Treatment of idiopathic sudden sensorineural hearing loss

CONCLUSION: The therapeutic role of corticosteroids and/or corticosteroids with antiviral agents for sudden sensorineural hearing loss (SSNHL) has yet to be fully elucidated; however, in cases where deafness is profound and of recent onset, a therapeutic trial is indicated. OBJECTIVES: To investigate treatment regimens and their efficacies, as well as evaluating the potential prognostic correlates and allowing comparison between local and national standards of care for SSNHL. PATIENTS AND METHODS: A retrospective evidence-based case series of 143 patients seen at the University of Rochester, Department of Otolaryngology between 1999 and 2002 was investigated. Treatment modalities included (1) observation, (2) steroids, and (3) steroids with antivirals. RESULTS: The study demonstrates that steroid treatment, alone or in combination with antivirals, results in a significant improvement rate compared with observation. Results indicate that the more expediently a patient with SSNHL is seen by an otolaryngologist, the better their prognosis.     

Intratympanic steroids for idiopathic sudden sensorineural hearing loss

OBJECTIVES: We undertook to evaluate the effectiveness of intratympanic (IT) steroid injections for treating idiopathic sudden sensorineural hearing loss (ISSHL) by performing a retrospective case series study in a private otology practice. METHODS: A total of 21 eligible patients with ISSHL were included. We defined ISSHL as a hearing loss of 20 dB or more at at least 3 consecutive audiometric frequencies that develops within 72 hours or less and cannot be attributed to any commonly identifiable cause of sudden hearing loss. Three IT injections of 0.4 mL of 62.5 mg/mL methylprednisolone solution were administered 1 week apart. The end point for the study was a clinically significant change in audiometric values, with a positive response determined to be a 10-dB or greater improvement in the 4-tone pure tone average and/ or a 15% or greater improvement in the word discrimination score. Audiometric data were recorded just before therapy and 1 week after the last IT treatment. The potentially confounding variables recorded included age, sex, "prompt treatment" (defined as treatment within 14 days from onset), concurrent or prior treatment with oral steroids, and severity of hearing loss. RESULTS: The overall response rate to the IT steroid protocol was 67% (14 of 21 patients), with a 95% confidence interval of 43% to 85%. Backward stepwise logistic regression identified "prompt treatment" as the only variable that significantly affected the outcome. The response rate of the "prompt treatment" cohort was 91% (10 of 11 patients), with a 95% confidence interval of 59% to 100%. CONCLUSIONS: These findings support the use of IT steroids as an early intervention in the treatment of ISSHL.     

Alpha-interferon for the treatment of idiopathic sudden sensorineural hearing loss

We have employed alpha-interferon (IFN-α), an anti-viral agent, in the treatment of severe idiopathic sudden sensorineural hearing loss (ISSHL). Forty-two patients were studied and had an average hearing ability of ≥ 70 dB before treatment. We also examined 2′-5′ oligoadenylate synthetase (2,5A-S) activity, one of the parameters indicating anti-viral activity of IFN, to investigate the relationship between the suppression of viral proliferation and prognosis and explain the pathogenesis of ISSHL. Complete recovery was found in 27 patients (64.3%) after IFN therapy. Increased 2,5A-S activity was observed on the 3rd day of IFN therapy in 24 of the 27 patients who completely recovered. No severe adverse events were reported after IFN therapy. Findings suggest that IFN therapy may be effective and safe in the treatment of ISSHL and calls for further investigation.     

Modified intratympanic treatment for idiopathic sudden sensorineural hearing loss

Steroids are the only proven drugs in the treatment for idiopathic sudden sensorineural hearing loss. In the recent studies, it has been suggested that, steroids delivered through the intratympanic route obtained higher perilymph levels, resulting in better hearing outcomes. The purpose of this study is to compare the hearing outcomes of the two routes of steroid treatment: intratympanic route and systemic route. In this prospective study, 60 consecutive patients with idiopathic sensorineural hearing loss treated between January 2005 and September 2008 were enrolled: 29 were in the intratympanic steroid group (ITSG) and 31 were in the systemic steroid group (SSG). In the ITSG, 5 intratympanic injections of dexamethasone were performed with the dose of 4 mg/ml, consecutively. Oral methylprednisolone was given at the dose of 1 mg/kg, tapered every 2 days and stopped at 10 days, in the SSG. The pure tone averages (PTA), speech discrimination scores (SDS) and the percentage of the patients who made an improvement more than 10 dB were analyzed on the tenth day and 2 months after the treatment statistically. The improvement in PTA on tenth day and second month after treatment was 31.38 and 37.55 dB, in the ITSG and 19.35 and 20.68 dB in the SSG, respectively. The improvement in SDS in the same time period was 35.24 and 37.52% in the ITSG and 20.13 and 19.61% in the SSG, respectively. Also, 25 of the 29 patients (86.2%) in the ITSG and 16 of the 31 patients (51.6%) in the SSG made an improvement more than 10 dB on PTA in the second month control. Intratympanic steroids gave better hearing results than systemic steroids with no systemic side effects. Studies with more sample sizes will identify the best steroid for injection, application time, frequency and dose.     

