Search Results (26)

Background/Objectives: This study presents a case of chronic internal carotid artery [ICA] occlusion initially misinterpreted as ICA agenesis on magnetic resonance angiography (MRA). The report underscores the importance of retrospective review of prior imaging, particularly computed tomography angiography [CTA], in establishing the correct diagnosis. Case Report: A 70-year-old man presented with persistent headache, pulsatile tinnitus, and intermittent dizziness. Neurological examination and laboratory results were unremarkable. Initial cranial MRA demonstrated absence of flow in the left ICA, raising suspicion of congenital agenesis. However, retrospective evaluation of a CTA performed nine years earlier revealed a well-formed left carotid canal without ICA opacification, confirming the diagnosis of chronic ICA occlusion. Results: Current imaging again showed lack of enhancement in the left ICA, with adequate cerebral perfusion supplied via the contralateral ICA and vertebrobasilar system. Recognition of the preserved carotid canal on earlier CTA clarified the diagnosis as chronic occlusion rather than agenesis. Although surgical or endovascular revascularization was recommended, the patient opted for conservative management. At three months of follow-up, symptoms had improved and clinical monitoring continues. Conclusions: This case underscores the importance of distinguishing ICA agenesis from chronic occlusion, particularly by evaluating the carotid canal on CT. The presence of a carotid canal strongly indicates prior patency of the ICA and supports a diagnosis of occlusion. Careful differentiation is critical to avoid misinterpretation and to guide appropriate clinical management. In addition, reviewing prior imaging can be valuable when current findings are inconclusive or potentially misleading. Since this is a single case report, these observations should be regarded as hypothesis-generating rather than definitive, and further studies are needed to validate their broader applicability. Full article

Dural arteriovenous fistulas (dAVFs) are a heterogeneous group of intracranial vascular anomalies characterized by abnormal arteriovenous shunting within the dura mater. While they are often considered acquired lesions—associated with trauma, surgery, venous sinus stenosis, or thrombosis—their precise etiology remains unclear in many cases. The clinical presentation of dAVFs varies widely depending on location and venous drainage patterns. Benign forms may manifest as pulsatile tinnitus or headache, whereas lesions with retrograde venous drainage and cortical venous reflux are considered aggressive and carry a heightened risk of hemorrhage and progressive neurological decline. Multiple classification systems, primarily based on angioarchitecture and venous outflow characteristics, have been developed to stratify risk and guide treatment strategies, as these features largely determine the natural history and clinical course of dAVFs. Endovascular embolization, microsurgical disconnection, and stereotactic radiosurgery (SRS) represent the mainstays of treatment, aiming to prevent hemorrhage or rebleeding and to alleviate symptoms related to venous congestion. Over the past two decades, advances in endovascular techniques have driven a paradigm shift in management, positioning embolization as the first-line therapy for most dAVFs. This review begins with a comprehensive overview of dAVF pathogenesis, classification systems, and angioarchitecture. It then focuses on the endovascular management of dAVFs, offering a detailed appraisal of current and emerging techniques, key technical considerations, and lesion-specific treatment strategies. Finally, we discuss the role of microsurgery and SRS. Full article

