强直性脊柱炎患者的听力学及抗膜迷路蛋白抗体的检测

目的 对强直性脊柱炎（ａｎｋｙｌｏｓｉｎｇ ｓｐｏｎｄｙｌｉｔｉｓ,ＡＳ）患者进行听力学检测并分析听力减退的特点。方法 采用问卷调查、临床耳鼻咽喉科检查、听力学测试、抗膜迷路抗体检查、血清免疫学检查方法,对确诊为ＡＳ的３４例（６８耳）患者的临床资料加以分析。结果 ３４例中主诉听力减退１１例,纯音测听发现听力减退２４例（４１耳）,占６０．３％（４１／６８）,２耳见鼓膜穿孔为混合性聋,３９耳为感生聋,     

热休克蛋白70抗体检测对自身免疫性聋诊断的意义

目的 分析自身免疫性聋的听力学特点，探讨用免疫印迹法(Western blot)检测热休克蛋白70抗体在自身免疫性耳聋诊断中的意义。方法自身免疫性聋14例28耳，其它感音神经性聋8例16耳，正常对照6例12耳，采用免疫印迹法检测各组受试者血清中的热休克蛋白70抗体，并分别作临床听力学检查。结果自身免疫性耳聋者纯音听力图多数为平坦型，TEOAE和／或DPOAE未引出，听性脑干反应多数正常，轻、中度耳聋者可引出镫骨肌声发射，该组8例(57．1％)热休克蛋白70抗体阳性。其它感音神经性聋者纯音听力图多数为高频减退型和平坦型，TEOAE和DPOAE未引出，重度耳聋者ABR未引出，轻、中度耳聋者可引出镫骨肌声发射，1例(12．5%)热休克蛋白70抗体阳性。正常对照组1例(16．7％)热休克蛋白70抗体阳性。结论免疫印迹法检测热休克蛋白70抗体对自身免疫性耳聋诊断有参考价值，对该病的确诊还应结合临床听力学特点。     

73例波动性感音神经性聋病因及临床特点分析

目的探讨波动性感音神经性聋(FSNHL)的病因及其相应的临床特点,分析引起误诊及漏诊的原因,为临床诊疗提供借鉴。方法回顾性分析73例波动性感音神经性聋的临床表现、听力学及影像学特征。结果①病因:按照发病率高低依次为低频感音神经性聋(LSNHL)29%(21/73),梅尼埃病(MD)23%(17/73),内耳畸形16%(12/73),迟发性膜迷路积水11%(8/73),自身免疫性感音神经性聋(ASNHL)7%(5/73),早期听神经瘤6%(4/73),噪声性聋4%(3/73)及不明原因者4%(3/73)。本组病例未见听神经病、外淋巴漏及耳周血管变异等病因。②LSNHL、MD早期多表现为低频听力波动;早期听神经瘤及噪声性聋表现为高频听力波动;ASNHL及双侧内耳畸形多为双侧对称性感音神经性聋;③容易误诊及漏诊的疾病:双侧对称性前庭扩大畸形可于成年后出现对称性、波动性、进行性感音神经性聋,发病常有导致颅压及腹压骤变的诱因,影像检查可与ASNHL鉴别;单耳高频波动性感音神经性聋需警惕早期听神经瘤,增强MRI应作为常规检查以避免漏诊。结论波动性感音神经性聋病因众多,表现各异;诊断除需关注病史、症状及听力学特征外,发病诱因及职业环境可提供重要诊断线索。     

