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
The purpose of this study was to investigate the presence of endolymphatic hydrops(EH)in both affected and unaffected ears of patients with pantonal unilateral idiopathic sudden sensorineural hearing loss(ISSNHL)using...The purpose of this study was to investigate the presence of endolymphatic hydrops(EH)in both affected and unaffected ears of patients with pantonal unilateral idiopathic sudden sensorineural hearing loss(ISSNHL)using three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging(3D-FLAIR MRI)and further evaluate the significance of EH in this disorder.Twenty-seven ISSHL patients were enrolled in this study.3D-FLAIR MRI was performed 24 h after intratympanic injection of gadolinium-diethylenetriaminepentaacetic acid(Gd-DPTA).The incidences of EH in the affected ears and contralateral unaffected ears were compared and the correlations of EH with vertigo or prognosis were analyzed using the Chi-square test.The results showed that the incidence of EH was 68.0%(17/25)in the affected ears and 34.8%(8/23)in the unaffected ears.There was a statistically significant difference between affected ears and unaffected ears in regard to the incidence of EH(P<0.05).There were no significant correlations of EH with vertigo(P=1.000)or with prognosis(P=0.359)in the affected ears.In conclusion,there is EH in the inner ear of patients with pantonal ISSNHL;EH is not related to vertigo,a concomitant symptom of ISSNHL,and the prognosis of this condition.The presence of EH may be a secondary reaction following the impairment of the inner ears with pantonal ISSNHL.展开更多
BACKGROUND Acute otitis media(AOM)is a common disease that is more prevalent in children.Most studies concerning AOM-associated sudden sensorineural hearing loss are case reports and retrospective in nature,hence the ...BACKGROUND Acute otitis media(AOM)is a common disease that is more prevalent in children.Most studies concerning AOM-associated sudden sensorineural hearing loss are case reports and retrospective in nature,hence the etiology of AOM-associated sudden hearing loss has not been fully established.AIM To analyze audiological characteristics of AOM-associated sudden hearing loss and evaluate efficacy of combined tympanostomy tube placement(TTP)and intratympanic methylprednisolone.METHODS Eight adult patients who were diagnosed with AOM-associated sudden hearing loss and ineffectively treated by conventional medical therapy were enrolled in this study.Basic data were collected,and pure tone audiometry was performed to assess the audiological characteristics.Combination therapy with TTP and intratympanic methylprednisolone injection was given to the patients.RESULTS Mixed or sensorineural hearing loss was observed at high frequencies(2–4 kHz).All the cases in this study were cured after TTP and intratympanic methylprednisolone.After treatment,the average hearing threshold at affected frequencies was significantly lower than those in the pretreatment group(P<0.05)and was similar to that in the healthy ears(P>0.05).CONCLUSION AOM rarely induces sudden sensorineural hearing loss.Combination therapy with TTP and intratympanic methylprednisolone injection may be effective after failure of conventional medical treatment.展开更多
Introduction Sudden sensorineural hearing loss(SSNHL)is defined as a hearing loss of at least 30 dB over three contiguous frequencies occurring in less than 3 days[1].Vertigo and profound hearing loss are considered p...Introduction Sudden sensorineural hearing loss(SSNHL)is defined as a hearing loss of at least 30 dB over three contiguous frequencies occurring in less than 3 days[1].Vertigo and profound hearing loss are considered poor prognostic factors in SSNHL[2-4].The most common diseases associated with vertigo in SSNHL include BPPV,vestibular neu-[3]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
BACKGROUND Sudden hearing loss(SHL)is associated with serious systematic conditions such as neoplasms,vascular events,autoimmune diseases,infections,and iatrogenic injury.Some authors report that SHL can be an early w...BACKGROUND Sudden hearing loss(SHL)is associated with serious systematic conditions such as neoplasms,vascular events,autoimmune diseases,infections,and iatrogenic injury.Some authors report that SHL can be an early warning sign of impending vertebrobasilar ischemic stroke.It is important to distinguish stroke from benign disease.CASE SUMMARY A 48-year-old male patient presented with SHL and vertigo as first symptoms.Diffusion-weighted imaging revealed high signal intensity in the left posterior inferior cerebellar artery territory of the cerebellar hemisphere and high signal intensity in the right pons and bridge cerebellar arm,confirming that the patient had cerebral infarction.Treatment with antiplatelet drugs,steroid antiinflammatory drugs,and neurotrophic nerve therapy promoted blood circulation and removed blood stasis,and the symptoms of the patient were significantly improved.CONCLUSION SHL and vertigo could be the initial symptoms of vertebrobasilar ischemic stroke.展开更多
