi Hervochon, Diane Picard, Yann Nguyen, Ghizlene Lahlou, Georges Lamas, Frédéric Tankere","doi":"10.1007/s00405-025-09399-5","DOIUrl":"10.1007/s00405-025-09399-5","url":null,"abstract":"<p><strong>Introduction: </strong>The incidence of otosclerosis appears to have been declining since the late 1970s. We conducted a retrospective nationwide study to assess the evolution of the annual number of stapes surgeries and to analyze the changes in technical practices, specifically comparing laser techniques to cold instruments.</p><p><strong>Methods: </strong>We examined the French national dataset from the \"Programme de Médicalisation des Systèmes d'Information\" (PMSI). Within this national database, we collected data on the number of stapes surgical procedures performed using cold instruments and laser technology from 2013 to 2022 in France.</p><p><strong>Results: </strong>The total number of stapes surgeries has decreased each year, dropping from 4,268 procedures in 2013 to 3,007 procedures in 2022, which represents a decline of 29.5% over a decade. On average, 162 fewer procedures were performed each year compared to the previous year. The use of laser technology increased from 42% of cases in 2013 to 61% of cases in 2022. Laser techniques were more commonly employed in university hospitals and private non-profit clinics than in general hospitals and private clinics.</p><p><strong>Conclusion: </strong>The number of stapes surgeries is decreasing linearly and consistently, raising concerns regarding the training of young surgeons. Furthermore, laser techniques are gradually replacing cold instruments in clinical practice.</p>","PeriodicalId":11952,"journal":{"name":"European Archives of Oto-Rhino-Laryngology","volume":" ","pages":"4615-4620"},"PeriodicalIF":2.2,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143978213","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
期刊
European Archives of Oto-Rhino-Laryngology
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