ArticleLiterature Review

Pulsatile tinnitus: Contemporary assessment and management

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Abstract

Pulsatile tinnitus is an uncommon otologic symptom, which often presents a diagnostic and management dilemma to the otolaryngologist. The majority of patients with pulsatile tinnitus have a treatable cause. Failure to establish correct diagnosis may have disastrous consequences, because a potentially life-threatening, underlying disorder may be present. The purpose of this review is to familiarize the otolaryngologist with the most common causes, evaluation, and management of pulsatile tinnitus. The pathophysiology, classification, various causes, evaluation, and management of the most common causes of pulsatile tinnitus are presented in this review. Pulsatile tinnitus deserves a thorough evaluation and, in the majority of cases, there is a treatable underlying cause. The possibility of a life-threatening cause needs to be ruled out in every patient with pulsatile tinnitus. The otolaryngologist should be familiar with the evaluation and management of this symptom.

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... Venous pulsatile tinnitus (VPT) is defined as the perception of a sound in the absence of an external stimulus, synchronous with the heartbeat and interrupted by compression of the ipsilateral internal jugular vein [1,2]. VPT can signif-icantly reduce patients' quality of life leading to insomnia or severe depression [3]. ...
... Since the first cases of stenting in dural venous sinuses in the 1990s [9,10], LSS stenting has proven to be an effective treatment option, regardless of whether the stenosis is associated with IIH and/or disabling VPT [3,5,[11][12][13]. Even when the clinical criteria of VPT [1,2] and the radiological criteria of LSS [3] are present, the decision to place a stent is confirmed by recording the trans-stenotic pressure gradient (TSG) [14]; however, there is no consensus on the gradient threshold to confirm this indication (4-10 mm Hg) [15]. Furthermore, the use of endovascular microcatheters for pressure measurement has limited accuracy with an error margin that can be up to 3 mm Hg [16]. ...
... The characteristics of the population with VPT caused by LSS in our study were similar to those in the literature [1,2]: they were overweight young adults, with a clear female predominance. We do not report any serious clinical adverse events after stenting. ...
Article
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Background and Purpose Lateral sinus stenosis is the most common cause of venous pulsatile tinnitus (VPT). Stenting is an effective treatment after demonstration of a trans-stenotic pressure gradient; however, pressure measurement has many technical limitations. In 2018, a study showed that a combined approach with intravascular velocity measurement could be effective in identifying most appropriate candidates for stenting. The aim of the present study was to evaluate a new strategy using this biomarker for the indication of stenting even without a significant pressure gradient. Material and Methods Consecutive patients with disabling VPT were included from 2016 to 2019 and analyzed retrospectively. Intrasinusal pressures were measured and blood flow velocities (with a dual-sensor guidewire) were used for the indication of stenting independent of the pressure gradient. We evaluated the clinical outcome after stenting based on this new biomarker. Results A total of 41 patients were treated according to this strategy. At last follow-up (mean = 30.2 months), 32/33 patients (97%) treated by stenting showed complete resolution or a significant decrease in VPT intensity. The use of velocity as the threshold for indicating stenting identified 8 patients (24%) missed by the pressure gradient. Their clinical outcome after stenting was excellent and no complications occurred. Conclusion Measurement of sinus blood flow velocity provides a hemodynamic explanation of disease and may be a better tool than pressure gradient for the indication of stenting in VPT.
... P ulsatile tinnitus (PT) is the auditory perception of rhythmic noise synchronous with the heartbeat (1). PT may be debilitating, impacting quality of life and sleep (2). ...
... The choice of imaging study should be on the basis of clinical findings. MRI of the head and internal auditory canal without and with contrast material remains the study of choice for patients with non-PT or when there is associated vertigo, dizziness, or hearing loss to exclude cerebellopontine angle cistern or internal auditory canal lesions (1). MRI of the brain and MR venography are the appropriate initial studies in patients with clinical suspicion of IIH because they are more effective in ruling out a mass lesion and a dural sinus thrombosis (22), although IIH can be assessed reliably at CT. CT angiography and venography of the head and neck can be performed as the first examination in patients with PT (Fig 1). ...
... It helps to assess the arterial and venous systems when evaluating vessel size, patency, stenosis, symmetry, dissection, aneurysms, and diverticula. High-spatial-resolution images in bone, softtissue algorithms, and multiplanar reconstructed images permit evaluation of the ear structures, venous sinus walls, and nonvascular causes of PT (1,4,12,13,16,17). MRI or CT can be used as first-line imaging method to identify dural arteriovenous fistulas because of their noninvasive nature (14,23). ...
Article
Venous variants and pathologic abnormalities are the most common causes of pulsatile tinnitus. These conditions include causes of turbulence within normally located veins and sinuses, and abnormally enlarged or abnormally located veins in close transmissive proximity to the conductive auditory pathway. Such disorders include pathologic abnormalities of the lateral sinus (transverse sinus stenosis and sigmoid sinus wall anomalies), abnormalities and variants of the emissary veins, and anomalies of the jugular bulb and jugular vein. Despite being the most common causes for pulsatile tinnitus, venous variants and pathologic abnormalities are often overlooked in the workup of pulsatile tinnitus. Such oversights can result in delayed patient care and prolonged patient discomfort. Advances in both cerebrovascular imaging and endovascular techniques allow for improved diagnostic accuracy and an increasing range of endovascular therapeutic options to address pulsatile tinnitus. This review illustrates the venous causes of pulsatile tinnitus and demonstrates the associated endovascular treatment. © RSNA, 2021.
... Meanwhile, pulsatile tinnitus (PT) is caused by altered vascular hemodynamics, such as turbulent blood flow or vibration of a dehiscent vascular wall, or abnormal perception of the sound of normal flow, such as third-window lesions due to bony defects of the inner ear. Venous PT is characterized by auditory perception of a pulse-synchronous sound, which is usually suppressed by compressing internal jugular vein on the symptomatic side (4,5). ...
