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
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
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
BACKGROUND Our study contributes to the further understanding of the mechanism of foot reflexology.Foot reflexology has been reported to affect hearing recovery,but no physiological evidence has been provided.This lac...BACKGROUND Our study contributes to the further understanding of the mechanism of foot reflexology.Foot reflexology has been reported to affect hearing recovery,but no physiological evidence has been provided.This lack of evidence hampers the acceptance of the technique in clinical practice.CASE SUMMARY A girl was taken to North Sichuan Medical University Affiliated Hospital for a hearing screen by her parents.Her parents reported that her hearing level was the same as when she was born.The girl was diagnosed with sensorineural hearing loss(SNHL)by a doctor in the otolaryngology department.After we introduced the foot reflexology project,the parents agreed to participate in the experiment.After 6 months of foot reflexology treatment,the hearing threshold of the girl recovered to a normal level,below 30 dB.CONCLUSION Foot reflexology should be encouraged in clinical practice and for families of infants with SNHL.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
Background: Sudden sensorineural hearing loss(SSNHL) is a prevalent emergency in ear, nose, and throat practice. Previous studies have demonstrated that intratympanic steroid therapy(IST) can serve as a salvage treatm...Background: Sudden sensorineural hearing loss(SSNHL) is a prevalent emergency in ear, nose, and throat practice. Previous studies have demonstrated that intratympanic steroid therapy(IST) can serve as a salvage treatment for SSNHL after the failure of systemic steroid therapy(SST).Objective: This study aimed to analyze the efficacy of modified IST involving the insertion of a tympanic tube and gelfoam as a salvage treatment for patients with SSNHL, and to explore its associated factors.Methods: Totally, 74 patients who were aged 22–81 years with SSNHL were enrolled and allocated to either the control group(n = 25) or the treatment group(n = 49) based on their treatment modalities. All patients received SST lasting for at least 7 days. Subsequently, patients in the treatment group, after SST failure, underwent IST twice a week for 2–6 weeks, while the control group did not. Efficacy was assessed by the improvement in pure tone average at the affected frequency at the beginning and end of IST.Results: Hearing improvement in all patients after IST in the treatment group was 9.71 ± 14.84 dB, with significant improvement at affected frequencies(250-8000 Hz) compared with the control group(P < 0.05). The findings indicated the duration from the onset of SSNHL to the beginning of IST as an independent factor for pure tone average improvement after treatment(P = 0.002), whereas age, duration of SST, and time of IST were not(P > 0.05).Conclusion: The modified IST was demonstrated to be a safe and effective method as a salvage treatment for SSNHL. This study explored the efficacy of a modified IST approach, incorporating the utilization of tympanic tubes and gelfoam as key components. The findings underscore the advantages of gelfoam as a strategic drug carrier placed in the round window niche. By minimizing drug loss, extending action time, and increasing perilymph concentration, gelfoam enhances the therapeutic impact of IST, contributing to improved hearing outcomes in patients with SSNHL.展开更多
Objective:The aim of this study was to examine angiotensin converting enzyme(ACE)insertion/deletion,alpha adducin,and interleukin-10(IL-10)gene polymorphisms(GPs)in terms of both idiopathic sudden sensorineural hearin...Objective:The aim of this study was to examine angiotensin converting enzyme(ACE)insertion/deletion,alpha adducin,and interleukin-10(IL-10)gene polymorphisms(GPs)in terms of both idiopathic sudden sensorineural hearing loss(ISSNHL)risk and their potential prognostic effects.Methods:The study group consisted of 70 patients and the control group consisted of 50 patients.Venous blood samples were analyzed for relevant GPs via kompetitive allele-specific polymerase chain reaction.Age,sex,affected side,tinnitus,and vertiginous symptom status,number of days between symptom onset and hospital admission,pure tone audiometry results at admission and after treatment were included in the study.Data were compared statistically.Results:The D allele of ACE insertion/deletion GP was significantly more frequent in patients with ISSNHL than in the control group(p=0.032).II genotype was associated with a reduced risk of ISSNHL(p=0.036).The amount of hearing loss was significantly higher in patients with the TT genotype(p=0.027)and T allele of the IL-10 GP(p=0.035)than in the patients without this allele.Severe hearing loss was a poor prognostic factor(p=0.008).Conclusions:The D allele of ACE insertion/deletion GP may be involved in the ISSNHL etiology.Due to the association of this allele with occlusive vascular pathologies,ischemia is believed to be a common pathway in the etiopathogenesis of ISSNHL.展开更多
Currently,treatment options for infant sensorineural hearing loss(SNHL)are limited.This article describes a novel case of SNHL in an infant successfully treated with foot reflexology,along with observed brain activity...Currently,treatment options for infant sensorineural hearing loss(SNHL)are limited.This article describes a novel case of SNHL in an infant successfully treated with foot reflexology,along with observed brain activity changes before and after treatment,as indicated by functional magnetic resonance imaging.Hence,this commentary discusses the case and our viewpoints regarding foot reflexology for treating SNHL.展开更多