Objectives: Tinnitus, defined as the perception of sound without an external stimulus, is a complex condition with unclear etiology. Emerging evidence suggests a link between vascular dysfunction, particularly arterial stiffness, and tinnitus. This study aimed to evaluate vertebral artery stiffness in patients with idiopathic subjective tinnitus and assess the utility of Doppler ultrasonography as a non-invasive diagnostic tool. Materials and Methods: In this prospective study, 31 patients with idiopathic tinnitus (11 males, 20 females; mean age: 50.42 ± 9.64 years) and 24 healthy controls (12 males, 12 females; mean age: 39.67 ± 10.63 years) underwent comprehensive clinical evaluations, including pure tone audiometry, blood tests, and vertebrobasilar Doppler ultrasonography. Vertebral artery stiffness index (VAS), resistive index (RI), and pulsatility index (PI) were measured bilaterally. Results: A total of 31 patients with idiopathic subjective tinnitus and 24 healthy controls were evaluated. The mean age was significantly higher in the tinnitus group compared to controls (50.42 ± 9.64 vs. 39.67 ± 10.63 years, p < 0.001). Lipid profile analysis revealed significantly higher levels of total cholesterol (193.6 ± 47.28 vs. 167.5 ± 28.99 mg/dL, p = 0.021), LDL (149.4 ± 37.9 vs. 106.1 ± 10.7 mg/dL, p < 0.005), and triglycerides (202.2 ± 83.5 vs. 148.6 ± 26.4 mg/dL, p < 0.005) in tinnitus patients. Doppler ultrasonography demonstrated significantly higher vertebral artery stiffness values in the tinnitus group (left: 2.87 ± 0.72 vs. 2.12 ± 0.22; right: 2.99 ± 0.77 vs. 2.14 ± 0.5; both p < 0.005). Similarly, pulsatility index (PI) was significantly elevated in patients compared to controls (left: 2.45 ± 1.2 vs. 1.2 ± 0.43; right: 2.49 ± 1.02 vs. 1.19 ± 0.42; both p < 0.005). No significant differences were observed in resistive index (RI) or vertebral artery diameters between groups. Among tinnitus patients, PI and VAS were significantly higher on the side corresponding to reported tinnitus symptoms (p < 0.05), suggesting a lateralized vascular contribution. Conclusions: The findings suggested a potential relationship between idiopathic subjective tinnitus and vertebral artery stiffness. We demonstrated the utility of Doppler ultrasonography, a cost-effective and non-invasive imaging modality, for evaluating vascular parameters in tinnitus patients, paving the way for broader clinical applications. By uncovering a significant association between vertebral artery stiffness and tinnitus, our findings suggest that vascular health assessments could enhance diagnostic and therapeutic strategies for tinnitus management. Full article

Background: Venous sinus stenting is a promising treatment for intracranial venous disorders, such as idiopathic intracranial hypertension and pulsatile tinnitus, associated with transverse sinus stenosis. The VIVA Stent System (VSS) is a novel self-expanding braided venous stent designed to navigate tortuous cerebral venous anatomy. This preclinical study assessed the safety, thrombogenicity, and performance of the VSS in a swine model. Methods: Fifteen swine underwent bilateral internal mammary vein stenting with either the VSS (n = 9) or the PRECISE^® PRO RX stent (n = 6, reference). Fluoroscopy and thrombogenicity assessments were conducted on the day of stenting, clinical pathology analysis was carried out throughout the in-life phase, and CT Venography was performed before sacrifice. Animals were sacrificed at 30 ± 3 or 180 ± 11 days post-stenting for necropsy and histological evaluation. Results: Fluoroscopic angiography confirmed the successful VSS deployment with complete venous wall apposition and no vessel damage. The VSS achieved the highest scores on a four-point Likert scale for most performance parameters. No thrombus formation was observed on either delivery system. CT Venography confirmed vessel patency, no stent migration, and complete stent integrity. Histopathology showed a mild, expected foreign body reaction at 30 days, which resolved by 180 days, indicating normal healing progression. Both stents showed increased luminal diameter and decreased wall thickness at 180 days, suggesting vessel recovery. No adverse reactions were observed in non-target organs. Conclusions: The VSS exhibited favorable safety, procedural performance, and thromboresistance in a swine model, supporting its potential clinical use for treating transverse sinus stenosis and related conditions. Full article

Pulsatile tinnitus (PT) is a subtype of tinnitus characterized by a perception of heartbeat-synchronous sound. It represents approximately 5–10% of all tinnitus cases and may have either a vascular or non-vascular etiology. Accurate diagnosis is crucial due to the potentially serious implications this condition can entail. Assessment through anamnesis and physical examination may often suggest a diagnosis of PT, but it is rarely definitive. Therefore, a comprehensive and specific imaging diagnostic protocol is essential when evaluating PT. A lack of consensus has been identified regarding the use of a standardized protocol for both pulsatile and non-pulsatile tinnitus, whether unilateral or bilateral. Consequently, neuroradiologists, otologists, and otoneurologists from a tertiary hospital have developed a new imaging diagnostic protocol for PT. The aim of this article is to present an updated approach to the diagnostic and therapeutic management of PT, aiming to establish a protocol that serves as a guide for clinicians assessing this symptom. In patients with bilateral PT, systemic conditions leading to increased cardiac output should generally be ruled out; in unilateral cases, focused imaging studies should be performed to exclude organic etiologies at the cervical and cranial levels. Full article

