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Bilateral squamous cell carcinoma of the temporal bone: A report of two cases and a systematic review of the literature 双侧颞骨鳞状细胞癌:两例报告及文献系统复习。
IF 1.4 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-10-01 DOI: 10.1016/j.joto.2024.07.006
N. Hoogenes , M. Tomasoni , P. Merkus , B.I. Lissenberg-Witte , C.R. Leemans , A. Deganello , C.F. Smit

Objective

two new cases of temporal bone squamous cell carcinoma (TBSCC) with a bilateral occurrence are presented. Furthermore, a review of the literature was performed and the yearly incidence was calculated.

Methods

A systematic review of the literature was conducted using PRISMA guidelines.

Results

Twenty-two more cases were found in literature. With a total of 24 cases, the calculated yearly incidence of bilateral TBSCC is 49:10^12 A history of chronic otitis or regional radiotherapy was found in respectively 50% and 12% of patients. In nine patients, the tumors developed synchronously (within 6 months) and in 13 metachronously.

Conclusions

The calculated incidence is 89 times higher than mathematically expected considering the incidence of unilateral cases. An explanation might be a history of chronic otitis or prior radiotherapy. The tumor staging of both the first tumor group and the contralateral tumor group are similar to unilateral temporal bone squamous cell carcinoma series.
目的:报告两例新发双侧颞骨鳞状细胞癌(TBSCC)。此外,回顾了文献并计算了年发病率。方法:采用PRISMA指南对文献进行系统回顾。结果:文献共发现22例。共24例,计算双侧TBSCC年发病率为49:10^12,分别有50%和12%的患者有慢性中耳炎或局部放疗史。在9例患者中,肿瘤同步发生(6个月内),13例异时发生。结论:考虑到单侧病例的发生率,计算出的发生率比数学上预计的高89倍。一种解释可能是慢性中耳炎病史或既往放疗。第一肿瘤组和对侧肿瘤组的肿瘤分期与单侧颞骨鳞状细胞癌系列相似。
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引用次数: 0
Effect analysis of unilateral deaf adults wearing bone-conduction hearing aids: A case report 成人单侧耳聋配戴骨传导助听器1例效果分析。
IF 1.4 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-10-01 DOI: 10.1016/j.joto.2024.07.009
Chenchen Xiang , Yi Wang , Xiaozhu Cao , Weiman Xv , Jing Liu , Nan Li , Lumeng Han , QIan Wang , Fei Ji
The prevalence of unilateral deafness (SSD) or asymmetric hearing loss (AHL) among patients with hearing impairments ranges from 7.2% to 15.0%, indicating a relatively significant proportion. However, these individuals often overlook their hearing loss, resulting in delayed or inadequate treatment. This oversight can lead to a lack of binaural summation and squelch effect, as well as the head shadow effect, which can significantly impact their speech recognition and sound localization abilities, especially in noisy environments.Recently, a groundbreaking Sound Bite™ Pinyin® Bone Conduction Hearing Aid (HA) device has been has been introduced as a viable alternative to traditional percutaneous stimulation hearing assistance devices. This innovative device harnesses bone conduction technology to convey sound vibrations directly to the inner ear via the bones of the jaw and skull, effectively bypassing the air conduction pathway that is commonly compromised in individuals with hearing loss.
This report details the evaluation and adjustment process of a HA device worn by a 31-year-old female patient who suffers from a congenital ossicular chain deformity in her right ear, while maintaining normal hearing in her left ear.The report comprehensively covers the hearing thresholds of the 31-year-old female patient on the day of fitting the hearing aid and one month later. It also presents assessments of the hearing aid's performance through the Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire, conducted three days after adjustment, one month later, and one year later.Furthermore, the report details the evaluation of the