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.     

Delayed vertigo and profound sensorineural hearing loss

Episodic vertigo, similar to that of Meniere's disease, can occasionally develop years to decades after an ear has suffered a profound sensorineural hearing loss. Although the pathogenesis of this process is unknown, it is theorized that delayed endolymphatic hydrops develops. An inflammatory reaction could cause obstruction of the endolymphatic duct or interfere with venous drainage from the region of the endolymphatic sac, leading to hydrops. This condition is reviewed, and two adult patients with severe episodic vertigo and a unilateral profound sensorineural hearing loss which occurred in childhood are presented. Their evaluation and management are discussed.     

Cochlear implantation in autistic children with profound sensorineural hearing loss

Introduction

Cochlear implants have become the method of choice for the treatment of severe-to-profound hearing loss in both children and adults. Its benefits are well documented in the pediatric and adult population. Also deaf children with additional needs, including autism, have been covered by this treatment.

Preserved otoacoustic emission in a child with a profound unilateral sensorineural hearing loss

We report a case of profound unilateral sensorineural hearing loss with good response of otoacoustic emission. The patient was a 5-year-old boy, who was diagnosed to have unilateral hearing impairment on pure tone audiometry at the first visit. The affected ear showed the absence of auditory brainstem response; however, its transiently evoked otoacoutic emission and distortion product otoacoustic emission were considered to be normal. These findings indicated that the outer hair cell of cochlea was not impaired and that the impairment should be localized between inner hair cells, primary afferent fiber or its synapses, spiral ganglion of the cochlea and acoustic fiber, or at a combination of these areas. That is, evaluation of otoacoustic emission was useful in determining the region of impairment in sensorineural hearing loss. Further follow-up will be necessary to differentiate the present case from auditory neuropathy.     

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.     

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.     

突发性极重度聋与全聋的预后特点及差异

Objective To clarify the different prognostic characteristics between profound sudden sensorineural hearing loss (SSNHL) and total SSNHL. Methods The patients with SSNHL who visited Eye Ear Nose and Throat Hospital from June 2007 to September 2008 were reviewed retrospectively. All the 204 patients, with pure tone average (PTA) threshold more than 90 dB, were enrolled and divided into two groups, including total SSNHL and profound SSNHL groups. The relationship between recovery rate and prognostic factors including the age, complications, time period between onset and therapy was analyzed.Results There were 57 cases of total SSNHL and 147 cases of profound SSNHL in this series. Tinnitus was complained in more than 90% of the patients in both groups, which was higher than that of dizziness and ear fullness. Dizziness was present in 64. 9% (37/57) patient with total SSNHL group and 45.6% (67/147)patients with profound SSNHL, which had significant difference between the two groups (x2 = 5.72,P =0. 017). The PTA threshold improvement in total SSNHL group and profound SSNHL group was (36. 4 ±19.3) dB and (40. 2 ±21.3) dB respectively, which was no significant difference between the two groups (t = 1. 165 ,P =0. 245). The cured patients were all those received therapy within 1 week following the onset of SSNHL, which was of 2. 6% (1/38) patients in the total SSNHL group and 14. 3% (14/98) patients in the profound SSNHL group(P =0. 045). Furthermore, 3.5% (2/57) patients in total SSNHL group as well as 29. 9% (44/147) patients in profound SSNHL group obtained a good result with PTA threshold ≤50 dB after therapy(x2 = 15.92,P = 0. 001 ). In addition, the favorable prognosis was related with the onsettherapy time point( P = 0. 001 ), but not related to the patients' age. Conclusion Profound SSNHL and total SSNHL though both with PTA threshold > 90 dB had significant differences recovery rate and need to be studied separately.     

突发性极重度聋与全聋的预后特点及差异

目的 研究突发性极重度聋和全聋的预后特点及差异.方法 回顾性研究复旦大学附属眼耳鼻喉科医院2007年6月至2008年9月收治的初始平均纯音听阈(pure tone average,PTA)>90 dB、随访完整的204例患者,分为全聋和极重度聋两组,对两组的预后进行比较,并对预后与年龄、并发症、发病-就诊时间进行相关性分析.结果 全聋组57例,极重度聋组147例,耳鸣、眩晕、耳闷三大伴随症状中,耳鸣的伴随率最高,两组均达90%以上,眩晕的伴随率全聋组为64.9%(37/57),极重度聋组为45.6%(67/147),两组间差异有统计学意义(x2=5.72,P=0.017).治疗后全聋组PTA下降(36.4±19.3)dB,极重度聋组下降(40.2±21. 3)dB,差异无统计学意义(t=1.165,P=0.245).两组痊愈者均为1周内接受治疗者,全聋组痊愈率为2.6%(1/38),极重度聋组为14.3%(14/98),两组间差异有统计学意义(Fisher确切概率法,P=0.045).全聋组治疗后PTA≤50 dB的患者占3.5%(2/57),极重度聋组达29.9%(44/147),两组间差异有统计学意义(x2=15.92,P=0.001).两组的预后与发病-就诊的时间有关(P值均为0.01),与年龄无关.结论 初始PTA>90 dB的突发性聋可分为极重度聋与全聋,两者预后有差异,需分开进行研究.     

