Radiological and intraoperative findings of a rare case of vestibular paroxysmia.

Vestibular paroxysmia (VP) is a rare condition. The pathogenesis is linked to a neurovascular conflict (NVC) between an abnormal arterial loop and the VII/VIII cranial nerve complex in the cerebello-pontine angle. Due to its rarity, intraoperative findings are only anecdotally reported. Here we reported on a case of VP, showing the radiological images and the intraoperative surgical video of microvascular decompression (MVD). Further we discussed our findings considering the pertinent literature. We think that in case of VP the concordance between the side of tinnitus/hypoacusia and the side of NVC on magnetic resonance imaging should be always looked for before considering MVD as a therapeutic option.

Abnormal Rhomboid Lip and Choroid Plexus Should be Valued in Microvascular Decompression for Vestibulocochlear Diseases.

BACKGROUND: Surgical outcomes for functional vestibulocochlear diseases vary, and the influencing factors are not fully understood. The role of a rhomboid lip (RL) and choroid plexus (CP) in microvascular decompression (MVD) of the vestibulocochlear nerve has not been studied. This study aims to evaluate the surgical efficacy of MVD for vestibulocochlear diseases, with and without addressing the RL and CP, to enhance our understanding of their etiology. METHODS: A total of 15 patients who underwent MVD for the vestibulocochlear nerve between 2013 and 2022 were retrospectively identified and followed up. The patients were classified into 4 categories: vestibular paroxysmia (VP), benign positional paroxysmal vertigo (BPPV), and Meniere disease (MD). The fourth was a "tinnitus" group. The relief of symptoms, recurrence, satisfaction after surgery, available relevant imaging studies, and intraoperative observation data were evaluated. RESULTS: Following MVD, 6 of the 7 patients in the VP group, the 1 patient in the BPPV group, and 1 of 2 patients in the MD group were completely relieved of vertigo. The seventh VP patient showed significant improvement. The 5 patients in the "tinnitus" group remained unchanged. Retrospectively, 4 patients from the VP, BPPV, and MD groups who underwent RL incision and CP excision were also free of vertigo, although vascular compression was not confirmed in these cases. CONCLUSIONS: MVD is generally considered a useful treatment for VP and could also be effective in managing recurrent vertigo caused by BPPV and MD. Our results highlight the potential role of an abnormal RL and CP in the development of vertigo symptoms. Patients presenting with "tinnitus" require further investigation and might not be suitable for MVD.

The "Near"-Narrowed Internal Auditory Canal Syndrome in Adults: Clinical Aspects, Audio-Vestibular Findings, and Radiological Criteria for Diagnosis.

INTRODUCTION: Vestibular Paroxysmia (VP) refers to short attacks of vertigo, spontaneous or triggered by head movements, and implies the presence of a compressive vascular loop in contact with the cochleovestibular nerve (CVN). Classically, a narrowed internal auditory canal (IAC) corresponds to a diameter of less than 2 mm on CT, usually associated with a hypoplastic CVN on MRI. The aim of this study was to discuss a distinct clinical entity mimicking VP in relation to a "near"-narrowed IAC (NNIAC) and to propose radiological criteria for its diagnosis. METHODS: Radiological measurements of the IAC were compared between three groups: the study group (SG, subjects with a clinical presentation suggestive of VP, but whose MRI of the inner ear and pontocerebellar angle excluded a compressive vascular loop) and two control groups (adult and children) with normal vestibular evaluations and no history of vertigo. RESULTS: 59 subjects (18 M and 41 F) were included in the SG. The main symptoms of NNIAC were positional vertigo, exercise- or rapid head movements-induced vertigo, and dizziness. The statistical analysis in the study group showed that the threshold values for diagnosis were 3.3 mm (in tomodensitometry) and 2.9 mm (in MRI) in coronal sections of IAC. Although a significantly lower mean value for axial IAC diameter was found in SG compared with controls, the statistics did not reveal a threshold due to the large inter-individual variations in IAC measurements in normal subjects. There was no significant difference in IAC diameter between the adult and pediatric controls. CONCLUSIONS: In the present study, we report a new anatomopathological condition that appears to be responsible for a clinical picture very similar-but not identical-to VP in association with the presence of an NNIAC. The diagnosis requires a careful analysis of the IAC's shape and diameters in both axial and coronal planes.