Background:Evidence suggests that glucocorticoids are important in the treatment of sudden hearing loss(SHL)and Meniere’s disease(MD).However,different glucocorticoid administration methods may have a significant imp...Background:Evidence suggests that glucocorticoids are important in the treatment of sudden hearing loss(SHL)and Meniere’s disease(MD).However,different glucocorticoid administration methods may have a significant impact on treatment outcomes.Objective:This study aimed to investigate effects of different glucocorticoid administration methods on sudden hearing loss and Meniere’s disease.Methods:In this study,glucocorticoids were administered orally in 18 patients,by retroauricular injection in 15 patients and by intratympanic injection in 15 patients.White blood cell(WBC)count,serum Kt,fasting plasma glucose(FPG),body temperature,heart rate and blood pressure were used to evaluate effects of glucocorticoids on patients with hearing loss.Visual analog scale(VAS)of pain and sleep disorders were also surveyed,and pure tone audiometry(PTA)results were compared among groups to evaluate efficacy of different glucocorticoids administration methods.Result:WBC count,heart rate and blood pressure were higher in patients taking oral glucocorticoids,while body temperature,serum Kt and FPG levels did not change in all three groups.However,patients who received intratympanic injection of glucocorticoids experienced more pain,while those taking oral glucocorticoids reported more sleep impairment.Treatment efficacy on hearing loss was not significantly different among the three groups.Conclusion:These findings suggest that systemic glucocorticoid administration can result in greater whole body responses than local administration,but with similar hearing treatment efficacy.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
Objective:Recent studies have shown that chronic inflammation contributes to the development of sudden sensorineural hearing loss(SSNHL).Some hematologic parameters have also been linked to the prognosis of SSNHL.Howe...Objective:Recent studies have shown that chronic inflammation contributes to the development of sudden sensorineural hearing loss(SSNHL).Some hematologic parameters have also been linked to the prognosis of SSNHL.However,the prognostic value of such hematological factors is not conclusive.This study explored the association of routine hematological parameters with SSNHL.Methods:A systematic literature search was conducted in PubMed,Cochrane Library,Web of Science and Embase to identify eligible studies.Standardized mean deviation(SMD)and the 95%confidence interval(CI)were retried from relevant studies for analysis.Heterogeneity,subgroup,and publication bias analyses were performed.Results:A total of 18 studies involving 1505 SSNHL patients and 1466 healthy persons were enrolled in the final analysis.The study population included 699 responders and 458 non-responders to treatment.Pooled results revealed that the neutrophil/lymphocyte ratio(NLR)and platelet/lymphocyte ratio(PLR)value in the SSNHL patient group were higher than in the healthy group(SMD=1.05,95%CI:0.86,1.24,p<0.001,SMD=0.52,95%CI:0.26,0.78,p<0.001,respectively).However,there was no significant difference in the mean platelet volumes(MPV)between the groups(SMD=0.03,95%CI:0.44,0.49,p=0.91).Notably,NLR and PLR values were evidently higher in the unrecovered group than in the recovered group(SMD=0.63,95%CI:1.02,0.23,p=0.002,SMD=0.4,95%CI:0.76,0.03,p=0.03,respectively).However,the MPV value was similar in both groups(SMD=0.35,95%CI:1.14,0.44,p=0.38).Conclusions:Our results show that NLR and PLR values can predict the onset and prognosis of SSNHL.展开更多
Objective To establish an animal model of sudden onset sensorineural hearing loss (SSNHL) to study its mechanisms. Materials and methods The inner ear was exposed to 3-nitropropionic acid at 0.5 mol/L (3-NP (H)) and 0...Objective To establish an animal model of sudden onset sensorineural hearing loss (SSNHL) to study its mechanisms. Materials and methods The inner ear was exposed to 3-nitropropionic acid at 0.5 mol/L (3-NP (H)) and 0.3 mol/L (3-NP (L)) through the round window membrane for 30 minutes in 50 male guinea pigs. Thresholds of auditory brainstem responses(ABR) were established before the treatment and retested at 4 hours, 1 day, 3 days and 6 days following 3-NP exposure. Control animals were treated with phosphate buffered saline (PBS) and their ABRs were retested at 4 hours and 1 day after the treatment. Animals were monitored for nystagmus and postural signs of vestibular dysfunction, using a digital video camera, following the treatment procedure. Specimens were taken at 12 hours, 1 day, 3 days and 7 days following 3-NP(H) exposure and embedded in JB4 for light microscopy observation. Results ABRs were lost in all animals tested at 4 hours following 3-NP (H) exposure. The rate of complete ABR loss decreased as post-treatment test time increased. ABRs were lost in 80% (4/5) of the animals at 1 day after exposure to 3-NP (L). Spontaneous horizontal nystagmus with a fast phase away from the treated ear developed in all 3-NP (H)-treated animals and in 20 % ( 1/5) of the animals exposed to 3-NP (L), except for the one treated bilaterally. Various degree of postural disturbances consistent with unilateral vestibular dysfunction, such as spontaneous barrel rolling towards the exposure side while walking, were seen in all animals exposed to 3-NP(H) and 40% (2/5) of animals exposed to 3-NP(L), except for the one animal treated bilaterally, which showed no signs of imbalance. Both nystagmus and postural disturbances resolved in 2 days following 3-NP exposure. Histological study showed temporary edema tin the organ or Corti, Claudius cells and the inner sulcus cells 3 days after 3-NP (H) treatment. Enlargement of intercellular space in the spiral prominence was first noticed at 12 hours post-3-NP (H) exposure, progressed at day 3 and recovered at day 7. Vacuoles in the cellular plasm and nucleus was seen as early as at 12 hours post-3-NP exposure in the spiral ganglion cells, and signs of degeneration were visible at day 7. Conclusion Inner ear exposure to 3-NP through the round window membrane appears to reproduce clinic manifestations and may serve as a legitimate animal model of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