BACKGROUND Numerous studies have found that patients experiencing sudden sensorineural hearing loss(SSHL),with or without accompanying vertigo,often show impaired vestibular function.However,there is a dearth of studi...BACKGROUND Numerous studies have found that patients experiencing sudden sensorineural hearing loss(SSHL),with or without accompanying vertigo,often show impaired vestibular function.However,there is a dearth of studies analyzing vestibularevoked myogenic potentials(VEMPs)in SSHL patients across various age groups.AIM To investigate vestibular condition in SSHL patients across various age demographics.METHODS Clinical data of 84 SSHL patients were investigated retrospectively.Audiometry,cervical vestibular evoked myogenic potentials(c-VEMPs),and ocular vestibular evoked myogenic potentials(o-VEMPs)were conducted on these patients.Parameters assessed included the latencies of P1 and N1 waves,as well as the amplitudes of P1–N1 waves.Moreover,the study evaluated the influence of factors such as sex,affected side,configuration of hearing loss,and presence of accompanying vertigo.RESULTS Among the 84 SSHL patients,no significant differences were observed among the three groups in terms of gender,affected side,and the presence or absence of vertigo.Group II(aged 41–60 years)had the highest number of SSHL cases.The rates of absent o-VEMPs in the affected ears were 20.83%,31.58%,and 22.72%for the three age groups,respectively,with no statistically significant difference among them.The rates of absent c-VEMPs in the affected ears were 8.3%,34.21%,and 18.18%for the three age groups,respectively,with significant differences.In the unaffected ears,there were differences observed in the extraction rates of o-VEMPs in the unaffected ears among the age groups.In the three age groups,no significant differences were noted in the three age groups in the latencies of P1 and N1 waves or in the amplitude of N1–P1 waves for c-VEMPs and o-VEMPs,either on the affected side or on the unaffected side,across the three age groups.CONCLUSION The extraction rate of VEMPs is more valuable than parameters.Regardless of the presence of vertigo,vestibular organs are involved in SSHL.Notably,SSHL patients aged 41–60 appear more susceptible to damage to the inferior vestibular nerve and saccule.展开更多
BACKGROUND Understanding the impact of early sensory deficits on brain development is essential for understanding developmental processes and developing potential interventions.While previous studies have looked into ...BACKGROUND Understanding the impact of early sensory deficits on brain development is essential for understanding developmental processes and developing potential interventions.While previous studies have looked into the impact of prenatal experiences on language development,there is a lack of research on how these experiences affect early language and brain function development in individuals with sensorineural hearing loss(SNHL).AIM To investigate SNHL effects on early brain development and connectivity in 4-month-olds vs healthy newborns and controls.METHODS The research involved analyzing the functional brain networks of 65 infants,categorized into three groups:28 healthy newborns,224-month-old participants with SNHL,and 15 age-matched healthy participants.The resting-state functional connectivity was measured and compared between the groups using functional near-infrared spectroscopy and graph theory to assess the brain network properties.RESULTS Significant differences were found in resting-state functional connectivity between participants with SNHL and age-matched controls,indicating a developmental lag in brain connectivity for those with SNHL.Surprisingly,SNHL participants showed better connectivity development compared to healthy newborns,with connectivity strengths of 0.13±0.04 for SNHL,0.16±0.08 for controls,and 0.098±0.04 for newborns.Graph theory analysis revealed enhanced global brain network properties for the SNHL group,suggesting higher communication efficiency at 4 months.No significant differences were noted in network properties between 4-month-old SNHL participants and neonates.A unique pattern of central hubs was observed in the SNHL group,with 2 hubs in the left hemisphere compared to 6 in controls.CONCLUSION 4-month-old infants with SNHL have a distinct brain network pattern with efficient long-distance information transmission but less effective local communication compared to age-matched controls.展开更多
BACKGROUND Sudden sensorineural hearing loss(SSNHL),characterized by a rapid and unexplained loss of hearing,particularly at moderate to high frequencies,presents a significant clinical challenge.The therapeutic use o...BACKGROUND Sudden sensorineural hearing loss(SSNHL),characterized by a rapid and unexplained loss of hearing,particularly at moderate to high frequencies,presents a significant clinical challenge.The therapeutic use of methylprednisolone sodium succinate(MPSS)via different administration routes,in combination with conventional medications,remains a topic of interest.AIM To compare the therapeutic efficacy of MPSS administered via different routes in combination with conventional drugs for the treatment of mid-to high-frequency SSNHL.METHODS The medical records of 109 patients with mid-to high-frequency SSNHL were analyzed.The patients were divided into three groups based on the route of administration:Group A[intratympanic(IT)injection of MPSS combined with mecobalamin and Ginkgo biloba leaf extract injection],Group B(intravenous injection of MPSS combined with mecobalamin and Ginkgo biloba leaf extract injection),and Group C(single IT injection of MPSS).The intervention effects were compared and analyzed.RESULTS The posttreatment auditory thresholds in Group A(21.23±3.34)were significantly lower than those in Groups B(28.52±3.36)and C(30.23±4.21;P<0.05).Group A also exhibited a significantly greater speech recognition rate(92.23±5.34)than Groups