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The Role of Measuring Preoperative Social Maturation Score in Children With Additional Needs Who Underwent Cochlear Implantation 测量术前社交成熟度评分对接受人工耳蜗植入术的有额外需求儿童的作用
IF 1.1 Q3 OTORHINOLARYNGOLOGY Pub Date : 2024-04-10 DOI: 10.7874/jao.2024.00101
Ji Won Choi, Yun Ji Lee, W. Kang, J. Ahn, Hong Ju Park, Jong Woo Chung
Background and Objectives: Additional needs refer to specific requirements or support for individuals with disabilities or syndromes. Intellectual ability is a crucial outcome determinant of a cochlear implant. The social quotient (SQ) is an indirect predictor of intellectual capacity and social skills. This study aimed to investigate the clinical significance of the SQ on children with additional needs who received cochlear implants. Subjects andMethods: This study included 24 patients with diagnosed developmental delays and syndromes, who demonstrated SQ scores of <70. Preoperative social skills were evaluated using the SQ. All patients underwent cochlear implantation (CI) surgery before 7 years of age. Outcomes were evaluated using the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) and Categories of Auditory Performance (CAP) scores. Data were collected through a retrospective chart review.Results: Children were categorized into three groups based on their SQ. There were no correlations between the preoperative SQ and IT-MAIS or CAP scores at 2 and 5 years of follow-up postoperatively. The CI outcomes of children with low SQ (<70) differed from those with normal development (SQ>70). In the low-SQ group, inner ear anomalies were observed in 10 (41.7%) patients. Although not statistically significant, these children exhibited a trend of lower average outcomes than children without inner ear anomalies.Conclusions: CI outcomes in children with additional needs positively affected auditory performance. Postoperative auditory and language skills tended to improve slowly in children with additional needs and a lower SQ. Over time, development gradually became more comparable to the other groups of children. However, this improvement was less than that observed in children without additional needs. Our findings support CI for children with additional needs as part of long-term auditory rehabilitation following surgery.
背景和目标:额外需求是指对残疾或综合症患者的特殊要求或支持。智力是决定人工耳蜗植入效果的关键因素。社交商数(SQ)是智力和社交能力的间接预测指标。本研究旨在调查社交商数对接受人工耳蜗植入的有额外需求儿童的临床意义。研究对象和方法:本研究包括 24 名确诊为发育迟缓和综合症的患者,他们的 SQ 评分均为 70 分。)在低 SQ 组中,有 10 名患者(41.7%)出现内耳异常。虽然没有统计学意义,但与没有内耳异常的儿童相比,这些儿童的平均治疗效果呈下降趋势:结论:有额外需求的儿童的 CI 结果对听觉表现有积极影响。有额外需求且 SQ 较低的儿童的术后听觉和语言技能往往改善缓慢。随着时间的推移,他们的发展逐渐与其他组别的儿童相当。但是,这种改善程度低于无额外需求儿童。我们的研究结果支持对有额外需求的儿童进行 CI,作为术后长期听觉康复的一部分。
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引用次数: 0
Application of Genetic Information to Cochlear Implantation in Clinical Practice 遗传信息在人工耳蜗植入临床实践中的应用
IF 1.1 Q3 OTORHINOLARYNGOLOGY Pub Date : 2024-04-10 DOI: 10.7874/jao.2024.00080
Chen-Chi Wu
Cochlear implantation is currently the treatment of choice for children with severe-to-profound sensorineural hearing impairment (SNHI). However, the outcomes with cochlear implant (CI) vary significantly among recipients. Genetic diagnosis offers direct clues regarding the pathogenesis of SNHI, which facilitates the development of personalized medicine for potential candidates for CI. In this article, I present a comprehensive overview of the usefulness of genetic information in clinical decision-making for CI. Genetically confirmed diagnosis enables clinicians to: 1) monitor the evolution of SNHI and determine the optimal surgical timing, 2) predict the potential benefits of CI in patients with identified genetic etiology, and 3) select CI devices/electrodes tailored to patients with specific genetic mutations.