Sudden deafness is one of the commonest acute symptoms in otolaryngology.In recent years,the incidence of sudden deafness is on the rise all over the world,but its etiology is still unclear,and the prognosis is greatl...Sudden deafness is one of the commonest acute symptoms in otolaryngology.In recent years,the incidence of sudden deafness is on the rise all over the world,but its etiology is still unclear,and the prognosis is greatly different.Traditional Chinese medicine(TCM)believes that the onset of sudden deafness is related to the liver,gallbladder,heart and triple energizer meridians.This article mainly explored the key points of common syndromes,TCM nursing methods and health guidance of sudden deafness.展开更多
Objective: To assess the outcomes of intratympanic steroids in the management of idiopathic sudden sensorineural hearing loss. Study Design: Retrospective study. Setting: Academic tertiary care medical centre. Materia...Objective: To assess the outcomes of intratympanic steroids in the management of idiopathic sudden sensorineural hearing loss. Study Design: Retrospective study. Setting: Academic tertiary care medical centre. Materials and Methods: A total of 67 patients with idiopathic sudden sensorineural hearing loss were included in our study. Pre- and post-injection audiograms were taken to assess the outcomes in improvement in hearing. Hearing outcomes were assessed with respect to duration of onset of treatment and age of the patient. Improvement in the speech discrimination score was also assessed. Results: A total of 67 patients were included in our study of which unilateral cases were 59 and bilateral cases were 8. Subjectively 35 patients reported improvement in hearing after the therapy while objectively we found 44 ears [41 patients] had improvement. The mean PTA pre ITS was 62.7 and after ITS it was 56.3. Maximum improvement was noted at 2000 Hz and 1000 Hz. Conclusion: Intratympanic steroids can be offered as a first line therapy for idiopathic sudden sensorineural hearing loss as it is minimally invasive, and can be performed as an office based procedure with no systemic side effects. No major complications have been reported in our study and results have been satisfactory.展开更多
Objective To explore the relationship between sudden sensorineural hearing loss(SSNHL) and vascular risk factors(including serum lipids and uric acid).Method This is a retrospective analysis of 100 cases of SSNHL seen...Objective To explore the relationship between sudden sensorineural hearing loss(SSNHL) and vascular risk factors(including serum lipids and uric acid).Method This is a retrospective analysis of 100 cases of SSNHL seen at the Drum Tower Hospital,Nanjing Medical University,between Jan.2007 and Apr.2008。 Patient history,blood test results and imaging scans were analyzed.Levels of triglyceride(TG),cholesterols(CHO),high density lipoprotein-cholesterol(HDL-CH),low density lipoprotein-cholesterol(LDL-CH),apolipoprotein AI(ApoAI),apolipoprotein B(ApoB) and uric acid(UA) from these patients were compared with a control group of 56 patients treated for vocal cord polyps or nasal septum deviation during the same period.Patients with hypertension,diabetes,heart,brain,liver or kidney disorders are excluded from the present investigation.Results HDLCH level was higher and UA level lower in the study group than the control group(P < 0.05).HDL-CH and UA showed no significant differences among different age-groups(P > 0.05).There were no significant differences in the levels of TG,CHO,LDL-CH,ApoAI and ApoB(P > 0.05).Conclusion These data indicate that metabolic disturbances of serum lipids and/or uric acid may be potential risk factors for展开更多
Objectives:This study aimed to determine the prognostic value of otoacoustic emissions(OAEs)in idiopathic sudden sensorineural hearing loss patients.Methods:The study included 30 subjects with unilateral idiopathic su...Objectives:This study aimed to determine the prognostic value of otoacoustic emissions(OAEs)in idiopathic sudden sensorineural hearing loss patients.Methods:The study included 30 subjects with unilateral idiopathic sudden sensorineural hearing loss(ISSNHL).Each patient was evaluated four times:at baseline and after one week,one month,and three months of treatment.During each visit,each patient was subjected to full audiological history,otoscopic examination,basic audiological evaluations,and transiently evoked and distortion product otoacoustic emission(TEOAEs&DEOAEs).Results:The hearing thresholds(frequency range 250e8000 Hz)and word recognition scores of patients with detectable TEOAEs and DPOAEs improved significantly,whereas no significant improvements were observed in those with no response.Conclusion:Hearing improvement is better in patients with detectable TEOAEs and DPOAEs.As a result,TEOAEs and DPOAEs are recommended as routine tests in all SSNHL patients to predict outcomes and monitor treatment as TEOAEs and DPOAEs reflect the cochlear OHCs activity.展开更多