B and C.The disappearance time of tinnitus,time to hearing recovery,and disappearance time of vertigo in Group A were significantly shorter than those in Groups B and C(P<0.05).The total effective rate in Group A(97.56%)was significantly greater than that in Groups B and C(77.14%and 78.79%,χ^(2)=7.898,P=0.019).Moreover,the incidence of adverse reactions in Groups A and C was significantly lower than that in Group B(4.88%,3.03%vs 2.57%,χ^(2)=11.443,P=0.003),and the recurrence rate in Group A was significantly lower than that in Groups B and C(2.44%vs 20.00%vs 21.21%,χ^(2)=7.120,P=0.028).CONCLUSION IT injection of MPSS combined with conventional treatment demonstrates superior efficacy and safety compared to systemic administration via intravenous infusion and a single IT injection of MPSS.This approach effectively improves patients'hearing and reduces the risk of disease recurrence.展开更多
Hereditary hearing loss(HHL),a genetic disorder that impairs auditory function,significantly affects quality of life and incurs substantial economic losses for society.To investigate the underlying causes of HHL and e...Hereditary hearing loss(HHL),a genetic disorder that impairs auditory function,significantly affects quality of life and incurs substantial economic losses for society.To investigate the underlying causes of HHL and evaluate therapeutic outcomes,appropriate animal models are necessary.Pigs have been extensively used as valuable large animal models in biomedical research.In this review,we highlight the advantages of pig models in terms of ear anatomy,inner ear morphology,and electrophysiological characteristics,as well as recent advancements in the development of distinct genetically modified porcine models of hearing loss.Additionally,we discuss the prospects,challenges,and recommendations regarding the use pig models in HHL research.Overall,this review provides insights and perspectives for future studies on HHL using porcine models.展开更多
Sudden sensorineural hearing loss(SSNHL)is an enigmatic entity,with obscure pathophysiology and debatable efficacy of the treatment agents used.An underlying cause is identified in only 10-15%of cases.The management o...Sudden sensorineural hearing loss(SSNHL)is an enigmatic entity,with obscure pathophysiology and debatable efficacy of the treatment agents used.An underlying cause is identified in only 10-15%of cases.The management of the remaining patients,classified as‘idiopathic’,is empirical,and is conventionally with systemic steroids,vasodilator therapy,rheological agents,and antioxidants,to list a few amongst the host of the agents employed for the treatment.The availability of conflicting outcomes and lack of conclusive evidence has resulted in the propagation of consensus-based treatment protocols.In the present review,we discuss the various controversial issues and newer developments in the management of idiopathic SSNHL.The current review aims to present a narrative outlook of the updated evidence base available from PUBMED,augmented with relevant designated publications.展开更多
Acute sensorineural hearing loss is an uncommon phenomenon in dentistry.We describe the case of a 79-year-old male who presented with acute sensorineural hearing loss occurring 2 days after a tooth extraction procedur...Acute sensorineural hearing loss is an uncommon phenomenon in dentistry.We describe the case of a 79-year-old male who presented with acute sensorineural hearing loss occurring 2 days after a tooth extraction procedure under local anesthesia.Possible mechanisms are discussed.He was treated with vasodilators(Ginaton and Alprostadil Injection)and Mecobalamin injection with benefit.High dose oral steroids(1 mg/kg)and low molecular weight dextran were used.展开更多
Objective Hyperbaric oxygen treatment(HBOT)has demonstrated efficacy in improving hearing levels of patients with idiopathic sudden sensorineural hearing loss(ISSHL);however,the underlying mechanisms are not well unde...Objective Hyperbaric oxygen treatment(HBOT)has demonstrated efficacy in improving hearing levels of patients with idiopathic sudden sensorineural hearing loss(ISSHL);however,the underlying mechanisms are not well understood.HBOT alleviates the inflammatory response,which is mediated by Toll-like receptor(TLR)4 and nuclear factor(NF)-κB.In this study we investigated whether HBOT attenuates inflammation in ISHHL patients via alteration of TLR4 and NF-κB expression.Methods ISHHL patients(n=120)and healthy control subjects(n=20)were enrolled in this study.Patients were randomly divided into medicine group treated with medicine only(n=60)and HBO group receiving both HBOT and medicine(n=60).Audiometric testing was performed pre-and posttreatment.TLR4,NF-кB,and TNF-αexpression in peripheral blood of ISSHL patients and healthy control subjects was assessed by ELISA before and after treatment.Results TLR4,NF-κB,and TNF-αlevels were upregulated in ISSHL patients relative to healthy control subjects;the levels were decreased following treatment and were lower in the HBO group than that in the medicine group post-treatment(P<0.05 and P<0.01).Conclusion HBOT alleviates hearing loss in ISSHL patients by suppressing the inflammatory response induced by TLR4 and NF-κB signaling.展开更多
