N-acetylcysteine as a single therapy for sudden deafness

Conclusion: Like NAC ameliorates hearing loss from acoustic trauma in the inner ear, NAC may also rescue hearing loss from sudden deafness confined to the inner ear.

Objective: This study assesses the effect of N-acetyl-L-cysteine (NAC) as a single therapy for sudden deafness.

Methods: Thirty-five sudden deafness patients with neither systemic disorders nor central signs in electronystagmography were treated with NAC alone and assigned to Group A. For comparison, another 35 sudden deafness patients treated by corticosteroids and plasma expander were assigned to Group B. There were no significant differences between the two groups in terms of age, sex, laterality, and pre-treatment mean hearing level. All patients underwent an inner ear test battery comprising audiometry, and ocular vestibular-evoked myogenic potential (oVEMP), cervical VEMP (cVEMP), and caloric tests.

Results: Groups A and B did not significantly differ in the pre-treatment mean hearing level, and percentages of abnormal oVEMP, cVEMP, and caloric tests, indicating that the involvement severity of sudden deafness between the two groups was similar. However, Group A (43?±?27?dB) showed significantly greater mean hearing gain than Group B (21?±?28?dB), and Group A (91%) revealed better improved rate of hearing than Group B (57%).     

The relationship between hearing loss and vestibular dysfunction in patients with sudden sensorineural hearing loss

Objective: To investigate the relationship between hearing loss and vestibular dysfunction in patients with sudden sensorineural hearing loss (SSHL).

Methods: Clinical data including the symptom of vertigo of 149 SSHL patients were investigated retrospectively. Pure tone audiometry, ocular vestibular-evoked myogenic potential (oVEMP) and cervical vestibular-evoked myogenic potential (cVEMP) evoked by air-conducted sound (ACS), and caloric test were employed for cochlear and vestibular function assessment. The relationship between hearing level and vestibular dysfunction was analyzed.

Results: The pure tone averages (PTAs) (mean?±?SD) of SSHL patients with and without vertigo were 88.81?±?21.74 dB HL and 72.49?±?21.88 dB HL (Z?=??4.411, p?=?0.000), respectively. The PTAs of SSHL patients with abnormal and normal caloric test were 84.71?±?22.54 dB HL and 70.41?±?24.07 dB HL (t?=??2.665, p?=?0.009), respectively. Conversely, vertigo and abnormal caloric results also happened more frequently in patients with profound hearing loss. However, no consistent tendency could be found among vestibular evoked myogenic potentials (VEMPs) responses or hearing loss.

Conclusions: SSHL patients with vertigo or abnormal caloric test displayed worse hearing loss; and vice versa, vertigo and abnormal caloric results happened more frequently in SSHL patients with profound hearing loss.     

Bilateral Meniere’s disease assessed by an inner ear test battery

Conclusion: An inner ear test battery may suggest bilateral involvement and aid in ‘grading’ of potential disease in a patient with actively symptomatic Meniere’s disease (MD). Objectives: This study applied an inner ear test battery in patients with bilateral MD to map the inner ear deficits in each ear. Methods: From 2009 to 2012, 100 (20%) of 498 MD patients were diagnosed with bilateral involvement, which was defined as established MD in one ear, and the opposite ear had inner ear symptoms combined with documented hearing loss. Each patient underwent audiometry, and ocular vestibular-evoked myogenic potential (oVEMP), cervical VEMP (cVEMP), and caloric tests. Results: Grading of inner ear deficits was based on the number of abnormal results in the inner ear test battery. Of 100 patients with bilateral MD, 54% had the same grade and 46% had different grades in their 2 ears. On the other hand, based on four-tone average, 79% had the same Meniere stage and 21% had different stages in their two ears. The difference between ears with asymmetric grades (46%) and ears with asymmetric stages (21%) accounts for 25% of bilateral MD patients, which may be caused by the saccular hydrops in the opposite ear.     