... Therefore, better objective tests are required to identify lesions causing PT and predict symptomatic improvement after treatment. Previous studies reported that sigmoid sinus dehiscence (SS-Deh) and sigmoid sinus diverticulum (SS-Div), which were the most frequent vascular abnormalities in patients with venous PT, were also found in individuals without PT (4,(9)(10)(11). ...
Article
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Venous pulsatile tinnitus (PT) is characterized by an auditory perception of pulse-synchronous sound, suppressed by compression of the ipsilateral internal jugular vein. We sought to determine the preoperative prognostic significance of the effect of ipsilateral neck manual compression on the PT loudness and audiometric changes in patients with sigmoid sinus dehiscences (SS-Deh) and diverticula (SS-Div) by comparing postoperative improvements in ipsilateral low-frequency hearing loss (LFHL) in pure-tone audiogram (PTA) and PT symptoms. Twenty-two subjects with PT originating from SS-Deh/Div were recruited. Air-conduction hearing thresholds were measured using PTA at three time points: twice preoperatively (with neutral neck position and with ipsilateral manual compression of internal jugular vein) and once at 3-months postoperatively with neutral neck position. We defined a positive neck compression effect as a threshold improvement of ≥ 10 dB HL at 250 or 500 Hz after manual neck compression. All but two subjects presented with ipsilateral LFHL in the neutral position. The average hearing threshold in the neutral position markedly improved after manual neck compression, indicating that LFHL originated from the masking effect of venous PT. All subjects had subjective improvements in PT and LFHL after sigmoid sinus surgeries, confirming that LFHL resulted from the masking effect of PT. Additionally, improvement of LFHL after neck compression could be regarded as a positive prognostic indicator after surgery. Collectively, elimination of PT loudness and improvement of LFHL with manual compression over the ipsilateral neck may suggest the venous origin of the PT and predict a favorable outcome following repair of SS-Deh/SS-Div.
... 24 Dissections have been reported associated with events such as sports, shaving, or childbirth as well as spontaneously. 18,25,26 These patients may present with deafness, vertigo, and facial nerve weakness and even nausea, vomiting, and cranial nerve involvement with more severe diseases. 27 PT can be the sole presenting symptom. ...
... They are less often associated with PT, although cases have been documented. 25,36 The mean age (30) of occurrence of intracranial dissections is significantly younger than those that occur extracranially. 37 These dural dissections have been associated with trauma, severe stretch, or compression; Marfan syndrome; and Ehlers-Danlos type IV. ...
Article
Traditionally in the domain of the otolaryngologist, pulsatile tinnitus (PT) has become increasingly relevant to neurosurgeons. PT may prove to be a harbinger of life-threatening pathology; however, often, it is a marker of a more benign process. Irrespectively, the neurosurgeon should be familiar with the many potential etiologies of this unique and challenging patient population. In this review, we discuss the myriad causes of PT, categorized by pulse-phase rhythmicity.
... While setting apart the sigmoid sinus and airy honeycomb-like mastoid cavity, this solid sigmoid plate is deemed to preclude the vascular sounds in the posterior fossa from transmitting to the inner ear. (1)(2)(3)(4)(5)(6) Unfortunately, in some individuals, the sigmoid plate is not intact. A growing number of clinical studies have indicated a strong possibility that the unremitting transverse sinus jet-flow impingement erodes the sigmoid plate. ...
... Venous PT is the self-perception of the vascular sound derived from the dural venous sinus. (1,(5)(6)(7)(8)(9) It is estimated that approximately 20 to 48% of venous PT patients present SSWAs. (9,10) Through extrapolation of surgical successes, PT associated with a dehiscent sigmoid plate is hypothesized to emerge under two major scenarios (see also Fig. 1). ...
Article
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The vibrations of a thinned sigmoid plate and exposed sinus vessel wall in patients with sigmoid sinus wall anomalies (SSWAs) have long been speculated to be a pivotal cause of venous pulsatile tinnitus (PT). This is the first in vivo study to investigate whether vibrations resulting from SSWAs induce PT using multiple sensor systems. A confocal laser displacement sensor system was intraoperatively deployed to gauge displacements of the focal anatomical structure in 12 subjects with venous PT with or without SSWAs. Doppler ultrasonography was performed to detect the blood flow and psychoacoustic characteristics of PT. Computational fluid dynamics (CFD) and piezoelectric sensors were deployed to assess the relationship between the vessel wall pressure and hemodynamics. The ultrasonographic examination showed that the PT pitch matched the blood flow sampled at the center of the sinus lumen in all subjects and that there was no statistical difference between the displacements of the sigmoid plate and vessel wall before separation, indicating that PT is strongly associated with flow-induced noise rather than vibration-induced noise. In addition, the quantitative water occlusion test can be potentially indicative for the surgical and mechanistic study of PT.
... [5][6][7] Consequently, surgical interventions have been utilized to correct anatomical anomalies or reconstruct bony deficiencies, which have achieved satisfactory outcomes. 8,9 However, surgical intervention does not relieve PT in all patients. 10 Therefore, further investigation exploring potential underlying factors is desirable. ...
... In the remaining 173 patients, variable structural abnormalities or deficiency surrounding the JF region were identified. An ipsilateral dominant JF was identified in 131 patients (67.2%) with PT, including the right side (113, 58.0%) or left side (18,9.2%); a balanced distribution of the reflux jugular bulb was present in another 40 patients (20.5%). ...
