[Clinical analysis of 50 patients with abnormal pulsatile tinnitus in sigmoid sinus].

Objective: To analyze the disease characteristics, diagnosis and treatment methods of venous pulsatile tinnitus treated by intervention of sigmoid sinus. Methods: Fifty patients (from Shandong Provincial ENT Hospital, Shandong University between February 2014 and July 2020) with venous pulsatile tinnitus treated by sigmoid sinus surgery were analyzed retrospectively. The tinnitus characteristics, imaging findings, surgical methods, intraoperative findings and postoperative tinnitus changes were recorded. The patients were followed up for 6-12 months. The sign rank sum test was used to analyze the difference in tinnitus grading before and after surgery. There were 50 patients with unilateral venous pulsatile tinnitus, including 49 females and 1 male. The age ranged from 17 to 67 years, with a median age of 44 years. There were 45 cases of right tinnitus and 5 cases of left tinnitus. The degree of tinnitus before operation was grade Ⅱ or above, including 4 cases of gradeⅡ, 11 cases of grade Ⅲ, 22 cases of grade Ⅳ and 13 cases of grade Ⅴ. Results: Thirty-seven cases were cured, 8 cases were ineffective (no change in tinnitus), 3 cases were markedly effective (tinnitus grade decreased by 3 in 2 cases, 4 in 1 case), and 2 cases were effective (tinnitus grade decreased by 1). The difference of tinnitus grade before and after operation was statistically significant (Z=-5.70,P<0.05). Temporal bone CT showed 36 cases of sigmoid diverticulum (including 17 cases with sigmoid sinus dehiscence), 12 cases of sigmoid sinus dehiscence and 2 cases of absence of the temporal bone cortex abutting to sigmoid sinus. Thirty-five cases were performed with closure of sigmoid sinus diverticulum, 4 cases were performed with resurfacing of the sigmoid plate, 5 cases were performed with narrowing of sigmoid sinus, 4 cases were performed with simple opening of pre sigmoid mastoid air chamber, 1 case of opening was performed with pre sigmoid mastoid air chamber combined with narrowing of sigmoid sinus, and 1 case was performed with opening of pre sigmoid mastoid air chamber combined with closure of sigmoid sinus diverticulum. Conclusions: Venous pulsatile tinnitus is common in women. The common causes may be sigmoid sinus wall abnormalities such as sigmoid sinus diverticulum and perisigmoid bone defect. Imaging examinations are helpful for diagnosis. Venous pulsatile tinnitus can be treated with surgery.

Sigmoid sinus diverticulum without pulsatile tinnitus occurring concurrently with chronic otitis media mucosal type.

Sigmoid sinus diverticulum is a rare vascular anomaly often associated with pulsatile tinnitus. It can occur in cases of chronic otitis media squamous type (unsafe type) due to dehiscence of the sigmoid sinus plate caused by cholesteatoma. The presentation of which is that of pulsatile tinnitus. However, we present an unusual case of sigmoid sinus diverticulum occurring concurrently with chronic otitis media mucosal type (safe type) but in the absence of pulsatile tinnitus. This case report highlights the diagnostic challenges and management of this rare clinical scenario.

Sigmoid Sinus Wall Anomalies Can Progress and May Not Be Congenital.

OBJECTIVE: Sigmoid sinus wall anomalies (SSWA) are closely linked to venous pulsatile tinnitus (PT). This study aims to demonstrate that SSWA develops progressively rather than being congenital. METHODS: We retrospectively analyzed 42 PT patients with SSWA who had at least two non-operative CT scans at our clinic. CT images were longitudinally assessed to track SSWA progression, while MRI and Doppler ultrasound evaluated transverse sinus stenosis and venous hemodynamics. Changes in PT perception were tracked using the tinnitus handicap inventory (THI) questionnaire. RESULTS: Among the 42 SSWA patients, 12 (28.6%) exhibited progression. Anastomosis between diploic vein and diverticulum was significantly higher compared to the dehiscence cohort (p < 0.01). Within the diverticulum group, seven individuals (30.4%) experienced enlargement, with a mean diverticular wall expansion of 5.9% ± 11.4%. Progressive erosion was observed in two cases (12.5%) in the dehiscence cohort, with a mean sigmoid plate erosion of 3.8% ± 10.1%. In cases progressing from dehiscence to diverticulum, three subjects transitioned, with a mean sigmoid sinus wall length expansion of 43.8% ± 31.9%. SSWA progression showed a significant negative correlation with QBILATERAL (r = -0.857, p = 0.014), and there was a significant difference between initial and revisit THI scores (p < 0.01). CONCLUSION: SSWA can undergo morphological progression, indicating it is a progressive clinical condition rather than congenital. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:4707-4715, 2024.