Objectives:To evaluate the pathological changes in the blood-perilymph,blood-endolymph,and bloodnerve barriers of a patient with idiopathic sudden sensorineural hearing loss(SSNHL).Methods:Potential ossification or fi...Objectives:To evaluate the pathological changes in the blood-perilymph,blood-endolymph,and bloodnerve barriers of a patient with idiopathic sudden sensorineural hearing loss(SSNHL).Methods:Potential ossification or fibrosis in the inner ear was evaluated using temporal bone CT and MRI acquired using the 3-dimensional T2-weighted sampling perfection with application-optimized contrasts using a flip angle evolution sequence.Pathological changes in the barriers were analyzed by MRI obtained 4 h after a single-dose intravenous injection of gadolinium chelate using a medium inversiontime inversion recovery imaging with magnitude reconstruction sequence.Results:The perilymph was absent,while significant enhancements of the vestibulocochlear nerve and the endolymphatic compartments were detected.Conclusion:Significant injuries in the blood-endolymph and blood-vestibulocochlear nerve barriers and disabled perilymph production may contribute to the development of SSNHL with poor response to treatments.展开更多
Rationale:Dengue is the most important human arboviral disease.Neurological manifestations occur rarely in dengue.To the best of our knowledge,there is only one reported case of dengue-associated sudden sensorineural ...Rationale:Dengue is the most important human arboviral disease.Neurological manifestations occur rarely in dengue.To the best of our knowledge,there is only one reported case of dengue-associated sudden sensorineural hearing loss(SSNHL)in Brazil untill now.Patient concerns:A 42-year-old man was presented to the emergency department with unconsciousness and generalized tonicclonic seizures.Diagnosis:Dengue-associated aseptic meningitis,acute kidney injury,and SSNHL.Interventions:The patient was treated with anticonvulsants and thiamine and underwent mechanical ventilation.He received combined ceftriaxone and acyclovir,which were later switched to meropenem,acyclovir and ampicillin empirically until culture results became available.He also required hemodialysis and plasmapheresis sessions and fresh frozen plasma and buffy coat transfusions until definitive diagnosis.Outcomes:The patient was discharged after improvement of his general condition and of his blood test results,but hearing loss remained.A six-month follow-up visit showed persistent deafness.Lessons:Dengue should be included in the differential diagnosis of patients from dengue-endemic areas presenting an acute febrile disease with neurological manifestations.To the best of our knowledge,this is the second reported case of dengueassociated SSNHL,suggesting an association between dengue and development of SSNHL.展开更多
Objective To investigate the genetic causes of sudden sensorineural hearing loss(SSNHL)patients in China.This study focused on analyzing variations of coding sequence of common genes related to deafness,revealing the ...Objective To investigate the genetic causes of sudden sensorineural hearing loss(SSNHL)patients in China.This study focused on analyzing variations of coding sequence of common genes related to deafness,revealing the molecular pathogenesis of sudden deafness from a genomics perspective,discovering molecular markers associated with the onset of deafness,and then supplying prevention to high-risk populations,classifying disease according to accurate etiology,and choosing a much more precision therapy.Methods We retrospectively analyzed the clinical characteristics of 51 patients diagnosed as SSNHL with vertigo treated in the Chinese PLA General Hospital.In this study,mutation screening of 307 nuclear genes and mitochondrial genome responsible for human or mouse deafness was performed on the 51 cases of unilateral sudden deafness patients with vertigo.Results We identified 51 cases of unilateral sudden deafness,including 2 cases of low-mid frequency hearing impairment,18 cases of mid-high frequency hearing loss,11 cases of flat-type hearing loss,and 20 cases of all frequency hearing loss.Among the 51 cases,8(15.69%)cases of GJB2 heterozygous variations,1(1.96%)case of GJB3 heterozygous variations,5(9.8%)cases of SLC26A4 heterozygous variations,2(3.92%)cases of COCH heterozygous variations,14(27.45%)cases of CDH23 heterozygous variations,14(27.45%)cases of OTOF heterozygous variations,1(1.96%)case of SLC17A8 heterozygous variations and 2(3.92%)cases of KCNE1 heterozygous variations.No mtDNA gene variations were identified.Conclusion SSNHL has some relationship with hereditary in Chinese population,but its complex genetic pathogenic mechanisms need further study.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 pr...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 Ig G 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 immuneassociated 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 PIK3 CD gene, a C896 T substitute in exon 7 was detected. This mutation causes primary immunodeficiency and is an autosomal dominant disease.Conclusion: The PIK3 CD C896T mutation responsible for primary immunodeficiency may contribute to the onset of bilateral SSNHL with subsequent rapid progression.展开更多
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the National Natural Science Foundation of China(Nos.81271072,81700909).