Article
Objective Structural anomalies of the jugular foramen (JF) and adjacent structures may contribute to development of pulsatile tinnitus (PT). The goal of this study was to assess anatomical variants in the ipsilateral JF region in patients with PT and to explore possible predisposing factors for PT. Materials and Methods One hundred ninety-five patients with PT who underwent CT angiography and venography of the temporal bone were retrospectively analyzed. Anatomic variants including dominance of the ipsilateral JF, bony deficiency of the sigmoid sinus and internal carotid artery canal, high riding or dehiscent jugular bulb, dehiscence of the superior semicircular canal, tumors in the JF region, or cerebellopontine angle were assessed. Results Of 195 patients with PT, the prevalence of a dominant JF on the ipsilateral side of patients with PT was 67.2%. Furthermore, the dominant JF demonstrated a significant correlation with the presence of ipsilateral PT (p < 0.001). No anatomical variants were present in 22 patients (11.3%), whereas in patients with structural variants, bony deficiency of the sigmoid sinus was most common (65.6%), followed by high riding (54.9%) or dehiscent jugular bulb (14.4%). Dehiscent internal carotid artery canal (3.1%) and superior semicircular canal (4.1%) were occasionally identified, while arteriovenous fistula, arterial aneurysm and tumors arising from the JF region or cerebellopontine angle were rarely encountered. Conclusion Structural abnormalities of the JF and adjacent structures may predispose to the development of PT. Knowledge of these anatomical variants in the JF region may help establish a clinical strategy for addressing PT.
... IIH is considered the most common cause of venous PT and the most common cause of PT overall (up to 39%) [22]. Other common symptoms and signs include headache (often worse supine, with Valsalva, and in the morning), visual disturbances, hearing loss, ear fullness, cranial nerve palsies (most commonly fifth, sixth and seventh) and papilledema [23]. ...
Article
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Purpose of Review The purpose of this review is to provide an updated approach to the evaluation and management of pulsatile tinnitus (PT), an uncommon but often treatable subtype of tinnitus. Recent Findings Secondary PT can be due to either vascular or non-vascular etiologies, including, but not limited to: neoplasm, arteriovenous malformation or fistula, idiopathic intracranial hypertension, dural venous sinus stenosis, otoacoustic etiologies (e.g., otosclerosis, patulous eustachian tube) and bony defects (e.g., superior semicircular canal dehiscence). Computed tomography (CT) and magnetic resonance imaging (MRI) imaging have comparable diagnostic yield, though each may be more sensitive to specific etiologies. If initial vascular imaging is negative and a vascular etiology is strongly suspected, digital subtraction angiography (DSA) may further aid in the diagnosis. Many vascular etiologies of PT can be managed endovascularly, often leading to PT improvement or resolution. Notably, venous sinus stenting is an emerging therapy for PT secondary to idiopathic intracranial hypertension with venous sinus stenosis. Summary Careful history and physical exam can help establish the differential diagnosis for PT and guide subsequent evaluation and management. Additional studies on the efficacy and long-term outcome of venous sinus stenting for venous stenosis are warranted.
... [1][2][3][4] In this particular form of PT, irregularities in the temporal bone or vascular structures within the transverse-sigmoid sinus can result in the transmission of blood flow sounds through the air conduction route to the inner ear, situated within the mastoid cavity. 4,5 Alongside distinctive physical and radiological examinations, alternative objective techniques, like acoustic sensing and water occlusion tests, play a pivotal role in discerning the sources of vascular PT. [5][6][7][8] In addition to SSWA, transverse sinus stenosis (TSS) and symptoms associated with idiopathic intracranial hypertension (IIH) often coexist in individuals with venous PT. [9][10][11][12] Several post hoc analyses of surgical and radiological studies have delved into this correlation. [12][13][14] However, it's worth noting that IIH-related symptoms are less prevalent among Asian patient populations. ...
Article
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 Q BILATERAL ( 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.
... The hyperdynamic blood flow may also result from arterial hypertension [60]. Thyrotoxicosis or pregnancy can also present with PT due to increased cardiac output [7,61], and a correction of these underlying medical conditions can abate the symptom. ...
Chapter
Pulsatile tinnitus (PT) is characterized by an auditory perception of pulse-synchronous sound. An alteration in the vascular hemodynamics causing turbulent flow, a vibration of a dehiscent vascular wall, or third window lesions are potential mechanisms of PT. Vascular PT can be subdivided into arterial, arteriovenous, and venous lesions. When the patient complains of psychoacoustic characteristics of PT, detailed physical examination and audiological evaluation complemented by etiology-targeted laboratory tests can provide diagnostic clues. Moreover, radiological evaluations such as brain magnetic resonance imaging (MRI) with angiography, temporal bone high-resolution computed tomography (CT), Doppler ultrasonography, and classical transfemoral cerebral angiography are recommended. Of various pathologies causing PT, dehiscence or diverticulum of the sigmoid sinus or jugular bulb is the most common cause that can be cured by surgical or interventional treatments. In addition, benign intracranial hypertension (BIH) is a common cause of PT. Although 15–50% of patients present with no definite diagnosis even after a meticulous diagnostic workup, the identification of the causal vascular pathology is vital for the optimal treatment of PT because successful surgical or interventional management is available in many cases.
... For a significant fraction of patients the underlying diagnosis remains elusive, a condition referred to as idiopathic pulsatile tinnitus. A typical evaluation entails imaging of the intra-cranial and cervical vasculature with MR or CT angiogram, fundoscopic examination to evaluate for idiopathic intracranial hypertension, and otological evaluation [1]. ...
Article
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Background: Pulsatile tinnitus is a pulse synchronous audible whooshing sensation that arises from turbulent flow in stenotic intracranial or upper cervical arteries, amidst other pathologies, yet is often idiopathic in origin. Raynaud’s syndrome involves peripheral vasoconstriction, but possibly also cerebral vasoconstriction. The presence of cerebral vasoconstriction in Raynaud’s was evaluated by ascertaining the prevalence of pulsatile tinnitus compared to a control population.
... (1) This type of tinnitus is subcategorized under objective tinnitus, for which anatomical lesions or vascular sound can be objectively found or perceived by an observer. (2) Venous PT is the most common vascular PT, (3) which can be silenced or reduced by digital compression over the ipsilateral internal jugular vein (IJV). Despite various causative and contributory factors of PT, the most common abnormal anatomical findings are sigmoid sinus wall anomalies (SSWAs), namely, sigmoid sinus wall dehiscence and sigmoid sinus diverticula. ...