Background and Clinical Significance: The levator claviculae muscle (also known as cleidocervicalis) is a vestigial muscle located in the posterior triangle of the neck, extending from the upper cervical transverse processus to the clavicle. It has been detected in ~2% of humans, but is rarely documented in the radiologic or anatomic literature. When found on physical exam, it is usually mis-identified as lymphadenopathy, metastasis, cysts, an aneurysm, or other masses. It has been implicated in a few cases of thoracic outlet syndrome. Case Presentation: Herein, we describe a 25-year-old man with a weightlifting history, who was found to have a right levator claviculae muscle in the setting of unilateral mixed neurovascular thoracic outlet syndrome. The patient presented with right-sided extremity paresthesias, pain in the neck, shoulder, and arm, and symptom exacerbation with overhead activities. He also described intermittent unilateral pulsatile tinnitus during strenuous exercise. On physical exam, he was found to have a right carotid bruit, unequal systolic blood pressures, and positive Roos and Adson’s testing. The variant muscle was identified with a modified exam maneuver, and was further characterized with sonography and MRI. Symptoms were managed with activity restriction and NSAIDs. We reviewed 17 cases of levator claviculae variant muscles in patients. Conclusions: The presence of levator claviculae muscles has been detected in patients with thoracic outlet syndrome, but never in a patient with an audible bruit and pulsatile tinnitus. This physical exam maneuver, used in conjunction with multimodal imaging, successfully aided diagnosis and direct medical management in this case. Full article

Objective: Comparing the clinical outcomes of glomus tympanicum (GT) resections by transcanal endoscopic ear surgery (TEES), microscopic- and endoscopic-assisted microscopic approaches. Methods: Adult patients conducting exclusive operations for GT within the tympanomastoid cavity were recruited in this retrospective cohort study at five international tertiary referral centers. GT resections were conducted by TEES, microscopic- and endoscopic-assisted microscopic approaches based on modified Fisch–Mattox classifications. Clinical characteristics and surgical outcomes were recorded and analyzed. Results: A total of 46 patients were included. A longer operative time was seen in more advanced GTs (A1: 106.73 ± 9.33 min, A2: 133.21 ± 13.47 min, B1: 176.88 ± 18.69 min, p = 0.005), while no significant differences were observed in the mean operative times among various surgical approaches. Preoperatively, 89.1% of patients experienced pulsatile tinnitus, and 56.5% exhibited conductive hearing loss. Postoperatively, only one patient continued to experience tinnitus (p < 0.001), and two patients had persistent hearing loss (p < 0.001). Higher disease grades correlated with poorer preoperative air-conduction thresholds (p = 0.015), while the differences in air-conduction thresholds before and after surgery did not demonstrate statistical significance across different tumor stages (p = 0.894) and surgical approaches (p = 0.257). The median follow-up period was 4 years, and only one recurrent case was found (2.2%, 1/46), which was treated by TEES and involved a B1 glomus tumor. Conclusions: Similar and excellent surgical outcomes were found among the TEES and microscope- and endoscopic-assisted microscopic approaches in early-stage GTs. Full article