Objective:To investigate immune-related genetic background in bilateral sudden sensorineural hearing loss (SSNHL). Case report and methods: The case is a 45-year-old man presenting with a 7-year history of bilateral p...Objective:To investigate immune-related genetic background in bilateral sudden sensorineural hearing loss (SSNHL). Case report and methods: The case is a 45-year-old man presenting with a 7-year history of bilateral profound SSNHL. Blood biochemical testing demonstrated increased levels of total cholesterol (5.88 mmol/L). Tests for hepatitis B showed a positive antibody against the hepatitis B core antigen. Complement C3 was below the normal value, and complement C4 and IgG were in the lower range of normal values. CT images showed a normal inner ear and vestibular aqueduct but round window membranous ossification on both sides. A total number of 232 immune-associated genes were sequenced using the next generation sequencing technique. Results: Mutations were detected in 5 genes, including the phosphoinositide 3-kinase catalytic subunit delta (PIK3CD), caspase recruitment domain-containing protein 9 (CARD9), complement factor H-related (CFHR2), immunoglobulin lambda-like polypeptide 1 Protein (IGLL1), and transmembrane channel-like gene family 8 (TMC8). In the PIK3CD gene, a C896T substitute in exon 7 was detected. This mutation causes primary immunodeficiency and is an autosomal dominant disease. Conclusion: The PIK3CD C896T mutation responsible for primary immunodeficiency may contribute to the onset of bilateral SSNHL with subsequent rapid progression.展开更多
Objectives:To evaluate hearing outcome of salvage treatment with intratympanic steroids(ITS)in idiopathic sudden sensorineural hearing loss(ISSNHL)refractory to initial systemic steroid(SS)therapy.Material and methods...Objectives:To evaluate hearing outcome of salvage treatment with intratympanic steroids(ITS)in idiopathic sudden sensorineural hearing loss(ISSNHL)refractory to initial systemic steroid(SS)therapy.Material and methods:A retrospective medical chart review was conducted on 54 consecutive patients with ISSNHL refractory to SS.Salvage treatment with a low dose intratympanic dexamethasone(4 mg/ml)was offered after one week of primary treatment.Patients were divided into two groups:25 patients accepted ITS(treatment group)and 29 patients did not undergo additional treatment(control group).A pure tone average(PTA)gain of at least 10 dB was considered hearing improvement.Results:Hearing improvement rate was higher in ITS group compared to control group(40%vs.13.8%,p=0.035).A mean PTA improvement of 8.6±9.8 dB was observed in the ITS group and,whereas the control group had an average hearing gain of 0.7±2 dB(p<0.001).Audiometric analysis revealed a significant hearing gain in ITS group at all tested frequencies compared to control group(p<0.05).Analysis of the selected variables,identified intratympanic steroid treatment as the only independent prognostic factor for hearing improvement(OR=4.2,95%CI:1.1e15.7;p=0.04).Conclusion:Intratympanic low dose dexamethasone is effective in patients with incomplete hearing recovery after primary systemic steroid treatment.展开更多
BACKGROUND As sudden sensorineural hearing loss(SSNHL)rarely occurs in pregnant women,there is a lack of knowledge and relevant research on its management.AIM To investigate the effect of intratympanic dexamethasone i...BACKGROUND As sudden sensorineural hearing loss(SSNHL)rarely occurs in pregnant women,there is a lack of knowledge and relevant research on its management.AIM To investigate the effect of intratympanic dexamethasone injection in the treatment of pregnant patients with SSNHL.METHODS A retrospective chart review was made for the period between June 2017 and August 2019 at our Department of Otorhinolaryngology-Head and Neck Surgery.Pregnant women who met the criteria for SSNHL were included and grouped based on the therapeutic modalities.The treatment group received intratympanic dexamethasone(2.5 mg)q.o.d.for a total of four times,while the control group received no medication other than bed rest and medical observations.All the patients were under close care of obstetricians.Pure-tone audiograms were performed before and after treatment.RESULTS Eleven patients who met the inclusion criteria were assigned to the treatment group(n=7)and the control group(n=4).The mean age of patients was 31.2±3.8 years;the right ear was affected in seven(63.64%)cases.Two patients(18.2%)suffered from vertigo,10(90.9%)suffered from tinnitus and 6(54.5%)suffered from aural fullness.The time from onset to clinic visit was relatively short,with a mean time of 1.3±0.9 d.All the women were within the second or third trimester;the average gestation period was 26.0±6.2 wk.The pure-tone averages at onset between the two groups were similar.After one wk of therapy,the treatment group had a curative rate of 57.1%and a significantly better hearing threshold and greater improvement compared to the control group(all P<0.05).Some patients experienced transient discomfort from intratympanic injections that disappeared after getting rest,while none had permanent complications.All patients delivered healthy full-term neonates with an average Apgar score of 9.7±0.5.CONCLUSION Intratympanic dexamethasone injections can be used as a first-line therapy in pregnant women with SSNHL.展开更多
In this article,the mechanism of inheritance behind inherited hearing loss and genetic susceptibilityin noise-induced hearing loss are reviewed.Conventional treatments for sensorineural hearing loss(SNHL),i.e.hearing ...In this article,the mechanism of inheritance behind inherited hearing loss and genetic susceptibilityin noise-induced hearing loss are reviewed.Conventional treatments for sensorineural hearing loss(SNHL),i.e.hearing aid and cochlear implant,are effective for some cases,but not without limitations.For example,they provide little benefit for patients of profound SNHL or neural hearing loss,especially when the hearing loss is in poor dynamic range and with low frequency resolution.We emphasize the most recent evidence-based treatment in this field,which includes gene therapy and allotransplantation of stem cells.Their promising results have shown that they might be options of treatment for profound SNHL and neural hearing loss.Although some treatments are still at the experimental stage,it is helpful to be aware of the novel therapies and endeavour to explore the feasibility of their clinical application.展开更多
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.
