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
Blast injuries are common among the military service members and veterans.One of the devastating effects of blast wave induced TBI is either temporary or permanent hearing loss.Treating hearing loss using minocycline ...Blast injuries are common among the military service members and veterans.One of the devastating effects of blast wave induced TBI is either temporary or permanent hearing loss.Treating hearing loss using minocycline is restricted by optimal drug concentration,route of administration,and its half-life.Therefore,therapeutic approach using novel therapeutic delivery method is in great need.Among the different delivery methods,nanotechnology-based drug delivery is desirable,which can achieve longer systemic circulation,pass through some biological barriers and specifically targets desired sites.The current study aimed to examine therapeutic effect of minocycline and its nanoparticle formulation in moderate blast induced hearing loss rat model through central auditory system.The I.v.administered nanoparticle at reduced dose and frequency than regularly administered toxic dose.After moderate blast exposure,rats had hearing impairment as determined by ABR at 7-and 30-days post exposure.In chronic condition,free minocycline also showed the significant reduction in ABR threshold.In central auditory system,it is found in this study that minocycline nanoparticles ameliorate excitation in inferior colliculus;and astrocytes and microglia activation after the blast exposure is reduced by minocycline nanoparticles administration.The study demonstrated that in moderate blast induced hearing loss,minocycline and its nanoparticle formulation exhibited the optimal therapeutic effect on the recovery of the ABR impairment and a protective effect through central auditory system.In conclusion,targeted and non-targeted nanoparticle formulation have therapeutic effect on blast induced hearing loss.展开更多
Background Sudden sensorineural hearing loss(SSNHL)is a common disease in otology,and steroids play an important role in its treatment.Steroids can be administered systemically or locally,and the efficacies of differe...Background Sudden sensorineural hearing loss(SSNHL)is a common disease in otology,and steroids play an important role in its treatment.Steroids can be administered systemically or locally,and the efficacies of different administration routes remain controversial.Methods We searched the Cochrane,EMBASE,PubMed,Web of Science,CNKI,Wanfang and Weipu databases for randomized controlled trials(RCTs)on glucocorticoid treatments for SSNHL to compare the efficacy of topical and systemic steroid administration.The Review Manager 5.4 software was used for synthesis of data:the rate of reported hearing improvement and change in pure-tone audiometry(PTA).Results In all the included studies,when intratympanic administration was compared to systemic therapies,the risk difference(RD)using reported hearing improvement as an outcome measure was 0.08(95%CI:0.01–0.14,I2=45%).Using PTA changes as an outcome measure in 4 studies,the mean difference(MD)was 10.43 dB(95%CI:3.68–17.18,I2=81%).Hearing improvement RD was also compared among different types of steroid,recovery criteria,follow-up times and diagnostic criteria,and showed no significant differences exception for recovery criteria(>10 dB)(RD-0.06,95%CI:0.14-0.2,I2=0%).Conclusion As the initial treatment for SSNHL,topical steroids seem to be superior to systemic steroid administration,especially in patients with contraindications to systemic steroids usage.However,further verification based on high-quality research is needed.展开更多
Objective:To evaluate the efficacy and safety of intratympanic and systemic steroid therapies in the initial treatment of Sudden Sensorineural Hearing Loss (SSNHL) patients. Methods:A comprehensive search of PubMed, ...Objective:To evaluate the efficacy and safety of intratympanic and systemic steroid therapies in the initial treatment of Sudden Sensorineural Hearing Loss (SSNHL) patients. Methods:A comprehensive search of PubMed, Wanfang database and CNKI (China National Knowledge Infrastructure) was performed covering the period from January 1990 to July 2014. A meta-analysis was conducted after filtering by the criteria of Cochrane Collaboration. Three hundred fifty six subjects in nine studies allocated to the group of intratympanic steroid therapies and 343 controls receiving systemic steroid therapies met the criteria for meta-analysis. The data were extracted and analyzed using the RevMan 5.3 meta-analysis software. Results: The total effectiveness rate in SSNHL patients receiving intratympanic steroid therapies did not differ statistically from patients receiving systemic therapies (RR ? 1.08, 95%CI ? 0.99e1.99, P ? 0.10), although the rate of full hearing recovery in this group differed significantly from patients receiving systemic therapies (RR ? 1.29, 95%CI ? 1.00e1.66, P ? 0.05). Conclusion: Local steroid therapy appears to generate higher rate of complete hearing recovery than systemic steroid treatment as an initial treatment for SSNHL, which may be especially useful for patients in whom systemic steroids are contraindicated.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