全聋型突发性耳聋患者的预后与前庭症状及前庭功能关系的回顾性分析

目的 探讨全聋型突发性耳聋患者的预后与前庭症状及前庭功能的关系。 方法 回顾性分析52例单侧全聋型突发性耳聋患者的前庭症状,以及眼肌前庭诱发肌源性电位(oVEMP)、颈肌前庭诱发肌源性电位(cVEMP)、视频头脉冲试验(vHIT)和冷热试验结果,观察这些患者的听力结果与前庭症状及前庭功能的关系。 结果 在52例单侧全聋型突发性耳聋患者中,有前庭症状、前庭功能异常的全聋型患者与无前庭症状者、功能正常者相比疗效差异有统计学意义。oVEMP、cVEMP、vHIT和冷热试验四项结果异常的全聋型患者显示出更低的治疗总有效率,oVEMP、cVEMP、vHIT和冷热试验正常的突发性耳聋患者有更高的听力恢复数值。 结论 有前庭症状、前庭功能异常的全聋型突发性耳聋患者疗效较差,oVEMP、cVEMP、vHIT和冷热试验正常的全聋型突发性耳聋患者有更好的听力恢复。前庭功能异常提示突发性聋患者的内耳病变范围较大、程度较重。     

Potential application of ocular and cervical vestibular‐evoked myogenic potentials in meniere's disease: A review

By stimulating the ear with air‐conducted sound or bone‐conducted vibration stimuli, vestibular‐evoked myogenic potential (VEMP) can be recorded on the contracted neck muscles, termed cervical VEMP (cVEMP), and on the extraocular muscles, termed ocular VEMP (oVEMP). These two electrophysiological tests expand the test battery for clinicians to explore the dynamic otolithic function, adding a potential usefulness to the sacculocollic reflex and vestibulo‐ocular reflex, respectively. The inner ear test battery, including audiometry, and cVEMP, oVEMP and caloric tests, is designed for complete evaluation of the inner ear function, namely, the cochlea, saccule, utricle, and semicircular canals, respectively. Using this test battery to study the localization and prevalence of hydrops formation reveals that the declining function in the cochlea, saccule, utricle, and semicircular canals mimics the declining sequence of hydrops formation in temporal bone studies. This study reviewed the physiological results in Meniere's patients via the inner ear test battery, especially the potential application of the oVEMP and cVEMP tests, to correlate with the histopathological findings of Meniere's disease. Laryngoscope, 2012     

Lermoyez syndrome revisited: 100-year mystery

Abstract

Objective: This study revisited seven patients with Lermoyez syndrome over the past 25 years using an inner ear test battery to elucidate its mechanism.

Methods: From 1992 to 2017, we have experienced 4096 patients with Meniere’s disease (MD) and seven patients (5 males and 2 females, 8 ears) with Lermoyez syndrome. Two of the Lermoyez patients were elderly, aged 71 and 85 years. An inner ear test battery comprising audiometry, ocular and cervical vestibular-evoked myogenic potential (oVEMP and cVEMP) tests, and caloric test were performed.

Results: Significant improvement of mean hearing levels (MHLs) was identified at low and middle frequencies after vertiginous attack, but not at high frequencies. Inner ear deficits in Lermoyez patients ran from abnormal hearing (100%) to abnormal cVEMP (43%), caloric (38%) and oVEMP (0) tests, exhibiting a significantly declining sequence. This declining sequence differed from that in MD.

Conclusions: Lermoyez syndrome is extremely rare, with prevalence relative to MD of 0.2%. The mechanism is considered as blockage in the ductus reuniens caused by dislodged saccular otoconia. Aging and trauma are two precipitating factors for the dislodged saccular otoconia, which may explain why Lermoyez syndrome occurs most frequently in males and some elderly.     

前庭诱发肌源性电位预测突聋患者疗效的临床价值

目的 分析单侧突发性耳聋患者的疗效,探讨前庭功能与预后的关系。 方法 回顾性分析59例(59耳)突发性耳聋患者的临床资料,观察临床疗效,根据前庭功能结果探讨对预后的影响。 结果 59例突发性耳聋患者在治疗前共进行了183项前庭功能检查,眼性前庭诱发肌源性电位(oVEMP)和颈性前庭诱发肌源性电位(cVEMP)异常的突聋患者显示出更低的治疗总有效率;oVEMP和cVEMP正常的突聋患者受损频率听力提高更明显。冷热试验(Caloric Test)和视频头脉冲试验(vHIT)的正常与否对突聋患者的总有效率和受损听力的提高无影响。cVEMP和oVEMP都异常的患者治疗无效率比仅其中一项异常的患者高,受损听力恢复程度也比仅其中一项异常的患者差。 结论 oVEMP和cVEMP异常的突聋患者疗效较差,oVEMP和cVEMP正常的突聋患者有更好的听力恢复。oVEMP和cVEMP功能可能是预测突发性聋患者预后的有效指标。前庭功能异常提示突发性聋患者的内耳病变范围较大、程度较重。     

