Tullio现象与Ramsay Hunt综合征的前庭诱发的肌源性电位

目的应用前庭诱发的肌源性电位(vestibular evoked myogenic potentials,VEMPs),观察Tullio现象与Ramsay Hunt综合征的电位特点,为诊断提供客观依据.方法Tullio现象与Ramsay Hunt综合征各1例,通过已经建立的VEMPs检查方法观察两者的电位引出情况.结果正常人VEMP刺激声在阈上85dB nHL可引出VEMPs.本文Tullio现象患者在刺激强度降至39dB nHL时仍可引出.而健侧在69dB nHL时就已不能引出.Ramsay Hunt综合征在阈上105dBHL右侧不能引出VEMP;而左侧可以引出.结论VEMPs可用来了解前庭下神经的功能状态.由于VEMPs的检查特点,可用于动态观察前庭神经病变后的恢复情况.     

3D-FLAIR MRI findings in a patient with Ramsay Hunt syndrome

Three-dimensional, fluid-attenuated inversion recovery (3D-FLAIR) of magnetic resonance imaging (MRI) has recently been developed to detect hemorrhage or high concentrations of protein. We present a patient with Ramsay Hunt syndrome, in whom high signals in the cochlear and vestibular apparatus were identified with 3D-FLAIR. The high signal areas in 3D-FLAIR were not detected by T1- and T2-weighted MRI in this case. This is the first report of high concentrations of protein in the inner ear in Ramsay Hunt syndrome using 3D-FLAIR, and suggests that high concentrations of protein in the inner ear are associated with hearing deterioration in some patients with Ramsay Hunt syndrome. 3D-FLAIR could be a useful diagnostic tool in the early stages of Ramsay Hunt syndrome.     

Contrast-enhanced magnetic resonance imaging of facial nerve swelling in patients with severe Ramsay Hunt syndrome

ObjectiveIn Ramsay Hunt syndrome, contrast enhancement of magnetic resonance (MR) imaging seen in the affected facial nerve in the majority of cases, but its clinical significance has not been well investigated to date. The aim of this study was to elucidate the clinical significance of this imaging sign by quantitively investigating the correlation between the signal increase and swelling of the facial nerve. We also investigated the temporal change in this sign and its correlation with recovery.MethodsWe retrospectively evaluated swelling of the facial nerve in 16 patients with severe Ramsay Hunt syndrome who underwent both contrast-enhanced magnetic resonance imaging and facial nerve decompression surgery via a transmastoid approach alone or in combination with a middle cranial approach. All the patients had a Yanagihara score of ≤8 points and facial nerve degeneration of ≥90% confirmed by either a nerve excitability test or electroneurography. Swelling of the facial nerve was evaluated intraoperatively using a 4-point grading system.ResultsA significant correlation was observed between contrast enhancement on T1-weighted images and facial nerve swelling in the labyrinthine segment, geniculate ganglion, and pyramidal segment (P = 0.030, P = 0.018, and P = 0.037, respectively). Furthermore, the contrast enhancement increased significantly as more time elapsed after the onset of facial palsy (mean ± standard error, 14.7 ± 2.3 days, range, 5–42 days) in the geniculate ganglion and pyramidal segment (correlation coefficient, 0.546 and 0.689, P = 0.022 and P = 0.002, respectively). Patients with good recovery (Yanagihara score of ≥36) showed significantly lower contrast enhancement in the tympanic and mastoid segments (P = 0.021 and 0.020, respectively) than those who with poor recovery.ConclusionIn particular segments of the facial nerve, contrast enhancement on T1-weighted image correlated with facial nerve swelling and recovery. These observations underscore the clinical significance of contrast enhancement on T1-weighted images in patients with Ramsay Hunt syndrome.     

Swelling of the intratemporal facial nerve in Ramsay Hunt syndrome

Although Ramsay Hunt syndrome is one of the most important diseases causing peripheral facial palsy, the detailed pathology of the disease in the intratemporal facial nerve remains unclear. The purpose of this study was to increase knowledge of the pathogenesis of the syndrome by means of surgical findings. Between April 1976 and March 1997 we performed subtotal decompression of the facial nerve in 74 patients with severe Ramsay Hunt syndrome. The grade of nerve swelling was assessed using a microscope and recorded in a standardized form. The relationships between nerve swelling, the timing of surgery and the swelling of each segment were analyzed. Pronounced neural swelling, involving the geniculate ganglion and the horizontal segment, was consistent finding in the acute phase. Although the incidence of pronounced swelling of the horizontal segment gradually declined with time after onset, in most cases nerve swelling persisted even beyond the 16th week after onset. These data suggest that diffuse viral neuritis occurs throughout the intratemporal facial nerve. We assume that the viral inflammatory swelling involving the geniculate ganglion and horizontal segment is mostly responsible for the acute facial palsy in the acute phase.     

