Geniculate Ganglion Research Articles

Identification of a postnatal period of interdependent neurogenesis and apoptosis in peripheral neurons

ABSTRACT During neurogenesis, excessive numbers of neurons are produced in most regions of the central and peripheral nervous systems. Nonessential neurons are eliminated by apoptosis, or programmed cell death. This has been most thoroughly characterized in the peripheral nervous system (PNS) where targets of innervation play a key role in this process. As maturing neurons project axons towards their targets of innervation, they become dependent upon these targets for survival. Survival factors, also called neurotrophic factors, are produced by targets, inhibit apoptosis cascades, and promote further growth and differentiation. Because neurotrophic factors are limited, as is target size, neurons that do not correctly and efficiently innervate targets undergo apoptosis ( Levi-Montalcini, 1987; Davies, 1996). Thus, excessive neurogenesis acts to ensure that sufficient numbers of neurons are produced during development. In the superior cervical ganglion (SCG), this process of neurogenesis and subsequent apoptosis is reported to be complete by postnatal day 3-4 (P3-P4) in mice. Surprisingly, we observed significant numbers of apoptotic neurons out to P14, and neurogenesis was still present at P14 as well. In both the SCG and geniculate ganglion (GG), postnatal neurogenesis was dependent on apoptosis because little or no postnatal neurogenesis was observed in Bax-/- mice, in which apoptosis is eliminated. These results indicate that both neurogenesis and apoptosis continue to occur well after birth in peripheral ganglia, and that neurogenesis depends on apoptosis, suggesting that neurogenesis continues postnatally to replace neurons that are eliminated during synaptic refinement.

Ramsay hunt syndrome: a case report

Ramsay hunt syndrome (RHS) is a neurological condition comprising of complications with varicella zoster virus (VZV) which results in inflammation of geniculate ganglion. In the days to weeks following zoster, various cranial nerve palsies can develop because the varicella-zoster virus is latent in the cranial nerve ganglia. The trigeminal and facial nerves were the most commonly affected by zoster, based on the cranial nerve involvement. Another common impacted nerve was the vestibulocochlear nerve. As VZV is exclusively human pathogen, research on VZV latency is limited to only human ganglia examination. This case report describes about Ramsay Hunt Syndrome induced or also called as Herpes Zoster Oticus caused by Varicella zoster virus. Diagnosis mainly involved laboratory investigations, patient history, treatment data and other relevant information co-related from various literature. A 46-year-old female patient was diagnosed Ramsay hunt syndrome on the basis of clinical findings as right facial palsy, blisters over right pinna, otalgia and right ear erythematous lesions. Patient also exhibited deviation of angle of mouth to left side and unable to close right eye. This was accompanied by elevated erythrocyte sedimentation rate (ESR) exhibiting acute inflammation. Hemoglobin was low and total leukocyte count was high. Following that, corticosteroids and antivirals were prescribed to assist her medical condition. Remission is slow for RHS, hence patient was also advised for rehabilitation therapy to improvise her lower motor neuron facial palsy. Recurrence is possible in long term so it is imperative to initiate early treatment within 72 hours as soon as the symptoms are observed for better patient care.

Deciphering Peripheral Taste Neuron Diversity: Using Genetic Identity to Bridge Taste Bud Innervation Patterns and Functional Responses.