人工耳蜗植入术是目前治疗重度至永久性感音神经性听力损伤(SNHI)儿童的首选方法。然而,人工耳蜗植入术(CI)的效果在不同受术者之间存在很大差异。基因诊断为感音神经性听力障碍(SNHI)的发病机制提供了直接线索,有助于为潜在的 CI 候选者开发个性化药物。在本文中,我将全面概述基因信息在 CI 临床决策中的作用。通过基因确诊,临床医生可以1)监测 SNHI 的演变并确定最佳手术时机;2)预测 CI 对已确定遗传病因的患者的潜在益处;3)为具有特定基因突变的患者选择量身定制的 CI 设备/电极。
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引用次数: 0
Updates on Genetic Hearing Loss: From Diagnosis to Targeted Therapies 遗传性听力损失的最新进展:从诊断到靶向治疗
IF 1.1 Q3 OTORHINOLARYNGOLOGY Pub Date : 2024-04-10 DOI: 10.7874/jao.2024.00157
Y. Yun, Sang-Yeon Lee
Sensorineural hearing loss (SNHL) is the most common sensory disorder, with a high Mendelian genetic contribution. Considering the genotypic and phenotypic heterogeneity of SNHL, the advent of next-generation sequencing technologies has revolutionized knowledge on its genomic architecture. Nonetheless, the conventional application of panel and exome sequencing in real-world practice is being challenged by the emerging need to explore the diagnostic capability of whole-genome sequencing, which enables the detection of both noncoding and structural variations. Small molecules and gene therapies represent good examples of how breakthroughs in genetic understanding can be translated into targeted therapies for SNHL. For example, targeted small molecules have been used to ameliorate autoinflammatory hearing loss caused by gain-of-function variants of NLRP3 and inner ear proteinopathy with OSBPL2 variants underlying dysfunctional autophagy. Strikingly, the successful outcomes of the first-in-human trial of OTOF gene therapy highlighted its potential in the treatment of various forms of genetic hearing loss. clustered regularly interspaced short palindromic repeats (CRISPR)-based technologies are currently being developed for site-specific genome editing to treat human genetic disorders. These advancements have led to an era of genotype- and mechanism-based precision medicine in SNHL practice.
感音神经性听力损失(SNHL)是最常见的感官疾病,具有很高的孟德尔遗传率。考虑到感音神经性听力损失的基因型和表型异质性,新一代测序技术的出现彻底改变了人们对其基因组结构的认识。然而,在现实世界中,传统的面板测序和外显子组测序正受到挑战,因为人们需要探索全基因组测序的诊断能力,因为全基因组测序可以检测非编码变异和结构变异。小分子药物和基因疗法是遗传学方面的突破性进展如何转化为 SNHL 靶向疗法的良好范例。例如,靶向小分子药物已被用于改善由 NLRP3 功能增益变异引起的自身炎症性听力损失,以及由 OSBPL2 变异引起的内耳蛋白病,其根本原因是自噬功能障碍。引人注目的是,OTOF 基因疗法首次人体试验的成功结果突显了它在治疗各种形式的遗传性听力损失方面的潜力。目前正在开发基于聚类规则间隔短回文重复序列(CRISPR)的技术,用于治疗人类遗传疾病的特定位点基因组编辑。这些技术的进步使基于基因型和机理的精准医疗时代在遗传性听力损失的实践中到来。
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引用次数: 0
In Memory of Professor Seung Ha Oh and Highlights From the 14th Asia Pacific Symposium on Cochlear Implant and Related Sciences (APSCI), Seoul, Korea 纪念 Seung Ha Oh 教授和第 14 届亚太人工耳蜗及相关科学研讨会(APSCI)花絮,韩国首尔
IF 1.1 Q3 OTORHINOLARYNGOLOGY Pub Date : 2024-04-10 DOI: 10.7874/jao.2024.00283
Jun Ho Lee
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引用次数: 0
Comparison of Two Clinical Devices for the Measurement of Distortion Product Otoacoustic Emissions in Normal-Hearing Adults. 两种用于测量正常听力成年人失真产物耳声发射的临床设备的比较。
IF 1.1 Q3 OTORHINOLARYNGOLOGY Pub Date : 2024-04-01 Epub Date: 2024-02-22 DOI: 10.7874/jao.2023.00269
Oscar M Cañete, Mouhamad El-Haj-Ali, Michal Fereczkowski

