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Objective: This study uses retrospective longitudinal data from a large unselected cohort of patients with peripheral facial paralysis to determine the prevalence and patient characteristic predictors of sequelae receiving intervention., Study Design: Retrospective case review., Setting: Karolinska University Hospital in Stockholm Sweden serves as the only tertiary facial palsy center in the region. Here, patients are diagnosed, are followed up, and undergo all major interventions., Patients: All adult patients presenting with peripheral facial palsy due to idiopathic, zoster, or Borrelia origin at Karolinska, January 1, 2010 to December 31, 2011 with follow-up until December 2022., Interventions: Patient charts were studied to identify patient characteristics, etiology, initial treatment, severity of palsy, and treatments targeting sequelae., Main Outcome Measures: Types of initial and late treatments were noted. Sunnybrook and/or House-Brackmann scales were used for palsy grading., Results: Five hundred twenty-five patients were included. Thirty-three patients (6.3%) received botulinum toxin injections and/or surgical treatment. In this subgroup, 67% received corticosteroids compared to 85% of all patients ( p = 0.005), cardiovascular disease prevalence was higher (23 and 42%, respectively, p = 0.009). For 81 patients (15%), follow-up was discontinued although the last measurement was Sunnybrook less than 70 or House-Brackmann 3 to 6., Conclusions: Of patients with peripheral facial palsy, 6.3% underwent injections and/or surgical treatment within 12 years. However, due to a rather large proportion not presenting for follow-up, this might be an underestimation. Patients receiving late injections and/or surgical treatment had more comorbidities and received corticosteroid treatment to a significantly lower extent in the acute phase of disease., Competing Interests: Conflicts of interest: No conflicts of interest are reported., (Copyright © 2024, Otology & Neurotology, Inc.)

Objective: The prognostic value of electroneurography (ENoG) for predicting the incidence of synkinesis is reportedly about 40 % using the formal standard method (ENoG-SM). However, the prognostic value of ENoG using the newly developed midline method (ENoG-MM) has not been determined. The aim of this study was to demonstrate the optimal prognostic value and advantages of ENoG-MM for predicting the incidence of synkinesis., Methods: Participants were 573 patients treated for peripheral facial palsy including Bell's palsy or Ramsay Hunt syndrome. We investigated the clinical presence of any oral-ocular or ocular-oral synkinesis from the medical records. ENoG-MM and ENoG-SM were performed 10-14 days after symptom onset. In ENoG-MM, compound muscle action potentials were recorded by placing the anode on the mental protuberance and the cathode on the philtrum. In ENoG-SM, electrodes were placed on the nasolabial fold. Synkinesis was clinically assessed at the end of follow-up or at >1 year after onset. The sensitivity and specificity of ENoG values for predicting the incidence of synkinesis were compared between ENoG-MM and ENoG-SM at every 5 % around 40 % (range, 30-50 %)., Results: At every 5 % of ENoG values around 40 %, ENoG-MM provided higher sensitivity and lower specificity for predicting the incidence of synkinesis compared with ENoG-SM. In particular, when the cut-off value was set at 45 %, sensitivity was 100 % and 95.3 % with ENoG-MM and ENoG-SM, respectively., Conclusion: In peripheral facial palsy, ENoG-MM offered higher sensitivity than ENoG-SM for predicting synkinesis. ENoG-MM is useful for screening patients at risk of developing synkinesis. In clinical practice, an ENoG-MM cut-off value of 45 % must be the optimal prognostic value because of the 100 % sensitivity., Competing Interests: Disclosure of interest The authors declare no conflicts of interest in association with the present study., (Copyright © 2024. Published by Elsevier B.V.)

Objective: This study aimed to reveal the efficacy of physical therapy for patients with peripheral facial palsy., Methods: A literature search was conducted using PubMed, Ichushi-Web, and Cochrane Central Register of Controlled Trials. Published randomized controlled trials comparing the physical therapy versus placebo/non-treatment for peripheral facial palsy such as Bell's palsy, Ramsay Hunt syndrome, and traumatic facial palsy were included for meta-analysis. The primary outcome was non-recovery at the end of the follow-up. Non-recovery was defined according to the authors' definition. The secondary outcomes were the composite score of the Sunnybrook facial grading system and sequelae (presence of synkinesis or hemifacial spasm) at the end of the follow-up. Data was analyzed using Review Manager software and pooled risk ratio (RR) or mean difference (MD) with 95% confidence intervals (CI) were calculated., Results: Seven randomized controlled trials met the eligible criteria. The data on non-recovery from four studies was obtained and included 418 participants in the meta-analysis. Physical therapy might reduce non-recovery (RR = 0.51 [95% CI = 0.31-0.83], low quality). Pooling the data of composite score of the Sunnybrook facial grading system from three studies (166 participants) revealed that physical therapy might increase the composite scores (MD = 12.1 [95% CI = 3.11-21.0], low quality). Moreover, we obtained data on sequelae from two articles (179 participants). The evidence was very uncertain about the effect of physical therapy on reduction of sequelae (RR = 0.64 [95% CI = 0.07-5.95], very low quality)., Conclusion: The evidence suggested that physical therapy reduces non-recovery in patients with peripheral facial palsy and improves the composite score of the Sunnybrook facial grading system, whereas the efficacy of physical therapy in reducing sequelae remained uncertain. The included studies had high risk of bias, imprecision, or inconsistency; therefore, the certainty of evidence was low or very low. Further well-designed randomized controlled trials are needed to confirm its efficacy., Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interest in association with the present study., (Copyright © 2023. Published by Elsevier B.V.)

