Comparison of hearing recovery criteria in sudden sensorineural hearing loss

The countless methods available to analyze hearing recovery in idiopathic sudden sensorineural hearing loss (ISSHL) cases hinder the comparison of the various treatments found in the literature.ObjectiveThis paper aims to compare the different criteria for hearing recovery in ISSHL found in the literature.Materials and MethodsThis is an observational clinical cohort study from a prospective protocol in patients with ISSHL, treated between 2000 and 2010. Five criteria were considered for significant hearing recovery and four for complete recovery by pure tone audiometry, using non-parametric tests and multiple comparisons at a significance level of 5%. After determining the stricter criteria for hearing recovery, vocal audiometry parameters were added.ResultsThere was a significant difference between the criteria (p < 0.001) as they were analyzed together. Mild auditory recovery occurred in only 35 (27.6%) patients. When speech audiometry was added, only 34 patients (26.8%) showed significant improvement.ConclusionsThere is a lack of consistency among the criteria used for hearing recovery. The criterion of change of functional category by one degree into at least mild hearing recovery was the stricter. Speech audiometry did not prove essential to define significant hearing recovery.     

双侧与单侧突发性聋的临床特点及疗效分析

目的：分析与比较双侧与单侧突发性聋的临床特点与疗效。方法：对333例突发性聋患者（单侧315例，双侧18例）资料进行回顾性研究。结果：双侧突发性聋占突发性聋患者的5．4％，单侧和双侧突发性聋的发病年龄、性别比例、就诊时间、伴随症状差异无统计学意义。5例（27．8％）双侧突发性聋患者并发有糖尿病，概率高于单侧的9．8％（P〈0．05）。单侧者突发性聋的听力损失程度比双侧者严重，但它的疗效较好（二者总有效率分别为58．4％和13．9％）。就诊越早，越早使用激素，突发性聋的疗效越好。结论：单侧突发性聋更常见，双侧突发性聋并发糖尿病者更多。双侧突发性聋预后比单侧差。越早激素治疗疗效越好。     

语后聋患者人工耳蜗植入后听觉行为及言语可懂度分级的相关因素分析

目的：应用听觉行为分级（CAP）和言语可懂度分级（SIR）评估语后聋患者人工耳蜗植入后言语交流状况,探讨其得分与听力下降时间、植入时间、术前助听器使用情况的关系。方法：回顾分析23例语后聋人工耳蜗植入者的临床资料,收集其听力下降时间、植入时间、术前助听器使用情况等相关资料,应用CAP、SIR分级评估其言语交流状况。运用IBM SPSS Statistics19软件进行统计描述及结果分析。结果：CAP分级平均得分为6．13分,统计分析示CAP分级与听力下降时间、植入时间、术前助听器使用时间无显著相关关系（均P〉0．05）。患者均为语后聋,其SIR分级的平均得分为4．91分。结论：语后聋患者人工耳蜗植入后CAP分级与听力下降时间、植入时间、术前助听器使用情况无相关性。这可能是由于CAP分级的精确性和细节上的欠缺或该研究样本量限制,未显示出其与3个变量之间的关系。SIR不适用于评估语后聋患者人工耳蜗植人后的言语状况。     

