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
This editorial critically evaluates the application of foot reflexology as a treatment for sensorineural hearing loss(SNHL)in infants,as proposed in a case report published in the World of Clinical Cases.SNHL is a con...This editorial critically evaluates the application of foot reflexology as a treatment for sensorineural hearing loss(SNHL)in infants,as proposed in a case report published in the World of Clinical Cases.SNHL is a condition characterized by damage to the cochlea or the neural pathways that transmit auditory information to the brain.The etiology of SNHL is often complex,involving genetic mutations,prenatal factors,or perinatal insults.Reflexology,an alternative therapy involving the application of pressure to specific points on the feet,is based on the hypothesis that these points correspond to different organs and systems in the body,including the auditory system.However,the biological plausibility and clinical efficacy of foot reflexology in addressing SNHL lack empirical support.This editorial examines the pathophysiology of SNHL,assesses the clinical claims of reflexology practitioners,and emphasizes the necessity of evidence-based approaches in treating infant hearing loss.While complementary therapies may provide ancillary benefits,they should not supplant validated medical treatments in managing SNHL in infants.Further research is needed to evaluate the safety and efficacy of foot reflexology and other alternative therapies in pediatric audiology.展开更多
The cochlea is one of the most complex organs in the human body,exhibiting a complex interplay of characteristics in acoustic,mechanical,electrical,and biological functions.Functional cochlea models are an essential p...The cochlea is one of the most complex organs in the human body,exhibiting a complex interplay of characteristics in acoustic,mechanical,electrical,and biological functions.Functional cochlea models are an essential platform for studying hearing mechanics and are crucial for developing next-generation auditory prostheses and artificial hearing systems for sensorineural hearing restoration.Recent advances in additive manufacturing,organ-on-a-chip models,drug delivery platforms,and artificial intelligence have provided valuable insights into how to manufacture artificial cochlea models that more accurately replicate the complex anatomy and physiology of the inner ear.This paper reviews recent advancements in the applications of advanced manufacturing techniques in reproducing the physical,biological,and intelligent functions of the cochlea.It also outlines the current challenges to developing mechanically,electrically,and anatomically accurate functional models of the inner ear.Finally,this review identifies the major requirements and outlook for impactful research in this field going forward.Through interdisciplinary collaboration and innovation,these functional cochlea models are poised to drive significant advancements in hearing treatments,and ultimately enhance the quality of life for individuals with hearing loss.展开更多
Background Sudden sensorineural hearing loss(SSNHL)is a rapid condition of hearing impairment,typically occurring over 72 hours and affecting the inner ear or auditory nerve pathways.The etiology of SSNHL remains larg...Background Sudden sensorineural hearing loss(SSNHL)is a rapid condition of hearing impairment,typically occurring over 72 hours and affecting the inner ear or auditory nerve pathways.The etiology of SSNHL remains largely idiopathic,with potential causes including viral infections,vascular occlusions,and autoimmune disorders.Traditional treatment primarily involves systemic corticosteroids,but their efficacy is inconsistent,leading to exploring alternative and adjunctive therapies such as intratympanic steroid(ITS)injections.Objective This Systematic Review and Meta-Analysis intends to determine the effectiveness of ITS injections as an initial treatment,combined therapy,or salvage treatment for SSNHL compared to systemic steroids alone.Methods An inclusive study was performed through the PubMed,Scopus,Web of Science,and Cochrane Library databases,encompassing publications published from 2015 to 2024.Case-control studies,cohort studies,randomized controlled trials(RCTs),and systematic reviews were included.Two separate reviewers read the studies and extracted data on steroid administration methods,study outcomes,and risk of bias(ROB)using the Cochrane Collaboration Tool and RevMan 5.4.Results The review included 12 studies with varied designs,including prospective RCTs,systematic reviews,and retrospective analyses.Combined ITS and systemic steroid therapy showed superior hearing recovery compared to systemic steroids alone in several studies.ITS alone demonstrated variable efficacy,with some studies indicating benefits,particularly as a salvage therapy.ROB assessment revealed variability in methodological rigor,with studies like those of Li&Ding et al.(2020)and Devantier et al.(2022)showing low risk,while others exhibited higher risks,particularly in random sequence generation and allocation concealment.Conclusion The findings suggest that ITS injections,particularly when combined with systemic steroids,can enhance hearing recovery in SSNHL patients.However,variability in study outcomes and methodological quality underscores the need for standardized protocols and further high-quality RCTs.This analysis underscores the prospective advantages of ITS therapy while stressing the need for stringent study designs to enhance SSNHL treatment methodologies.展开更多
Objectives:Sudden sensorineural hearing loss(SSNHL)is a sudden onset condition of unknown etiology,often accompanied by tinnitus and vertigo,significantly affecting patients'quality of life.While some individuals ...Objectives:Sudden sensorineural hearing loss(SSNHL)is a sudden onset condition of unknown etiology,often accompanied by tinnitus and vertigo,significantly affecting patients'quality of life.While some individuals experience spontaneous recovery,the unpredictability of treatment outcomes and potential complications pose clinical challenges.Recent studies suggest a potential link between thyroid hormone levels-particularly free triiodothyronine(FT3)and thyroid-stimulating hormone(TSH)-and both the pathogenesis and prognosis of SSNHL.However,the precise mechanisms remain unclear.This study aims to investigate the correlation between thyroid hormone abnormalities and hearing recovery in SSNHL patients,providing insights to enhance diagnostic and therapeutic approaches.Methods:This prospective observational study included 66 SSNHL patients admitted to the Affiliated Hospital of Qingdao University between April 2024 and February 2025.Serum TSH and FT3 levels were measured using chemiluminescence immunoassay,while hearing function was evaluated through pure-tone audiometry following standard guidelines.The“posttreatment hearing gap(PTHG)”was introduced as a quantitative measure of treatment efficacy.All patients received comprehensive treatment,including glucocorticoid therapy and hyperbaric oxygen therapy,and were classified into effective and ineffective groups based on hearing recovery outcomes.Results:No significant differences in gender or comorbidities(tinnitus,ear fullness,dizziness)were observed between the abnormal thyroid function group(n=45)and the normal thyroid function group(n=21)(all p>0.05).Similarly,initial TSH and FT3 levels did not differ significantly among patients with varying degrees of hearing impairment(mild,moderate,severe)(p>0.05).However,during treatment,ΔFT3 demonstrated a significant negative correlation with PTHG(r=-0.739,p=0.002),suggesting that a greater increase inΔFT3 is associated with improved hearing recovery.Additionally,TSH levels showed a significant positive correlation withΔFT3(p<0.05).Conclusions:Abnormal thyroid function in SSNHL patients was not associated with the severity of hearing loss.However,dynamic changes in FT3 during treatment showed a significant correlation with therapeutic efficacy,suggesting that monitoringΔFT3 may have clinical value in prognostic assessment.Nonetheless,this study had a limited sample size,and the potential influence of hormone therapy on thyroid function was not accounted for.Therefore,the role of thyroid hormones in SSNHL warrants further validation through large-scale prospective studies and mechanistic investigations.展开更多