Article
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Venous pulsatile tinnitus (PT) arises from the motion of blood flow. However, the correlation between flow velocity and amplitude remains undiscovered. In this study, retroauricular colorcoded Doppler (RCCD) and transcranial color-coded Doppler (TCCD) ultrasound examination techniques were deployed to assess the hemoacoustics at the ipsilateral internal jugular vein (IJV), intradiverticular, mainstream sinus, and transverse sinus regions. Auto- and crosscorrelation analyses were used to analyze the correlations between flow velocity and amplitude. Furthermore, the Mel-frequency cepstrum coefficients were calculated, and the Melspectrogram was used to exhibit the human perception of PT. The mainstream sinus flow had the highest coefficient (cross-correlation coefficient = 0.781) among the sensed locations. The cross-correlation coefficient of the IJV was the second largest and close to that of the mainstream sinus flow. The transverse sinus flow had the lowest cross-correlation coefficient. Additionally, the transverse sinus septum was visualized for the first time using the RCCD technique in this study. In conclusion, cross-correlation analysis indicates that the amplitude of vascular sound is highly correlated to the vascular flow velocity. The Mel-spectrogram demonstrates the outcome of the human perception of PT, and its use can be extended to future psychoacoustic studies.
... Pulsatile tinnitus (PT) is defined as the perception of a sound in the absence of external stimulus, synchronous with the heartbeat [1]. It may have a venous origin when the sound is generated by the venous flow near the inner ear. ...
... Contralateral venous compression, the Muller maneuver, and rotation of the head to the opposite side will increase it. 12,17,19,25 In tinnitus of arterial origin, strong compression of the carotid artery will decrease or eliminate the tinnitus. Slight compression on the ipsilateral veins, the Valsalva and Muller maneuvers, and rotation of the head toward the ipsilateral or opposite side has no effect. ...
Article
Somatosound (somatic tinnitus) is associated with vascular, musculoskeletal, respiratory, or temporomandibular joint disorders. Several studies of its management have been widely reported, but only few presented long-term follow-up results. The purposes of this paper are to review the causes and management, present cases with long-term follow-up, together with previously reported cases in literatures. We treated nine patients with somatosound of vascular, hematologic, endocrinologic, muscular, and cervical origin. Follow-up were conducted routinely, and the final results were collated in 3 to 11 years. Patients with non-life-threatening causes were given counseling and palliative management. Their tinnitus becomes tolerable, gradually decreased, and even disappeared. Patients with life-threatening causes were treated immediately. Among all, there were three cases which to the best of our knowledge, are the first reported of its causes. One case was caused by a compensatory of increasing blood flow in internal carotid artery (ICA) secondary to contralateral ICA stenosis. Another had a combination of anemia and an ipsilateral jugular bulb diverticulum. The tinnitus disappeared after the anemia treated. The last was patient with hyperthyroidism. The tinnitus disappeared by controlling the condition. Even when the causes are benign and the available treatments may carry risks, the patients should not be left unmanaged. Symptomatic treatment should be given, such as counseling, sound therapy, and palliative management. Our long-term observation indicated that overall outcomes are positive when the etiologies are identified early and managed properly.
... A lthough tinnitus is a common symptom, with an incidence of 4%-20%, pulsatile tinnitus affects <10% of all tinnitus patients. [1] Unlike permanent tinnitus, pulsatile tinnitus is mainly related to vascular pathologies, which cause a change in blood volume and pressure or a change in vessel lumen. [2] The differential diagnosis of pulsatile tinnitus includes dural arteriovenous fistulas, dural sinus stenosis, glomus tumors of the jugular foramen, or atherosclerotic diseases of the carotid artery. ...
Article
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Fibromuscular dysplasia (FMD) is a known cause of pulsatile tinnitus that can, on rare occasion, evolve into an incapacitating condition. It is a noninflammatory and nonatherosclerotic arteriopathy of unknown cause that affects medium-sized vessels, such as the carotid and renal arteries, occurring mainly in women. We describe a 72-year-old woman suffering from pulsatile tinnitus refractory to medical treatment who was successfully treated with Casper stent in the carotid artery. The different treatment strategies published in the literature were reviewed.
... Sismanis and other authors propose that the management of patients with vascular tinnitus must focus on the underlying cause, involving evaluation and follow-up not only by otolaryngologist (ENTs), but also by physicians from different specialties such as neurologists, neurosurgeons, and ophthalmologists. 16 Thereby, in case a DAVFs or other intracranial vascular malformations are diagnosed based on imaging tests, the patient should be referred for evaluation from a neurosurgical team to consider other diagnostic and management options. Treatment options include embolization, radiosurgery, open surgical intervention, or wait-and-see strategies. ...
Article
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Introduction Tinnitus is characterized as the conscious and involuntary perception of sound, and it affects ∼ 30% of the population. Despite careful physical examination, the etiology of tinnitus can be established for only 30% of patients. Tinnitus is a common symptom of cerebral arteriovenous fistulas and results from increased blood flow through the dural venous sinuses, leading to turbulent arterial flow, mainly related to sigmoid and transverse sinus lesions. Objectives To analyze the frequency of tinnitus, patient profile, and endovascular treatment characteristics in individuals diagnosed with cerebral arteriovenous fistulas. Methods A retrospective and observational study based on reviewed data from medical records on the PHILIPS Tasy system (Philips Healthcare, Cambridge, MA, USA) at the neurosurgery and interventional neuroradiology service of Hospital Santa Isabel in Blumenau–state of Santa Catarina, Brazil. Results The profile of 68 individuals diagnosed with cerebral arteriovenous fistula who underwent endovascular treatment were analyzed. Most patients were female, aged 31 to 60. Tinnitus affected 18 individuals. Dural fistulas were the most prevalent in the sample, and computed tomography alone was the most used diagnostic method for initial investigation. Conclusion The prevalence of this symptom in patients diagnosed with cerebral arteriovenous fistula was found in 26.5% of this sample, mainly in women with associated comorbidities. Tinnitus remission was observed in all patients who underwent endovascular treatment to correct cerebral fistula.