Vestibular and cochlear nerve enhancement on MRI and its correlation with vestibulocochlear functional deficits in patients with Ramsay Hunt syndrome

ObjectiveThe correlation between enhancement of the vestibulocochlear nerves on gadolinium-enhanced magnetic resonance imaging (MRI) and vestibulocochlear functional deficits was examined in patients with Ramsay Hunt syndrome (RHS).MethodsNineteen patients with RHS who showed herpes zoster oticus, peripheral facial palsy, and vertigo were enrolled. Canal paresis (CP) in the caloric test, abnormal response to ocular and cervical vestibular myogenic potentials (oVEMP and cVEMP), and refractory sensorineural hearing loss were evaluated. MRI images perpendicular to the internal auditory canal were reconstructed to identify the superior (SVN) and inferior vestibular nerves (IVN) and the cochlear nerve (CV). The signal intensity increase (SIinc) of the four-nerve enhancement was calculated as an index.ResultsAmong RHS patients, 79%, 53%, 17% and 26% showed CP in the caloric test, abnormal responses to oVEMP and cVEMP, and refractory sensorineural hearing loss, respectively. SIinc rates of the SVN were significantly increased in RHS patients with CP in the caloric test, and with abnormal responses to oVEMP and cVEMP. SIinc rates of the SVN tended to increase in RHS patients with refractory sensorineural hearing loss (p = 0.052). SIinc rates of the IVN were significantly increased in RHS patients with abnormal responses to oVEMP and cVEMP, and refractory sensorineural hearing loss, but not in those with CP in the caloric test. SIinc rates of the CN were significantly increased in RHS patients with CP in the caloric test, abnormal response to oVEMP and refractory sensorineural hearing loss, but not in those with abnormal response to cVEMP.ConclusionIn patients with RHS, the origin of vertigo may be superior vestibular neuritis, which is affected by reactive varicella-zoster virus from the geniculate ganglion of the facial nerve through the faciovestibular anastomosis. The results also suggested that in some RHS patients, inferior vestibular neuritis contributes to the development of vertigo and that the origin of refractory sensorineural hearing loss is cochlear neuritis.     

Sensory organization test principally reflects utricular function

Conclusion: Our study showed that sensory organization test (SOT) could principally reflect utricular function, and our cut-score of VEST ratio (the indicator of inputs from the vestibular system in balance performance) in SOT could help to screen patients with unilateral peripheral vestibular dysfunction in non-fallers.

Objectives: We aimed to know SOT reflects the function of which vestibular end organs and to assess the possibility of screening patients with peripheral vestibular dysfunction in non-fall population by means of SOT.

Methods: A total of 75 patients with unilateral peripheral vestibular dysfunction underwent SOT, ocular vestibular-evoked myogenic potential (oVEMP), cervical vestibular-evoked myogenic potential (cVEMP) and caloric tests.

Results: Regression model indicated that oVEMP, as an independent variable, exerted the greatest influence on VEST ratio, followed by age and cVEMP. There were no significant differences in VEST ratio among groups with different extents of peripheral vestibular involvement (p?>?.05). VEST ratio in the vestibular impairment groups was significantly lower than in the control group and significantly higher than in fall group (p?p?=?.013). The cut-score between populations with functional and dysfunctional vestibule was 0.649.     