Objective: To study the relationship between cortical auditory evoked potential (CAEP) thresholds and behavioral thresholds in pediatric populations with sensorineural hearing loss (SNHL). Methods: Fifteen children (m...Objective: To study the relationship between cortical auditory evoked potential (CAEP) thresholds and behavioral thresholds in pediatric populations with sensorineural hearing loss (SNHL). Methods: Fifteen children (mean age 6.8 years) with bilateral SNHL underwent behavioral pure-tone audiometry and CAEP testing at 0.5, 1, 2, and 4 kHz. CAEP thresholds were determined using tone bursts, and correlations between CAEP and pure-tone thresholds were analyzed using Pearson correlation and t-tests. Results: A strong positive correlation was observed between P1 thresholds and behavioral thresholds across all test frequencies: 0.5 kHz (r = 0.765, p Conclusion: The strong correlation between P1 and behavioral thresholds demonstrates the reliability of CAEP testing for estimating auditory thresholds in children. These findings support the use of CAEP testing as a reliable objective tool for threshold estimation, particularly in cases where behavioral responses cannot be reliably obtained. When adjusted with frequency-specific correction values, CAEP testing provides a reliable method for assessing hearing thresholds in pediatric populations.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
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
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
Early intervention for sensorineural hearing loss(SNHL)in childhood is crucial for auditory and language development.In recent years,innovative auditory stimulation techniques and speech therapy strategies,such as mid...Early intervention for sensorineural hearing loss(SNHL)in childhood is crucial for auditory and language development.In recent years,innovative auditory stimulation techniques and speech therapy strategies,such as middle ear implants,cochlear implants,auditory brainstem implants,and midbrain implants,have provided new avenues for improving patient outcomes.Additionally,basic research advancements in cell reprogramming and regeneration,stem cell therapy,and targeted drug delivery offer promising approaches to meet the individualized needs of children with SNHL.However,many challenges and unresolved issues remain in the treatment of SNHL.This article comments on the case report,which describes a female pediatric patient with SNHL who underwent foot reflexology which led to the normalization of hearing thresholds.Reflexology is considered to have potential benefits in physical rehabilitation,but its efficacy in hearing restoration requires further scientific validation through rigorous clinical trials and large-scale prospective studies.展开更多
Background:Even though various research has demonstrated the connection between consanguinity and health issues,consanguineous marriage still common in Egypt with high prevalence rate 35.3%,23.5%in semi-urban and 17.7...Background:Even though various research has demonstrated the connection between consanguinity and health issues,consanguineous marriage still common in Egypt with high prevalence rate 35.3%,23.5%in semi-urban and 17.7%in urban areas.Methods:This study was conducted on 434native Arabic-speaking Egyptians children(3-10 years)who visited Special Needs Clinic at National Research Centre,among one year presenting with a hearing or/and language problem.They were subjected to psychometric assessment,Conners'Parent Rating Scales-Revised,Autism Diagnostic Interview Revised,Childhood Autism Rating Scale,audiological assessment,and Arabic Preschool Language Scale-4.Participants were classified into 6 subgroups according to the cause of language delay.We estimated the frequency and degree of consanguinity in each group and the association between consanguinity and hearing and language problems.Results:The prevalence of the consanguineous marriage in all participants(n=434)was31.6%with the mean inbreeding coefficient of 0.01208.First cousins'marriages were the most common type of consanguineous marriages(50.3%of consanguineous marriages).Among all participants in the 6 subgroups(n=434),language delay secondary to cognitive delay showed the highest percentage of consanguineous marriages(35%)followed by autism spectrum disorder(32.8%).No significant association between consanguinity and language problems.Among the 78 participants with sensorineural hearing loss,35.9%of consanguineous couples(no.=28 cases)were reported versus 64.1%of non-consanguineous couples(no.=50 cases).Conclusions:The Frequency of consanguineous marriages in our participants was 31.6%which was close to that reported in Egyptian population.Absence of significant association between consanguinity and language problems warrants further investigation and point to the role of genetic-environment interplay in cases of language delay.展开更多
Purpose To investigate the perinatal risk factors for conductive hearing loss(CHL)in infancy and develop an initial prediction model to facilitate accurate diagnosis and early detection of CHL.Method This retrospectiv...Purpose To investigate the perinatal risk factors for conductive hearing loss(CHL)in infancy and develop an initial prediction model to facilitate accurate diagnosis and early detection of CHL.Method This retrospective study utilized data from the Newborn Cohort Study of Hearing Loss(ChiCTR2100049765).Infants who underwent diagnostic audiological assessments at our hospital between January 2003 and June 2024 were included.Data analysis was conducted using R(version 4.4.1)to construct an initial prediction model for CHL in infancy,applying the LASSO regression technique.Results A total of 661 infants(1322 ears)were included,with 1253 ears in the normal hearing group and 69 ears in the CHL group.Statistically significant differences were observed between the groups in the following factors:parent-reported infant response to sound,craniofacial deformities,neonatal hemolysis,jaundice treatment,and neonatal hypoglycemia.A multivariate prediction model and nomogram for CHL in infancy were developed and validated,achieving an accuracy of 92.5%and a specificity of 91.3%.Conclusions This study identified key risk factors for CHL in infancy and developed a preliminary predictive model,improving the diagnostic accuracy for CHL.Improved diagnostic precision can decrease misdiagnoses,reduce delays in treatment,and limit unnecessary antimicrobial prescriptions for infants.展开更多