[Dolichoectasia of the basilar artery caused by cystic medial degeneration, as a cause of neurovascular conflict with damage to the trigeminal, facial and vestibulocochlear nerves]. / Dolikhoektaziya bazilyarnoi arterii, obuslovlennaya kistoznoi medial'noi degeneratsiei, kak prichina neirovaskulyarnogo konflikta s porazheniem troinichnogo, litsevogo i vestibulokokhlearnogo nervov.

Cystic medial degeneration (Gsell-Erdheim syndrome, cystic medial necrosis) is considered to be a nonspecific histological manifestation of a group of diseases characterized by degenerative changes in the media, affecting primarily the aorta and adjacent branches, which leads to destruction of the vessel wall, followed by its expansion and, possibly, rupture. The authors describe a case of a 65-year-old female patient with a neurovascular conflict of the three cranial nerves with dolichoectatic basilar artery due to cystic medial degeneration. As a result, the patient has clinical manifestations in the form of hemifacial spasm, trigeminal neuralgia and vestibular paroxysmia. Data from instrumental studies and treatment provided are presented. Neurovascular conflict can be identified in various diseases and is characterized by the complex etiology. The most common clinical manifestations of neurovascular conflict are trigeminal neuralgia, hemifacial spasm, glossopharyngeal neuralgia, and vestibular paroxysmia.

Vestibular paroxysmia associated with congenital vascular malformation: A case report.

Vestibular paroxysmia (VP) is an uncommon paroxysmal disease, characterized by vertigo, tinnitus, and postural unsteadiness. The main reason of VP is neurovascular cross compression, while few cases of VP accompanied with congenital vascular malformation were reported. Here, we describe a 22-year-old patient with VP caused by congenital anterior inferior cerebellar artery (AICA) malformation who completely recovered after taking oral medicine. This report shows that VP caused by congenital vascular malformation can occur in adults and that oral medication is effective.

Correlation of Preoperative High-Resolution Neurovascular Imaging and Surgical Success in Neurovascular Compression Syndromes.

BACKGROUND: Neurovascular compression syndromes (NVCSs) are a group of neurological disorders characterized by compression of a cranial nerve and include trigeminal neuralgia (TN), hemifacial spasm, geniculate neuralgia, glossopharyngeal neuralgia, vestibular paroxysmia, and trochlear palsy. The European Academy of Neurology recommends constructive interference in steady-state/fast imaging employing steady-state (CISS/FIESTA) magnetic resonance imaging (MRI) in the evaluation of medically intractable TN, but similar recommendations do not exist for the remaining NVCSs. METHODS: We conducted a retrospective chart review of 300 patients with an NVCS who underwent microvascular decompression (MVD) by a single neurosurgeon from 2004 to 2021. Data were collected on preoperative diagnosis, pre- and postoperative symptoms, presence/absence of preoperative high-spatial-resolution CISS/FIESTA MRI, and intraoperative findings. Rates of symptomatic improvement were used as a correlate of surgical success. RESULTS: The rate of symptomatic improvement in the patients with a preoperative CISS/FIESTA MRI was 5.8% greater than those without preoperative high-spatial-resolution neuroimaging (98.8% vs. 93%, respectively; P = 0.008). Stratified by diagnosis, patients with TN had the greatest difference in surgical success between the 2 groups (99.3% vs. 92.9%, n = 268; P = 0.006). No statistically significant differences were observed in the other NVCSs, although positive trends were noted. CONCLUSIONS: Preoperative CISS/FIESTA MRI correlated with greater rates of surgical success in cases of medically intractable TN; however, definitive conclusions could not be made regarding the remaining NVCSs. We support the recommendation that this imaging modality be included as part of the standard of practice for the evaluation and management of TN and encourage future studies to further elucidate this relationship for the less common NVCSs using a larger cohort.

Vestibular paroxysmia entails vestibular nerve function, microstructure and endolymphatic space changes linked to root-entry zone neurovascular compression.

