Aberrant cerebral blood flow and functional connectivity in patients with vestibular migraine: a resting-state ASL and fMRI study.

BACKGROUND: Prior neuroimaging studies on vestibular migraine (VM) have extensively certified the functional and structural alterations in multiple brain regions and networks. However, few studies have assessed the cerebral blood flow (CBF) in VM patients using arterial spin labeling (ASL). The present study aimed to investigate CBF and functional connectivity (FC) alterations in VM patients during interictal periods. METHODS: We evaluated 52 VM patients and 46 healthy controls (HC) who received resting-state pseudo-continuous ASL and functional magnetic resonance imaging (fMRI) scanning. Comparisons of voxel-based CBF and seed-based FC were performed between the two groups. Brain regions showed significant group differences in CBF analyses were chosen as seeds in FC analyses. Additionally, the associations between abnormal imaging results and clinical features were explored. RESULTS: Compared with HC, VM patients showed higher normalized CBF in the right precentral gyrus (PreCG), left postcentral gyrus (PostCG), left superior frontal gyrus and bilateral insular (p < 0.05, FDR corrected). Furthermore, VM patients exhibited increased FC between the right PreCG and areas of the left PostCG, left cuneus and right lingual gyrus (p < 0.05, FDR corrected). In addition, we observed decreased FC between the left insular and regions of the left thalamus and right anterior cingulate cortex, as well as increased FC between the left insular and right fusiform gyrus in VM patients (p < 0.05, FDR corrected). Moreover, these variations in brain perfusion and FC were significantly correlated with multiple clinical features including frequency of migraine symptoms, frequency of vestibular symptoms and disease duration of VM (all p < 0.05). CONCLUSIONS: Patients with VM during interictal period showed hyperperfusion and abnormal resting-state FC in brain regions potentially contributed to disrupted multi-sensory and autonomic processing, as well as impaired ocular motor control, pain modulation and emotional regulation. Our study provided novel insights into the complex neuropathology of VM from a CBF perspective.

Suppression of inner ear signal intensity on fluid-attenuated inversion recovery magnetic resonance imaging in cats with vestibular disease.

OBJECTIVES: Otitis media/interna (OMI) is the most common cause of peripheral vestibular disease in cats. The inner ear contains endolymph and perilymph, with perilymph being very similar in composition to cerebrospinal fluid (CSF). As a very-low-protein fluid, it would be expected that normal perilymph should suppress on fluid-attenuated inversion recovery (FLAIR) MRI sequences. Based on this, we hypothesized that MRI FLAIR sequences should provide a non-invasive way of diagnosing inflammatory/infectious diseases such as OMI in cats, something that has previously been demonstrated in humans and, more recently, in dogs. METHODS: This was a retrospective cohort study in which 41 cats met the inclusion criteria. They were placed into one of four groups, based on presenting complaint: clinical OMI (group A); inflammatory central nervous system (CNS) disease (group B); non-inflammatory structural disease (group C); and normal brain MRI (control group; group D). Transverse T2-weighted and FLAIR MRI sequences at the level of the inner ears bilaterally were compared in each group. The inner ear was selected as a region of interest using Horos, with a FLAIR suppression ratio calculated to account for variability in signal intensity between MRIs. This FLAIR suppression ratio was then compared between groups. Statistical analyses were performed by an experienced statistician, with a general linear model used to compare mean FLAIR suppression ratio, CSF nucleated cell count and CSF protein concentration between groups. RESULTS: The OMI group (group A) had significantly lower FLAIR suppression scores compared with all other groups. The CSF cell count was also significantly increased in the OMI (group A) and inflammatory CNS disease (group B) groups compared with the control group (group D). CONCLUSIONS AND RELEVANCE: This study demonstrates the utility of MRI FLAIR sequences in diagnosing presumptive OMI in cats, similarly to in humans and dogs. This study is relevant to practicing veterinary neurologists and radiologists in interpreting MRI findings in cats with suspected OMI.

Using magnetic resonance imaging to improve diagnosis of peripheral vestibular disorders.