Introduction: Acute facial palsy, characterized by sudden hemifacial weakness, significantly impacts an individual's quality of life. Despite several predisposing factors identified for acute facial palsy, the specific relationship between diabetes mellitus (DM) and acute facial palsy has not been comprehensively explored in recent studies. The aim of the study was to assess the risk of acute facial palsy in patients with DM using a nationwide population sample cohort., Methods: DM cohort and non-DM cohort were built using the Korean National Health Insurance Service-Sample Cohort which represents the entire population of the Republic of Korea from January 2002 to December 2019. The DM cohort comprised 92,872 patients with a record of medication and a diagnosis of DM. Individuals who had facial palsy before the diagnosis of DM were excluded. A comparison cohort comprised 1,012,021 individuals without DM matched sociodemographically in a 1:4 ratio. The incidence of Bell's palsy (BP) and Ramsay Hunt syndrome (RHS) were evaluated in both cohorts. The risk factors for acute facial palsy were also assessed., Results: Among the 92,868 patients in the DM cohort, the incidence rate (IR) of BP and RHS were 31.42 (confidence interval [CI], 30.24-32.63) and 4.58 per 10,000 person-years (CI, 4.14-5.05), respectively. Among the 371,392 individuals in the non-DM cohort, the IR of BP was 22.11 per 10,000 person-years (CI, 21.62-22.59) and the IR of RHS was 2.85 per 10,000 person-years (CI, 2.68-3.02). IR ratios for BP and RHS were 1.42 (CI, 1.36-1.48) and 1.61 (CI, 1.43-1.80). In multivariate analysis, DM (hazard ratio [HR] 1.428), age (HR 1.008), and high comorbidity score (HR 1.051) were associated with increased risk of BP, and male (HR 0.803) and living in metropolis (HR 0.966) decreased the risk of BP. And DM (HR 1.615), high comorbidity score (HR 1.078), and living in metropolis (HR 1.201) were associated with increased risk for RHS., Conclusion: This study suggests that patients with DM had an increased risk of acute facial palsy including BP and RHS., (© 2023 The Author(s). Published by S. Karger AG, Basel.)

Purpose: To observe vestibular impairment patterns in patients with Ramsay Hunt syndrome with dizziness (RHS_D) and sudden sensorineural hearing loss with dizziness (SSNHL_D) using hierarchical cluster analysis (HCA) to interpret results with possible mechanisms., Methods: The data of 30 RHS_D and 81 SSNHL_D patients from January 2017 to August 2022 in a single tertiary referral center were retrospectively analyzed. Video head impulse test (vHIT) and vestibular evoked myogenic potential (VEMP) were used for vestibular analysis of peripheral vestibular organs, and the results of vHIT and VEMP were analyzed. HCA was used to analyze vestibular impairment patterns., Results: In RHS_D patients, the lateral semicircular canal (LSCC) was the most impaired semicircular canal (SCC), followed by the anterior semicircular canal (ASCC) and the posterior semicircular canal (PSCC), and the utricle was more impaired than the saccule. In SSNHL_D patients, the PSCC was the most impaired SCC, followed by the LSCC and the ASCC, and the utricle was more impaired than the saccule. In HCA of RHS_D patients, the ASCC and utricle were initially clustered, followed by the LSCC, PSCC and saccule in order. In the HCA of SSNHL_D patients, the PSCC was solely merged and independently clustered., Conclusion: There were different patterns of vestibular impairments between RHS_D and SSNHL_D patients. The vestibular analysis and HCA results of SSNHL_D showed tendency of skip lesion, which could be explained by vascular pathophysiology., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Ramsay Hunt syndrome (RHS) is a manifestation of reactivated varicella-zoster virus (VZV) from the geniculate ganglion. Data on clinical features and outcomes of patients with RHS and concurrent VZV meningitis (henceforth RHS meningitis) are limited. Thus, we conducted a nationwide population-based cohort study of all adults hospitalized for RHS meningitis at the departments of infectious diseases in Denmark from 2015 to 2020. Patients with VZV meningitis without cranial nerve palsies were included for comparison. In total, 37 patients with RHS meningitis (mean annual incidence: 1.6/1 000 000 adults) and 162 with VZV meningitis without cranial nerve palsies were included. In RHS meningitis, the median age was 52 years (interquartile range: 35-64), and in addition to peripheral facial nerve palsy (100%), dizziness (46%), and hearing loss (35%) were common symptoms. The triad of headache, neck stiffness, and photophobia/hyperacusis was less common in RHS meningitis than in VZV meningitis without cranial nerve palsies (0/27 [0%] vs. 24/143 [17%]; p = 0.02). At 30 days after discharge, 18/36 (50%) patients with RHS meningitis had persistent peripheral facial nerve palsy, with no statistically significant difference between those treated with and without adjuvant glucocorticoids (6/16 [38%] vs. 12/20 [60%]; p = 0.18). Additional sequelae of RHS meningitis included dizziness (29%), neuralgia (14%), tinnitus/hyperacusis (11%), hearing loss (9%), headache (9%), fatigue (6%), and concentration difficulties (3%). In conclusion, clinical features and outcomes of RHS meningitis were primarily related to cranial neuropathies., (© 2023 Wiley Periodicals LLC.)

Background: Several studies point out the association of facial palsy with psychiatric distress such as depression and anxiety. However, there have been few studies on this association based on population-based data., Objectives: To investigate the risk for depression and anxiety disorders in patients with Bell's palsy and in those with Ramsay Hunt syndrome., Materials and Methods: This retrospective study included data from the Korean National Health Insurance Service National Sample Cohort. From 2006 to 2015, patients with Bell's palsy and those with Ramsay Hunt syndrome were defined using diagnostic and medication claim codes. Patients with depression or anxiety were defined using diagnostic and medication claim codes. Occurrences of depression and anxiety were included in analyses., Results: There were significantly higher risks for depressive and anxiety disorders in the group of patients with Bell's palsy than in the control group, as well as in the group of patients with Ramsay Hunt syndrome than in the control group., Conclusions and Significance: There were significantly higher risks for depression and anxiety in patients with facial palsy including Bell's palsy and Ramsay Hunt syndrome based on population-based data from Korea.

Ramsay Hunt syndrome is due to reactivation of varicella zoster virus (VZV) dormant in the geniculate ganglion of the facial nerve. The diagnosis is typically based on clinical triad of ipsilateral facial paralysis, otalgia, and vesicles in the auditory canal or the auricle. However, Ramsay Hunt syndrome may occur without skin eruption in up to one third of patients. Moreover, the involvement of other cranial nerves in addition to the facial nerve has been also reported. Herein, we reported a case report of a man who developed a multiple cranial neuropathy caused by VZV reactivation without skin vesicular eruption. The present case underlines a possible diagnostic challenge that clinicians may hit when facing a common disorder such as peripheral facial palsy. Indeed, clinicians must be aware that Ramsay Hunt syndrome may develop without skin vesicular eruption as well it may be complicated by multiple cranial nerve involvement. Antiviral therapy is effective in VZV reactivation for recovery of nerve function., (© 2023. The Author(s).)