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.
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.
基金Graduate Student Project of Xi’an International Studies University,No.2021BS012Nanchong City-Universities Project,No.22SXCXTD0004.
文摘BACKGROUND Our study contributes to the further understanding of the mechanism of foot reflexology.Foot reflexology has been reported to affect hearing recovery,but no physiological evidence has been provided.This lack of evidence hampers the acceptance of the technique in clinical practice.CASE SUMMARY A girl was taken to North Sichuan Medical University Affiliated Hospital for a hearing screen by her parents.Her parents reported that her hearing level was the same as when she was born.The girl was diagnosed with sensorineural hearing loss(SNHL)by a doctor in the otolaryngology department.After we introduced the foot reflexology project,the parents agreed to participate in the experiment.After 6 months of foot reflexology treatment,the hearing threshold of the girl recovered to a normal level,below 30 dB.CONCLUSION Foot reflexology should be encouraged in clinical practice and for families of infants with SNHL.
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Background: Sudden sensorineural hearing loss(SSNHL) is a prevalent emergency in ear, nose, and throat practice. Previous studies have demonstrated that intratympanic steroid therapy(IST) can serve as a salvage treatment for SSNHL after the failure of systemic steroid therapy(SST).Objective: This study aimed to analyze the efficacy of modified IST involving the insertion of a tympanic tube and gelfoam as a salvage treatment for patients with SSNHL, and to explore its associated factors.Methods: Totally, 74 patients who were aged 22–81 years with SSNHL were enrolled and allocated to either the control group(n = 25) or the treatment group(n = 49) based on their treatment modalities. All patients received SST lasting for at least 7 days. Subsequently, patients in the treatment group, after SST failure, underwent IST twice a week for 2–6 weeks, while the control group did not. Efficacy was assessed by the improvement in pure tone average at the affected frequency at the beginning and end of IST.Results: Hearing improvement in all patients after IST in the treatment group was 9.71 ± 14.84 dB, with significant improvement at affected frequencies(250-8000 Hz) compared with the control group(P < 0.05). The findings indicated the duration from the onset of SSNHL to the beginning of IST as an independent factor for pure tone average improvement after treatment(P = 0.002), whereas age, duration of SST, and time of IST were not(P > 0.05).Conclusion: The modified IST was demonstrated to be a safe and effective method as a salvage treatment for SSNHL. This study explored the efficacy of a modified IST approach, incorporating the utilization of tympanic tubes and gelfoam as key components. The findings underscore the advantages of gelfoam as a strategic drug carrier placed in the round window niche. By minimizing drug loss, extending action time, and increasing perilymph concentration, gelfoam enhances the therapeutic impact of IST, contributing to improved hearing outcomes in patients with SSNHL.
基金supported by The Coordinatorship of Scientific Research Projects Department,Süleyman Demirel University(Grant Number:TTU-2021-8402).
文摘Objective:The aim of this study was to examine angiotensin converting enzyme(ACE)insertion/deletion,alpha adducin,and interleukin-10(IL-10)gene polymorphisms(GPs)in terms of both idiopathic sudden sensorineural hearing loss(ISSNHL)risk and their potential prognostic effects.Methods:The study group consisted of 70 patients and the control group consisted of 50 patients.Venous blood samples were analyzed for relevant GPs via kompetitive allele-specific polymerase chain reaction.Age,sex,affected side,tinnitus,and vertiginous symptom status,number of days between symptom onset and hospital admission,pure tone audiometry results at admission and after treatment were included in the study.Data were compared statistically.Results:The D allele of ACE insertion/deletion GP was significantly more frequent in patients with ISSNHL than in the control group(p=0.032).II genotype was associated with a reduced risk of ISSNHL(p=0.036).The amount of hearing loss was significantly higher in patients with the TT genotype(p=0.027)and T allele of the IL-10 GP(p=0.035)than in the patients without this allele.Severe hearing loss was a poor prognostic factor(p=0.008).Conclusions:The D allele of ACE insertion/deletion GP may be involved in the ISSNHL etiology.Due to the association of this allele with occlusive vascular pathologies,ischemia is believed to be a common pathway in the etiopathogenesis of ISSNHL.
文摘Currently,treatment options for infant sensorineural hearing loss(SNHL)are limited.This article describes a novel case of SNHL in an infant successfully treated with foot reflexology,along with observed brain activity changes before and after treatment,as indicated by functional magnetic resonance imaging.Hence,this commentary discusses the case and our viewpoints regarding foot reflexology for treating SNHL.
