目的 比较韶音后挂式骨导助听器对不同类型听力损失患者的听力干预短期效果,探讨其临床应用前景。方法 55例听力损失患者(年龄18~82岁;传导性听力损失9例,感音神经性听力损失15例,混合性听力损失31例;左右耳0.5、1、2、4 kHz四个频率的...目的 比较韶音后挂式骨导助听器对不同类型听力损失患者的听力干预短期效果,探讨其临床应用前景。方法 55例听力损失患者(年龄18~82岁;传导性听力损失9例,感音神经性听力损失15例,混合性听力损失31例;左右耳0.5、1、2、4 kHz四个频率的骨导纯音听阈均≤60 dB HL)配戴韶音后挂式骨导助听器,分别于配戴助听器前和配戴第14±2 d行声场总体听阈、单音节识别率及安静环境语句识别阈测试,比较配戴助听器前后的结果差异。并于配戴第14±2 d使用IOI-HA问卷对助听器使用效果进行评估。结果 患者配戴后挂式骨导式助听器后声场四个频率平均听阈(39.3±4.9 dB HL)较配戴前(56.5±8.2 dB HL)显著改善,差异有统计学意义(P<0.001)。患者助听前单音节识别率(给声强度:患者助听前双音节言语识别阈减5 dB)为29.8%±11.4%,配戴第14±2 d为72.4%±14.4%,配戴后单音节识别率显著提高,差异有统计学意义(P<0.001)。患者语句识别阈由配戴前的48.6±9.7 dB HL降至34.3±5.6 dB HL,差异有统计学意义(P<0.001)。配戴14±2 d时IOI-HA问卷评估总分平均值为29.0±3.8分。结论 后挂式骨导助听器可显著提高传导性、0.5~4 kHz骨导纯音听阈不超过60 dB HL的混合性及感音神经性听力损失患者的听力及言语识别能力。展开更多
Aminoglycosides are a widely used class of antibacterials renowned for their effectiveness and broad antimicrobial spectrum.However,their use leads to irreversible hearing damage by causing apoptosis of hair cells as ...Aminoglycosides are a widely used class of antibacterials renowned for their effectiveness and broad antimicrobial spectrum.However,their use leads to irreversible hearing damage by causing apoptosis of hair cells as their direct target.In addition,the hearing damage caused by aminoglycosides involves damage of spiral ganglion neurons upon exposure.To investigate the mechanisms underlying spiral ganglion neuron degeneration induced by aminoglycosides,we used a C57BL/6J mouse model treated with kanamycin.We found that the mice exhibited auditory deficits following the acute loss of outer hair cells.Spiral ganglion neurons displayed hallmarks of pyroptosis and exhibited progressive degeneration over time.Transcriptomic profiling of these neurons showed significant upregulation of genes associated with inflammation and immune response,particularly those related to the NLRP3 inflammasome.Activation of the canonical pyroptotic pathway in spiral ganglion neurons was observed,accompanied by infiltration of macrophages and the release of proinflammatory cytokines.Pharmacological intervention targeting NLRP3 using Mcc950 and genetic intervention using NLRP3 knockout ameliorated spiral ganglion neuron degeneration in the injury model.These findings suggest that NLRP3 inflammasome-mediated pyroptosis plays a role in aminoglycoside-induced spiral ganglion neuron degeneration.Inhibition of this pathway may offer a potential therapeutic strategy for treating sensorineural hearing loss by reducing spiral ganglion neuron degeneration.展开更多
目的:通过循证医学方法系统评价电针治疗突发性耳聋的临床疗效。方法:系统检索中国知网、万方、维普、Pubmed、Embase、Web of Science、Cochrane Library数据库中自建库至2024年10月7日以来电针治疗突发性耳聋的随机对照试验,由2名研...目的:通过循证医学方法系统评价电针治疗突发性耳聋的临床疗效。方法:系统检索中国知网、万方、维普、Pubmed、Embase、Web of Science、Cochrane Library数据库中自建库至2024年10月7日以来电针治疗突发性耳聋的随机对照试验,由2名研究者参考Cochrane协作网提供的偏倚风险评估工具,采用RevMan 5.3软件进行Meta分析。结果:共纳入8篇文献,样本量为638例,试验组321例,对照组317例。Meta分析结果显示,试验组电针治疗突发性耳聋的总有效率(RR=1.23,95%CI[1.07,1.41],P=0.003)、治愈率(RR=1.73,95%CI[1.28,2.34],P=0.0004)、眩晕总有效率(RR=1.25,95%CI[0.99,1.57],P=0.06)均高于对照组;试验组平均听阈值低于对照组(MD=-6.29,95%CI[-9.01,-3.56],P<0.00001),差异均有统计学意义(P<0.05)。结论:电针治疗突发性耳聋可以提高突发性耳聋总有效率、治愈率、眩晕总有效率,改善平均听阈值。展开更多
