Outcomes research analysis of continuous intratympanic glucocorticoid delivery in patients with acute severe to profound hearing loss: basis for planning randomized controlled trials

CONCLUSIONS: The data presented herein form the basis for conducting randomized placebo-controlled clinical trials evaluating the safety and efficacy of salvage treatment in patients with idiopathic sudden severe sensorineural hearing loss (but not anacusis) refractory to initial systemic therapy. Comparison of different application protocols and drug delivery systems will allow assessment of the value of continuous versus intermittent intratympanic glucocorticoid drug delivery. OBJECTIVES: To describe and critically evaluate the results of continuous intratympanic glucocorticoid delivery in patients with acute unilateral severe and profound sensorineural hearing loss refractory to initial systemic therapy and to compare the outcome with a historical control group. MATERIAL AND METHODS: In a retrospective chart review, treatment results were analyzed in 23 patients with acute severe and profound hearing loss and failure of systemic standard therapy who received a continuous intratympanic delivery of glucocorticoids as a salvage treatment. Audiological results were compared within the local therapy group and with the results of an historical control group who did not receive salvage treatment. The study and control groups were matched with respect to hearing loss after initial systemic treatment failure. RESULTS: The average pure-tone threshold after intratympanic salvage treatment showed a statistically significant improvement of 15 dB (95% CI 7-24 dB; p<0.001). After exclusion of patients with complete anacusis, i.e. a non-measurable hearing threshold, the local therapy group showed a significantly better improvement (mean 19 dB; 95% CI 6-32 dB) than the historical control group (mean 5 dB; 95% CI -2-11 dB; p<0.05).     

Different prognoses in patients with profound sudden sensorineural hearing loss

Background: Inner ear hemorrhage is increasingly recognized as a cochlear lesion that can cause profound sudden sensorineural hearing loss (SSNHL).

Objectives: To investigate changes of cochlear and vestibular function and to compare therapeutic recovery from profound SSNHL induced by different etiologies.

Material and methods: Eighty patients with profound SSNHL (≥90?dB) were divided into an inner ear hemorrhage group and a non-inner ear hemorrhage group by MRI. Statistical analysis was performed to compare the therapeutic effects from vertigo and hearing loss and the outcomes of follow-up in the two groups.

Results: There were significant differences between the two groups in terms of the overall 14-day therapeutic response rate (20 vs. 48%), the incidence of imbalance (26.7 vs. 6%), the incidence of semicircular canal dysfunction on the affected side (60 vs. 20%), the incidence of abnormal C-VEMP and O-VEMP on the affected side (63.3 vs. 38%; and 60 vs. 30%, respectively), the average hearing threshold (74.2?±?10.7 vs. 53.6?±?11.4?dB), and the word recognition score (65.5?±?21.7 vs. 83.5?±?24.5%) at a 12-month follow-up.

Conclusions and significance: A higher percentage of patients with profound SSNHL induced by inner ear hemorrhage were associated with vertigo and had a poor prognosis.     

Prevalence of labyrinthine ossification in CT and MR imaging of patients with acute deafness to severe sensorineural hearing loss

Abstract

Objective: To evaluate the prevalence of labyrinthine ossification, and especially cochlear ossification, in a cohort of patients with unilateral sudden deafness or severe sensorineural hearing loss. Design: Retrospective data collection. Study sample: Sixty-four consecutive patients with unilateral sudden deafness or severe sensorineural hearing loss and either high-resolution CT (HRCT) of the temporal bone (isotropic spatial resolution ≤ 0.8 mm; n = 18) or high resolution CISS MRI (isotropic spatial resolution ≤ 1 mm; n = 55) were included. Nine patients underwent both imaging modalities. A standardized reading regarding labyrinthine ossifications was performed by an experienced head and neck radiologist blinded to clinical symptoms. Results: Radiologic signs of cochlear ossification were present in 14 patients (12 CT and 2 MRI). Eight patients showed unilateral and six patients bilateral signs of cochlear ossification. In all except one of the unilateral cases, the deafened ear was affected. Conclusions: Signs of cochlear ossification were found in an unexpectedly high rate (14/64, 22%) of patients with acute deafness. The data suggest HRCT of the temporal bone to be more sensitive to detect labyrinthine ossification than MRI. HRCT of the temporal bone should therefore be considered in patients with impaired recovery of acute deafness to exclude cochlear ossification; if present, and, in cases of early signs, the patient should be evaluated further to facilitate early cochlear implantation before progression impedes electrode insertion, reflecting latest developments considering cochlea implants for single-sided deafness to be effective.     