Background and objectives: Otoacoustic emissions (OAEs) are low-intensity sounds generated by the cochlea and associated with the function of the outer hair cells. Since OAE measurements do not require active participation of a listener, OAEs are considered an objective measure of cochlear function. While distortion-product OAEs (DPOAEs) are commonly used in clinical practice, limited information is available on the performance of various clinical devices. This study compared two commercial clinical devices, Titan and Eclipse from Interacoustics, and collected normative data for DPOAEs in people with normal hearing. The data collection and analysis were focused on signal and noise amplitudes as well as signal-to-noise ratios (SNRs).

Subjects and methods: Sixty-three participants with normal hearing (age 23.2±2.0 years) were included in the study. DPOAEs were measured at 31 frequencies ranging from 500 to 10,000 Hz.

Results: DPOAE amplitude differed across frequencies. Additionally, a high number of unreliable responses were observed at the edge frequencies (<828 Hz and >6,072 Hz). There were no significant differences between the two devices in terms of DPOAE amplitudes, but the recorded-noise levels (amplitude) differed significantly. SNRs of the recorded OAEs were frequency dependent (higher frequencies showed larger SNRs), and significant differences in terms of SNR were found between the two devices.

Conclusions: Despite the above-mentioned differences, the OAEs recorded with both devices met the pass criteria for the SNR (≥6 dB) consistently across frequencies, and thus the differences do not compromise the test outcomes. The frequency dependence of the OAE amplitudes and the corresponding SNRs may be relevant for clinical practice.

背景和目的:耳声发射(OAE)是耳蜗产生的低强度声音,与外毛细胞的功能有关。由于 OAE 测量不需要听者的积极参与,因此 OAE 被认为是衡量耳蜗功能的客观指标。虽然畸变产物 OAE(DPOAE)在临床实践中得到了普遍应用,但有关各种临床设备性能的信息却很有限。本研究比较了 Interacoustics 的 Titan 和 Eclipse 两款商用临床设备,并收集了听力正常者的 DPOAEs 标准数据。数据收集和分析的重点是信号和噪声幅度以及信噪比(SNR):研究对象和方法:63 名听力正常的参与者(年龄为 23.2±2.0 岁)。在 500 至 10,000 Hz 的 31 个频率范围内测量了 DPOAE:结果:不同频率的 DPOAE 振幅不同。此外,在边缘频率(6,072 Hz)处观察到大量不可靠的反应。两种设备在 DPOAE 振幅方面没有明显差异,但记录的噪声水平(振幅)却有很大不同。记录的 OAE 的信噪比与频率有关(频率越高,信噪比越大),两种设备在信噪比方面存在显著差异:结论:尽管存在上述差异,但使用两种设备记录的 OAEs 在不同频率下均符合信噪比合格标准(≥6 dB),因此这些差异不会影响测试结果。OAE 振幅和相应信噪比的频率依赖性可能与临床实践有关。
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引用次数: 0
Effect of Vestibular Rehabilitation on Sleep Quality and Depression in the Elderly With Chronic Dizziness: A Prospective Study. 前庭康复对慢性头晕老年人睡眠质量和抑郁的影响:前瞻性研究。
IF 1.1 Q2 Health Professions Pub Date : 2024-04-01 Epub Date: 2023-12-07 DOI: 10.7874/jao.2023.00171
Zahra Hosseini Dastgerdi, Nasrin Gohari, Mobina Mehrabifard, Hasti Seifi, Bahare Khavarghazalani

Background and objectives: Dizziness and the accompanying complaints, including sleep disorders and depression, are common among the elderly. This study investigated the effect of vestibular rehabilitation on complaints of dizziness, sleep problems, and the severity of depression in older people with chronic dizziness.

Subjects and methods: The study included 25 participants with chronic dizziness accompanied by comorbid sleep disturbance (Pittsburgh Sleep Quality Index [PSQI] global score >5). Participants completed the Dizziness Handicap Inventory (three sub-scales: physical, emotional, and functional), the PSQI, and the Persian version of the shortened Beck Depression Inventory (BDI-13) before and after the vestibular rehabilitation.

Results: The findings showed that the handicap caused by dizziness, the severity of depression, and the quality of sleep in the study participants improved significantly after the intervention (p<0.05).