patient's sound localization ability, comparing her performance before and after wearing the hearing aid device. Additionally, it includes measurements of her speech recognition ability for monosyllabic words and Yang Yang Ge words, which are specific tests in the Chinese language, conducted two years after the initial fitting of the hearing aid.The discovery that bone-anchored hearing devices can significantly enhance hearing thresholds in patients with unilateral conductive hearing loss represents a significant milestone. These devices not only improve speech recognition ability but also enhance sound localization in noisy environments. This improvement is accompanied by a high level of subjective satisfaction among patients, indicating a positive impact on their overall quality of life.
听力障碍患者中单侧耳聋(SSD)或不对称听力损失(AHL)的患病率为7.2% ~ 15.0%,占比较显著。然而,这些人往往忽视他们的听力损失,导致延迟或不充分的治疗。这种疏忽可能导致双耳求和和静音效果的缺乏,以及头部阴影效应,这可能会严重影响他们的语音识别和声音定位能力,特别是在嘈杂的环境中。最近,突破性的Sound Bite™拼音®骨传导助听器(HA)设备已经推出,作为传统经皮刺激助听器的可行替代方案。这种创新的设备利用骨传导技术,通过颌骨和颅骨将声音振动直接传递到内耳,有效地绕过了听力损失患者通常受损的空气传导途径。本报告详细介绍了一名患有先天性右耳听骨链畸形的31岁女性患者在保持左耳正常听力的情况下佩戴HA装置的评估和调整过程。该报告全面涵盖了该31岁女性患者配戴助听器当日及一个月后的听力阈值。它还通过助听器效益简要介绍(APHAB)问卷对助听器的性能进行评估,在调整后三天,一个月和一年后进行。此外,该报告还详细评估了患者的声音定位能力,比较了患者佩戴助听器前后的表现。此外,它还包括测量她对单音节单词和洋洋歌单词的语音识别能力,这是在初次安装助听器两年后进行的汉语特定测试。骨锚定助听器的发现可以显著提高单侧传导性听力损失患者的听力阈值,这是一个重要的里程碑。这些设备不仅提高了语音识别能力,还增强了嘈杂环境下的声音定位能力。这种改善伴随着患者的高水平主观满意度,表明对他们的整体生活质量有积极的影响。
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引用次数: 0
Analysis and improving countermeasures of medical disputes from the perspective of legal changes 法律变迁视角下的医疗纠纷分析及完善对策
IF 1.4 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-10-01 DOI: 10.1016/j.joto.2024.05.004
Zhang Linlin , Zhang Shimeng
Under the background of medical disputes growing in number, scale and intensity, tracing back legal changes in medical field as a breakthrough point, this paper took a legal perspective to illustrate changes in medical dispute settlements from legislative orientation to legal system improvement. In view of the fact that early legislation in medical field was biased towards identification and punishment of doctors’ responsibility, and later intensive legislation in balancing increasing "medical trouble" phenomenon with limited effects and difficulties to abide by the law, this paper proposed to improve doctor-patient dispute settlements system in China referencing from foreign law experience, to reduce investigation of doctors at the judicial level, and to establish a settlement mechanism on doctors' apology at the legislative level, so as to promote a healthy development of doctor-patient relationship.
在医疗纠纷数量、规模和强度不断增加的背景下,本文以追溯医疗领域的法律变迁为切入点,从法律视角来阐述医疗纠纷解决从立法导向到法制完善的变化。针对早期医疗领域的立法偏向于认定和处罚医生的责任,后来在平衡日益增多的“医患纠纷”现象与效果有限、难以遵纪守法之间的强化立法,本文借鉴国外法律经验,提出完善中国医患纠纷解决制度,减少对医生的司法追究。并在立法层面建立医生道歉的解决机制,促进医患关系的健康发展。
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引用次数: 0
Lessons learned in delayed identification of a misplaced electrode array in the vestibule 前厅错位电极阵列延迟识别的经验教训。
IF 1.4 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-10-01 DOI: 10.1016/j.joto.2024.03.003
Huey Ting Diong , Yuhan Wong , Shermaine Png , Yeow Seng Raymond Ngo