文摘The purpose of this study was to investigate the presence of endolymphatic hydrops(EH)in both affected and unaffected ears of patients with pantonal unilateral idiopathic sudden sensorineural hearing loss(ISSNHL)using three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging(3D-FLAIR MRI)and further evaluate the significance of EH in this disorder.Twenty-seven ISSHL patients were enrolled in this study.3D-FLAIR MRI was performed 24 h after intratympanic injection of gadolinium-diethylenetriaminepentaacetic acid(Gd-DPTA).The incidences of EH in the affected ears and contralateral unaffected ears were compared and the correlations of EH with vertigo or prognosis were analyzed using the Chi-square test.The results showed that the incidence of EH was 68.0%(17/25)in the affected ears and 34.8%(8/23)in the unaffected ears.There was a statistically significant difference between affected ears and unaffected ears in regard to the incidence of EH(P<0.05).There were no significant correlations of EH with vertigo(P=1.000)or with prognosis(P=0.359)in the affected ears.In conclusion,there is EH in the inner ear of patients with pantonal ISSNHL;EH is not related to vertigo,a concomitant symptom of ISSNHL,and the prognosis of this condition.The presence of EH may be a secondary reaction following the impairment of the inner ears with pantonal ISSNHL.
文摘BACKGROUND Acute otitis media(AOM)is a common disease that is more prevalent in children.Most studies concerning AOM-associated sudden sensorineural hearing loss are case reports and retrospective in nature,hence the etiology of AOM-associated sudden hearing loss has not been fully established.AIM To analyze audiological characteristics of AOM-associated sudden hearing loss and evaluate efficacy of combined tympanostomy tube placement(TTP)and intratympanic methylprednisolone.METHODS Eight adult patients who were diagnosed with AOM-associated sudden hearing loss and ineffectively treated by conventional medical therapy were enrolled in this study.Basic data were collected,and pure tone audiometry was performed to assess the audiological characteristics.Combination therapy with TTP and intratympanic methylprednisolone injection was given to the patients.RESULTS Mixed or sensorineural hearing loss was observed at high frequencies(2–4 kHz).All the cases in this study were cured after TTP and intratympanic methylprednisolone.After treatment,the average hearing threshold at affected frequencies was significantly lower than those in the pretreatment group(P<0.05)and was similar to that in the healthy ears(P>0.05).CONCLUSION AOM rarely induces sudden sensorineural hearing loss.Combination therapy with TTP and intratympanic methylprednisolone injection may be effective after failure of conventional medical treatment.
文摘Introduction Sudden sensorineural hearing loss(SSNHL)is defined as a hearing loss of at least 30 dB over three contiguous frequencies occurring in less than 3 days[1].Vertigo and profound hearing loss are considered poor prognostic factors in SSNHL[2-4].The most common diseases associated with vertigo in SSNHL include BPPV,vestibular neu-[3]
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.
文摘BACKGROUND Sudden hearing loss(SHL)is associated with serious systematic conditions such as neoplasms,vascular events,autoimmune diseases,infections,and iatrogenic injury.Some authors report that SHL can be an early warning sign of impending vertebrobasilar ischemic stroke.It is important to distinguish stroke from benign disease.CASE SUMMARY A 48-year-old male patient presented with SHL and vertigo as first symptoms.Diffusion-weighted imaging revealed high signal intensity in the left posterior inferior cerebellar artery territory of the cerebellar hemisphere and high signal intensity in the right pons and bridge cerebellar arm,confirming that the patient had cerebral infarction.Treatment with antiplatelet drugs,steroid antiinflammatory drugs,and neurotrophic nerve therapy promoted blood circulation and removed blood stasis,and the symptoms of the patient were significantly improved.CONCLUSION SHL and vertigo could be the initial symptoms of vertebrobasilar ischemic stroke.