... PT can be caused by vascular disorders including stenosis or tortuosity of the carotid artery, intraluminal webs resulting in turbulent flow, and other vascular abnormalities including aneurysms, dissections, or arteriovenous fistulas, all potentially present in patients with FMD. [10][11][12] Another potential contributor to PT in patients with FMD is the presence of an S-curve, or extreme arterial tortuosity, of the internal carotid artery, resulting in turbulent blood flow and PT. A review of 116 patients with FMD found 32% of patients had an S-curve present in the internal carotid artery. ...
Article
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Background Fibromuscular dysplasia (FMD) is a nonatherosclerotic arterial disease that has a variable presentation including pulsatile tinnitus (PT). The frequency and characteristics of PT in FMD are not well understood. The objective of this study was to evaluate the frequency of PT in FMD and compare characteristics between patients with and without PT. Methods and Results Data were queried from the US Registry for FMD from 2009 to 2020. The primary outcomes were frequency of PT among the FMD population and prevalence of baseline characteristics, signs/symptoms, and vascular bed involvement in patients with and without PT. Of 2613 patients with FMD who were included in the analysis, 972 (37.2%) reported PT. Univariable analysis and multivariable logistic regression were performed to explore factors associated with PT. Compared with those without PT, patients with PT were more likely to have involvement of the extracranial carotid artery (90.0% versus 78.6%; odds ratio, 1.49; P =0.005) and to have higher prevalence of other neurovascular signs/symptoms including headache (82.5% versus 62.7%; odds ratio, 1.82; P <0.001), dizziness (44.9% versus 22.9%; odds ratio, 2.01; P <0.001), and cervical bruit (37.5% versus 15.8%; odds ratio, 2.73; P <0.001) compared with those without PT. Conclusions PT is common among patients with FMD. Patients with FMD who present with PT have higher rates of neurovascular signs/symptoms, cervical bruit, and involvement of the extracranial carotid arteries. The coexistence of the 2 conditions should be recognized, and providers who evaluate patients with PT should be aware of FMD as a potential cause.
... The objectification of venous pulsatile tinnitus (PT), a common form of objective tinnitus characterized by selfperception of the pulse-synchronous extradural sinus flow sound, has recently been increasingly documented [3][4][5][16][17][18][19], of which the trans-external auditory canal and Doppler ultrasound-recording techniques are the commonly implemented methodologies to capture in vivo acoustic characteristics of PT [9,11,[13][14][15]. It has been suggested that analyzing the frequency features of PT has a high potential for differentiating the vascular type [13]. ...
Article
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.
... Tinnitus describes distressing otological symptoms associated with the perception of sound without corresponding acoustic processing in the cochlea [8]. Pulsatile tinnitus accounts for less than 10% of tinnitus [9]. Two possible causes of PT have been postulated: ...
Article
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Introduction: Pulsatile tinnitus (PT) can be very distressing for the patient. An identifiable abnormality is rarely detected. Dural AV malformation is responsible for arterial PT. Venous PT has rarely been attributed to an obvious abnormality on venogram. Dehiscent high jugular bulb or sigmoid sinus have been thought to be potential cause for venous PT. Ligation of internal jugular vein (IJV) has been advocated as a definitive surgical treatment. To our knowledge the use of acellular dermal matrix for treatment of venous PT has not been reported previously. Objectives: To share our experience of a successful treatment of PT using acellular dermis. Methodology: Case report and literature review. Case description: A 23-year-old Caucasian female presented with right-sided PT of 9 months duration. All clinical and audiological investigations were normal. MRI brain and internal auditory canals was normal but the CT scan showed a high right jugular bulb. It also showed dehiscence of the right sigmoid plate with herniation of sigmoid sinus into the mastoid. She underwent transmastoid correction of dehiscent sigmoid sinus and jugular bulb. Acellular dermis was used for extra luminal packing of mastoid cavity and hypotympanum. The patient made a good post-operative recovery and reported resolution of tinnitus on recovering from anaesthesia. The patient was discharged home the following day. There were no sequelae from surgery. The patient has remained symptom-free 11 years following her treatment. Conclusion: The surgical goal of dehiscent sigmoid sinus correction can be accomplished with acellular dermis packing. Traditionally ligation of the IJV or rigid correction of herniated sinus has been recommended; however, we have demonstrated that a relatively thick pliable acellular dermis is more than adequate to correct herniation of the sigmoid venous sinus.
... Headache (especially migraine), pulsatile tinnitus, dizziness, or light-headedness are non-specific symptoms that may be the consequence of cerebrovascular FMD. However, patients can be asymptomatic or may present with focal neurological deficits, such as transient ischemic attack or stroke [3,4,9,[52][53][54][55][56]. In case of cervical artery dissection (ceAD), these focal neurological symptoms may be accompanied by neck, face, or head pain [6]. ...
Article
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Fibromuscular dysplasia (FMD) is a non-atherosclerotic vascular disease that may involve medium-sized muscular arteries throughout the body. The pathogenesis of FMD remains poorly understood, but a combination of genetic and environmental factors may be involved. The majority of FMD patients are women, but men may have a more progressive disease, especially when smoking. Besides the classical phenotype of string of beads or focal stenosis, arterial aneurysms, dissections, and tortuosity are frequent manifestations of the disease. However, the differential diagnosis of FMD is extensive and includes imaging artefacts as well as other arterial diseases. Diagnosis is based on CT-, MR-, or conventional catheter-based angiography during work-up of clinical manifestations, but clinically silent lesions may be found incidentally. Arterial hypertension and neurological symptoms are the most frequent clinical presentations, as renal and cerebrovascular arteries are the most commonly involved. However, involvement of most arteries throughout the body has been reported, resulting in a variety of clinical symptoms. The management of FMD depends on the vascular phenotype as well on the clinical picture. Ongoing FMD-related research will elaborate in depth the current progress in improved understandings of the disease's clinical manifestations, epidemiology, natural history and pathogenesis. This review is focused on the clinical management of adult FMD in daily practice.