基金the Innovative Program of Hebei Provincial Eye Hospital,No.2023ZZ107.
文摘BACKGROUND Numerous studies have found that patients experiencing sudden sensorineural hearing loss(SSHL),with or without accompanying vertigo,often show impaired vestibular function.However,there is a dearth of studies analyzing vestibularevoked myogenic potentials(VEMPs)in SSHL patients across various age groups.AIM To investigate vestibular condition in SSHL patients across various age demographics.METHODS Clinical data of 84 SSHL patients were investigated retrospectively.Audiometry,cervical vestibular evoked myogenic potentials(c-VEMPs),and ocular vestibular evoked myogenic potentials(o-VEMPs)were conducted on these patients.Parameters assessed included the latencies of P1 and N1 waves,as well as the amplitudes of P1–N1 waves.Moreover,the study evaluated the influence of factors such as sex,affected side,configuration of hearing loss,and presence of accompanying vertigo.RESULTS Among the 84 SSHL patients,no significant differences were observed among the three groups in terms of gender,affected side,and the presence or absence of vertigo.Group II(aged 41–60 years)had the highest number of SSHL cases.The rates of absent o-VEMPs in the affected ears were 20.83%,31.58%,and 22.72%for the three age groups,respectively,with no statistically significant difference among them.The rates of absent c-VEMPs in the affected ears were 8.3%,34.21%,and 18.18%for the three age groups,respectively,with significant differences.In the unaffected ears,there were differences observed in the extraction rates of o-VEMPs in the unaffected ears among the age groups.In the three age groups,no significant differences were noted in the three age groups in the latencies of P1 and N1 waves or in the amplitude of N1–P1 waves for c-VEMPs and o-VEMPs,either on the affected side or on the unaffected side,across the three age groups.CONCLUSION The extraction rate of VEMPs is more valuable than parameters.Regardless of the presence of vertigo,vestibular organs are involved in SSHL.Notably,SSHL patients aged 41–60 appear more susceptible to damage to the inferior vestibular nerve and saccule.
基金Supported by the National Social Science Foundation,No.18BY0911.
文摘BACKGROUND Understanding the impact of early sensory deficits on brain development is essential for understanding developmental processes and developing potential interventions.While previous studies have looked into the impact of prenatal experiences on language development,there is a lack of research on how these experiences affect early language and brain function development in individuals with sensorineural hearing loss(SNHL).AIM To investigate SNHL effects on early brain development and connectivity in 4-month-olds vs healthy newborns and controls.METHODS The research involved analyzing the functional brain networks of 65 infants,categorized into three groups:28 healthy newborns,224-month-old participants with SNHL,and 15 age-matched healthy participants.The resting-state functional connectivity was measured and compared between the groups using functional near-infrared spectroscopy and graph theory to assess the brain network properties.RESULTS Significant differences were found in resting-state functional connectivity between participants with SNHL and age-matched controls,indicating a developmental lag in brain connectivity for those with SNHL.Surprisingly,SNHL participants showed better connectivity development compared to healthy newborns,with connectivity strengths of 0.13±0.04 for SNHL,0.16±0.08 for controls,and 0.098±0.04 for newborns.Graph theory analysis revealed enhanced global brain network properties for the SNHL group,suggesting higher communication efficiency at 4 months.No significant differences were noted in network properties between 4-month-old SNHL participants and neonates.A unique pattern of central hubs was observed in the SNHL group,with 2 hubs in the left hemisphere compared to 6 in controls.CONCLUSION 4-month-old infants with SNHL have a distinct brain network pattern with efficient long-distance information transmission but less effective local communication compared to age-matched controls.
文摘BACKGROUND Sudden sensorineural hearing loss(SSNHL),characterized by a rapid and unexplained loss of hearing,particularly at moderate to high frequencies,presents a significant clinical challenge.The therapeutic use of methylprednisolone sodium succinate(MPSS)via different administration routes,in combination with conventional medications,remains a topic of interest.AIM To compare the therapeutic efficacy of MPSS administered via different routes in combination with conventional drugs for the treatment of mid-to high-frequency SSNHL.METHODS The medical records of 109 patients with mid-to high-frequency SSNHL were analyzed.The patients were divided into three groups based on the route of administration:Group A[intratympanic(IT)injection of MPSS combined with mecobalamin and Ginkgo biloba leaf extract injection],Group B(intravenous injection of MPSS combined with mecobalamin and Ginkgo biloba leaf extract injection),and Group C(single IT injection of MPSS).The intervention effects were compared and analyzed.RESULTS The posttreatment auditory thresholds in Group A(21.23±3.34)were significantly lower than those in Groups B(28.52±3.36)and C(30.23±4.21;P<0.05).Group A also exhibited a significantly greater speech recognition rate(92.23±5.34)than Groups B and C.The disappearance time of tinnitus,time to hearing recovery,and disappearance time of vertigo in Group A were significantly shorter than those in Groups B and C(P<0.05).The total effective rate in Group A(97.56%)was significantly greater than that in Groups B and C(77.14%and 78.79%,χ^(2)=7.898,P=0.019).Moreover,the incidence of adverse reactions in Groups A and C was significantly lower than that in Group B(4.88%,3.03%vs 2.57%,χ^(2)=11.443,P=0.003),and the recurrence rate in Group A was significantly lower than that in Groups B and C(2.44%vs 20.00%vs 21.21%,χ^(2)=7.120,P=0.028).CONCLUSION IT injection of MPSS combined with conventional treatment demonstrates superior efficacy and safety compared to systemic administration via intravenous infusion and a single IT injection of MPSS.This approach effectively improves patients'hearing and reduces the risk of disease recurrence.