突发性聋伴耳鸣患者耳鸣特征

目的 分析研究突发性聋伴耳鸣患者的耳鸣特点、耳鸣与听力损失的关系、耳鸣对患者的影响程度, 探讨突聋患者耳鸣的产生机制。 方法 突发性聋伴有耳鸣患者231例常规采集病史, 填写耳鸣致残量表(THI)及视觉模拟得分表(VAS), 同时进行纯音听阈测试、耳声发射、耳鸣检测及掩蔽试验等检查。分析患者的耳鸣特点、耳鸣与听力的关系、耳鸣的THI及VAS得分以及它们特点。 结果 ①听力损失特点：低中频下降型20例(占8.7%), 中高频下降型60例(占26.0%), 平坦型44例(占19.0%), 全聋型79例(占34.2%), 不规则型28例(占12.1%);②耳鸣频率:低频耳鸣(≤500 Hz)49例(占21.2%), 中频耳鸣(51～2 000 Hz)54例(23.4%), 高频耳鸣(>2 000 Hz)122例(52.8%), 未匹配6例(占2.6%)。其中听力损失类型为低中频下降型的患者中, 低频、中频、高频耳鸣的比例分别为75%、15%、10%;中高频下降型患者中, 低频、中频、高频耳鸣的比例分别为13.3%、26.7%、60.0%;全聋型患者中, 低频、中频、高频耳鸣的比例分别为17.1%、19.0%、63.3%。听力下降最明显的频率对数与耳鸣频率对数呈线性关系, r=0.592, P<0.01;③耳鸣响度:0～30 dB HL 45例(占19.5%), 31～60 dB HL 60例(占26.0%), 60～90 dB HL 102例(占44.2%), 大于90 dB HL 18例(占27.8%), 未匹配6例(占2.6%)。耳鸣响度与听力损失程度(250～4 000 Hz平均听阈)有相关性, 相关系数r=0.216, P=0.001<0.05。④耳鸣致残级别:按照Newman等依据THI得分将耳鸣残疾分级, 其中1级27例(占11.7%), 2级耳鸣44例(占19.0%), 3级66例(占28.6%), 4级94例(占40.7%)。⑤THI及VAS得分特点:THI得分与听力损失程度无相关性r=0.087, P=0.287>0.05。VAS得分与听力损失程度无相关性r=0.002, P=0.982>0.05。THI得分与耳鸣频率对数无相关性, 相关系数r=-0.056, P=0.402>0.05。VAS得分与耳鸣频率对数无相关性, 相关系数r=-0.003, P=0.970>0.05。THI得分与耳鸣响度无相关性, 相关系数r=0.039, P=0.563>0.05。VAS得分与耳鸣响度无相关性, 相关系数r=0.136, P=0.110>0.05。结论 ①突发性聋伴耳鸣患者中高频耳鸣最常见;②耳鸣频率与听力损失类型显著相关, 低中频下降型以低频耳鸣多见, 而中高频下降型及全聋型以高频耳鸣多见;③听力损失最大的频率与耳鸣的频率有较高的一致性;④突发性聋伴耳鸣患者急性期的耳鸣致残程度以3～4级为多;⑤THI及VAS得分与听力损失的程度、耳鸣的频率、耳鸣的响度均无相关性。     

The effect of side of implantation on unilateral cochlear implant performance in patients with prelingual and postlingual sensorineural hearing loss: A systematic review

Objective

Cerebral lateralisation of language processing leads to a right ear advantage in normal hearing subjects. The aim of this study was to present a systematic overview of the effect of implantation side on postoperative cochlear implant performance in patients with symmetrical severe to profound sensorineural hearing loss.

Data sources

PubMed, Embase and The Cochrane Library databases.

Research methods

Databases were searched from database inception up to 9 January 2017 for cochlear implant and side and all synonyms. Title, abstract and full‐text of retrieved articles were screened for eligibility. Then, directness of evidence and risk of bias were assessed. For the included articles, study characteristics and outcome data (hearing and language development) were extracted.

Results

2541 unique articles were screened, of which twenty were eligible for critical appraisal. No randomised controlled trials were identified. Twelve studies with a high directness of evidence remained for data extraction. Four of six studies including children with pre‐lingual sensorineural hearing loss and four of seven studies investigating adults with postlingual sensorineural hearing loss found a right ear advantage in at least one outcome measurement related to cochlear implant performance.

Conclusion

The available evidence on the effect of side of implantation is of low quality, as study populations and outcome measures are heterogeneous. The majority of studies reveals evidence for a right ear advantage in prelingually deafened children as well as postlingually deafened adults. In view of the present evidence and as no left ear advantage was identified, we cautiously advise implanting the cochlear implant in the right ear when other prognostic factors do not favour the left ear and sensorineural hearing loss is symmetrical.     

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.     