To report a case of cochlear implantation with a misplaced electrode array in the vestibule and the causes for the delay in identification. A 23-year-old male with left single-sided deafness underwent cochlear implantation. The intraoperative assessment did not reveal any major red flags of electrode array misplacement. He did not display any vestibular symptoms postoperatively but showed poor speech performance, even though the aided tone audiometry revealed good sound detection thresholds. High-resolution computed tomography (HRCT) showed that the entire perimodiolar electrode array was situated within the vestibule, and a revision surgery was conducted. Retrospective analysis of the neural response telemetry (NRT) revealed subtle differences in responses between the misplaced and correctly placed electrode arrays. Unlike previously reported cases, the patient did not display vestibular symptoms despite the misplacement of the electrode in the vestibule due to existing weakness in otolithic function. Further investigation is warranted when a motivated patient with normal inner ear anatomy does not show benefit with the cochlear implant post-operatively.
报告1例耳蜗前庭电极排列错位的人工耳蜗植入术,并分析其诊断延误的原因。一位23岁的左单侧耳聋男性接受了人工耳蜗植入手术。术中评估未发现任何电极阵列错位的主要危险信号。他术后未出现任何前庭症状,但言语表现不佳,尽管辅助音调测听显示良好的声音检测阈值。高分辨率计算机断层扫描(HRCT)显示整个臼齿周围电极阵列位于前庭内,并进行了翻修手术。神经反应遥测(NRT)的回顾性分析揭示了错误放置和正确放置电极阵列之间的细微差异。与先前报道的病例不同,尽管由于耳石功能薄弱而将电极放置在前庭,但该患者并未表现出前庭症状。当一个内耳解剖正常的患者术后没有表现出人工耳蜗植入的益处时,进一步的研究是有必要的。
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引用次数: 0
Exploration of preschool hearing screening at primary healthcare centers in Riyadh, Saudi Arabia: A cross-sectional study 沙特阿拉伯利雅得初级卫生保健中心学龄前听力筛查的探索:一项横断面研究。
IF 1.4 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-10-01 DOI: 10.1016/j.joto.2024.10.001
Ahmad A. Alanazi , Rayan A. Almutairi , Khalid S. Alsuhaibani , Yazzan Alruwaily , Hellal A. Alqudiey , Mohammed A. Alabbas , Shuaa R. Alanazi
The hearing status of children should be examined throughout early childhood, even if they have passed the newborn hearing loss because hearing loss can occur at any time and may affect their ability to learn. Preschool hearing screening (PHS) is vital to continue screening throughout early childhood. The current practice of PHS in the primary healthcare centers (PHCs) in Saudi Arabia is unknown. The purpose of this study was to investigate PHS in the PHCs. This cross-sectional descriptive study used an in-person-administered questionnaire to collect data. A total of 106 out of 120 participants (male = 61; female = 45) representing the PHCs in Riyadh were interviewed. Most of the participants were aged 31–40 years and held a bachelor's degree as the highest academic qualification with limited years of experience. PHS was mostly performed through subjective measures by asking the parents (71.7%) and the child (65.1%). The audiometric evaluation was conducted for preschool children in only half of the PHCs. General practitioners and nurses usually perform PHS. Substantial gaps in the practice of PHS were identified. The lack of training and appropriate instruments and the need for audiological services were the main challenges. Incorporating robust and objective protocols for PHS into the educational system is a valuable strategy for identifying hearing loss early and reducing its impact through the establishment of effective intervention plans.
儿童的听力状况应在整个幼儿期进行检查,即使他们已经度过了新生儿听力损失期,因为听力损失随时可能发生,并可能影响他们的学习能力。学龄前听力筛查(PHS)是至关重要的继续筛查整个幼儿期。沙特阿拉伯初级卫生保健中心(PHCs)目前的小灵通做法尚不清楚。本研究的目的是调查PHCs的小灵通。本横断面描述性研究采用个人管理问卷来收集数据。120名参与者中,共有106人(男61人;代表利雅得初级保健中心的妇女(45岁)接受了采访。大多数参与者年龄在31-40岁之间,拥有学士学位作为最高学历,但工作年限有限。PHS主要通过询问父母(71.7%)和儿童(65.1%)的主观测量方式进行。只有一半的初级保健中心对学龄前儿童进行了听力评估。全科医生和护士通常执行小灵通。确定了小灵通实践中的重大差距。缺乏训练和适当的仪器以及需要听力学服务是主要的挑战。将健全客观的小灵通方案纳入教育系统是早期发现听力损失并通过建立有效的干预计划减少其影响的一种有价值的策略。
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引用次数: 0
Diverse phenotype of Ménière's disease associated with family history, thyroid disorder, migraine and associated disorders 与家族史、甲状腺疾病、偏头痛及相关疾病相关的msami病多样表型
IF 1.4 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-10-01 DOI: 10.1016/j.joto.2024.07.005
Ilmari Pyykkö , Vinay , Jing Zou , Artur Vetkas , Vinaya Manchaiah