Dural sinus malformation presenting with proptosis: report and review of a rare entity.

Dural sinus malformation (DSM) is a rare vascular malformation characterized by the dilatation of a dural venous sinus with or without an anomalous jugular bulb. Its presentation with venous-reflux-related secondary proptosis is anecdotal, with only six such cases reported so far. We report a 17-month-old boy who presented with a progressive proptosis of the right eye secondary to a DSM of the transverse sinus and torcula. Following endovascular embolization of the arterio-venous fistula, complete thrombosis of the venous lake and improvement in proptosis was noted at 6-month follow-up. Prognosis of this rare malformation is variable and dependent on specific angio-architectural features.

The Petrosquamosal Sinus: A Rare Vascular Variant Encountered During Cochlear Implantation Surgery.

The petrosquamosal sinus is a rare and almost unknown emissary vein of the temporal bone. It traverses the middle cranial fossa along the petrosquamosal fissure and connects dural sinuses with extracranial venous networks. It is known to undergo normal involution during fetal and early postnatal life, and therefore is rarely found in humans. When encountered during surgery, it can be a significant source of bleeding hindering proper visualization of important surgical landmarks. Here we report the case of a PSS, encountered in a child during cochlear implantation surgery. We discuss its clinical significance and impact on the surgical approach. Laryngoscope, 134:4111-4113, 2024.

Magnetic Resonance Imaging of Dural Sinus Malformation in a Fetus: A Case Report.

BACKGROUND: Dural sinus malformation (DSM) is a rather rare congenital condition that can be encountered in the fetus and infants. The cause and etiology of DSM remain unclear. Obstetric ultrasound plays a key role in screening fetal brain malformations, and MRI is frequently used as a complementary method to confirm the diagnosis and provide more details. OBJECTIVE: Here, we present a fetus with DSM by multiple imaging methods to help better understand the imaging characteristics of this malformation. CASE PRESENTATION: A 22-year-old primipara was referred to our hospital at 25 weeks of gestation following the detection of a fetal intracranial mass without any symptoms. A prenatal ultrasound performed in our hospital at 25 + 2 gestational weeks showed a large anechoic mass with liquid dark space, while no blood flow was detected. After the initial evaluation, this primipara received a prenatal MRI in our hospital. This examination at 25 + 5 gestational weeks delineated a fan-shaped mass in the torcular herophili, which was iso-to hyperintense on T1WI and hypointense on T2WI. At the lower part of this lesion, a quasi-circular hyperintense on T1WI and a signal slightly hyperintense on T2WI could be seen. Meanwhile, the adjacent brain parenchyma was compressed by the mass. CONCLUSION: We reviewed the current literature to obtain a better understanding of the mechanisms, imaging characteristics, and survival status of DSM. Although the primipara of the present study regretfully opted for elective termination of pregnancy, the reevaluation of DSM survival deserves more attention because of the better survival data from recent studies.

Remodeling of venous drainage after the treatment of dural sinus malformation with arteriovenous shunts in a neonate: a case report and literature review.

Dural sinus malformations (DSMs) are rare congenital vascular diseases characterized by a giant venous pouch with or without arteriovenous shunts. We present a neonatal case of DSM that was diagnosed prenatally and treated via endovascular intervention in the early postnatal period. The patient presented with a large DSM involving the torcular Herophilion prenatal magnetic resonance imaging (MRI). Enlargement of the head circumference and respiratory failure rapidly progressed after birth. On the 5th day after birth, the neonate underwent endovascular occlusion via the umbilical artery. The arteriovenous shunt was occluded, and the reflux from the enlarged venous pouch to the dural sinus was decreased. No additional procedure other than ventriculoperitoneal shunting was required. The neonate's development slowly caught up to normal parameters. Follow-up MRI demonstrated the successful development of the venous drainage system. DSMs are characterized by an abnormally dilated dural sinus, which can block the venous return and ultimately increase intracranial pressure and cerebral ischemia. Long-term follow-up indicates that an abnormally developed dural sinus can be reconstructed by appropriate and timely treatment.

Cerebral neurovascular embryology, anatomic variations, and congenital brain arteriovenous lesions.