听神经病谱系障碍患者的临床及听力学特征分析

目的探讨听神经病谱系障碍(auditory neuropathy spectrum disorder,ANSD)的临床和听力学特征。方法分析14例(22耳)ANSD患者的临床资料及听力学检查结果,14例均行声导抗测试、听性脑干反应(auditory brainstem re-sponse,ABR)测试及畸变产物耳声发射(distortion product otoacoustic emission,DPOAE)测试,9例行纯音听阈测试,6例行听性稳态反应(auditory steady-state response,ASSR)测试。5例行颞骨薄层CT检查,9例行MRI检查。结果 14例(22耳)患者中,双侧ANSD 8例,单侧ANSD 6例。9例(14耳)行纯音测听的患者听力图以低频损失为主呈上升型7耳(占50%),覆盆型2耳(占14.29%),平坦型1耳(占7.14%),下降型1耳(占7.14%),全聋型3耳(占21.43%)。低频组(250Hz、500Hz)的平均阈值较中(1k Hz、2k Hz)、高频组(4k Hz、8k Hz)明显升高。声导抗鼓室图均为A型,镫骨肌反射消失。11例(6例单侧ANSD,共16耳)ABR未引出;其余3例(共6耳)ABR严重异常。14例DPOAE均正常引出。6例(9耳)ASSR结果均为极重度聋。结论 ANSD的临床表现具有明显的个体差异,这种个体差异产生的原因目前尚无法确定。对疑似ANSD的感音神经性聋患者,应行全面的听力学检查以明确诊断,对确诊的患者应定期随访。     

大前庭水管综合征患者的听力学及前庭诱发肌源性电位检测的特点

目的:探讨大前庭水管综合征(LVAS)患者的听力学、前庭诱发肌源性电位(VEMP)检测的特点及诊断意义。方法:对30例(60耳)LVAS患者行纯音测听、声导抗、ABR、畸变产物耳声发射(DPOAE)、VEMP及冷热实验检测,分析其诊断意义。结果:30例(60耳)LVAS患者均呈进行性、波动性听力下降,16例在听力波动性下降时伴眩晕。纯音测听主要呈感音神经性聋,但47耳(94.0%)在中耳功能正常的情况下于低频250、500Hz分别出现(43±17)dBHL及(33±18)dBHL骨气导差,呈混合性聋。ABR检测18耳(64.3%)于(3.06±0.52)ms引出声诱发短潜伏期负反应。42耳VEMP振幅均值为(147.10±107.55)μV,19耳反应阈为75dBnHL,7耳反应阈为65dBnHL。结论:根据进行性波动性听力减退、感音神经性聋在中耳功能正常的情况下出现低频骨气导差,ABR测试引出声诱发短潜伏期负反应及VEMP呈现高振幅低阈值的特征性提示,有助于临床医生得出LVAS的初步印象,可进一步行影像学检查以确诊。     

听神经病

目的介绍一种特殊的感音神经性听力疾患-听神经病,探讨其临床特征及听力学特点.方法报道5例听神经病患者,2例为成人;3例为儿童.记录患者的临床资料,并对患者进行纯音测听、脑干电反应测听、耳声发射、耳蜗电图及语言辨别率等听力学检查.结果5例均主诉听力下降,听力学检查纯音听阈为轻、中度感音神经性聋,与纯音测听不相符的语言辨别率明显下降,不能引出脑干诱发电位(ABR),耳蜗电图基本正常,畸变产物耳声发射(DPOAE)基本正常.提示外毛细胞功能正常,病变可能在听神经.结论听神经病是一种主诉听力下降,纯音听阈为轻、中度感音神经性聋,不能引出脑干诱发电位(ABR),畸变产物耳声发射正常的听力疾患,临床上应与其他感音神经性聋区别.     

岩尖胆脂瘤诊断及治疗问题

岩尖胆脂瘤不常见。在诊断及治疗上存在着一些疑难问题。该文对14例岩尖胆脂瘤病例作了回顾性评价及分类。14例均有耳漏及听力减退症状。6例为重度感音神经性聋;4例传导聋;2例为混合聋;(正常2例)。术中所见及放射线检查病变直接累及迷路者最多(11例),如迷路上气房病变9例。面神经功能障碍(HouseⅡ～Ⅵ度)占半数,面神经膝状节是最易受累部位,往往是迷路上骨坏死或迷路坏死的结果。治疗均采用了经岩骨手术径路。10例经迷路-耳蜗径路,去除胆脂瘤并切除游动或全长的颞骨内面神经;4例经迷路,     