同步检测眼性和颈性前庭诱发肌源性电位的可行性研究

目的 探讨同步检测眼性前庭诱发肌源性电位(ocular vestibular-evoked myogenic potential,oVEMP)和颈性前庭诱发肌源性电位(cervical vestibular-evoked myogenic potential,cVEMP)的可行性.方法 对20例正常人及23例梅尼埃病患者分别进行oVEMP和cVEMP同步检测和单独检测,两种检测方式所用仪器和坐姿相同,检测参数设置相同,分别比较两种检测方式下两组对象oVEMP和cVEMP的振幅、潜伏期、波间期及双侧耳不对称比.结果 正常人左、右耳的oVEMP及cVEMP的引出率、振幅、潜伏期、波间期与不对称比在两种形式同步检测与各方式单独检测之间无显著差异;两种方式同步检测时梅尼埃病患者的患耳及对侧耳的oVEMP及cVEMP引出率、振幅、潜伏期、波间期及不对称比与各方式单独检测的结果也无显著差异.结论 对正常人和梅尼埃病患者,oVEMP和cVEMP检测可同步进行,但检测参数应与单独检测时设置一致.     

Vestibular functions in patients with idiopathic sudden sensorineural hearing loss and its relation to prognosis

ObjectiveBecause of the anatomically close relationship between the cochlea and the vestibular organs, cochlear function disorders may be accompanied by vestibular disorders. This study aimed to evaluate vestibular functions in patients with idiopathic sudden sensorineural hearing loss using VEMP, caloric test, and VNG test battery and its relation to prognosis.Materials and MethodsFor this study, 42 patients aged 18–55 years with idiopathic sudden sensorineural hearing loss and 30 volunteers who had no hearing and balance problems were included. Audiometry, cVEMP, oVEMP, caloric tests, and VNG tests were performed. Moreover, the effects of age, sex, time of admission, degree and configuration of hearing loss, accompanying vertigo, tinnitus, and ear fullness on improvement in hearing were evaluated.ResultsOf the 42 patients in the study group, 26 (56.52%) were male, 20 (43.48%) were female, and the mean age was 41.54 ± 12.23 years. Of the 30 individuals in the control group, 19 (63.3%) were male, 11 (36.7%) were female, and the mean age was 39.53 ± 13.03 years. There was no significant difference in the incidence of sudden sensorineural hearing loss in terms of sex and age, and the prognosis was better for female patients. Early admission to treatment was a factor of good prognosis; profound hearing loss, bilateral hearing loss and vertigo were factors of poor prognosis. Prognosis was better in patients with rising type audiogram configuration, while the prognosis was worse in patients with flat, descending and total hearing loss. Tinnitus and ear fullness had no effect on the prognosis. No anomalies were observed in VNG findings. Moreover, abnormal caloric response was higher in patients with profound hearing loss and total hearing configuration. Shortening was observed in cVEMP / oVEMP P1 and N1 latency after treatment. Furthermore, there was an improvement in abnormal responses after treatment.ConclusionIn this study, vestibular function was affected in patients with idiopathic sudden sensorineural hearing loss. The present study can help the development of a clinical strategy in the evaluation of the vestibular system in idiopathic SSNHL, patient follow-up, patient information, and the implementation of vestibular rehabilitation. Note that additional studies involving larger patients series are required.     

Evolution of Meniere’s Disease from MD 1.0, via MD 1.5, to MD 2.0

Abstract

Background: Elder Meniere’s disease (MD) patients ultimately lose their vestibular function.

Objective: This study utilized an inner ear test battery to investigate evolution of MD.

Methods: Total 278 elder MD patients aged >65 years were divided into three groups. Ninety-four patients with bilateral MD (188 ears) were assigned to Group A. The remaining 184 patients with unilateral MD were further divided into two groups. Group B consisted of 20 affected ears with normal vestibular function on the opposite ears, while Group C indicated 184 unaffected ears. All patients underwent an inner ear test battery.

Results: Inner ear deficits in Group B declined from the cochlea via the saccule, utricle to semicircular canals. In contrast, Groups A and C did not significantly differ in the abnormality rates of cervical vestibular-evoked myogenic potential (cVEMP), ocular VEMP and caloric tests, indicating that Group C (unaffected ears) may partly share the same mechanism like Group A (affected ears), namely aging and hydropic effects.

Conclusion and significance: Evolution of MD may progress from unilateral MD (MD 1.0), via unilateral MD coupled with asymptomatic hydrops on opposite ear (MD 1.5), toward bilateral MD (MD 2.0), where the number 1.0-2.0 means the number of clinically affected ears.     