Sensorineural hearing loss(SNHL),the most commonly-occurring form of hearing loss,is caused mainly by injury to or the loss of hair cells and spiral ganglion neurons in the cochlea.Numerous environmental and physiolog...Sensorineural hearing loss(SNHL),the most commonly-occurring form of hearing loss,is caused mainly by injury to or the loss of hair cells and spiral ganglion neurons in the cochlea.Numerous environmental and physiological factors have been shown to cause acquired SNHL,such as ototoxic drugs,noise exposure,aging,infections,and diseases.Several programmed cell death(PCD)pathways have been reported to be involved in SNHL,especially some novel PCD pathways that have only recently been reported,such as ferroptosis,necroptosis,and pyroptosis.Here we summarize these PCD pathways and their roles and mechanisms in SNHL,aiming to provide new insights and potential therapeutic strategies for SNHL by targeting these PCD pathways.展开更多
ObjectivesThe PTPRQ gene is essential for preserving the structure and function of stereocilia in inner ear.However,research on splicing mutations within this gene is limited.This study aims to investigate novel splic...ObjectivesThe PTPRQ gene is essential for preserving the structure and function of stereocilia in inner ear.However,research on splicing mutations within this gene is limited.This study aims to investigate novel splicing mutations in PTPRQ,clarify their molecular mechanisms,and provide new insights into the genetic factors associated with hearing loss,ultimately enhancing diagnostic accuracy.MethodClinical data and peripheral blood samples were obtained from members of a family with congenital hearing loss.Variants were identified through high-throughput sequencing and confirmed by Sanger sequencing to ensure genealogical co-segregation.The splicing effects of PTPRQ variants were evaluated using bioinformatics tools and minigene assays.ResultsWe used whole exome sequencing to identify novel double compound heterozygous splice-altering variants(c.5426+1 G>A and c.6603-3 T>G)in the PTPRQ gene with DFNB84A.We molecularly characterized these variants,and they were found to co-segregate with the disease within the family.Minigene assays and Sanger sequencing confirmed that the c.6603-3 T>G variant caused exon 43 skipping,resulting in a frameshift mutation(p.Ser2201ArgfsTer112).Further bioinformatic analysis supported these findings.ConclusionsThis study identifies a novel compound heterozygous splicing variant in the PTPRQ gene in a Chinese family with DFNB84A,expanding the known spectrum of PTPRQ mutations.These findings enhance the understanding of PTPRQ-related hearing loss and may aid in early diagnosis,prevention,and therapeutic strategies.展开更多
BACKGROUND Otitis media with effusion(OME),glue ear,serous otitis media,or secretory otitis media is a common paediatric condition.Two widely used surgical interventions for OME are myringotomy alone and myringotomy w...BACKGROUND Otitis media with effusion(OME),glue ear,serous otitis media,or secretory otitis media is a common paediatric condition.Two widely used surgical interventions for OME are myringotomy alone and myringotomy with tympanostomy tube.While both procedures aim to improve hearing outcomes,the efficacy of these approaches has been a subject of ongoing research and debate.AIM To compare the efficacy of myringotomy alone and myringotomy with tympa-nostomy tube.METHODS In this comparative study,66 patients diagnosed as OME meeting the inclusion criteria were selected via ear,nose and throat department.They were divided into two groups randomly,each of 33 patients.In the first group(Group A)myrin-gotomy alone was performed while in the second group(Group B)myringotomy with tympanostomy tube placement was performed.We observed hearing outcome by pure tone audiogram(PTA)pre operatively.Patients were followed up and re-assessed in outdoor patient department at 4th week postoperatively again by PTA.RESULTS The mean age of the patients in Group A was 10.96±2.76 SD but the mean age of the patients in Group B was 10.22±2.73 SD(P=0.1056).In Group A,males were 63.6%and females were 36.3%.Also in Group B,males were 63.6%and females were 36.3%(P=1.0).In group A,post operative hearing gain using pure tone audiometry at one month was 20.45±3.78 SD while in group B,post operative hearing gain using pure tone audiometry at one month was 23.84±3.69 SD(P=0.00005).However,ear discharge was noted in 3.03%cases in group A and 15.15%cases in group B(P=0.035).By applying independent t-test,the P<0.05 indicated that there is a significant association between Group B and hearing improvement at 4th week.CONCLUSION Our study concluded that myringotomy with tympanostomy tube seems to have better hearing results than myringotomy alone in treatment of OME.展开更多
Hearing loss is one of the most prevalent sensory disorders affecting the human nervous system.Liquid–liquid phase separation(LLPS)is a physiological process that facilitates the reversible and dynamic assembly of bi...Hearing loss is one of the most prevalent sensory disorders affecting the human nervous system.Liquid–liquid phase separation(LLPS)is a physiological process that facilitates the reversible and dynamic assembly of biomolecular condensates.Increasing evidence suggests that LLPS plays a significant role in the pathogenesis of hereditary hearing loss.Nevertheless,there is a conspicuous lack of systematic investigations exploring the impact of LLPS abnormalities on the etiology of hereditary hearing loss.In this review,we examine the mechanisms by which dysfunctions in LLPS contribute to hereditary hearing loss,specifically focusing on its effects on mechanoelectrical transduction in hair bundles,transcriptional regulation,post-transcriptional modifications,the actin cytoskeleton,ion homeostasis within the inner ear,and energy and redox homeostasis.Furthermore,we evaluate the considerable potential of targeting LLPS as a therapeutic approach for hearing loss and propose innovative perspectives on LLPS that may guide future research initiatives in the field of auditory disorders.展开更多
BACKGROUND Managing refractory sudden sensorineural hearing loss(RSSHL)in patients with diabetes mellitus(DM)presents significant therapeutic challenges,highlighting the importance of identifying effective treatment s...BACKGROUND Managing refractory sudden sensorineural hearing loss(RSSHL)in patients with diabetes mellitus(DM)presents significant therapeutic challenges,highlighting the importance of identifying effective treatment strategies.AIM To analyze the therapeutic effectiveness of intratympanic injection plus retroauricular injection for RSSHL complicated with DM.METHODS This study included 84 patients with RSSHL complicated with DM from April 2021 to April 2024,all receiving routine treatment.Participants were categorized into the control group(40 cases),receiving an intratympanic injection of methylprednisolone sodium succinate(MPSS),and the research group(44 cases),treated with retroauricular MPSS injection the next day in addition to the treatment administered in the control group.The efficacy,adverse reactions(tympanic membrane perforation,middle ear infections,burning sensation,vertigo,and tinnitus),blood glucose(BG)[fasting BG(FBG),2-hour postprandial BG(2hPBG),and glycosylated hemoglobin(HbA1c)],hearing thresholds at different frequencies(250 Hz,500 Hz,and 1000 Hz),serum biochemical indexes[interleukin(IL)-6,C-reactive protein(CRP),and procalcitonin(PCT)],and quality of life assessed by the short-form 36 item health survey(SF-36)were comparatively analyzed.RESULTS The research group demonstrated a markedly higher total effectiveness rate(81.82%vs 60.00%,P=0.027)and a comparable incidence of total adverse reactions than the control group.Further,the research group exhibited notably reduced FBG,2hPBG,HbA1c,IL-6,CRP,and PCT post-treatment(P<0.01),which were lower compared with the pre-treatment levels and the control group(P<0.05),as well as reduced hearing thresholds at different frequencies(250 Hz,500 Hz,and 1000 Hz,P<0.05).Furthermore,the post-treatment SF-36 scores of the research group in terms of energy,social functioning,role functioning,physical functioning,mental health,and overall health were all significantly improved than the pre-treatment levels and the control group(P<0.05).CONCLUSION The above results indicate that intratympanic plus retroauricular injections of MPSS are effective in treating RSSHL complicated with DM without increasing the incidence of adverse reactions,which has a health promotion value.