Although congenital sensorineural hearing loss (SHL) in the bilateral cochleae mainly results from genetic abnormalities, chronic SHL progressing in later life is often influenced by systemic immune disturbances, incl...Although congenital sensorineural hearing loss (SHL) in the bilateral cochleae mainly results from genetic abnormalities, chronic SHL progressing in later life is often influenced by systemic immune disturbances, including autoimmunity, chronic inflammation, and immunosenescence. We have investigated the relationship between the inner ear and systemic immunity and reviewed the possibilities to prevent SHL, including autoimmune SHL and age-related SHL. We also demonstrated two lymphocyte populations, interleukin 1 receptor type II (IL-1R2)-positive T cells (T1R2) and naturally occurring regulatory T cells (nTregs) in CD4+ T cells, which increase with aging, suppress host immune function and promote organ degeneration. Alterations in systemic immunity by fewer microbial antigen challenges in the living environment, elimination of immune suppressive lymphocytes, or immune rejuvenation with a reconstituted thymus may contribute not only to renew the cochlear function in SHL, but also to extend the healthy life of functional organs in a vigorous and youthful body, one of humanity’s greatest dreams.展开更多
Background: Lassa fever is a viral haemorrhagic fever associated with several complications which include hearing loss. Hearing loss in Lassa fever may become residual with its negative impact on the quality of life. ...Background: Lassa fever is a viral haemorrhagic fever associated with several complications which include hearing loss. Hearing loss in Lassa fever may become residual with its negative impact on the quality of life. Purpose: This paper is a review of various studies on Lassa fever related hearing loss. Methods: Systematic literature review was done on articles on Lassa fever patients and Lassa fever survivors. Relevant articles published in English between 1969 and 2021 were searched for using Google Scholar and PubMed databases. Eight studies which met the inclusion criteria were finally selected and included in the review. The variables of interest were extracted from the full-text articles. Results: Lassa fever related hearing loss occurs in both sexes and in all age groups. The incidence of self-reported hearing loss ranged from 12.5% to 30% with a mean of 17.6%. The incidence of audiometrically determined sensorineural hearing loss ranged from 13.5% to 29% with a mean of 22.1%. Hearing loss persisted in 64% of those that were followed up. Cases of hearing loss varied in severity, ranging from mild to profound. No successful specific treatment for Lassa fever related hearing loss was reported apart from hearing rehabilitation. Conclusion: Hearing loss among Lassa fever patients remains a major challenge. Lack of effective treatment for Lassa fever related hearing loss is a serious gap in the management of Lassa fever patients which should be addressed in future research.展开更多
Telemedicine is defined as a system that allows healthcare workers to deliver their services and consultations to the patients remotely without the need of their physical presence within academic institutions, hospita...Telemedicine is defined as a system that allows healthcare workers to deliver their services and consultations to the patients remotely without the need of their physical presence within academic institutions, hospitals, and medical clinics aiming to reduce the personal contact and limiting it to the need only. The field of telemedicine is growing every day and facilitating more flexible services for patients around the world especially during COVID-19 pandemic. Methods: This systematic review was conducted according to the Cochrane Handbook for Systematic Reviews. Database search was made on several databases including PubMed/Medline, Scopus, Web of Science, Publons, EMBASE, and Google Scholar using the relevant keywords. Inclusion criteria for articles in our study included the following original research (primary research articles), addressing the efficacy of teleaudiology for hearing aids programming and satisfaction of patients. Results: Seven studies were included in this systematic review article. Included studies were assessed using Cochrane handbook guidelines. Conclusions: The results of this work based on the currently available literature denote that remote programming for the hearing aids using modern technology is effective and provide comparable results with the standard face-to-face clinic programming, even for patients with no previous experience in hearing aids fitting in either of in-person physical presence programming or remotely over the internet using teleconferencing which is known as teleaudiology. Most of the results were positive and support the continuity to develop better facilities to improve the teleaudiology to be an essential part of hearing aids programming with its different types. Minimal results provided negative impact from the participating patients, this resulted because of the non-upgraded infrastructure and facilities of the audiology clinic or the personal computer of the patient in his home/ workplace or both sides don’t have enough specifications to smoothly perform this modern approach.