Noise-induced hearing loss is the primary non-genetic factor contributing to auditory dysfunction.However,there are currently no effective pharmacological interventions for patients with noise-induced hearing loss.Her...Noise-induced hearing loss is the primary non-genetic factor contributing to auditory dysfunction.However,there are currently no effective pharmacological interventions for patients with noise-induced hearing loss.Here,we present evidence suggesting that the lysine-specific demethylase 1 inhibitor–tranylcypromine is an otoprotective agent that could be used to treat noise-induced hearing loss,and elucidate its underlying regulatory mechanisms.We established a mouse model of permanent threshold shift hearing loss by exposing the mice to white broadband noise at a sound pressure level of 120 d B for 4 hours.We found that tranylcypromine treatment led to the upregulation of Sestrin2(SESN2)and activation of the autophagy markers light chain 3B and lysosome-associated membrane glycoprotein 1 in the cochleae of mice treated with tranylcypromine.The noise exposure group treated with tranylcypromine showed significantly lower average auditory brainstem response hearing thresholds at click,4,8,and 16 k Hz frequencies compared with the noise exposure group treated with saline.These findings indicate that tranylcypromine treatment resulted in increased SESN2,light chain 3B,and lysosome-associated membrane glycoprotein 1 expression after noise exposure,leading to a reduction in levels of 4-hydroxynonenal and cleaved caspase-3,thereby reducing noise-induced hair cell loss.Additionally,immunoblot analysis demonstrated that treatment with tranylcypromine upregulated SESN2 expression via the autophagy pathway.Tranylcypromine treatment also reduced the production of NOD-like receptor family pyrin domaincontaining 3(NLRP3)production.In conclusion,our results showed that tranylcypromine treatment ameliorated cochlear inflammation by promoting the expression of SESN2,which induced autophagy,thereby restricting NLRP3-related inflammasome signaling,alleviating cochlear hair cell loss,and protecting hearing function.These findings suggest that inhibiting lysine-specific demethylase 1 is a potential therapeutic strategy for preventing hair cell loss and noise-induced hearing loss.展开更多
Hearing loss is the third leading cause of human disability.Age-related hearing loss,one type of acquired sensorineural hearing loss,is largely responsible for this escalating global health burden.Noise-induced,ototox...Hearing loss is the third leading cause of human disability.Age-related hearing loss,one type of acquired sensorineural hearing loss,is largely responsible for this escalating global health burden.Noise-induced,ototoxic,and idiopathic sudden sensorineural are other less common types of acquired hearing loss.The etiology of these conditions is complex and multi-fa ctorial involving an interplay of genetic and environmental factors.Oxidative stress has recently been proposed as a likely linking cause in most types of acquired sensorineural hearing loss.Short non-coding RNA sequences known as microRNAs(miRNAs)have increasingly been shown to play a role in cellular hypoxia and oxidative stress responses including promoting an apoptotic response.Sensory hair cell death is a central histopathological finding in sensorineural hearing loss.As these cells do not regenerate in humans,it underlies the irreversibility of human age-related hearing loss.Ovid EMBASE,Ovid MEDLINE,Web of Science Core Collection,and ClinicalTrials.gov databases over the period August 1,2018 to July 31,2023 were searched with"hearing loss,""hypoxamiRs,""hypoxia,""microRNAs,""ischemia,"and"oxidative stress"text words for English language primary study publications or registered clinical trials.Registe red clinical trials known to the senior author we re also assessed.A total of 222studies were thus identified.After excluding duplicates,editorials,retra ctions,secondary