听神经病在纯音听阈测听和声导抗检查中的特征性表现

目的 探讨听神经病在纯音听阈测听及声导抗检查中的临床听力学特点及诊断要点.方法 回顾性分析中山大学附属第三医院耳鼻喉科收治的17例(32耳)听神经病确诊患者在纯音听阈测听、声导抗检查中的听力学特点.结果 17例患者中15例为双侧发病,呈左右对称性听力曲线;26耳以轻至中度低频感音性聋为主(听力图上升型);病程<5年的听力损失主要为轻度、中度听力障碍(17/32耳),病程>5年的听力损失主要为重度、极重度听力障碍.16例(31耳)声导抗为"A"型鼓室图,15例(30耳)同侧及交叉镫骨肌声反射均未引出,2例(2耳)镫骨肌声反射阈值升高.结论 听神经病在纯音听阔测听及声导抗检查中主要表现为:(1)为双侧对称性、渐进性听力下降;(2)早期为低频上升型听力图,后期为全频听力下降;(3)呈"A"型鼓室图,镫骨肌声反射阈值升高或引不出;(4)患耳无响度重振现象.     

听神经病在纯音听阈测听和声导抗检查中的特征性表现

目的 探讨听神经病在纯音听阈测听及声导抗检查中的临床听力学特点及诊断要点.方法 回顾性分析中山大学附属第三医院耳鼻喉科收治的17例(32耳)听神经病确诊患者在纯音听阈测听、声导抗检查中的听力学特点.结果 17例患者中15例为双侧发病,呈左右对称性听力曲线;26耳以轻至中度低频感音性聋为主(听力图上升型);病程<5年的听力损失主要为轻度、中度听力障碍(17/32耳),病程>5年的听力损失主要为重度、极重度听力障碍.16例(31耳)声导抗为"A"型鼓室图,15例(30耳)同侧及交叉镫骨肌声反射均未引出,2例(2耳)镫骨肌声反射阈值升高.结论 听神经病在纯音听阔测听及声导抗检查中主要表现为:(1)为双侧对称性、渐进性听力下降;(2)早期为低频上升型听力图,后期为全频听力下降;(3)呈"A"型鼓室图,镫骨肌声反射阈值升高或引不出;(4)患耳无响度重振现象.     

听性脑干反应和纯音听阈在听神经瘤早期诊断中的应用

目的 探讨采用听性脑干反应和纯音听阈对早期诊断听神经瘤的临床应用价值.方法 回顾性分析了111例听神经瘤患者的临床资料、纯音听阈、听性脑干反应及增强磁共振结果,采用线性回归分析纯音听阈均值与肿瘤体积、病程是否存在相关性,采用卡方检验分析不同肿瘤体积在听性脑干反应异常发生率上是否存在差异.结果 听神经瘤引起感音神经性耳聋,纯音听阈均值与病程存在显著地相关性(P=0.000);听性脑干反应诊断听神经瘤的敏感度和特异度分别为98.2％和93.6％,肿瘤最大径＞3 cm与≤3 cm两组,在患侧和对侧Ⅲ～Ⅳ波间期异常发生率上,差异均具有统计学意义(P值分别为0.038和0.045).结论 听性脑干反应联合纯音测听是早期诊断听神经瘤的有效方法.     

伴耳鸣和听力下降的重症肌无力(附7例报告)

目的探讨重症肌无力(MG)患者伴有耳鸣和听力下降的诊断与鉴别诊断及可能的机制。方法7例伴耳鸣和听力下降的MG患者在注射新斯的明前后进行脑干诱发电位(BAEP)和电测听检查,并随访。结果所有患者在抗胆碱酯酶药治疗前BAEP检查均异常,治疗后检查BAEP好转或恢复正常,耳鸣和听力下降症状6例消失,1例好转。结论MG患者的耳鸣和听力下降可能与听神经传导通路和中耳听小骨肌神经-肌肉接头受累有关,无需特殊治疗。     