Background: Ophthalmoplegic migraine (OM) is an uncommon variant of migraine characterised by headache and cranial nerve palsy, posing significant diagnostic and therapeutic challenges. Objective: This study aimed to describe an extremely rare OM variant with a partial therapeutic response. Clinical Case: A 34-year-old pregnant woman in gestational week 19.1 (G6P2A3) with a history of three consecutive spontaneous abortions presented at the emergency services with insidious onset and mild-to-moderate-intensity pulsatile bifrontal headache for 15 days, and the positional changes exacerbated this. At peak intensity, she experienced nausea, vomiting, tinnitus, and photophobia without phonophobia or osmophobia, prompting multiple visits to the emergency department. Despite a broad range of treatments, including intravenous fluids, analgesia, pericranial blocks, and preventive management, there was a non-significative improvement in the symptomatology described above. However, spontaneous resolution of this clinical picture was observed during the postpartum period. Results: This case highlights the complexity of ophthalmoplegic migraine, especially in the context of pregnancy, and raises questions about the underlying pathophysiological mechanisms. The absence of structural lesions on neuroimaging and postpartum resolution suggests a potential association with the hormonal and physiological changes associated with pregnancy. Conclusions: Despite limited scientific evidence, this report contributes to expanding the knowledge of this rare entity and emphasises the importance of a multidisciplinary approach to its management. Full article

Objective: to identify advanced or “so-called inoperable” cases of tympanojugular paragangliomas (PGLs) and analyze how each case is surgically managed and followed afterward. Study Design: a retrospective case series study. Methods: Out of 262 type C and D TJPs and more than 10 cases of advanced or so-called inoperable cases, files of 6 patients with a diagnosis of advanced tympanojugular PGLs who were referred to an otology and skull-base center between 1996 and 2021 were reviewed to analyze management and surgical outcomes. The criteria for choosing these cases involve having one or more of the following features: (1) a large-sized tumor; (2) a single ipsilateral internal carotid artery (ICA); (3) involvement of the vertebral artery; (4) a considerable involvement of the ICA; (5) an extension to the clivus, foramen magnum, and cavernous sinus; (6) large intradural involvement (IDE); and (7) bilateral or multiple PGLs. Results: The age range at presentation was 25–43 years old, with a mean of 40.5 years: two females and four males. The presenting symptoms were glossal atrophy, hearing loss, pulsatile tinnitus, dysphonia, shoulder weakness, and diplopia. The modified Infratemporal Fossa Approach (ITFA) with a transcondylar–transtubercular extension is the principal approach in most cases, with additional approaches being used accordingly. Conclusions: The contemporary introduction of carotid artery stenting with the direct and indirect embolization of PGLs has made it possible to operate on many cases, which was otherwise considered impossible to treat surgically. Generally, the key is to stage the removal of the tumor in multiple stages during the management of complex PGLs to decrease surgical morbidities. A crucial aspect is to centralize the treatment of PGLs in referral centers with experienced surgeons who are trained to plan the stages and manage possible surgical complications. Full article

The pathology of idiopathic intracranial hypertension (IIH), a disease characterized by papillary edema and increased intracranial pressure (IICP), is not yet understood; this disease significantly affects quality of life due to symptoms including vision loss, headache, and pulsatile tinnitus. By contrast, superficial siderosis (SS), a disorder in which hemosiderin is deposited on the surface of the cerebral cortex and cerebellum, potentially causes cerebellar ataxia or hearing loss. So far, no cases of IIH with infratentorial and supratentorial cortical SS have been reported. Herein, we report a case of a 31-year-old woman with obesity who developed this condition. The patient suddenly developed headache and dizziness, had difficulty walking, and subsequently became aware of diplopia. Fundus examination revealed bilateral optic nerve congestive papillae and right eye abducens disturbance. Head magnetic resonance imaging (MRI) showed prominent SS on the cerebellar surface and cerebral cortex. Lumbar puncture revealed IICP of 32 cmH₂O, consistent with the diagnostic criteria for IIH, and treatment with oral acetazolamide was started; subsequently, the intracranial pressure decreased to 20 cmH₂O. Her abduction disorder disappeared, and the swelling of the optic papilla improved. She was now able return to her life as a teacher without any sequelae. SS is caused by persistent slight hemorrhage into the subarachnoid space. In this case, both infratentorial and supratentorial cortical superficial SS was observed. Although cases of IIH complicated by SS are rare, it should be kept in mind that a causal relationship between IIH and SS was inferred from our case. Our findings also suggest that cerebrospinal fluid dynamic analysis using MRI is effective in diagnosing IIH and in determining the efficacy of treatment. Full article