Influence of cochlear implantation on vestibular function

Conclusion Vestibular function in patients can be damaged following cochlear implantation. Therefore, assessing the pre-operative vestibular status, carefully choosing the side of implantation, and preserving function by using minimally invasive surgical techniques are important. Objectives The aim of this study was to assess the influence of cochlear implantation on vestibular function in patients with severe and profound sensorineural hearing loss, and to analyze a possible correlation between the changes in vestibular testing and post-operative vestibular symptoms. Methods Thirty-four patients were evaluated for vestibular function using the cervical and ocular vestibular-evoked myogenic potentials (cVEMP and oVEMP, respectively), and 29 patients underwent caloric tests pre-operatively and 4 weeks post-operatively. Results Before surgery, the cVEMPs were recorded bilaterally in 22 patients, unilaterally in eight patients, and absent bilaterally in four patients. The oVEMPs were recorded bilaterally in 19 patients, unilaterally in six patients, and absent bilaterally in nine patients. After implantation, the cVEMPs were absent in 10 patients and the oVEMPs were absent in seven patients on the implanted side. Caloric tests demonstrated canal paresis in 17 patients, and normal responses were recorded in 12 of the 29 patients pre-operatively. There was a significant decrease post-implantation in the ear implanted, with the exception of two patients. Two patients presented with vertigo and another two patients reported slight unsteadiness post-operatively, but all symptoms resolved within 7 days. The impaired vestibular function did not correlate with vestibular symptoms, age, or gender. Function on the contralateral side remained unaffected.     

Sequence of vestibular deficits in patients with noise-induced hearing loss

This study utilized audiometry, and cervical vestibular-evoked myogenic potential (cVEMP), ocular VEMP (oVEMP) and caloric tests to investigate the sequence of vestibular deficits in patients with noise-induced hearing loss (NIHL). Thirty patients with NIHL underwent an inner ear test battery. Another 30 normal controls with age- and sex-matched were included for comparison. The abnormal percentages of the audiometry, and cVEMP, oVEMP and caloric tests were 100, 70, 57 and 33 % in NIHL patients, which showed significant differences from 13, 13, 7 and 3 % in normal controls, respectively. A significantly decreasing trend among the four tests, with the sequence of damage from the cochlea, followed by the saccule, utricle, and semicircular canals was noted in NIHL patients, but not in normal controls. In conclusion, the decreasing order of abnormal percentages in the function of the cochlea, saccule, utricle and semicircular canals after chronic noise exposure further supports that the pars inferior (cochlea and saccule) is more vulnerable to noise exposure than the pars superior (utricle and semicircular canals).     

前庭诱发肌源性电位检查的点评

借由气导声刺激或骨导振动刺激可成功诱发前庭诱发肌源性电位(vestibular-evoked myogenic potential, VEMP)。从颈部胸锁乳突肌表面记录得到的VEMP称为颈肌前庭诱发肌源性电位(cVEMP),从眼外肌表面记录得到的VEMP称为眼肌前庭诱发肌源性电位(oVEMP)。这两项新兴的耳神经科学功能检查开启了科学家探索耳石器官(球囊与椭圆囊)的纪元,分别用来检测“球囊-颈肌反射”和“椭圆囊-眼肌反射”神经通路。内耳功能检查序列,包括听力检查、cVEMP检查、oVEMP检查、温度试验检查等,可以对内耳终末器官诸如耳蜗、球囊、椭圆囊及半规管的功能进行全面检测,目前已经广泛应用于动物及人体。这一系列内耳功能检测项目将有助于描绘内耳终末器官受侵犯的范围、厘清过去医学上的盲点,进一步阐明内耳及中枢前庭系病变的机制。     

Is otolithic vertigo accompanied by hearing loss caused by sacculocochlear endolymphatic hydrops?

Conclusion: Otolithic vertigo is sometimes accompanied by hearing loss. Otolithic vertigo accompanied by hearing loss seems to be caused by sacculocochlear endolymphatic hydrops. Objectives: To clarify the lesion site and pathophysiology of otolithic vertigo (OV) accompanied by hearing loss. Methods: The clinical records of four patients (two men and two women) that had been diagnosed with OV accompanied by hearing loss according to pre-determined diagnostic criteria were reviewed. Results: The patients’ main symptoms involved a sensation of movement in the pitch plane. All of the patients had low frequency-dominant hearing loss and either exhibited decreased cervical vestibular evoked myogenic potentials (cVEMP) or did not produce cVEMP. Two patients produced normal ocular VEMP (oVEMP). Caloric tests obtained normal results in all patients.     