面肌痉挛患者微血管减压术后听力情况临床分析

目的研究微血管减压术对患者听力的影响,探讨听力受损的原因及其相关因素。方法回顾性研究102名面肌痉挛患者围手术期听力情况,同时使用t检验、χ2检验和多元Logistic回归分析患者术后听力减退的相关因素。结果术后电测听结果显示:24名(23.5%)患者纯音听阈均值降低大于5dB,其中:手术侧13耳,非手术侧12耳。统计学研究发现,术后患者是否出现纯音听阈降低,与患者责任血管有关(P0.05)。结论微血管减压术术前及术后应常规做双耳电测听检查。微血管减压术后PTA降低是由多种因素造成的。对于多根责任血管共同压迫的HFS患者,术者应尤其注意。     

传导性耳聋合并鼓室段面神经畸形患者的听力重建

目的探讨听力重建技术在传导性耳聋合并鼓室段面神经畸形患者中的应用价值。方法对我院2014-01—2015-010耳鼻喉科收治的18例传导性耳聋合并鼓室段面神经畸形患者的临床资料进行回顾性分析,从胚胎解剖学和外科技术角度分析患者鼓室段面神经走形位置状态、砧镫骨、前庭窗畸形状态、听骨链重建方式,为临床治疗总结经验。结果根据术中处理方式将患者分为4组:A组(置入TORP组7例)、B组(置入PORP 3例)、C组(置入Piston组4例)、D组(于鼓阶起始处置入TORP组4例);A组2例外耳道均正常,镫骨上结构出现异常,底板活动度相对较差,面神经覆盖部分前庭窗,置入TORP术后患者听力恢复良好;B组均为先天性小耳畸形,多伴随外耳道闭锁,镫骨上结构正常完整,面神经覆盖底板,低垂挤压镫骨上结构,置入PORP术后复查2组听力均有所提升;C组多存在砧骨、镫骨上结构异常,前庭窗骨性闭锁,面神经骨质缺损,置入Piston术后患者听力均有所提升;D组镫骨呈块状结构,底板均为闭合骨质,面神经骨质缺损,覆盖前庭窗,于鼓阶起始处置入TORP术后1例恢复良好,未出现并发症,另1例改善不明显。结论传导性耳聋合并鼓室段面神经畸形患者的听力重建技术应用效果突出,临床实践中要结合患者的具体情况科学选择听力重建技术。     

微血管减压术治疗面肌痉挛致术后听力障碍临床分析

目的探讨微血管减压术（MVD）治疗面肌痉挛（HFS）术后听力障碍的因素及预防措施。方法面肌痉挛MVD后发生听力障碍的患者75例，采用电测听对听力进行检测。结果术后7d内听力下降组电测听示：B级20例，C级21例，D级10例，E级15例，F级9例。半年后听力正常组2例发生迟发性听力障碍，电测听为C级；听力下降组电测听复查示：A级13例。B级11例，C级19例，D级8例，E级15例，F级9例。结论引起MVD术后听力障碍因素复杂，多不可恢复，预防为主。     

突发性耳聋43例诊治体会

我科自2003-01～2009-06对43例患者采用综合治疗方法,取得满意效果,现报告如下。1资料与方法1.1病例选择标准所有病例均采用中华医学会耳鼻咽喉科学会1997年发布标准,行声导抗及纯音测听检查,且行颅脑CT或MRI排除听神经鞘瘤等颅脑疾患。     

