Vestibular mapping in Ramsay-Hunt syndrome and idiopathic sudden sensorineural hearing loss.

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.

Clinical analysis of acute peripheral facial palsy in older adults.

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.

Distribution of Medical Service Use for Facial Palsy Between Medicine and Traditional Korean Medicine Based on Population-Based Data of Korea.

We investigated the distribution of medical service uses for Bell's palsy and Ramsay Hunt syndrome between medicine and traditional Korean medicine using the National Health Insurance Service National Sample Cohort data of Korea from 2006 to 2015. Patients were identified with diagnostic codes and medication or treatment claim codes. For Bell's palsy, there were 5,970 (68.8%) patients who used traditional Korean medical service only, whereas for Ramsay Hunt syndrome, there were 749 (93.6%) patients who used medical service only. The proportion of traditional Korean medical service use was higher than that of medical service use in patients with Bell's palsy, while the opposite was found in patients with Ramsay Hunt syndrome.

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.

Síndrome de Ramsay Hunt: reporte de un caso clínico / Ramsay Hunt Syndrome: clinical case report

El virus varicela zoster produce cuadros clínicos por reactivación del mismo en años posteriores a la primoinfección, en sujetos con condiciones particulares, como situaciones de estrés, inmunodepresión, radioterapia, estados infecciosos que cursen con fiebre, entre otros. El síndrome de Ramsay Hunt es uno de ellos. Se caracteriza por una tríada de parálisis facial periférica, vesículas herpéticas en oído externo y otalgia. Objetivo: pr esentar un caso típico del síndr ome de Ramsay Hunt, métodos diagnósticos y tratamiento realizado. Presentación de caso clínico: paciente femenina, de 21 años, originaria de Tegucigalpa, Honduras, se presentó al Hospital Escuela Universitario por cuadro de seis días de evolución, de cambios inflamatorios en el oído externo izquierdo, en la región del pabellón auricular y el conducto auditivo externo, con presencia de vesículas herpéticas eritematosas con costras y restos hemáticos, otorrea, hiperemia, prurito y otalgia intermitente. También refirió parálisis de hemicara izquierda de cuatro días de evolución. Examen físico: conducto auditivo externo izquierdo presentaba vesículas con costras. Hubo ptosis palpebral izquierda e incapacidad para realizar las mímicas faciales. Tratamiento intrahospitalario: aciclovir 800 mg vía oral cada 6 horas, dexametasona 8 mg IV cada 8 horas, diclofenaco 75 mg IV cada 12 horas; citidina-5- monofosfato disódico, más uridin-5-trifosfato trisódico, 1 comprimido cada 12 horas; mupirocina ungüento al 1Universidad Nacional Autónoma de Honduras, Facultad de Ciencias Médicas, Tegucigalpa, Honduras. ORCID 0000-0002-2346-4209 2Universidad Nacional Autónoma de Honduras, Facultad de Ciencias Médicas. Departamento de Cirugía. Tegucigalpa, Honduras. 3Hospital Escuela Universitario, Departamento de Cirugía, Tegucigalpa, Honduras. Autor de correspondencia: Paola Andrea Hincapié Gaviria, paolahincapie23@gmail.com Recibido: 10/07/2020 Aceptado: 03/03/2021 al 2%, aplicado en oído externo cada 8 horas y ejercicios de fisioterapia. Conclusión: el síndrome de Ramsay Hunt se diagnóstica, fundamentalmente, mediante hallazgos clínicos. El tratamiento empírico y adecuado, acompañado de una buena terapia posterior, anticipa un pronóstico favorable sin secuelas neurológicas...(AU)

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.

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.

Síndrome de Ramsay Hunt. Informe de un caso y revisión de la literatura / Ramsay Hunt syndrome. Case report and literature review

RESUMEN Se presentó paciente masculino de 68 años de edad, con antecedentes de síndrome de Parkinson posinfarto cerebral isquémico antiguo e hipertensión arterial sistémica, con la intensión de describir la experiencia del diagnóstico y tratamiento del síndrome de Ramsay Hunt. El paciente acudió a consulta de Medicina Interna del Policlínico Universitario "Omar Ranedo Pubillones", Guantánamo, por presentar dolor en hemicara izquierda y otalgia homolateral, cefalea y febrícula. Luego, presentó lesiones vesiculosas de aspecto herpético. Se concluyó el diagnóstico definitivo de síndrome de Ramsay Hunt tipo II y se remitió a consulta de Otorrinolaringología, donde se confirmó el diagnóstico. Luego del tratamiento evolucionó satisfactoriamente. Aunque el síndrome de Ramsay Hunt no es un problema de salud, se revela la necesidad de que el médico del nivel primario de atención a la salud se familiarice con las bases teóricas para su diagnóstico y se connota la importancia de la evaluación interdisciplinaria para esta finalidad.

ABSTRACT It was reported a 68 year old male patient with a personal pathological history of post- ischemic stroke Parkinson's syndrome and systemic arterial hypertension and with the intention of describing the experience of diagnosis and treatment of Ramsay Hunt syndrome. Patient presented to his primary health physician at the Univirsity Clinic "Omar Ranedo Pubillones" in Guantanamo, presenting pain in right side of the face and earache, headache and fever. Then, he presented vesicular lesions with a herpetic appearance. It was concluded the definitive diagnosis of Ramsay Hunt syndrome type II and it was referred to Otolaryngology consultation, where the diagnosis was confirmed. After treatment patient's improvement was good. Although Ramsay Hunt syndrome is not a health problem, shows the need for primary care physicians to become familiar with the theoretical basis for its diagnosis and the importance of interdisciplinary evaluation for this purpose.