Combining magnetic resonance imaging (MRI) sequences that permit the determination of vestibular nerve angulation (NA = change of nerve caliber or direction), structural nerve integrity via diffusion tensor imaging (DTI), and exclusion of endolymphatic hydrops (ELH) via delayed gadolinium-enhanced MRI of the inner ear (iMRI) could increase the diagnostic accuracy in patients with vestibular paroxysmia (VP). Thirty-six participants were examined, 18 with VP (52.6 ± 18.1 years) and 18 age-matched with normal vestibulocochlear testing (NP 50.3 ± 16.5 years). This study investigated whether (i) NA, (ii) DTI changes, or (iii) ELH occur in VP, and (iv) to what extent said parameters relate. Methods included vestibulocochlear testing and MRI data analyses for neurovascular compression (NVC) and NA verification, DTI and ELS quantification. As a result, (i) NA increased NVC specificity. (ii) DTI structural integrity was reduced on the side affected by VP (p < 0.05). (iii) 61.1% VP showed mild ELH and higher asymmetry indices than NP (p > 0.05). (iv) "Disease duration" and "total number of attacks" correlated with the decreased structural integrity of the affected nerve in DTI (p < 0.001). NVC distance within the nerve's root-entry zone correlated with nerve function (Roh = 0.72, p < 0.001), nerve integrity loss (Roh = - 0.638, p < 0.001), and ELS volume (Roh = - 0.604, p < 0.001) in VP. In conclusion, this study is the first to link eighth cranial nerve function, microstructure, and ELS changes in VP to clinical features and increased vulnerability of NVC in the root-entry zone. Combined MRI with NVC or NA verification, DTI and ELS quantification increased the diagnostic accuracy at group-level but did not suffice to diagnose VP on a single-subject level due to individual variability and lack of diagnostic specificity.

Case report: Bitter vertigo.

Background: There are many causes of episodes of vertigo and very few causes of episodes of changes in taste, and the combination of the two is very rare. Here, we describe a patient with recurrent short episodes of vertigo in combination with simultaneous episodes of recurrent paroxysmal dysgeusia and altered feeling on the left side of face. The symptoms were caused by compression of the vestibulocochlear nerve and the facial nerve due to dolichoectasia of the basilar artery. Methods: The patient was diagnosed in our routine clinical practice and underwent a complete neurological and neuro-otological examination, including video head impulse test, caloric irrigation, ocular and cervical vestibular evoked myogenic potentials, acoustic-evoked potentials, neuro-orthoptic examination, cranial MRI, and MR angiography. The patient was seen twice for follow-up. Case: A 71-year-old patient primarily presented with a 2-year history of recurrent short episodes of spinning vertigo. Each of the episodes began with an altered feeling on the left side of the face, followed by a bitter taste on the left half of the tongue, and subsequently vertigo lasting for up to 15 s. The frequency of the attacks was high: up to 80 times per day. Laboratory tests revealed signs of a peripheral vestibular deficit on the left side. There were no signs of sensory or motor deficits or of altered taste between the episodes. An MRI of the brain showed an elongated basilar artery leading to an indentation of the facial and vestibulocochlear nerves on the left side. Conclusion: We propose a neurovascular compression in the proximal part of two cranial nerves because of pulsatile compression by the elongated basilar artery with ephatic discharges as the cause of the recurrent episodes. Consistent with the theory of ephatic discharges, treatment with the sodium channel blocker lacosamide for over six months with a final dosage of 200 mg per day p.o. led to a significant reduction of the attack frequency and intensity. This treatment option with a sodium channel blocker should therefore not only be considered in vestibular paroxysmia but also in cases of paroxysmal dysgeusia.

Vestibular paroxysmia: Long-term clinical outcome after treatment.

Objective: To study the long-term treatment outcome of vestibular paroxysmia (VP). Study design: Retrospective study. Setting: Tertiary referral hospital. Methods: We analyzed records of 29 consecutive patients who were diagnosed with VP and who were treated with VP-specific anticonvulsants for at least 3 months. Patients were followed for a minimum of 6 months. We recorded and assessed starting and target dosage of medications, time to achieve adequate therapeutic response, adverse effects, and the rates of short-term and long-term remission without medication. Results: All 29 patients were started on oxcarbazepine as first-line treatment, and 93.1% and 100% of patients reported good-to-excellent therapeutic response within 2 and 4 weeks, respectively. Three patients switched to other anticonvulsants at 3 months. At long-term follow-up (8-56 months), most (84.6%) oxcarbazepine-treated patients maintained good therapeutic response at doses between 300 and 600 mg/day. Eleven (37.9%) patients experienced complete remission without medication for more than 1 month, of which six (20.7%) had long-term remission off medication for more than 12 months. Nineteen (65.5%) patients had neurovascular compression (NVC) of vestibulocochlear nerve on MRI, but its presence or absence did not predict treatment response or remission. Conclusion: Low-dose oxcarbazepine monotherapy for VP is effective over the long term and is generally well-tolerated. About 20% of patients with VP in our study had long-term remission off medication.