特发性震颤听力障碍特点及其本质分析

目的部分特发性震颤(ET)患者存在听力障碍,但是针对性研究较少。通过对ET患者听力障碍特点的研究,推测ET本质特征,为ET的临床诊疗提供可供参考的策略。方法采用平行组设计,选取曲靖市第二人民医院神经内科住院部和门诊73名ET患者(146耳)和60名健康体检对照者(120耳),分别进行简易智力状态检查量表(MMSE)评分、问卷调查、纯音听力测试(PTA)和畸变产物耳声发射(DPOAE)检测,同时测定DPOAE的幅值。结果调查问卷中ET组听力下降耳数与对照组比较,差异有统计学意义(P=0.027);客观听力测试中,ET组DPOAE和PTA检出听力下降耳数与对照组比较,差异有统计学意义(P=0.019,P=0.046)。问卷调查中ET组耳鸣发生率与对照组比较,差异有统计学意义(P=0.013);ET组者在高频耳鸣发生率与对照组比较,差异有统计学意义(P=0.000)。ET病程与DPOAE下降幅值之间关系回归分析结果显示,两者呈线性相关(r=0.948, P=0.005)。结论听力障碍可能是ET的另一个经典非运动症状(NMS),推测ET可能为神经系统变性疾病。     

中型听神经瘤术中听力保存及影响因素

目的 探讨中型听神经瘤听力保存的影响因素.方法 回顾性研究了乙状窦后入路听神经瘤显微手术58例.其中,试图听力保存的中型肿瘤22例.术前、术后行听力学检测纯音听阈(PTA)、言语识别率(SDS)和听觉诱发电位(BAEP),进行对比分析.结果 全部58例病人中,听力得以较好保存的4例.除去未考虑听力保存的大型肿瘤,22例中型肿瘤中听力得以保存的(较好保存PTA 60dB,SDS≥50%;部分保存PTA≤60dB,SDS≥50%)9例.12例肿瘤和耳蜗神经粘连重,10例没有粘连或粘连轻.结论 肿瘤与耳蜗神经的分界面是否粘连是听力保存的重要影响因素.术中神经监测对指导肿瘤切除是有益的,但术者的局部解剖知识和显微外科技术仍是手术成功的最基本因素.     

Primary auditory neuropathy - an enigma

OBJECTIVES: To report audiological and neuroradiological findings in patients with primary auditory neuropathy (PAN) from a tertiary institution in south India. MATERIAL AND METHODS: The auditory profiles in 24 patients were analyzed along with neurological and radiological findings. RESULTS: Puretone audiometry showed varied hearing acuity and configuration. The syllable recognition score (n=15) was above 80% except in three. The dichotic digit test (n=11) showed bilateral suppression except in two who showed unilateral suppression. The auditory brainstem responses and the acoustic stapedius reflex were absent with distortion product otoacoustic emission (DPOAE) present in all. The neurological and radiological studies were normal. CONCLUSION: The audiological profile is consistent with the presence of DPOAE and absence of acoustic stapedius reflex and auditory brainstem responses. In patients with adequately preserved syllable recognition in quiet conditions, there was a significantly poor performance on dichotic digit test. As these patients had no neurological deficits and absence of specific etiological factors, we propose to use the term PAN for this entity.     

Facioscapulohumeral muscular dystrophy (FSH) and hearing loss

It has been reported that cochlea is the lesion of hearing loss in FSH. However, the details of this lesion are not yet sufficiently known. We performed detailed audiologic studies to examine hearing loss in FSH. We experienced 2 cases of FSH associated with hearing loss. Case 1 was a girl aged 5 years, and case 2 a boy aged 15 years. Clinical findings, EMG and muscle biopsy gave a diagnosis of FSH in both cases. Hearing loss was evaluated by pure tone audiography, speech audiography, tympanometry, stapedial reflex, auditory brain stem response and electrocochleography. In case 1, pure tone audiograms revealed high tone hearing loss without an A-B gap. On speech audiography, the maximum articulation score was 100% and proved normal. The tympanogram was type A. Stapedial reflex was normal bilaterally. The threshold of the 5th wave increased markedly on auditory brain stem response. On electrocochleography, the H-curve of the input-output function curves of action potential was recorded, but the L-curve was absent. There were no complaints of hearing loss in case 2, but pure tone audiograms revealed high-tone hearing loss without an A-B gap. The tympanogram was type A. Stapedial reflex was normal bilaterally. On auditory brain stem response, threshold was increased and latency was prolonged when intensity was lowered. The electrocochleograms were almost normal. It has been reported that, in electrocochleography, the L-curve represents the function of the outer hair cells and the H-curve that of the inner hair cells. The electrocochleograms in case 1 showed damage to the outer hair cells.(ABSTRACT TRUNCATED AT 250 WORDS)     