This study aimed to investigate the effect of the transverse sinus (TS) stenosis (TSS) position caused by arachnoid granulation on patients with venous pulsatile tinnitus (VPT) and to further identify the types of TSS that are of therapeutic significance for patients. Multiphysics interaction models of six patients with moderate TSS caused by arachnoid granulation and virtual stent placement in TSS were reconstructed, including three patients with TSS located in the middle segment of the TS (group 1) and three patients with TTS in the middle and proximal involvement segment of the TS (group 2). The transient multiphysics interaction simulation method was applied to elucidate the differences in biomechanical and acoustic parameters between the two groups. The results revealed that the blood flow pattern at the TS and sigmoid sinus junction was significantly changed depending on the stenosis position. Preoperative patients had increased blood flow in the TSS region and TSS downstream where the blood flow impacted the vessel wall. In group 1, the postoperative blood flow pattern, average wall pressure, vessel wall vibration, and sound pressure level of the three patients were comparable to the preoperative state. However, the postoperative blood flow velocity decreased in group 2. The postoperative average wall pressure, vessel wall vibration, and sound pressure level of the three patients were significantly improved compared with the preoperative state. Intravascular intervention therapy should be considered for patients with moderate TSS caused by arachnoid granulations in the middle and proximal involvement segment of the TS. TSS might not be considered the cause of VPT symptoms in patients with moderate TSS caused by arachnoid granulation in the middle segment of the TS. Full article

Objective: To analyze the long-term facial function as well as overall postoperative condition in surgically treated tympanomastoid PGL patients. Study Design: Retrospective study. Method: The medical records of patients with surgically managed class A and B tympanomastoid PGLs between 1983 and 2023 were thoroughly evaluated. Result: Our center has treated a total of 213 cases of tympanomastoid PGL surgically. The mean age of patients was 54, and the male-to-female ratio was 1:6. The most common symptoms at presentation were hearing loss (80%), pulsatile tinnitus (77%), and vertigo (15%). According to the modified Fisch classification, 45% of the cases were classified as class A (A1 and A2), while 55% were classified as class B (B1, B2, and B3). All class A and most class B1 and B2 tumors were removed either with transcanal or retroauricular-transcanal approaches. However, more advanced class B3 lesions were removed with subtotal petrosectomy (SP) along with middle ear obliteration. Facial nerve outcome was excellent in all class A and B cases, while chances of postoperative paresis slightly increased with the size and extent of the tumor (p < 0.05). The hearing outcome is excellent for class A1, A2, B1, and B2 tumors, whereas more advanced class B3 cases have a loss of air conduction (AC) and increased bone conduction (BC) threshold (p < 0.05). Complete surgical removal was achieved in 97% of our cases. The most common late complication was permanent TM perforation (7%), and the recurrence rate was 3%. Conclusions: Tympanomastoid PGL represents the most common neoplasm of the middle ear space. The most frequent presenting symptoms include pulsatile tinnitus and hearing loss, whereas the presence of retrotympanic mass was evident in all cases at the time of initial otoscopic evaluation. Proper documentation of facial function and audiometric evaluation are crucial elements of preoperative workup. The most preferred preoperative radiologic examination is high-resolution computer tomography (HRCT), whereas magnetic resonance imaging (MRI) with or without gadolinium enhancement is reserved for cases with a dilemma of carotid artery or jugular bulb involvement. The main goal of tympanomastoid PGL treatment is complete disease removal with preservation of hearing and facial functions. Surgical treatment remains the preferred treatment modality with the benefits of complete disease removal, lower rate of recurrence and complication, and acceptable postoperative hearing level. Here, we present our 40 years of experience, which, to the very best of our knowledge, is the largest series of tympanomastoid PGL in the English literature. Full article