Neurovascular compression syndrome:Trigeminal neuralgia, hemifacial spasm, vestibular paroxysmia, glossopharyngeal neuralgia, four case reports and review of literature.

Neurovascular compression syndrome is caused by vessels touching a cranial nerve, resulting in clinical manifestations of abnormal sensory or motor symptoms. The most common manifestations are trigeminal neuralgia and hemifacial spasm. However, neurovascular compression of the vestibular nerve or glossopharyngeal nerve are rare. In this article, we describe four typical cases of neurovascular compression syndrome. In addition, we analyze the main features of the etiology, neuroimaging, and treatment of this disease.

The Narrowed Internal Auditory Canal: A Distinct Etiology of Pediatric Vestibular Paroxysmia.

Vestibular paroxysmia (VP) is a disorder encountered in the pediatric population that etiology has been attributed to neurovascular cross-compression syndrome (NVCC). The purpose of this study was to report a new probable pathological condition, the narrowed internal auditory canal (IAC), which appears to be involved in the development of a clinical picture of VP in the pediatric population. A retrospective descriptive comparative study was conducted to compare clinical, electrophysiological, radiological, and therapeutic outcomes in both etiologies. Overall, 16 pediatric patients suffering from VP were included and divided into two groups: patients with narrowed internal auditory (Group 1) were compared to those with NVCC syndrome (Group 2). Patients in both groups were similar in terms of auditory complaints, as well as hearing, vestibular, and electrophysiological status. A narrowed IAC was encountered in the adolescent age category and females, especially those with rapid growth. The diagnosis requires a careful analysis of the shape and diameters of the IAC. Radiologic measurements in the axial plane do not seem to be sufficient to confirm the diagnosis, and, therefore, an analysis of diameters in the coronal plane is required. Treatment with sodium-channel blockers drugs showed promising results not only by relieving vertigo but also by normalizing the electrophysiological findings. In conclusion, a narrowed IAC can be considered in patients suffering from VP.

Vestibular paroxysmia: clinical characteristics and long-term course.

In 2016, the Bárány Society defined new diagnostic criteria for the neurovascular compression syndrome of the eighth nerve, called "vestibular paroxysmia" (VP), differentiating between definite (dVP) and probable (pVP) forms. The aim of this study was (1) to describe clinical symptoms and laboratory findings in a well-diagnosed large patient cohort according to those criteria, and (2) to evaluate the long-term course over years in dVP. We identified 146 patients (73 dVP, 73 pVP) from our tertiary dizziness center registry. Data of structured history-taking, clinical neurological, neuro-ophthalmological/-otological examinations as well as MRI imaging were extracted for analyses. Overall, attack frequency ranged between 5 and 30 attacks per day; spinning vertigo was the most frequent type. In two-thirds of patients, attacks occurred spontaneously; in one-quarter, they were triggered by head movements. The majority (approximately 70%) reported no accompanying symptoms; in those with symptoms, mild unilateral cochlear symptoms prevailed. One-third of patients initially showed hyperventilation-induced nystagmus without specific direction, and a deviation of the subjective visual vertical between 3° and 6°. Complete loss of peripheral vestibular function was never evident. dVP and pVP significantly differed concerning the vertigo type, e.g., spinning vertigo was more frequent in dVP. Fortunately, three-quarters of dVP patients remained attack-free during follow-up (mean 4.8 years, standardized questionnaire), more than half of them even without any medication. Patients with ongoing attacks showed significantly higher attack frequency at baseline, but reported persistent frequency reduction. Overall, the long-term prognosis of VP appears favorable, not necessarily requiring ongoing treatment.

Application of ABR in pathogenic neurovascular compression of the 8th cranial nerve in vestibular paroxysmia.