... Although transmastoid SS resurfacing/reshaping demonstrates favorable outcomes in most studies, not all patients with PT and SS-Div/SS-Deh experience complete resolution of symptoms after this surgery 23,25 . In addition, a discrepancy between the radiological findings and symptoms has been reportedly observed in nearly half of the patients 29 , necessitating objective tests in order to identify whether vascular pathologies on radiological modalities are causative lesions for the generation of PT, and to verify if surgical interventions really improve the symptom. For example, Subject 1 suffered from rightsided PT, and was diagnosed with SS-Div/SS-Deh and HJBD simultaneously. ...
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A dominant sigmoid sinus with either diverticulum or dehiscence (SS-Div/SS-Deh) is a common cause of pulsatile tinnitus (PT). For PT originating from SS-Div/SS-Deh, an etiology-specific and secure reconstruction using firm materials is vital for optimal outcomes. As a follow-up to our previous reports on transmastoid SS resurfacing or reshaping for SS-Div/SS-Deh, this study aimed to evaluate the long-term results of transmastoid resurfacing/reshaping. We retrospectively reviewed 20 PT patients who were diagnosed with SS-Div/SS-Deh, underwent transmastoid resurfacing/reshaping, and were followed up for more than 1 year postoperatively. For PT, immediate and long-term changes (> 1 year) in loudness and annoyance were analyzed using the visual analog scale (VAS). Additionally, pre and postoperative objective measurements of PT using transcanal sound recording and spectro-temporal analysis (TSR-STA), imaging results, and audiological findings were comprehensively analyzed. Significant improvements in PT were sustained or enhanced for > 1 year (median follow-up period: 37 months, range: 12-54 months). On TSR-STA, both peak and root mean square amplitudes decreased after surgery. Also, the average pure-tone threshold at 250 Hz improved after surgery. Thus, our long-term follow-up data confirmed that the surgical management of PT originating from SS-Div/SS-Deh is successful with regard to both objective and subjective measures.
... Turbulent blood flow through vessels in the head and neck can be transmitted to the inner ear through surrounding bony and soft-tissue structures, contributing to the perception of pulsatile tinnitus [9]. In up to 30% of cases, no cause is identified. ...
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Introduction: Pulsatile tinnitus is a relatively common presentation in otolaryngology clinics, most cases of which have a treatable cause. This presentation warrants a thorough workup to identify treatable, and rule out life-threatening, etiologies. We present a case of a patient with pulsatile tinnitus arising from multiple dilated venous channels in the head and neck. Case Presentation. We present the case of a 65-year-old Caucasian female with a two-year history of progressive, bilateral pulsatile tinnitus, which had become debilitating. Computed-tomographic angiography (CTA) studies ruled out an intracranial vascular cause for her symptoms. However, computed tomography (CT) scanning and magnetic resonance imaging (MRI) revealed multiple dilated bilateral, low-flow, venous channels throughout the head and neck. The proximity of such dilated venous channels to the temporal bone provides a route for sound to be transmitted to the inner ear. Conclusion: Arterial, venous, and systemic etiologies can cause pulsatile tinnitus. Arteriovenous malformations (AVMs) of the head and neck represent less than 1% of cases. In our patient, dilated low-flow venous malformations are the likely source of her symptoms, which is the first reported case in the literature.
Chapter
Hearing loss is the symptom most commonly seen in association with tinnitus. According to the various models of tinnitus development, hearing loss also represents the crucial trigger mechanism for the development of tinnitus. Since different structures such as the outer ear, the middle ear and the inner ear are involved in the peripheral hearing process, a distinction is also made between conductive hearing loss, sensorineural hearing loss and retrocochlear hearing loss, depending on the localization of the pathology. This chapter highlights the main causes of conductive hearing loss and sensorineural hearing loss. A detailed clarification of the causes of hearing loss in tinnitus is important, as in many cases this also leads to specific therapies, which ideally will result in an improvement of hearing loss and tinnitus symptoms. In the case of middle ear hearing loss, these therapeutic approaches are primarily surgical in nature (tympanoplasty, stapes surgery), in the case of sensorineural hearing loss, the improvement is generally based on a prosthetic fitting with hearing aids or cochlear implants.
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This chapter discusses cerebrovascular diseases as the cause of tinnitus as well as the underlying mechanisms, the diagnostic approach, and the treatment options, when possible.
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This chapter describes the tasks of the otolaryngologist when examining a patient with tinnitus. This is of great importance because the otolaryngologist is often the first point of contact for the patient. The detailed history and also the physical examination can provide the first clues to possible causes of tinnitus. Subsequently, the combination with the audiological examination can provide the basis for successful counseling. The otolaryngological examination is of particular importance in cases of objective tinnitus. Here, objectifiable findings can be obtained in otomicroscopy, such as pulsating movements of the tympanic membrane or middle-ear masses shining through the membrane. Last but not least, conditions such as chronic otitis media or otosclerosis, which in many cases are accompanied by tinnitus, can also be detected.
Chapter
This chapter describes the imaging techniques used in neuroradiology to investigate patients with tinnitus in more detail. A radiological examination is not part of a routine evaluation, but is reserved for certain conditions. These may include cases of objective tinnitus, especially with pulse-synchronous sounds, in which vascular pathology is suspected. Another important point is the assessment of the middle ear and the cochlear and retrocochlear structures in cases of asymmetric hearing loss and tinnitus on the worse side. Mainly, techniques of computed tomography (CT) and magnetic resonance imaging (MRI) and MR angiography (MRA) are used. In addition to finding the underlying cause of the tinnitus, neuroradiological examination can then also point the way to adequate therapy. In some cases of pulsatile tinnitus, therapy of the tinnitus is possible with invasive neuroradiological techniques in the context of digital subtraction angiography (e.g. coiling, stenting and embolization).
