Task force of the Brazilian Society of Otology - evaluation and management of peripheral facial palsy.

OBJECTIVE: To review key evidence-based recommendations for the diagnosis and treatment of peripheral facial palsy in children and adults. METHODS: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on peripheral facial palsy were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. RESULTS: The topics were divided into 2 main parts: (1) Evaluation and diagnosis of facial palsy: electrophysiologic tests, idiopathic facial palsy, Ramsay Hunt syndrome, traumatic peripheral facial palsy, recurrent peripheral facial palsy, facial nerve tumors, and peripheral facial palsy in children; and (2) Rehabilitation procedures: surgical decompression of the facial nerve, facial nerve grafting, surgical treatment of long-term peripheral facial palsy, and non-surgical rehabilitation of the facial nerve. CONCLUSIONS: Peripheral facial palsy is a condition of diverse etiology. Treatment should be individualized according to the cause of facial nerve dysfunction, but the literature presents better evidence-based recommendations for systemic corticosteroid therapy.

Ramsay Hunt Syndrome in Asymptomatic COVID-19 Infection: A Case Report and a Literature Review.

(1) Background: COVID-19 infection has affected almost 6 million people worldwide. Geniculate Ganglion Zoster resulting in Ramsay Hunt Syndrome (RHS) has been rarely described in this context. (2) Methods: Here, a case of RHS in the context of asymptomatic COVID-19 infection is reported followed by a literature review of the previously published cases (PubMed research combining "COVID-19" and "Ramsay Hunt Syndrome" or their abbreviations/synonyms, searching for data published at any time till October 2023). (3) Results: Five cases have been previously published (age range: 25-67 years; n = 3 males). Three patients were known to be immunocompetent prior to infection, one was receiving corticotherapy for lung disease, and one had an unspecified immune status. RHS predominantly involved both facial and vestibulocochlear nerves, with one case exclusively involving the facial nerve as the presented case. Regarding facial nerve palsy, three were right-sided (like the current report) and two were left-sided. Two cases were asymptomatic to COVID-19 (like the present patient), one had mild fatigue, and two had classical COVID-19 symptoms preceding RHS symptoms. Workup included serological testing against Varicella Zoster Virus and PCR assays that can detect the viral DNA in saliva, blood, tears, exudates, and cerebrospinal fluid. The treatment combined antiviral and corticosteroid therapies which yielded heterogeneous outcomes that might be related to some demographic and clinical data. (4) Conclusions: RHS rarely occurs in the context of COVID-19. Early recognition is important. Management seems to be similar to the classical condition. Some data may help predict facial nerve recovery.

Ramsay Hunt syndrome and concurrent varicella-zoster virus meningitis in Denmark: A nationwide cohort study.

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.

Audiological Profiling and Importance of Vestibular Rehabilitation for a Patient with Ramsay Hunt Syndrome: Case Report.

The case report highlights the importance of the rehabilitative approach and the role of audiology in managing patients with Ramsay Hunt Syndrome (RHS). RHS is a rare condition characterized by neuropathies involving multiple cranial nerves. Out of three neurological variants noted in the literature, RHS type II is characterized by hearing loss, tinnitus, and vertigo. The current case report is of a 37-year-old female diagnosed with RHS type II who reported with the complaints of right-sided headache and chronic otalgia. The progression of the disease caused hearing loss and tinnitus on the right side. Subsequently, the patient also developed signs of imbalance, which were not reported till 2 weeks after the onset of other symptoms. Three audiological evaluations were done during the initial visit, treatment phase, and post-treatment. It also helped identify the need for vestibular rehabilitation therapy and medical treatment. A comprehensive team approach and timely intervention aided in the prevention of the long-lasting effects of RHS in this patient. Awareness about the roles of professionals in assessment and management can help significantly improve the quality of life of individuals, especially in syndromes and multiple disabilities.

Cochleo-Vestibular Disorders in Herpes Zoster Oticus: A Literature Review and a Case of Bilateral Vestibular Hypofunction in Unilateral HZO.