Objective: Recurrent facial palsy is relatively rare and its clinical details of recurrent facial palsy are not well known. We analyzed recurrent facial palsy cases and clarified its characteristics, especially the difference between ipsilateral and alternative palsies. The analysis aimed to obtain information about recurrent facial palsy that would be useful for delivering explanations to patients and help improve recurrent facial palsy treatments based on the etiology., Methods: We picked up data from the chart and analyzed the clinical characteristics of patients with recurrent facial palsy from 1243 facial palsy patients (Bell's palsy, VZV-related palsy (Ramsay Hunt syndrome and zoster sine herpete [ZSH])) between 2006 and 2020., Results: Recurrent facial palsy was observed in 104 of 1243 patients (8.4%). There were 35 cases (34%) of ipsilateral palsy and 69 cases (66%) of alternative palsy. The mean age at the onset of the first palsy was 38.9 years old in the ipsilateral group and 48.4 years old in the alternative group, and a significant difference was observed between them. The number of recurrences ranged from 1 to 4. Among the ipsilateral group, 6 patients experienced more than second recurrence. In two cases, the condition failed to resolve after the second recurrence. A serological examination confirmed that 4 cases had recurrent VZV-related palsy (both the first and second palsies were VZV-related) and all of them initially had ZSH: no cases had Hunt syndrome as the first palsy., Conclusions: The VZV-specific immunity obtained with ZSH might be insufficient to suppress VZV reactivation, and VZV vaccination should be recommended for ZSH patients to prevent further recurrence of VZV-related facial palsy. More than 2 ipsilateral recurrent episodes may be a risk factor for incomplete recovery., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to declare., (Copyright © 2022 Japanese Society of Otorhinolaryngology-Head and Neck Surgery, Inc. Published by Elsevier B.V. All rights reserved.)

Background: Ramsay-Hunt syndrome (RHS) due to varicella zoster virus (VZV) infection is commonly reported in individuals aged at least 50 years or immunocompromised individuals. VZV infection may invade the central nervous system (CNS) and cause meningitis or encephalitis, which are more likely to occur in patients with chronic diseases such as diabetes and chronic renal failure. However, cases with VZV-induced concurrent RHS and CNS infections are rare., Case Presentation: Two young male patients, aged 32 and 43 years, with no underlying disease developed VZV meningitis, followed by RHS involving cranial nerves VII and VIII. Both patients presented with symptoms of peripheral facial palsy, and dizziness accompanied by tinnitus and hearing loss, which appeared several days after the onset of fever and headache. These symptoms were documented as facial neuropathy and sensorineural hearing loss in the electrophysiologic studies. Lymphocyte-dominant pleocytosis and VZV positivity were confirmed from cerebrospinal fluid examination and polymerase chain reaction, respectively. The patients were treated with intravenous acyclovir and oral steroids simultaneously. Following the treatment completion, both patients were relieved of their headaches and fever; however, facial palsy, dizziness, and tinnitus persisted. They were followed up at the outpatient clinic., Conclusion: These cases confirmed that RHS and CNS infections can co-exist even in young adults with normal immune function and more importantly, that CNS infection can precede RHS. Since early detection and treatment of RHS improve the prognosis, it is critical to closely monitor patients with VZV meningitis or encephalitis considering the possible superimposition of RHS., (© 2023. The Author(s).)

Background: Fear of falling (FOF) is a common and major health concern in older adults. The consequences of FOF include reduced physical performance, social activity, and health-related quality of life. Ramsay Hunt syndrome (RHS) is a herpes zoster-related facial nerve dysfunction accompanied by an erythematous vesicular rash on the ear or mouth that may complicate ipsilateral facial paralysis and otalgia, vertigo, tinnitus, hearing loss, and meningoencephalitis. However, repeated falls and subsequent FOF due to RHS have not been reported in older adults., Case Presentation: A 65-year-old woman diagnosed with RHS experienced repeated falls during hospital admission and after discharge. Despite recovery of balance and no subsequent falls, the patient presented with persistent FOF at the geriatric outpatient follow-up visit 1 year after the RHS episode. The fear sensation impaired the patient's instrumental daily activities and was confirmed by documentation of serial comprehensive geriatric assessments, especially the Timed Up and Go test scores., Conclusions: RHS may cause repeated falls and FOF, leading to impairment in daily activities and psychosocial function in older adults. Therefore, clinicians should be mindful of falls and FOF when caring for older patients with RHS and should develop multidimensional strategies for fall prevention and FOF., (© 2022. The Author(s).)

Objective: Acute facial palsy is a consequence of various diseases, with the number of patients increasing with advancing age. This study aimed to analyse the clinical characteristics of acute peripheral facial palsy in older adults., Methods: A total of 30 patients with a mean age of 68.4 ± 9.1 years were included in the study. All patients received a standardised investigation and follow up. The hospital charts of the patients with acute facial palsy were reviewed retrospectively., Results: The predominant causes of acute facial palsy in older adults were: Bell's palsy, Ramsay Hunt syndrome, trauma, otitis media and malignancy. At baseline, complete and incomplete facial palsies were seen in 26.7 per cent and 73.3 per cent of patients, respectively. The overall rates of good recovery, partial recovery and no recovery were 66.7 per cent, 10 per cent and 23.3 per cent, respectively. Increased age led to a significantly lower level of recovery in older adults., Conclusion: Bell's palsy and Ramsay Hunt syndrome were the most common aetiologies of acute facial palsy in older adults, and such patients are likely to have incomplete recovery. Active early treatment is necessary for achieving good outcomes in older adults.

