葛根素注射液联合巴曲酶治疗突发性耳聋的临床疗效观察

目的 研究葛根素注射液联合巴曲酶治疗突发性耳聋的临床效果。方法 选择2016年2月—2016年12月在榆林市星元医院进行诊治的突发性耳聋患者78例，随机分为两组，每组各39例。对照组给予巴曲酶注射液，每次10 BU，加入500 mL生理盐水中稀释后进行静脉滴注，每两天1次。观察组在对照组基础上联合静脉滴注葛根素注射液治疗，将0.4 g葛根素注射液加入250 mL生理盐水中进行静脉滴注，每天1次。两组均治疗10 d。比较两组的临床治疗效果，以及治疗前后的活化部分凝血活酶时间、凝血酶时间、凝血酶原时间和血浆纤维蛋白原，全血黏度低切、血细胞比容、全血黏度高切、血浆黏度和纯音听阈值。结果 观察组的有效率为89.74%，明显高于对照组的71.79%，差异有统计学意义（P<0.05）。两组治疗后的凝血酶时间、活化部分凝血活酶时间以及凝血酶原时间均明显升高，血浆纤维蛋白原明显降低，同组治疗前后比较差异有统计学意义（P<0.05）；且两组间相比差异有统计学意义（P<0.05）。两组治疗后的全血黏度低切、血细胞比容、全血黏度高切及血浆黏度均明显降低，同组治疗前后比较差异有统计学意义（P<0.05）；且观察组明显低于对照组，差异有统计学意义（P<0.05）。两组治疗后的纯音听阈值均明显改善，同组治疗前后比较差异有统计学意义（P<0.05）；且观察组明显优于对照组，差异有统计学意义（P<0.05）。结论 葛根素注射液联合巴曲酶治疗突发性耳聋的临床效果明显优于单独使用巴曲酶，不仅可以有效改善患者的临床症状还可以改善血液流变学状态以及血液高凝状态。     

糖皮质激素治疗突发性耳聋的临床效果观察

目的：研究分析糖皮质激素治疗突发性耳聋的临床效果。方法：选取在我院治疗的突发性耳聋患者80例（2014年5月～2015年5月）。将其动态随机化分2组，研究组和对照组各40例。对照组患者给予非激素常规治疗，研究组患者在常规治疗的基础上给予糖皮质激素进行治疗，对比两组患者突发性耳聋的临床疗效。结果：研究组患者治愈率为27．50％，总有效率为87．50％，与对照组患者对比存在明显差异（P＜0．05）。结论：采用糖皮质激素对突发性耳聋患者进行治疗的效果显著，能有效改善患者听力情况，在临床上可以广泛应用。     

金纳多对突发性耳聋患者血流动力学的影响

目的:探讨金纳多对突发性耳聋患者血清hs-CRP、HMGB1、ADP、RES及血流动力学的影响。方法:将2009年8月～2012年5月收治的78例突发性耳聋患者随机分为对照组(常规治疗组)和观察组(加用金多纳组),每组各39例,将两组不同严重程度者治疗前及治疗后10d、20d血清hs-CRP、HMGB1、ADP、RES及血流动力学指标进行比较。结果:观察组轻度、中度及重度者治疗后基底动脉血流动力学指标异常率低于对照组,hs-CRP、HMGB1、ADP、RES改善幅度大于对照组,P均<0.05。结论:金纳多可有效改善突发性耳聋者的hs-CRP、HMGB1、ADP、RES及血流动力学。     

CT和MRI诊断儿童先天性耳聋的对比

目的 对比先天性耳聋患儿的宝石CT和MRI特点。方法 回顾性分析60例临床诊断的先天性耳聋患儿的宝石CT及MRI资料,将原始数据传至配套工作站,采用MIP、MRP、VR进行重建,分析两种检查的一致性。结果 CT显示耳部骨性结构具有优势,MRI显示耳部神经具有优势。2种检查对于显示乳突炎并大前庭导水管综合征、大前庭导水管综合征及耳蜗半规管畸形的一致性较好（Kappa>0.75）,显示半规管狭窄的一致性尚可（Kappa=0.485）。结论 CT、MR检查诊断儿童先天性耳聋各有优势,可优势互补,具有重要的临床应用价值。     

突发性聋合并耳鸣患者实施护理对降低心理负面情绪的影响

目的：观察不同护理干预方法对突发性耳聋合并耳鸣患者负面情绪的影响。方法：研究对象为本院耳鼻喉科80例突发性耳聋合并耳鸣患者,其中接受常规护理的40例患者为对照组,在常规护理基础上接受综合护理的40例患者为观察组,比较两组患者护理前后的匹兹堡睡眠质量指数、汉密顿焦虑量表评分和抑郁自评量表评分。结果：护理前,两组患者的各项观察指标比较,差异无统计学意义（P>0.05）;护理后,观察组各项观察指标均优于对照组,比较差异有统计学意义（P<0.05）。结论：给予突发性耳聋合并耳鸣患者一定的护理干预可改善患者的睡眠质量和心理状态,且综合性护理的改善效果优于常规护理。     

Homozygous and compound heterozygous mutations in the ATP6V1B1 gene in patients with renal tubular acidosis and sensorineural hearing loss

