既往存在感音神经性听力损失突发性聋患者临床特征与疗效分析

目的探讨既往存在感音神经性听力损失突发性聋患者临床特征与疗效。方法对61例(63耳)既往存在感音神经性听力损失突发性聋患者的临床资料进行系统性分析,包括临床表现、疗效评估等,与同期收入院的既往不存在感音神经性听力损失突发性聋患者资料对比,分析发病和影响疗效的高危因素,总结该病发生、发展及预后的特点。结果既往存在和不存在感音神经性听力损失突聋患者的性别、现患耳侧别、病程差异无统计学意义(P值均>0.05),发病年龄差异具有统计学意义(P<0.05)。既往存在和不存在感音神经性听力损失突聋患者的听力曲线类型、听力损失程度差异无统计学意义(P值均>0.05),既往存在感音神经性听力损失突聋患者总有效率低于既往不存在感音神经性听力损失突聋患者总有效率,差异有显著统计学意义(χ²=6.915,P=0.009),既往存在感音神经性听力损失突聋患者痊愈率低于既往不存在感音神经性听力损失突聋患者痊愈率,差异无统计学意义(χ²=0.221,P=0.638)。结论既往存在感音神经性听力损失突聋患者病因复杂,疗效较差,应加大研究制定个性化的诊疗方案。     

Audiometric Profile in an Indian Family with Alport's Syndrome

An audiometric profile of some of the members of an Indian family with Alport's syndrome has been presented. The findings of audiometric and vestibular testing suggest an end-organ lesion. It has been suggested that an ingerent weakness of ectodermal derivatives in this syndrome, which is mediated through an inborn error of metabolism, accounts for the end-organ hearing loss, cataract and kidney involvement in such cases.     

听神经病的临床表现和听力学检查结果分析

目的 探讨听神经病的临床表现和听力学特征。方法 分析23例听神经 临床表现、纯音听阈、声导抗、言语听力、听性脑干反应（ABR）和畸变产物耳声发射（DPOAE）检查。结果 听神经病多发于青少年,妇性多于男性。纯音听阈呈轻、中度升高,以双耳对称的上升型听阈图为主。言语识别率显著下降,且与纯音听阈不成比例。同侧、对侧镫骨肌反射引不出,ABR明显异常,DPOAE正常或基本正常。结论 听神经病的临床表现和系统的听力学检查有其显著特征,不仅对认识该病,而且对明确诊断有重要意义。     

SNPscan法与Sanger测序法用于感音神经性聋患者常见聋病基因检测的对比研究

目的 比较SNPscan法与Sanger测序法用于甘肃省部分感音神经性聋患者常见聋病基因检测的优缺点.方法 在知情同意的前提下,抽取甘肃省东乡族(122例)、裕固族(11例)、保安族(18例)三个特有少数民族共151例中重度至极重度感音神经性聋患者静脉血,用磁珠法提取基因组DNA,应用SNPscan法对GJB2、SLC26A4和mtDNA基因常见115个突变位点进行筛查,采用Sanger测序法对GJB2基因的第二个外显子、SLC26A4基因P8和P18两个外显子、mtDNA的两个常见突变位点进行测序,比较两种方法基因筛查的结果.结果 经SNPscan法检测151例患者GJB2、SLC26A4、mtDNA A1555G和mtDNA C1494T基因突变的总检出率为23.18%(35/151),其中东乡族、裕固族、保安族的检出率分别为21.31%(26/122)、54.54%(6/11)、16.67%(3/18),与Sanger测序法的筛查结果一致,差异无统计学意义(P>0.05);SNPscan法在三个基因的致病基因型检出率分别为11.26%(17/151)、1.32%(2/151)、0.66%(1/151),而Sanger测序法的检出率分别为9.93%(15/151)、1.32%(2/151)、0.66%(1/151),差异无统计学意义(P>0.05).完成上述三种基因突变筛查SNPscan法耗时约10天,而Sanger测序法耗时约1个月,且前者费用较低,可同时对多个位点进行检测.结论 与Sanger测序法相比较,SNPscan法用于聋病基因检测耗费时间短,通量较高,成本较低,得到更多的有意义的突变位点,有利于降低假阴性率,因此,在进行全部外显子测序前先进行SNPscan法筛查更有意义.     