Hearing impairment in Parkinson's disease: Expanding the nonmotor phenotype

The objective of this study was to evaluate hearing impairment in patients affected by Parkinson's disease compared with hearing scores observed in normal age‐ and sex‐matched controls. One hundred eighteen consecutive patients with a clinical diagnosis of Parkinson's disease were screened. Severity of motor symptoms and staging were measured with the Unified Parkinson's Disease Rating Scale (section III) and the Hoehn and Yahr scale. Audiometric evaluation consisted of a comprehensive audiologic case history and questionnaire, visual otoscopic examination, acoustic immittance measures (tympanogram and acoustic reflexes), pure tone audiometry, and measurement of brain stem auditory‐evoked potentials. Healthy age‐ and sex‐matched subjects were selected as the control group. One hundred six of 118 patients were enrolled. Pure tone audiometry revealed age‐dependent high‐frequency hearing loss in patients with Parkinson's disease compared with both normative values and values for healthy age‐ and sex‐matched controls (75/106 [71%], χ² = 5.959, P = .02; 92/106 [86.8%] vs 60/106 [56.6%], χ² = 23.804, P < .001, respectively). Pure tone audiometry scores correlated with Hoehn and Yahr scale scores (P < .05). Brain stem auditory‐evoked potentials were normal in all patients. Our patients with Parkinson's disease showed age‐dependent peripheral, unilateral, or bilateral hearing impairment. Whether these auditory deficits are intrinsic to Parkinson's disease or secondary to a more complex impaired processing of sensorial inputs occurring over the course of illness remains to be determined. Because α‐synuclein is located predominately in the efferent neuronal system within the inner ear, it could affect susceptibility to noise‐induced hearing loss or presbycusis. It is feasible that the natural aging process combined with neurodegenerative changes intrinsic to Parkinson's disease might interfere with cochlear transduction mechanisms, thus anticipating presbycusis. © 2012 Movement Disorder Society     

Central hyperacusis with phonophobia in multiple sclerosis

Hearing disorders are a well-described symptom in patients with multiple sclerosis (MS). Unilateral or bilateral hyperacusis or deafness in patients with normal sound audiometry is often attributed to demyelinating lesions in the central auditory pathway. Less known in MS is a central phonophobia, whereby acoustic stimuli provoke unpleasant and painful paresthesia and lead to the corresponding avoidance behaviour. In our comparison collective, patient 1 described acute shooting pain attacks in his right cheek each time set off by the ringing of the telephone. Patient 2 complained of intensified, unbearable noise sensations when hearing nonlanguage acoustic stimuli. Patient 3 noticed hearing unpleasant echoes and disorders of the directional hearing. All patients had a clinical brainstem syndrome. ENT inspection, sound audiometry and stapedius reflex were normal. All three patients had pathologically changed auditory evoked potentials (AEPs) with indications of a brainstem lesion, and in magnetic resonance imaging (MRI) demyelinating lesions in the ipsilateral pons and in the central auditory pathway. The origin we presume in case 1 is an abnormal impulse conduction from the leminiscus lateralis to the central trigeminus pathway and, in the other cases, a disturbance in the central sensory modulation. All patients developed in the further course a clinically definite MS. Having excluded peripheral causes for a hyperacusis, such as, e.g., an idiopathic facial nerve palsy or myasthenia gravis, one should always consider the possibility of MS in a case of central phonophobia. Therapeutic possibilities include the giving of serotonin reuptake inhibitors or acoustic lenses for clearly definable disturbing frequencies.     