突发性极重度聋与全聋的预后特点及差异

Objective To clarify the different prognostic characteristics between profound sudden sensorineural hearing loss (SSNHL) and total SSNHL. Methods The patients with SSNHL who visited Eye Ear Nose and Throat Hospital from June 2007 to September 2008 were reviewed retrospectively. All the 204 patients, with pure tone average (PTA) threshold more than 90 dB, were enrolled and divided into two groups, including total SSNHL and profound SSNHL groups. The relationship between recovery rate and prognostic factors including the age, complications, time period between onset and therapy was analyzed.Results There were 57 cases of total SSNHL and 147 cases of profound SSNHL in this series. Tinnitus was complained in more than 90% of the patients in both groups, which was higher than that of dizziness and ear fullness. Dizziness was present in 64. 9% (37/57) patient with total SSNHL group and 45.6% (67/147)patients with profound SSNHL, which had significant difference between the two groups (x2 = 5.72,P =0. 017). The PTA threshold improvement in total SSNHL group and profound SSNHL group was (36. 4 ±19.3) dB and (40. 2 ±21.3) dB respectively, which was no significant difference between the two groups (t = 1. 165 ,P =0. 245). The cured patients were all those received therapy within 1 week following the onset of SSNHL, which was of 2. 6% (1/38) patients in the total SSNHL group and 14. 3% (14/98) patients in the profound SSNHL group(P =0. 045). Furthermore, 3.5% (2/57) patients in total SSNHL group as well as 29. 9% (44/147) patients in profound SSNHL group obtained a good result with PTA threshold ≤50 dB after therapy(x2 = 15.92,P = 0. 001 ). In addition, the favorable prognosis was related with the onsettherapy time point( P = 0. 001 ), but not related to the patients' age. Conclusion Profound SSNHL and total SSNHL though both with PTA threshold > 90 dB had significant differences recovery rate and need to be studied separately.     

Analysis of p1 latency in normal hearing and profound sensorineural hearing loss

Objectives

P1 is a robust positivity at a latency of 50-150 msec in the auditory evoked potential of young children. It has been reported that over the first 2-3 years of life, there is a rapid decrease of the latency and the mean P1 latency in adults with normal hearing is approximately 60 msec. This study was designed to evaluate the change of the P1 latency in Koreans with normal hearing according to age and to compare this with the P1 latency of young patients with profound sensorineural hearing loss before and/or after cochlear implantation.

Methods

Among the patients who visited the Department of Otorhinolaryngology at Seoul National University Hospital from June 2007 to September 2009, the P1 response was recorded in 53 patients in the normal hearing group, in 13 patients in the pre-cochlear implantation (CI) group and in 10 patients in the post-CI group. A synthesized consonant-vowel syllable /ba/ was used to elicit the evoked responses. The evoked responses were collected using the center of the frontal head. For each subject, an individual grand average waveform was computed by averaging the ten recordings. The P1 latency was visually identified as a robust positivity in the waveform.

Results

For the normal hearing group, the P1 latency showed the pattern of shortening as the age increased (coefficient, -0.758; P<0.001). For the pre-CI group, 10 cases showed delayed latencies and 3 cases did not show the P1 wave. For the post-CI group, the P1 latencies showed a less delayed tendency than those of the pre-CI group, but this was not statistically different.

Conclusion

This report provides the standard value of the P1 latency at each age in Koreans for the first time and the findings support that the maturation of the central auditory pathways could be measured objectively using the P1 latency.     

Edaravone,a free radical scavenger,in the treatment of idiopathic sudden sensorineural hearing loss with profound hearing loss

Objective

Edaravone, a free radical scavenger, is a clinical drug that is widely used to reduce neuronal damage after acute cerebral infarction in Japan since 2001. The aim of this study was to investigate whether edaravone could improve treatment result in idiopathic sudden sensorineural hearing loss (ISSHL) patients with severe hearing loss.

Methods

Between 2004 and 2006, 14 patients of ISSHL with the mean hearing levels equal or over 90 dB at the initial visit were treated with edaravone. 14 counterpart control patients were selected from 45 patients who had similar prognostic factors and were treated with hyperbaric oxygenation therapy (HBO) in the past decade.

Results

There were no significant differences between edaravone group and the control group in hearing recovery.

Conclusion

We considered that edaravone was not able to bring remarkable effect compared with conventional treatment regimen for ISSHL.     