Article
Head and neck trauma-induced pulsatile tinnitus (PT) should be approached with caution, as it can rarely be attributed to an arteriovenous fistula (AVF). We present a 26-year-old male with a history of blunt trauma who presented delayed PT with direct AVF between the ascending pharyngeal artery (APA) and the internal jugular vein (IJV). The patient underwent occlusion of the fistula with transarterial embolization using coils and PT was completely resolved, confirming successful treatment. The delayed manifestation of PT in the APA-IJV fistula is probably due to the gradual formation of a pseudoaneurysm and subsequent AVF. This case highlights the importance of investigating PT in head trauma patients, as it can be a sign of AVF and possible complications. Overall, this case contributes to understanding delayed PT with AVF and emphasizes the importance of prompt diagnosis and treatment of AVF in patients with head and neck trauma.
Article
OBJECTIVE Patients with pulsatile tinnitus (PT) are often referred for digital subtraction angiography (DSA) to exclude cranial dural arteriovenous fistula (DAVF). Because DSA is not without risk, the authors studied the sensitivity and specificity of an automated detection algorithm that analyses external ear canal sound measurements to evaluate the presence of DAVF in PT patients. METHODS Between 2015 and 2021, external ear canal sound measurements of 72 PT patients were collected prospectively at our tertiary tinnitus clinic preceding referral of these patients to the neurosurgical department for counseling about DSA. The measurements were analyzed with an algorithm that objectifies a pulsatile sound as a PT coherence index (PTCI) per frequency band. RESULTS Ultimately, DSA was performed in 49 PT patients. DAVF was revealed in 15 patients (31%). PTCI cutoff values of 0.7 (at 250 Hz) and 0.6 (at 1000 Hz) resulted in sensitivity of 100% (78%–100%) and specificities of 62% (44%–78%) and 68% (49%–83%), respectively, for detecting DAVF on DSA. CONCLUSIONS Analysis of external ear canal sound measurements in PT patients showed 100% sensitivity for detecting DAVF on DSA. This analysis can potentially be used as a screening tool to help clinicians and PT patients to decide on the necessity of DSA. Retrospectively, the use of sound measurement in our cohort would have narrowed the indication for DSA to 23 of 49 cases (47%) without missing a DAVF.
Article
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Background Pericarditis is a common pericardial disorder that is frequently accompanied by a pericardial friction rub, which can be detected during a physical examination. Although patients’ awareness of cardiac murmurs and vascular bruits has been extensively reported, there are no reports on patients’ self-awareness of a pericardial friction rub. Case Summary We present the first case of a patient with acute pericarditis associated with objective self-awareness of a pericardial friction rub, which we recorded with an electronic stethoscope and confirmed the sound with the patient. The patient had a recent history of 3-vessel coronary artery bypass grafting and presented with a progressively worsening, rhythmic, and "sandpaper-scratching" sound in both ears. The sound was more pronounced in the left lateral decubitus position. The symptom resolved with colchicine therapy and was associated with concomitant resolution of the pericardial friction rub. Discussion This is the first documented case of a patient demonstrating objective self-awareness of a pericardial rub resulting from acute pericarditis associated with post-pericardiotomy syndrome. Tinnitus refers to the perception of an auditory sensation that can be subjective or objective, depending on whether it is heard only by the individual or can also be heard by an observer. While objective tinnitus caused by cardiovascular conditions has been previously reported, no cases have attributed pericardial friction rub as the underlying cause. Therefore, we suggest using the term pericardial rub tinnitus to describe this unique phenomenon.
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Objective The objective of this study is to assess diagnostic yield of imaging modalities used to evaluate patients presenting with pulsatile tinnitus (PT). Databases Reviewed PubMed, Embase, and Scopus were queried using the search terms “pulsatile tinnitus,” “pulse-synchronous tinnitus,” and “pulse synchronous tinnitus” with no date limitations. Methods Studies that reported diagnostic imaging for patients presenting with PT were included. Data were reviewed for sample size, gender, age, imaging study, indications, and diagnoses. The primary outcome measure from aggregated data was the yield of positive diagnoses made with each imaging modality. The quality of evidence was assessed for risk of bias. Results From an initial search of 1145 articles, 17 manuscripts met inclusion criteria, of which 12 studies evaluated individual imaging modalities. The number of unique patients included was 1232. The diagnostic yield varied between modalities: carotid ultrasound (21%, 95% confidence interval [CI]: 12%–35%), CT temporal bone (65%, CI: 20%–93%), computed tomographic angiography (86%, CI: 80%–90%), and MRI/magnetic resonance angiography (58%, CI: 43%–72%). Conclusion Studies on the diagnostic approach to PT are limited by heterogeneity in both inclusion criteria and reporting standards. A wide range of imaging modalities are used in practice during the initial evaluation of PT, and the diagnostic yield for imaging can be improved by utilizing more specific clinical indications.
Article
Objective: Many but not all patients with idiopathic intracranial hypertension (IIH) have pulsatile tinnitus (PT). However, little is known about why some patients with IIH develop PT and others do not. The purpose of this study was to determine if any of the classic magnetic resonance imaging (MRI)-detectable markers of IIH differ between patients with and without PT, thereby shedding light on potential pathophysiology. Methods: A retrospective age-matched cohort study of patients with documented IIH (diagnosed by neuro-ophthalmologist) was performed. All patients had MRI performed around the time of diagnosis. MRIs were assessed for 16 variables known to be associated with IIH (e.g., pituitary displacement/empty sella, optic nerve tortuosity, transverse sinus stenosis, inferior cerebellar tonsils, arachnoid granulations, slit-like ventricles) by two blinded neuroradiologists. All binary variables were analyzed via χ2 test with Yates correction, or Fisher exact when appropriate. Continuous variables were analyzed via Student t test. Inter-rater reliability for binary variables was assessed by Cohen κ. For continuous variables, intraclass correlation coefficient was calculated. Results: Forty age-matched patients with IIH met the inclusion criteria (20 with PT, 20 without PT). For all known binary MRI findings associated with IIH, there were no statistically significant differences between groups. Likewise, there were no statistically significant differences for continuous variables. Conclusions: The classic MRI findings associated with IIH do not differ between patients with and without PT, suggesting that systemic (rather than localized intrinsic or extrinsic) factors may play a critical role in the pathophysiology.