research studies,and non-English language articles,39 primary studies and clinical trials underwent full-text screening.This resulted in 11 animal,in vitro,and/or human subject journal articles and 8 registered clinical trial database entries which form the basis of this narrative review.MiRNAs miR-34a and miR-29b levels increase with age in mice.These miRNAs were demonstrated in human neuroblastoma and murine cochlear cell lines to target Sirtuin 1/peroxisome proliferato r-activated receptor gamma coactivator-1-alpha(SIRT1/P GC-1α),SIRT1p53,and SIRT1/hypoxia-inducible factor 1-alpha signaling pathways resulting in increased apoptosis.Furthermore,hypoxia and oxidative stress had a similar adve rse apoptotic effect,which was inhibited by resve ratrol and a myocardial inhibitorassociated transcript,a miR-29b competing endogenous mRNA.Gentamicin reduced miR-182-5p levels and increased cochlear oxidative stress and cell death in mice-an effect that was corrected by inner ear stem cell-derived exosomes.There is ongoing work seeking to determine if these findings can be effectively translated to humans.展开更多
Foot reflexology is a non-invasive and safe complementary therapy that works by massaging the reflex zones of the feet and exerts systemic or whole-body regulation through meridian nerve conduction.This therapy is com...Foot reflexology is a non-invasive and safe complementary therapy that works by massaging the reflex zones of the feet and exerts systemic or whole-body regulation through meridian nerve conduction.This therapy is commonly used in the treatment of various conditions such as autism and Parkinson's disease.However,there is limited reporting on the use of foot reflexology therapy for infants with sensorineural hearing loss(SNHL).Currently,there is no definitive conclusion on how foot reflexology therapy can influence hearing.This editorial holds some guiding significance regarding this clinical issue.The aim is to present physiological evidence of how foot reflexology therapy can impact infants with SNHL,thereby enhancing clinician’s awareness of foot reflexology in treating infants with SNHL.展开更多
目的 研究一个家系中2例非综合征型遗传性聋患者的致病原因。方法 收集该耳聋家系临床检查结果资料,采集静脉血后提取DNA,应用全外显子组测序技术分析可疑致病基因,并通过Sanger测序对变异进行验证。结果 该家系中2代5人,先证者(Ⅱ-2,9...目的 研究一个家系中2例非综合征型遗传性聋患者的致病原因。方法 收集该耳聋家系临床检查结果资料,采集静脉血后提取DNA,应用全外显子组测序技术分析可疑致病基因,并通过Sanger测序对变异进行验证。结果 该家系中2代5人,先证者(Ⅱ-2,9岁)和其弟弟(Ⅱ-3)均为迟发性感音神经性听力损失,患者父母听力正常。先证者弟弟(Ⅱ-3)携带相同突变位点。基因检测结果显示,先证者携带CDH23基因c.4762C>T(p.ARG1588TRP)和c.6604G>A(p.ASP2202ASN)。根据美国医学遗传学与基因组学学会(American College of Medical Genetics and Genomics, ACMG)遗传变异分类标准与指南分析显示,c.4762C>T和c.6604G>A位点均为致病突变。蛋白功能分析发现,变异后氨基酸改变造成蛋白结构发生改变,进而影响蛋白质功能。结论 CDH23基因c.4762C>T和c.6604G>A复合杂合突变位点组合很可能是该家系耳聋致病基因。展开更多
文摘目的 比较韶音后挂式骨导助听器对不同类型听力损失患者的听力干预短期效果,探讨其临床应用前景。方法 55例听力损失患者(年龄18~82岁;传导性听力损失9例,感音神经性听力损失15例,混合性听力损失31例;左右耳0.5、1、2、4 kHz四个频率的骨导纯音听阈均≤60 dB HL)配戴韶音后挂式骨导助听器,分别于配戴助听器前和配戴第14±2 d行声场总体听阈、单音节识别率及安静环境语句识别阈测试,比较配戴助听器前后的结果差异。并于配戴第14±2 d使用IOI-HA问卷对助听器使用效果进行评估。结果 患者配戴后挂式骨导式助听器后声场四个频率平均听阈(39.3±4.9 dB HL)较配戴前(56.5±8.2 dB HL)显著改善,差异有统计学意义(P<0.001)。患者助听前单音节识别率(给声强度:患者助听前双音节言语识别阈减5 dB)为29.8%±11.4%,配戴第14±2 d为72.4%±14.4%,配戴后单音节识别率显著提高,差异有统计学意义(P<0.001)。患者语句识别阈由配戴前的48.6±9.7 dB HL降至34.3±5.6 dB HL,差异有统计学意义(P<0.001)。配戴14±2 d时IOI-HA问卷评估总分平均值为29.0±3.8分。结论 后挂式骨导助听器可显著提高传导性、0.5~4 kHz骨导纯音听阈不超过60 dB HL的混合性及感音神经性听力损失患者的听力及言语识别能力。
基金supported by the National Natural Science Foundation of China,Nos.81800919(to YX),82171140(to PW)the International Cooperation and Exchange of the National Natural Science Foundation of China,Nos.82020108008(to HS),81720108010(to SY).