基金the Science and Technology Program of Guangzhou(#201803010093)Special Cultivation Project of Sun Yat-sen University(#2018122819965)。
文摘Background:Evidence suggests that glucocorticoids are important in the treatment of sudden hearing loss(SHL)and Meniere’s disease(MD).However,different glucocorticoid administration methods may have a significant impact on treatment outcomes.Objective:This study aimed to investigate effects of different glucocorticoid administration methods on sudden hearing loss and Meniere’s disease.Methods:In this study,glucocorticoids were administered orally in 18 patients,by retroauricular injection in 15 patients and by intratympanic injection in 15 patients.White blood cell(WBC)count,serum Kt,fasting plasma glucose(FPG),body temperature,heart rate and blood pressure were used to evaluate effects of glucocorticoids on patients with hearing loss.Visual analog scale(VAS)of pain and sleep disorders were also surveyed,and pure tone audiometry(PTA)results were compared among groups to evaluate efficacy of different glucocorticoids administration methods.Result:WBC count,heart rate and blood pressure were higher in patients taking oral glucocorticoids,while body temperature,serum Kt and FPG levels did not change in all three groups.However,patients who received intratympanic injection of glucocorticoids experienced more pain,while those taking oral glucocorticoids reported more sleep impairment.Treatment efficacy on hearing loss was not significantly different among the three groups.Conclusion:These findings suggest that systemic glucocorticoid administration can result in greater whole body responses than local administration,but with similar hearing treatment efficacy.
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Objective:Recent studies have shown that chronic inflammation contributes to the development of sudden sensorineural hearing loss(SSNHL).Some hematologic parameters have also been linked to the prognosis of SSNHL.However,the prognostic value of such hematological factors is not conclusive.This study explored the association of routine hematological parameters with SSNHL.Methods:A systematic literature search was conducted in PubMed,Cochrane Library,Web of Science and Embase to identify eligible studies.Standardized mean deviation(SMD)and the 95%confidence interval(CI)were retried from relevant studies for analysis.Heterogeneity,subgroup,and publication bias analyses were performed.Results:A total of 18 studies involving 1505 SSNHL patients and 1466 healthy persons were enrolled in the final analysis.The study population included 699 responders and 458 non-responders to treatment.Pooled results revealed that the neutrophil/lymphocyte ratio(NLR)and platelet/lymphocyte ratio(PLR)value in the SSNHL patient group were higher than in the healthy group(SMD=1.05,95%CI:0.86,1.24,p<0.001,SMD=0.52,95%CI:0.26,0.78,p<0.001,respectively).However,there was no significant difference in the mean platelet volumes(MPV)between the groups(SMD=0.03,95%CI:0.44,0.49,p=0.91).Notably,NLR and PLR values were evidently higher in the unrecovered group than in the recovered group(SMD=0.63,95%CI:1.02,0.23,p=0.002,SMD=0.4,95%CI:0.76,0.03,p=0.03,respectively).However,the MPV value was similar in both groups(SMD=0.35,95%CI:1.14,0.44,p=0.38).Conclusions:Our results show that NLR and PLR values can predict the onset and prognosis of SSNHL.
基金Supported by grants from National Natural Science Foundation of China: 30572019Xinhua Hospital Foundation, Shanghai Jiaotong University School of Medicine.