Intratympanic corticosteroids for sudden idiopathic sensorineural hearing loss.

OBJECTIVE: To determine whether instillation of intratympanic steroids is effective in the treatment of sudden idiopathic sensorineural hearing loss. METHODS: A retrospective chart review of all patients who underwent intratympanic steroid treatment (methylprednisolone and/or dexamethasone) between 1996 and 2002 at a tertiary care university otology clinic. Thirty-three patients were identified, of which 26 met inclusion criteria for having an idiopathic hearing loss. Pretreatment and posttreatment pure-tone audiograms and speech discrimination scores were compared. RESULTS: Overall, there was a 27.2 +/- 5.7 dB improvement in the pure-tone thresholds and a 25.4 +/- 6.2% improvement in speech discrimination scores. Those treated within 10 days of onset had a statistically significant better outcome than those treated after 10 days. No adverse reactions or complications were reported. CONCLUSION: Instillation of intratympanic steroids represents a safe and potentially effective treatment of sudden idiopathic sensorineural hearing loss.     

突发性聋临床分析

目的探讨突发性聋的治疗方法及预后因素。方法96例突发性聋病例采用川芎嗪、皮质类固醇激素、抗病毒药物为主的综合治疗,其中43例辅以高压氧治疗,分析患者年龄、初诊听力损失程度、听力曲线类型、治疗早晚、有无眩晕与预后的关系。结果痊愈25例,显效46例,痊愈率为26.04%,痊愈、显效率为73.96%;重度听力损失组,高压氧治疗组痊愈、显效率高于非高压氧治疗组(P<0.05);年龄18～40岁组,发病后10天内就诊组,轻、中度听力损失组,初诊听力曲线为平坦型和上升型组,无眩晕组疗效分别好于对照组(P<0.05)。结论年龄18～40岁,发病后10天内就诊,轻、中度听力损失组,初诊听力曲线为平坦型和上升型,无眩晕者预后好。     

The pathology of idiopathic sudden sensorineural hearing loss

Summary We examined the temporal bone pathologies in 12 ears with idiopathic sudden sensorineural hearing loss, and found that the lesions present in these specimens and in 10 others reported in the literature are similar to lesions occurring in known cases of viral cochleitis. These lesions are unlike those resulting from known vascular causes.Supported by grant no. 5 R01 NS05881 from the National Institute of Neurological and Communicative Disorders and Stroke     

The pathology of idiopathic sudden sensorineural hearing loss

We examined the temporal bone pathologies in 12 ears with idiopathic sudden sensorineural hearing loss, and found that the lesions present in these specimens and in 10 others reported in the literature are similar to lesions occurring in known cases of viral cochleitis. These lesions are unlike those resulting from known vascular causes.     

A follow-up study of patients suffering from sudden sensorineural hearing loss.

Sudden sensorineural hearing loss (S-SNHL) is a common problem with a high recovery rate. However, little is known of the long-term prognosis of affected patients. The purpose of this follow-up study was to evaluate the long-term hearing results of S-SNHL patients. The sample consisted of 168 patients with S-SNHL treated with carbogen inhalation and/or anticoagulant therapy during the period 1982-89. A questionnaire was sent to these patients, and audiological investigations were carried out in a selection of these patients in 1997. Comparison of the different treatment methods showed that the difference observed in improvement of hearing was statistically significant between the carbogen inhalation and anticoagulant treatment groups. The hearing improvement achieved was stable for, on average, 8 years of follow-up. During the follow-up period, Ménière's disease was diagnosed in only 1 of the 116 patients who answered the questionnaire and no cases of acoustic neurinoma were diagnosed, indicating that establishment of a careful patient history and clinical and audiological investigations are sufficient for the diagnosis of S-SNHL. In general, the hearing improvement achieved in S-SNHL patients is stable during long-term follow-up.     

