Features of Audio-Vestibular Deficit and 3D-FLAIR Temporal Bone MRI in Patients with Herpes Zoster Oticus.

Herpes zoster oticus (HZO) is characterized by otalgia and erythematous vesicles in the auricle or external auditory canal. Ramsay Hunt syndrome (RHS) can be diagnosed when facial nerve palsy is accompanied by these symptoms of HZO, and in this case, audio-vestibular symptoms such as hearing loss or dizziness often develop. Recently, 3D-fluid-attenuated inversion recovery sequence (3D-FLAIR) magnetic resonance imaging (MRI) has been introduced in order to evaluate the inner ear structure pathology. The purpose of this study was to investigate the audio-vestibular characteristics in correlation with temporal bone MRI findings in HZO patients. From September 2018 to June 2022, 18 patients with HZO participated in the study. Thirteen patients (77%) showed high-signal intensity in the inner ear structures in 4 h post-contrast 3D-FLAIR images. In a bithermal caloric test, the lateral semicircular canal showed high signal intensity in 4 h post-contrast 3D-FLAIR images in 75% of patients with abnormal canal paresis. While the cochlea showed high signal intensity in 4 h post-contrast 3D-FLAIR images in 75% of patients with hearing loss, the vestibulo-cochlear nerve showed enhancement in post-contrast T1-weighted images in only 33% of patients with hearing loss. The present study demonstrates that audio-vestibular deficits are well-correlated with increased signal intensity of the inner ear endorgans in 4 h post contrast 3D-FLAIR MRI.

Mastoid effusion on temporal bone MRI in patients with Bell's palsy and Ramsay Hunt syndrome.

This study aimed to investigate the incidence of mastoid effusion on temporal bone magnetic resonance imaging (MRI) in patients with Bell's palsy (BP) and Ramsay Hunt syndrome (RHS), and evaluate the usefulness of mastoid effusion in early differential diagnosis between BP and RHS. The incidence of mastoid effusion on 3.0 T-temporal bone MRI, which was conducted within 10 days after the onset of acute facial nerve palsy, was compared between 131 patients with BP and 33 patients with RHS. Findings of mastoid cavity on temporal bone MRI were classified into three groups as normal mastoid, mastoid effusion, and sclerotic change, and the incidence of ipsilesional mastoid effusion was significantly higher in RHS than BP (P < 0.001). Tympanic membrane was normal in 7 of 14 RHS patients with mastoid effusion, and injected without middle ear effusion in 7 patients. This study highlights significantly higher incidence of ipsilesional mastoid effusion in RHS than BP, and suggests that the presence of mastoid effusion may provide additional information for differential diagnosis between RHS and BP.

Electroneurography value as an indicator of high risk for the development of moderate-to-severe synkinesis after Bell's palsy and Ramsay Hunt syndrome.

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

Atypical clinical manifestations of herpes zoster oticus: diagnostic usefulness of magnetic resonance imaging.

Typical symptoms of Ramsay Hunt syndrome (RHS) consist of painful vesicular eruptions in the external ear, unilateral facial palsy, and/or vestibulocochlear deficit. When RHS patients show atypical clinical manifestations, correct diagnosis can be delayed, and ideal treatment timing for antiviral therapy may be missed. The aim of this study is to describe RHS patients with atypical clinical manifestations and evaluate the usefulness of magnetic resonance imaging (MRI) for early differential diagnosis. We retrospectively reviewed the clinical data and investigated the findings of internal auditory canal (IAC) MRI of seven patients diagnosed with RHS presenting "atypical" clinical manifestations between January 2013 and December 2016. "Typical" symptoms of RHS consist of herpetic vesicular eruption and facial palsy with or without vestibulocochlear deficit. Regardless of symptomatic presentations, IAC MRI demonstrated post-contrast enhancement of cranial nerve (CN) VII, CN VIII, and IAC dura in patients with atypical clinical manifestations. In cases with multiple lower CN palsy, enhancement along the involved nerve was observed on IAC MRI. When RHS was complicated by acute parotiditis, diffuse enhancement of the parotid gland was demonstrated. The present study shows that in IAC MRI of RHS patients with atypical clinical manifestations, post-contrast enhancement was not confined to the facial nerve but also observed in CN VIII and IAC dura regardless of the symptoms, which may facilitate early diagnosis of RHS.

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

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

Ramsay Hunt syndrome associated with solitary nucleus, spinal trigeminal nucleus and tract, and vestibular nucleus involvement on sequential magnetic resonance imaging.

Ramsay Hunt syndrome (RHS) is an acute peripheral facial nerve paralysis typically accompanied by erythematous vesicular lesions of the auricular skin. The etiology is considered to be geniculate ganglionitis due to reactivation of varicella-zoster virus (VZV). Encephalitis is a rare but serious complication of VZV reactivation. Clarifying the regional and temporal evolution of the lesions on magnetic resonance imaging (MRI) would help with understanding the pathology of the lesion, but this information is lacking in encephalitis with RHS. Therefore, here, we reviewed sequential MR images in three RHS cases complicated by brainstem lesions. All the regions of the lesions represent specific neuronal structures-the ipsilateral solitary nucleus (SN) and spinal trigeminal nucleus and tract (STNT) in case 1; bilateral SN, ipsilateral STNT, and vestibular nucleus in case 2; ipsilateral SN and vestibular nucleus in case 3-and this seems to account for the persistent robust symptoms. Case 1 initially showed no abnormalities on MRI and cases 2 and 3 showed weak signals on the first MRI which subsequently plateaued. These observations suggest the timeframe within which it becomes possible to detect regional and temporal evolution, namely, that the distribution of the affected regions expands between weeks 2 and 5 after onset of facial paralysis. These observations and the findings of a literature review indicate that the SN, STNT, and vestibular nucleus are relatively prone to developing encephalitis after RHS.