Pure word deafness in two patients with subcortical lesions

We report two patients with pure word deafness (PWD) with tumour in the III ventricle region with obstructive hydrocephalus. A diagnosis of PWD was made in these two patients in view of impaired verbal comprehension in the presence of adequate hearing, intact acoustic stapedius reflex and well preserved environmental sound perception. Return of verbal comprehension following the radiation therapy observed is probably due to the reduction of the tumour mass and the release of thalamocortical auditory pathways from its compressive effect. Our findings support the hypothesis of the presence of discrete auditory pathways for mediation of verbal and non-verbal stimuli independently.     

Cochlear origin of hearing loss in MELAS syndrome

There have been few studies investigating the mechanism and nature of the hearing loss that occurs in the mitochondrial disorders. We studied 18 patients with the MELAS A3243G point mutation from four different kindreds. Pure tone audiometry, speech discrimination testing, acoustic reflexes, tympanometry, and brain stem auditory evoked responses were performed to localize the site of pathology in the auditory pathways. In 12 patients, we performed electrocochleography and otoacoustic emissions to assess cochlear involvement. Neuroimaging and promontory nerve stimulation were performed to exclude retrocochlear pathology. Audiological testing confirmed sensorineural hearing loss in 14 of the 18 patients studied; hearing loss was usually gradual in onset, was symmetrical, and initially affected the higher frequencies. In some patients, there were features that distinguished the hearing loss from presbyacusis, including a young age at onset, asymmetrical involvement, stepwise progression, and partial recovery. We treated one patient who had profound bilateral hearing loss with cochlear implantion; this restored good functional hearing. Hearing loss in MELAS syndrome appears to be due to dysfunction of the cochlea, probably resultiong from metabolic failure of the stria vascularis and outer hair cells. Cochlear implantation is a therapeutic option worth considering in those patients who become deaf.     

Neurophysiologic,audiometric and vestibular function tests in patients with hyperostosis cranialis interna

Objective

Hyperostosis cranialis interna (HCI) is an autosomal dominant sclerosing bone dysplasia affecting the skull base and the calvaria, characterized by cranial nerve deficits due to stenosis of neuroforamina. The aim of this study is to describe the value of several neurophysiological, audiometric and vestibular tests related to the clinical course of the disorder.

Methods

Ten affected subjects and 13 unaffected family members were recruited and tested with visual evoked potentials, masseter reflex, blink reflex, pure tone and speech audiometry, stapedial reflexes, otoacoustic emissions, brainstem evoked response audiometry and electronystagmography.

Results

Due to the symmetrical bilateral nature of this disease, the sensitivity of visual evoked potentials (VEPs), masseter reflex and blink reflex is decreased (25–37.5%), therefore reducing the value of single registration. Increased hearing thresholds and increased BERA latency times were found in 60–70%. The inter-peak latency I–V parameter in BERA has the ability to determine nerve encroachment reliably. 50% of the patients had vestibular abnormalities. No patient had disease-related absence of otoacoustic emissions, because the cochlea is not affected.

Conclusion

In patients with HCI and similar craniofacial sclerosing bone dysplasias we advise monitoring of vestibulocochlear nerve function with tone and speech audiometry, BERA and vestibular tests. VEPs are important to monitor optic nerve function in combination with radiological and ophthalmologic examination. We do not advise the routine use of blink and masseter reflex.     

The stapedius reflex in the diagnosis of myasthenia gravis

The contralateral stapedius reflex, elicited by a clearly-defined auditory stimulus, was studied in 3 patients with myasthenia gravis. The reflex was assessed by recording the change which occurred in the acoustic impedance of the middle ear. In all patients a decay (decrement) of the reflex was found, interpreted as a sign of increased fatiguability at the neuromuscular junction of the stapedius muscle. The reflex recovered rapidly after the intravenous injection of a cholinesterase inhibitor (Tensilone?g;).It is concluded that the stapedius reflex test can be of value in the diagnosis of myasthenia gravis, since it can be well-standardized, involves a muscle innervated by the facial nerve and is not disturbing to the patient, whose active cooperation is not necessary.