Objective

To better understand the clinical phenotype of Ménière's disease (MD), we examined family history, thyroid disorder, migraine, and associated disorders in complaints of people living with MD.

Method

We designed the study as a retrospective and examined data gathered from 912 participants with MD. Their data were originally collected by the Finnish Ménière Federation (FMF). The survey data included individual case histories for environmental factors, comorbidities, disease-specific complaints, impact-related questions, cognitive complaints, health-related quality of life (HRQoL), and sense of coherence (SOC).

Results

We observed significant differences between those with and without sporadic occurrence, family history, thyroid disorder, and migraine-associated complaints. Family history explained 20% of variability in patient complaints. Patients with a family history of MD whose disease started at younger age experienced balance problems, more severe vertigo spells, more severe vestibular drop attacks (VDA), and less nausea, although they had good SOC. Thyroid disorder explained 14% of variability in patient complaints. MD patients with a thyroid disorder comorbidity suffered more often from constant dizziness, balance problems, greater impact of hearing problems, cognitive complaints, and poor HRQoL. Migraine explained 12% of variability in patients’ complaints and was associated with poor SOC and cognitive balance problems. MD patients with both thyroid disorder and migraine used antidepressants more often than other groups. Logistic regression analysis showed comorbidities of ischemic brain disorder (among 7.1%), kidney insufficiency (among 1.2%), and diabetes (among 7.3%) had statistically significant but restricted association with balance and gait problems, VDA, and reduced HRQoL.

Conclusions

Family history of MD and thyroid disorder or migraine comorbidities in MD influence the complaint pattern and partially explain complex symptom profiles, including symptoms of cognitive problems. Confounders play a minimal role in complaint profile and impact of MD whereas comorbidities influence the complaint structure and partly explain the complex symptom profile in MD.
目的:为了更好地了解m懊悔病(MD)的临床表型,我们研究了m懊悔病患者的家族史、甲状腺疾病、偏头痛和相关疾病。方法:我们设计了一项回顾性研究,并检查了912名MD患者的数据。他们的数据最初由芬兰m懊悔病联合会(FMF)收集。调查数据包括环境因素、合并症、疾病特异性投诉、影响相关问题、认知投诉、健康相关生活质量(HRQoL)和连贯感(SOC)的个人病史。结果:我们观察到有和没有散发性、家族史、甲状腺疾病和偏头痛相关投诉的患者之间存在显著差异。家族病史解释了患者投诉中20%的差异。有MD家族史且发病年龄较轻的患者会出现平衡问题、更严重的眩晕期、更严重的前庭下降发作(VDA)和更少的恶心,尽管他们有良好的SOC。甲状腺疾病解释了患者主诉中14%的变异性。伴有甲状腺疾病共病的MD患者更常出现持续的头晕、平衡问题、更大的听力问题影响、认知问题和较差的HRQoL。偏头痛解释了患者抱怨中12%的变异性,并与SOC差和认知平衡问题相关。同时患有甲状腺疾病和偏头痛的MD患者比其他组更常使用抗抑郁药。Logistic回归分析显示,缺血性脑疾病(7.1%)、肾功能不全(1.2%)和糖尿病(7.3%)的合并症与平衡和步态问题、VDA和HRQoL降低有统计学意义,但相关性有限。结论:MD和甲状腺疾病或偏头痛合并症的家族史影响MD的主诉模式,部分解释了复杂的症状特征,包括认知问题的症状。混杂因素在MD的主诉特征和影响中起最小作用,而合并症影响主诉结构并部分解释MD的复杂症状特征。
{"title":"Diverse phenotype of Ménière's disease associated with family history, thyroid disorder, migraine and associated disorders","authors":"Ilmari Pyykkö ,&nbsp;Vinay ,&nbsp;Jing Zou ,&nbsp;Artur Vetkas ,&nbsp;Vinaya Manchaiah","doi":"10.1016/j.joto.2024.07.005","DOIUrl":"10.1016/j.joto.2024.07.005","url":null,"abstract":"<div><h3>Objective</h3><div>To better understand the clinical phenotype of Ménière's disease (MD), we examined family history, thyroid disorder, migraine, and associated disorders in complaints of people living with MD.</div></div><div><h3>Method</h3><div>We designed the study as a retrospective and examined data gathered from 912 participants with MD. Their data were originally collected by the Finnish Ménière Federation (FMF). The survey data included individual case histories for environmental factors, comorbidities, disease-specific complaints, impact-related questions, cognitive complaints, health-related quality of life (HRQoL), and sense of coherence (SOC).</div></div><div><h3>Results</h3><div>We observed significant differences between those with and without sporadic occurrence, family history, thyroid disorder, and migraine-associated complaints. Family history explained 20% of variability in patient complaints. Patients with a family history of MD whose disease started at younger age experienced balance problems, more severe vertigo spells, more severe vestibular drop attacks (VDA), and less nausea, although they had good SOC. Thyroid disorder explained 14% of variability in patient complaints. MD patients with a thyroid disorder comorbidity suffered more often from constant dizziness, balance problems, greater impact of hearing problems, cognitive complaints, and poor HRQoL. Migraine explained 12% of variability in patients’ complaints and was associated with poor SOC and cognitive balance problems. MD patients with both thyroid disorder and migraine used antidepressants more often than other groups. Logistic regression analysis showed comorbidities of ischemic brain disorder (among 7.1%), kidney insufficiency (among 1.2%), and diabetes (among 7.3%) had statistically significant but restricted association with balance and gait problems, VDA, and reduced HRQoL.</div></div><div><h3>Conclusions</h3><div>Family history of MD and thyroid disorder or migraine comorbidities in MD influence the complaint pattern and partially explain complex symptom profiles, including symptoms of cognitive problems. Confounders play a minimal role in complaint profile and impact of MD whereas comorbidities influence the complaint structure and partly explain the complex symptom profile in MD.</div></div>","PeriodicalId":37466,"journal":{"name":"Journal of Otology","volume":"19 4","pages":"Pages 185-192"},"PeriodicalIF":1.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11701336/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142961022","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
Investigating the expression profiles of cysteine string proteins (CSPs) in cochlear tissue 半胱氨酸链蛋白(CSPs)在耳蜗组织中的表达谱研究。
IF 1.4 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-10-01 DOI: 10.1016/j.joto.2024.06.001
Shili Zhang , Caiji Wang , Yuehua Qiao , Xi Shi