The varicella-zoster virus (VZV), a member of the Herpesviridae family, causes both the initial varicella infection and subsequent zoster episodes. Disorders of the eighth cranial nerve are common in people with herpes zoster oticus (HZO). We performed a review of the literature on different databases including PubMed and SCOPUS, focusing on cochlear and vestibular symptoms; 38 studies were considered in our review. A high percentage of cases of HZO provokes cochlear and vestibular symptoms, hearing loss and vertigo, whose onset is normally preceded by vesicles on the external ear. It is still under debate if the sites of damage are the inferior/superior vestibular nerves and cochlear nerves or a direct localization of the infection in the inner ear. The involvement of other contiguous cranial nerves has also been reported in a few cases. We report the case of a patient with single-side HZO presenting clinical manifestations of cochleo-vestibular damage without neurological and meningeal signs; after 15 days, the patient developed a new episode of vertigo with clinical findings of acute contralateral vestibular loss. To our knowledge, only three other such cases have been published. An autoimmune etiology may be considered to explain these findings.

Herpes Zoster Oticus with Concurrent Hearing Loss: A Study on Clinical Characteristics and Prognosis.

This study aimed to analyze the clinical characteristics and treatment prognoses of patients with Herpes Zoster Oticus (HZO) and concurrent hearing loss (HL). Various clinical characteristics of 192 patients with HZO, with or without concurrent HL, from 2016 to 2020 were retrospectively analyzed through a chart review. All patients were followed-up until recovery or up to 12 months. Demographic and clinical findings were compared between the groups, and the recovery rates of facial palsy, hearing, and other clinical features were analyzed. Facial palsy recovery was analyzed using the House-Brackmann (HB) grading system, and hearing recovery rates were analyzed using the Siegel criteria. Of the 192 patients diagnosed with HZO, 142 had no hearing loss (HZO without HL), and 50 had hearing loss (HZO with HL). While both groups had similar ages, treatment timings, and underlying diseases, the HZO w HL group had a significantly higher rate of dizziness and tinnitus, but received more intratympanic steroid injections. In terms of facial palsy, there were no significant differences in the initial HB grade or recovery rates between the groups. Within the HZO w HL group, hearing loss severity varied, with 52% experiencing mild loss and only 16% achieving complete recovery. Descending-type audiograms were the most common at 66%. In patients with HZO, there was no statistically significant difference between the degree of initial facial paralysis and the degree of recovery of the final facial paralysis according to concurrent HL. However, among patients with concurrent HL, the hearing recovery rate in the HZO group was low.

Clinical and Epidemiological Profile of Herpes Zoster and Its Complications in a Tertiary Care Center of Bihar: A Prospective Study.

Background Herpes zoster is a common viral infection caused by reactivation of the varicella-zoster virus (VZV) characterized by the presence of a segmental distribution of painful grouped vesicles on an erythematous base. It is associated with several complications like zoster-associated pain (ZAP), postherpetic neuralgia (PHN), pigmentary changes, scarring, secondary infections, and dermatosis as well as severe systemic complications. Aims/objectives The aim of the study was to analyze the various clinical and epidemiological patterns of herpes zoster and post-herpetic complications. Materials and methods We conducted a single-center observational cross-sectional study on 72 patients with herpes zoster and post-herpetic complications attending the dermatology outpatient department (OPD) to understand its various clinical and epidemiological patterns. A detailed history taking regarding the onset, progression, and complications of the disease, as well as the type, duration, and severity of pain, was taken, followed by a general physical, systemic, and cutaneous examination, along with investigations wherever needed. Results A total of 72 patients were included in the study, comprising 32 (44.4%) patients suffering from herpes zoster and 40 (55.5%) patients suffering from post-herpetic complaints. The minimum age was 14 years, the maximum age was 83 years, and the mean age in our study was 52 ± 17 years. The most commonly affected age group was 41-60 years. A total of 52 males and 22 females were included in the study, resulting in a male-to-female ratio of 2.3:1. The thoracic dermatome was the most commonly involved dermatome, observed in 43 (59.7%) patients, and the left side was more commonly affected, seen in 41 (56.9%) patients. Among the total 72 patients, 26 (36.1%) had co-morbidities, with hypertension (18%) being the most common, followed by diabetes mellitus (12.5%). Regarding the post-herpetic complaints encountered in our study, the most common was post-herpetic neuralgia, seen in 31 (77.5%) patients, followed by post-herpetic pigmentation (macular), observed in 22 (55%) patients, and scarring (papules, plaques, hypertrophic scar, and keloid), observed in 17 (42.5%) patients. Conclusion A broader understanding of the clinical and epidemiological factors of herpes zoster and post-herpetic complications is important as this disease constitutes a considerable burden in a tertiary care center and if not treated adequately, the after-effects might last for many years altogether. Hence, early diagnosis and initiation of adequate antiviral therapy as well as pain management is the key aspect of management.