Ramsay Hunt syndrome: characteristics and patient self-assessed long-term facial palsy outcome.

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.

Herpes zoster oticus with meningitis masquerading as malignant otitis externa.

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.

[Ramsey-Hunt syndrome with initial syndrome of hoarseness: a case report].

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.

Ramsay Hunt syndrome: a rare complication of herpes zoster infection in a lung cancer patient.

Objectives: Ramsay Hunt syndrome (RHS) is a rare complication of varicella zoster virus (VZV) reactivation with high morbidity, requiring swift and specific treatment. Methods: We discuss the epidemiology, diagnosis and treatment of RHS using a case report of a cancer patient who presented with disseminated VZV reactivation, including RHS. Results: A 68-year old man with stage IV lung adenocarcinoma, for which he received pemetrexed maintenance chemotherapy, presented to the emergency department with a progressive skin rash despite broad spectrum antibiotics, vertigo, and diminished hearing. Valacyclovir was started for a varicella zoster virus (VZV) reactivation. Despite antiviral therapy, his symptoms progressed with late appearance of vesicles in the external ear canal, leading to a diagnosis of Ramsay Hunt syndrome (RHS). Conclusion: RHS is a rare complication of VZV reactivation and can lead to peripheral facial nerve palsy, hearing loss, vestibular problems, and vesicles in the dermatome of the cranial nerve. Timely initiation of steroids and antiviral therapy lead to recovery of the facial nerve in >75% of cases, although the optimal therapy is still unclear.

Typical or Atypical Ramsay-Hunt Syndrome in Delayed Facial Palsy After Stapedectomy?

OBJECTIVES: The aim of this study was to define the typical pattern for varicella zoster virus (VZV) reactivation in delayed facial palsy (DFP) after stapedectomy for otosclerosis. MATERIALS AND METHODS: Review of the relevant literature, personal casistics, and case-report. RESULTS: In total, 48 cases of DFP after stapes surgery have been described so far, including the reported case with exclusive manifestation of atypical Ramsay Hunt syndrome (RH); in the personal series of 1253 stapedectomies, DFP occurred in only one case (0.08%). Complete DFP (House-Brackmann grade VI) rapidly developed 12 days after surgery; RH appeared 2 days later, confirming the role of VZV. The DFP started improving after 8 weeks and completely recovered 6 months later. CONCLUSION: Acute otalgia prior to DFP should raise the suspicion of VZV reactivation. Atypical RH is the most frequent pattern that occurs in DFP after stapedectomy.

Novel pre-therapeutic scoring system using patient and haematological data to predict facial palsy prognosis.

OBJECTIVES: We describe a novel scoring system, the facial Palsy Prognosis Prediction score (PPP score), which we test for reliability in predicting pre-therapeutic prognosis of facial palsy. We aimed to use readily available patient data that all clinicians have access to before starting treatment. DESIGN: Multicenter case series with chart review. SETTING: Three tertiary care hospitals. PARTICIPANTS: We obtained haematological and demographic data from 468 facial palsy patients who were treated between 2010 and 2014 in three tertiary care hospitals. Patients were categorised as having Bell's palsy or Ramsey Hunt's palsy. MAIN OUTCOME MEASURES: We compared the data of recovered and unrecovered patients. PPP scores consisted of combinatorial threshold values of continuous patient data (eg platelet count) and categorical variables (eg gender) that best predicted recovery. We created separate PPP scores for Bell's palsy patients (PPP-B) and for Ramsey Hunt's palsy patients (PPP-H). RESULTS: The PPP-B score included age (≥65 years), gender (male) and neutrophil-to-lymphocyte ratio (≥2.9). The PPP-H score included age (≥50 years), monocyte rate (≥6.0%), mean corpuscular volume (≥95 fl) and platelet count (≤200 000 /µL). Patient recovery rate significantly decreased with increasing PPP scores (both PPP-B and PPP-H) in a step-wise manner. PPP scores (ie PPP-B score and PPP-H score) ≥2 were associated with worse than average prognosis. CONCLUSIONS: Palsy Prognosis Prediction scores are useful for predicting prognosis of facial palsy before beginning treatment.

Magnetic Resonance Imaging Evidence of Varicella Zoster Virus Polyneuropathy: Involvement of the Glossopharyngeal and Vagus Nerves Associated With Ramsay Hunt Syndrome.

The involvement of lower cranial nerve palsies is less frequent in Ramsay Hunt syndrome caused by varicella zoster virus (VZV). The authors report 1 of extremely rare patients of radiologically proven polyneuropathy of VZV infection with magnetic resonance imaging findings of VII, IX, and X cranial nerve involvement is a 62-year-old female patient, who initially presented with Ramsay Hunt syndrome. Varicella zoster virus infection should be considered even in patients who show unilateral palsy of the lower cranial nerves associated with laryngeal paralysis. Thin-section T2W and T1W images with a contrast agent should be added to the imaging protocol to show the subtle involvement.

Synchronous presentation of trigeminal, glossopharyngeal and geniculate neuralgias in a single patient.

Orofacial pain disorders can present as a diagnostic and therapeutic challenge for oral health care providers. Odontogenic and nonodontogenic sources of orofacial pain should be considered and cranial neuralgias may be included in the differential diagnosis. Synchronous presentation of multiple cranial neuralgias is a rare occurrence. We report a case of a patient with a synchronous presentation of trigeminal, glossopharyngeal, and geniculate neuralgias. To our knowledge, this is the first case of a synchronous presentation of these conditions reported to date.