Objective: Few large-scale investigations have been conducted on treatment of House-Brackmann grade VI (HB grade VI) Ramsay Hunt syndrome (RHS) patients. We compared recovery rates among patients receiving a normal-dose corticosteroid (prednisolone [PSL] 60 mg/d) or high-dose corticosteroid (PSL 200 mg/d), both with or without an antiviral agents. Recovery rates were also examined based on the order of presentation of herpetic vesicles versus facial palsy., Study Design: Retrospective case review., Setting: Tertiary referral center., Patients: A total of 128 patients with HB grade VI RHS were treated in our department between 1995 and 2017. These patients were divided into four treatment groups based on corticosteroid dosage and use of an antiviral agent., Methods: We assessed treatment outcomes for HB grade VI patients together with logistic regression analysis to investigate factors that can impact treatment outcomes, that is, sex, age, days to start of treatment, PSL dosage, and antiviral agent administration., Results: Recovery rates were best in the high-dose corticosteroid group with an antiviral agent (71.1%) in comparison with the normal-dose corticosteroid group with an antiviral agent (60.0%) or high-dose corticosteroid alone (57.1%). Significant factors for treatment outcomes were high-dose corticosteroid administration and early initiation of treatment. A better recovery rate was also found when the herpetic vesicles appeared before facial palsy., Conclusion: We showed that a combination of a high-dose corticosteroid and antiviral agent produced the best outcomes for patients with HB grade VI RHS. However, our results were not statistically significant because of small sample size., Competing Interests: The authors have no other funding, financial relationships, or conflicts of interest to disclose., (Copyright © 2022, Otology & Neurotology, Inc.)

Background: Ramsay Hunt Syndrome (RHS) is a neurotological disorder involving the reactivation of the varicella-zoster virus (VZV) in the geniculate ganglion of the facial nerve (Sweeney and Gilden in J Neurol Neurosurg Psychiatry 71:149-154, 2001). The characteristic presentation involves ipsilateral lower motor neuron type facial paresis, auricular pain with or without hearing impairment, and vesicular lesions of the external auditory canal and outer ear. Involvement of the facial and vestibulocochlear nerve is typical in RHS, whilst multiple cranial neuropathies are rare and associated with poorer prognosis and systemic complications (Arya et al. in Am J Case Rep 19:68-71, 2017; Shinha and Krishna in IDCases 2:47-48, 2015; Shim et al. in Acta Otolaryngol 131:210-215, 2011; Coleman et al. in J Voice 26:e27-e28, 2012; Morelli et al. in Neurol Sci 29:497-498, 2008;). Likely mechanisms involved in the pathogenesis of cranial polyneuropathy include direct peri-neural and trans-axonal spread of viral inflammation between contiguous cranial nerves and haematogenous dissemination between nerves with shared blood supply. Impairments in speech, swallowing, hearing, and oculo-protection can contribute to morbidity and requires a multidisciplinary approach to patient care., Methods: We present a rare case of RHS with multiple cranial neuropathies followed by a comprehensive review of current literature with regard to the pathophysiology, diagnostic workup, and the management strategies employed in these patients., Conclusion: RHSs with multiple cranial neuropathies are important to recognise as they are associated with significant morbidity and poor prognosis. A multidisciplinary approach to patient management is required to address the several complications that can arise from cranial nerve deficits, especially in regard to speech and swallow., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Rationale: Ramsay Hunt syndrome is a type of herpes zoster infection involving geniculate ganglion and facial nerve. Unilateral facial palsy, otalgia, and painful vesicular rash on the auricle and external auditory canal are the typical symptoms. Although postherpetic neuralgia (PHN) is a devastating complication of herpes zoster infection, PHN following Ramsay Hunt syndrome has rarely been reported., Patient Concerns: A 55-year-old immunocompetent female patient visited our pain clinic, for left-sided refractory otalgia (PHN) that persisted for 3 months after she was diagnosed with Ramsay Hunt syndrome. Although facial palsy and tinnitus had recovered within 2 to 4 weeks after symptom onset, the patient had been experiencing a persistent and severe otalgia radiating to mandibular angle, temporal and upper cervical area of neuropathic nature., Diagnoses: The patient's pain persisted despite conservative medication and administration of ultrasound-guided stellate ganglion block, facial nerve block, and great auricular nerve block several times., Interventions: The patient was treated with the application of ultrasound-guided pulsed radiofrequency (PRF) to the great auricular nerve., Outcomes: The patient experienced significant pain reduction more than 50% on a numeric rating scale after 2 weeks of PRF treatment., Lessons: Chronic otalgia might be a type of PHN after Ramsay Hunt syndrome with cervical nerve involvement. PRF treatment to the great auricular nerve can be a therapeutic option for refractory otalgia following Ramsay Hunt syndrome., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Objective: The 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)., Methods: Nineteen 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., Results: Among 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., Conclusion: In 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., Competing Interests: Declaration of Competing Interest The authors declare that they have no conflict of interest., (Copyright © 2020. Published by Elsevier B.V.)

A 63-year-old Japanese female in an immunocompetent state developed right Ramsay Hunt syndrome and left shoulder pain, and left upper limb motor paresis with herpes zoster (HZ) duplex in the right auricle and left shoulder regions. With her Ramsay Hunt syndrome, neural deafness, tinnitus and vestibular symptoms were observed, and she lacked facial nerve palsy. Cerebrospinal fluid (CSF) findings revealed an increase in lymphocytes (21 cells/μl) and protein content (29 mg/dl), and polymerase chain reaction for varicella-zoster virus DNA in CSF was negative. Cervical root MRI using 3D Nerve VIEW (Philips) imaging showed high-intensity lesions on the C5-C8 spinal roots with contrast enhancements. No abnormalities were observed in the median or ulnar motor sensory nerve conduction velocity conduction studies including the F wave. PubMed search revealed no report of a patient with this profile, and to the best of our knowledge HZ duplex with concomitant neurological impairments has not been reported. We compare our present case with several similar cases from the literature.