Objective

This study aims to explore the expression patterns of cysteine string protein alpha (CSPα) and cysteine string protein beta (CSPβ) in the mammalian inner ear, with an emphasis on their temporal dynamics during the developmental stages of C57BL/6 mice.

Methods

We utilized immunofluorescence staining to assess the localization and distribution of CSPα and CSPβ within the inner ears of C57BL/6 mice and miniature pigs. Additionally, this method facilitated the investigation of their temporal expression profiles.

Results

In adult C57BL/6 mice and miniature pigs, CSPα and CSPβ were identified in the cytoplasm of inner hair cells and spiral ganglion cells, yet were absent in outer hair cells. Both proteins were found to colocalize with Ctbp2 on the basal side of the cytoplasm in inner hair cells' basilar membrane. Expression of CSPα was observed at the nerve fiber termini at the basilar membrane's base of inner and outer hair cells 10 days postnatally in C57BL/6 mice. Notably, expression of both CSPα and CSPβ in the cytoplasm of inner hair cells emerged on the 12th day post-birth, aligning with the timeline for registering cochlear potentials. The expression levels of both proteins increased with age, but were consistently absent in outer hair cells. Contrastingly, expression of CSPα and CSPβ was present in the cytoplasm of inner hair cells in miniature pigs as early as one day post-birth, yet remained absent in the three rows of outer hair cells.