Usefulness of High-Resolution 3D Multi-Sequences for Peripheral Facial Palsy: Differentiation Between Bell's Palsy and Ramsay Hunt Syndrome.

OBJECTIVE: To investigate the usefulness of magnetic resonance imaging (MRI) including three-dimensional (3D) sequences in the differentiation between Bell's palsy (BP) and Ramsay Hunt syndrome (RHS). STUDY DESIGN: A prospective study. SETTING: Tertiary care center. PATIENTS: Twenty patients: 15 patients with BP and five patients with RHS. INTERVENTION: Diagnostic. MAIN OUTCOME MEASURE: Clinical diagnosis (BP or RHS). RESULTS: The presence of hyperintensity on 3D-fluid-attenuated inversion recovery sequence (3D-FLAIR) and enhancement on gadolinium-enhanced (CE)-3D-FLAIR and CE-3D-T1-weighted image (3D-T1WI) along the internal auditory canal (IAC) wall were significantly associated with RHS (p < 0.05). Hyperintensity in the inner ear was observed on pre- and postcontrast 3D-FLAIR, and enhancement of the cranial nerve (CN)-VIII was observed only on CE-3D-FLAIR. The presence of these findings also showed significant relationships with RHS (p < 0.05). Moreover, thickening of the CN-VII in the fundus of the IAC in 3D-constructive interference on steady state sequence (3D-CISS) also showed a significant association with RHS (p < 0.05). In contrast, the presence of hyperintensity of the CN-VII in the fundus of the IAC on 3D-FLAIR did not demonstrate a significant relationship (p = 0.95), and enhancement in this region was observed in all cases on CE-3D-FLAIR and gadolinium-enhanced-three-dimensional-T1-weighted gradient echo sequence (CE-3D-T1WI). CONCLUSIONS: 3D MRI sequences are useful for differentiating RHS from BP. In particular, the enhancement in the CN-VIII and/or along the IAC wall are valuable findings, and CE-3D-FLAIR is the most useful sequence to evaluate these findings. Thickening of the CN-VII on 3D-CISS is also an important finding.

Slowly progressing varicella zoster brainstem encephalitis complicating Ramsay Hunt syndrome in an immunocompetent patient: case report and review of the literature.

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

Facial palsy and atrial fibrillation: a special case of Ramsay Hunt syndrome.

Ramsay Hunt syndrome (herpes zoster oticus) is a rare complication of latent Varicella Zoster virus infection. It can be complicated by permanent hearing loss, loss of taste and postherpetic neuralgia. Although Ramsay Hunt syndrome most prominently involves the facial nerve, a number of other cranial nerves can be involved such as the vestibulocochlear, glossopharyngeal and the vagus nerve. We report on a case of Ramsay Hunt syndrome with cranial polyneuritis complicated by atrial fibrillation. Vagal involvement as evidenced by physical examination and MRI findings was present in our patient. We hypothesise that viral vagal neuritis likely contributed to the development of atrial fibrillation in our patient through involvement of the autonomic components of the vagus nerve.

Geniculate neuralgia: clinical, radiologic, and intraoperative correlates.

OBJECTIVE: Geniculate neuralgia is an uncommon pain syndrome that can be severe and disabling and is difficult to diagnose. METHODS: The literature was reviewed for geniculate neuralgia, including anatomy, presentation, and treatment. A case illustration was presented that demonstrates the novel brainstem functional imaging findings for geniculate neuralgia. A 39-year-old man presented with a history of left "deep" ear pain within his ear canal. He noted occasional pain on the left side of his face around the ear. He had been treated with neuropathic pain medications without relief. His wife described suicidal ideations discussed by her husband because of the intense pain. RESULTS: The patient's neurologic examination was normal, and otolaryngologic consultation revealed no underlying structural disorder. Anatomic imaging revealed a tortuous vertebral artery-posterior inferior cerebellar artery complex with the posterior inferior cerebellar artery loop impinging on the root entry zone of the nervus intermedius-vestibulocochlear nerve complex and just inferior to the root entry zone of the facial nerve and a small anterior inferior cerebellar artery loop interposed between the cranial nerve VII-VIII complex and the hypoglossal and glossopharyngeal nerves. A left-sided retromastoid craniotomy was performed, and the nervus intermedius was transected. An arterial loop in contact with the lower cranial nerves at the level of the brainstem was mobilized with a polytetrafluoroethylene implant. CONCLUSIONS: The patient indicated complete relief of his preoperative pain after surgery. He has remained pain-free with intact hearing and balance.