展开更多
Introduction:Hearing loss,particularly congenital hearing loss,poses significant challenges to affected individuals and their families.Recent advancements in regenerative medicine have fueled interest in stem cell the...Introduction:Hearing loss,particularly congenital hearing loss,poses significant challenges to affected individuals and their families.Recent advancements in regenerative medicine have fueled interest in stem cell therapy as a potential solution for hearing restoration.This review discusses the feasibility of using stem cells to regenerate damaged cochlear structures and auditory neurons,focusing on their differentiation potential and integration into the cochlear environment.Discussion:Congenital hearing loss remains a significant global health challenge,with genetic mutations and syndromic conditions contributing to its high prevalence.Advances in regenerative medicine have led to increased interest in stem cell therapy as a potential solution for auditory restoration.Research on embryonic stem cells(ESCs),induced pluripotent stem cells(i PSCs),and mesenchymal stem cells(MSCs)has demonstrated their ability to differentiate into auditory cell types,offering a potential avenue for cochlear regeneration.Gene therapies also present a promising approach for addressing hereditary hearing loss by targeting specific genetic mutations.i PSCs in particular show promise,especially when combined with gene therapy for hereditary hearing loss.Despite encouraging preclinical findings,persistent hurdles remain:immune rejection,limited cell survival,difficulty with cochlear integration,and complex ethical and regulatory considerations.Addressing these barriers is crucial for safe and effective translation into clinical care.In Pakistan,there is a critical need to promote advancements in this field.Positioning Pakistan as an illustrative case within broader low-and middle-income country(LMIC)disparities underscores how limited infrastructure,funding gaps,and weak regulatory frameworks constrain progress and highlight opportunities for regional collaboration.Conclusion:As stem cell research advances,it is crucial to address ethical and regulatory considerations to ensure the responsible translation of these therapies into clinical practice.By integrating stem cell therapy with gene editing techniques and biomaterial scaffolding,the future of regenerative treatment for congenital hearing loss holds transformative potential.展开更多
Vibration cutting has emerged as a promising method for creating surface functional microstructures.However,achieving precise tool setting is a time-consuming process that significantly impacts process efficiency.This...Vibration cutting has emerged as a promising method for creating surface functional microstructures.However,achieving precise tool setting is a time-consuming process that significantly impacts process efficiency.This study proposes an intelligent approach for tool setting in vibration cutting using machine vision and hearing,divided into two steps.In the first step,machine vision is employed to achieve rough precision in tool setting within tens of micrometers.Subsequently,in the second step,machine hearing utilizes sound pickup to capture vibration audio signals,enabling fine tool adjustment within 1μm precision.The relationship between the spectral intensity of vibration audio and cutting depth is analyzed to establish criteria for tool–workpiece contact.Finally,the efficacy of this approach is validated on an ultra-precision platform,demonstrating that the automated tool-setting process takes no more than 74 s.The total cost of the vision and hearing sensors is less than$1500.展开更多
Hearing loss,which currently affects more than 430 million individuals globally and is projected to exceed 700 million by 2050,predominantly manifests as sensorineural hearing loss(SNHL),for which existing technologie...Hearing loss,which currently affects more than 430 million individuals globally and is projected to exceed 700 million by 2050,predominantly manifests as sensorineural hearing loss(SNHL),for which existing technologies such as hearing aids and cochlear implants fail to restore natural auditory function.Research focusing on protecting inner ear hair cells(HCs)from harmful factors through the regulation of epigenetic modifications has gained significant attention in otology for its role in regulating gene expression without altering the DNA sequence,suggesting potential strategies for preventing and treating SNHL.By synthesizing relevant studies on the inner ear,this review summarizes the emerging roles of histone modifications,DNA methylation,and noncoding RNAs in HC damage,with a focus on their therapeutic potential through epigenetic modulation.Moreover,this review examines the therapeutic potential of epigenetic regulation for the prevention and treatment of SNHL,emphasizing the application of small-molecule epigenetic compounds and their efficacy in modulating gene expression to preserve and restore auditory function.展开更多
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This editorial critically evaluates the application of foot reflexology as a treatment for sensorineural hearing loss(SNHL)in infants,as proposed in a case report published in the World of Clinical Cases.SNHL is a condition characterized by damage to the cochlea or the neural pathways that transmit auditory information to the brain.The etiology of SNHL is often complex,involving genetic mutations,prenatal factors,or perinatal insults.Reflexology,an alternative therapy involving the application of pressure to specific points on the feet,is based on the hypothesis that these points correspond to different organs and systems in the body,including the auditory system.However,the biological plausibility and clinical efficacy of foot reflexology in addressing SNHL lack empirical support.This editorial examines the pathophysiology of SNHL,assesses the clinical claims of reflexology practitioners,and emphasizes the necessity of evidence-based approaches in treating infant hearing loss.While complementary therapies may provide ancillary benefits,they should not supplant validated medical treatments in managing SNHL in infants.Further research is needed to evaluate the safety and efficacy of foot reflexology and other alternative therapies in pediatric audiology.