Pediatric sudden sensorineural hearing loss after diving

OBJECTIVE: Sensorineural hearing loss after diving from a low height has been rarely reported especially in children. METHODS: We present and discuss a new case of pediatric sudden sensorineural hearing loss after diving. RESULTS AND CONCLUSION: Medical and surgical approaches (indications and timing of explorative tympanotomy) to this occurrence are still controversial.     

Role of cytomegalovirus in sensorineural hearing loss of children: a case-control study Tehran, Iran

BACKGROUND: Congenital CMV is the most common cause of nonhereditary sensorineural hearing loss in children. More than 40% of deafness cases with an unknown cause are due to congenital CMV. When CMV infection is diagnosed in the first year of life, treatment with gancyclovir is recommended and is associated with diminished occurrence of SNHL. Previous studies in Tehran showed congenital CMV infection developed in 2.6% of neonates. OBJECTIVE: To determine the role of cytomegalovirus in sensorineural hearing loss in children by comparing the CMV antibodies quantitatively between SNHL and controls. METHODS AND MATERIALS: This case-control study was done in the ENT Department of Hazrat Rasoul Akram Hospital in Tehran (2002-2003). This study was carried out based on diagnostic parameters (AAO criteria) for SNHL and a healthy control. We compared the specific cytomegalovirus antibodies (IgM and IgG measured by ELISA method) in 95 blood samples of infants with SNHL (mean age=35+/-30 months) and 63 healthy matched infants (mean age=38.7+/-27.3 months) as controls. 59.6% of cases were male; 40.4% were female. Acute and previous immunity to cytomegalovirus (IgM and IgG) was found in 34.7% and 72.6% of SNHL children, acute infection detected in 3.5% of controls, previous immunity (IgG) detected in 94.7% of them. Acute CMV infection was higher in the cases (P-value=0.000) but previous immunity (CMV-IgG) was higher in the controls (P-value=0.001). Mean age of cases with acute infection (CMV-IgM) was 40 months and for previous immunity (CMV-IgG) was 35 months in SNHL cases and 40 months in controls, respectively. CONCLUSION: Cytomegalovirus is one of the most common infectious agents in SNHL children compared to the healthy children. Probably both congenital and acquired CMV can induce progressive hearing loss in our cases. We prefer at least in our country to consider seropositive (CMV-IgM) SNHL children (less than 1 year old) as congenital form. But we are not able to differentiate the congenital from the acquired infection in seropositive (CMV-IgM) SNHL children after first year of life. It should be subjected to randomized controlled trial for treatment of acquired type of CMV infection in SNHL children with ganciclovir.     

Hereditary Deafness Syndromes with Onset in Adult Life

The types of hereditary deafness in man can be separated from each other by the type of hearing loss and its age of onset, by the mode of transmission and by the associated defects caused by the abnormal gene. In this report we reviewed the clinical findings, laboratory findings, etiology, treatment, prognosis, and the diagnostic features of the 12 known types of hereditary deafness which begin in adult life.     