Background: Ramsay Hunt syndrome (RHS) is caused by a reactivation of varicella-zoster virus (VZV) infection, and it is characterized by the symptoms of facial paralysis, otalgia, auricular rash, and/or an oral lesion. Elderly patients or immunocompromised patients, deep pain at the initial visit and no prompt treatment are significant predictors of postherpetic neuralgia (PHN). When PHN occurs, especially involved cranial polyneuropathy, multiple modalities should be administered for patients with the intractable PHN. The use of thermography in the follow-up of PHN secondary to RHS with multicranial nerve involvement has not yet been described yet in the literature., Case Presentation: The patient was a 78-year-old man with the chief complaint of a 3-month history of PHN secondary to RHS with polycranial nerve (V, VII, VIII, and IX) involvement. Multimodality therapy with oral gabapentin, pulsed radiofrequency (PRF) application to the Gasserian ganglion for pain in the trigeminal nerve region, linear-polarized near-infrared light irradiation for pain in the facial nerve region, and 2% lidocaine spray for pain in the glossopharyngeal nerve region was used to the treat patient, and follow-up evaluations included thermography. This comprehensive treatment obviously improved the quality of life, resulting in considerable pain relief, as indicated by a decrease in the numerical rating scale (NRS) score from 9 to 3 and a decrease in thermal imaging temperature from higher to average temperature on the ipsilateral side compared with the contralateral side. Lidocaine spray on the tonsillar branches of the glossopharyngeal nerve resulted in an improvement in odynophagia, and the NRS score decreased from 9 to 0 for glossopharyngeal neuralgia after three applications., Conclusion: Although the use of thermography in the follow-up of RHS with multiple cranial nerve (V, VII, VIII, and IX) involvement is very rare, in this patient, thermal imaging showed the efficacy of combination therapy (oral gabapentin, 2% lidocaine sprayed, PRF application and linear-polarized near-infrared light irradiation) and that is a good option for treatment.

Background: The presence of dizziness has been reported as a negative prognostic factor for recovery of facial palsy in Ramsay Hunt syndrome (RHS). The aim of this study was to investigate the incidence and patterns of nystagmus in RHS patients without dizziness, and discuss possible mechanisms. We also compared the severity and prognosis of facial palsy between RHS patients with and without dizziness., Methods: From January 2014 to January 2019, 36 patients diagnosed with RHS (27 with dizziness and 9 without dizziness) were included. Patterns of nystagmus were examined and categorized using video-nystagmography. House-Brackmann(HB) grade of facial palsy was compared between RHS patients with and without dizziness., Results: Not only RHS patients with dizziness exhibited nystagmus in most cases (96%, 26 of 27) but also as many as 67% (6 of 9) of RHS patients without dizziness exhibited nystagmus, though the intensity was remarkably weak. In both groups of RHS with and without dizziness, direction-fixed nystagmus and direction-changing positional nystagmus were observed. Initial HB grade and recovery of facial palsy after treatment were not significantly different between RHS with and without dizziness., Conclusion: Various patterns of nystagmus including direction-fixed and positional direction-changing nystagmus were observed in RHS patients, and inflammation of the vestibular nerve and inner ear end organs may be responsible for the production of nystagmus in these patients. The results support that the evaluation of vestibular function may be necessary even in RHS patients who do not complain of dizziness or vertigo.

Introduction: Herpes zoster laryngitis (HZL) is a recently recognized rare disease, easily mistaken for common viral laryngopharyngitis. There are only a few case reports in the English literature. No study has evaluated the clinical characteristics of HZL. In this study, we analyzed the clinical characteristics of HZL and compared them to those of Ramsay Hunt syndrome (RHS)., Materials and Methods: Seventy-three patients who were initially diagnosed with HZL or RHS were enrolled in this study. Their medical records, including laryngoscopic findings, were analyzed retrospectively. The demographic factors, cranial nerve involvement, and recovery rate of both groups were evaluated., Results: Sixty patients in the non-HZL group and 13 patients in the HZL group were analyzed. Five more patients in the non-HZL group were newly identified with HZL during the retrospective chart review. The mean age of the patients in the HZL group was higher than that of the non-HZL group (p = 0.016). The prevalence of hypertension was higher in the HZL group (p = 0.012). Patients with multiple cranial nerve involvement were more common in the HZL group (p < 0.001). In addition, the prognosis of facial weakness (p = 0.002) and multisensory dizziness (p = 0.006) was poor in HZL group., Conclusion: This study showed that a considerable proportion of HZL cases were misdiagnosed or overlooked if not suspected. Considering the poor prognosis of HZL patients with facial paralysis and dizziness, HZL should be diagnosed earlier and treated properly.

Ramsay Hunt syndrome involving the vagus nerve is very rare. We herein describe a 53-year-old man who developed severe pharyngeal pain after alcohol intoxication. Antibiotic treatment was ineffective. Laryngoscopy showed an ulcer on the right side of the epiglottis. As the condition progressed, the patient developed hoarseness. He then gradually developed multiple herpes lesions on the right side of the head, face, and neck along with right peripheral facial paralysis. Corticosteroid, analgesic, antiviral, and nutritional nerve therapy resulted in disappearance of the herpes lesions, epiglottis ulcer, pharyngeal pain, and right head and face pain. The facial paralysis slightly improved, but the hoarseness did not improve. The patient was discharged with an unsatisfactory outcome, and he attempted treatment with acupuncture. After 6 months, the right facial paralysis and hoarseness disappeared. Our case emphasizes the importance of early diagnosis and treatment of atypical Ramsay Hunt syndrome as well as timely communication, enhancement of trust, and reduction of disputes between doctors and patients.

Purpose: To explore the characteristics, medical treatments, and long-term facial palsy outcome in Ramsay Hunt syndrome., Methods: Patient questionnaire including self-assessment of long-term facial palsy outcome and retrospective chart review. Initial facial palsy grade was compared to self-assessed or patient record stated palsy outcome. Occurrence of different characteristics (blisters, hearing loss, vertigo, etc.) of the syndrome were assessed., Results: Altogether 120 patients were included of which 81 answered the questionnaire. All but one patient had received virus medication (aciclovir, valaciclovir), and half received simultaneous corticosteroids. If the medication was started within 72 h of Ramsay Hunt diagnosis, facial palsy recovered totally or with only slight sequelae in over 80% of the patients. Only a minority of the patients experienced varicella blisters simultaneously with facial palsy, blisters more often preceded or followed the palsy. Approximately 20% of the patients had their blisters in hidden places in the ear canal or mouth., Conclusions: The long-term outcome of facial palsy in medically treated Ramsay Hunt syndrome was approaching the outcome of Bell's palsy. It is crucial to ask and inform the patient about the blisters and look for them since, more often than not, the blisters precede or follow the palsy and can be in areas not easily seen.