Peripheral taste neurons exhibit functional, genetic, and morphological diversity, yet understanding how or if these attributes combine into taste neuron types remains unclear. In this study, we used male and female mice to relate taste bud innervation patterns to the function of a subset of proenkephalin-expressing (Penk+) taste neurons. We found that taste arbors (the portion of the axon within the taste bud) stemming from Penk+ neurons displayed diverse branching patterns and lacked stereotypical endings. The range in complexity observed for individual taste arbors from Penk+ neurons mirrored the entire population, suggesting that taste arbor morphologies are not primarily regulated by neuron type. Notably, the distinguishing feature of arbors from Penk+ neurons was their propensity to come within 110 nm (in apposition with) different types of taste-transducing cells within the taste bud. This finding is contrary to the expectation of genetically defined taste neuron types that functionally represent a single stimulus. Consistently, further investigation of Penk+ neuron function revealed that they are more likely to respond to innately aversive stimuli -sour, bitter and high salt concentrations - as compared to the full taste population. Penk+ neurons are less likely to respond to non-aversive stimuli -sucrose, umami, and low salt- compared to the full population. Our data support the presence of a genetically defined neuron type in the geniculate ganglion that is responsive to innately aversive stimuli. This implies that genetic expression might categorize peripheral taste neurons into hedonic groups, rather than simply identifying neurons that respond to a single stimulus.Significance Statement Peripheral taste neuron coding has been heavily debated. Our study delves into this issue by leveraging genetic expression in a specific neuron subset to relate peripheral innervation patterns to functional taste responses. We examined a taste neuron type that appears to be in apposition with multiple taste-transducing cell types and responds to innately aversive concentrations of sour, bitter, and high NaCl stimuli. These collective observations suggest that genetic markers can delineate groups of neurons sharing similar hedonic responses rather than categorizing neurons solely based on individual taste qualities.

Geniculate ganglion diverticulum: a potential imaging marker in patients with idiopathic intracranial hypertension

PurposeThe diagnosis of idiopathic intracranial hypertension (IIH) is often challenging in patients who do not present with classic symptoms. Brain MRI can play a pivotal role, as several imaging findings, such as an empty sella appearance (ESA), have been shown to be associated with IIH. Yet, none of the MRI signs have been shown to have a high sensitivity and specificity. In this study, we tested the hypothesis that presence of a geniculate ganglion diverticulum (GGD) is a potential imaging marker for the detection of IIH. Materials and methodsThis is an IRB-approved, single-institution, retrospective, observational study. Brain MRI examinations of patients referred to Radiology by Otology clinic over a period of 10 years were reviewed. 244 MRI exams fulfilling inclusion and exclusion criteria were independently screened for the presence of GGD and ESA by two Neuroradiology fellows. Electronic medical records (EMR) of patients in this study were reviewed for presence of clinical manifestations of IIH. Receiver operator characteristic (ROC) curves were generated to estimate the accuracy of each covariate in diagnosing IIH. The area under each ROC curve (AUC) was calculated to identify an accurate prognostic covariate. Statistical analysis was done using R programming language V 4.2.2. ResultsGGD was identified in MRI exams of 51 patients. A 2:1 propensity score (PS) matching for age, gender, and Body Mass Index (BMI) was used to select non-GGD control group for comparison with the GGD group. There was strong agreement between the 2 reviewers (kappa = 0.81, agreement = 95 %). Twelve patients in this study were diagnosed with IIH. There was a high incidence of GGD (OR = 12.19, 95 % CI (2.56, 58.10)) and ESA (OR = 4.97, 95 % CI (1.47, 16.74)) in IIH patients. The AUC observed in GGD for predicting IIH was 0.771 (0.655–0.888), specificity = 0.709 (0.638–0.780), and sensitivity = 0.833 (0.583–1). The AUC observed for ESA in predicting IIH was 0.682 (0.532–0.831), specificity = 0.780 (0.709–0.844), and sensitivity = 0.583 (0.333–0.833). ConclusionGGD is potentially a novel imaging marker of IIH with sensitivity higher than and specificity comparable to that of ESA. Clinical relevance statementPresence of GGD should raise the possibility of IIH.

