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.     

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).     

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.     

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%).     

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。     

Acute hearing loss in patients with hematological disorders

Objective: This study adopted an inner ear test battery to investigate the causes of acute sensorineural hearing loss in patients with hematological disorders. Methods: During the past 20 years, the authors have experienced 14 patients with hematological disorders, i.e. leukemia or aplastic anemia, having acute sensorineural hearing loss. An inner ear test battery comprising audiometry and cervical vestibular-evoked myogenic potential (cVEMP), ocular VEMP (oVEMP), and caloric tests was performed. Results: Diagnoses comprised of sudden sensorineural hearing loss in 12 patients and endolymphatic hydrops in four patients (two patients had one ear with sudden sensorineural hearing loss while the other ear had endolymphatic hydrops). Percentages of recruitment phenomenon showed a significant difference between endolymphatic hydrops and sudden sensorineural hearing loss. Abnormal percentages for mean hearing level (86%), cVEMP test (71%), oVEMP test (25%), and caloric test (14%) exhibited a significant sequential decline in these patients. Conclusion: Acute sensorineural hearing loss in a patient with leukemia or aplastic anemia may be related to either sudden sensorineural hearing loss or endolymphatic hydrops. A significant sequential decline in the function of the cochlea, saccule, utricle, and semicircular canals indicates that the pars inferior is more vulnerable to blood insult than the pars superior.     

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     

A longitudinal study of changes in distortion-product otoacoustic emissions and pure-tone thresholds in an industrial setting

In a longitudinal study of 76 noise-exposed industrial workers, 34 (68 ears) of them were available for final evaluation after 9 years of observation. Distortion-product otoacoustic emissions (DP) at 65/55 dB SPL and pure-tone audiometry were used to detect noise-induced inner ear changes. Repeated measures analyses of variance were made on the subjects and in a control group, whereas significant threshold shifts (STS) and significant emission shifts (SES) were calculated. Both hearing thresholds and DP showed a high-frequency deterioration, but there was absence of statistical correlation between elevation of hearing thresholds and decrease of DP. There was no clear pattern between individual changes in audiometric thresholds and DP, and all combinations were present: ears with only STS, ears with only SES, ears with both STS and SES and ears with absence of STS and SES. Audiometric changes were maximal at 4 and 6 kHz and DP changes at 2 and 3 kHz. Since significant individual emission changes do not necessarily follow the same pattern as the group-averaged results, the use of DP for monitoring of individual subjects is not advised. However, its use in conjunction with audiometry in hearing conservation programs is highly recommended.     

A longitudinal study of changes in evoked otoacoustic emissions and pure-tone thresholds as measured in a hearing conservation program

Non-linear transient-evoked otoacoustic emissions (TEOAEs) at 74 dB pSPL, distortion-product otoacoustic emissions (DPOAEs) at 65/45 dB SPL and pure-tone audiometry were used to detect noise-induced, inner ear changes in a longitudinal study. Repeated-measures ANOVAs were made on the Noise (n=69) and Quiet (n=42) groups. The Noise group's hearing thresholds increased by 1.2 dB and DPOAE amplitude decreased by ?0.9 dB. For both groups, TEOAE amplitude decreased by approximately ?0.6 dB. Eight of 12 ears with permanent threshold shift (PTS) and 10 of 13 ears with temporary threshold shift (TTS) showed TEOAE decrements or low baseline TEOAE amplitudes. Fewer TTS and PTS ears also showed DPOAE decrements, and there was never a DPOAE decrement without a corresponding TEOAE decrement or low TEOAE baseline. Some TTS ears showed permanent emission decrements. Although otoacoustic emissions show promise in detecting noise-induced inner ear changes, it is premature to use them in hearing conservation programs.     