感音神经性听力损失的MRI特征

感音神经性听力损失(SNHL)是耳科常见疾病,主要表现为听力损失伴或不伴耳鸣、眩晕、耳闷等症状,SNHL病因复杂多样,目前尚未有一个确切的病因。近年来磁共振影像技术(MRI)得到了较好的发展和运用,内听道MRI在显示软组织方面更具优势,可用来研究SNHL患者的内耳疾病,如研究小脑前下动脉来进一步阐述内耳微循环,轧造影显示梅尼埃病患者膜迷路积水,以及功能MRI对脑区活动的研究等等。本文主要以内听道MRI影像学特征来探讨SNHL的潜在病因,为耳科医生诊疗SNHL提供新思路。     

极重度感音神经性聋患者耳蜗微音电位测试研究

目的:报告极重度感音神经性聋患者人工耳蜗植入术中圆窗耳蜗微音电位(CM)测试结果.方法:40例患者全身麻醉下人工耳蜗植入手术过程中,行术中圆窗CM测试,测出CM与术前畸变耳声发射(DPOAE)结果进行比较.结果:40例患者术中圆窗CM 0.5、1.0、2.0、4.0 kHz引出率分别为90.0%、97.5%、100.0%、92.5%;术前DPOAE 0.5、1.0、2.0、4.0 kHz引出率分别为2.5%、2.5%、17.5%、2.5%.结论:同一极重度感音神经性聋患者CM引出率明显高于DPOAE引出率,术中圆窗CM测试可以较准确客观地评估极重度感音神经性聋患者的部分耳蜗功能.     

Antiphospholipid antibodies in patients with sensorineural hearing loss

Sensorineural hearing loss can be associated with autoimmune diseases and the presence of antiphospholipid antibodies. Sixty patients (mean age 47 years, range 18–76 years) with sudden sensorineural hearing loss were studied with audiograms, stapedial thresholds, otoacoustic emissions, positional and caloric testing. The serologic testing included antibodies against phosphatidylserine and ₂-glycoprotein. Additionally, a group of 34 patients (mean age 65 years, range 31–81 years) with normal tension glaucoma was examined because in a previous study these patients were reported to have elevated concentrations of antiphospholipid antibodies with a coincidence of progressive sensorineural hearing loss. The baseline for antiphospholipid antibody levels was established in a control group of 40 healthy blood donors. In 12 of the 60 patients with sudden sensorineural hearing loss, levels of antiphospholipid antibodies were elevated. Antiphosphatidylserine IgM antibodies were significantly lower compared to controls and patients with the combination of hearing loss and normal tension glaucoma (Fishers exact two-sided test, P<0.01). Our data suggest that antibodies against ₂-glycoprotein seem to coincidence with an acute event, such as sudden sensorineural hearing loss, whereas antibodies against phosphatidylserine IgG are detectable in the prolonged sequel, such as in patients with progressive sensorineural hearing loss and normal tension glaucoma.     

Vestibular system in patients with sensorineural hearing loss

A near localization of anatomical structures of an inner ear and possibility of damage caused by the same etiopathogenic factor implies that sensorineural hearing loss may be accompanied by vertigo and dizziness. The aim of a study was to evaluate a frequency of vestibular system dysfunction in patients with sensorineural hearing loss and to analyse it according to severity and type of the hearing loss. We evaluated a group of 126 persons which were treated in ENT Department during the last year from sensorineural hearing loss. In all subjects' audiological and ENG examinations were performed. A 50% of patients complained of vertigo and 30% of them--of dizziness. Abnormal ENG recordings were registered in 72% of persons especially in positional and visual ocular-motor tests. In the group without vertigo incorrect ENG tests were often recorded. In the group with vertigo the peripheral and mixed lesions in ENG examination were noted statistically more frequent. We found lack of correlation between magnitude and type of hearing loss and vestibular impairment. Only in the group-residual hearing/deafness were observed significantly more frequent peripheral vestibular dysfunction.     

Asymmetric hearing loss in a random population of patients with mild to moderate sensorineural hearing loss

OBJECTIVES: We evaluated the correlation of asymmetric hearing loss, in a random population of patients with mild to moderate sensorineural hearing loss, to several clinical factors such as age, sex, handedness, and noise exposure. METHODS: We randomly selected, from 8 hearing institutes in Israel, 429 patients with sensorineural hearing loss of at least 30 dB at one frequency and a speech reception threshold not exceeding 30 dB. Patients with middle ear disease or retrocochlear disorders were excluded. The results of audiometric examinations were compared binaurally and in relation to the selected factors. RESULTS: The left ear's hearing threshold level was significantly higher than that of the right ear at all frequencies except 1.0 kHz (p < .05). One hundred fifty patients (35%) had asymmetric hearing loss (more than 10 dB difference between ears). In most of the patients (85%) the binaural difference in hearing threshold level, at any frequency, was less than 20 dB. CONCLUSIONS: Age, handedness, and sex were not found to be correlated to asymmetric hearing loss. Noise exposure was found to be correlated to asymmetric hearing loss.