Hearing-related,health-related quality of life in patients who have undergone otosclerosis surgery: A long-term follow-up study

Objectives: The aims of the study were to assess health-related quality of life and hearing-related disability in subjects with otosclerosis 30 years after surgery. Design: An observational study was performed. Medical records were reviewed, a clinical examination as well as audiometric assessments were performed. Generic health-related quality of life was assessed by the SF-36v2 and hearing disability by a shortened version of SSQ (speech spatial and qualities of hearing scale). Study sample: Sixty-five individuals, who had undergone stapedectomy in 1977-79 at a tertiary referral center. Results: Generic health-related quality of life according to SF-36 subscale scores was comparable to that of an age- and sex-matched reference population. The SF-36 mental component summary score (MCS) was, however, significantly better than that of the reference population. The mental and physical summary component scores correlated significantly to hearing disability measured by the SSQ but not to hearing impairment. Hearing disability was displayed in all SSQ sub-scores, especially in more complex listening situations and in the localization of sounds. Conclusions: This study shows that individuals with otosclerosis, 30 years after surgery, have a good generic health-related quality of life, despite moderate to severe hearing loss and significant hearing disabilities.     

糖尿病早期内耳损伤听力学改变的实验研究

目的研究糖尿病内耳损伤早期的听力学改变及其机制。方法建立糖尿病动物模型,每日检测动物血糖,持续2周。实验前后检测ABR及DPOAE,2周后检测血脂、超氧化物岐化酶（SOD）、谷胱甘肽过氧化物酶（GSHPx）、血清丙二醛（MDA）,并与对照组比较。结果模型组大鼠的血糖、血脂增高,GSH-Px的活性下降,ABR阈值无显著变化,DPOAE的反应幅值下降,与正常组比较差异有统计学意义（P〈0．05）。结论糖尿病造成大鼠内耳损害的机制与机体代谢紊乱引起内耳毛细胞的氧化损伤有关;这种损伤所致的耳声发射的异常改变早于听性脑干反应异常的出现。     

甘肃省375例非综合征型聋患者聋病易感基因突变检测分析

目的 通过对甘肃省部分非综合征型聋患者进行聋病易感基因筛查,从分子水平了解其遗传病因及特点.方法 采集甘肃省375例非综合征型聋患者的外周静脉血5~10 ml,提取基因组DNA,运用SNPscan技术检测GJB2基因2个外显子36个突变位点、SLC26A4基因21个外显子77个突变位点和mtDNA12SrRNA A1555G及C1494T突变.结果 375例非综合征型聋患者中,23例携带mtDNA12SrRNA A1555G均质性突变(6.13%, 23/375),2例携带mtDNA12SrRNA C1494T均质性突变(0.53%, 2/375);检出GJB2基因突变致聋者42例(11.20%, 42/375),其中纯合突变31例(8.27%, 31/375)、复合杂合突变11例(2.93%, 11/375),GJB2基因单杂合突变携带者25例(6.67%,25/375),c.235delC为最常见的突变类型,等位基因频率为8.80%(66/750);检出SLC26A4基因突变致聋者29例(7.73%, 29/375),其中纯合突变17例(4.53%, 17/375)、复合杂合突变12例(3.20%, 12/375),SLC26A4基因单杂合突变携带者14例(3.73%,14/375),c.919-2A>G和c.2168A>G为其最主要的突变类型,等位基因频率分别为5.20%(39/750)和2.0%(15/750).结论 甘肃省部分非综合征型聋患者mt DNA12SrRNA A1555G突变检出率明显高于全国水平(2.83%,57/2016),而GJB2、SLC26A4基因突变检出率与全国水平相近;三个常见聋病易感基因筛查可为25.60%的本组耳聋患者提供明确的分子病因学诊断.     

听觉障碍青少年群体认同特点研究

目的 探讨听觉障碍青少年的群体认同特点.方法 对79名听觉障碍青少年和76名健听青少年实施<群体认同问卷>调查.采用验证性因素分析考察问卷的信度和效度指标;采用相关分析考察群体认同不同成分之间的关系,采用方差分析比较听觉障碍青少年和健听青少年在群体认同上的差异.结果 除了群体评价部分,<群体认同问卷>应用于听觉障碍青少年具有较好的信度和效度;性别、年级对听觉障碍青少年群体认同没有显著性影响;听觉障碍青少年的群体认同成分之间存在着极其显著的相关;在群体认知、情感投入和群体评价上,听觉障碍青少年显著低于健听青少年,在体验承诺上两者不存在显著性差异.结论 需要通过心理健康教育等措施提高听觉障碍青少年的群体认同水平.     

Frequency-specific hearing results after stapes surgery for Chinese population otosclerosis with different degrees of hearing loss

Abstract

Background: Frequency-specific hearing studies are important for predicting hearing results and the prognosis after stapes surgery, to prepare for the rehabilitation of frequency-specific hearing in patients with otosclerosis.

Objectives: To evaluate outcomes of stapes surgery of Chinese otosclerosis patients with different degrees of hearing loss.

Methods: We conducted a retrospective analysis of 213 otosclerosis patients who underwent stapes surgery in our hospital. Pre- and post-operative audiometric evaluation using conventional audiometry.