Ramsay Hunt Syndrome: A Rare Disease of Multiple Cranial Nerve Involvement

Ramsay Hunt Syndrome (RHS) also known as herpes zoster oticus is a viral disease, a member of the human herpes virus family, is a late complication of varicella-zoster virus infection that results in inflammation of the geniculate ganglion of cranial nerve VII. Ramsay Hunt is a clinical diagnosis. 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 RHS which is usually accompanied by vestibulocochlear abnormalities. Diagnosis is often missed or delayed, which can lead to an increased incidence of long-term complications. The condition is self-limiting, but treatment is targeted at decreasing the total duration of the illness as well as providing analgesia and preventing the complications that can occur. This activity reviews the role of the inter professional team in the diagnosis and treatment of RHS. Mugda Med Coll J. 2024; 7(1): 48-52

Surgical results for one-stage VII/VIII schwannoma resection and hemihypoglossal-facial neurorrhaphy

BackgroundThis retrospective study evaluated the outcomes of patients undergoing one-stage resection of VII/VIII schwannomas and hemihypoglossal-facial neurorrhaphy via the translabyrinthine approach (TLA). MethodsThe study encompassed ten consecutive patients with unilateral hearing loss (six women, four men, mean age: 49.5 ± 12.1 years) who underwent surgery. The cohort included two patients with vestibular schwannomas (VSs), four with facial nerve schwannomas (FNSs) (two originating from the geniculate ganglion of the facial nerve and two from the cerebellopontine angle), one with VS regrowth, and three with residual VSs. Preoperative facial nerve function, assessed using the House–Brackmann (HB) scale, was Grade V in one and Grade VI in nine patients. The mean preoperative duration of facial paralysis was 7.5 ± 6.9 months. ResultsAll patients underwent gross total resection. Postoperatively, one patient experienced cerebrospinal fluid leaks, which were successfully managed with lumbar drains and surgical revisions. At follow-up, facial nerve function improved in all patients: HB Grade V to III in one, HB Grade VI to III in one, HB Grade VI to IV in seven, and Grade VI to V in one. No tumor recurrence was observed during the follow-up period (mean duration: 16.6 ± 9.3 months), and no patient had hemilingual atrophy. ConclusionThe TLA for one-stage resection of VII/VIII schwannomas and facial nerve reconstruction is effective in treating both regrowth and residual VSs and FNSs in the cerebellopontine angle or petrosal bone in patients with severe preoperative facial palsy. This technique facilitates simultaneous tumor removal and nerve anastomosis, thereby reducing the need for multiple surgical interventions in patients with hearing loss and compromised facial nerve function.

Clinical case of genous schwannoma

Schwannomas are benign tumors that arise from Schwann cells and account for approximately 5% of all benign soft tissue tumors, are characterized by slow growth (1–2 mm per year) and are clinically asymptomatic for a long time.Material and methods. A review of literary sources for 2000–2023 was carried out, sources from the Russian scientific electronic library eLIBRARY, RSCI databases, and PubMed were used. A clinical case of patient with geniculate ganglion schwannoma is presented.Results. Schwannomas can occur at any age, no gender specific characteristics have been identified, the incidence most often occurs between the third and sixth decades, and there is an association with genetic disorders, in particular with neurofibromatosis. The dominant diagnostic methods remain MRI and MSCT of the brain.Conclusion. The presented clinical case demonstrates a long-term, asymptomatic course of geniculate ganglion schwannoma with intracranial spread. The surgical treatment contributed to improving the patient’s quality of life.

Detailed Radiomorphometric Analysis of the Surgical Corridor for the Suprageniculate Approach.

The suprageniculate fossa (SGF) is located between the geniculate ganglion, the middle cranial fossa (MCF) and the anterior semicircular canal (ASCC). An endoscopic transcanal approach has been recently proposed to treat the different lesions in this area. The aim of the study is to describe the anatomical pathway of this approach by measuring the dimensions of its boundaries while checking their correlation with the pneumatization of the SGF area. This is a retrospective anatomical analysis of Cone Beam CT scans of 80 patients, for a total of 160 temporal bones analyzed. Two checkpoints were measured for the SGF route, as an internal and an external window. These are triangles between the MCF dura, the geniculate ganglion and the ASCC on parasagittal and axial planes. The pneumatization of the SGF was also assessed, classified and correlated with the measured dimensions. The depth of the SGF was 7.5 ± 1.8 mm. The width of the external window was 7.5 ± 1.9, 5.6 ± 2.4 and 1.6 ± 1.6 mm for the posterior, middle and anterior points of measurement, respectively. The height of the internal window was 7.6 ± 1.2, 4.5 ± 1.5 and 1.7 ± 1.7 mm for the posterior, middle and anterior points of measurement, respectively. Type A pneumatization was found in 87 cases, type B in 34 and type C in 39. The degree of pneumatization directly correlated to the depth and height of the fossa. The suprageniculate approach route is defined by the internal and external windows which should be evaluated during a pre-surgery imaging assessment. The detailed anatomy of the approach and the novel classification of the pneumatization of the SGF are here described which may be useful to plan a safer procedure with minimal complications.