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。     

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。     

Long-term functional outcome and satisfaction of patients with an active middle ear implant for sensorineural hearing loss compared to a matched population with conventional hearing aids

The objectives of the study were to review the results of an active middle ear implant for sensorineural hearing loss in patients who were unable to wear or did not benefit from conventional hearing aids in comparison to patients with a matched degree of hearing loss successfully fitted with a conventional hearing aid. A retrospective chart review of 10 patients (10 ears) after implantation of an active middle ear implant and 12 patients (13 ears) with conventional hearing aids in one tertiary referral center was performed. Intervention for sensorineural hearing loss was the implantation of an active middle ear implant in one group or fitting of conventional hearing aids in the other group. Outcome measures were pure-tone audiometry (auditory thresholds, functional gain), speech audiometry (Freiburg Monosyllabic Test in quiet and in noise) and a quality-of-life questionnaire (Glasgow Benefit Inventory). Average functional gain was 25.2 ± 8.6 and 14.6 ± 10.8 dB, speech recognition score in noise was 36.6 ± 18.4 and 31.2 ± 19.2 % and in quiet was 66.0 ± 23.2 and 61.5 ± 23.8 %, Glasgow Benefit Inventory total score was 38.3 ± 32.3 and 24.8 ± 22.2 in patients with active middle ear implants and conventional hearing aids, respectively. In two patient groups matched for degree of sensorineural hearing loss, active middle ear implants provided comparable speech recognition and superior functional gain and quality of life compared to conventional hearing aids. Level of evidence: 4     

A longitudinal study of changes in evoked otoacoustic emissions and pure-tone thresholds as measured in a hearing conservation program

Non-linear transient-evoked otoacoustic emissions (TEOAEs) at 74dB pSPL, distortion-product otoacoustic emissions (DPOAEs) at 65/45dB SPL and pure-tone audiometry were used to detect noise-induced, inner car changes in a longitudinal study. Repeated-measures ANOVAs were made on the Noise (n=69) and Quiet (n=42) groups. The Noise group's hearing thresholds increased by 1.2 dB and DPOAE amplitude decreased by -0.9 dB. For both groups, TEOAE amplitude decreased by approximately -0.6 dB. Eight of 12 ears with permanent threshold shift (PTS) and 10 of 13 ears with temporary threshold shift (TTS) showed TEOAE decrements or low baseline TEOAE amplitudes. Fewer TTS and PTS ears also showed DPOAE decrements, and there was never a DPOAE decrement without a corresponding TEOAE decrement or low TEOAE baseline. Some TTS ears showed permanent emission decrements. Although otoacoustic emissions show promise in detecting noise-induced inner ear changes, it is premature to use them in hearing conservation programs.     

Predisposing factors for inner ear hearing loss association with chronic otitis media

The aim of the present study was to investigate the consequences of chronic otitis media on inner ear function. Retrospective analysis of conventional pure-tone audiometry tests was carried out on 344 patients who were scheduled for surgical treatment of unilateral chronic otitis media without other risk factors for sensorineural hearing loss. Bone conduction thresholds of diseased ears were compared with those of contralateral, non-diseased ears. Selected clinical features were assessed among diseased ears to examine possible influences on inner ear function. Mean bone conduction threshold differences varied from 0.6 dB at 0.5 kHz to 3.7 dB at 4 kHz. These differences augmented with increasing duration of middle ear disease. Impaired hearing by bone conduction thresholds of diseased ears correlated with increased age at every frequency and with an interruption of the ossicular chain only at higher frequencies. The severity of sensorineural hearing loss correlated with longer duration of middle ear disease. Thus, surgical treatment of dry and apparently stable tympanic membrane perforation is warranted.     

Predisposing factors for inner ear hearing loss association with chronic otitis media

The aim of the present study was to investigate the consequences of chronic otitis media on inner ear function. Retrospective analysis of conventional pure-tone audiometry tests was carried out on 344 patients who were scheduled for surgical treatment of unilateral chronic otitis media without other risk factors for sensorineural hearing loss. Bone conduction thresholds of diseased ears were compared with those of contralateral, non-diseased ears. Selected clinical features were assessed among diseased ears to examine possible influences on inner ear function. Mean bone conduction threshold differences varied from 0.6?dB at 0.5?kHz to 3.7?dB at 4?kHz. These differences augmented with increasing duration of middle ear disease. Impaired hearing by bone conduction thresholds of diseased ears correlated with increased age at every frequency and with an interruption of the ossicular chain only at higher frequencies. The severity of sensorineural hearing loss correlated with longer duration of middle ear disease. Thus, surgical treatment of dry and apparently stable tympanic membrane perforation is warranted.     

