急性低频感音神经性聋预后相关因素研究进展

急性低频感音神经性聋指急性发作的感音神经性听力损失, 以低频听力下降为主, 可伴自听增强、耳鸣及耳闷胀感, 多不伴眩晕及眼震。目前, 对急性低频感音神经性聋的基础研究和临床分析之结果各有所见。本文就性别、年龄、病程、伴随症状、听力学检查、治疗方法、基础疾病与生活事件对急性低频感音神经性聋预后的影响进行文献综述, 供临床参考及应用。     

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。     

急性低频感音神经性聋研究进展

随着医师对原因不明的、急性起病的低频感音神经性听力损失研究的关注,关于急性低频感音神经性聋的临床症状与特点、临床诊断及听力学特征、病因病理、临床治疗及预后的报道愈多.本文就以上述文献报道内容做一综述.     

感音神经性聋高频听力易损机制

感音神经性聋是临床常见的疾病,包括噪声性聋、老年性聋、药物性聋、突发性聋等。感音神经性聋多以高频听力损失为主要表现,或由高频听力下降开始（Cole,1988;Mur-phy,1991）。以往人们对高频听力损失的认识比较局限,为何感音神经性聋高频听力比低频更容易受损,并没有明确的结论,本文就感音神经性聋高频听力损失的原因和机理做一综述,以期为采取合适的预防和治疗方案提供依据。     

人感染猪链球菌致极重度感音神经性耳聋一例

目的探讨人感染猪链球菌致感音神经性耳聋的临床特点。方法复习文献回顾性分析人感染猪链球菌致感音神经性耳聋1例。结果人感染猪链球菌致感音神经性耳聋1例临床主要表现为在感染早期即出现不可逆的双侧极重度感音神经性耳聋,治疗困难,预后差。结论人感染猪链球菌所致感音神经性耳聋发生率高,多为双耳急性起病,进展迅速,多出现于急性感染的早中期,多为重度~极重度耳聋,一般无前庭神经及其他脑神经受损症状,治疗困难,预后差,绝大多数遗留永久性听力损害。     

Hunt综合征39例分析

为提高Ｈｕｎｔ综合征的诊治水平，报告Ｈｕｎｔ综合征３９例，２３例获早期诊断，１６例分别误诊为Ｂｅｌｌ面瘫，突发性聋，疱疹性咽炎和急性化脓性中耳炎，均用强的松或地塞米松加抗病毒药物治疗。２７例痊愈，１２例后遗面瘫，其中１１例伴感音神经性聋，且８例为重度聋。认为：①不明原因的特发性面瘫合并感音神经性聋，即使无耳部疱疹，也应考虑Ｈｕｎｔ综合征；②治疗以皮质类固醇和抗病毒剂等保守措施为主，且口服和静脉给药     

母系遗传性聋家系的纯音测听及畸变产物耳声发射测试的相关分析

目的：探讨母系遗传非综合征性聋的听力学特征及畸变产物耳声发射测试对其听改变早期诊断的优越性。方法：对六个母系遗传家系成员共102人进行纯音测听、DPOAE评估其听力，比较纯音测听与DPOAE检测耳蜗早期病变的灵敏性。结果：纯音测听为感音神经性聋的38例中，PTA≥45d B HL的18例，DPOAE反应缺失；PTA＜45dB HL的20例，DPOAE高频或高、中频振幅下降或缺失；纯音测听正常者中4例DPOAE显示高频或高中频振幅下降。结论：母系遗传非综合征性聋的听力损害为双侧、对称性、进行性感音神经性聋，早期表现高频损害，DPOAE可在纯音听阈改变之前，发现早期耳蜗轻微的病理变化，对早期诊断及遗传咨询有较大指导意义。     

表现为非进行性耳聋的Ⅱ型Usher综合征

Usher综合征是一种常染色体隐性遗传病,它以感音神经性聋和继发于色素性视网膜炎的渐进性视力下降为特征。Usher综合征临床上可分为3型:Ⅰ型(Usher Ⅰ)表现为先天性极重度感音神经性聋,前庭器官可以是正常的(cUsher Ⅰ)或功能缺陷的(vcUsherⅠ):Ⅱ型(UsherⅡ)以轻至重度感音神经性聋和正常的前庭功能为特征:Ⅲ型(Usher Ⅲ)表现为进行性听力下降,散光和/或远视。目前关于Usher Ⅱ是否表现为进行性听力下降存在争议。     

重视大前庭水管综合征的 早期诊断和听力保护

大前庭水管综合征(large vestibular aqueduct syndrome,LVAS)是一种以渐进性、波动性听力下降为主要特征的感音神经性听力损失,可同时伴有反复发作的耳鸣或眩晕等一系列临床症候群;听力检查通常表现为感音神经性聋,也有部分患者表现为混合性聋.该病是上世纪70年代末随着CT技术问世而被发现的一种内耳畸形所致的听力障碍性疾病,1978年被正式命名为LVAS[1]综合.     