Zoster Polyneuritis Cranialis and Intracranial Vasculopathy.

A case report of varicella zoster virus (VZV) producing cranial polyneuropathy with intracranial vasculopathy afflicting the internal carotid artery, in a 45-year lady is presented. The clinical features included appearance of zosteriform rash in the left external auditory canal and concha, affliction of ophthalmic and maxillary divisions of V, VI, VII, VIII, IX and X cranial nerves. Contrast MRI brain demonstrated attenuated flow void in the left internal carotid artery. The diagnosis was confirmed with raised level of IgG VZV antibody in serum and detection of VZV IgG antibody in CSF, although PCR for VZV DNA in CSF was negative. The significance lies in atypical presentation, the diagnostic dilemma it poses, poor prognosis of cranial nerve function and the need for multidisciplinary approach to patient management and rehabilitation.

A Rare Case of Vocal Cord Paralysis in the Setting of Ramsay Hunt Syndrome.

Ramsay Hunt syndrome (RHS) with concomitant vocal cord paralysis (VCP) is a rare finding. This case is particularly rare because the patient lacked the symptoms of otalgia or hearing loss when in fact, a majority of cases typically demonstrate both hearing loss and otalgia. Unique to this case is also the fact that it was complicated by a concomitant infarction of the splenium corpus callosum and a right temporal meningioma. The purpose of this study was to bring awareness to the fact that RHS can cause multiple cranial nerve neuropathies including VCP and should be included in the differential diagnosis for VCP.

Efficacy and safety of twice-daily tramadol hydrochloride bilayer sustained-release tablets with an immediate release component for postherpetic neuralgia: Results of a Phase III, randomized, double-blind, placebo-controlled, treatment-withdrawal study.

BACKGROUND: We investigated the efficacy and safety of twice-daily bilayer sustained-release tramadol hydrochloride tablets (35% immediate-release; 65% sustained-release) in patients with postherpetic neuralgia. METHODS: This was a Phase III treatment-withdrawal study with 1-4-week dose-escalation, 1-week fixed-dose, and 4-week randomized, double-blind, placebo-controlled withdrawal periods performed at 43 medical institutions in Japan. Patients aged ≥20 years, ≥3 months after the onset of herpes zoster with localized, persistent pain despite fixed-dose analgesics for ≥2 weeks before enrollment were eligible. Patients started tramadol at 100 mg/day and its dose escalated to a maximum of 400 mg/day to achieve a reduction in their Numeric Rating Scale (NRS) for pain of ≥2 points. Eligible patients were randomized to continue tramadol or switched to placebo for 4 weeks (double-blind period). Patients were withdrawn due to inadequate analgesia (NRS deteriorated on ≥2 consecutive days) or their request. RESULTS: Overall, 252 patients started tramadol and 173 were randomized (tramadol: 85; placebo: 88). Tramadol was superior to placebo for the primary endpoint (time from randomization to an inadequate analgesic effect) with log-rank test p = 0.0005. The hazard ratio was 0.353 (95% confidence interval 0.190-0.657) in favor of tramadol and fewer patients in the tramadol group experienced inadequate analgesic effects (16.9% vs. 39.8%). Adverse events in ≥10% of patients in the open-label period were constipation (43.8%), nausea (34.9%), somnolence (18.5%), and dizziness (11.6%). The frequencies of adverse events in the double-blind period were similar in both groups. CONCLUSION: Sustained-release tramadol tablets with an immediate-release component are effective and well tolerated for managing postherpetic neuralgia.