基金support from the UCL GRS/ORS scholarshipUCL Fellowship Incubator Award+9 种基金supported by the NIHR Cambridge Biomedical Research Centre(NIHR203312)funded by the Royal National Institute for Deaf People(RNID,G100138)funded by the Rosetrees Trust Enterprise Fellowship(EF2020100099)RNID Flexigrant(F112)Wellcome Trust Developing Concept Fund(RG93172/BANCE/40181)by the Evelyn Trustfunded by the Woolf Fisher Trust,New Zealandthe Cambridge Commonwealth,European,&International Trustby Trinity CollegeUniversity of Cambridge。
文摘The cochlea is one of the most complex organs in the human body,exhibiting a complex interplay of characteristics in acoustic,mechanical,electrical,and biological functions.Functional cochlea models are an essential platform for studying hearing mechanics and are crucial for developing next-generation auditory prostheses and artificial hearing systems for sensorineural hearing restoration.Recent advances in additive manufacturing,organ-on-a-chip models,drug delivery platforms,and artificial intelligence have provided valuable insights into how to manufacture artificial cochlea models that more accurately replicate the complex anatomy and physiology of the inner ear.This paper reviews recent advancements in the applications of advanced manufacturing techniques in reproducing the physical,biological,and intelligent functions of the cochlea.It also outlines the current challenges to developing mechanically,electrically,and anatomically accurate functional models of the inner ear.Finally,this review identifies the major requirements and outlook for impactful research in this field going forward.Through interdisciplinary collaboration and innovation,these functional cochlea models are poised to drive significant advancements in hearing treatments,and ultimately enhance the quality of life for individuals with hearing loss.
文摘Background Sudden sensorineural hearing loss(SSNHL)is a rapid condition of hearing impairment,typically occurring over 72 hours and affecting the inner ear or auditory nerve pathways.The etiology of SSNHL remains largely idiopathic,with potential causes including viral infections,vascular occlusions,and autoimmune disorders.Traditional treatment primarily involves systemic corticosteroids,but their efficacy is inconsistent,leading to exploring alternative and adjunctive therapies such as intratympanic steroid(ITS)injections.Objective This Systematic Review and Meta-Analysis intends to determine the effectiveness of ITS injections as an initial treatment,combined therapy,or salvage treatment for SSNHL compared to systemic steroids alone.Methods An inclusive study was performed through the PubMed,Scopus,Web of Science,and Cochrane Library databases,encompassing publications published from 2015 to 2024.Case-control studies,cohort studies,randomized controlled trials(RCTs),and systematic reviews were included.Two separate reviewers read the studies and extracted data on steroid administration methods,study outcomes,and risk of bias(ROB)using the Cochrane Collaboration Tool and RevMan 5.4.Results The review included 12 studies with varied designs,including prospective RCTs,systematic reviews,and retrospective analyses.Combined ITS and systemic steroid therapy showed superior hearing recovery compared to systemic steroids alone in several studies.ITS alone demonstrated variable efficacy,with some studies indicating benefits,particularly as a salvage therapy.ROB assessment revealed variability in methodological rigor,with studies like those of Li&Ding et al.(2020)and Devantier et al.(2022)showing low risk,while others exhibited higher risks,particularly in random sequence generation and allocation concealment.Conclusion The findings suggest that ITS injections,particularly when combined with systemic steroids,can enhance hearing recovery in SSNHL patients.However,variability in study outcomes and methodological quality underscores the need for standardized protocols and further high-quality RCTs.This analysis underscores the prospective advantages of ITS therapy while stressing the need for stringent study designs to enhance SSNHL treatment methodologies.
文摘Objectives:Sudden sensorineural hearing loss(SSNHL)is a sudden onset condition of unknown etiology,often accompanied by tinnitus and vertigo,significantly affecting patients'quality of life.While some individuals experience spontaneous recovery,the unpredictability of treatment outcomes and potential complications pose clinical challenges.Recent studies suggest a potential link between thyroid hormone levels-particularly free triiodothyronine(FT3)and thyroid-stimulating hormone(TSH)-and both the pathogenesis and prognosis of SSNHL.However,the precise mechanisms remain unclear.This study aims to investigate the correlation between thyroid hormone abnormalities and hearing recovery in SSNHL patients,providing insights to enhance diagnostic and therapeutic approaches.Methods:This prospective observational study included 66 SSNHL patients admitted to the Affiliated Hospital of Qingdao University between April 2024 and February 2025.Serum TSH and FT3 levels were measured using chemiluminescence immunoassay,while hearing function was evaluated through pure-tone audiometry following standard guidelines.The“posttreatment hearing gap(PTHG)”was introduced as a quantitative measure of treatment efficacy.All patients received comprehensive treatment,including glucocorticoid therapy and hyperbaric oxygen therapy,and were classified into effective and ineffective groups based on hearing recovery outcomes.Results:No significant differences in gender or comorbidities(tinnitus,ear fullness,dizziness)were observed between the abnormal thyroid function group(n=45)and the normal thyroid function group(n=21)(all p>0.05).Similarly,initial TSH and FT3 levels did not differ significantly among patients with varying degrees of hearing impairment(mild,moderate,severe)(p>0.05).However,during treatment,ΔFT3 demonstrated a significant negative correlation with PTHG(r=-0.739,p=0.002),suggesting that a greater increase inΔFT3 is associated with improved hearing recovery.Additionally,TSH levels showed a significant positive correlation withΔFT3(p<0.05).Conclusions:Abnormal thyroid function in SSNHL patients was not associated with the severity of hearing loss.However,dynamic changes in FT3 during treatment showed a significant correlation with therapeutic efficacy,suggesting that monitoringΔFT3 may have clinical value in prognostic assessment.Nonetheless,this study had a limited sample size,and the potential influence of hormone therapy on thyroid function was not accounted for.Therefore,the role of thyroid hormones in SSNHL warrants further validation through large-scale prospective studies and mechanistic investigations.