Ramsay Hunt syndrome (RHS) occurs due to reactivation of latent Varicella Zoster Virus (VZV) infection in the geniculate ganglion of the facial nerve. Major clinical symptoms include ipsilateral facial paralysis, otic pain, and herpetic vesicles (rashes) along the nerve with accompanying ear pain. Rarely clinical findings include retrograde transaxonal spread of the virus from the ganglion into the brain parenchyma with developing the encephalitis or multiple cranial nerve involvement. We describe here a patient with both RHS along with complicating brainstem encephalitis developed due to the coinfection of VZV and EBV.

Introduction: Ramsay Hunt syndrome (RHS) is a rare manifestation of varicella-zoster virus (VZV) reactivation in geniculate ganglion. It usually manifests with a characteristic triad of symptoms including ipsilateral ear pain, vesicles in the external auditory canal, and facial nerve palsy., Case: We present a case report showing RHS additionally manifested by meningitis and involvement of VIII cranial nerve. Clinical course was complicated by acute kidney injury induced by acyclovir therapy., Results: Despite the involvement of the geniculate ganglion and vestibulocochlear nerve in the course of herpes zoster, and the occurrence of acute kidney injury, the patient fully recovered., Conclusions: A complete recovery of cranial nerves VII and VIII in the course of RHS can be achieved.

Several case studies have suggested the usefulness of peripheral nerve blocks in the management of various types of chronic pain that are unresponsive to standard medical treatment. We report here the case of a patient with severe neuralgia, secondary to Ramsay Hunt syndrome that was refractory to standard drug therapy. As a last resort, a block of the terminal branches of nervus intermedius was performed. Despite transient facial paralysis, pain was markedly reduced for 3 months with self-reported improved quality of life. To our knowledge, this block has never been described previously.

Background: The degree of distress caused by the sequelae of peripheral facial nerve palsy usually depends on the severity of synkinesis. Objective: To clarify whether electroneurography (ENoG) can predict the severity of synkinesis after peripheral facial nerve palsy. Materials and methods: One-hundred and fourteen patients treated for facial nerve palsy at our hospital from April 2014-September 2016 were retrospectively reviewed. ENoG was performed 10-16 days after symptom onset. Patients were classified into Groups A (ENoG value 10%-20%, n  = 9) and B (ENoG value <10%, n  = 21). Eight months after symptom onset, electrophysiological and symptomatic outcomes were evaluated as the aberrant regeneration ratio of the blink reflex and the total synkinesis score of the Sunnybrook facial grading system, respectively. The outcomes of the groups were compared. Results: Group B had a significantly higher median aberrant regeneration ratio (0% versus 87%, p =.015), median total synkinesis score (1 versus 3, p  < .001), and incidence of moderate-to-severe synkinesis (0% versus 57.2%, p =.003) than did Group A. Conclusions and significance: Patients with an ENoG value of <10% have higher risks of aberrant regeneration and moderate-to-severe synkinesis than those with an ENoG value of 10%-20%. Patients with ENoG values of <10% may require rehabilitation to prevent synkinesis.

This is a case of an 85-year-old woman whom was admitted with otalgia and an abducens nerve palsy alongside a Pseudomonas otitis externa; she was presumed to have malignant otitis externa. However, despite optimum treatment and resolution of her otitis externa, she went on to develop an ipsilateral facial nerve palsy and sensorineural hearing loss. After further investigation, it was discovered that varicella-zoster meningitis was causing her polyneuropathy. She eventually responded to antivirals and steroids and, at follow-up, her sixth and seventh cranial nerve palsies had completely resolved, though a hearing deficit remained. This case highlights the importance of keeping a diagnosis under review, with the help of the multidisciplinary team, when the clinical course is not progressing as expected., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: Patients with Ramsay-Hunt syndrome have a poorer prognosis than patients with Bell's palsy. Factors of metabolic syndrome affecting prognosis were therefore compared between patients with Ramsay-Hunt syndrome and those with Bell's palsy., Methods: This retrospective study included 106 with Ramsay-Hunt syndrome and 182 with Bell's palsy. Age, sex, body mass index, blood pressure, blood test results, and ENoG results, stratified by House-Brackmann grade, were compared in patients with Ramsay-Hunt syndrome and Bell's palsy. Both groups of patients were treated with steroids and the antiviral agent famciclovir., Results: Age, sex, body mass index, dyslipidemia, triglyceride, diabetes, hypertension, and onset of palsy did not differ in patients with Ramsay-Hunt syndrome and Bell's palsy. Rates of favorable recovery in patients with severe facial palsy and DM were lower in patients with Ramsay-Hunt syndrome than with Bell's palsy and were also lower in low-weight, normal weight, and overweight patients with Ramsay-Hunt syndrome than with Bell's palsy. Rates of favorable recovery in patients with severe facial palsy and normal HDL, as well as in patients with severe facial palsy and < 10% ENoG, were lower in patients with Ramsay-Hunt syndrome than with Bell's palsy., Conclusions: Among patients with severe facial palsy, along with diabetes and < 10% ENoG, unfavorable recovery rates were significantly higher in those with Ramsay-Hunt syndrome than with Bell's palsy.