Diagnosis and treatment of 68 cases of traumatic facial nerve paralysis

Objective:To summarize the clinical characteristics and therapeutic effect of traumatic facial nerve palsy. Methods:Sixty-eight cases of traumatic facial nerve palsy were retrospectively analyzed from January 2015 to May 2023. Results:The median course of disease was 33 days. The facial nerve function of the patients was grade HB-Ⅱin 2 cases, grade HB-Ⅲ in 4 cases, grade HB-Ⅳin 16 cases, grade HB-Ⅴ in 37 cases（38 ears）, and grade HB-Ⅵ in 9 cases. 42 cases occurred immediately after injury and 26 cases were delayed. CT examination of temporal bone revealed longitudinal fractures in 51 cases（52 ears） , transverse fractures in 6 cases and mixed fractures in 4 cases. No definite temporal bone fracture was found in the remaining 7 cases. The segments of facial nerve injury in 49 cases（50 ears） were geniculate ganglion and adjacent, in 7 cases were vertical segment, in 7 cases were horizontal segment, in 2 cases were horizontal segment and vertical segment; and the other 3 cases could not be evaluated. Conservative treatment with glucocorticoids was used in 23 ears and surgery was used in 46 ears. Patients were followed up 6-24 months after treatment, including 20 cases of grade HB-Ⅰ, 19 cases of grade HB-Ⅱ, 23 cases（24 ears） of grade HB-Ⅲ, 4 cases of grade HB-Ⅳ, and 1 case of grade HB-Ⅴ.One patient was lost to follow-up. After treatment, the facial nerve function of patients was significantly improved（P<0.05）, and there were significant differences between conservative treatment group and surgical treatment group in the course of facial nerve palsy, the ratio of facial palsy immediately after injury, the nerve function before treatment and the nerve function after treatment（P<0.05）. There were no significant differences in age, sex, hearing condition, temporal bone fracture, facial nerve injury segment and rate of favorable neurologic outcomes（P>0.05）. The comparison of patients with neurodegeneration rate>90% and ≤90% showed that the facial nerve function of patients with neurodegeneration rate>90% before treatment was significantly worse（P<0.05）, but there was no significant difference between the facial nerve function after treatment（P>0.05）. There was no significant difference in facial nerve function between middle fossa approach group and mastoid approach group（P>0.05）. Conclusion:Patients with traumatic facial nerve palsy should be evaluated individually. Patients with mild facial nerve palsy, low neurodegeneration rate and short course of disease can be treated conservatively and followed up closely. Patients with severe facial nerve palsy, high neurodegeneration rate and more than 6 weeks of disease can be actively considered surgery. Good prognosis can be obtained by correct evaluation and treatment.

Analysis of clinical manifestations and imaging features of facial nerve schwannomas