Conclusion

CSPα and CSPβ exhibit predominant and specific expression in inner hair cells and spiral ganglion cells. A unique expression pattern was observed for CSPα, which was also present at the nerve fiber endings of both inner and outer hair cells. The developmental expression trajectory of CSPα and CSPβ in mouse inner hair cells is characterized by an initial absence, followed by a gradual increase. Moreover, the timing of expression onset between mice and miniature pigs indicates distinct temporal dynamics, suggesting a potential role in auditory development.
目的:探讨哺乳动物内耳半胱氨酸弦蛋白α (CSPα)和半胱氨酸弦蛋白β (CSPβ)在C57BL/6小鼠发育阶段的表达规律,重点研究其时间动态。方法:采用免疫荧光染色法观察CSPα和CSPβ在C57BL/6小鼠和小型猪内耳的定位和分布。此外,这种方法有助于研究他们的时间表达谱。结果:成年C57BL/6小鼠和小型猪的内毛细胞和螺旋神经节细胞细胞质中检测到CSPα和CSPβ,而外毛细胞中不存在CSPα和CSPβ。这两种蛋白均与Ctbp2共定位于内毛细胞基底膜细胞质基侧。CSPα在C57BL/6小鼠出生后10天内、外毛细胞基底膜基部的神经纤维末梢表达。值得注意的是,出生后第12天,内毛细胞细胞质中均出现CSPα和CSPβ的表达,与耳蜗电位记录时间一致。这两种蛋白的表达水平随着年龄的增长而增加,但在外毛细胞中一直不存在。相比之下,CSPα和CSPβ早在出生后1天就在小型猪的内毛细胞细胞质中表达,而在三排外毛细胞中不表达。结论:CSPα和CSPβ在内毛细胞和螺旋神经节细胞中具有显性和特异性表达。CSPα在毛细胞内外神经纤维末梢均有独特的表达模式。CSPα和CSPβ在小鼠内毛细胞中的发育表达轨迹具有先缺失后逐渐增加的特点。此外,小鼠和小型猪之间的表达开始时间表明了不同的时间动态,表明听觉发育的潜在作用。
{"title":"Investigating the expression profiles of cysteine string proteins (CSPs) in cochlear tissue","authors":"Shili Zhang ,&nbsp;Caiji Wang ,&nbsp;Yuehua Qiao ,&nbsp;Xi Shi","doi":"10.1016/j.joto.2024.06.001","DOIUrl":"10.1016/j.joto.2024.06.001","url":null,"abstract":"<div><h3>Objective</h3><div>This study aims to explore the expression patterns of cysteine string protein alpha (CSPα) and cysteine string protein beta (CSPβ) in the mammalian inner ear, with an emphasis on their temporal dynamics during the developmental stages of C57BL/6 mice.</div></div><div><h3>Methods</h3><div>We utilized immunofluorescence staining to assess the localization and distribution of CSPα and CSPβ within the inner ears of C57BL/6 mice and miniature pigs. Additionally, this method facilitated the investigation of their temporal expression profiles.</div></div><div><h3>Results</h3><div>In adult C57BL/6 mice and miniature pigs, CSPα and CSPβ were identified in the cytoplasm of inner hair cells and spiral ganglion cells, yet were absent in outer hair cells. Both proteins were found to colocalize with Ctbp2 on the basal side of the cytoplasm in inner hair cells' basilar membrane. Expression of CSPα was observed at the nerve fiber termini at the basilar membrane's base of inner and outer hair cells 10 days postnatally in C57BL/6 mice. Notably, expression of both CSPα and CSPβ in the cytoplasm of inner hair cells emerged on the 12th day post-birth, aligning with the timeline for registering cochlear potentials. The expression levels of both proteins increased with age, but were consistently absent in outer hair cells. Contrastingly, expression of CSPα and CSPβ was present in the cytoplasm of inner hair cells in miniature pigs as early as one day post-birth, yet remained absent in the three rows of outer hair cells.