Purpose: To examine the etiology, clinical course, and management of recurrent peripheral facial nerve paralysis., Methods: Retrospective review at a single tertiary academic center and systematic review of the literature. Clinical presentation, laboratory and imaging findings, treatment and outcome for all cases of recurrent ipsilateral, recurrent contralateral, and bilateral simultaneous cases of facial paralysis are reviewed., Results: Between 2000 and 2017, 53 patients [41.5% men, 29 median age of onset (range 2.5 wk-75 yr)] were evaluated for recurrent facial nerve paralysis at the authors' institution. Twenty-two (41.5%) cases presented with ipsilateral recurrences only, while the remaining 31 patients (58.5%) had at least 1 episode of contralateral recurrent paralysis. No cases of bilateral simultaneous facial nerve paralysis were observed. The median number of paretic events for all patients was 3 (range 2-20). The median nadir House-Brackmann score was 4, with a median recovery to House-Brackmann grade 1.5 over a mean recovery time of 61.8 days (range 1-420 d). Diagnostic evaluation confirmed Melkersson-Rosenthal syndrome in four (7.5%) cases, neurosarcoidosis in two (3.7%), traumatic neuroma in one (1.9%), Ramsay Hunt syndrome in one (1.9%), granulomatosis with polyangiitis in one (1.9%), and neoplastic causes in three (5.7%) cases [facial nerve schwannoma (n = 2; 3.7%), metastatic squamous cell carcinoma to the deep lobe of the parotid gland (n = 1; 1.9%)]; ultimately, 77.4% (41) of cases were deemed idiopathic. Facial nerve decompression via a middle cranial fossa approach was performed in three (5.7%) cases without subsequent episodes of paralysis., Conclusion: Recurrent facial nerve paralysis is uncommon and few studies have evaluated this unique population. Recurrent ipsilateral and contralateral episodes are most commonly attributed to idiopathic facial nerve paralysis (i.e., Bell's palsy); however, a subset harbor neoplastic causes or local manifestations of underlying systemic disease. A comprehensive diagnostic evaluation is warranted in patients presenting with recurrent facial nerve paralysis and therapeutic considerations including facial nerve decompression can be considered in select cases.

Summary Ramsey-Hunt syndrome is caused by the varicella zoster virus, which mainly affects the facial nerve. The typical clinical features of Ramsey-Hunt syndrome are peripheral facial paralysis and ear herpes. In this case, initial symptoms were hoarseness and coughing, afterwards typical symptom occurred 5 days later which were earache, late-onset herpes, and facial paralysis. Ramsey-Hunt syndrome are difficult to diagnose because of its seemingly unrelated initial symptoms., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Background: Prediction of the severity and outcomes of Ramsay Hunt syndrome (RHS) is difficult., Objectives: The aim of this study was to evaluate the predictive power of the neutrophil-to-lymphocyte ratio (NLR) for the severity and outcomes of RHS., Material and Methods: From 2007 to 2017, a retrospective study of 102 hospitalized patients with RHS was conducted. The degree of paralysis was assessed by determining the House-Brackmann (H-B) grade. Obesity, hypertension, and diabetes mellitus were assessed, and the blood NLR and platelet-to-lymphocyte ratio were determined. The patients received steroids and antivirals, and were followed in the outpatient department at 1, 4, 12, and 24 weeks., Results: The H-B grade of the high-NLR group was significantly higher than that of the normal-NLR group (p = 0.039), and the probability of complete recovery was significantly lower in the high-NLR group (p = 0.048)., Conclusions: Patients with RHS who have an elevated NLR have poor outcomes in terms of the H-B grade. Therefore, the NLR may be useful for evaluating the prognosis of patients with RHS., (© 2019 S. Karger AG, Basel.)

The article contains literature review on etiology, natural history and treatment of Ramsay Hunt syndrome. Differential diagnosis in case of 8 th cranial nerve involvement is discussed. We present a case of the patient with Ramsay Hunt syndrome with hearing loss and vertigo is described. Clinical symptoms and diagnosis of sensorineural hearing loss and acute unilateral vestibulopathy are presented. The successful treatment of the patient resulted in complete facial nerve recovery, hearing improvement and partial recovery of vestibular function.

Objective: This article describes an atypical herpes zoster (HZ) infection heralded by prodromic dental pain., Background: Trigeminal HZ is associated with any of the three divisions of the trigeminal nerve, and Ramsay Hunt syndrome is a rare variant of HZ infection of the ear, often accompanied with unilateral facial nerve palsy., Materials and Methods: An 87-year-old man sought remedy for tooth-like pain. Within a week, he developed cutaneous, intraoral and otic eruptions, hearing loss, and mild facial palsy., Results: After ruling out odontogenic pain, he was diagnosed with trigeminal HZ and Ramsay Hunt syndrome., Conclusion: Herpes zoster infection should be included as a possible aetiologic factor with atypical presentations of odontogenic pain. Diagnosis of trigeminal HZ infection and the Ramsay Hunt syndrome may lead to timely intervention with antivirals and improved patient outcomes., (© 2018 Gerodontology Association and John Wiley & Sons Ltd.)