儿童Turner综合征听力学特点

目的探讨中国Turner综合征患儿听力学特点及其相关因素。方法收集2013年5月至2015年7月经我院内分泌科确诊为Turner综合征的18例女性患儿,对其进行耳科学检查及听力学检查(纯音测听、声导抗及声反射阈)。结果18例患儿最小年龄5.5岁,最大16岁,中位数12.50岁。听力正常9耳,占25.00%;传导性耳聋15耳,41.67%,感音神经性聋12耳,占33.33%,其中全频感音神经性聋2耳,中频"U"型感音神经性聋6耳,高频听力下降4耳。对单体型/等臂型与嵌顿型不同核型间就听力损失表型进行χ2检验,P=0.988>0.05,差异无统计学意义。骨导PTA及4k Hz骨导阈值与年龄进行一元线性相关分析,r值分别为0.564及0.606,相应P值均为0.000<0.01,统计学上具有极其显著差异。结论 TS综合征患儿染色体核型与听力损失表型间无相关联系。骨导PTA及4k Hz骨导阈值与年龄变化线性相关,即随着年龄增长骨导PTA及4k Hz骨导阈值均升高。故随着年龄增长,TS患者相对出现感音神经性聋的风险加大,且听力损失程度增加,建议TS患者长期随访,定期检查听力,及时给予相关治疗。     

听力筛查未通过婴幼儿的听力学特点及原因

目的探讨听力筛查未通过婴幼儿的听力学特点及原因。方法应用听性脑干反应（auditory brainstem response,ABR）、40Hz听觉相关电位（40 Hz auditory event related potential,40 Hz AERP）、畸变产物耳声发射（distortion product otoacoustic emissions,DPOAE）、鼓室声导抗及声反射方法,对126例听力筛查未通过的婴幼儿进行听力学诊断性检查,确认听力损失的程度及性质,并对听力损失婴幼儿的原因进行分析。结果126例（252耳）婴幼儿中,感音神经性聋61例（48.41%）,传导性聋48例（38.09%）,ABR反应阈值正常范围17例（13.50%）。此组患儿既往病史有母孕期感染病史21例、先兆流产9例、高龄6例、延期妊娠7例、伴全身系统疾病10例;新生儿期黄疸13例、缺氧窒息18例、早产儿及低体重8例、伴其他新生儿疾病8例、有听力损失家族史5例,颅面畸形3例,中枢神经系统疾病6例,第2胎9例。结论听力筛查未通过的婴幼儿听力评估后有不同的表现,本组感音神经性聋较传导性聋所占比例高,多数听力损失患儿伴有高危因素。     

感音神经性聋病人的抗内耳血管抗体测定的研究

目的 :研究某些感音神经性聋患者血清中是否存在抗内耳血管抗体。方法 :通过临床详细询问病史、纯音测听、声导抗测试及耳声发射筛查 ,选择耳蜗性聋患者 4 0 7例为研究对象 ,其中突发性耳聋 35例 ,Ménière′s病 4 1例 ,不明原因耳蜗性耳聋 331例。以豚鼠内耳石蜡切片作为抗原 ,用间接免疫荧光法检测患者血清中的抗内耳血管抗体。结果 :4 0 7例患者中有 6 9例血清中抗内耳血管抗体阳性 ,阳性率为 16 .95 %。其抗体类型有抗毛细血管内皮抗体 ,抗血管壁弹力纤维抗体 ,抗血管壁内膜和外膜抗体等。 6 9例患者中 ,单纯抗血管壁抗体阳性者 31例 ,占 4 4 .93% ,其余 38例患者还同时存在其它抗内耳抗体。在 6 9例患者中临床诊断突发聋者 12例 ,Ménière′s病者 13例 ,不明原因耳蜗性聋者 4 4例 ,占6 3.77%。 6 9例患者纯音测听结果显示低频听力下降者 4 2例 ,平坦型听力下降者 2 4例 ,高频听力下降者 3例。结论 :某些感音神经性聋患者血清中存在各种类型的抗内耳血管抗体。     