Continuous hemicrania as the initial manifestation of Ramsay Hunt syndrome: a case report / Hemicrania contínua como manifestação inicial da síndrome de Ramsay Hunt: relato de caso

Introduction:Ramsay-Hunt syndrome, also called otic zoster, is a rare complication of herpes zoster. The syndrome is characterized by peripheral facial nerve palsy and an erythematous vesicular eruption in the ear or mouth. Preceding the appearance of the vesicles, unilateral otalgia or neck pain may occur more commonly. However, persistent hemicrania is infrequent in the preeruptive phase. Objective:To present an atypical case of Ramsay Hunt syndrome with continuous unilateral headache preceding the onset of other symptoms and signs of the syndrome. Case report:Report of a 69-year-old woman who presented subacute onset of moderate to severe left hemicrania with no autonomic signs. Eight days after the start and continuous headache maintenance, she presented with peripheral facial paralysis. After four days, she noticed the presence of vesicles in the left ear and odynophagia. She developed nausea with several episodes of vomiting and severe imbalance that made it impossible for her to walk unassisted. On physical examination, she presented vesicles in the left ear and oropharynx, left peripheral facial palsy (House Brackmann grade IV), left hypoacusis, nystagmus, and vestibular gait. Diagnostic tests for screening several metabolic diseases and diagnosis of infection (including HIV) were unremarkable. Brain computed tomography and cerebrospinal fluid analysis showed no abnormalities. Conclusion:Ramsay-Hunt syndrome mainly involves the facial and vestibulocochlear nerves, causing peripheral facial palsy, otalgia, hypoacusis, and, less frequently, imbalance. Although pain is a frequent manifestation of the preeruptive phase of RHS, unilateral headache is not common in this scenario. On the other hand, it is a prevalent complaint in the emergency department and has several different etiologies. Hence, diagnosing RHS when patients present exclusively unilateral headaches is challenging for clinicians. Physicians must consider RHS a vital differential diagnosis of sided-locked headaches, avoiding diagnostic errors and treatment delays.

Introdução: A síndrome de Ramsay-Hunt, também chamada de ótico-zóster, é uma complicação rara do herpes-zóster. A síndrome é caracterizada por paralisia do nervo facial periférico e erupção vesicular eritematosa no ouvido ou na boca. Precedendo o aparecimento das vesículas, pode ocorrer mais comumente otalgia unilateral ou dor cervical. No entanto, a hemicrania persistente é pouco frequente na fase pré-eruptiva. Objetivo: Apresentar um caso atípico de síndrome de Ramsay Hunt com cefaleia unilateral contínua precedendo o aparecimento de outros sintomas e sinais da síndrome. Relato de caso: Relato de uma mulher de 69 anos que apresentou hemicrania esquerda de início subagudo, moderada a grave, sem sinais autonômicos. Oito dias após o início e manutenção contínua da cefaleia, apresentou paralisia facial periférica. Após quatro dias, notou presença de vesículas em orelha esquerda e odinofagia. Ela desenvolveu náuseas com vários episódios de vômitos e desequilíbrio grave que a impossibilitou de andar sem ajuda. Ao exame físico apresentava vesículas em orelha esquerda e orofaringe, paralisia facial periférica esquerda (grau IV de House Brackmann), hipoacusia esquerda, nistagmo e marcha vestibular. Os testes de diagnóstico para rastreio de diversas doenças metabólicas e diagnóstico de infeções (incluindo VIH) não apresentaram resultados dignos de nota. A tomografia computadorizada de cérebro e a análise do líquido cefalorraquidiano não mostraram alterações. Conclusão: A síndrome de Ramsay-Hunt envolve principalmente os nervos facial e vestibulococlear, causando paralisia facial periférica, otalgia, hipoacusia e, menos frequentemente, desequilíbrio. Embora a dor seja uma manifestação frequente da fase pré-eruptiva da SHI, a cefaleia unilateral não é comum neste cenário. Por outro lado, é uma queixa prevalente no serviço de urgência e tem diversas etiologias. Portanto, diagnosticar SHI quando os pacientes apresentam dores de cabeça exclusivamente unilaterais é um desafio para os médicos. Os médicos devem considerar a SHI um diagnóstico diferencial vital de cefaleias laterais, evitando erros de diagnóstico e atrasos no tratamento.