Peripheral vestibular disorders are caused by pathology of the inner ear. The majority of these disorders are diagnosed with a detailed history and vestibular physical exam. Imaging is rarely a part of diagnostic work up as the pathologies of these disorders are "invisible," or undetectable on imaging. However, these "invisible" diagnoses are becoming increasingly visible with advancements in imaging technology. Developments in magnetic resonance imaging are allowing for increased spatial resolution and better image contrast, improving our ability to see soft tissue structures including the membranous labyrinth, sensory epithelia and nerves. With these improvements in imaging, clinicians will be able to understand better atypical presentations of peripheral vestibular disorders, disease intractable to traditional therapy, disorders with unclear pathoetiology and disease only seen on histopathological studies. This review assesses the current state of imaging in the neurotology clinic with a special focus on magnetic resonance imaging and then gathers diseases identified by vestibular testing and histopathological studies that could be better understood with developments in imaging. In doing so, we hope to guide advancement in neurotologic imaging and apprise clinicians of the utility of imaging in peripheral vestibular disease diagnosis.

Neuroimaging Systematic Review in Persistent Postural-Perceptual Dizziness: The Elaborate Alterations in the Delicate Network to Remain Balanced.

OBJECTIVES: Persistent postural-perceptual dizziness (PPPD) is a chronic functional vestibular disorder that may have normal physical examination, clinical laboratory testing and vestibular evaluation. However, advances in neuroimaging have provided new insights in brain functional connectivity and structure in patients with PPPD. This systematic review was aimed at identifying significant structural or alterations in functional connectivity in patients with PPPD. DATABASES REVIEWED: Science Direct, Pubmed, Embase via Ovid databases, and Cochrane library. METHODS: This review following the guidelines of PRISMA, systematically and independently examined papers published up to March 2021 which fulfilled the predetermined criteria. PROSPERO Registration (CRD42020222334). RESULTS: A total of 15 studies were included (MRI = 4, SPECT = 1, resting state fMRI = 4, task-based fMRI = 5, task-based fMRI + MRI = 1). Significant changes in the gray matter volume, cortical folding, blood flow, and connectivity were seen at different brain regions involved in vestibular, visual, emotion, and motor processing. CONCLUSION: There is a multisensory dimension to the impairment resulting in chronic compensatory changes in PPPD that is evident by the significant alterations in multiple networks involved in maintaining balance. These changes observed offer some explanation for the symptoms that a PPPD patient may experience.Systematic Review Registration: This study is registered with PROSPERO (CRD42020222334).

Neuroimaging in Kabuki syndrome and another KMT2D-related disorder.

Recognition of distinct phenotypic features is an important component of genetic diagnosis. Although CHARGE syndrome, Kabuki syndrome, and a recently delineated KMT2D Ex 38/39 allelic disorder exhibit significant overlap, differences on neuroimaging may help distinguish these conditions and guide genetic testing and variant interpretation. We present an infant clinically diagnosed with CHARGE syndrome but subsequently found to have a de novo missense variant in exon 38 of KMT2D, the gene implicated in both Kabuki syndrome and a distinct KMT2D allelic disorder. We compare her brain and inner ear morphology to a retrospective cohort of 21 patients with classic Kabuki syndrome and to typical CHARGE syndrome findings described in the literature. Thirteen of the 21 Kabuki syndrome patients had temporal bone imaging (5/13 CT, 12/13 MRI) and/or brain MRI (12/13) which revealed findings distinct from both CHARGE syndrome and the KMT2D allelic disorder. Our findings further elucidate the spectrum of inner ear dysmorphology distinguishing Kabuki syndrome and the KMT2D allelic disorder from CHARGE syndrome, suggesting that these three disorders may be differentiated at least in part by their inner ear anomalies.

Acetazolamida enmascara un tumor hipofisario bajo el diagnóstico de migraña vestibular / Acetazolamide conceals a pituitary macroadenoma in a patient with criteria of vestibular migraine

No disponible

Dynamic whole-brain metabolic connectivity during vestibular compensation in the rat.