文摘Objective: To study the relationship between cortical auditory evoked potential (CAEP) thresholds and behavioral thresholds in pediatric populations with sensorineural hearing loss (SNHL). Methods: Fifteen children (mean age 6.8 years) with bilateral SNHL underwent behavioral pure-tone audiometry and CAEP testing at 0.5, 1, 2, and 4 kHz. CAEP thresholds were determined using tone bursts, and correlations between CAEP and pure-tone thresholds were analyzed using Pearson correlation and t-tests. Results: A strong positive correlation was observed between P1 thresholds and behavioral thresholds across all test frequencies: 0.5 kHz (r = 0.765, p Conclusion: The strong correlation between P1 and behavioral thresholds demonstrates the reliability of CAEP testing for estimating auditory thresholds in children. These findings support the use of CAEP testing as a reliable objective tool for threshold estimation, particularly in cases where behavioral responses cannot be reliably obtained. When adjusted with frequency-specific correction values, CAEP testing provides a reliable method for assessing hearing thresholds in pediatric populations.
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 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.
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Early intervention for sensorineural hearing loss(SNHL)in childhood is crucial for auditory and language development.In recent years,innovative auditory stimulation techniques and speech therapy strategies,such as middle ear implants,cochlear implants,auditory brainstem implants,and midbrain implants,have provided new avenues for improving patient outcomes.Additionally,basic research advancements in cell reprogramming and regeneration,stem cell therapy,and targeted drug delivery offer promising approaches to meet the individualized needs of children with SNHL.However,many challenges and unresolved issues remain in the treatment of SNHL.This article comments on the case report,which describes a female pediatric patient with SNHL who underwent foot reflexology which led to the normalization of hearing thresholds.Reflexology is considered to have potential benefits in physical rehabilitation,but its efficacy in hearing restoration requires further scientific validation through rigorous clinical trials and large-scale prospective studies.
文摘Background:Even though various research has demonstrated the connection between consanguinity and health issues,consanguineous marriage still common in Egypt with high prevalence rate 35.3%,23.5%in semi-urban and 17.7%in urban areas.Methods:This study was conducted on 434native Arabic-speaking Egyptians children(3-10 years)who visited Special Needs Clinic at National Research Centre,among one year presenting with a hearing or/and language problem.They were subjected to psychometric assessment,Conners'Parent Rating Scales-Revised,Autism Diagnostic Interview Revised,Childhood Autism Rating Scale,audiological assessment,and Arabic Preschool Language Scale-4.Participants were classified into 6 subgroups according to the cause of language delay.We estimated the frequency and degree of consanguinity in each group and the association between consanguinity and hearing and language problems.Results:The prevalence of the consanguineous marriage in all participants(n=434)was31.6%with the mean inbreeding coefficient of 0.01208.First cousins'marriages were the most common type of consanguineous marriages(50.3%of consanguineous marriages).Among all participants in the 6 subgroups(n=434),language delay secondary to cognitive delay showed the highest percentage of consanguineous marriages(35%)followed by autism spectrum disorder(32.8%).No significant association between consanguinity and language problems.Among the 78 participants with sensorineural hearing loss,35.9%of consanguineous couples(no.=28 cases)were reported versus 64.1%of non-consanguineous couples(no.=50 cases).Conclusions:The Frequency of consanguineous marriages in our participants was 31.6%which was close to that reported in Egyptian population.Absence of significant association between consanguinity and language problems warrants further investigation and point to the role of genetic-environment interplay in cases of language delay.
基金supported by the grants of the National Key Research and Development Program of China(Grant No.2023YFC2508400)the National Natural Science Foundation of China(Grant No.82350005).
文摘Purpose To investigate the perinatal risk factors for conductive hearing loss(CHL)in infancy and develop an initial prediction model to facilitate accurate diagnosis and early detection of CHL.Method This retrospective study utilized data from the Newborn Cohort Study of Hearing Loss(ChiCTR2100049765).Infants who underwent diagnostic audiological assessments at our hospital between January 2003 and June 2024 were included.Data analysis was conducted using R(version 4.4.1)to construct an initial prediction model for CHL in infancy,applying the LASSO regression technique.Results A total of 661 infants(1322 ears)were included,with 1253 ears in the normal hearing group and 69 ears in the CHL group.Statistically significant differences were observed between the groups in the following factors:parent-reported infant response to sound,craniofacial deformities,neonatal hemolysis,jaundice treatment,and neonatal hypoglycemia.A multivariate prediction model and nomogram for CHL in infancy were developed and validated,achieving an accuracy of 92.5%and a specificity of 91.3%.Conclusions This study identified key risk factors for CHL in infancy and developed a preliminary predictive model,improving the diagnostic accuracy for CHL.Improved diagnostic precision can decrease misdiagnoses,reduce delays in treatment,and limit unnecessary antimicrobial prescriptions for infants.