急性低频感音神经性听力损失的临床研究

目的　探讨急性低频感音神经性听力损失(acutelow tonesensorineuralhearingloss,ALHL)的病因病理、临床特点和诊治方法。方法　选择30例ALHL患者为研究对象,在详细采集病史和耳科检查的基础上,行系统的听力学检测,包括纯音测听、声导抗测试、听性脑干反应(auditorybrainstemresponse, ABR)和耳声发射(otoacousticemissions,OAE)。所有患者接受为期15天的皮质类固醇激素试验性治疗,疗程结束后随访6~14个月(平均10 2个月)。结果　本组患者以青年为主,临床主诉多为低音调耳鸣、耳闷和听力下降,耳科检查未见异常。所有患者(30例31耳)纯音测听显示轻到中度的低频感音神经性听力损失;鼓室图呈“A”型, 26耳引出镫骨肌声反射,其中14耳Metz试验阳性。受检的20耳中,ABR均正常;初诊时14耳瞬态诱发性耳声发射未通过,畸变产物耳声发射听力图表现为0 .5及0 .75kHz两个频率点上异常。经激素治疗后, 24耳听力完全恢复, 4耳部分恢复, 3耳无变化,总有效率90. 3%。结论　ALHL以突发的低音调耳鸣、听力下降和耳闷为主要表现,常单耳发病,听力学定位诊断提示为蜗性病变,仅累及低频区。本病尚无特效疗法,皮质类固醇激素可能是一种有希望的治疗药物。     

急性低频下降型感音神经性听力损失的临床研究

目的：研究急性低频下降型感音神经性听力损失（ALHL）的病因、诊断和治疗方法。方法：选择55例ALHL患者为研究对象,经详细询问病史和耳专科检查后,行纯音测听,声导抗测听和听性脑干反应（ABR）,随机分为泼尼松组和对照组治疗,疗程结束后随访6个月。结果：两组患者多以青年为主,主要症状为耳闷,低音调耳鸣和听力减退,耳科检查无殊。所有患者（55例58耳）纯音听阈示轻到中度低频感音神经性聋,鼓室导抗图“A”型,49耳镫骨肌反射引出,其中40耳Metz试验阳性。受检的45耳中ABR均正常。治疗后泼尼松组效果优于对照组（P〈O．05）。结论：ALHL以突发的低音调耳鸣,听力减退和耳闷为主要表现,常单耳发病,听力学定位诊断提示为蜗性病变,仅累及低频区。本病对皮质类固醇激素有一定的治疗效果。     

Sudden sensorineural hearing loss associated with inner ear anomaly.

OBJECTIVE: This study was conducted to evaluate the frequency of inner ear anomaly in patients with sudden sensorineural hearing loss and in control subjects. STUDY DESIGN: Retrospective case review. SETTING: A tertiary referral center. PATIENTS AND INTERVENTION: We evaluated 366 patients (165 men and 201 women; age range, 3-91 yr) with sudden sensorineural hearing loss and 228 control subjects without sensorineural hearing loss using magnetic resonance imaging. Three hundred fifty-six patients had unilateral and 10 patients had bilateral sudden sensorineural hearing loss. RESULTS: Eleven (2.9%) of 376 ears with sudden sensorineural hearing loss had inner ear anomaly. Nine patients (2.5%) had inner ear anomaly associated with sudden sensorineural hearing loss, but none of the 228 control subjects had the anomaly. The current study demonstrated that the frequency of inner ear anomaly in patients with sudden sensorineural hearing loss was significantly higher than in control subjects. CONCLUSION: Our study reveals that inner ear anomaly may be associated with sudden sensorineural hearing loss in 2.5% of patients.     

Ear involvement in Wegener's granulomatosis

In 16 of 19 patients with biopsy-proved Wegener's granulomatosis the early manifestations were limited to the ear and nose. The audiological data of 13 patients revealed middle ear involvement in 16 of 26 ears. Twenty-one of 26 ears presented with a low to moderate sensorineural hearing loss. One ear remained deaf after a sudden hearing loss in the early stage of the disease. Serologically, 4 of 6 tested patients with sensorineural hearing loss demonstrated antibodies against sarcolemma. One patient showed antinuclear antibodies. It is remarkable that these antibodies can often be detected in classic inner ear disorders. The course of inner ear function, serum findings and the success of immunosuppressive therapy in Wegener's granulomatosis are comparable with immunologically mediated vasculitis in the inner ear.     

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.     