Unilateral damage to the inner ear results in an acute vestibular syndrome, which is compensated within days to weeks due to adaptive cerebral plasticity. This process, called central vestibular compensation (VC), involves a wide range of functional and structural mechanisms at the cellular and network level. The short-term dynamics of whole-brain functional network recruitment and recalibration during VC has not been depicted in vivo. The purpose of this study was to investigate the interplay of separate and distinct brain regions and in vivo networks in the course of VC by sequential [18F]-FDG-PET-based statistical and graph theoretical analysis with the aim of revealing the metabolic connectome before and 1, 3, 7, and 15 days post unilateral labyrinthectomy (UL) in the rat. Temporal changes in metabolic brain connectivity were determined by Pearson's correlation (|r| > 0.5, p < 0.001) of regional cerebral glucose metabolism (rCGM) in 57 segmented brain regions. Metabolic connectivity analysis was compared to univariate voxel-wise statistical analysis of rCGM over time and to behavioral scores of static and dynamic sensorimotor recovery. Univariate statistical analysis revealed an ipsilesional relative rCGM decrease (compared to baseline) and a contralesional rCGM increase in vestibular and limbic networks and an increase in bilateral cerebellar and sensorimotor networks. Quantitative analysis of the metabolic connections showed a maximal increase from baseline to day 3 post UL (interhemispheric: 2-fold, ipsilesional: 3-fold, contralesional: 12-fold) and a gradual decline until day 15 post UL, which paralleled the dynamics of vestibular symptoms. In graph theoretical analysis, an increase in connectivity occurred especially within brain regions associated with brainstem-cerebellar and thalamocortical vestibular networks and cortical sensorimotor networks. At the symptom peak (day 3 post UL), brain networks were found to be organized in large ensembles of distinct and highly connected hubs of brain regions, which separated again with progressing VC. Thus, we found rapid changes in network organization at the subcortical and cortical level and in both hemispheres, which may indicate an initial functional substitution of vestibular loss and subsequent recalibration and reorganization of sensorimotor networks during VC.

Calculated Decisions: Alberta stroke program early ct score (ASPECTS).

The ASPECTS determines middle cerebral artery stroke severity using available computed tomography data.

MRI utricle diameter asymmetry is significantly greater in dogs with idiopathic vestibular syndrome compared with unaffected dogs.

Idiopathic vestibular syndrome (IVS) is the most common cause of acute unilateral peripheral vestibular dysfunction in older dogs. The purpose of this retrospective, cross-sectional study was to characterize morphological changes in the utricle of dogs affected by IVS, using MRI. To evaluate differences between affected and unaffected utricles, the ratio of the largest to the smallest utricle diameter was obtained, as measured on transverse T2-weighted images, and defined as the utricle asymmetricity ratio (UAR). Out of 137 patients diagnosed with IVS after excluding other vestibular diseases by MRI, 101 were eligible for inclusion. Additionally, 31 older dogs with no signs of vestibular disorders or other intracranial diseases were included as a control group. The disease group was divided into two subgroups in which the direction of head tilt and nystagmus symptoms versus the decreased utricle diameters were consistent or inconsistent. The medians of UARs of the IVS and control groups were 0.83 (range 0.37-1.00) and 0.98 (0.70-1.00), respectively. The medians of the UARs of the consistent and inconsistent IVS subgroups were 0.82 (0.37-0.99) and 0.90 (0.74-1.00), respectively. The UAR of the IVS group was significantly decreased than that of the control group and UAR of the consistent sub-group was significantly decreased than that of the inconsistent sub-group (P < .01). In conclusion, significant asymmetry of utricle diameter was identified in dogs with IVS versus unaffected dogs. We propose that canine IVS may possibly be correlated with structural atrophy of the vestibular system.

Inner ear fluid-attenuated inversion recovery MRI signal intensity in dogs with vestibular disease.

The inner ear contains endolymph and perilymph. The second is comparable and in continuity with the cerebrospinal fluid (CSF) so it is expected to suppress in fluid-attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI) if normal. Even though inner ear FLAIR abnormalities have been extensively described in humans with inner ear disease, its diagnostic value in dogs is yet to be proven. The goal of this retrospective cohort study was to investigate the diagnostic utility of FLAIR MRI in dogs with vestibular disease. A review of medical records identified 101 dogs that had brain MRI performed because of vestibular signs. Based on the final diagnosis, patients were allocated to three groups: otitis media/interna, idiopathic vestibular disease, and central vestibular disease. Additionally, a control group (n = 73) included dogs with normal MRI and without vestibular signs. Inner ears were delineated using a region of interest, and signal intensity was measured in FLAIR and T2-weighted images. The percentages of suppression in FLAIR were calculated and compared between affected and unaffected sides of each individual and between groups using a general linear mixed model. Correlation between suppression and CSF cell count and protein concentration was assessed. Affected inner ears in dogs with otitis media/interna had decreased suppression in FLAIR compared to the unaffected side (P < .001), and all other groups (P < .01). No significant correlation was detected between CSF results and suppression. These results show the diagnostic value of FLAIR in otitis media/interna due to lack of suppression in the affected inner ear.