Cerebral neurovascular development is a complex and coordinated process driven by the changing spatial and temporal metabolic demands of the developing brain. Familiarity with the process is helpful in understanding neurovascular anatomic variants and congenital arteriovenous shunting lesions encountered in endovascular neuroradiological practice. Herein, the processes of vasculogenesis and angiogenesis are reviewed, followed by examination of the morphogenesis of the cerebral arterial and venous systems. Common arterial anatomic variants are reviewed with an emphasis on their development. Finally, endothelial genetic mutations affecting angiogenesis are examined to consider their probable role in the development of three types of congenital brain arteriovenous fistulas: vein of Galen malformations, pial arteriovenous fistulas, and dural sinus malformations.

Study on the correlation between hemodynamic status of the transverse sinus-sigmoid sinus and the clinical efficacy of sigmoid sinus wall reconstruction.

BACKGROUND AND PURPOSE: For patients with pulsatile tinnitus who have both transverse sinus stenosis and sigmoid sinus wall anomalies, sigmoid sinus wall reconstruction surgery is the first-choice treatment when the trans-stenotic pressure gradient less than 10 mmHg. However, not all patients are cured by surgery. We hypothesized the abnormal hemodynamics caused by transverse sinus stenosis is associated with the clinical efficacy of surgery. METHODS: Eight pulsatile tinnitus patients treated with surgery were retrospectively reviewed (4 rehabilitated, 4 nonrehabilitated). All patients had radiologically diagnosed transverse sinus stenosis and sigmoid sinus wall anomalies. A numerical simulation of the hemodynamics of the transverse sinus-sigmoid sinus was performed using computational fluid dynamics technology. Changes in the blood flow patterns before and after surgery were observed. The blood flow velocity at the stenosis, vorticity of blood flow in the sigmoid sinus and wall pressure distribution in the sigmoid sinus wall anomalies area were compared. RESULTS: The blood flow velocity in the stenosis (preoperative P = 0.04, postoperative P = 0.004) and vorticity in the sigmoid sinus (preoperative P = 0.02, postoperative P = 0.007) pre- and post-surgery were significantly higher in the non-rehabilitation group than in the rehabilitation group. No significant difference was found in the wall pressure distribution in the sigmoid sinus wall anomalies area (preoperative P = 0.12, postoperative P = 0.24). CONCLUSIONS: There is a clear correlation between the abnormal hemodynamic status caused by transverse sinus stenosis and the clinical efficacy of surgery. The blood flow velocity at the stenosis and vorticity of blood flow in the sigmoid sinus are factors influencing the clinical efficacy of surgery.

Associations among Audiometric, Doppler Hydroacoustic, and Subjective Outcomes of Venous Pulsatile Tinnitus.

OBJECTIVE: Venous pulsatile tinnitus (PT) has received increasing attention recently. As analyses of psychophysical and neuropsychological dimensions of venous PT are lacking, this study aimed to quantitatively and qualitatively investigate the correlation among audiometric, hydroacoustic, and subjective outcomes in patients with PT. METHODS: Fifty-five venous PT patients, with or without sigmoid sinus wall anomalies (SSWAs), were subdivided into SSWAs (n = 30) and non-SSWAs (n = 25) groups. Audiometric and hemodynamic evaluations were assessed. Questionnaires including the Tinnitus Handicap Inventory, Hospital Anxiety and Depression Scale (HADS), and Athens Insomnia Scale (AIS) were deployed to evaluate the psychological impacts of PT. RESULTS: Among 55 subjects, PT frequency-related pure-tone audiometry (PTA) was significantly different between ipsilesional non-PT frequency-related PTA (p < 0.01), ipsilateral jugular vein compression PTA (p < 0.01), and contralesional ear PTA (p < 0.01). In contrast with the pulsatility index and flow velocity, bilateral EOET and flow volume were significantly different (p < 0.01). Of the 3 questionnaire types, there was a strong correlation between HADS anxiety and AIS scores (r = 0.658, p < 0.01). The duration of PT was not correlated with subjective outcomes, and there was no statistical significance found among audiometric, hemodynamic, and subjective outcomes between SSWAs and non-SSWAs groups. CONCLUSIONS: (1) The duration of PT was irrelevant to the increase of PTA. (2) Venous PT is the perception of vascular flow sound, in which hydroacoustic characteristics can be highly independent. (3) Anxiety, depression, and sleep disorders commonly prevail among PT patients.

Fetal Cerebral Sinovenous Thrombosis and Dural Sinus Malformation.