耳带状疱疹10例临床分析

报道10例耳带状疱疹综合征（RamsayHuntsyndrome）病人临床资料,就其临床表现,治疗方法及疗程疗效进行分析。疱疾病毒常侵犯面神经,也可侵犯第Ⅴ、Ⅷ、Ⅸ、Ⅹ等颅神经,从而产生不同的疤疹征候群。耳带状疱疹引起面瘫,三叉神经节炎的面部疱疹和颈神经炎的颈部疱疹也可引起面瘫。本组病例经抗病毒药剂及神经营养药物治疗,预后较好,面神经恢复满意者5例,较好2例,差者1例。患者较长时间疱疹区剧烈疼痛是值得今后研究的问题。     

Acyclovir improves recovery rate of facial nerve palsy in Ramsay Hunt syndrome

OBJECTIVE: Although the antiviral agent, acyclovir, is currently employed for the treatment in Ramsay Hunt syndrome, the benefit of acyclovir on facial nerve is still unknown and remains controversial. This study was designed to evaluate the effect of acyclovir in facial nerve recovery in Ramsay Hunt syndrome. METHODS: To evaluate drug effect on facial nerve function, evaluation of the facial voluntary movement and nerve excitability testing were performed. We have used an infusion therapy of acyclovir in combination with a high dose of steroid (AS), which was started within 7 days of onset of facial nerve palsy in 91 patients with Ramsay Hunt syndrome. The results were compared with those of 47 patients whose therapy was steroid alone started within 7 days of onset. RESULTS: Out of 91 patients treated with AS, nerve exitability was good in 68 (75%), while it was poor in 17 and absent in six. Of 47 patients treated with steroid alone, nerve exitability was good in 25 (53%), while it was poor in 11 and absent in 11. There was statistically significant difference between AS and steroid therapy in the posttreatment degree of nerve function. Complete recovery to grade I in facial voluntary movement was attained in 82 of 91 patients (90%) in the AS therapy, while out of 47 patients treated with steroid alone complete recovery to grade I was attained in only 30 (64%). A statistically significant difference in the recovery rate of facial nerve function was induced between AS and steroid therapy. CONCLUSION: The AS therapy was proved to keep good degree of nerve function indicated with nerve excitability testing and improve recovery rate of facial nerve in Ramsay Hunt syndrome. Based on this study, we now believe that the AS therapy is an advisable treatment modality to improve the recovery rate of facial nerve function in Ramsay Hunt syndrome.     

28例Ramsay Hunt综合征病例临床分析

目的 :探讨 Ramsay Hunt综合征的早期诊断、合理治疗及预后情况。方法 :回顾性分析 1990年 1月～ 2 0 0 1年5月收治的 2 8例 Ramsay Hunt综合征的临床资料 ,全部应用糖皮质激素及抗病毒剂治疗 ,其中面瘫患者全部应用血管扩张剂 ,2例行面神经减压术。结果 :2 8例中出现面瘫 2 5例 ,保守治疗 16例治愈 ,占 6 4 % ;好转 6例 ,占 2 4 % ;无效 3例 ,占 12 % (其中 2例经面神经减压好转 )。伴听力下降者 13例 ,5例轻度聋完全恢复 ;5例中度聋 ,3例恢复 ,2例好转 ;3例重度聋仅 1例听力提高 2 0 d BHL。 3例后遗神经痛。结论 :应注意临床上少见类型的 Ramsay Hunt综合征 ,减少误诊 ,尽早给予合理治疗 ,提高治愈率     

Electrocochleography and brain-stem potentials in Ramsay Hunt syndrome

Audiometric investigations and electrophysiologic recordings of cochlear and brain-stem auditory evoked potentials (BAEPs) were performed in 13 patients to elucidate further the type of hearing disorders in Ramsay Hunt syndrome. Transtympanic electrocochleography showed no enhancement of summating potential and did not suggest secondary endolymphatic hydrops. The recording of BAEPs was clearly abnormal in several of the 13 patients. The striking feature of the abnormalities in these patients was the prolongation of the latencies of waves III and V with the preservation of wave I, which clearly suggests retrocochlear involvement. In all the patients tested, abnormalities of the BAEPs were present only on the affected side. It is possible, on the basis of BAEP findings, to suggest that in Ramsay Hunt syndrome both cochlear and retrocochlear involvement may occur.     