Effect of single-drug treatment on idiopathic sudden sensorineural hearing loss

OBJECTIVES: In order to evaluate the effect of a medical administration for the sudden deafness patients, single-drug treatment for idiopathic sudden sensorineural hearing loss (ISSHL) was assessed at multi-centers participating in the Acute Severe Hearing Loss Study Group sponsored by the Ministry of Health, Labor and Welfare of Japan. METHODS: The subjects consisted of ISSHL patients who were (1) 20 years of age or older, (2) diagnosed within 2 weeks after the onset of hearing loss, (3) showing a mean hearing level of 40-90 dB at five frequencies from 250 to 4000 Hz, (4) previously untreated, and (5) with normal for age in hearing of the opposite ear. The drugs used in this study were ATP, alprostadil, hydrocortisone and amidotrizoate, which were administered intravenously, and beraprost sodium and betamethasone, which were given orally. Two drugs were assigned to each center, one of which was selected according to the code hidden in envelopes and administered for 1 week. The treatment after the single-drug administration was conducted at the discretion of each center. The hearing gain and recovery rate at 1 week after the initiation of single-drug treatment and at 1 month or over when the hearing level was fixed, were evaluated based on the criteria for hearing recovery prepared by the Acute Severe Hearing Loss Study Group. RESULTS: There was no statistically significant difference in the recovery rate among drugs either at 1 week after the initiation of single-drug treatment or at the time of fixed hearing level. At the time when the hearing level was fixed, a statistically significant difference in the complete recovery rate was detected only between amidotrizoate and beraprost sodium. CONCLUSION: From these results, we could not find any specific drugs recommended for ISSNHL. In evaluating the effect of the drugs, however, several problems in the clinical trial for ISSHL should be considered.     

Glucocorticoid influence on prognosis of idiopathic sudden sensorineural hearing loss

IntroductionIdiopathic Sudden Sensorineural Hearing Loss (ISSHL) is defined when a loss of at least 30 dB occurs in over 3 continuous frequencies, in up to 72 hours, of which etiology is not established, despite adequate investigation. Different types of treatment regimens have been proposed, but only glucocorticoids have shown some evidence of benefit in the literature.ObjectiveTo analyze whether the type of treatment or time of treatment with glucocorticoids have any influence on hearing recovery in ISSHL.MethodsObservational retrospective cohort study. One hundred twenty-seven patients with ISSHL, treated at outpatient clinics between the years 2000 and 2010, were studied. We evaluated the prognostic correlation of the type of treatment and time to treatment with glucocorticoids and ISSHL.ResultsThe absolute hearing gain and the relative hearing gain was as follows: 23.6 dB and 37.2%. Complete recovery was observed in 15.7% of patients, significant recovery in 27.6% and recovery in 57.5%.ConclusionIn this study, there was no difference between the use and nonuse of glucocorticoids in hearing improvement. However, when started within seven days after onset, the use of glucocorticoids was a factor of better prognosis.     

影响突发性聋预后因素的临床研究

目的 研究影响突发性聋预后的相关因素,提高突发性聋预后的判断.方法 回顾性分析2006年1月至2007年3月复旦大学附属眼耳鼻喉科医院收治的突发性聋患者,首先去除初始听阈≤40 dB的低频聋患者,得到882例进行疗效分析.初始听阈＞40 dB的患者按不同的初始听阈形态与严重程度分为下坡型组(69例)、上坡型组(24例)、平坦型组(139例)、凹陷型组(44例)、极重度聋(126例)和全聋(86例)6组.结果 病程3 d内疗效最佳,其次是1、2周内,超过2周疗效差,3周内和1个月内、1个月后差异无统计学意义.病程2周内患者初始听阈＞40 dB组中凹陷型组与其他组相比预后最佳,恢复率达97.7%.极重度聋组和其他组(除全聋组)相比,治愈率(23.8%)及恢复率(57.9%)低,但好于全聋组.全聋组的预后最差,无效率达67.4%.合并有糖尿病、高血压患者的预后与其他不伴此病的患者相比差异具有统计学意义(H=4.455,P=0.0348).年龄与预后有关,年龄越大,预后也越差,以50岁为界,＜50岁的患者的预后要好于≥50岁的患者,差异有统计学意义(H=7.739,P=0.0054).结论 病程大于2周的患者疗效差,不同的初始听阈形态与听力损失程度是影响突发性聋预后的重要因素.年龄越大预后越差.合并有高血压、糖尿病的患者,其预后比无任何合并症的患者差.     