基金supported by the National Key Research and Development Program of China (2021YFA0805902,2022YFF0710703)National Natural Science Foundation of China (32201257)+1 种基金Science and Technology Innovation Project of Xiongan New Area (2022XAGG0121)Young Elite Scientists Sponsorship Program by the China Association for Science and Technology (2019QNRC001)。
文摘Hereditary hearing loss(HHL),a genetic disorder that impairs auditory function,significantly affects quality of life and incurs substantial economic losses for society.To investigate the underlying causes of HHL and evaluate therapeutic outcomes,appropriate animal models are necessary.Pigs have been extensively used as valuable large animal models in biomedical research.In this review,we highlight the advantages of pig models in terms of ear anatomy,inner ear morphology,and electrophysiological characteristics,as well as recent advancements in the development of distinct genetically modified porcine models of hearing loss.Additionally,we discuss the prospects,challenges,and recommendations regarding the use pig models in HHL research.Overall,this review provides insights and perspectives for future studies on HHL using porcine models.
文摘Sudden sensorineural hearing loss(SSNHL)is an enigmatic entity,with obscure pathophysiology and debatable efficacy of the treatment agents used.An underlying cause is identified in only 10-15%of cases.The management of the remaining patients,classified as‘idiopathic’,is empirical,and is conventionally with systemic steroids,vasodilator therapy,rheological agents,and antioxidants,to list a few amongst the host of the agents employed for the treatment.The availability of conflicting outcomes and lack of conclusive evidence has resulted in the propagation of consensus-based treatment protocols.In the present review,we discuss the various controversial issues and newer developments in the management of idiopathic SSNHL.The current review aims to present a narrative outlook of the updated evidence base available from PUBMED,augmented with relevant designated publications.
基金supported by grants from the Youth cultivation project of military medical science(16QNP133)Medical big data research and development project of PLA general hospital(2018MBD-015)+3 种基金the capital health research and development of special project(2016-1-5014)the National Natural Science Foundation of China(81670940,81770991)Financial supports from the Beijing Nova Program(xxjh2015105)clinical support fund of General Hospital of the PLA are acknowledged
文摘Acute sensorineural hearing loss is an uncommon phenomenon in dentistry.We describe the case of a 79-year-old male who presented with acute sensorineural hearing loss occurring 2 days after a tooth extraction procedure under local anesthesia.Possible mechanisms are discussed.He was treated with vasodilators(Ginaton and Alprostadil Injection)and Mecobalamin injection with benefit.High dose oral steroids(1 mg/kg)and low molecular weight dextran were used.
基金supported by Beijing Science and Technology Special Fund[grants number 2161100000116067]。
文摘Objective Hyperbaric oxygen treatment(HBOT)has demonstrated efficacy in improving hearing levels of patients with idiopathic sudden sensorineural hearing loss(ISSHL);however,the underlying mechanisms are not well understood.HBOT alleviates the inflammatory response,which is mediated by Toll-like receptor(TLR)4 and nuclear factor(NF)-κB.In this study we investigated whether HBOT attenuates inflammation in ISHHL patients via alteration of TLR4 and NF-κB expression.Methods ISHHL patients(n=120)and healthy control subjects(n=20)were enrolled in this study.Patients were randomly divided into medicine group treated with medicine only(n=60)and HBO group receiving both HBOT and medicine(n=60).Audiometric testing was performed pre-and posttreatment.TLR4,NF-кB,and TNF-αexpression in peripheral blood of ISSHL patients and healthy control subjects was assessed by ELISA before and after treatment.Results TLR4,NF-κB,and TNF-αlevels were upregulated in ISSHL patients relative to healthy control subjects;the levels were decreased following treatment and were lower in the HBO group than that in the medicine group post-treatment(P<0.05 and P<0.01).Conclusion HBOT alleviates hearing loss in ISSHL patients by suppressing the inflammatory response induced by TLR4 and NF-κB signaling.