Varicella-zoster virus load and cochleovestibular symptoms in Ramsay Hunt syndrome

OBJECTIVES: The mechanism by which varicella-zoster virus (VZV) reactivation causes cochleovestibular symptoms (CVSs) in patients with Ramsay Hunt syndrome (RHS) remains to be elucidated. The present study analyzed the relationship between VZV load and the onset of CVSs in RHS. METHODS: The subjects consisted of 56 patients with RHS; 29 exhibited CVSs and facial paralysis (FP; group 1), and 27 exhibited FP without CVSs (group 2). The VZV DNA copy number in the saliva was measured with a quantitative polymerase chain reaction. Anti-VZV antibodies were assayed by an enzyme-linked immunosorbent assay with paired sera. RESULTS: There was no significant difference in maximum viral copy number between the two groups. In group 1, CVSs occurred at various times between the early phase and the regression phase of VZV reactivation. In some patients, CVSs occurred in the early phase of VZV reactivation, before the onset of zoster lesions and FP. CONCLUSIONS: There are various different patterns in the development of eighth cranial nerve dysfunction, which is caused by progression of neuritis or labyrinthitis following VZV reactivation. Our data suggest that CVSs in RHS may also be caused by reactivation of VZV in the spiral and/or vestibular ganglia.     

Detection of varicella-zoster virus DNA in tear fluid and saliva of patients with Ramsay Hunt syndrome

Ramsay Hunt syndrome develops when the varicella-zoster virus (VZV) is reactivated. In the present study, we examined the secretion kinetics of VZV DNA in the tear fluid, submandibular gland saliva and parotid gland saliva of 15 patients with Ramsay Hunt syndrome. The presence of VZV DNA was detected using PCR and a microplate hybridization method. Hybridization signals were measured using the fluorescence density of an enzymatic reaction product using fluoroscan and a system involving streptavidin-conjugated beta-galactosidase. The results were converted into numerical values and used to estimate the number of virus DNA copies. VZV DNA was detected in the tear fluid, submandibular gland saliva and parotid gland saliva of the Ramsay Hunt syndrome patients. The rate of VZV DNA detection in the submandibular gland saliva was 72%, and the detection rate in the parotid gland saliva was 57%. The detection rate in the tear fluid was 27%, which is significantly lower than other two detection rates. Regarding the submandibular gland saliva and the parotid gland saliva, the VZV DNA was detected in samples collected at a comparatively early stage of onset. In the tear fluid, the detection rate increased significantly in samples collected 2 weeks after onset or later. Thus, differences in the detection rate were observed depending on the type of secretory gland and the timing of the sample collection. The VZV DNA in the tear fluid is thought to derive from the ganglion trigeminale. The increase and decrease in the number of VZV DNA copies detected in samples collected at different times is considered to substantiate VZV reactivation in Ramsay Hunt syndrome.     

Analysis of prognostic factors in Bell's palsy and Ramsay Hunt syndrome

OBJECTIVE: This study evaluated the prognostic factors in Bell's palsy and Ramsay Hunt syndrome (HZO). METHODS: A retrospective, institutional review board-approved study at a university-based hospital. A total of 81 patients consisting of 55 Bell's palsy patients and 26 HZO patients were enrolled in this study. The treatment consisted uniformly in all cases, and acyclovir was administered in the case of Ramsay Hunt syndrome. All patients were followed up until they recovered or for least up 6 months. RESULTS: The recovery rates to House-Brackmann grade II or better were 96.3% in those with Bell's palsy and 84.6% in those with HZO. In the HZO cases, older patients had a poorer initial and final status, and less chance of making a complete recovery than the younger patients. The HZO patients without diabetes mellitus had a higher chance of recovery, a higher chance of complete recovery, and a better final status. In addition, HZO patients without essential hypertension had a higher degree of recovery. HZO patients not suffering from vertigo had a higher chance of recovery. CONCLUSION: There was no prognostic factor found in the Bell's palsy patients in this study. The prognostic factors of HZO were age, diabetus mellitus, essential hypertension and vertigo.     