Objective:To explore the clinical manifestations and imaging characteristics, and to clarify the imaging value in the diagnosis of facial nerve schwannomas. Methods:Retrospectively analyze the data of 23 patients with facial nerve schwannomas confirmed by surgery and pathology in the Department of Otorhinolaryngology of the First Affiliated Hospital of the Air Force Military Medical University from September 2020 to September 2022, including 8 males and 15 females, aged 18-66 years old. Summarize and analyze their clinical symptoms, specialized examinations, and imaging findings. Results:The clinical manifestations were facial nerve paralysis in 15 cases（2 cases of HB Ⅳ, 6 cases of HB Ⅴ, 7 cases of HB Ⅵ）, hearing loss in 14 cases（5 cases of conductive deafness, 2 cases of mixed deafness, and 7 cases of severe sensorineural hearing loss）, 8 cases tinnitus, 7 cases ear pain, 4 cases dizziness, 4 cases headache, 2 cases ear pus, and parotid gland tumors in 6 cases presenting as local masses. Endoscopic examination revealed 8 cases of external ear canal tumors and 3 cases of intratympanic tumors. Combining temporal bone HRCT, MRI enhanced scanning, and CPR imaging techniques, 1 case involved the internal auditory canal segment, 2 cases in the tympanic segment, 6 cases in the parotid gland area. A total of 14 cases involved two or more segments of the internal auditory canal segment, the labyrinthine segment, geniculate ganglion, the tympanic segment, and the mastoid segment. When the tumors were large, adjacent structures were involved. It was found that 8 cases invaded the external auditory canal and tympanic cavity, ossicles were displaced or bony destruction; 3 cases invaded the jugular foramen area, and 1 case grew to the middle cranial fossa region with temporal lobe brain parenchymal compression. Conclusion:The clinical manifestations of facial nerve schwannomas are diverse. The combination of various imaging techniques will be conducive to topical and qualitative diagnosis and provide an important basis for treatment strategies.

Clinical characteristics and prognosis of two anastomosis techniques in the treatment of facial nerve defects

Objective:To investigate the characteristics and prognosis of two anastomosis techniques in repairing facial nerve defects. Methods:A retrospective analysis was conducted on 30 patients who underwent facial nerve anastomosis（direct or rerouting） for facial nerve defects in our department from January 2012 to December 2021. Among them, 21 were male and 9 were female, with an average age of（37.53±11.33） years, all with unilateral onset. Preoperative House-Brackmann（H-B） facial nerve function grades were Ⅳ in 2 cases, Ⅴ in 9 cases, and Ⅵin 19 cases. The duration of facial paralysis before surgery was within 6 months in 21 cases, 6-12 months in 6 cases, and over 1 year in 3 cases. The causes of facial paralysis included 14 cases of cholesteatoma, 6 cases of facial neurioma, 6 cases of trauma, and 4 cases of middle ear surgery injury. Surgical approaches included 9 cases of the middle cranial fossa approach, 8 cases of labyrinthine-otic approach, 7 cases of mastoid-epitympanum approach, and 6 cases of retroauricular lateral neck approach. Results:All patients were followed up for more than 2 years. The direct anastomosis was performed in 10 cases: 6 cases with defects located in the extratemporal segment and 4 cases in the tympanic segment. Rerouting anastomosis was performed in 20 cases: 11 cases with defects located in the labyrinthine-geniculate ganglion, 4 cases from the internal auditory canal to the geniculate ganglion, 3 cases in the internal auditory canal, and 2 cases in the horizontal-pyramid segment. Postoperative H-B facial nerve grades were Ⅱ in 2 cases, Ⅲ in 20 cases, and Ⅳ in 8 cases, with 73.3%（22/30） of patients achieving H-B grade Ⅲ or better. Conclusion:Both direct and rerouting anastomosis techniques can effectively repair facial nerve defects, with no significant difference in efficacy between the two techniques. Most patients can achieve H-B grade Ⅲ or better facial nerve function recovery. Preoperative facial nerve function and duration of facial paralysis are the main prognostic factors affecting the outcome of facial nerve anastomosis.

Peripheral Facial Palsy in Geniculate Herpes Zoster Revealing HIV Infection: A Case Report

Herpes zoster of the geniculate ganglion is characterized by severe paroxysmal ear pain accompanied by an erythematous vesicular rash involving the external auditory canal. Most people with herpes zoster have associated immunodepression of any type. We report a case of PFP in a patient living with HIV during geniculate herpes zoster.

Aberrant Course of the Intratemporal Facial Nerve in Children with Congenital Hearing Loss.