BACKGROUND: Fetal cerebral sinovenous thrombosis (CSVT) and dural sinus malformation (DSM) are rare types of fetal cerebral venous pathology that are becoming increasingly recognized as fetal imaging advances. Fetal DSMs are a common source of fetal CSVT, although CSVT may occur without a DSM. The literature on these disorders is limited. METHODS: Cases of fetal CSVT and DSM were identified retrospectively through a query of the Indiana University Health fetal imaging archive from 2007 to 2021. RESULTS: Seven cases were identified, all of whom were alive at birth. A DSM was present in six. Treatments after birth included enoxaparin sodium (3), embolization (3), and shunt placements (1). Five cases had documented regression or complete resolution of the thrombus and/or malformation. One was lost to follow-up, one died from complications of hydrocephalus at nine months, one was receiving physical and occupational therapy at last follow-up at three months, one had concern for autism and mild gait abnormality at 21 months, two had concern for speech delay (18 months and 24 months), and one had normal development at most recent follow-up (four years). CONCLUSIONS: Positive short-term outcomes may occur for some cases of fetal CSVT and DSM. However, risk factors and best treatments are not clear, and long-term outcome data are limited. There is a need for further study.

Successful endovascular therapy for an adolescent patient with cerebral venous sinus thrombosis: A case report.

RATIONALE: Cerebral venous sinus thrombosis associated with protein S deficiency is rare in adolescent patients and has high disability and fatality.Patient concerns: A 15-year-old male student presented in the hospital with sudden headache, nausea, and vomiting and was diagnosed with protein S deficiency by gene testing. DIAGNOSES: Cerebral venous sinus thrombosis due to protein S deficiency was diagnosed in this adolescent patient, who underwent successful endovascular therapy (EVT). INTERVENTIONS: The patient was treated with standard anti-coagulation therapy including low-molecular-weight heparin (90 IU/kg/Q12 h) and dehydrant (mannitol 125 mL Q8 h); however, the symptoms were not alleviated. Successful EVT was implemented. OUTCOMES: Both the superior sagittal sinus and bilateral transverse sinus were recanalized after thrombus clearance. The patient achieved a complete recovery without any other stroke recurrence during follow-up. LESSONS: EVT can be performed with favorable and effective clinical outcomes in adolescent cerebral venous sinus thrombosis patients with protein S deficiency. EVT associated with standard anti-coagulation therapy may improve the prognosis and reduce mortality among such patients.

Dural Venous Sinus Variations in Idiopathic Subarachnoid Hemorrhage: A New Indicator of the Venous Origin with Diagnostic Usefulness?

OBJECTIVE: Venous hypertension associated with a primitive basal vein of Rosenthal (BVR) has been noted as the most likely cause of idiopathic subarachnoid hemorrhage (iSAH). Other types of venous drainage variations have been scarcely studied but may further explain the cases not associated with a BVR anomaly. Our aim was to investigate if dural venous sinus (DVS) anomalies are related with iSAH. METHODS: A total of 76 patients diagnosed with iSAH were identified from a prospectively maintained database and their angiographic findings compared with 76 patients diagnosed with aneurysmal subarachnoid hemorrhage. RESULTS: On top of the BVR variations, our data showed a higher prevalence of transverse sinus hypoplasia (47.4% vs. 28.9%; P = 0.019), superior petrosal sinus hypoplasia (32.9% vs. 13.2%; P = 0.003), and clival plexus hyperplasia (65.8% vs. 43.4%; P = 0.005) in patients with iSAH. Analyzing by total number of angiograms, the iSAH group showed also a higher prevalence of inferior petrosal sinus hyperplasia (36.2% vs. 25%; P = 0.003). Of the patients with iSAH without a primitive BVR, 84% harbored ≥1 perimesencephalic DVS variation and the overall number of venous drainage variations was significantly higher in patients with iSAH. CONCLUSIONS: In addition to the well-documented BVR anomalies, there seems to be a significant relationship of other DVS variations in patients with iSAH. Transverse sinus hypoplasia, superior petrosal sinus hypoplasia, inferior petrosal sinus hyperplasia, and clival plexus hyperplasia were significantly more frequent in patients with iSAH. The presence of ≥3 of those variations would increase the suspicion of a nonaneurysmatic subarachnoid hemorrhage and could help avoid a second angiogram.

Hydroacoustic analysis and extraluminal compression surgical insights of venous pulsatile tinnitus.