不同时机鼓室内注射糖皮质激素治疗重度以上突发性聋的疗效分析

目的 观察全身联合不同时机鼓室内注射糖皮质激素治疗重度以上突发性聋的疗效,探讨鼓室内注射时机,分析影响重度以上突发性聋预后的因素.方法 回顾性分析108例重度以上突发性聋患者的临床资料及治疗效果,其中全身联合初始鼓室组64例,全身联合延后鼓室组44例;对两组患者出院时与随访时疗效分别进行比较,并分析治疗有效组和无效组临...     

Intratympanic steroid injection as a first-line therapy in uremia patients with sudden sensorineural hearing loss

Conclusions: ITSI as a first-line therapy in uremia patients with SSNHL offers a valid and safe treatment compared with intravenous systemic steroid treatment. A specific pathophysiology caused by possible sodium pump paralysis may be explained for uremia patients with SSNHL. Objective: To compare the efficacy of intratympanic steroid injection (ITSI) with that of systemic intravenous steroids as a first-line therapy in uremia patients with sudden sensorineural hearing loss (SSNHL). Materials and methods: A total of 23 consecutive uremia patients with SSNHL were enrolled in this study. Patients were divided into two groups: the ITSI group (n = 15) and the non-ITSI group (n = 8), in which patients received intravenous systemic steroid treatment. The two groups were homogeneous in all respects. Results: The hearing improvement and relative gain were statistically significant between the two groups. The value of hearing gain (ΔPTA = PTA _pre – PTA _post) in the ITSI group and the non-ITSI group was 24.6 ± 16.4dB and 8.4 ± 19.3dB. The value of relative gain (ΔPTA/PTA_pre) in the ITIS group and the non-ITSI group was 31.1 ± 22% and 9.4 ± 20.5%. In the ITSI group, 11 patients (73.3%) exhibited hearing recovery (ΔPTA > 10 dB).     

Efficacy of intratympanic corticosteroid,intravenous batroxobin and combined treatment for sudden sensorineural hearing loss with type-2 diabetes

Background: Intratympanic corticosteroid (IC), intravenous batroxobin (IB) as the treatment for sudden sensorineural hearing loss (SSNHL) has been reported. However, the data on combination therapy (CT) was scarce.

Objective: The aim of this retrospective study was to compare the efficacy of IC, IB, and CT in the treatment of SSNHL with diabetes.

Material and Methods: A total of 212 SSNHL patients with diabetes, who were initially treated within 14 days of onset of disease, were divided into three groups by treatment modality. The hearing recovery was evaluated by the results of pure-tone test after completion of treatment. The prognostic factors, including age, severity of initial hearing loss, duration to onset of treatment, and audiometric curve type, were further compared.

Results: There was a significant difference in hearing recovery by the treatment (p?<?.05). Recovery rates in the CT group were significantly higher in patients with early treatment than with delayed treatment (p?=?.021). However, duration and recovery rate was not significantly correlated in IC and IB group (p?>?.05). In patients recieving early treatment, the recovery rate in CT group was significantly higher than that in IC (p?=?.013) and IB group (p?=?.029). Regardless of treatment, the recovery rates were higher in patients with flat and ascending audiograms (p?<?.05).

Conclusions and Significance: Patients receiving combined therapy, especially in the early stage of SSNHL, could achieve significantly superior recovery in the treatment of SSNHL with diabetes, compared with those using IC or IB alone.     

Severe to profound hearing loss in patients with progressed Alport's syndrome

Conclusion: The concept of hearing loss severity must be redefined, as there is a clear need for more active hearing management in Alport's syndrome patients with severe and profound hearing loss. Objectives: Sensorineural hearing loss (SNHL) caused by Alport's syndrome generally does not exceed 60–70 dB, because a cochlear lesion is responsible for this hearing loss. Careful management of renal function improves the prognosis and the longevity of Alport's syndrome patients; it is useful to reassess SNHL caused by Alport's syndrome. Patients and methods: Thirty-two patients with Alport's syndrome were analyzed retrospectively. Pure tone audiograms (PTAs), speech audiograms, and transiently evoked otoacoustic emissions (TEOAEs) were performed. Hearing loss severity was compared to duration of disease and severity of renal dysfunction. We also evaluated the correlation between OAEs and PTAs according to the hypothesis that evoked OAEs would be abnormal even in early stage SNHL in Alport's syndrome. Results: The level of hearing was positively correlated with disease duration. The hearing of the end-stage renal disease (ESRD) group, whose hearing threshold could exceed 70 dB, was worse than that of the non-ESRD group. OAEs were found in patients with normal hearing and mild hearing loss and had no significant early detection value.     