中度和重度聋儿助听后早期听觉能力及言语可懂度发育

目的：评价耳聋儿童助听后1年内听觉及言语能力发展变化趋势,探讨耳聋程度对聋儿的听觉及言语发育水平的影响,为聋儿的有效康复提供临床参考资料。方法：患儿29例,男19例,女10例。助听器选配年龄3～8岁,平均5．6岁。根据听力损失程度将患儿分为中度听力损失组（14例）和重度听力损失组（15例）。分别使用听觉行为分级（CAP）和言语可懂度分级（SIR）问卷在助听前及助听后1、3、6、9、12个月时对患儿的听觉能力及言语可懂度发育情况进行评估。结果：经单因素方差分析,中度和重度聋患儿CAP平均得分在助听前差异有统计学意义（P〉0．05）。在助听后1、3、6、9、12个月均差异无统计学意义（P〉0．05）。同样中度和重度聋患儿SIR平均得分在助听前差异有统计学意义（P〈0．05）。在助听后1、3、6、9、12个月均差异无统计学意义（P〉0．05）。中度聋患儿cAP平均得分助听前与助听后6、9、12个月差异有统计学意义（P〈0．05）。中度聋患儿SIR平均得分助听前与助听后6、9、12个月差异有统计学意义（P〈0．05）;助听后1个月与12个月差异有统计学意义（P〈0．05）。重度聋患儿CAP平均得分助听前与助听后3、6、9、12个月差异有统计学意义（P〈0．05）,助听后1个月与9、12个月差异有统计学意义（P〈0．05）。重度聋患儿SIR平均得分助听前与助听后3、6、9、12个月差异有统计学意义（P〈0．05）,助听后1个月与6、9、12个月差异有统计学意义（P〈0．05）。结论：中度和重度聋组患儿助听后1年内早期听觉及言语能力有显著提高,但各自有不同的发育特点和规律。     

Magnetic resonance imaging of endolymphatic space in patients with sensorineural hearing loss: comparison between fluctuating and idiopathic sudden sensorineural hearing loss

Abstract

Background: Recently, 3-Tesla magnetic resonance imaging (MRI) after an intravenous gadolinium injection has been used to describe the endolymphatic space (ELS).

Objectives: This study described the histopathological differences between idiopathic sudden sensorineural hearing loss (ISSNHL) and fluctuating sensorineural hearing loss (FSNHL) by examining the ELS. Additionally, the relationship between the affected cochlear and vestibular ELS/total fluid space (TFS) volume ratio and the duration from the onset to MRI in patients with FSNHL were evaluated.

Material and methods: This study included 205 individuals without vertigo: 47 controls, 94 with ISSNHL, and 64 with FSNHL. The TFS and ELS volumes were measured and the ELS/TFS volume ratios (%) were evaluated.

Results: The cochlear and vestibular ELS/TFS volume ratios of the affected ear in patients with FSNHL were significantly higher than that in those with ISSNHL. There was no correlation between the duration from FSNHL onset to the MRI scan in the affected cochlea and vestibule.

Conclusion and significance: There were differences in the form of hearing fluctuation and the extended ELS volume between ISSNHL and FSNHL. ISSNHL cases with severe ELS extension were likely to change to FSNHL.     

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.     

Intratympanic dexamethasone with hyaluronic acid in the treatment of idiopathic sudden sensorineural hearing loss after failure of intravenous steroid and vasoactive therapy

The purpose of this prospective study was to test whether intratympanic application of dexamethasone/hyaluronic acid improves hearing outcome in patients with pantonal idiopathic sudden sensorineural hearing loss (ISSHL), in patients with sudden deafness or sudden profound SHL and in patients with predominant high-frequency ISSHL who are refractory to intravenous steroid and vasoactive therapy. The study took place in an academic tertiary referral hospital involving 21 patients with pantonal ISSHL, 10 patients with sudden deafness or sudden profound SHL and 9 patients with a high-frequency ISSHL. Intratympanic dexamethasone/hyaluronic acid was administered in the affected ear. Hearing was evaluated by means of standard pure-tone audiometry. The differences between pure-tone hearing thresholds by air conduction before intravenous therapy and before the beginning of the intratympanic therapy, as well as before and after intratympanic therapy, were calculated. Statistical analysis was performed by means of the Wilcoxons test for paired samples. Intratympanic injection of dexamethasone/hyaluronic acid results in a significant global (pantonal) improvement in hearing in patients with pantonal ISSHL. It also effects improvement in hearing at selected frequencies (namely at 1.5 and 3 kHz) in patients with a predominant high-frequency ISSHL and at selected frequencies (namely at 0.5, 0.75 and 1 kHz) in patients with sudden deafness or sudden profound SHL. Neither systemic nor local side effects were observed. Intratympanic administration of dexamethasone/hyaluronic acid provides a safe and efficacious therapeutic option for the treatment of patients with pantonal and high-frequency ISSHL who dont respond to intravenous steroid and vasoactive therapy.Abbreviations dB HL decibel hearing level - (IS)SHL (idiopathic sudden) sensorineural hearing loss - kHz kiloHertz     

Effect of treatment with Ginkgo biloba extract EGb 761 (oral) on unilateral idiopathic sudden hearing loss in a prospective randomized double-blind study of 106 outpatients