OBJECTIVE: This study aimed to quantitatively and qualitatively evaluate the hydroacoustic changes from "presence" to "disappearance" of pulsatile tinnitus (PT) with the extraluminal compression surgical technique. The recent issues of concern pertaining to the hydroacoustic characteristics of sigmoid sinus wall anomalies and distal transverse sinus stenosis (dTSS) were discussed. METHODS: This study was based on a retrospective case series. Seventy-seven patients with PT and transverse-sigmoid sinus enlargement with or without transverse-sigmoid sinus junction anomalies and transverse sinus stenosis (TSS) who had undergone extraluminal compression surgery under local anesthesia were included. Management of intractable intraoperative challenges and techniques for reversal extraluminal compression were introduced. Anatomical measurements, intraoperative color-coded Doppler ultrasonography, spectro-temporal analysis, and computational fluid dynamics were employed to analyze the hydroacoustic characteristics of PT. RESULTS: The efficacy of the extraluminal compression technique was evident with the significant reduction in peak turbulent kinetic energy, vorticity, and mean pressure gradient at the transverse-sigmoid junction, resulting in over 20% reduction in PT amplitude. dTSS is a common finding in patients with PT exhibiting transverse-sigmoid sinus enlargement. Patients with dTSS presented with significant differences in hemodynamic characteristics as compared to those without. Linear regression analysis showed that the flow disturbance (turbulent kinetic energy and vorticity) was closely associated with the degree of dTSS, whereas the flow amplitude was not related to the degree or location of TSS. Low-pulsatory vortex flow at the transverse-sigmoid junction was visualized during an intraoperative color-coded Doppler examination, and the displayed low-frequency PT sound corresponded to the patients' subjective perception of PT. CONCLUSION: (1) A reduction of over 20% of the flow-induced noise is the therapeutic goal of extraluminal compression technique. Since reductions in the magnitude of hemodynamic parameters, including turbulent kinetic energy, vorticity, and mean pressure gradient, render the flow-induced noise inaudible, besides sigmoid sinus wall anomalies, it is likely that PT develops from the aggregation of flow-based pathologies. (2) Although dTSS and diverticulum may greatly affect the hemodynamics at the transverse-sigmoid junction, in contrast to dehiscence, dTSS and diverticulum may not be the limiting factors for PT development.

Transmastoid Hydroxyapatite Resurfacing for Sigmoid Sinus Wall Anomalies Causing Pulsatile Tinnitus.

OBJECTIVE: Comprising 4% of tinnitus, pulsatile tinnitus (PT) can be particularly difficult for affected patients as well as surgeons looking to address their symptoms. Often the cause is not identified but can be secondary to turbulent flow in or near the sigmoid sinus, particularly if there is an identifiable sigmoid sinus dehiscence (SSDe) and/or diverticulum (SSDi). These sigmoid sinus wall anomalies (SSWA) may be treated with transmastoid sigmoid sinus resurfacing; however, this intervention remains relatively novel and its technique, materials used, resolution success, and complications need to be continuously reviewed. METHODS: A retrospective case series of patients with PT due to SSWA at a tertiary referral center was reviewed. A total of 6 patients (7 ears) treated by transmastoid resurfacing using hydroxyapatite (HA) were retrospectively assessed. Pre-operative demographics and symptoms, pre- and post-operative hearing results, and post-operative outcomes were reviewed. RESULTS: All patients were female with an average BMI of 32.9 (±5.4) and a mean age of 45.5 years (±15.3). Mean follow-up was 648 days. Objective tinnitus was noted in all ears with SSDi (100%); however, no objective tinnitus was noted with purely SSDe. In 100% of ears, PT was diminished with ipsilateral jugular compression and was amplified with contralateral head turn. Pre-operative symptoms of PT resolved in all patients, but delayed recurrence (>1 year) occurred in 1 ear (14%). No patient had pre- or post-operative hearing loss. No major complications were encountered. CONCLUSIONS: Transmastoid resurfacing for SSWA with HA bone cement is a safe, reliable intervention in properly identified PT patients.

Two cases of persistent falcine and occipital sinuses.

BACKGROUND: The coexistence of falcine and occipital sinuses is rare and its natural course has not been reported. CASE REPORTS: Two patients with persistent falcine and occipital sinuses are described. Both patients had straight sinuses. In one, both the transverse and sigmoid sinuses were hypoplastic and the patient had an acquired Chiari I malformation. The other patient had no other venous anomalies and had a normal posterior cranial fossa. CONCLUSION: The coexistence of falcine and occipital sinuses can lead to an acquired Chiari I malformation. These cases suggest the importance of checking other venous and brain anomalies in this situation.