Ramsay Hunt Syndrome With Cranial Polyneuropathy and Delayed Facial Nerve Palsy: A Case Report.

Herpes zoster oticus is a viral disease caused by the reactivation of the varicella-zoster virus at the geniculate ganglion. The hallmark of the condition is multiple unilateral erythematous vesicles, which are distributed over the auricle and preceded by severe otalgia. If these symptoms are associated with facial nerve palsy, the condition is called Ramsay Hunt syndrome (RHS) which is usually accompanied by vestibulocochlear abnormalities. A 42-year-old woman came to our clinic with sudden onset of right-sided severe otalgia and several erythematous vesicles on the auricle two days ago. She provided a history of dysphagia and hoarseness for 10 days. After two days, ipsilateral facial nerve paralysis was noted. The patient was immunocompetent with an unremarkable medical history. Physical examination revealed the following: the vesicles distributed over the right auricle, external auditory canal, and eardrum; right sensorineural deafness; deviated uvula to the left side; absent gag reflex on the right side; right vocal cord palsy; and lower motor facial nerve paralysis of House-Brackmann grade III. The pure tone audiogram confirmed the diagnosis of right-sided sensorineural deafness. Acyclovir therapy and prednisolone tablets at a loading dose were initiated. At the four-month follow-up, the presenting manifestations were improved. Here, we report a case of RHS with early glossopharyngeal and vagus nerve palsy, followed by pain, vesicular eruptions, sensorineural hearing loss, and delayed onset of facial nerve paralysis. The condition resolved completely on medical treatment with acyclovir and prednisolone.

[Clinical and therapeutic features of herpes zoster oticus: a case report]. / Zona otitique, aspects cliniques et thérapeutiques: à propos d'un cas.

Ear shingles, also called herpes zoster oticus, are a viral infection of the outer, middle and/or inner ear due to varicella zoster virus (VZV). Patients with ear involvement associated with facial palsy are clinically diagnosed with Ramsay-Hunt´s syndrome. We here report the case of a 25-year-old patient with herpes zoster oticus associated with peripheral facial palsy, without other associated signs. This study and literature review investigate the various clinical, para-clinical and evolutionary features of herpes zoster oticus and the therapeutic approaches.

Where are the vesicles? A case report of Ramsay Hunt syndrome.

A 1-year-10-month-old child developed left-sided Ramsay Hunt syndrome (RHS) without vesicles 1 month after an episode of varicella zoster infection. No ear symptoms, including hearing loss, tinnitus, or imbalance, were reported. The external ear and otoscopic examinations were unremarkable. He achieved adequate recovery with corticosteroid treatment. This case report discusses the unusual presentation of RHS without vesicles, the diagnostic dilemma in young children, varicella zoster virus hepatitis, treatment modalities, and the role of vaccination in its prevention.

Ramsay Hunt syndrome with multiple cranial neuropathy: a literature review.

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.

Ramsay Hunt syndrome: long-term facial palsy outcome assessed face-to-face by three different grading scales and compared to patient self-assessment.

PURPOSE: To determine the long-term facial palsy outcome of Ramsay Hunt Syndrome by face-to-face grading by House-Brackmann Grading System, Facial Nerve Grading System 2.0, and Sunnybrook Facial Grading System concomitantly. To compare the applicability of the grading scales. To compare patients' self-assessed facial palsy outcome results to gradings performed by the investigator. To compare the face-to-face assessed facial palsy outcome to the initial palsy grade. METHODS: Fifty-seven patients self-assessed their facial palsy outcome and came to a one-time follow-up visit. The palsy outcome was graded by one investigator using the three above-mentioned grading systems concomitantly. The median time from syndrome onset to follow-up visit was 6.6 years. RESULT: A good long-term face-to-face assessed palsy outcome was enjoyed by 84% of the patients. Trying to assess only one House-Brackmann grade to represent the palsy outcome was impossible for most patients. Facial Nerve Grading System 2.0 worked better, but needed adjustments and certain sequelae findings needed to be neglected for it to be executable. The Sunnybrook system worked the best. Nearly 20% of the patients assessed themselves differently from the investigator: both better and worse. CONCLUSION: The Sunnybrook scale was the most applicable system used. With antiviral medication, the outcome of facial palsy in Ramsay Hunt syndrome starts to resemble that of Bell's palsy and emphasizes the importance of recognizing the syndrome and treating it accordingly. The results give hope to patients instead of the gloomy prospects that have stigmatized the syndrome.