Conclusions: Vestibular rehabilitation is an effective intervention to reduce dizziness handicap, comorbid sleep disturbance, and depression.

背景和目的: :头晕及其伴随的主诉,包括睡眠障碍和抑郁,在老年人中很常见。本研究调查了前庭康复对患有慢性头晕的老年人的头晕主诉、睡眠问题和抑郁严重程度的影响:研究包括25名患有慢性头晕并伴有睡眠障碍(匹兹堡睡眠质量指数[PSQI]总分大于5分)的参与者。参与者在前庭康复治疗前后填写了头晕障碍量表(三个分量表:身体、情绪和功能)、匹兹堡睡眠质量指数和波斯语版贝克抑郁简易量表(BDI-13):结果:研究结果表明,干预后,研究对象因头晕造成的障碍、抑郁的严重程度和睡眠质量都有了明显改善(p 结论:前庭康复治疗是一种有效的治疗方法:前庭康复是减少头晕障碍、合并睡眠障碍和抑郁的有效干预措施。
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引用次数: 0
Comparison of Speech Perception Performance According to Prosody Change Between People With Normal Hearing and Cochlear Implant Users. 根据前奏变化比较正常听力者与人工耳蜗使用者的语音感知能力
IF 1.1 Q2 Health Professions Pub Date : 2024-04-01 Epub Date: 2023-12-07 DOI: 10.7874/jao.2023.00234
Eun Yeon Kim, Hye Yoon Seol

Background and objectives: Cochlear implants (CIs) are well known to improve audibility and speech recognition in individuals with hearing loss, but some individuals still struggle with many aspects in communication, such as prosody. This study explores how prosodic elements are perceived by those with normal hearing (NH) and CIs.

Subjects and methods: Thirteen individuals with NH and thirteen CI users participated in this study and completed speech perception, speech prosody perception, speech prosody production, pitch difference discrimination, and melodic contour perception testing.

Results: NH listeners performed significantly better than CI users on speech perception, speech prosody perception (except for words with neutral meaning and a negative prosody change and when words were repeated twice), pitch difference discrimination, and melodic contour perception testing. No statistical significance was observed for speech prosody production for both groups.

Conclusions: Compared to NH listeners, CI users had limited ability to recognize prosodic elements. The study findings highlight the necessity of an assessment tool and signal processing algorithm for CIs, specifically targeting prosodic elements in clinical settings.

背景和目的:众所周知,人工耳蜗(CI)可提高听力损失患者的听力和语音识别能力,但有些患者在交流中仍有许多困难,如模仿发音:众所周知,人工耳蜗(CI)可提高听力损失者的听力和语音识别能力,但有些人在交流的许多方面仍有困难,例如前音。本研究探讨了听力正常(NH)和 CI 患者如何感知前音元素:13名听力正常者和13名CI使用者参与了本研究,并完成了言语感知、言语前奏感知、言语前奏制作、音高差异辨别和旋律轮廓感知测试:在言语感知、言语前音感知(中性含义和负面前音变化的词语以及重复两次的词语除外)、音高差异辨别和旋律轮廓感知测试中,NH 听者的表现明显优于 CI 使用者。两组听者在语音拟声方面均无统计学意义:与正常听者相比,CI 使用者识别前奏元素的能力有限。研究结果突出表明,有必要为 CI 设计一种评估工具和信号处理算法,特别是针对临床环境中的前音元素。
{"title":"Comparison of Speech Perception Performance According to Prosody Change Between People With Normal Hearing and Cochlear Implant Users.","authors":"Eun Yeon Kim, Hye Yoon Seol","doi":"10.7874/jao.2023.00234","DOIUrl":"10.7874/jao.2023.00234","url":null,"abstract":"<p><strong>Background and objectives: </strong>Cochlear implants (CIs) are well known to improve audibility and speech recognition in individuals with hearing loss, but some individuals still struggle with many aspects in communication, such as prosody. This study explores how prosodic elements are perceived by those with normal hearing (NH) and CIs.</p><p><strong>Subjects and methods: </strong>Thirteen individuals with NH and thirteen CI users participated in this study and completed speech perception, speech prosody perception, speech prosody production, pitch difference discrimination, and melodic contour perception testing.</p><p><strong>Results: </strong>NH listeners performed significantly better than CI users on speech perception, speech prosody perception (except for words with neutral meaning and a negative prosody change and when words were repeated twice), pitch difference discrimination, and melodic contour perception testing. No statistical significance was observed for speech prosody production for both groups.</p><p><strong>Conclusions: </strong>Compared to NH listeners, CI users had limited ability to recognize prosodic elements. The study findings highlight the necessity of an assessment tool and signal processing algorithm for CIs, specifically targeting prosodic elements in clinical settings.</p>","PeriodicalId":44886,"journal":{"name":"Journal of Audiology and Otology","volume":" ","pages":"119-125"},"PeriodicalIF":1.1,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11065548/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138488678","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Relationship Between Chloroquine or Hydroxychloroquine Use and Hearing Disorders: A Systematic Review and Meta-Analysis. 使用氯喹或羟氯喹与听力障碍之间的关系:系统回顾与元分析》。
IF 1.1 Q2 Health Professions Pub Date : 2024-04-01 Epub Date: 2024-02-22 DOI: 10.7874/jao.2023.00157
Maria Renata José, Jéssica da Silva Ortega, Jordana Batista Correia Baran, Débora Lüders, Claudia Giglio de Oliveira Gonçalves, Bianca Simone Zeigelboim, Karinna Veríssimo Meira Taveira, José Fernando Polanski, Rosane Sampaio Santos, Camila de Castro Corrêa, Cristiano Miranda de Araujo