急性低频感音神经性聋的临床分析

目的:评估急性低频感音神经性聋的临床疗效以及相关因素对预后的影响.方法:从398例突发性聋患者中筛选出41例急性低频听力下降者,分析经糖皮质激素等综合治疗后的效果,探讨患者的性别、病程和发病年龄与疗效的相关性.结果:急性低频感音神经性聋占突发性聋的10.30%,其中女性患者占70.73%,明显高于男性.初诊时3个低频和...     

Preserved otoacoustic emissions in postparotitis profound unilateral hearing loss: a case report

We report a case of a profound unilateral sensorineural hearing loss following epidemic parotitis, with good response of otoacoustic emissions. The patient was a 12-year-old girl who had developed a unilateral hearing impairment 2 weeks after the onset of mumps. Pure tone audiometry confirmed a profound left sensorineural hearing loss. The affected ear showed an absence of auditory brain stem responses, whereas transient evoked otoacoustic emissions and distortion product otoacoustic emissions were preserved. Epidemic parotitis virus is likely responsible for an impairment of inner hair cells, primary afferent fibers or their synapses, or a combination of these areas, and it does not seem to have a specific tropism for cochlear outer hair cells. Further follow-up will be necessary to differentiate the present case from auditory neuropathy.     

Autoimmune sensorineural hearing loss: is it still a clinical diagnosis?

Inner ear involvement with sensorineural hearing loss (SNHL) has been reported in many autoimmune disorders including ulcerative colitis. The pathogenetic mechanism of hearing loss in ulcerative colitis is thought to be immune mediated. Diagnostic tests are being developed to identify inner ear autoantibodies, that may be the cause of such hearing loss. The only test that is currently available for clinical use is the Otoblot test. This, however, tests only for antibodies against bovine heat shock protein 70 which is only one of the many cross-reacting proteins against the inner ear in suspected immune-mediated hearing loss. The clinical response to steroid therapy is thus the mainstay in the diagnosis of immune-mediated hearing loss. This paper presents a series of patients with clinically suspected autoimmune hearing loss. Diagnostic assays for this condition are discussed along with a review of the recent advances in the pathogenesis and laboratory diagnosis of immune-mediated sensorineural hearing loss.     

The value of enhanced magnetic resonance imaging in the evaluation of endocochlear disease.

BACKGROUND: Gadolinium-enhanced magnetic resonance imaging (GdMRI) is routinely used in the evaluation and management of suspected retrocochlear pathology such as vestibular schwannoma. However, its value in the evaluation and diagnosis of cochlear pathology associated with sensorineural hearing loss (SNHL) has been less clear. STUDY DESIGN: Retrospective review of case histories and imaging studies of patients with SNHL and cochlear enhancement on GdMRI diagnosed between 1998 and 2000. RESULTS: Five patients with SNHL who required gadolinium administration to establish the diagnosis of endocochlear disease were identified. Diagnosed lesions included an intralabyrinthine schwannoma, intracochlear hemorrhage, radiation-induced ischemic change, autoimmune labyrinthitis, and meningogenic labyrinthitis. In these illustrative cases, the GdMRI demonstrated intrinsic high signal or contrast enhancement within the cochlea and labyrinth in the absence of a retrocochlear mass. In one patient with meningogenic labyrinthitis, cochlear enhancement on MRI led to prompt cochlear implantation before the potential development of cochlear ossification. CONCLUSION: Our experience suggests that GdMRI plays a crucial role in the diagnosis of cochlear pathology associated with sensorineural hearing loss and may directly impact patient management.     

Sensorineural hearing loss as a probable serious adverse drug reaction associated with low-dose oral azithromycin

BACKGROUND: Hearing loss as a possible side effect of azithromycin has been recognized since 1994. Most reports suggesting a link between sensorineural hearing loss (SNHL) and this drug have been in association with prolonged doses for treatment of Mycobacterium lung disease. Mild-moderate, gradual, and reversible SNHL in the speech frequencies has been most often reported. MATERIALS AND METHODS: We describe irreversible SNHL in a patient in association with low-dose oral azithromycin prescribed for acute otitis media. We summarize the available evidence, including a systematic literature review, in support of a possible causal association between SNHL and low-dose azithromycin therapy. INTERPRETATION/DISCUSSION: Physicians should be aware of the potential for even low-dose, oral azithromycin to cause irreversible SNHL as a serious adverse drug reaction in some patients.     