Ramsay Hunt Syndrome in a Patient with Ulcerative Colitis Treated with Infliximab.

Ramsay Hunt syndrome is a rare complication of herpes zoster that results from reactivation of varicella-zoster virus in the geniculate ganglion of the VII cranial nerve. Immunosuppression can lead to reactivation of latent varicella-zoster virus, resulting in herpes zoster. Here, we present a case of Ramsay Hunt syndrome in a patient with ulcerative colitis under treatment with infliximab. LEARNING POINTS: Ramsay Hunt syndrome is a rare form of herpes zoster and characterized by the presence of otalgia, facial palsy, vertigo and vesicular rash in the external ear or on the oropharynx.Treatment with TNF inhibitors increases the risk of its development.Although not generally recommended, vaccination may reduce the risk of herpes zoster recurrence during TNF inhibitor therapy.

Nystagmus in Ramsay Hunt syndrome with or without dizziness.

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.

Síndrome de Ramsay Hunt: revisión narrativa / Ramsay Hunt Syndrome: Narrative Review

El síndrome de Ramsay Hunt (SRH) corresponde a la asociación de la parálisis facial periférica con una erupción vesicular localizada en el pabellón auricular, causada por el compromiso del ganglio geniculado secundario a una infección por el virus de la varicela-zóster (VVZ). Este síndrome es la segunda causa más común de parálisis facial atraumática y representa aproximadamente el 10 %-12 % de las parálisis faciales agudas, con una incidencia anual de 5 por cada 100 000 habitantes en Estados Unidos. El diagnóstico es principalmente clínico y entre las manifestaciones más destacadas se encuentran síntomas neurológicos como otalgia, tinnitus, hipoacusia asociada con parálisis facial junto a lesiones herpéticas características. Dentro de las complicaciones que se pueden presentar en esta entidad se encuentra, principalmente, la neuralgia posherpética, seguida de otras menos frecuentes como la encefalitis, el herpes zóster oftálmico y la mielitis. El manejo actual del SRH se basa en la aplicación de terapias duales con corticosteroides asociados a terapia antiviral, lo cual ha demostrado que el inicio temprano del tratamiento mejora el pronóstico y disminuye la aparición de complicaciones. El pronóstico de esta patología es inferior en comparación a patologías menos severas que comprometen el nervio facial (como la parálisis de Bell) y se ve impactado por varios factores como el inicio oportuno de tratamiento, el grupo etario y la presencia de comorbilidades.

Ramsay Hunt syndrome corresponds to the association of peripheral facial paralysis with a vesicular eruption located in the pinna, caused by the involvement of the geniculate ganglion secondary to infection by the varicella zoster virus. This syndrome is the second causes of atraumatic facial paralysis, representing approximately 10 %-12 % of acute facial paralysis, with an annual incidence of 5 per 100,000 inhabitants. The diagnosis is mainly clinical and among the most prominent manifestations are neurological symptoms such as otalgia, tinnitus, hypoacusis associated with facial paralysis together with characteristic herpetic lesions. Among the complications that may occur in this entity is mainly postherpetic neuralgia, followed by less frequent ones such as encephalitis, ophthalmic herpes zoster and myelitis. Current management of Ramsay Hunt syndrome is based on the application of dual therapies consisting of corticosteroids associated with antiviral therapy, showing that early initiation of treatment improves prognosis and reduces the appearance of complications. The prognosis of this pathology is inferior compared to less severe pathologies that compromise the facial nerve (Bell's palsy) and is impacted by several factors such as the timely initiation of treatment, the age group, and the presence of comorbidities.

Clinical characteristics of herpes zoster laryngitis.

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.