Background and objectives: Chloroquine and its analog hydroxychloroquine are derivatives of 4-aminoquinoline and are regularly used in the treatment of malaria and autoimmune diseases. Among the side effects of these drugs, alterations associated with the auditory system are frequently mentioned. Thus, the aim of this systematic review is to systematically review publications on hearing disorders and chloroquine or hydroxychloroquine use.

Materials and methods: Inclusion criteria were observational or interventional studies on audiological assessment in participants who were using chloroquine or hydroxychloroquine. The methodological quality was independently assessed by two reviewers using the Meta-Analysis of Statistics: assessment and review Instrument. The certainty of the evidence was assessed using the GRADE tool.

Results: A total of 1,372 non-duplicate papers were screened, out of which 17 were included in the final qualitative synthesis, and 5 studies in the meta-analysis. The odds ratio for the two subgroups evaluated did not show significance with no heterogeneity between the effects observed between the different diseases (I2=0%) and obtaining the global estimate of 0.76 (95% confidence interval [CI]=0.41-1.39; p>0.05). Despite the inclusion of papers with different disease samples, the heterogeneity observed in the analysis was low (I2= 0%) and prediction interval (95% PI=0.32-1.80; p>0.05) remained close to that estimated by the CI (95% CI=0.41-1.39; p>0.05). The certainty of the evidence assessed by the GRADE tool was considered very low due to the risk of bias, indirect evidence, and imprecision.

Conclusions: The findings of this study suggest that the use of chloroquine/hydroxychloroquine is not associated with hearing disorders.