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
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
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
Avian vocal communication represents one of the most intricate forms of animal language,playing a critical role in behavioral interactions.Both peripheral and central auditory-vocal pathways are essential for precisel...Avian vocal communication represents one of the most intricate forms of animal language,playing a critical role in behavioral interactions.Both peripheral and central auditory-vocal pathways are essential for precisely integrating acoustic signals,ensuring effective communication.Like humans,songbirds exhibit vocal learning behaviors supported by complex neural mechanisms.However,unlike most mammals,songbirds possess the remarkable ability to regenerate damaged auditory cells.These capabilities offer unique opportunities to explore how birds adjust their vocal behavior and auditory processing in response to dynamic environmental conditions.Recent studies have advanced our understanding of the plasticity of avian vocal communication system,yet the vocal diversity and neurophysiological mechanisms underlying vocalization and hearing have often been examined independently.A comprehensive overview of how these systems interact and adapt in birds remains lacking.To address this gap,this review synthesizes the peripheral and central features of avian vocalization and hearing,while also exploring the mechanisms that drive the remarkable plasticity of these systems.Furthermore,it explores seasonal variations in bird vocalization and hearing and adaptations to environmental noise,focusing on how hormonal,neural,and ecological factors together shape vocal behavior and auditory sensitivity.Avian vocal communication systems present an exceptional model for studying the integration of peripheral and central vocal-auditory pathways and their adaptive responses to ever-changing environments.This review underscores the dynamic interactions between avian vocal communication systems and environmental stimuli,offering new insights into broader principles of sensory processing,and neuroplasticity.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
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
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
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.
基金funding provided by Faculty seed grant (FSG) from NJIT, Newark, USA " (NC and Venkatesan Perumal).
文摘Blast injuries are common among the military service members and veterans.One of the devastating effects of blast wave induced TBI is either temporary or permanent hearing loss.Treating hearing loss using minocycline is restricted by optimal drug concentration,route of administration,and its half-life.Therefore,therapeutic approach using novel therapeutic delivery method is in great need.Among the different delivery methods,nanotechnology-based drug delivery is desirable,which can achieve longer systemic circulation,pass through some biological barriers and specifically targets desired sites.The current study aimed to examine therapeutic effect of minocycline and its nanoparticle formulation in moderate blast induced hearing loss rat model through central auditory system.The I.v.administered nanoparticle at reduced dose and frequency than regularly administered toxic dose.After moderate blast exposure,rats had hearing impairment as determined by ABR at 7-and 30-days post exposure.In chronic condition,free minocycline also showed the significant reduction in ABR threshold.In central auditory system,it is found in this study that minocycline nanoparticles ameliorate excitation in inferior colliculus;and astrocytes and microglia activation after the blast exposure is reduced by minocycline nanoparticles administration.The study demonstrated that in moderate blast induced hearing loss,minocycline and its nanoparticle formulation exhibited the optimal therapeutic effect on the recovery of the ABR impairment and a protective effect through central auditory system.In conclusion,targeted and non-targeted nanoparticle formulation have therapeutic effect on blast induced hearing loss.