Coenzyme Q-10 treatment of patients with a 7445A--->G mitochondrial DNA mutation stops the progression of hearing loss

CONCLUSION: CoQ 10 may be helpful in delaying the progression of hearing loss in patients with the 7445A-->G mitochondrial mutation. Objective. To assess the effect of an antioxidant drug (Coenzyme Q-10) on the hearing level of patients with the mitochondrial DNA 7445A-->G mutation and associated sensorineural hearing loss (SNHL). Material and methods. We identified three patients with bilateral non-syndromic SNHL harboring the mitochondrial 7445A-->G mutation. Two patients had a family history of hearing loss with a strong matrilineal inheritance. The other patient did not have a family history of hearing loss. Two patients (1 with familial and 1 with sporadic SNHL) received treatment with 75 mg of Coenzyme Q-10 (CoQ10) twice a day for 1 year. The remaining patient with a familial form of hearing loss did not agree to take the treatment. Average bone conduction pure-tone thresholds for 0.5, 1, 2 and 4 kHz were obtained before and after diagnosis of mitochondrial hearing loss, and before and after treatment with CoQ10. Results. CoQ10-treated patients did not show any additional deterioration of their SNHL after 12 (familial case) and 13 months (sporadic case). The progression rate of SNHL was 6 dB/year in the 2 years prior to initiation of treatment in the familial case who received CoQ10 treatment. One year after being diagnosed with mitochondrial hearing loss, the patient who refused CoQ10 treatment exhibited an 11-dB deterioration of his hearing thresholds. There were no side-effects related to treatment with CoQ10.     

Auditory and facial nerve dysfunction in patients with hemifacial microsomia

BACKGROUND: Hemifacial microsomia (HFM) is a common craniofacial disorder characterized by a wide spectrum of anomalies, including conductive hearing loss due to external and middle ear deformities. However, the prevalence of sensorineural hearing loss (SNHL) as well as facial nerve dysfunction is underappreciated. OBJECTIVE: To determine the frequency of auditory and facial nerve dysfunction and its relationship to more severe forms of bilateral HFM. DESIGN: Retrospective medical record review to characterize the clinical severity of HFM and the prevalence and nature of the associated auditory and facial nerve dysfunction. SETTING: Center for Craniofacial Anomalies at the University of California, San Francisco, Medical Center. PATIENTS: Ninety-nine pediatric patients with HFM evaluated at the University of California, San Francisco, Medical Center. MAIN OUTCOME MEASURES: The prevalence of SNHL and facial nerve dysfunction in this patient population and any associations between these 2 characteristics. RESULTS: Hearing loss was present in 74 (75%) of 99 patients, with a conductive component in 73 patients. Sensorineural hearing loss was present in 11 patients ( 11%), with mixed hearing loss in most patients. Fourteen patients required rehabilitation with auditory amplification. Nearly a quarter of the patients (22 [22%] of 99) had facial nerve dysfunction, but only 1 patient had facial palsy on the same side as the SNHL. There was a statistically significant association between having auricular abnormalities and conductive hearing loss or SNHL (P = .30 and .80, respectively). However, there was no statistically significant association between bilateral HFM and the occurrence of either SNHL or facial paralysis, nor was there an association between auditory and facial nerve dysfunction. CONCLUSIONS: Sensorineural hearing loss and facial nerve dysfunction are common in HFM. These findings have important implications in the treatment of patients with HFM.     

Bilateral sudden sensorineural hearing loss caused by Charcot-Marie-Tooth disease

Charcot-Marie-Tooth (CMT) disease or hereditary motor and sensory neuropathy (HMSN) is a relatively common neurological syndrome, which has seldom been associated with hearing dysfunction, particularly sudden sensorineural hearing loss (SNHL). Families with autosomal dominant, autosomal recessive and X-linked forms of inheritance have been described. Sudden sensorineural hearing loss is a frustrating and frightening condition, especially if the hearing loss is bilateral. Regarding the site of the lesion, the evidence from the literature on HMSN suggests that either the VIIIth nerve or central auditory pathways are primarily involved in patients with hearing loss. We report the first case in the English literature of a patient with Charcot-Marie-Tooth type II disease presenting bilateral SNHL in the course of his disease. The patient was hospitalized for 15 days, and undergoing treatment without any audiological improvement. Detailed clinical, audiological and laboratory examination was performed. The aetiology and prognostic indicators of bilateral SNHL are discussed, as well as, the incidence of hearing loss in CMT patients.     

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.     

Cochlear implantation in a human immunodeficiency virus-infected patient

OBJECTIVES/HYPOTHESIS: Patients infected with HIV have an increased risk of developing sensorineural hearing loss (SNHL), yet pathogenesis of SNHL in HIV infection is still poorly understood. In subjects affected by bilateral profound or total SNHL, cochlear implantation may be the only possibility to restore a hearing level that allows them to have an acceptable quality of life. STUDY DESIGN: Case report. METHODS: A retrospective chart review of a HIV type 1-seropositive profoundly deafened patient who underwent cochlear implantation. RESULTS: To date, with a follow-up of 4 years, the patient has not experienced any complication and has regained useful open-set speech perception. CONCLUSIONS: Cochlear impairment with preserved auditory pathways can be responsible for profound SNHL in HIV-infected patients. Cochlear implantation can restore a social hearing in these patients, dramatically improving their quality of life. The surgical procedure can be safely performed when keeping in mind that the general condition of the patient is the decisive factor for or against surgery.