The round window sign: a sensitive sign to detect perilymphatic fistulae on delayed postcontrast 3D-FLAIR sequence.

OBJECTIVES: The aim of this study is to assess the diagnostic performance of a new MR sign, named the round window sign (RWS), to diagnose perilymphatic fistula (PLF) in a population of patients with chronic cochleo-vestibular symptoms, classified as definite or probable Menière's disease (MD). METHODS: A total of 164 patients (mean age 52 ± 35 years) with chronic cochleo-vestibular symptoms underwent MRI, between 4 and 5 h after intravenous gadoteric acid injection (Dotarem®, 0.1 mmol/kg). MRI exploration was carried out on a 3-T Achieva® TX scanner. We analyzed the presence of the RWS, defined as a nodular FLAIR high signal in the round window (RW) and the presence of associated saccular hydrops. When this RWS was present, a temporal bone CT scan was performed and the RW was analyzed. RESULTS: Of the 164 patients with definite MD (85 patients) or probable MD (79 patients), we found the RWS in 18 (11%), and 17/18 were classified into the group of probable MD. All these 18 patients showed other MR sequences considered as normal, including heavily weighted T2 imaging. Among these 18 patients, the temporal bone CT examination presented a filling of the RW in 13 patients (72%) and no filling of the RW in 5 patients (28%). Seven patients were surgically managed confirming in vivo the PLF diagnosis. The RWS was associated with the presence of a saccular hydrops in 4 cases. CONCLUSION: Delayed postcontrast 3D-FLAIR may reveal perilymphatic fistulae in patients with probable Menière's disease using the round window sign. KEY POINTS: • MRI with delayed acquisition can detect perilymphatic fistulae with perfect sensitivity, based on the presence of the round window sign. • This visual sign is only visible on a 3D-FLAIR sequence. • 3D-FLAIR sequence with delayed acquisition is more sensitive than temporal bone CT scan examination in detecting PLF.

Clinical signs, MRI findings and outcome in dogs with peripheral vestibular disease: a retrospective study.

BACKGROUND: Vestibular dysfunction is relatively common in dogs, with a prevalence of 0.08% reported in primary veterinary care in the UK. There are several studies investigating how to differentiate between peripheral and central vestibular disease but only limited information regarding the possible underlying causes for peripheral vestibular dysfunction in dogs. This study therefore aimed to describe the clinical signs, magnetic resonance imaging findings (MRI), underlying causes and outcome in a large population of dogs diagnosed with peripheral vestibular disease. RESULTS: One hundred eighty-eight patients were included in the study with a median age of 6.9 years (range 3 months to 14.6 years). Neurological abnormalities included head tilt (n = 185), ataxia (n = 123), facial paralysis (n = 103), nystagmus (n = 97), positional strabismus (n = 93) and Horner syndrome (n = 7). The most prevalent diagnosis was idiopathic vestibular disease (n = 128), followed by otitis media and/or interna (n = 49), hypothyroidism (n = 7), suspected congenital vestibular disease (n = 2), neoplasia (n = 1) and cholesteatoma (n = 1). Long-term follow-up revealed persistence of head tilt (n = 50), facial paresis (n = 41) and ataxia (n = 6) in some cases. Recurrence of clinical signs was observed in 26 dogs. Increasing age was associated with a mild increased chance of diagnosis of idiopathic vestibular syndrome rather than otitis media and/or interna (P = 0.022, OR = 0.866; CI 0.765-0.980). History of previous vestibular episodes (P = 0.017, OR = 3.533; CI 1.251-9.981) was associated with an increased likelihood of resolution of the clinical signs whilst contrast enhancement of cranial nerves VII and/or VIII on MRI (P = 0.018, OR = 0.432; CI 0.251-0.868) was associated with a decreased chance of resolution of the clinical signs. CONCLUSIONS: Idiopathic vestibular disease is the most common cause of peripheral vestibular dysfunction in dogs and it is associated with advanced age. Incomplete recovery from peripheral vestibular disease is common, especially in dogs presenting with cranial nerve enhancement on MRI but less so if there is previous history of vestibular episodes.

Phenotypic expansion of KMT2D-related disorder: Beyond Kabuki syndrome.