文摘Background Sudden sensorineural hearing loss(SSNHL)is a common disease in otology,and steroids play an important role in its treatment.Steroids can be administered systemically or locally,and the efficacies of different administration routes remain controversial.Methods We searched the Cochrane,EMBASE,PubMed,Web of Science,CNKI,Wanfang and Weipu databases for randomized controlled trials(RCTs)on glucocorticoid treatments for SSNHL to compare the efficacy of topical and systemic steroid administration.The Review Manager 5.4 software was used for synthesis of data:the rate of reported hearing improvement and change in pure-tone audiometry(PTA).Results In all the included studies,when intratympanic administration was compared to systemic therapies,the risk difference(RD)using reported hearing improvement as an outcome measure was 0.08(95%CI:0.01–0.14,I2=45%).Using PTA changes as an outcome measure in 4 studies,the mean difference(MD)was 10.43 dB(95%CI:3.68–17.18,I2=81%).Hearing improvement RD was also compared among different types of steroid,recovery criteria,follow-up times and diagnostic criteria,and showed no significant differences exception for recovery criteria(>10 dB)(RD-0.06,95%CI:0.14-0.2,I2=0%).Conclusion As the initial treatment for SSNHL,topical steroids seem to be superior to systemic steroid administration,especially in patients with contraindications to systemic steroids usage.However,further verification based on high-quality research is needed.
文摘Objective:To evaluate the efficacy and safety of intratympanic and systemic steroid therapies in the initial treatment of Sudden Sensorineural Hearing Loss (SSNHL) patients. Methods:A comprehensive search of PubMed, Wanfang database and CNKI (China National Knowledge Infrastructure) was performed covering the period from January 1990 to July 2014. A meta-analysis was conducted after filtering by the criteria of Cochrane Collaboration. Three hundred fifty six subjects in nine studies allocated to the group of intratympanic steroid therapies and 343 controls receiving systemic steroid therapies met the criteria for meta-analysis. The data were extracted and analyzed using the RevMan 5.3 meta-analysis software. Results: The total effectiveness rate in SSNHL patients receiving intratympanic steroid therapies did not differ statistically from patients receiving systemic therapies (RR ? 1.08, 95%CI ? 0.99e1.99, P ? 0.10), although the rate of full hearing recovery in this group differed significantly from patients receiving systemic therapies (RR ? 1.29, 95%CI ? 1.00e1.66, P ? 0.05). Conclusion: Local steroid therapy appears to generate higher rate of complete hearing recovery than systemic steroid treatment as an initial treatment for SSNHL, which may be especially useful for patients in whom systemic steroids are contraindicated.
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Although congenital sensorineural hearing loss (SHL) in the bilateral cochleae mainly results from genetic abnormalities, chronic SHL progressing in later life is often influenced by systemic immune disturbances, including autoimmunity, chronic inflammation, and immunosenescence. We have investigated the relationship between the inner ear and systemic immunity and reviewed the possibilities to prevent SHL, including autoimmune SHL and age-related SHL. We also demonstrated two lymphocyte populations, interleukin 1 receptor type II (IL-1R2)-positive T cells (T1R2) and naturally occurring regulatory T cells (nTregs) in CD4+ T cells, which increase with aging, suppress host immune function and promote organ degeneration. Alterations in systemic immunity by fewer microbial antigen challenges in the living environment, elimination of immune suppressive lymphocytes, or immune rejuvenation with a reconstituted thymus may contribute not only to renew the cochlear function in SHL, but also to extend the healthy life of functional organs in a vigorous and youthful body, one of humanity’s greatest dreams.
