A novel PIK3CD C896T mutation detected in bilateral sudden sensorineural hearing loss using next generation sequencing:An indication of primary immunodeficiency

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.

Cerebellar artery infarction with sudden hearing loss and vertigo as initial symptoms: A case report

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.

Impacts of different methylprednisolone administration routes in patients with sudden hearing loss or Meniere’s disease

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.

Creutzfeldt-Jakob disease presenting with bilateral hearing loss:A case report

BACKGROUND Sporadic Creutzfeldt-Jakob disease(s CJD)is a prion disease characterized as a fatal transmissible neurodegenerative disorder.Dizziness is often the first presenting symptom of s CJD,but hearing loss as an early manifestation is very rare.CASE SUMMARY A 76-year-old man presented with bilateral sudden hearing impairment and dizziness for 10 d.He was taking medications for hypertension and diabetes.He denied any difficulty with activities of daily living or hearing impairment before the onset of symptoms.Pure tone audiometry showed bilateral severe hearing impairment.Brain magnetic resonance imaging(MRI)and laboratory tests were within normal limits.Given his diagnosis of sudden sensory hearing loss,the patient received corticosteroid treatment but it was ineffective.Two weeks later,he complained of aggravated gait impairment,disorientation,and cognitive impairment.Repeat brain MRI showed diffuse cortical high signal intensities on diffusion-weighted imaging.In cerebrospinal fluid analysis,the real-time quaking-induced conversion assay was positive,and 14-3-3 protein was detected in the by western blotting.Considering all the data,we diagnosed probable s CJD,and the patient’s symptoms rapidly progressed into akinetic mutism.CONCLUSION For patients with abrupt bilateral hearing impairment,especially in the elderly,various differential diagnoses,including s CJD,should be considered.

CASE REPORT: TOTAL SUDDEN HEARING LOSS WITH BENIGN PAROXYSMAL POSITIONAL VERTIGO

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]

Intratympanic vs systemic use of steroids as first-line treatment for sudden hearing loss:A meta-analysis of randomized,controlled trials

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.

Clinical characteristics of sudden hearing loss during pregnancy

Objective:The objective of this study was to explore the clinical characteristics and management of sudden hearing loss(HL)during pregnancy,thus better guiding the clinical practice.Methods:The clinical and follow-up data of 17 patients(17 ears)with sudden HL during pregnancy were analyzed retrospectively(the observe group).Twelve nonpregnant female patients(12 ears)with sudden HL of similar clinical characteristics were selected as the control group.The prognosis of the two groups was compared.All the patients were followed up after delivery,and two of them were readmitted to the hospital 1–2 months after delivery.Results:The observe group had better improvement in hearing and a higher response rate compared to the control group.The pure tone hearing and speech recognition rate of patients could still be improved after the readmitted treatment,and the hearing could partially recover spontaneously during follow-up.The laboratory indicators that affect the inflammatory response and coagulation pathway were significantly different between the two groups.Conclusions:The hearing condition of sudden HL during pregnancy is severe,and the prognosis of these patients is better than nonpregnant patients of similar clinical characteristics.Postpartum treatment is still effective,and some patients showed self-healing with time during follow-up.The inflammatory response and coagulation function may affect the hearing of patients through a metabolic pathway.

Modified intratympanic steroid therapy for sudden sensorineural hearing loss via tympanic tube and gelfoam as a salvage treatment

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.

Clinical characteristics and analysis of vestibular-evoked myogenic potentials in patients with sudden sensorineural hearing loss in different ages

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.

Therapeutic efficacy of methylprednisolone sodium succinate via diverse administration routes for mid-to high-frequency sudden sensorineural hearing loss

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.