Relationship between CT findings and sensorineural hearing loss in chronic otitis media

Objective

To investigate the relationships between the temporal bone CT findings and sensorineural hearing loss in ears with non-cholesteatomatous chronic otitis media (COM).

Methods

Preoperative bone conduction (BC) hearing thresholds of 266 patients (304 ears) with COM were compared with those of 342 normal individuals (440 ears) by audiometry. The incidence of abnormal BC threshold at lower frequencies (250–1000 Hz) and at higher frequencies (2000–4000 Hz) were examined and the differences between control and COM groups were compared by using χ2 test. In the COM group, the cross-sectional area of the mastoid air cells based on the axial CT image (n = 255) were correlated with the results of BC threshold.

Results

The percentage in the COM group exceeds 15% in their 50s at lower frequencies while in their 40s at higher frequencies. The BC thresholds were significantly better in the group with normal mastoid area than in those with smaller mastoid area at each decadal age group. The BC impairment from COM becomes worse as the course of the disease progresses and deteriorated from 40s rapidly, especially at higher frequencies and in the group with smaller mastoid area.

Conclusion

These results recommend that early treatment, including surgery, should be considered as early as possible before BC impairment occurs, especially for COM cases with smaller mastoid area, which may indicate the existence of more severe otitis media since earlier childhood.     

Is sensorineural hearing loss with chronic otitis media due to infection or aging in older patients?

Objective

To clarify true incidence of sensorineural hearing loss in ears with chronic otitis media (COM).

Methods

Bone conduction (BC) hearing thresholds of 180 preoperative patients (207 ears) with COM and 226 normal individuals (289 ears) were measured by audiometry, and the percentage of ears with BC thresholds being higher than normal range was evaluated in the COM group. In the COM group, the size of the perforation on the eardrum (n = 196) and the cross-sectional area of the mastoid air cells based on the axial CT image (n = 103) were also measured and correlated with the results of BC threshold.

Results

The percentage of ears with BC thresholds being higher than normal range calculated from comparison to the control group tended to increase with age, ranging from 4.5% in the 20s to 34.1% in the 60s with an average of 26.6%. The increase in the BC thresholds did not correlate with the size of eardrum perforation, but correlated well with the size of the mastoid air cells.

Conclusion

These results may suggest that all measures for early cure, including surgery, should be considered as early as possible for patients with COM.     

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.     

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.     

Tuning characteristics of ocular and cervical vestibular evoked myogenic potentials in intact and dehiscent ears

Cervical and ocular vestibular evoked myogenic potentials (cVEMPs and oVEMPs) to air-conducted tone bursts (250-2000 Hz) were recorded in 14 patients with superior canal dehiscence (SCD) and 32 healthy controls. For cVEMPs, the most common 'optimal frequency' in control ears (48.2%) was 500 Hz; for oVEMPs, it was 1000 Hz (51.8%). We found a significant interaction between age and frequency, with a shift towards higher-frequency tuning in older subjects. cVEMP and oVEMP tuning in SCD was characterised by a broadening of amplitude and threshold tuning curves. The tendency of cVEMPs to tune to lower frequencies compared to oVEMP was enhanced in SCD. Differences in cVEMP and oVEMP 'optimal frequencies', demonstrated in 57.1% intact ears and 81.3% dehiscent ears, imply differences in the recruitment of hair cells generating these two reflexes. Age-matched oVEMP amplitudes provided excellent separation between SCD and control ears. Although cVEMP amplitudes overlapped between SCD and control ears, better separation was achieved by using a 2-kHz stimulus.