文摘Background: Lassa fever is a viral haemorrhagic fever associated with several complications which include hearing loss. Hearing loss in Lassa fever may become residual with its negative impact on the quality of life. Purpose: This paper is a review of various studies on Lassa fever related hearing loss. Methods: Systematic literature review was done on articles on Lassa fever patients and Lassa fever survivors. Relevant articles published in English between 1969 and 2021 were searched for using Google Scholar and PubMed databases. Eight studies which met the inclusion criteria were finally selected and included in the review. The variables of interest were extracted from the full-text articles. Results: Lassa fever related hearing loss occurs in both sexes and in all age groups. The incidence of self-reported hearing loss ranged from 12.5% to 30% with a mean of 17.6%. The incidence of audiometrically determined sensorineural hearing loss ranged from 13.5% to 29% with a mean of 22.1%. Hearing loss persisted in 64% of those that were followed up. Cases of hearing loss varied in severity, ranging from mild to profound. No successful specific treatment for Lassa fever related hearing loss was reported apart from hearing rehabilitation. Conclusion: Hearing loss among Lassa fever patients remains a major challenge. Lack of effective treatment for Lassa fever related hearing loss is a serious gap in the management of Lassa fever patients which should be addressed in future research.
文摘Telemedicine is defined as a system that allows healthcare workers to deliver their services and consultations to the patients remotely without the need of their physical presence within academic institutions, hospitals, and medical clinics aiming to reduce the personal contact and limiting it to the need only. The field of telemedicine is growing every day and facilitating more flexible services for patients around the world especially during COVID-19 pandemic. Methods: This systematic review was conducted according to the Cochrane Handbook for Systematic Reviews. Database search was made on several databases including PubMed/Medline, Scopus, Web of Science, Publons, EMBASE, and Google Scholar using the relevant keywords. Inclusion criteria for articles in our study included the following original research (primary research articles), addressing the efficacy of teleaudiology for hearing aids programming and satisfaction of patients. Results: Seven studies were included in this systematic review article. Included studies were assessed using Cochrane handbook guidelines. Conclusions: The results of this work based on the currently available literature denote that remote programming for the hearing aids using modern technology is effective and provide comparable results with the standard face-to-face clinic programming, even for patients with no previous experience in hearing aids fitting in either of in-person physical presence programming or remotely over the internet using teleconferencing which is known as teleaudiology. Most of the results were positive and support the continuity to develop better facilities to improve the teleaudiology to be an essential part of hearing aids programming with its different types. Minimal results provided negative impact from the participating patients, this resulted because of the non-upgraded infrastructure and facilities of the audiology clinic or the personal computer of the patient in his home/ workplace or both sides don’t have enough specifications to smoothly perform this modern approach.
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.
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 National Natural Science Foundation of China(NSFC,32471572)to D.L.the NSFC(32401298)the Hebei Natural Science Foundation(C2023205016)to L.W。
文摘Avian vocal communication represents one of the most intricate forms of animal language,playing a critical role in behavioral interactions.Both peripheral and central auditory-vocal pathways are essential for precisely integrating acoustic signals,ensuring effective communication.Like humans,songbirds exhibit vocal learning behaviors supported by complex neural mechanisms.However,unlike most mammals,songbirds possess the remarkable ability to regenerate damaged auditory cells.These capabilities offer unique opportunities to explore how birds adjust their vocal behavior and auditory processing in response to dynamic environmental conditions.Recent studies have advanced our understanding of the plasticity of avian vocal communication system,yet the vocal diversity and neurophysiological mechanisms underlying vocalization and hearing have often been examined independently.A comprehensive overview of how these systems interact and adapt in birds remains lacking.To address this gap,this review synthesizes the peripheral and central features of avian vocalization and hearing,while also exploring the mechanisms that drive the remarkable plasticity of these systems.Furthermore,it explores seasonal variations in bird vocalization and hearing and adaptations to environmental noise,focusing on how hormonal,neural,and ecological factors together shape vocal behavior and auditory sensitivity.Avian vocal communication systems present an exceptional model for studying the integration of peripheral and central vocal-auditory pathways and their adaptive responses to ever-changing environments.This review underscores the dynamic interactions between avian vocal communication systems and environmental stimuli,offering new insights into broader principles of sensory processing,and neuroplasticity.
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.
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.
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.