Prognostic factors for vestibular impairment in sensorineural hearing loss

The clinical course and prognosis in sensorineural hearing loss (SNHL) may be even worse if vestibular system is also involved, especially due to near location of anatomical structures in the inner ear. The aim of the study was to determine prognostic value of some clinical, audiological and demographic factors associated with SNHL in predicting a possibility of vestibular impairment. The study was conducted on 124 consecutive patients (183 ears) diagnosed for sensorineural hearing loss during 1 year in our department. In all of them, audiological (pure-tone, speech and impedance audiometry, ABR) and ENG examinations (visual ocular–motor, positional, kinetic and caloric tests) were performed. The correlations between ENG outcome and the following variables associated with sensorineural hearing loss were investigated: audiological (degree and location of hearing loss, audiogram configuration), clinical (tinnitus, vertigo, dizziness) and demographic (age, sex) factors. Normal ENG was recorded in 26.6%, vestibular impairment of peripheral type in 38.7%, and central type in 34.7% of the patients. In a multivariate stepwise linear regression analysis, the degree of hearing loss was the main variable correlating with abnormal ENG result. Tinnitus and location of hearing loss were also found to be the two other variables which, to some minor extent, can influence the ENG outcome. Peripheral vestibular impairment was observed more frequently in patients with residual hearing/deafness. The degree of hearing loss, presence of tinnitus and location of hearing loss are factors predicting the possibility of abnormal ENG outcome in sensorineural hearing loss.     

Auditory function in women with autoimmune inner ear diseases and their offspring

Objective

The precise cause of congenital sensorineural hearing loss (CSNHL) is unclear in many cases. In a previous study we found that offspring from guinea pigs with autoimmune sensorineural hearing loss (ASNHL) exhibited signs of SNHL. Here we studied women with autoimmune inner ear diseases (AIED) and their offspring. Our aim was to determine if autoimmune damage may be one of the causes of CSNHL.

Methods

Thirty-eight pregnant women with AIED were recruited. Thirty-three had ASNHL; one with autoimmune delayed endolymphatic hydrops (ADEH) and four with autoimmune Meniere's disease (AIMD). The following were assessed in all women: audiogram, auditory brain stem response (ABR), otoacoustic emission (OAE), vestibular function test and presence of inner ear antigens. The following were assessed in offspring from these women: OAE, ABR and presence of inner ear antigens.

Results

Five of the 38 children born to women with AIED had SNHL (an incidence much higher than normal). OAEs were not inducible in these children shortly after birth or within 46-100 days after birth. Abnormal ABR findings were apparent in these five children and inner ear antigens were detected in three of the five children (the mother's of these children were also positive for inner ear antigens).

Conclusions

These preliminary findings suggest that the prevalence of congenital ASNHL may be increased in offspring born to women with AIED.     

Audiometric shape and presbycusis

The aim of this study was to describe the prevalence of specific audiogram configurations in a healthy, otologically screened population between 55 and 65 years old. The audiograms of 1147 subjects (549 males and 598 females between 55 and 65 years old) were collected through population registries and classified according to the configuration of hearing loss. Gender and noise/solvent-exposure effects on the prevalence of the different audiogram shapes were determined statistically. In our population ‘Flat’ audiograms were most dominantly represented (37%) followed by ‘High frequency Gently sloping’ audiograms (35%) and ‘High frequency Steeply sloping’ audiograms (27%). ‘Low frequency Ascending’ audiograms, ‘Mid frequency U-shape’ audiograms and ‘Mid frequency Reverse U-shape’ audiograms were very rare (together less than 1%). The ‘Flat’-configuration was significantly more common in females, whereas the ‘High frequency Steeply sloping’-configuration was more common in males. Exposure to noise and/or solvents did not change this finding. In addition, females with a ‘Flat’ audiogram had a significantly larger amount of overall hearing loss compared to males. Furthermore, our data reveal a significant association between the prevalence of ‘High frequency Steeply sloping’ audiograms and the degree of noise/solvent exposure, despite a relatively high proportion of non-exposed subjects showing a ‘High frequency Steeply sloping’ audiogram as well.     

Evoked Response Audiometry: Influence of Somatosensation

Averaged vertex response thresholds evoked with the oscillator on the mastoid process and tibia in patients deaf since birth or early childhood, patients with sensorineural hearing loss acquired during adulthood, and subjects with normal hearing were compared and the following conclusions were reached:

(1) This study provided the first objective documentation of the pseudoauditory nature of the low-freqency air-bone gap. This vibratory phenomenon is acute in individuals who have been deaf since early childhood.