Results: The post-op ABG was less than 20?dB in 94.52% of all cases after surgery. There was no statistical significance of the post-op ABG among different groups at all frequencies except extremely severe group (p?<?.05). There was a significant improvement of both AC and BC thresholds in post-op period and the most significant improvement was found in severe group. There were no serious post-op complications and no re-operations during the follow-up period.

Conclusion: Stapes surgery is a suitable treatment option for otosclerosis with mild to extremely severe HL. The mild to moderate hearing loss groups had the most significant improvement of AC thresholds in the low frequency region, and the severe to extremely severe groups appeared in the high frequency region.     

Peripheral and central hearing impairment and their relationship with cognition: a review

Objective: To consider the relationships between both peripheral and central hearing impairment and cognition.

Design: Narrative review.

Study sample: Numerous studies exploring the relationship between hearing impairment and cognitive function, particularly in an older population.

Results: In addition to the well-documented relationship between peripheral hearing loss and cognition highlighted in previous comprehensive reviews, there is also some evidence to suggest that there is a relationship between central hearing impairment and cognition. Further research is required to better understand this relationship and its effects on hearing aid benefit in people with both peripheral hearing loss and central hearing impairment.

Conclusions: To fully understand the relationship between hearing impairment and cognitive impairment, not only peripheral but central hearing needs to be considered. Such knowledge could be of benefit in the clinical management of people with both peripheral hearing loss and central hearing impairment.     

PCR-GeneScan法检测遗传性聋相关基因GJB2 235 delC和mtDNA A1555G突变

目的:检测GJB2 235delC杂合突变和mtDNA A1555G突变。方法:对120例样本进行诊断试验,其中测序GJB2 235delC杂合突变样本16例,mtDNA A1555G突变17例。用PCR方法对目标片段进行扩增,PCR产物在3100DNA sequencer(ABI)上聚丙烯酰胺胶毛细管电泳,GeneScan、GeneMarker软件数据分析。结果:120例样本均得到检测结果,检出GJB2 235delC杂合突变样本17例,mtDNA A1555G突变17例,1例正常样本误诊为235delC杂合突变而出现假阳性。结论:PCR-GeneScan技术可以同时检测2种不同基因的突变,单管多重PCR和GeneScan荧光标记法结合是同时检测多种突变一种新的思路,而且可能是一种有效的方法。     

听障儿童声母最小音位对比式识别能力的个案研究

目的通过对一名听障儿童声母最小音位对比式识别能力进行评估与干预训练,验证分层训练方法对提高其声母最小音位对比式识别能力的有效性。方法以一名3岁、双耳配戴助听器的听障儿童为被试,采用分层训练的方法进行为期6周的干预训练。训练前、后分别使用声母最小音位对比式识别能力评估词表进行评估,训练过程中采用单一被试法（B1-B2设计）、声母最小音位对比式识别能力评估A监控词表进行监控。结果训练后,该儿童声母识别的正确率较训练前有了大幅度提高（由62.1％上升为93．3％）,并且通过了同龄儿童的参考标准;处理前期和处理后期两组数据存在极显著差异（P〈0.01）。同时,个案在日常生活中的识别能力也有了一定程度的提高。结论本研究设计的训练方案对提高听障儿童声母最小音位对比式识别能力是有效的。     

突发性聋伴眩晕患者血管损伤因素相关检查结果的分析

目的 总结突发性聋伴眩晕四种听力类型患者的临床特点,分析与血管损伤因素相关检查的临床价值及意义,了解血管因素在突发性聋伴眩晕发病机制中的作用.方法 收集我院2015年10月至2017年4月住院治疗的突发性聋伴眩晕病例,按听力曲线类型分成低频下降型、高频下降型、全频下降型、全聋型4组,分析其颅多普勒(TCD)、颈部血管彩色多普勒超声、MRI检查结果异常的分布情况和血浆同型半胱氨酸(HCY)水平.结果 突发性聋伴眩晕病例中全聋型占35.7％、高频听力下降型24.5％,低频听力下降型20.4％,平坦下降型19.4％.血管相关检查结果显示,血浆同型半胱氨酸异常检出率为83.7％,MRI为67.3％,颈部彩超为52.0％,TCD为17.3％.低频下降型患者血管相关检查异常率明显低于其他类型患者,差异有统计学意义(P＜0.05).结论 突发性聋伴眩晕病人中,低频下降型血管因素影响较小.同型半胱氨酸和MRI检查是突发性聋伴眩晕病人血管损伤性因素的敏感指标.     

耳后药物注射治疗突发性聋32例

目的：观察耳后注射糖皮质激素治疗突发性聋的疗效。方法65例突聋患者分为耳后注射组（32例）和鼓室给药组（33例）,除常规治疗外,两组分别经耳后注射和鼓膜穿刺给予地塞米松0．5 mg,1次／3 d,疗程14 d。结果耳后注射组和鼓室给药组听力改善分别为（13．2±8．6）dB 和（14．6±11．1）dB,两组治疗前后比较差异均有统计学意义（P ＜0．05）,组间比较差异无统计学意义（P ＞0．05）,不良反应试验组低于对照组（P ＜0．05）。结论突聋患者耳后局部注射糖皮质激素效果明显、操作简单、不良反应低,可作为鼓室给药有禁忌时的备选疗法。