Mohebbi N, Vargas‐Poussou R, Hegemann SCA, Schuknecht B, Kistler AD, Wüthrich RP, Wagner CA. Homozygous and compound heterozygous mutations in the ATP6V1B1 gene in patients with renal tubular acidosis and sensorineural hearing loss. Distal renal tubular acidosis (dRTA) is characterized by the inability to excrete acid in the renal collecting ducts resulting in inappropriately alkaline urine and hyperchloremic (normal anion gap) metabolic acidosis in the context of a normal (or near‐normal) glomerular filtration rate. Inborn dRTA can be due to autosomal dominant or recessive gene defects. Clinical symptoms vary from mild acidosis, incidental detection of kidney stones or renal tract calcification to severe findings such as failure to thrive, severe metabolic acidosis, and nephrocalcinosis. The majority of patients with recessive dRTA present with sensorineural hearing loss (SNHL). Few cases with abnormal widening of the vestibular aqueduct have been described with dRTA. Mutations in three different genes have been identified, namely SLC4A1, ATP6V1B1, and ATP6V0A4. Patients with mutations in the ATP6V1B1 proton pump subunit develop dRTA and in most of the cases sensorineural hearing loss early in childhood. We present two patients from two different and non‐consanguineous families with dRTA and SNHL. Direct sequencing of the ATP6V1B1 gene revealed that one patient harbors two homozygous mutations and the other one is a compound heterozygous. To our knowledge, this is the first case in the literature describing homozygosity in the same dRTA gene on both alleles.     

New Tmc1 Deafness Mutations Impact Mechanotransduction in Auditory Hair Cells

Transmembrane channel-like protein isoform 1 (TMC1) is a major component of the mechano-electrical transducer (MET) channel in cochlear hair cells and is subject to numerous mutations causing deafness. We report a new dominant human deafness mutation, TMC1 p.T422K, and have characterized the homologous mouse mutant, Tmc1 p.T416K, which caused deafness and outer hair cell (OHC) loss by the fourth postnatal week. MET channels showed decreased Ca²⁺ permeability and resting open probability, but no change in single-channel conductance or expression. Three adjacent deafness mutations are TMC1 p.L416R, p.G417R, and p.M418K, the last homologous to the mouse Beethoven that exhibits similar channel effects. All substitute a positive for a neutral residue, which could produce charge screening in the channel pore or influence binding of an accessory subunit. Channel properties were compared in mice of both sexes between dominant (Tmc1 p.T416K, Tmc1 p.D569N) and recessive (Tmc1 p.W554L, Tmc1 p.D528N) mutations of residues near the putative pore of the channel. Tmc1 p.W554L and p.D569N exhibit reduced maximum current with no effect on single-channel conductance, implying a smaller number of channels transported to the stereociliary tips; this may stem from impaired TMC1 binding to LHFPL5. Tmc1 p.D528N, located in the pore''s narrowest region, uniquely caused large reductions in MET channel conductance and block by dihydrostreptomycin (DHS). For Tmc1 p.T416K and Tmc1 p.D528N, transduction loss occurred between P15 and P20. We propose two mechanisms linking channel mutations and deafness: decreased Ca²⁺ permeability, common to all mutants, and decreased resting open probability in low Ca²⁺, confined to dominant mutations.SIGNIFICANCE STATEMENT Transmembrane channel-like protein isoform 1 (TMC1) is thought to be a major component of the mechanotransducer channel in auditory hair cells, but the protein organization and channel structure are still uncertain. We made four mouse lines harboring Tmc1 point mutations that alter channel properties, causing hair cell degeneration and deafness. These include a mouse homolog of a new human deafness mutation pT416K that decreased channel Ca²⁺ permeability by introducing a positively-charged amino acid in the putative pore. All mutations are consistent with the channel structure predicted from modeling, but only one, p.D528N near the external face of the pore, substantially reduced channel conductance and Ca²⁺ permeability and virtually abolished block by dihydrostreptomycin (DHS), strongly endorsing its siting within the pore.     

方剑乔教授虚实辨证治疗耳鸣耳聋经验采撷

[目的]总结方剑乔教授治疗耳鸣耳聋的临床经验,提炼其学术思想。[方法]通过临床跟师学习,收集病案,认真研习方师对耳鸣耳聋的诊疗思路与辨证施治要点,归纳整理方师针灸取穴与临证用药经验,从病因病机和治法治则两个方面总结分析方师论治本病的学术思想,并列举验案予以佐证。[结果]方师提出,耳鸣耳聋的诊治应重视虚实辨证,首辨虚实之病机,后辨病因,实证多责之于风火之邪上扰,虚症多责之于气虚或阴精亏损,主要是肾阴肾精的亏损。治疗上辨证与辨经相结合,采用针药相辅的治疗手段,补虚泻实,标本兼顾。针灸治疗方面以患侧的耳前三穴(耳门、听宫、听会)作为治疗耳聋耳鸣的基本穴,结合虚实辨证选择电针频率,虚证用疏波,实证用密波。中药治疗多以耳聋左慈丸加减,以奏三阴并补、利湿泄浊之效,对肝肾亏虚型患者尤为效佳。所举验案中患者为耳鸣耳聋(肾精亏虚证),治拟补肾益精、滋阴养窍,经针灸耳前三穴并随证加减,并结合左慈丸口服后,患者听力及耳鸣情况较前明显改善。[结论]方师治疗耳鸣耳聋,注重辨证与辨经相结合,采用针药相辅的治疗手段,补虚泻实,标本兼顾,临床疗效显著,能明显缓解患者耳鸣症状,提高听力水平。