(2) Evoked response audiometry (ERA) demonstrated a potential hazard in testing deaf children. If ERA shows a low-frequency air-bone gap, one should suspect that the response is of vibratory origin. Confirmation of the origin can be obtained by recording responses evoked by tibial stimulation.

(3) ERA bone-conduction thresholds accurately correlate with conventional bone-conduction thresholds.     

Epidemiology of hearing loss in childhood

During a 20-year period, half a million screening audiograms have been obtained on children at schools in Stockholm. The incidence of hearing loss in children of a particular age did not differ appreciably over the 14-year period analysed. In the age groups from 4 to 16, the incidence of temporary conductive hearing impairment decreased with age, whereas the incidence of permanent defects and particularly sensorineural high-frequency loss showed an increase with age.

For conductive hearing impairments there was no sex difference but for sensorineural defects there was a definite prepoderance of boys which was observed as early as 7 years of age.

The results of this general survey of the total material are supplemented and verified by the findings recorded in a more thorough examination at schools in Stockholm of all 13-year-old children born in 1948. The discussion of the results leads to the opinion that high-frequency loss in children would seem to be to a large extent of genetic origin.     

感音神经性耳聋的早期识别与临床干预

感音神经性耳聋是临床常见问题,若不能及时发现和正确干预,可导致儿童听觉言语发育迟缓,以及情感、心理和社会交往等综合能力的发展。新生儿听力筛查与新生儿基因筛查使得感音神经性耳聋的早期发现成为可能。儿童期的听力筛查工作很大程度上弥补了因迟发性、渐进性或获得性听力损失导致的新生儿听力筛查漏诊的不足。感音神经性耳聋通过主客观听力检测技术可获得早期诊断,通过药物治疗、选配助听装置等获得早期干预。永久性感音神经性听力损失作为一种慢性疾病,传统的以医生为中心的医疗服务模式不足以满足患者需求,以患者、家庭或社区为中心的医疗服务模式逐渐被重视。     

Alport综合征的临床及听力学表现

目的 分析Alport综合征的临床及听力学特点。方法 回顾性分析经我院确诊为Alport综合征的9例患者临床资料，包括耳科、肾脏和眼科情况。结果 听力学表现全部为双耳对称性感音神经性聋，高频下降型67％（6／9），谷型22％（2／9），平坦型11％（1／9）；肾脏主要表现为血尿、蛋白尿、慢性肾功能不全；眼科病变主要为前锥形晶体，黄斑周围视网膜斑点。5例患者肾活检电镜表现典型。5例患者家族史阳性。结论 Alport综合征的听力学表现为双耳感音神经性聋，因此遇到双耳感音神经性聋患者应注意肾脏情况、眼科情况和家族史，以利于本病的诊断。     

急性低频感音神经性聋的短期临床疗效分析

目的探讨小剂量、短疗程皮质类固醇激素对急性低频感音神经性聋的疗效。方法选取28例(30耳)急性低频感音神经性聋患者作为研究对象,随机分为强的松治疗组16例(耳)与对照组12例(14耳),两组患者在口服等剂量强的松基础上,对照组加用血管扩张剂和神经营养剂治疗,1周后观察疗效。结果两组患者总有效率(完全恢复和部分恢复)为83.3%(25/30),总治愈率(完全恢复)46.7%(14/30);激素治疗组的有效率87.5%(14/16),治愈率50.0%(8/16),对照组有效率78.6%(11/14),治愈率42.9%(6/14),两组间的疗效比较,差异无统计学意义(P>0.05)。结论急性低频感音神经性聋的治疗有别于突发性耳聋,治疗上无需使用血管扩张剂及神经营养剂等,初始小剂量皮质类固醇激素治疗本病有效。