Pathogenic variants in KMT2D, which encodes lysine specific methyltransferase 2D, cause autosomal dominant Kabuki syndrome, associated with distinctive dysmorphic features including arched eyebrows, long palpebral fissures with eversion of the lower lid, large protuberant ears, and fetal finger pads. Most disease-causing variants identified to date are putative loss-of-function alleles, although 15-20% of cases are attributed to missense variants. We describe here four patients (including one previously published patient) with de novo KMT2D missense variants and with shared but unusual clinical findings not typically seen in Kabuki syndrome, including athelia (absent nipples), choanal atresia, hypoparathyroidism, delayed or absent pubertal development, and extreme short stature. These individuals also lack the typical dysmorphic facial features found in Kabuki syndrome. Two of the four patients had severe interstitial lung disease. All of these variants cluster within a 40-amino-acid region of the protein that is located just N-terminal of an annotated coiled coil domain. These findings significantly expand the phenotypic spectrum of features associated with variants in KMT2D beyond those seen in Kabuki syndrome and suggest a possible new underlying disease mechanism for these patients.

Holoprosencephaly in Kabuki syndrome.

Kabuki syndrome is a rare, multi-systemic disorder of chromatin regulation due to mutations in either KMT2D or KDM6A that encode a H3K4 methyltransferase and an H3K27 demethylase, respectively. The associated clinical phenotype is a direct result of temporal and spatial changes in gene expression in various tissues including the brain. Although mild to moderate intellectual disability is frequently recognized in individuals with Kabuki syndrome, the identification of brain anomalies, mostly involving the hippocampus and related structures remains an exception. Recently, the first two cases with alobar holoprosencephaly and mutations in KMT2D have been reported in the medical literature. We identified a de novo, pathogenic KMT2D variant (c.6295C > T; p.R2099X) using trio whole-exome sequencing in a 2-year-old female with lobar holoprosencephaly, microcephaly and cranio-facial features of Kabuki syndrome. This report expands the spectrum of brain anomalies associated with Kabuki syndrome underscoring the important role of histone modification for early brain development.

The effect of canal diameter on audiologic results in patients with cochlear implantation with large vestibular aqueduct syndrome.

PURPOSE: To compare audiologic results according to vestibular aqueduct (VA) diameter in patients who have undergone cochlear implantation and were diagnosed with LVAS. METHODS: This was a retrospective study detailing the outcomes of 18 patients with LVAS and 18 patients undergone cochlear implants. VA diameter was assessed by magnetic resonance imaging and computed tomography. Categories of Auditory Perception (CAP) and Speech Intelligibility Rating (SIR) were assessed in all patients, and speech audiometry, including speech recognition thresholds (SRT) and word discrimination scores, was applied for all subjects who were able to perform these tests. All audiologic parameters were compared between patients with and without LVAS, and the relationship of these parameters with VA diameter was investigated. RESULTS: The control group consisted of 18 subjects (5 males, 13 females), ranging in age between 2 and 34 years (mean 13.17 ± 8.97 years). The research group consisted of 18 subjects (8 males, 10 females), ranging in age between 2 and 35 years (mean 13.28 ± 8.96 years). There was a statistically significant difference between the groups in terms of SIR and CAP pre-post differences (Mann-Whitney U test, p < 0.05), with higher averages in the LVAS group. No statistically significant correlations were found between VA diameter on computed tomography and magnetic resonance imaging and the audiologic variables collected. CONCLUSIONS: Patients with LVAS benefit from cochlear implant surgery and VA parameters do not affect audiologic parameters.

Altered spontaneous functional activity of the right precuneus and cuneus in patients with persistent postural-perceptual dizziness.

Persistent postural-perceptual dizziness (PPPD) is a functional vestibular disorder, and is the most common cause of chronic vestibular syndrome. However, the pathogenesis of PPPD is currently unclear. This study aimed to analyze the changes of brain spontaneous functional activities in PPPD patients during the resting state, and to explore the underlying pathogenesis of PPPD, particularly the abnormal integration of visual and vestibular information. Ten PPPD patients and 10 healthy controls were enrolled from January to June 2018, and baseline data were collected from all subjects. Videonystagmography (VNG), the vestibular caloric test, the video head impulse test (vHIT) and vestibular evoked myogenic potentials (VEMPs) were measured to exclude peripheral vestibular lesions. Functional MRI (fMRI) was conducted in PPPD patients and healthy controls. The amplitude of low frequency fluctuation (ALFF) and regional homogeneity (ReHo), and functional connectivity were calculated to explore changes in brain spontaneous functional activity during the resting state. Compared with healthy controls, ALFF and ReHo values in the right precuneus and cuneus were significantly lower in PPPD patients (both P < 0.05). Further seed-based functional connectivity analysis showed decreased functional connectivity between precuneus, cuneus and left precentral gyrus (P < 0.05). Our findings suggest that the spontaneous functional activity of cuneus and precuneus in PPPD patients were altered, potentially leading to abnormal integration of visual and vestibular information. Weakened functional connectivity between the precuneus and the precentral gyrus may be associated with aggravated symptoms during upright posture, active or passive movements.