Varicella-zoster virus DNA level and facial paralysis in Ramsay Hunt syndrome

We have investigated whether the copy number of varicella-zoster virus (VZV) in saliva correlates with the clinical symptoms in patients with Ramsay Hunt syndrome. A real-time quantitative polymerase chain reaction assay was used to examine the VZV DNA copy number in saliva samples from 37 patients. We detected VZV DNA in 6 of the 7 patients with oropharyngeal zoster lesions (86%) and in 17 of the 30 patients who had zoster lesions only on the skin (57%). Patients with oropharyngeal zoster lesions had a high VZV load in their saliva, and the difference between the copy number in patients with oropharyngeal zoster lesions and those without was around 10,000 copies per 50 microL. In addition, patients with oropharyngeal zoster lesions showed worse recovery of facial function than those without. It seems that the VZV DNA level in saliva reflects the kinetics of viral reactivation in the facial nerve, as well as in the oropharyngeal epithelium, in patients with Ramsay Hunt syndrome.     

Vestibular nerve section in patients with chronic otitis media

Retrolabyrinthine vestibular neurectomy preserves hearing and relieves intractable vertigo emanating from the inner ear. However, this approach must be modified in patients with prior "canal-wall-down" procedures. Traversing an exteriorized mastoid cavity risks bacterial contamination of the subarachnoid space. Three patients seen at the Otologic Medical Group with prior canal-wall-down procedures required vestibular neurectomy for persistent vertigo. Using the retrosigmoid approach, the vestibular nerve was sectioned without sacrificing hearing and without traversing a potentially infected mastoid cavity. It is recommended that this approach be considered in patients with intractable vertigo, serviceable hearing, and exteriorized mastoid cavities.     

Relationship between cochlear implant outcome and the diameter of the cochlear nerve depicted on MRI

This study aimed to evaluate the relationship between the diameters of the auditory and eighth cranial nerves and improvements in post-implant performance. Twenty prelingually deafened children (aged from 2.0 to 6.0 years) who received the Nucleus 24 cochlear implant participated in this study. All subjects had used their implant for at least 1 year after device connection. The diameters of cochlear and eighth cranial nerves were retrospectively measured on preoperative T2-weighted axial magnetic resonance image (MRI). In 17 of 20 subjects, the cochlear and eighth cranial nerves could be identified on MRI. The mean diameter of the cochlear and eighth cranial nerves were 0.9 +/- 0.2 mm and 1.2 +/- 0.3 mm, respectively. In the remaining three subjects, the cochlear and eighth cranial nerves could not be identified on MRI. These three subjects had significantly lower scores in the Infant-Toddler-Meaningful Auditory Integration Scale (IT-MAIS) than the other 17 subjects at 12 months post-implant. There was no significant correlation among the maximal diameters of the nerves and age, ECAP thresholds and IT-MAIS scores. A sufficient outcome from cochlear implantation can be expected when cochlear and eighth cranial nerves are depicted on MRI, regardless of the nerve diameters.     

Evaluation of cochlear nerve diameter and cross-sectional area in ANSD patients by 3.0-Tesla MRI

Conclusions: The size of cochlear nerve (CN) is atrophic in adult auditory neuropathy spectrum disorder (ANSD) patients compared with non-ANSD sensorineural hearing loss (SNHL) patients and normal hearing subjects, and CN deficiency is one of the lesions for ANSD patients. Objectives: To evaluate the dimensions of CN in adult ANSD patients on magnetic resonance imaging (MRI) and confirm the hypothesis that CN deficiency is one of the lesions for ANSD patients. Methods: Medical records and MRI of 24 adult ANSD patients reviewed retrospectively and 20 non-ANSD SNHL and 24 volunteers with normal hearing were recruited as control groups. The long diameter (LD), short diameter (SD), and cross-sectional area (CSA) of CN and facial nerve (FN) were measured. Results: Among the 24 ANSD patients, this study was able to reconstruct and measure the CN of 91.7% (22/24, total 43 ears) of patients and FN of 83.3% (20/24, total 38 ears) of patients. The mean values and standard deviations of LD, SD, and CSA of CN in ANSD patients were 0.65?±?0.20?mm, 0.44?±?0.15?mm, and 0.30?±?0.19?mm², respectively. They were significantly smaller in ANSD patients than in control groups (p?相似文献