Objective: To investigate factors affecting the effect of physical rehabilitation therapy for synkinesis as a sequela to facial nerve palsy., Methods: A total of 37 patients with peripheral facial nerve palsy in Teine-Keijinkai Hospital were enrolled in this study. All patients showed synkinesis at 6 months after the onset of facial nerve palsy and were instructed in physical rehabilitation by expert staff from their first visit. The degree of synkinesis was evaluated at 6, 9 and 12 months after the onset of facial nerve palsy based on Sunnybrook facial grading system score and asymmetry in eye opening width. The patients were divided into two groups by age, gender, cause of palsy, electroneurography (ENoG) value, onset of synkinesis, initial treatment and timing of the start of physical rehabilitation., Results: Female patients and younger patients did not show any deterioration in synkinesis. Patients in the lower ENoG group and the later onset of synkinesis group showed significant deterioration in synkinesis after the 6th month from onset of facial palsy., Conclusion: Physical rehabilitation was shown to prevent significant deterioration in synkinesis in female and younger patients with facial nerve palsy. Careful follow-up with regard to synkinesis is required in cases in which the facial nerve damage is thought to be severe., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Objective: We investigated whether the value of ENoG is a predictive factor for the development of facial synkinesis in patients with facial palsy., Methods: The degree of oral-ocular synkinesis was evaluated quantitatively by an asymmetry of the interpalpebral space width during the mouth movement (% eye opening). Twenty healthy volunteers without a history of facial palsy (12 men and 8 women; 25-65 years old; mean age: 42.3±9.7years) were included in the study to examine the normal range of % eye opening. Fifty-one patients with facial palsy including 38 with Bell palsy and 15 with herpes zoster oticus (28 men and 25 women; 11-86 years old; mean age: 54±19years) were enrolled to examine the relationship between the ENoG value 10-14days after the onset of facial palsy, and the % eye opening 12 months later. Receiver operating characteristic (ROC) curve for the ENoG value was then used to decide the optimum cut-off value as a predictor of the development of oral-ocular synkinesis., Results: We defined a % eye opening inferior to 85% as an index of the development of oral-ocular synkinesis. There was a significant correlation between the values of ENoG 10-14days after the onset of facial palsy and those of % eye opening 12 months later (ρ=0.81, p<0.001). The area under the ROC curve for the ENoG value was the predictor for the development of oral-ocular synkinesis at 0.913 (95%CI: 0.831-0.996, p<0.001). The optimum cut-off value of ENoG 10-14days after the onset of facial palsy was 46.5% to predict the development of oral-ocular synkinesis 12 months after the onset of facial palsy (sensitivity 97.1% and specificity 77.5%)., Conclusion: The value of ENoG 10-14days after the onset of facial palsy is a predictive factor for the development of facial synkinesis 12 months later. Since facial palsy patients with a ENoG value inferior to 46.5% have a high risk of developing synkinesis, they should receive the facial biofeedback rehabilitation with a mirror as a preventive therapy., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Objective: Ramsay Hunt syndrome (RHS) and Bell palsy (BP) are typically known as facial nerve motor syndromes and are primarily unilateral. The aim of this study was to challenge this assertion, because both conditions are also known to be associated with viruses that typically affect several nerves., Methods: Ten participants with RHS, 12 with BP, all clinically unilateral, and 12 healthy controls were prospectively enrolled. Electrogustometric thresholds were measured bilaterally in the areas of the chorda tympani, the glossopharyngeal, and the major petrosal nerve. Also bilaterally, the taste function was tested using chemogustometry with different tastant concentrations. Again bilaterally, the morphology of the mucosa and the vessels of the anterior fungiform papillae were examined by contact endoscopy. Statistically, RHS and BP participants were compared with the healthy controls, and the paretic sides of RHS and BP were compared pairwise with their mobile sides., Results: Electrogustometrically, perception was reduced bilaterally in RHS (10-19dB, p < 0.001) and BP (3-5dB, p = 0.011-0.030) in all 3 innervation areas. Chemogustometrically, it was also reduced bilaterally in RHS (20-70%) and BP (8-50%). Papillary atrophies were increased 100% in RHS (p = 0.001) and BP (p < 0.001). They were more increased on the paretic side in RHS (30%, p = 0.078) and BP (83%, p < 0.001)., Interpretation: In these 2 clinically unilateral conditions, the gustatory perception and morphology are bilaterally affected, more in RHS and more on the paretic side. BP, known as an isolated motor condition, appears to be a cranial polyneuritis. A bilateral examination and therapeutic gustatory monitoring might follow these observations in evidence-based practice. Ann Neurol 2018;83:807-815., (© 2018 American Neurological Association.)

Rationale: Ramsay-Hunt's syndrome (RHS) is a disorder characterized by facial paralysis, herpetic eruptions on the auricle, and otic pain due to the reactivation of latent varicella zoster virus in the geniculate ganglion. A few cases of multiple cranial nerve invasion including the vestibulocochlear nerve, glossopharyngeal nerve and vagus nerve have been reported. However, there has been no report about RHS with delayed onset multiple cranial nerve involvement causing severe aspiration, and a clinical course that improved after more than one year of dysphagia rehabilitation and percutaneous endoscopic gastrostomy (PEG). Here, we report on a 67-year old male with delayed onset swallowing difficulty after 16 days of RHS development., Patient Concern: Severe aspiration during swallowing., Diagnosis: Severe dysphagia caused by RHS with multiple cranial nerve involvement., Intervention: Application of percutaneous endoscopic gastrostomy (PEG) and rehabilitation therapy of dysphagia., Outcomes: After 13 months from symptom onset, his PAS improved from 7 to 2 in follow-up video-fluoroscopic swallowing study (VFSS). Then, he was re-admitted, and the PEG tube was removed and oral feeding was started., Lessons: This case gives us the lesson that optimal doses of acyclovir and corticosteroids are important to prevent progression of multiple cranial involvement in RHS, and swallowing difficulty in RHS patients with multiple cranial nerve involvement can be improved through long-term rehabilitation even if there is no improvement for more than one year.

Objectives: To investigate the incidence of positional nystagmus (PN) using a head-roll test in patients with Ramsay Hunt syndrome with vertigo (RHS&#95;V) and discuss possible mechanisms., Study Design: Retrospective study., Setting: Tertiary referral academic medical center., Patients: Twenty-eight patients with RHS&#95;V were enrolled., Main Outcome Measure: Eye movements were recorded at positions of head roll to the right or left, and PN was classified as direction-fixed or direction-changing. Vestibular function tests including caloric test were performed., Results: Direction-fixed nystagmus beating away from the affected side was the most common type of PN (61%), followed by direction-changing geotropic type (18%), direction-fixed nystagmus beating toward the affected side (14%), and direction-changing apogeotropic type (7%). The duration of nystagmus was longer than 60 seconds in all patients exhibiting direction-changing PN. Postcontrast T1-weighted internal auditory canal (IAC) magnetic resonance imaging showed enhancement of not only the facial and vestibulocochlear nerves, but also the inner ear structures or dura along the IAC, suggesting inflammatory changes within the labyrinthine membrane or IAC dura., Conclusion: Although direction-fixed PN was more commonly observed (75%), direction-changing PN was also observed in some RHS&#95;V patients (25%). The mechanism of direction-changing PN may be, at least in part, explained by the alteration of specific gravity of the lateral semicircular canal cupula or endolymph due to inflammation in the inner ear membrane.

A 56-year-old immunocompetent male developed brainstem encephalitis complicating Ramsay Hunt syndrome. The disease had a slowly progressing course of months after the triggering infection, much longer than previously reported. Furthermore, magnetic resonance imaging, physical-chemical, and cell count analyses on cerebrospinal fluid were normal, whereas polymerase chain reaction for varicella zoster virus DNA was positive. The simultaneous negativity of both imaging and basic CSF exams is very rare, although possible event which confirms the irreplaceable role of viral screening on CSF. A systematic review of similar reports with highlights on the unusual aspects of our case is also presented.