基金supported by grants from the National Key Research and Development Program of China(2023YFA1801804,2022YFA0807000,2021YFA1101300,2021YFA1101800,and 2020YFA0112503)the National Natural Science Foundation of China(82171149,82371166,81970892,82330033,82030029,92149304,82071053,and 82171144)+5 种基金the Shenzhen Science and Technology Program(JCYJ20230807114700001,JCYJ20210324125608022,and JCYJ20190814093401920)the Guangdong Basic and Applied Basic Research Foundation(2024A1515010548)the Science and Technology Department of Sichuan Province(2021YFS0371)the Jiangsu Provincial Scientific Research Center of Applied Mathematics(BK20233002)the Open Research Fund of Guangdong Academy of Medical Sciences(YKY‐KF202201)the Taishan Scholars Project-Young Experts Program of Shandong Province(tsqn202211357).
文摘Sensorineural hearing loss(SNHL),the most commonly-occurring form of hearing loss,is caused mainly by injury to or the loss of hair cells and spiral ganglion neurons in the cochlea.Numerous environmental and physiological factors have been shown to cause acquired SNHL,such as ototoxic drugs,noise exposure,aging,infections,and diseases.Several programmed cell death(PCD)pathways have been reported to be involved in SNHL,especially some novel PCD pathways that have only recently been reported,such as ferroptosis,necroptosis,and pyroptosis.Here we summarize these PCD pathways and their roles and mechanisms in SNHL,aiming to provide new insights and potential therapeutic strategies for SNHL by targeting these PCD pathways.
基金supported in part by the Natural Science Foundation of Shandong Province(no.ZR2022QH373,ZR2022QH292 and ZR2023MH2474).
文摘ObjectivesThe PTPRQ gene is essential for preserving the structure and function of stereocilia in inner ear.However,research on splicing mutations within this gene is limited.This study aims to investigate novel splicing mutations in PTPRQ,clarify their molecular mechanisms,and provide new insights into the genetic factors associated with hearing loss,ultimately enhancing diagnostic accuracy.MethodClinical data and peripheral blood samples were obtained from members of a family with congenital hearing loss.Variants were identified through high-throughput sequencing and confirmed by Sanger sequencing to ensure genealogical co-segregation.The splicing effects of PTPRQ variants were evaluated using bioinformatics tools and minigene assays.ResultsWe used whole exome sequencing to identify novel double compound heterozygous splice-altering variants(c.5426+1 G>A and c.6603-3 T>G)in the PTPRQ gene with DFNB84A.We molecularly characterized these variants,and they were found to co-segregate with the disease within the family.Minigene assays and Sanger sequencing confirmed that the c.6603-3 T>G variant caused exon 43 skipping,resulting in a frameshift mutation(p.Ser2201ArgfsTer112).Further bioinformatic analysis supported these findings.ConclusionsThis study identifies a novel compound heterozygous splicing variant in the PTPRQ gene in a Chinese family with DFNB84A,expanding the known spectrum of PTPRQ mutations.These findings enhance the understanding of PTPRQ-related hearing loss and may aid in early diagnosis,prevention,and therapeutic strategies.
文摘BACKGROUND Otitis media with effusion(OME),glue ear,serous otitis media,or secretory otitis media is a common paediatric condition.Two widely used surgical interventions for OME are myringotomy alone and myringotomy with tympanostomy tube.While both procedures aim to improve hearing outcomes,the efficacy of these approaches has been a subject of ongoing research and debate.AIM To compare the efficacy of myringotomy alone and myringotomy with tympa-nostomy tube.METHODS In this comparative study,66 patients diagnosed as OME meeting the inclusion criteria were selected via ear,nose and throat department.They were divided into two groups randomly,each of 33 patients.In the first group(Group A)myrin-gotomy alone was performed while in the second group(Group B)myringotomy with tympanostomy tube placement was performed.We observed hearing outcome by pure tone audiogram(PTA)pre operatively.Patients were followed up and re-assessed in outdoor patient department at 4th week postoperatively again by PTA.RESULTS The mean age of the patients in Group A was 10.96±2.76 SD but the mean age of the patients in Group B was 10.22±2.73 SD(P=0.1056).In Group A,males were 63.6%and females were 36.3%.Also in Group B,males were 63.6%and females were 36.3%(P=1.0).In group A,post operative hearing gain using pure tone audiometry at one month was 20.45±3.78 SD while in group B,post operative hearing gain using pure tone audiometry at one month was 23.84±3.69 SD(P=0.00005).However,ear discharge was noted in 3.03%cases in group A and 15.15%cases in group B(P=0.035).By applying independent t-test,the P<0.05 indicated that there is a significant association between Group B and hearing improvement at 4th week.CONCLUSION Our study concluded that myringotomy with tympanostomy tube seems to have better hearing results than myringotomy alone in treatment of OME.
基金supported by the National Natural Science Foundation of China(82430035)the Foundation for Innovative Research Groups of Hubei Province(2023AFA038)+1 种基金the National Key Research and Development Program of China(2021YFF0702303,2024YFC2511101,and 2023YFE0203200)the Fundamental Research Funds for the Central Universities(2024BRA019).
文摘Hearing loss is one of the most prevalent sensory disorders affecting the human nervous system.Liquid–liquid phase separation(LLPS)is a physiological process that facilitates the reversible and dynamic assembly of biomolecular condensates.Increasing evidence suggests that LLPS plays a significant role in the pathogenesis of hereditary hearing loss.Nevertheless,there is a conspicuous lack of systematic investigations exploring the impact of LLPS abnormalities on the etiology of hereditary hearing loss.In this review,we examine the mechanisms by which dysfunctions in LLPS contribute to hereditary hearing loss,specifically focusing on its effects on mechanoelectrical transduction in hair bundles,transcriptional regulation,post-transcriptional modifications,the actin cytoskeleton,ion homeostasis within the inner ear,and energy and redox homeostasis.Furthermore,we evaluate the considerable potential of targeting LLPS as a therapeutic approach for hearing loss and propose innovative perspectives on LLPS that may guide future research initiatives in the field of auditory disorders.