文摘Objective To establish an animal model of sudden onset sensorineural hearing loss (SSNHL) to study its mechanisms. Materials and methods The inner ear was exposed to 3-nitropropionic acid at 0.5 mol/L (3-NP (H)) and 0.3 mol/L (3-NP (L)) through the round window membrane for 30 minutes in 50 male guinea pigs. Thresholds of auditory brainstem responses(ABR) were established before the treatment and retested at 4 hours, 1 day, 3 days and 6 days following 3-NP exposure. Control animals were treated with phosphate buffered saline (PBS) and their ABRs were retested at 4 hours and 1 day after the treatment. Animals were monitored for nystagmus and postural signs of vestibular dysfunction, using a digital video camera, following the treatment procedure. Specimens were taken at 12 hours, 1 day, 3 days and 7 days following 3-NP(H) exposure and embedded in JB4 for light microscopy observation. Results ABRs were lost in all animals tested at 4 hours following 3-NP (H) exposure. The rate of complete ABR loss decreased as post-treatment test time increased. ABRs were lost in 80% (4/5) of the animals at 1 day after exposure to 3-NP (L). Spontaneous horizontal nystagmus with a fast phase away from the treated ear developed in all 3-NP (H)-treated animals and in 20 % ( 1/5) of the animals exposed to 3-NP (L), except for the one treated bilaterally. Various degree of postural disturbances consistent with unilateral vestibular dysfunction, such as spontaneous barrel rolling towards the exposure side while walking, were seen in all animals exposed to 3-NP(H) and 40% (2/5) of animals exposed to 3-NP(L), except for the one animal treated bilaterally, which showed no signs of imbalance. Both nystagmus and postural disturbances resolved in 2 days following 3-NP exposure. Histological study showed temporary edema tin the organ or Corti, Claudius cells and the inner sulcus cells 3 days after 3-NP (H) treatment. Enlargement of intercellular space in the spiral prominence was first noticed at 12 hours post-3-NP (H) exposure, progressed at day 3 and recovered at day 7. Vacuoles in the cellular plasm and nucleus was seen as early as at 12 hours post-3-NP exposure in the spiral ganglion cells, and signs of degeneration were visible at day 7. Conclusion Inner ear exposure to 3-NP through the round window membrane appears to reproduce clinic manifestations and may serve as a legitimate animal model of
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Sudden deafness is one of the commonest acute symptoms in otolaryngology.In recent years,the incidence of sudden deafness is on the rise all over the world,but its etiology is still unclear,and the prognosis is greatly different.Traditional Chinese medicine(TCM)believes that the onset of sudden deafness is related to the liver,gallbladder,heart and triple energizer meridians.This article mainly explored the key points of common syndromes,TCM nursing methods and health guidance of sudden deafness.
文摘Objective: To assess the outcomes of intratympanic steroids in the management of idiopathic sudden sensorineural hearing loss. Study Design: Retrospective study. Setting: Academic tertiary care medical centre. Materials and Methods: A total of 67 patients with idiopathic sudden sensorineural hearing loss were included in our study. Pre- and post-injection audiograms were taken to assess the outcomes in improvement in hearing. Hearing outcomes were assessed with respect to duration of onset of treatment and age of the patient. Improvement in the speech discrimination score was also assessed. Results: A total of 67 patients were included in our study of which unilateral cases were 59 and bilateral cases were 8. Subjectively 35 patients reported improvement in hearing after the therapy while objectively we found 44 ears [41 patients] had improvement. The mean PTA pre ITS was 62.7 and after ITS it was 56.3. Maximum improvement was noted at 2000 Hz and 1000 Hz. Conclusion: Intratympanic steroids can be offered as a first line therapy for idiopathic sudden sensorineural hearing loss as it is minimally invasive, and can be performed as an office based procedure with no systemic side effects. No major complications have been reported in our study and results have been satisfactory.
基金supported by a grant from Nanjing Medical Development Foundation(NO: YKK05098)
文摘Objective To explore the relationship between sudden sensorineural hearing loss(SSNHL) and vascular risk factors(including serum lipids and uric acid).Method This is a retrospective analysis of 100 cases of SSNHL seen at the Drum Tower Hospital,Nanjing Medical University,between Jan.2007 and Apr.2008。 Patient history,blood test results and imaging scans were analyzed.Levels of triglyceride(TG),cholesterols(CHO),high density lipoprotein-cholesterol(HDL-CH),low density lipoprotein-cholesterol(LDL-CH),apolipoprotein AI(ApoAI),apolipoprotein B(ApoB) and uric acid(UA) from these patients were compared with a control group of 56 patients treated for vocal cord polyps or nasal septum deviation during the same period.Patients with hypertension,diabetes,heart,brain,liver or kidney disorders are excluded from the present investigation.Results HDLCH level was higher and UA level lower in the study group than the control group(P < 0.05).HDL-CH and UA showed no significant differences among different age-groups(P > 0.05).There were no significant differences in the levels of TG,CHO,LDL-CH,ApoAI and ApoB(P > 0.05).Conclusion These data indicate that metabolic disturbances of serum lipids and/or uric acid may be potential risk factors for
文摘Objectives:This study aimed to determine the prognostic value of otoacoustic emissions(OAEs)in idiopathic sudden sensorineural hearing loss patients.Methods:The study included 30 subjects with unilateral idiopathic sudden sensorineural hearing loss(ISSNHL).Each patient was evaluated four times:at baseline and after one week,one month,and three months of treatment.During each visit,each patient was subjected to full audiological history,otoscopic examination,basic audiological evaluations,and transiently evoked and distortion product otoacoustic emission(TEOAEs&DEOAEs).Results:The hearing thresholds(frequency range 250e8000 Hz)and word recognition scores of patients with detectable TEOAEs and DPOAEs improved significantly,whereas no significant improvements were observed in those with no response.Conclusion:Hearing improvement is better in patients with detectable TEOAEs and DPOAEs.As a result,TEOAEs and DPOAEs are recommended as routine tests in all SSNHL patients to predict outcomes and monitor treatment as TEOAEs and DPOAEs reflect the cochlear OHCs activity.