Comparison between Bilateral and Unilateral Sudden Sensorineural Hearing Loss

Background：Bilateral sudden sensorineural hearing loss （BSSHL） is rare and assumed to be a different clinical entity compared to unilateral SSHL （USSHL）.This study examined the differences between the idiopathic BSSHL and USSHL.Methods：Forty-six sequential BSSHL patients （Se-BSSHL） and 68 simultaneous BSSHL （Si-BSSHL） were consecutively admitted between June 2008 and December 2015.Two sets of patients served as control groups：（1） USSHL patients with healthy contralateral ear and （2） USSHL patients with contralateral preexisting hearing loss （USSHLwCHL）.We retrospectively analyzed differences among four cohorts using analysis of variance,Kruskal-Wallis test,Welch＇s t-test,and Chi-square test as appropriate before and after propensity score matching （PSM） based on age,gender,and body mass index （BMI）.Results：The prevalence of idiopathic BSSHL was 8.6% （114/1329） among the total SSHL patients.In the total cohort,USSHL patients tended to be younger,female,and tended to have lower BMI,renal parameters,and total cholesterol in addition to higher high-density lipoprotein compared to the other three groups.Most routine blood indicators,some coagulation markers,and immunoglobulin M （H =13.4,P =0.004） were significantly different among the study groups.After PSM,the major significant differences were found in audiometric characteristics.Si-BSSHL and Se-BSSHL patients demonstrated similar hearing thresholds as USSHL but were significantly better than the USSHLwCHL patients across most frequencies before and after treatment （H =30.0,P 〈 0.001 for initial hearing and H =12.0,P =0.007 for final hearing）.Moreover,the BSSHL patients showed different hearing loss distribution patterns （more descending type,x2 =33.8,P =0.001） with less hearing gain （H =17.5,P 〈 0.001） compared to the USSHL patients.Conclusions：Idiopathic BSSHL is a relatively rare subtype of SSHL with a higher rate of descending audiogram type and inferior hearing outcome rather than being classified as a completely different disease entity compared to USSHL.

Reflections on Bilateral Cochlear Implants

The bilateral cochlear implant has increased in recent years in due to the search for auditory enhancements. There are many advantages to the users of the bilateral cochlear implant, which may include the location of the auditory sign, decreasing the head shadow effect for the contralateral ear and binaural summation of the hearing. In children it is also discussed the issue of auditory deprivation. The aim of this review was to present a reflection on issues related to the bilateral cochlear implant, allowing the reader to do a search and strengthen scientifically with this issue, giving theoretical foundation to better guide and advise their patients.

Audiological characteristics and exploratory treatment of a rare condition of acute-otitis-media-associated sudden sensorineural hearing loss

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.

Sudden sensorineural hearing loss(SSHL) following a local anesthetic dental procedure

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.

Sudden sensorineural hearing loss-A contemporary review of management issues

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.

Hyperbaric Oxygen Treatment Improves Hearing Level via Attenuating TLR4/NF-κB Mediated Inflammation in Sudden Sensorineural Hearing Loss Patients

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.

Idiopathic sudden sensorineural hearing loss:Effectiveness of salvage treatment with low-dose intratympanic dexamethasone

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.

Intratympanic dexamethasone injection for sudden sensorineural hearing loss in pregnancy

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.

The Role of Endolymphatic Hydrops in Patients with Pantonal Idiopathic Sudden Sensorineural Hearing Loss:A Cause or Secondary Reaction

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.

Assessment of Balance and Vestibular Functions in Patients with Idiopathic Sudden Sensorineural Hearing Loss

This study investigated the relationship among the severity of hearing impairment,vestibular function and balance function in patients with idiopathic sudden sensorineural hearing loss（ISSNHL）.A total of 35 ISSNHL patients（including 21 patients with vertigo） were enrolled.All of the patients underwent audiometry,sensory organization test（SOT）,caloric test,cervical vestibular-evoked myogenic potential（cV EMP） test and ocular vestibular-evoked myogenic potential（o VEMP） test.Significant relationship was found between vertigo and hearing loss grade（P=0.009）,and between SOT VEST grade and hearing loss grade（P=0.001）.The abnormal rate of o VEMP test was the highest,followed by the abnormal rates of caloric and c VEMP tests,not only in patients with vertigo but also in those without vertigo.The vestibular end organs were more susceptible to damage in patients with vertigo（compared with patients without vertigo）.Significant relationship was found between presence of vertigo and SOT VEST grade（P=0.010）.We demonstrated that vestibular end organs may be impaired not only in patients with vertigo but also in patients without vertigo.The cochlear and vestibular impairment could be more serious in patients with vertigo than in those without vertigo.Vertigo does not necessarily bear a causal relationship with the impairment of the vestibular end organs.SOT VEST grade could be used to reflect the presence of vertigo state in the ISSNHL patients.Apart from audiometry,the function of peripheral vestibular end organs and balance function should be evaluated to comprehensively understand ISSNHL.Better assessment of the condition will help us in clinical diagnosis,treatment and prognosis evaluation of ISSNHL.