双耳感音神经性聋患儿合并分泌性中耳炎的临床诊治

目的 探讨双耳感音神经性聋并发分泌性中耳炎患儿的症状特点, 为及时诊治此类患者提供临床依据。方法 收集经手术治疗的双耳感音神经性聋并发分泌性中耳炎患儿(A组)17例(34耳)的病历资料, 分析其误诊原因、临床特点及并发症发生率, 并与同期行手术治疗的单纯双耳单纯分泌性中耳炎患儿(B组)17例(34耳)进行鼓室粘连发生率的比较。手术前后应用听性脑干反应(ABR)检查随诊听力变化。结果 A组均以家属发现听力下降为首诊症状, 在当地首诊曾诊断为突发性聋7例, 耳闷塞感、耳鸣、耳痛等症状叙述不清, 均无法采集到确切的分泌性中耳炎发病时间;行双耳鼓膜置管时发现中耳粘连5例(7耳), 手术前后ABR检查Ⅴ波阈值改善0～30 dB nHL, 平均17.3 dB nHL, 手术前后Ⅴ波阈值改善, 差异有统计学意义(P < 0.05)。B组患儿无1例误诊, 首诊诉耳痛或耳鸣、耳闷塞感等耳部不适症状15例, 发病时间明确, 首诊诉听力下降2例, 鼓膜置管时中耳粘连1例(1耳)。A组并发症发生率高于B组, 差异有统计学意义(P < 0.05)。结论 双耳感音神经性聋并发分泌性中耳炎患儿临床病史采集困难, 易误诊, 临床并发症发生率高, 应及时干预;鼓膜置管对听力改善效果明显。     

鼻咽癌放疗后突发感音神经性聋14例临床分析

目的 探讨鼻咽癌放射治疗后突发感音神经性聋患者的临床特点及疗效,以利临床诊治。方法 回顾性分析14例（15耳）鼻咽癌放疗后突发感音神经性聋患者的临床特点及疗效。结果 鼻咽癌放射治疗后突发感音神经性聋的患者中男10例、女4例,左耳10耳、右耳5耳,平均发生时间为放疗后6.6年,突聋前多存在听力损失。250、500、1000、2000、4000 Hz的平均听阈（听力级）：突聋耳为(78.5±24.7) dB,非突聋耳为(57.0±32.4) dB。突聋耳73.33％ (11/15)为感音神经性聋,26.67％为混合性聋(4/15)。12例合并放疗后并发症,至少1例存在后循环障碍。治疗总有效率为26.67％ (4/15),其中4例复发且再治疗无效。结论 鼻咽癌放疗后患者发生突发感音神经性聋的程度较重,疗效较差,易复发。其发病机制可能与放疗所致后循环障碍有关。     

Ear involvement in Wegener's granulomatosis*

In 16 of 19 patients with biopsy-proved Wegener's granulomatosis the early manifestations were limited to the ear and nose. the audiological data of 13 patients revealed middle ear involvement in 16 of 26 ears. Twenty-one of 26 ears presented with a low to moderate sensorineural hearing loss. One ear remained deaf after a sudden hearing loss in the early stage ofthe disease. Serologically, 4 of 6 tested patients with sensorieneural hearing loss demonstrated antibodies against sarcolemma. One patient showed antinuclear antibodies. It is remarkable that these antibodies can often be detected in classic inner ear disorders. The course of inner ear function, serum findings and the success of immunosuppressive therapy in Wegener's granulomatosis are comparable with immunologically mediated vasculitis in the inner ear.     

A study of the relationship between hearing-aid usage and residual hearing

Fifty-eight children (from 4 to 19 years of age) with bilateral, symmetrical, sensorineural hearing loss, who had used a hearing aid in only one ear for 1 to 19 years were investigated to study the influence of hearing-aid upon the residual hearing between the used and no used ears. The results were as follows, 1) The average hearing level for the used ear at seven frequencies was 49. 5 dB of pre-hearing-aid usage and was 53. 8 dB of post-hearing-aid usage. The discrepancy of audiograms between the aided and unaided ear was not remarkable. 2) The hearings for both the aided and the unaided ears in Audiograms was progressive for a long time. But no significance relation was observed between changes in used-ear hearing of the aided and unaided ears. 3) When the hearing levels of the children were scrutinised on an individual basis, it was found that 9 cases (15.5%) in both ears, 2 cases (3.4%) in only used ear and 1 case (1.7%) in only no used ear out of 58 cases showed deterioration of hearing. 4) These data did not lead us to the conclusion that hearing-aid usage was detrimental on the residual hearing of children with sensorineural hearing loss.