基金the National Natural Science Foundation of China(81771006).
文摘Objectives:To evaluate the pathological changes in the blood-perilymph,blood-endolymph,and bloodnerve barriers of a patient with idiopathic sudden sensorineural hearing loss(SSNHL).Methods:Potential ossification or fibrosis in the inner ear was evaluated using temporal bone CT and MRI acquired using the 3-dimensional T2-weighted sampling perfection with application-optimized contrasts using a flip angle evolution sequence.Pathological changes in the barriers were analyzed by MRI obtained 4 h after a single-dose intravenous injection of gadolinium chelate using a medium inversiontime inversion recovery imaging with magnitude reconstruction sequence.Results:The perilymph was absent,while significant enhancements of the vestibulocochlear nerve and the endolymphatic compartments were detected.Conclusion:Significant injuries in the blood-endolymph and blood-vestibulocochlear nerve barriers and disabled perilymph production may contribute to the development of SSNHL with poor response to treatments.
文摘Rationale:Dengue is the most important human arboviral disease.Neurological manifestations occur rarely in dengue.To the best of our knowledge,there is only one reported case of dengue-associated sudden sensorineural hearing loss(SSNHL)in Brazil untill now.Patient concerns:A 42-year-old man was presented to the emergency department with unconsciousness and generalized tonicclonic seizures.Diagnosis:Dengue-associated aseptic meningitis,acute kidney injury,and SSNHL.Interventions:The patient was treated with anticonvulsants and thiamine and underwent mechanical ventilation.He received combined ceftriaxone and acyclovir,which were later switched to meropenem,acyclovir and ampicillin empirically until culture results became available.He also required hemodialysis and plasmapheresis sessions and fresh frozen plasma and buffy coat transfusions until definitive diagnosis.Outcomes:The patient was discharged after improvement of his general condition and of his blood test results,but hearing loss remained.A six-month follow-up visit showed persistent deafness.Lessons:Dengue should be included in the differential diagnosis of patients from dengue-endemic areas presenting an acute febrile disease with neurological manifestations.To the best of our knowledge,this is the second reported case of dengueassociated SSNHL,suggesting an association between dengue and development of SSNHL.
基金supported by the National Natural Science Foundation of China(No.81830028,No.81900950 and No.81900951).
文摘Objective To investigate the genetic causes of sudden sensorineural hearing loss(SSNHL)patients in China.This study focused on analyzing variations of coding sequence of common genes related to deafness,revealing the molecular pathogenesis of sudden deafness from a genomics perspective,discovering molecular markers associated with the onset of deafness,and then supplying prevention to high-risk populations,classifying disease according to accurate etiology,and choosing a much more precision therapy.Methods We retrospectively analyzed the clinical characteristics of 51 patients diagnosed as SSNHL with vertigo treated in the Chinese PLA General Hospital.In this study,mutation screening of 307 nuclear genes and mitochondrial genome responsible for human or mouse deafness was performed on the 51 cases of unilateral sudden deafness patients with vertigo.Results We identified 51 cases of unilateral sudden deafness,including 2 cases of low-mid frequency hearing impairment,18 cases of mid-high frequency hearing loss,11 cases of flat-type hearing loss,and 20 cases of all frequency hearing loss.Among the 51 cases,8(15.69%)cases of GJB2 heterozygous variations,1(1.96%)case of GJB3 heterozygous variations,5(9.8%)cases of SLC26A4 heterozygous variations,2(3.92%)cases of COCH heterozygous variations,14(27.45%)cases of CDH23 heterozygous variations,14(27.45%)cases of OTOF heterozygous variations,1(1.96%)case of SLC17A8 heterozygous variations and 2(3.92%)cases of KCNE1 heterozygous variations.No mtDNA gene variations were identified.Conclusion SSNHL has some relationship with hereditary in Chinese population,but its complex genetic pathogenic mechanisms need further study.
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 Ig G 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 immuneassociated 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 PIK3 CD gene, a C896 T substitute in exon 7 was detected. This mutation causes primary immunodeficiency and is an autosomal dominant disease.Conclusion: The PIK3 CD C896T mutation responsible for primary immunodeficiency may contribute to the onset of bilateral SSNHL with subsequent rapid progression.