</div></div><div><h3>Conclusion</h3><div>CSPα and CSPβ exhibit predominant and specific expression in inner hair cells and spiral ganglion cells. A unique expression pattern was observed for CSPα, which was also present at the nerve fiber endings of both inner and outer hair cells. The developmental expression trajectory of CSPα and CSPβ in mouse inner hair cells is characterized by an initial absence, followed by a gradual increase. Moreover, the timing of expression onset between mice and miniature pigs indicates distinct temporal dynamics, suggesting a potential role in auditory development.</div></div>","PeriodicalId":37466,"journal":{"name":"Journal of Otology","volume":"19 4","pages":"Pages 193-199"},"PeriodicalIF":1.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11701328/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142961052","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
Exploring the efficacy of (R)-PFI-2 hydrochloride in mitigating noise-induced hearing loss by targeting NLRP3 inflammasome and NF-κB pathway to reduce inner ear inflammation 探讨盐酸(R)-PFI-2通过靶向NLRP3炎性体和NF-κB通路减轻内耳炎症,减轻噪声性听力损失的疗效。
IF 1.4 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-10-01 DOI: 10.1016/j.joto.2024.07.008
Dawei Ren , Xuemin Chen , Hongdong Liu , Menghua Li , Liting Zheng , Pan Yong , Mohe Huang , Xi Shi , Yice Xu , Shujin Chen , Yan Zhang , Wei Zhu
Noise-induced hearing loss (NIHL) is primarily driven by inflammatory processes within the cochlea, where noise exposure triggers the activation of the NOD-like receptor protein 3 (NLRP3) inflammasome, leading to an inflammatory cascade. The interaction between increased NLRP3 expression and NF-κB activity can further amplify cochlear inflammation. Our findings reveal that (R)-PFI-2 hydrochloride, a selective inhibitor of the SETD7 enzyme, effectively inhibits the activation of the cochlear NF-κB pathway, suppresses the release of pro-inflammatory factors, and prevents inflammasome assembly. This intervention disrupts the perpetuating cycle of inflammation, thereby alleviating damage to cochlear hair cells attributed to acoustic trauma. Consequently, (R)-PFI-2 hydrochloride emerges as a promising pharmacological candidate for NIHL, targeting and moderating the excessive immune and inflammatory responses implicated in the pathology of hearing loss.
噪声性听力损失(NIHL)主要由耳蜗内的炎症过程驱动,其中噪声暴露触发nod样受体蛋白3 (NLRP3)炎性体的激活,导致炎症级联反应。NLRP3表达升高与NF-κB活性的相互作用可进一步放大耳蜗炎症。我们的研究结果表明,SETD7酶的选择性抑制剂(R)-PFI-2 hydrochloride可有效抑制耳蜗NF-κB通路的激活,抑制促炎因子的释放,阻止炎性小体的组装。这种干预破坏了炎症的持续循环,从而减轻了耳蜗毛细胞因声创伤造成的损伤。因此,(R)-PFI-2盐酸盐成为NIHL的有希望的药理学候选药物,靶向并调节与听力损失病理相关的过度免疫和炎症反应。
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引用次数: 0
Assessment of sensory impairment in older adults with dementia 老年痴呆患者感觉功能障碍的评估。
IF 1.4 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-10-01 DOI: 10.1016/j.joto.2024.11.001
Nikol Gottfriedová , Martina Kovalová , Eva Mrázková , Ondřej Machaczka , Veronika Koutná , Vladimír Janout , Jana Janoutová