Objective: Test of dose–response relationship for Ginkgo biloba extract EGb 761 (oral) in outpatients with acute idiopathic sudden sensorineural hearing loss (ISSHL) of at least 15 dB at one frequency within the speech range occurring less than 10 days before study inclusion. Design: Multicentre, randomized, double-blind phase III study comparing dosages of 120 mg twice daily and 12 mg twice daily over 8 weeks. Main endpoint: Recovery (in dB) of the auditory threshold from the initial measurement to the value on the last day of treatment, averaged over those frequencies from 0.25, 0.5, 1, 2, and 3 kHz for which the initial hearing loss amounted to 15 dB or more compared to the level on the opposite side. Patients: 106 patients with an average age of 44 ± 16 years and with hearing loss at affected frequencies 26 dB ± 9 dB included between December 1995 and July 1997. Results: Large majorities of both treatment groups recovered completely. In exploratory analyses of the 96 patients included according to the protocol, patients given the higher dose had less risk of not recovering well (≤10 dB residual hearing loss) (one-sided Fisher test: P = 0.0061), especially if they had no tinnitus (n = 44, P = 0.00702). Conclusion: A higher dosage of EGb 761 (oral) appears to speed up and secure the recovery of ISSHL patients, with a good chance that they will recover completely, even with little treatment. This was already observed after one week of treatment. We find it justified to treat patients who have unilateral ISSHL of less than 75 dB and neither tinnitus nor vertigo with 120 mg oral EGb 761 twice daily. Received: 20 November 2000 / Accepted: 10 April 2001     

Screening for delayed-onset hearing loss in preschool children who previously passed the newborn hearing screening

Objective

The goal of this study was to investigate the prevalence of delayed-onset hearing loss in preschool children who previously passed newborn hearing screening in Shanghai, China.

Methods

Between October 2009 and September 2010, 21,427 preschool children were enrolled from five areas of Shanghai, who had passed newborn hearing screening. Children were screened for delayed-onset hearing loss with pediatric audiometers. Children with positive results in initial and re-screening tests were assessed audiologically and for risk indicators according to the Year 2007 Statement of the American Joint Committee on Infant Hearing (JCIH).

Results

During the study period, 445 children (2.08%) were referred for audiologic assessment; 16 (0.75/1000, 95% CI 0.38-1.12) had permanent delayed-onset hearing loss. Of these, five (0.23/1000) had bilateral moderate hearing loss; seven (0.33/1000) had mild bilateral hearing loss; and four (0.19/1000) had unilateral moderate or mild hearing loss. JCIH risk indicators were established for six children (6/16, 37.5%), including parental concern regarding speech and language developmental delay, neonatal intensive care unit with assisted ventilation or hyperbilirubinemia, recurrent otitis media with effusion, craniofacial malformation, and family history (n = 1 for each). The remaining ten (62.5%) had no related risk factors.

Conclusions

A significant proportion of preschool children have undiagnosed delayed-onset hearing loss. Hearing screening in preschool is recommended for an early detection.     

R75Q de novo dominant mutation of GJB2 in a Chinese family with hearing loss and palmoplantar keratoderma

Objectives

Mutations in the GJB2 gene encoding connexin 26 (Cx26) are major causes of hereditary deafness. This study aimed to characterize the mutation profiles of the GJB2 gene in a Chinese family with sensorineural hearing loss.

Methods

A Chinese family that included three individuals with sensorineural hearing loss and palmoplantar keratoderma underwent complete physical examinations, audiological examinations including pure tone audiometry and auditory brainstem response, skin pathological examination, and temporal CT scans. The entire coding region of GJB2, GJB3, GJB6, and the coding exons (exon7 + 8 and 19) of SLC26A4, mitochondrial 12SrRNA, and tRNA Ser (UCN) were sequenced. Structural analysis was performed to detect the effects of mutation on the tertiary structure of Cx26.

Results

A dominant GJB2 mutation, c.224G>A (p.Arg75Gln, p.R75Q), was detected in the family. No other mutation was identified in GJB2, GJB3, GJB6, or the coding exons (exon7 + 8 and 19) of SLC26A4, mitochondrial 12SrRNA, and tRNA Ser (UCN). Structural analysis revealed that the p.R75Q mutation likely affects the structural stability and permeation properties of the Cx26 gap junction channel.

Conclusion

Our findings provide further evidence of a correlation between the p.R75Q mutation in Cx26 and a syndromic hearing impairment with palmoplantar keratoderma.