Posterior semicircular canal dehiscence (PSCD) has been demonstrated to result in a third mobile window mechanism (TMWM) in the inner ear similar to superior semicircular canal dehiscence (SSCD). Typical clinical and instrumental features of TMWM, including low-frequency conductive hearing loss (CHL), autophony, pulsatile tinnitus, sound/pressure-induced vertigo and enhanced vestibular-evoked myogenic potentials, have been widely described in cases with PSCD. Nevertheless, video-head impulse test (vHIT) results have been poorly investigated. Here, we present six patients with PSCD presenting with a clinical scenario consistent with a TMWM and an impaired vestibulo-ocular reflex (VOR) for the affected canal on vHIT. In two cases, an additional dehiscence between the facial nerve and the horizontal semicircular canal (HSC) was detected, leading to a concurrent VOR impairment for the HSC. While in SSCD, a VOR gain reduction could be ascribed to a spontaneous “auto-plugging” process due to a dural prolapse into the canal, the same pathomechanism is difficult to conceive in PSCD due to a different anatomical position, making a dural herniation less likely. Alternative putative pathomechanisms are discussed, including an endolymphatic flow dissipation during head impulses as already hypothesized in SSCD. The association of symptoms/signs consistent with TMWM and a reduced VOR gain for the posterior canal might address the diagnosis toward PSCD. Full article

To evaluate the efficacy of perivascular sympathectomy in managing adventitia layer-related long-segment tubular stenosis of cervical segment (C1) internal carotid arteries (ICAs) in a cohort where conventional medical and endovascular interventions were not viable options, we retrospectively analyzed 20 patients (8 males, 12 females, aged 41–63 years) who underwent perivascular sympathectomy for long-segment (>5 cm) tubular cervical ICA stenosis (non-atherosclerotic, non-intima related, and nondolichoarteriopathic) between 2017 and 2023. The procedure aimed to alleviate symptoms such as hemiparesis, pulsatile tinnitus, and migraines associated with transient ischemic attacks (TIAs). Preoperative and postoperative symptoms were assessed, and patient follow-up was conducted by MR angiography and perfusion studies. Postoperatively, 10 out of 11 migraine sufferers (90.9%) reported complete cessation of symptoms, while one patient (9.09%) experienced reduction in frequency and intensity. In cases of tinnitus, six out of nine patients (66.6%) reported complete resolution, two (22.2%) had reduced symptoms, and one (11.1%) saw no change. Regarding motor function, all 12 patients (100%) with initial hemiparesis (30–40% loss of motor function) showed complete recovery postoperatively. There was no TIA attack among the patients after the procedure in the mean two-year follow-up. Perivascular sympathectomy has shown promising results in alleviating symptoms and preventing recurrent cerebrovascular events in long-segment tubular stenosis of cervical ICAs. Full article

Objective: Vertebro-vertebral arteriovenous fistulae (VVFs) are a rare disorder characterized by a direct shunt between the extracranial vertebral artery and the veins of the vertebral venous plexus. This study aims to comprehensively review the characteristics and outcomes of endovascular treatments for VVFs at our center. Methods: A retrospective review was conducted on 14 patients diagnosed with a VVF who underwent endovascular treatment at Siriraj Hospital from January 2000 to January 2023. The study assessed patient demographics, presentation, fistula location, treatment strategies, endovascular techniques employed, and treatment outcomes. Results: Among the 14 patients, 11 (78.6%) were female, with an age range from 25 to 79 years (median: 50 years). Spontaneous VVFs were observed in 64.3% of the cases, including three associated with neurofibromatosis type 1 (NF-1). Iatrogenic injury accounted for two cases, and three patients had VVFs resulting from traffic accidents. A pulsatile neck mass and tinnitus, with or without neurological deficits, were common presenting symptoms. Active bleeding was observed in three cases with vascular injury, while unilateral proptosis, congestive heart failure, and incidental findings each presented in one patient. All the VVFs were successfully obliterated without major treatment complications. Parent vessel sacrifice was performed in 85.7% of the cases, while vertebral artery preservation was achieved in the remaining two patients. Embolic materials included detachable balloons, detachable coils, and n-butyl cyanoacrylate (NBCA) glue. All the presenting symptoms showed improvement, and no morbidity or mortality was observed. Conclusions: Endovascular embolization is a feasible and effective approach for achieving complete VVF obliteration with safety. Parent artery sacrifice should not be reluctantly performed, particularly when adequate collateral circulation is demonstrated. Full article