文摘Aminoglycosides are a widely used class of antibacterials renowned for their effectiveness and broad antimicrobial spectrum.However,their use leads to irreversible hearing damage by causing apoptosis of hair cells as their direct target.In addition,the hearing damage caused by aminoglycosides involves damage of spiral ganglion neurons upon exposure.To investigate the mechanisms underlying spiral ganglion neuron degeneration induced by aminoglycosides,we used a C57BL/6J mouse model treated with kanamycin.We found that the mice exhibited auditory deficits following the acute loss of outer hair cells.Spiral ganglion neurons displayed hallmarks of pyroptosis and exhibited progressive degeneration over time.Transcriptomic profiling of these neurons showed significant upregulation of genes associated with inflammation and immune response,particularly those related to the NLRP3 inflammasome.Activation of the canonical pyroptotic pathway in spiral ganglion neurons was observed,accompanied by infiltration of macrophages and the release of proinflammatory cytokines.Pharmacological intervention targeting NLRP3 using Mcc950 and genetic intervention using NLRP3 knockout ameliorated spiral ganglion neuron degeneration in the injury model.These findings suggest that NLRP3 inflammasome-mediated pyroptosis plays a role in aminoglycoside-induced spiral ganglion neuron degeneration.Inhibition of this pathway may offer a potential therapeutic strategy for treating sensorineural hearing loss by reducing spiral ganglion neuron degeneration.
文摘目的:通过循证医学方法系统评价电针治疗突发性耳聋的临床疗效。方法:系统检索中国知网、万方、维普、Pubmed、Embase、Web of Science、Cochrane Library数据库中自建库至2024年10月7日以来电针治疗突发性耳聋的随机对照试验,由2名研究者参考Cochrane协作网提供的偏倚风险评估工具,采用RevMan 5.3软件进行Meta分析。结果:共纳入8篇文献,样本量为638例,试验组321例,对照组317例。Meta分析结果显示,试验组电针治疗突发性耳聋的总有效率(RR=1.23,95%CI[1.07,1.41],P=0.003)、治愈率(RR=1.73,95%CI[1.28,2.34],P=0.0004)、眩晕总有效率(RR=1.25,95%CI[0.99,1.57],P=0.06)均高于对照组;试验组平均听阈值低于对照组(MD=-6.29,95%CI[-9.01,-3.56],P<0.00001),差异均有统计学意义(P<0.05)。结论:电针治疗突发性耳聋可以提高突发性耳聋总有效率、治愈率、眩晕总有效率,改善平均听阈值。
基金supported by the National Key Research and Development Program of China,No.2022YFC2402701(to WC)Key International(Regional)Joint Research Program of the National Natural Science Foundation of China,No.81820108009(to SY)+5 种基金the National Natural Science Foundation of China,Nos.81970890(to WC)and 82371148(to WG)Fujian Provincial Healthcare Young and Middle-aged Backbone Talent Training Project,No.2023GGA035(to XC)Spring City Planthe High-level Talent Promotion and Training Project of Kunming,No.2022SCP001(to SY)the Natural Science Foundation of Hainan Province of China,No.824MS052(to XS)the Sixth Medical Center of Chinese PLA General Hospital Innovation Cultivation,No.CXPY202116(to LX)。
文摘Noise-induced hearing loss is the primary non-genetic factor contributing to auditory dysfunction.However,there are currently no effective pharmacological interventions for patients with noise-induced hearing loss.Here,we present evidence suggesting that the lysine-specific demethylase 1 inhibitor–tranylcypromine is an otoprotective agent that could be used to treat noise-induced hearing loss,and elucidate its underlying regulatory mechanisms.We established a mouse model of permanent threshold shift hearing loss by exposing the mice to white broadband noise at a sound pressure level of 120 d B for 4 hours.We found that tranylcypromine treatment led to the upregulation of Sestrin2(SESN2)and activation of the autophagy markers light chain 3B and lysosome-associated membrane glycoprotein 1 in the cochleae of mice treated with tranylcypromine.The noise exposure group treated with tranylcypromine showed significantly lower average auditory brainstem response hearing thresholds at click,4,8,and 16 k Hz frequencies compared with the noise exposure group treated with saline.These findings indicate that tranylcypromine treatment resulted in increased SESN2,light chain 3B,and lysosome-associated membrane glycoprotein 1 expression after noise exposure,leading to a reduction in levels of 4-hydroxynonenal and cleaved caspase-3,thereby reducing noise-induced hair cell loss.Additionally,immunoblot analysis demonstrated that treatment with tranylcypromine upregulated SESN2 expression via the autophagy pathway.Tranylcypromine treatment also reduced the production of NOD-like receptor family pyrin domaincontaining 3(NLRP3)production.In conclusion,our results showed that tranylcypromine treatment ameliorated cochlear inflammation by promoting the expression of SESN2,which induced autophagy,thereby restricting NLRP3-related inflammasome signaling,alleviating cochlear hair cell loss,and protecting hearing function.These findings suggest that inhibiting lysine-specific demethylase 1 is a potential therapeutic strategy for preventing hair cell loss and noise-induced hearing loss.