Background

Over 55 million people worldwide are living with dementia. The rate of cognitive decline increases with age, and loss of senses may be a contributing factor.

Objectives

This study aimed to analyze hearing, olfactory function, and color vision in patients with dementia.

Materials and methods

The sample comprised 40 patients with dementia and 37 cognitively normal controls aged 41–85 years. All participants underwent conventional pure-tone audiometry and a screening version of the Hearing Handicap Inventory for Adults, the Odorized Markers Test of olfactory function and the Ishihara color vision test. The effects of comorbidities and lifestyle factors were also assessed.

Results

Patients with dementia had significantly worse hearing at almost all frequencies tested and significantly greater olfactory impairment than cognitively normal controls. Color vision impairment was found in less than 8% of the sample, with no significant difference between the groups. Impairment of two senses (hearing and olfaction) was significantly more common in patients with dementia than in controls.

Conclusion

Individuals with dementia were found to have sensory decline, namely hearing and olfactory impairment. Color vision was rarely impaired in the sample. Participants with dementia tended to have more multisensory impairments than controls.
背景:全世界有超过5500万人患有痴呆症。认知能力下降的速度随着年龄的增长而增加,而感官的丧失可能是一个促成因素。目的:本研究旨在分析痴呆患者的听力、嗅觉功能和色觉。材料与方法:40例痴呆患者和37例认知正常对照,年龄41 ~ 85岁。所有的参与者都接受了传统的纯音测听和筛查版的成人听力障碍量表,嗅觉功能的气味标记测试和石原色觉测试。还评估了合并症和生活方式因素的影响。结果:痴呆患者在几乎所有频率的听力测试中都比认知正常的对照组明显更差,嗅觉损伤也明显更大。色觉障碍在不到8%的样本中被发现,两组之间没有显著差异。两种感觉(听觉和嗅觉)的损伤在痴呆患者中明显比对照组更常见。结论:痴呆患者存在感觉功能下降,即听觉和嗅觉障碍。色觉在样本中很少受损。痴呆患者往往比对照组有更多的多感觉障碍。
{"title":"Assessment of sensory impairment in older adults with dementia","authors":"Nikol Gottfriedová ,&nbsp;Martina Kovalová ,&nbsp;Eva Mrázková ,&nbsp;Ondřej Machaczka ,&nbsp;Veronika Koutná ,&nbsp;Vladimír Janout ,&nbsp;Jana Janoutová","doi":"10.1016/j.joto.2024.11.001","DOIUrl":"10.1016/j.joto.2024.11.001","url":null,"abstract":"<div><h3>Background</h3><div>Over 55 million people worldwide are living with dementia. The rate of cognitive decline increases with age, and loss of senses may be a contributing factor.</div></div><div><h3>Objectives</h3><div>This study aimed to analyze hearing, olfactory function, and color vision in patients with dementia.</div></div><div><h3>Materials and methods</h3><div>The sample comprised 40 patients with dementia and 37 cognitively normal controls aged 41–85 years. All participants underwent conventional pure-tone audiometry and a screening version of the Hearing Handicap Inventory for Adults, the Odorized Markers Test of olfactory function and the Ishihara color vision test. The effects of comorbidities and lifestyle factors were also assessed.</div></div><div><h3>Results</h3><div>Patients with dementia had significantly worse hearing at almost all frequencies tested and significantly greater olfactory impairment than cognitively normal controls. Color vision impairment was found in less than 8% of the sample, with no significant difference between the groups. Impairment of two senses (hearing and olfaction) was significantly more common in patients with dementia than in controls.</div></div><div><h3>Conclusion</h3><div>Individuals with dementia were found to have sensory decline, namely hearing and olfactory impairment. Color vision was rarely impaired in the sample. Participants with dementia tended to have more multisensory impairments than controls.</div></div>","PeriodicalId":37466,"journal":{"name":"Journal of Otology","volume":"19 4","pages":"Pages 220-226"},"PeriodicalIF":1.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11701325/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142961020","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
Roles of supporting cells in the maintenance and regeneration of the damaged inner ear: A literature review 支持细胞在受损内耳维持和再生中的作用:文献综述。
IF 1.4 Q2 OTORHINOLARYNGOLOGY Pub Date : 2024-10-01 DOI: 10.1016/j.joto.2024.07.007
Jing-Ying Guo , Jun-Yi Xu , Shu-Sheng Gong , Guo-Peng Wang
The inner ear sensory epithelium consists of two major types of cells: hair cells (HCs) and supporting cells (SCs). Critical functions of HCs in the perception of mechanical stimulation and mechanosensory transduction have long been elucidated. SCs are indispensable components of the sensory epithelia, and they maintain the structural integrity and ionic environment of the inner ear. Once delicate inner ear epithelia sustain injuries (for example, due to ototoxic drugs or noise exposure), SCs respond immediately to serve as repairers of the epithelium and as adapters to become HC progenitors, aiming at morphological and functional recovery of the inner ear. This regenerative process is extensive in non-mammals, but is limited in the mammalian inner ear, especially in the mature cochlea. This review aimed to discuss the important roles of SCs in the repair of the mammalian inner ear.
内耳感觉上皮由两大类细胞组成:毛细胞(HCs)和支持细胞(SCs)。hc在机械刺激感知和机械感觉转导中的关键功能早已被阐明。SCs是感觉上皮细胞不可缺少的组成部分,它们维持内耳的结构完整性和离子环境。一旦脆弱的内耳上皮受到损伤(例如,由于耳毒性药物或噪音暴露),sc立即反应作为上皮的修复者和成为HC祖细胞的适配器,旨在内耳形态和功能的恢复。这种再生过程在非哺乳动物中广泛存在,但在哺乳动物内耳,尤其是成熟耳蜗中受到限制。本文就SCs在哺乳动物内耳修复中的重要作用进行综述。
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引用次数: 0
期刊
Journal of Otology
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