Vestibular evoked myogenic potentials in delayed endolymphatic hydrops

OBJECTIVE/HYPOTHESIS: Vestibular evoked myogenic potential (VEMP) has become an established test to explore the sacculo-collic reflex. The study aims to investigate the VEMPs in cases of delayed endolymphatic hydrops because greatly dilated saccule was observed in histopathological specimens of delayed endolymphatic hydrops. STUDY DESIGN: Prospective study. METHODS: Twenty patients with delayed endolymphatic hydrops were subjected to pure-tone audiometry, caloric testing, and VEMP test. Delayed VEMP was defined as the latency of peak I exceeding 22.6 milliseconds or of peak II exceeding 33.1 milliseconds. Interaural amplitude difference over the sum of amplitudes of both ears was measured, and when the ratio exceeded 0.36, it was identified as augmented VEMP or depressed VEMP depending on whether the amplitude of the lesioned side was greater or less than the opposite side. RESULTS: The VEMP test revealed that 9 patients (45%) were normal, 6 (30%) exhibited absent VEMPs, and 5 (25%) displayed abnormal VEMPs, including delayed VEMPs in 2, depressed VEMPs in 2, and augmented VEMPs in 1. The caloric test indicated that 9 (47%) of the 19 ears exhibited normal caloric response, whereas 10 ears displayed abnormal caloric responses including canal paresis in 8 and absent caloric response in 2. Six ears had preserved both the caloric response and the VEMPs, whereas no ear demonstrated both absent caloric response and absent VEMPs. CONCLUSIONS: The residual caloric as well as saccular functions after ear insult may determine whether delayed endolymphatic hydrops will occur. These findings suggest that patients with sudden deafness or juvenile unilateral total deafness should undergo caloric testing and VEMP test to predict the occurrence of delayed endolymphatic hydrops in the future.     

迟发性膜迷路积水的诊断

目的：探讨迟发性膜迷路积水（DEH）的诊断手段及意义。方法：15例DEH患者,分别行纯音听阈及耳蜗电图检查、前庭双温试验和前庭诱发的肌源性电位检查（VEMP）,用以诊断及判断DEH的侧别和病变累及范围。结果：15例患者均为中～重度以上感音神经性聋。积水与听力下降同侧10例,对侧4例,双侧1例。水平半规管和球囊均有积水5例,水平半规管积水6例,球囊积水2例。VEMP异常者7例,其中1例患侧p13-n23振幅消失,2例p13潜伏期延长,4例患侧p13-n23低振幅。结论：DEH的诊断除了纯音测听外,耳蜗电图检查、前庭双温试验和VEMP检查是重要的实验室检查,应列为诊断常规。     

Brainstem lesion in benign paroxysmal vertigo children: Evaluated by a combined ocular and cervical vestibular-evoked myogenic potential test

Objective

This study utilized a combined ocular vestibular-evoked myogenic potential (oVEMP) and cervical VEMP (cVEMP) test in children with benign paroxysmal vertigo (BPV) to investigate whether the upper or lower brainstem is more frequently affected in BPV children.

Methods

Fifteen BPV children aged 4-14 years, and 15 age- and sex-matched healthy children were enrolled. All subjects underwent pure tone audiometry, stabilometry, and a combined oVEMP and cVEMP test using acoustic stimulation.

Results

All BPV patients displayed normal hearing and clear oVEMPs. However, 11 (73%) of 15 BPV patients had delayed cVEMPs, showing significant difference when compared with 100% normal cVEMPs in healthy children. The sway path and sway area in stabilometry were significantly different between BPV and healthy children, regardless of whether their eyes were open or closed. However, neither the sway path nor sway area correlated significantly with cVEMP results.

Conclusion

Normal oVEMPs in BPV children indicate an intact vestibulo-ocular reflex pathway, which travels through the upper brainstem. In contrast, delayed cVEMPs in BPV children reflect a retrolabyrinthine lesion along the sacculo-collic reflex pathway, which descends via the lower brainstem. Hence, the lower brainstem is more frequently affected than the upper brainstem in children with BPV.