PURPOSE: To investigate the clinical value of electrophysiological tests in indicating pathogenic vascular contact of the 8th nerve in definite vestibular paroxysmia (VP) cases to provide a reference for decompression surgery. METHODS: We retrospectively analyzed patients who had vertigo, unilateral tinnitus, or hearing loss and exhibited vascular contact of the 8th cranial nerve by MRI. Participants were classified into the VP or non-VP group according to the criteria of the Bárány Society in 2016. The demographic characteristics and audiological and electrophysiological test results of the two groups were compared. Receiver operating characteristic (ROC) curves were calculated for ABR to determine the best parameters and cutoff values to predict the existence of pathological neurovascular contact in VP. RESULTS: Thirteen patients in the VP group and 66 patients in the non-VP group were included. VP patients had longer interpeak latency (IPL) I-III and wave III latency compared to non-VP patients (p < 0.001; p < 0.001). According to the ROC analyses, IPL I-III and wave III latency were the best indicators for the diagnosis of VP. The optimal cutoff for IPL I-III was 2.3 ms (sensitivity 84.6%, specificity 95.5%), and that for wave III latency was 4.0 ms (sensitivity 92.3%, specificity 77.3%). There were no differences in the PTA, caloric test, o-VEMP, or c-VEMP results between the two groups. CONCLUSION: Prolonged IPL I-III and the wave III latency of ABR strongly suggested that vascular contact of the 8th cranial nerve was pathological, which may provide some references for microvascular decompression surgery of VP.

Vascular Loops: The Innocent Bystander for Vestibular Paroxysmia.

INTRODUCTION: Vestibular paroxysmia (VP) is a condition with recurrent short bouts of vertigo and is thought to be part of a neurovascular compression syndrome caused by the vascular loop. However, this is still being debated as vascular loops are considered as normal variants with limited studies involving vertiginous patients. OBJECTIVES: The aim of this paper is to study the association between audiovestibular symptoms and the presence of vascular loops and to study the association between vestibular paroxysmia and vascular loops. DESIGN: This is a retrospective analysis of clinical, audiological and MRI findings of patients with and without vascular loops and vestibular paroxysmia from 2000 to 2020. RESULTS: A total of 470 MRI Internal Auditory Meatus scans were performed during the study period of which, 71 (15.1%) had vascular loops and 162 (34.5%) had normal MRI which were used as controls. From the 233 subjects recruited, there were 37 subjects with VP and 196 non VP subjects were used as controls. There was no association between the vascular loop and control groups in terms of co-morbidity and audiovestibular symptoms. The VP group had a significantly older mean age of 51.8 (SD ± 10.3) as compared to the non VP group with the mean age of 45.6 (SD ± 15.5). The VP group had higher number of patients presenting with hearing loss at 97.3% when compared with those without VP (80.1%) (P = .01). The odds of having a vascular loop giving rise to VP was not statistically significant at 0.82 (95% CI 0.3735-1.7989) P = .62. CONCLUSION: The vascular loop is a normal variant which may or may not give rise to audiovestibular symptoms or vestibular paroxysmia. Clinical assessment is still most important tool in deriving a diagnosis of VP and MRI may be useful to rule out other central causes.

Vestibular paroxysmia: Clinical features and imaging findings; a literature review.

According to the definition of neurovascular compression syndromes (NVCS), a vascular structure in direct contact with a cranial nerve is causing mechanical irritation of the neural tissue producing correlating symptoms. Vestibular paroxysmia is an example of a neurovascular compression which is caused by neurovascular contact between the eighth cranial nerve and a vessel. It is crucial to understand the unique anatomy of the vestibulocochlear nerve in order to study the syndrome which is the result of its compression. More specifically, the long transitional zone between central and peripheral myelin plays a central role in clinical significance, as the transitional zone is the structure most prone to mechanical injury. Imaging techniques of the eighth cranial nerve and the surrounding structures are substantial for the demonstration of clinically significant cases and potential surgical decompression. The goal of the current review is to present and study the existing literature on vestibular paroxysmia and to search for the most appropriate imaging technique for the syndrome. An extensive literature search of PubMed database was performed, and the studies were ranked based on evidence-based criteria, followed by descriptive statistics of the data. The present analysis indicates that 3D CISS MRI sequence is superior to any other sequence, in the most studies reviewed, regarding the imaging of neurovascular compression of the eighth cranial nerve.