背景和目的:氯喹及其类似物羟氯喹是 4-氨基喹啉的衍生物,经常用于治疗疟疾和自身免疫性疾病。在这些药物的副作用中,与听觉系统相关的改变经常被提及。因此,本系统综述旨在系统回顾有关听力障碍和氯喹或羟氯喹使用的出版物:纳入标准是对使用氯喹或羟氯喹的参与者进行听力评估的观察性或干预性研究。方法学质量由两名审稿人使用 "统计元分析:评估和审查工具 "进行独立评估。证据的确定性采用 GRADE 工具进行评估:共筛选出 1,372 篇非重复论文,其中 17 篇纳入最终定性综合,5 篇纳入荟萃分析。所评估的两个亚组的几率比并没有显示出显著性,不同疾病之间所观察到的效果也没有异质性(I2=0%),总体估计值为 0.76(95% 置信区间 [CI]=0.41-1.39; p>0.05)。尽管纳入了不同疾病样本的论文,但分析中观察到的异质性很低(I2= 0%),预测区间(95% PI=0.32-1.80;P>0.05)仍然接近 CI(95% CI=0.41-1.39;P>0.05)的估计值。由于存在偏倚风险、间接证据和不精确性,GRADE 工具评估的证据确定性很低:本研究结果表明,使用氯喹/羟氯喹与听力障碍无关。
{"title":"Relationship Between Chloroquine or Hydroxychloroquine Use and Hearing Disorders: A Systematic Review and Meta-Analysis.","authors":"Maria Renata José, Jéssica da Silva Ortega, Jordana Batista Correia Baran, Débora Lüders, Claudia Giglio de Oliveira Gonçalves, Bianca Simone Zeigelboim, Karinna Veríssimo Meira Taveira, José Fernando Polanski, Rosane Sampaio Santos, Camila de Castro Corrêa, Cristiano Miranda de Araujo","doi":"10.7874/jao.2023.00157","DOIUrl":"10.7874/jao.2023.00157","url":null,"abstract":"<p><strong>Background and objectives: </strong>Chloroquine and its analog hydroxychloroquine are derivatives of 4-aminoquinoline and are regularly used in the treatment of malaria and autoimmune diseases. Among the side effects of these drugs, alterations associated with the auditory system are frequently mentioned. Thus, the aim of this systematic review is to systematically review publications on hearing disorders and chloroquine or hydroxychloroquine use.</p><p><strong>Materials and methods: </strong>Inclusion criteria were observational or interventional studies on audiological assessment in participants who were using chloroquine or hydroxychloroquine. The methodological quality was independently assessed by two reviewers using the Meta-Analysis of Statistics: assessment and review Instrument. The certainty of the evidence was assessed using the GRADE tool.</p><p><strong>Results: </strong>A total of 1,372 non-duplicate papers were screened, out of which 17 were included in the final qualitative synthesis, and 5 studies in the meta-analysis. The odds ratio for the two subgroups evaluated did not show significance with no heterogeneity between the effects observed between the different diseases (I2=0%) and obtaining the global estimate of 0.76 (95% confidence interval [CI]=0.41-1.39; p>0.05). Despite the inclusion of papers with different disease samples, the heterogeneity observed in the analysis was low (I2= 0%) and prediction interval (95% PI=0.32-1.80; p>0.05) remained close to that estimated by the CI (95% CI=0.41-1.39; p>0.05). The certainty of the evidence assessed by the GRADE tool was considered very low due to the risk of bias, indirect evidence, and imprecision.</p><p><strong>Conclusions: </strong>The findings of this study suggest that the use of chloroquine/hydroxychloroquine is not associated with hearing disorders.</p>","PeriodicalId":44886,"journal":{"name":"Journal of Audiology and Otology","volume":" ","pages":"126-145"},"PeriodicalIF":1.1,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11065553/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139933456","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Translabyrinthine Excision of a Transmodiolar Intralabyrinthine Schwannoma Mimicking Meniere's Disease: A Case Report. 模仿美尼尔氏病的经迷路迷路内许旺瘤切除术:病例报告。
IF 1.1 Q3 OTORHINOLARYNGOLOGY Pub Date : 2024-04-01 Epub Date: 2024-02-22 DOI: 10.7874/jao.2023.00304
Sunil Goyal, Mahesh Ravunnikutty, Himanshu Swami, Sneha Yadav

Intralabyrinthine schwannomas (ILSs) are rare tumors involving the otic capsule. Notably, they are often misdiagnosed because their symptoms mimic those of other, more common inner ear pathologies. Diagnosis requires high-resolution contrast-enhanced magnetic resonance imaging (MRI), which reveals filling defects (using a T2-weighted MRI sequence) or focal enhancement (using a T1-weighted MRI sequence with gadolinium enhancement) in the inner ear. A 52-year-old male patient with intractable vertigo or single-sided deafness should raise suspicion of this clinical entity as a differential diagnosis. Translabyrinthine excision of the tumor along with auditory rehabilitation using a cochlear implant can provide good outcomes with minimal morbidity in carefully selected cases. Here, we present an interesting case of a transmodiolar ILS mimicking Meniere's disease, wherein surgery using the translabyrinthine approach and an extended cochleostomy yielded favorable outcomes.