Clinical associations of serum antiendothelial cell antibodies in patients with sudden sensorineural hearing loss

OBJECTIVES/HYPOTHESIS: The role of antiendothelial cell antibodies in systemic vasculitis has been reported. The aim of the study was to define the clinical associations of serum antiendothelial cell antibodies in patients with sudden sensorineural hearing loss. STUDY DESIGN: A prospective study in patients with sudden sensorineural hearing loss. METHODS: Serum samples were taken from 59 consecutive patients with sudden sensorineural hearing loss at time of presentation and from 28 normal control subjects. Indirect immunofluorescence assay was used to detect antiendothelial cell antibodies. RESULTS: The prevalence of antiendothelial cell antibody detection was 54% (32 of 59 patients), with a statistically significant difference between patients and control subjects (P =.0004). Antiendothelial cell antibody positivity was significantly associated with absent recovery of hearing loss (P =.0020). CONCLUSIONS: The cytotoxicity to endothelial cells of the inner ear by antiendothelial cell antibody-positive sera might play a role in causing the stria vascularis damage in immune-mediated sudden sensorineural deafness. The appearance of antiendothelial cell antibody is related to the poor outcome of hearing loss, and its detection could be helpful in the selection of particular patients with sensorineural hearing loss for specific immunosuppressive treatments.     

感音神经性聋患儿中先天性内耳畸形的构成、影像学及临床听力学特征

目的:分析婴幼儿、儿童先天性感音神经性聋(SNHL)中先天性内耳畸形的构成、影像学及临床听力学特征。方法:回顾性分析2005-02-2010-01上海交通大学医学院附属上海儿童医学中心耳鼻咽喉科诊治的860例先天性SNHL患儿中,经颞骨高分辨率CT及MRI发现有先天性内耳畸形的125例(225耳)患儿的听力学及影像学资料。结果:860例先天性SNHL患儿中有先天性内耳畸形者占14.5%;累及双侧98例(78.4%),单侧27例(21.6%)。225耳中167耳(74.2%)为极重度聋,36耳(16%)为重度聋,22耳(9.8%)为中度聋。该组内耳畸形中,前庭水管扩大最多见(75.6%),其次为前庭畸形(32%),再次为耳蜗前庭畸形(23.1%)。耳蜗前庭畸形中以Mondini畸形最常见(55.8%),其次为共同腔畸形(28.9%)。累及耳蜗的内耳畸形中极重度聋者明显多于未累及耳蜗的内耳畸形中极重度聋者。结论:对了解中国婴幼儿、儿童先天性SNHL中先天性内耳畸形的构成,对先天性SNHL的病因诊断以及对包括助听器、耳蜗植入等在内的干预策略的制订及其预后有一定意义。     

Feeling of ear fullness in acute sensorineural hearing loss

Conclusions. This study revealed two characteristic findings of the feeling of ear fullness (FEF) in acute sensorineural hearing loss (ASNHL). First, that the appearance of FEF was not associated with the auditory function on audiograms; and second, that the disappearance of FEF is clearly associated with the hearing prognosis. Objective. FEF appears frequently in patients with ASNHL. However, there are few reports on this symptom and its mechanism has yet to be clarified. So we tried to investigate the character of FEF in ASNHL. Patients and methods. In this study, observations were made during the period from the first medical examination to 2 months after obtaining a steady audiogram on 227 ears from unilateral ASNHL cases, and the relationship between FEF and the audiograms was analyzed. Results. The FEF prevalence at the first medical examination was about 80% for any audiogram shape or hearing threshold. Moreover, the better the hearing prognosis, the higher the improvement rate of FEF.     

扬州市特教学校耳聋学生常见耳聋突变基因调查报告

目的:调查扬州地区非综合征性聋患儿常见耳聋突变基因的发病情况。方法:选择扬州市特教学校90例中、重度非综合征性聋学生为研究对象。在苏北人民医院医学检测中心利用耳聋基因芯片诊断试剂盒筛查常见的耳聋相关基因的9个热点突变,包括GJB2(35delG、176del16、235delC及299delAT),GJB3(538C>T),SLC26A4(IVS7-2A>G、2168A>G)和mtDNA 12SrRNA(A>G、1494C>T)。结果:在90例耳聋患者中,基因芯片方法共检出携带致聋基因突变64例(71.1%)。其中,GJB2基因突变40例(44.4%),包括235delC纯合突变20例(22.2%),235delC单杂合突变4例(4.4%),235delC和299delAT复合杂合突变2例(2.2%);299de-lAT单杂合突变2例(2.2%),299delAT纯合突变2例(2.2%);176del16单杂合突变2例(2.2%),176del16纯合突变2例(2.2%),176del16和235delC复合杂合突变6例(6.7%)。SLC26A4基因突变22例(24.4%),包括IVS7-2A>G纯合突变2例,IVS7-2A>G和2168A>G复合杂合突变2例(2.2%),IVS7-2A>G单杂合突变18例(20.0%);mtDNA 12SrRNA A>G纯合突变2例(2.2%);未检出GJB3基因突变。结论:应用基因诊断技术可以在耳聋患者病因调查中进行快速筛查诊断,值得推广应用。     