基金Supported by 2023 Science and Technology Innovation Program(Key Laboratory of Sleep Respiratory Disorders)in Huai'an,No.HAP202304。
文摘BACKGROUND Managing refractory sudden sensorineural hearing loss(RSSHL)in patients with diabetes mellitus(DM)presents significant therapeutic challenges,highlighting the importance of identifying effective treatment strategies.AIM To analyze the therapeutic effectiveness of intratympanic injection plus retroauricular injection for RSSHL complicated with DM.METHODS This study included 84 patients with RSSHL complicated with DM from April 2021 to April 2024,all receiving routine treatment.Participants were categorized into the control group(40 cases),receiving an intratympanic injection of methylprednisolone sodium succinate(MPSS),and the research group(44 cases),treated with retroauricular MPSS injection the next day in addition to the treatment administered in the control group.The efficacy,adverse reactions(tympanic membrane perforation,middle ear infections,burning sensation,vertigo,and tinnitus),blood glucose(BG)[fasting BG(FBG),2-hour postprandial BG(2hPBG),and glycosylated hemoglobin(HbA1c)],hearing thresholds at different frequencies(250 Hz,500 Hz,and 1000 Hz),serum biochemical indexes[interleukin(IL)-6,C-reactive protein(CRP),and procalcitonin(PCT)],and quality of life assessed by the short-form 36 item health survey(SF-36)were comparatively analyzed.RESULTS The research group demonstrated a markedly higher total effectiveness rate(81.82%vs 60.00%,P=0.027)and a comparable incidence of total adverse reactions than the control group.Further,the research group exhibited notably reduced FBG,2hPBG,HbA1c,IL-6,CRP,and PCT post-treatment(P<0.01),which were lower compared with the pre-treatment levels and the control group(P<0.05),as well as reduced hearing thresholds at different frequencies(250 Hz,500 Hz,and 1000 Hz,P<0.05).Furthermore,the post-treatment SF-36 scores of the research group in terms of energy,social functioning,role functioning,physical functioning,mental health,and overall health were all significantly improved than the pre-treatment levels and the control group(P<0.05).CONCLUSION The above results indicate that intratympanic plus retroauricular injections of MPSS are effective in treating RSSHL complicated with DM without increasing the incidence of adverse reactions,which has a health promotion value.
文摘Introduction:Hearing loss,particularly congenital hearing loss,poses significant challenges to affected individuals and their families.Recent advancements in regenerative medicine have fueled interest in stem cell therapy as a potential solution for hearing restoration.This review discusses the feasibility of using stem cells to regenerate damaged cochlear structures and auditory neurons,focusing on their differentiation potential and integration into the cochlear environment.Discussion:Congenital hearing loss remains a significant global health challenge,with genetic mutations and syndromic conditions contributing to its high prevalence.Advances in regenerative medicine have led to increased interest in stem cell therapy as a potential solution for auditory restoration.Research on embryonic stem cells(ESCs),induced pluripotent stem cells(i PSCs),and mesenchymal stem cells(MSCs)has demonstrated their ability to differentiate into auditory cell types,offering a potential avenue for cochlear regeneration.Gene therapies also present a promising approach for addressing hereditary hearing loss by targeting specific genetic mutations.i PSCs in particular show promise,especially when combined with gene therapy for hereditary hearing loss.Despite encouraging preclinical findings,persistent hurdles remain:immune rejection,limited cell survival,difficulty with cochlear integration,and complex ethical and regulatory considerations.Addressing these barriers is crucial for safe and effective translation into clinical care.In Pakistan,there is a critical need to promote advancements in this field.Positioning Pakistan as an illustrative case within broader low-and middle-income country(LMIC)disparities underscores how limited infrastructure,funding gaps,and weak regulatory frameworks constrain progress and highlight opportunities for regional collaboration.Conclusion:As stem cell research advances,it is crucial to address ethical and regulatory considerations to ensure the responsible translation of these therapies into clinical practice.By integrating stem cell therapy with gene editing techniques and biomaterial scaffolding,the future of regenerative treatment for congenital hearing loss holds transformative potential.
基金the financial support for this research provided by the National Natural Science Foundation of China(Grant Nos.52275470,124115301,and 52105458)the Natural Science Foundation of Beijing(Grant No.3222009).
文摘Vibration cutting has emerged as a promising method for creating surface functional microstructures.However,achieving precise tool setting is a time-consuming process that significantly impacts process efficiency.This study proposes an intelligent approach for tool setting in vibration cutting using machine vision and hearing,divided into two steps.In the first step,machine vision is employed to achieve rough precision in tool setting within tens of micrometers.Subsequently,in the second step,machine hearing utilizes sound pickup to capture vibration audio signals,enabling fine tool adjustment within 1μm precision.The relationship between the spectral intensity of vibration audio and cutting depth is analyzed to establish criteria for tool–workpiece contact.Finally,the efficacy of this approach is validated on an ultra-precision platform,demonstrating that the automated tool-setting process takes no more than 74 s.The total cost of the vision and hearing sensors is less than$1500.
基金supported by the National Natural Science Foundation of China(Nos.82271158,82301312,82071045,82101219,82071048).
文摘Hearing loss,which currently affects more than 430 million individuals globally and is projected to exceed 700 million by 2050,predominantly manifests as sensorineural hearing loss(SNHL),for which existing technologies such as hearing aids and cochlear implants fail to restore natural auditory function.Research focusing on protecting inner ear hair cells(HCs)from harmful factors through the regulation of epigenetic modifications has gained significant attention in otology for its role in regulating gene expression without altering the DNA sequence,suggesting potential strategies for preventing and treating SNHL.By synthesizing relevant studies on the inner ear,this review summarizes the emerging roles of histone modifications,DNA methylation,and noncoding RNAs in HC damage,with a focus on their therapeutic potential through epigenetic modulation.Moreover,this review examines the therapeutic potential of epigenetic regulation for the prevention and treatment of SNHL,emphasizing the application of small-molecule epigenetic compounds and their efficacy in modulating gene expression to preserve and restore auditory function.