Congenital facial nerve (FN) malformations are uncommon. Our aim is to determine the clinical and radiological features of FN malformations along with the associated cochlear and vestibular malformations. We conducted a retrospective study including children with a profound sensorineural hearing loss who were candidates for cochlear implantation. We evaluated the presence of FN malformations through temporal bone computed tomography scan and magnetic resonance imaging. We recorded an aberrant FN course in five out of 165 patients in a total of 9 ears. They consisted of a bifurcation of the mastoid segment, an anterior or posterior displacement of the labyrinthine segment and a hypoplasia of the geniculate ganglion. Associated inner ear malformations included vestibular aqueduct dilation, cochlear hypoplasia and total labyrinthine aplasia. We noted a bilateral agenesis of the cochlear nerve in three patients. Facial nerve malformations should be suspected in patients presenting a congenital hearing loss especially in association with other temporal bone malformations. Their pre-operative discovery is helpful in planning the surgical procedure.

Combined microscope–endoscopy resection of petrous bone cholesteatoma with temporary facial nerve transposition versus nontransposition

PurposeThe narrow supralabyrinthine space affects surgical procedures. To study the effect of temporary transposition of geniculate ganglion of facial nerve versus nontransposition on lesion recurrence and facial nerve function in patients with petrous bone cholesteatoma.MethodsA total of 18 patients with petrous bone cholesteatoma involving the facial nerve were treated in our hospital from November 2016 to March 2023. The main surgical method is the extended supralabyrinthine approach assisted by a microscope and an endoscope. We collected and retrospectively analyzed their medical records.ResultsTemporary facial nerve transposition was performed in five patients, and nontransposition was performed in 13 patients. Cholesteatoma recurred in three patients with facial nerve nontransposition, whereas none in patients with facial nerve transposition. In this study, except for one case with a second operation, postoperative facial paralysis in other cases was improved to varying degrees, and there was no significant difference between the two groups.ConclusionTemporary transposition of geniculate ganglion of facial nerve will not affect the postoperative nerve function of patients and can reduce the possibility of cholesteatoma recurrence of the petrous bone.

Transcanal Endoscopic Facial Nerve Decompression in Posttraumatic Facial Paralysis: A Tertiary Care Experience

Purpose: Facial nerve decompression (FND) for traumatic facial palsy with lesion at geniculate ganglion and tympanic segment can be done through transcanal approach, by a microscope or an endoscope without opening the mastoid. The study analyzes the outcomes of transcanal endoscopic approach (TEA) for FND with regard to improvement in facial nerve (FN) function and hearing status, in lesions limited to perigeniculate and tympanic segment of FN. Methodology: This was a retrospective analysis of the case series from a tertiary care center, of the patients with posttraumatic FN paralysis, surgically treated with TEA. From January 2014 to December 2018, nine patients of traumatic facial palsy were selected for surgery. The patients posted for surgery were selected only when they had immediate-onset FN paralysis after trauma and not responding to treatment of systemic corticosteroid for 14 days with, House Brackmann grading (HBG) IV–VI, lesions confined to perigeniculate ganglion and tympanic segment of FN. Outcome analysis following TEA for FND was performed in terms of postoperative FN function and hearing improvement. Results: The TEA showed a postoperative improvement of FN function (HBG I/II) in 77.7% of patients within 6-month follow-up period. There was no significant improvement in FN function between 6th and 12th month. Postoperative hearing improvement was seen in eight cases, and there was no improvement in one case due to preoperative sensorineural hearing loss. Conclusion: TEA for FND is a direct approach for lesions confined to perigeniculate ganglion area and tympanic segment without involvement of mastoid segment of FN. It requires minimal bone drilling, provides proper visualization, and accurate surgical site assessment. Surgeries performed even in late presentations may have favorable outcomes.

Distinct expression patterns of Hedgehog signaling components in mouse gustatory system during postnatal tongue development and adult homeostasis.