迷走神经内分裂瘤(ILSs)是一种罕见的累及耳廓的肿瘤。值得注意的是,它们经常被误诊,因为其症状与其他更常见的内耳病变相似。诊断需要高分辨率对比增强磁共振成像(MRI),它能显示内耳充盈缺损(使用 T2 加权磁共振成像序列)或局灶增强(使用钆增强的 T1 加权磁共振成像序列)。一名 52 岁的男性患者患有顽固性眩晕或单侧耳聋,应将这种临床实体病作为鉴别诊断加以怀疑。经迷宫切除肿瘤,同时使用人工耳蜗进行听觉康复治疗,可为精心挑选的病例提供良好的治疗效果,且发病率极低。在此,我们介绍了一例有趣的模仿梅尼埃病的经迷路 ILS 病例,该病例采用迷走神经切断术和扩大耳蜗造口术,取得了良好的疗效。
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引用次数: 0
Can Cochlear Nerve Size Assessment With Magnetic Resonance Enhance the Understanding of Idiopathic Sudden Sensorineural Hearing Loss? 磁共振耳蜗神经大小评估能提高对特发性突发性感觉神经性听力损失的认识吗?
IF 1.1 Q3 OTORHINOLARYNGOLOGY Pub Date : 2024-01-01 Epub Date: 2023-10-20 DOI: 10.7874/jao.2023.00164
Hande Arslan, Meltem Özdemir, Rasime Pelin Kavak, Kemal Keseroğlu, Murad Mutlu, Mehmet Hakan Korkmaz

Background and objectives: Idiopathic sudden sensorineural hearing loss (ISSHL) is a rapid loss of hearing, exceeding 30 dB in at least 3 consecutive frequencies within 3 days, without any identifiable cause despite thorough investigations. Currently, the etiology and pathogenesis of ISSHL have not been fully elucidated. This study aimed to assess the size of the cochlear nerve in patients with ISSHL and explore its relationship with pretreatment audiograms and treatment response. Subjects and.

Methods: A total of 125 patients (59 [47.2%] women; mean age 47.7±13.8 years [minimum-maximum: 21-76]) and 60 healthy participants (27 [45%] women; mean age 45.7±16.8 years [minimum-maximum: 20-76]) as a control group were included in this study. The size of the cochlear nerve was assessed on the affected side, compared to the control group, as well as on the unaffected side. Pretreatment and posttreatment audiological values were also analyzed.

Results: The cross-sectional area (CSA), vertical diameter (VD), and horizontal diameter (HD) of the CN were found to be smaller on the affected side of ISSHL patients compared to the control group (p<0.01; p=0.04; p=0.02, respectively). In the study group (affected side of ISSHL patients), there were no significant differences in VD, HD, and CSA values between pretreatment audiogram types (p=0.23; p=0.53; p=0.39, respectively), and initial hearing levels (p=0.16; p=0.22; p=0.23, respectively). Furthermore, there were no significant differences in VD, HD, and CSA values between the recovery groups according to Furuhashi criteria (p=0.18; p=0.37; p=0.27, respectively).

Conclusions: The size of the CN may be a risk factor for ISSHL, but it does not affect the type of audiogram curves and was not prognostic in terms of treatment response.

背景和目的:特发性突发性感音神经性听力损失(ISSHL)是一种快速的听力损失,在3天内至少连续3个频率超过30dB,尽管进行了彻底的调查,但没有任何可确定的原因。目前,ISSHL的病因和发病机制尚未完全阐明。本研究旨在评估ISSHL患者耳蜗神经的大小,并探讨其与预处理听力图和治疗反应的关系。受试者和方法:本研究共纳入125名患者(59名[47.2%]女性;平均年龄47.7±13.8岁[最小-最大值:21-76])和60名健康参与者(27名[45%]女性,平均年龄45.7±16.8岁[最大-最小值:20-76])作为对照组。与对照组和未受影响侧相比,评估了受影响侧的耳蜗神经大小。还分析了治疗前和治疗后的听力学值。结果:与对照组相比,ISSHL患者患侧CN的横截面积(CSA)、垂直直径(VD)和水平直径(HD)较小(P结论:CN的大小可能是ISSHL的一个危险因素,但它不会影响听力图曲线的类型,也不是治疗反应的预后因素。
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引用次数: 0
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Journal of Audiology and Otology
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