Cardiogenic vertigo: characteristics and proposed diagnostic criteria.

Early identification of cardiogenic vertigo (CV) is necessary to prevent serious complications of cardiovascular diseases. However, the literature is limited to case reports without detailed clinical features or diagnostic criteria. The aim of this study was to define characteristics of CV and propose diagnostic criteria. This study included patients with CV diagnosed at Pusan National University and Keimyung University Hospitals. Demographic, clinical, laboratory, and treatment data were analyzed. Of 72 patients with clinically suspicious CV, 27 were finally included. The age ranged from 63 to 88 years (75.1 ± 7.2 years). Recurrent vertigo occurred without syncopal attacks in 52% [95% CI, 32-71], while it preceded (37% [19-58]) or followed (11% [2-29]) syncope. The patients with recurrent isolated vertigo had suffered from symptoms from 15 days to 5 years until final diagnosis (median 122 days). The vertigo lasted only for a few seconds (93% [76-99]) or a few minutes (7% [1-24]). Fourteen patients presented with spinning vertigo, and one of them showed spontaneous downbeat nystagmus during the attack. Accompanying symptoms including chest discomfort, palpitation, headache, arm twitching, and lightheadedness were found in 70% [50-86]. Between patients with and without syncope, there was no difference in clinical parameters and results of cardiac function tests. The most common cardiac abnormality during the attacks of vertigo was bradyarrhythmia (89% [71-98]). Cardiovascular diseases can develop recurrent isolated vertigo without or preceding syncope. Onset age, duration of vertigo, accompanying symptoms, and underlying cardiac diseases can aid in differentiation from other vestibular disorders. Early identification of CV would reduce morbidity and mortality associated with cardiac syncope.

Clinical long-term observation of the keyhole microvascular decompression with local anesthesia on diagnosis and treatment of vestibular paroxysmia.

Background: The pathophysiology and etiology of vestibular paroxysmia (VP) remains unclear, moreover, due to the lack of reliable diagnostic features for VP, the clinical diagnosis will be made mainly by exclusion.Aims/objectives: To evaluate the diagnostic value and curative effect of keyhole microvascular decompression with local anesthesia for VP.Material and methods: 54 patients with trigeminal neuralgia, hemifacial spasm and glossopharyngeal neuralgia underwent keyhole microvascular decompression with local anesthesia, twelve of whom were coexistent with VP. The evaluation of the vertigo after operation was performed with symptom report card for 12 patients with VP and the mean follow-up period was 116 months (range 114-118 months).Results: The cochleovestibular neurovascular compression at the root zone of vestibular nerve was found in 12 patients with VP, of whom 11 patients had the neurovascular compressive vertigo induced intra-operatively and the vertigo disappeared postoperatively, moreover, one patient had no neurovascular compressive vertigo induced intra-operatively and the vertigo was not improved significantly after operation. Of 12 patients with VP during the mean 116-month follow-up, 11 patients had no recurrence of neurovascular compressive vertigo and the effective control rate of vertigo was 91.7%.Conclusions and significance: Keyhole microvascular decompression with local anesthesia is not only an effective method for treating VP and controlling neurovascular compressive vertigo, but also has definite clinical significance in the diagnosis of VP.

Unilateral cochleovestibular nerve compression syndrome in a patient with bilateral IAC osteoma.

INTRODUCTION: Osteomas of the internal auditory canal are rarely reported in the literature. Patients may complain of disabling symptoms of dizziness, hearing loss, and vestibular dysfunction. We report the case of a patient with bilateral osteomas of the internal auditory canal (IAC) associated with bilateral neurovascular compression mainly affecting the right cochleovestibular nerve (VIII) and right anterior inferior cerebellar artery (AICA). OBSERVATION: This 75-year-old woman patient complained of disabling paroxysmal vertigo, typewriter tinnitus and hearing loss of the right ear. Temporal bone computed tomography showed bilateral osteoma arising from the posterior superior wall of the IAC. MRI sequences of the right VIII demonstrated compression by the right AICA against the inferior wall of the narrowed IAC. Treatment with oxcarbazepine allowed marked and lasting improvement of the patient's symptoms. CONCLUSION: To our knowledge, this is the first description of an ipsilateral neurovascular compression syndrome of the VIII secondary to the presence of an osteoma narrowing the IAC.