基金supported by the Ministry of Health Fund Industry of China,as part of project"Prevention,Intervention,and Extend Application of Deafness with Birth Defect"(contract#:201202005)the 1255 project of Changhai Hospital,Second Military Medical University,Shanghai,China
文摘Objective:To investigate immune-related genetic background in bilateral sudden sensorineural hearing loss (SSNHL). Case report and methods: The case is a 45-year-old man presenting with a 7-year history of bilateral profound SSNHL. Blood biochemical testing demonstrated increased levels of total cholesterol (5.88 mmol/L). Tests for hepatitis B showed a positive antibody against the hepatitis B core antigen. Complement C3 was below the normal value, and complement C4 and IgG were in the lower range of normal values. CT images showed a normal inner ear and vestibular aqueduct but round window membranous ossification on both sides. A total number of 232 immune-associated genes were sequenced using the next generation sequencing technique. Results: Mutations were detected in 5 genes, including the phosphoinositide 3-kinase catalytic subunit delta (PIK3CD), caspase recruitment domain-containing protein 9 (CARD9), complement factor H-related (CFHR2), immunoglobulin lambda-like polypeptide 1 Protein (IGLL1), and transmembrane channel-like gene family 8 (TMC8). In the PIK3CD gene, a C896T substitute in exon 7 was detected. This mutation causes primary immunodeficiency and is an autosomal dominant disease. Conclusion: The PIK3CD C896T mutation responsible for primary immunodeficiency may contribute to the onset of bilateral SSNHL with subsequent rapid progression.
文摘Objectives:To evaluate hearing outcome of salvage treatment with intratympanic steroids(ITS)in idiopathic sudden sensorineural hearing loss(ISSNHL)refractory to initial systemic steroid(SS)therapy.Material and methods:A retrospective medical chart review was conducted on 54 consecutive patients with ISSNHL refractory to SS.Salvage treatment with a low dose intratympanic dexamethasone(4 mg/ml)was offered after one week of primary treatment.Patients were divided into two groups:25 patients accepted ITS(treatment group)and 29 patients did not undergo additional treatment(control group).A pure tone average(PTA)gain of at least 10 dB was considered hearing improvement.Results:Hearing improvement rate was higher in ITS group compared to control group(40%vs.13.8%,p=0.035).A mean PTA improvement of 8.6±9.8 dB was observed in the ITS group and,whereas the control group had an average hearing gain of 0.7±2 dB(p<0.001).Audiometric analysis revealed a significant hearing gain in ITS group at all tested frequencies compared to control group(p<0.05).Analysis of the selected variables,identified intratympanic steroid treatment as the only independent prognostic factor for hearing improvement(OR=4.2,95%CI:1.1e15.7;p=0.04).Conclusion:Intratympanic low dose dexamethasone is effective in patients with incomplete hearing recovery after primary systemic steroid treatment.
基金Supported by the Health and Family Planning Commission of Shenzhen Municipality,No.SZXJ2017065.
文摘BACKGROUND As sudden sensorineural hearing loss(SSNHL)rarely occurs in pregnant women,there is a lack of knowledge and relevant research on its management.AIM To investigate the effect of intratympanic dexamethasone injection in the treatment of pregnant patients with SSNHL.METHODS A retrospective chart review was made for the period between June 2017 and August 2019 at our Department of Otorhinolaryngology-Head and Neck Surgery.Pregnant women who met the criteria for SSNHL were included and grouped based on the therapeutic modalities.The treatment group received intratympanic dexamethasone(2.5 mg)q.o.d.for a total of four times,while the control group received no medication other than bed rest and medical observations.All the patients were under close care of obstetricians.Pure-tone audiograms were performed before and after treatment.RESULTS Eleven patients who met the inclusion criteria were assigned to the treatment group(n=7)and the control group(n=4).The mean age of patients was 31.2±3.8 years;the right ear was affected in seven(63.64%)cases.Two patients(18.2%)suffered from vertigo,10(90.9%)suffered from tinnitus and 6(54.5%)suffered from aural fullness.The time from onset to clinic visit was relatively short,with a mean time of 1.3±0.9 d.All the women were within the second or third trimester;the average gestation period was 26.0±6.2 wk.The pure-tone averages at onset between the two groups were similar.After one wk of therapy,the treatment group had a curative rate of 57.1%and a significantly better hearing threshold and greater improvement compared to the control group(all P<0.05).Some patients experienced transient discomfort from intratympanic injections that disappeared after getting rest,while none had permanent complications.All patients delivered healthy full-term neonates with an average Apgar score of 9.7±0.5.CONCLUSION Intratympanic dexamethasone injections can be used as a first-line therapy in pregnant women with SSNHL.
文摘In this article,the mechanism of inheritance behind inherited hearing loss and genetic susceptibilityin noise-induced hearing loss are reviewed.Conventional treatments for sensorineural hearing loss(SNHL),i.e.hearing aid and cochlear implant,are effective for some cases,but not without limitations.For example,they provide little benefit for patients of profound SNHL or neural hearing loss,especially when the hearing loss is in poor dynamic range and with low frequency resolution.We emphasize the most recent evidence-based treatment in this field,which includes gene therapy and allotransplantation of stem cells.Their promising results have shown that they might be options of treatment for profound SNHL and neural hearing loss.Although some treatments are still at the experimental stage,it is helpful to be aware of the novel therapies and endeavour to explore the feasibility of their clinical application.