[Imaging characteristics of patients with large vestibular aqueduct syndrome and its relationship with the acoustically evoked short latency negative response].

Objective: To explore the imaging characteristics of large vestibular aqueduct syndrome (LVAS) patients and their relationship with the acoustically evoked short latency negative response (ANSR), so as to provide reference for the diagnosis of LVAS. Methods: Clinical data of 174 patients(334 ears) with LVAS diagnosed and treated by the Department of Otorhinolaryngology Head and Neck Surgery of the First Affiliated Hospital of Guangxi Medical University, from October 2009 to December 2017 were retrospectively analyzed, including 117 males and 57 females, aged from 5 months to 47 years old, with the median age of 4 years and 4 months. ABR and imaging data of patients were collected. Midpoint diameter and the outlet diameter of the vestibular aqueduct were measured on CT images, the midpoint diameter of the intraosseous parts and the extraosseous parts of enlarged endolymphatic sac(EES) were measured on MRI images. The correlation between the above measurements was analyzed by Pearson test using SPSS 17.0. According to whether ASNR was detected in ABR, the above data were divided into two groups, and the differences of the above imaging measurements were compared by the Independent-Sample Test. Results: The average midpoint diameter of the vestibular aqueduct was (1.87±0.58) mm (x±s, the following was the same), and the outlet diameter was (3.07±0.99) mm on CT; the average midpoint diameter of the intraosseous parts in enlarged endolymphatic sac(EES) was (2.39±1.37) mm, and the extraosseous parts was (2.50±2.18) mm on MRI. There was a correlation between the four measurements (P<0.05), among which the midpoint diameter of vestibular aqueduct was strongly positively correlated with the outlet diameter (r=0.760), and the remaining pairs were weakly correlated. ASNR was detected in 241 ears (72.16%,241/334) and undetected in 93 ears (27.84%, 93/334) of the 334 ears with LVAS. Midpoint diameter and the outlet diameter of the vestibular aqueduct in no ASNR group were smaller than the ASNR group, and the difference was statistically significant (t value was 2.814 and 2.754, P<0.05). There was no significant difference in the midpoint diameter of the intraosseous parts and the extraosseous parts of enlarged endolymphatic sac between the two groups, and the difference was no statistically significant(t value was 0.101 and 0.683, P>0.05). Conclusions: There is a strong positive correlation between the midpoint diameter of vestibular aqueduct and the outlet diameter in LVAS patients. There is a certain correlation between the size of vestibular aqueduct and the size of endolymphatic sac. The smaller the diameter of vestibular aqueduct, the lower the occurrence rate of ASNR.

Tumor-to-Tumor Metastasis of Hepatocellular Carcinoma to Vestibular Schwannoma.

BACKGROUND: Intracranial metastasis is a common complication of systemic malignancy. A rare subset of intracranial masses constitutes tumor-to-tumor metastasis, in which an extracranial neoplasm hematogenously spreads to an existing intracranial lesion. CASE DESCRIPTION: Here we present the unique case of a 59-year-old man with known hepatocellular carcinoma who presented with acute headaches, double vision, vertigo, and gait instability in the context of 2 years of right-sided hearing loss. Head imaging showed a heterogeneous right cerebellopontine angle mass extending into the porus acousticus with adjacent cerebellar edema. Histopathologic analysis after resection found coexisting hepatocellular carcinoma within a vestibular schwannoma. CONCLUSIONS: Tumor-to-tumor metastasis is an important consideration in the diagnostic work-up and treatment of patients with known systemic malignancy who present with a new intracranial lesion. This pathologic entity could be missed if this patient were treated with single-fraction radiosurgery such as Gamma Knife.