The Hedgehog (HH) pathway regulates embryonic development of anterior tongue taste fungiform papilla (FP) and the posterior circumvallate (CVP) and foliate (FOP) taste papillae. HH signaling also mediates taste organ maintenance and regeneration in adults. However, there are knowledge gaps in HH pathway component expression during postnatal taste organ differentiation and maturation. Importantly, the HH transcriptional effectors GLI1, GLI2 and GLI3 have not been investigated in early postnatal stages; the HH receptors PTCH1, GAS1, CDON and HHIP, required to either drive HH pathway activation or antagonism, also remain unexplored. Using lacZ reporter mouse models, we mapped expression of the HH ligand SHH, HH receptors, and GLI transcription factors in FP, CVP and FOP in early and late postnatal and adult stages. In adults we also studied the soft palate, and the geniculate and trigeminal ganglia, which extend afferent fibers to the anterior tongue. Shh and Gas1 are the only components that were consistently expressed within taste buds of all three papillae and the soft palate. In the first postnatal week, we observed broad expression of HH signaling components in FP and adjacent, non-taste filiform (FILIF) papillae in epithelium or stroma and tongue muscles. Notably, we observed elimination of Gli1 in FILIF and Gas1 in muscles, and downregulation of Ptch1 in lingual epithelium and of Cdon, Gas1 and Hhip in stroma from late postnatal stages. Further, HH receptor expression patterns in CVP and FOP epithelium differed from anterior FP. Among all the components, only known positive regulators of HH signaling, SHH, Ptch1, Gli1 and Gli2, were expressed in the ganglia. Our studies emphasize differential regulation of HH signaling in distinct postnatal developmental periods and in anterior versus posterior taste organs, and lay the foundation for functional studies to understand the roles of numerous HH signaling components in postnatal tongue development.

A Case of Ramsay Hunt Syndrome Showing Central Findings due to Brainstem Involvement

Ramsay Hunt syndrome occurs when the varicella zoster virus reactivates. Classic findings include the triad of facial paralysis, otic pain and herpetic lesions due to the pathogenesis associated with anterograde axonal reactivation of the varicella zoster virus in the geniculate ganglion. In addition to the classic triad, rare features such as a central type of vestibular function test may be observed due to the retrograde spread of the varicella zoster virus from the geniculate ganglion into the brain stem, including involvement of the vestibular nucleus. We present a case of Ramsay Hunt syndrome in a 57-year-old male patients, manifesting not only the typical triad of symptoms but also the unique features associated with brain stem involvement. This presented as direction-changing gaze-evoked nystagmus and a decrease in gain on both sides on video head impulse test. And brain magnetic resonance imaging showed a lesion in the vestibular nucleus of the brain stem.

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.

Contrast-enhanced MRI findings of patients with acute Bell palsy within 7 days of symptom onset: A retrospective study.

The brain magnetic resonance imaging (MRI) findings seen in patients with Bell palsy are abnormal contrast enhancement of affected facial nerves. Previous studies were conducted on a few patients, mainly those who had experienced palsy for several weeks. This study investigated the diagnostic usefulness of MRI by examining MRI findings of acute Bell palsy (within 7 days of symptom onset) in a large cohort. Among the patients with Bell palsy (idiopathic unilateral facial palsy) who visited the hospital, 123 patients who underwent contrast-enhanced MRI of the internal auditory canal within 7 days of symptom onset were reviewed retrospectively. MRI examination results were investigated along with the patient's clinical symptoms and electrodiagnostic test results. Based on the MRI results, the frequency of abnormal contrast enhancement and contrast-enhanced areas were investigated. Of the 123 patients, 13 (11%) had normal brain MRI results, and 110 (89%) had abnormal findings. The frequency of abnormal contrast enhancement was not significantly associated with test timing (P = .56). Of the 110 patients with abnormal findings, 65 (59%) showed contrast enhancement in the labyrinthine segment and 36 (33%) in both the labyrinthine segment and geniculate ganglion. Most patients with Bell palsy who are in the acute phase showed abnormal contrast enhancement in their facial nerves, and similar findings were even observed in the examination conducted on the day of symptom onset. Brain MRI helps in the diagnosis of acute Bell palsy.

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.