文摘Hearing loss is the third leading cause of human disability.Age-related hearing loss,one type of acquired sensorineural hearing loss,is largely responsible for this escalating global health burden.Noise-induced,ototoxic,and idiopathic sudden sensorineural are other less common types of acquired hearing loss.The etiology of these conditions is complex and multi-fa ctorial involving an interplay of genetic and environmental factors.Oxidative stress has recently been proposed as a likely linking cause in most types of acquired sensorineural hearing loss.Short non-coding RNA sequences known as microRNAs(miRNAs)have increasingly been shown to play a role in cellular hypoxia and oxidative stress responses including promoting an apoptotic response.Sensory hair cell death is a central histopathological finding in sensorineural hearing loss.As these cells do not regenerate in humans,it underlies the irreversibility of human age-related hearing loss.Ovid EMBASE,Ovid MEDLINE,Web of Science Core Collection,and ClinicalTrials.gov databases over the period August 1,2018 to July 31,2023 were searched with"hearing loss,""hypoxamiRs,""hypoxia,""microRNAs,""ischemia,"and"oxidative stress"text words for English language primary study publications or registered clinical trials.Registe red clinical trials known to the senior author we re also assessed.A total of 222studies were thus identified.After excluding duplicates,editorials,retra ctions,secondary research studies,and non-English language articles,39 primary studies and clinical trials underwent full-text screening.This resulted in 11 animal,in vitro,and/or human subject journal articles and 8 registered clinical trial database entries which form the basis of this narrative review.MiRNAs miR-34a and miR-29b levels increase with age in mice.These miRNAs were demonstrated in human neuroblastoma and murine cochlear cell lines to target Sirtuin 1/peroxisome proliferato r-activated receptor gamma coactivator-1-alpha(SIRT1/P GC-1α),SIRT1p53,and SIRT1/hypoxia-inducible factor 1-alpha signaling pathways resulting in increased apoptosis.Furthermore,hypoxia and oxidative stress had a similar adve rse apoptotic effect,which was inhibited by resve ratrol and a myocardial inhibitorassociated transcript,a miR-29b competing endogenous mRNA.Gentamicin reduced miR-182-5p levels and increased cochlear oxidative stress and cell death in mice-an effect that was corrected by inner ear stem cell-derived exosomes.There is ongoing work seeking to determine if these findings can be effectively translated to humans.
基金Supported by the Fundamental Research Funds for the Central Universities,No.2022CDJYGRH-004.
文摘Foot reflexology is a non-invasive and safe complementary therapy that works by massaging the reflex zones of the feet and exerts systemic or whole-body regulation through meridian nerve conduction.This therapy is commonly used in the treatment of various conditions such as autism and Parkinson's disease.However,there is limited reporting on the use of foot reflexology therapy for infants with sensorineural hearing loss(SNHL).Currently,there is no definitive conclusion on how foot reflexology therapy can influence hearing.This editorial holds some guiding significance regarding this clinical issue.The aim is to present physiological evidence of how foot reflexology therapy can impact infants with SNHL,thereby enhancing clinician’s awareness of foot reflexology in treating infants with SNHL.
文摘目的 研究一个家系中2例非综合征型遗传性聋患者的致病原因。方法 收集该耳聋家系临床检查结果资料,采集静脉血后提取DNA,应用全外显子组测序技术分析可疑致病基因,并通过Sanger测序对变异进行验证。结果 该家系中2代5人,先证者(Ⅱ-2,9岁)和其弟弟(Ⅱ-3)均为迟发性感音神经性听力损失,患者父母听力正常。先证者弟弟(Ⅱ-3)携带相同突变位点。基因检测结果显示,先证者携带CDH23基因c.4762C>T(p.ARG1588TRP)和c.6604G>A(p.ASP2202ASN)。根据美国医学遗传学与基因组学学会(American College of Medical Genetics and Genomics, ACMG)遗传变异分类标准与指南分析显示,c.4762C>T和c.6604G>A位点均为致病突变。蛋白功能分析发现,变异后氨基酸改变造成蛋白结构发生改变,进而影响蛋白质功能。结论 CDH23基因c.4762C>T和c.6604G>A复合杂合突变位点组合很可能是该家系耳聋致病基因。