鼓室内注射地塞米松治疗极重度以上突发性聋的临床研究

目的 评估鼓室内注射地塞米松在极重度以上突发性聋仞始治疗中的作用.方法 以发病2周内,未接受任何治疗,初始听力损失(250～4000 Hz均值)>90 dB的突发性聋患者78例作为研究对象,根据患者意愿分为全身激素+局部激素组(22例)、全身激素组(44例)和局部激素组(12例),各组均同时给予扩血管、营养神经的药物治疗和高压氧治疗.全身激素治疗采用地塞米松15 mg/d连用3 d,随后10 mg/d用3 d,最后5 mg/d用3 d.局部激素治疗采用鼓窜内注射5 mg/ml地塞米松0.8 ml,隔日1次,共5次.获取治疗后第10、20、30天的纯音测听结果并进行疗效评估.结果 治疗前影响预后的因素三组间基本匹配.治疗后纯音听阈均值(pure tone average,PTA)改善≥30 dB者所占比例,全身+局部激素组81.82%、局部激素组83.33%、全身激素组88.64%,三组间差异无统计学意义(P=0.726);治疗30 d后PTA改善值分别为:全身+局部激素组41.36 dB、局部激素组43.08 dB、全身激素组51.70 dB,三组间差异无统计学意义(F=1.58,P=0.2133).各组患者治疗后10 d听力改善最为显著,20 d听力基本稳定.纯音测听各频率听阈的改善程度由低频向高频逐渐递减.结论 局部激素治疗作为极重度以上突发性聋的初始治疗与全身激素治疗相比并无优越性.     

多频稳态诱发电位(ASSR)的临床应用研究(36例极重度感音神经性耳聋幼儿及32例成人感音神经性耳聋患者ASSR测试结果分析)

目的旨在探讨ASSR与ABR在极重度感音神经性耳聋幼儿及成人感音神经性耳聋患者测试中的临床应用价值.方法对36例(72耳)小于3岁极重度感音神经性耳聋幼儿分别行ASSR和ABR测试;对32例(64耳)成人感音神经性耳聋患者分别行ASSR和电测听测试.结果①极重度感音神经性耳聋幼儿ABR均未引出V波,而ASSR在0.5 kHz、1 kHz、2 kHz、4 kHz的引出率分别为66.67%、86.11%、88.89%、94.44%,ASSR在0.5 kHz、1 kHz、2 kHz、4 kHz的阈值均数、标准差分别为82.56±9.26 dB HL、90.31±6.94 dB HL、88.12±7.93 dB HL、88.62±8.12 dB HL.②对成人感音神经性耳聋患者0.5 kHz、1 kHz、2 kHz、4 kHz ASSR测试阈值与电测听语频听阈(dB HL)进行配对两两比较的t检验,各组P值均大于0.05,无显著性差异.结论ASSR有助于极重度感音神经性耳聋幼儿残余听力的客观评估,尤以高频听阈为佳;ASSR与电测听在感音神经性耳聋诊断上有良好的一致性.     

Prevalence of auditory neuropathy (AN) among infants and young children with severe to profound hearing loss

Auditory neuropathy is a challenging disorder and needs special habilitative/rehabilitative approach. This study aimed to detect its prevalence among infants and young children with severe to profound hearing loss. 112 infants and young children with age ranged 6-32 months were examined and diagnosed as having severe to profound hearing loss and were referred for hearing aid fitting. Those infants were reassessed in our centers for detecting cases with auditory neuropathy. The study group was subjected to immittancemetry, behavioral observation audiometry, ABR and cochlear microphonics.

Results

15 patients were found to have auditory neuropathy according to our criteria for diagnosis.

Conclusions

The prevalence of AN in the study group was 13.4%. CM were recommended to be tested routinely during ABR assessment whenever abnormal results are obtained.     

耳后注射甲强龙作为补救方案在中高频听力下降型突发性聋治疗中的疗效及可行性分析

目的探析中高频听力下降型突发性聋采取耳后注射甲强龙治疗的临床效果及可行性。方法选取2019年5月~2020年7月本院耳鼻喉科就诊的中高频听力下降型突发性聋患者,共纳入病例54例,以随机法分组,即常规组(n=27)和观察组(n=27),常规组予常规治疗,观察组采取耳后注射甲强龙,比较两组效果。结果观察组有效率相比于常规组显著提高(P<0.05);治疗3周后,观察组2000~8000Hz频率听力提高值与常规组比(P<0.05)。结论中高频听力下降型突发性聋采取耳后注射甲强龙治疗效果理想,值得推广。