Positive family history of idiopathic sudden sensorineural hearing loss

Idiopathic sudden sensorineural hearing loss (ISSNHL) is a heterogenic disease. Multiple factors influencing aetiology and prognosis are discussed. A retrospective clinical characterisation and analysis of family history of ISSNHL patients was performed to investigate influences on the disease. 186 inpatients diagnosed with ISSNHL were characterised by health records and a standardised questionnaire. Audiograms were observed. 75 controls that had never experienced an event of ISSNHL were questioned about family members being affected by ISSNHL. 63.4% of all patients could be assigned to at least one group with similar causes of ISSNHL (noise exposure, positive family history, infectious diseases, hypothyreoidism and fibromuscular dysplasia). A positive family history for ISSNHL has not been reported so far. Therefore, we accentuated the characterisation of patients with positive family history. 21.4% affirmed a positive family history. In ten families, at least two family members were reported as ISSNHL patients. In comparison with patients with negative family history, they tend to be younger, experience more events of ISSNHL and show less improvement of hearing abilities under therapeutic treatment (non-significant). Differences intensified between smokers with positive family history and non-smokers with negative family history. Differences concerning average age were statistically significant (p = 0.001). Within 75 controls 11 families were reported with one member being affected by ISSNHL. In the control group we did not detect any family with more than one ISSNHL patient. The results indicated that patients with positive family history tend to have an aggravated course of ISSNHL. Further studies should help to confirm these results and to identify environmental or genetic factors leading to ISSNHL. This might support a better understanding of the aetiology of ISSNHL and offer new possibilities for prevention and therapy.     

Prognostic factors of profound idiopathic sudden sensorineural hearing loss

Profound idiopathic sudden sensorineural hearing loss is thought to have a poor prognosis, but few studies have focused on this condition. We aimed to assess the impact of patient factors, audiologic parameters, and salvage intratympanic steroid injection therapy on the prognosis of profound idiopathic sudden sensorineural hearing loss. The demographic, clinical, and audiologic data, degree of hearing recovery, and efficacy of intratympanic steroid injection therapy in 576 patients with profound idiopathic sudden sensorineural hearing loss (mean age 56.2 ± 14.9 years) who had been admitted at four tertiary referral centers between 2000 and 2011 were retrospectively reviewed. The mean hearing level at the initial presentation was 108.1 ± 9.5 dB. Many patients experienced vertigo (52.1 %) and tinnitus (77.4 %). At the 2-month follow-up, 172 (29.8 %) patients showed some degree of hearing recovery, but only 21 (3.6 %) patients recovered normal hearing. Further, the 116 patients who had received salvage intratympanic steroid injections showed a better audiologic outcome (improvement, 26.1 ± 24.3 vs. 15.7 ± 22.1 dB; P = 0.000) than those who had not (n = 429). In conclusion, a higher degree of hearing loss at the initial presentation indicates a poorer prognosis. Salvage intratympanic steroid injection therapy may improve the hearing of patients with profound idiopathic sudden sensorineural hearing loss after the failure of systemic steroid therapy.     

Electrocochleographic findings in recurrent idiopathic sudden sensorineural hearing loss

Abstract Conclusion: The main differences in electrocochleography (ECochG) between recurrent and nonrecurrent idiopathic sudden sensorineural hearing loss (iSSNHL) were the high prevalence of an abnormally increased action potential (AP) recovery (iR) in nonrecurrent iSSNHL and the very low prevalence of iR in recurrent iSSNHL. An enhanced SP/AP ratio, low AP threshold, low initial hearing level, and up-sloping type of audiogram appear to be favorable prognostic factors in recurrent iSSNHL. Objectives: This study aimed to characterize differences in ECochG findings and other confounding prognostic factors between recurrent and nonrecurrent iSSNHL and to estimate how these findings are related to hearing outcome in recurrent iSSNHL. Methods: ECochG was performed transtympanically in 23 and 42 ears with recurrent and nonrecurrent iSSNHL, respectively. AP recovery was measured using a paired-click stimulation paradigm. Differences in ECochG findings associated with confounding prognostic factors between recurrent and nonrecurrent iSSNHL, and between three outcome groups were assessed. Results: iR was significantly more common in nonrecurrent iSSNHL (16 ears) than in recurrent iSSNHL (1 ear). In the outcome of recurrent iSSNHL, the prevalence rates of an enhanced summating potential (SP)/AP ratio, lower AP threshold, lower initial hearing level, and up-sloping type of audiogram were significantly higher in the cure group than in the improvement and no-recovery outcome groups.