Article
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Background In the 21st century, the prevalence of tinnitus is increasing, impacting approximately one in five people. It is a very complicated condition that significantly affects quality of life. Despite the availability of hundreds of tinnitus treatment options, none are very successful. In light of this, there has been a steady increase in studies on tinnitus treatments in the recent past. To comprehend them better, this study used bibliometric approaches to analyze and summarize 21st century scientific research accomplishments in tinnitus treatment. Methods The Web of Science Core Collection (WoSCC) was searched for papers that had been published and related to the treatment of tinnitus. VOSviewer, CiteSpace, R, and Tableau software programs were used to conduct bibliometric studies. To evaluate and visualize the results. Results 2,933 publications on tinnitus treatment were found in 74 countries. Between 2000 and 2021, publications increased steadily. Otolaryngology-Head & Neck Surgery had the highest impact factor, whereas Otology & Neurotology had the most magazines and the highest h, g, and m index. Langguth B was the most prolific author in terms of productivity during the past 21 years. Numerous eminent authors and organizations from multiple nations collaborated. With 626 papers, the United States of America (USA) contributed the most to this field, making them the leading contributor. Neuroplasticity, sound therapy, and cognitive behavioral therapy (CBT) have attracted the attention of researchers, leading to the development of innovative diagnostic and treatment strategies for tinnitus. Conclusion This bibliometric study provides a comprehensive analysis of worldwide publications, cooperation, and research hotspots in tinnitus therapy, revealing the present status of research on this issue and guiding tinnitus treatment research in the coming years.
Article
Objective: To describe the demographic, clinical, and radiologic findings in a consecutive series of patients presenting with a chief complaint of pulsatile tinnitus (PT). Study design: Retrospective review of 157 patients undergoing a combined arterial/venous phase computed tomographic (CT) imaging study. Setting: Tertiary referral center. Patients: Adult patients referred to neurotology faculty for evaluation of PT between 2016 and 2020. Interventions: Triple phase high-resolution arteriography/venography/temporal bone CT. Main outcome measures: Prevalence of osseous, venous, and/or arterial pathology, clinicodemographic characteristics. Results: One hundred fifty-seven adults (mean age, 52 years; 79.6% female) were evaluated. A history of migraine headaches was common (19.7%). The average body mass index was 30.0 (standard deviation, 6.8), and 17.2% of subjects had a diagnosis of obstructive sleep apnea. Idiopathic intracranial hypertension was diagnosed by elevated opening pressure on lumbar puncture in 13.4%. Comorbid depression and anxiety were common (25.5% and 26.1%, respectively). Overall, abnormalities were found in 79.0% of scans, with bilateral transverse sinus stenosis (TSS) seen in 38.9% and unilateral TSS found in 20.4%. Fifteen subjects (9.6%) had evidence of osseous etiologies, including superior canal dehiscence or thinning in 8.9% and sigmoid sinus dehiscence in one subject. There were 3 dural arteriovenous fistulae identified. Unilateral PT was ipsilateral to the side of TSS in 84.4% of subjects with unilateral TSS. Conclusion: In a large consecutive series of patients with PT referred for CT venography/arteriography, transverse sinus stenosis was the most common finding at 59%. Venous etiologies for PT should be suspected when patients are referred to neurotologists for evaluation.
Chapter
Biomechanics plays a key role in occurrence, prevention and rehabilitation of the landing injuries. Some factors can affect the biomechanical performance of landing. To evaluate the effect of various factors, we measured kinematic, kinetic and EMG properties of 16 subjects while they land with changed conditions. We found that landing on level ground with two legs was stable, and the body would be injured before dynamic postural stability was impaired. Compared with the dominant lower limb, the non-dominant limb has a more effective protective mechanism in that the ankle motion is restrained by higher flexor activities. Women are prone to transform the landing energy to the joint motion, whereas men are more likely to transform it to friction. The semi-rigid stabilizer was helpful for men in increasing shank muscle activities. For women, high stabilizer rigidity had little influence on the muscle activities, and it could contribute to larger injury risk. Terrain stiffness did not appear to influence ankle biomechanics.
Chapter
Pulsatile tinnitus (PT), a common otology symptom, is synchronous with patient’s heartbeat. PT is generally objective, induced by cranial vascular sound, and its mechanism is a biomechanical problem. Multiple etiologies and treatments were proposed in clinical studies, but without quantitative analysis in view of biomechanics. In this chapter, both numerical and experimental models were developed for quantification of generation, propagation and reception of PT sound in view of biomechanics. Based on these models, two commonly reported etiologies were analyzed, including sigmoid sinus cortical plate dehiscence/thinness and hyperpneumatization of temporal bone air cells. It is indicated in results that dehiscence of sigmoid sinus cortical plate would directly induce PT, while thinness would not. Normal pneumatization temporal bone cavity would amplify PT sound mostly, but the pneumatization grade would not be the direct etiology of PT. Based on the findings, we propose that PT patients with sigmoid sinus cortical plate dehiscence undergo plate resurfacing surgery, while the patients with thinness should not receive resurfacing surgery. For patients with only hyperpneumatization shown in radiology data, other etiologies should be searched and considered.
Article
Objective: To determine the relationship, if any, between dural venous sinus arachnoid granulations (AGs) and pulsatile tinnitus. Study design: Retrospective case-control study. Methods: Between October 1999 and March 2020, magnetic resonance imaging of patients with tinnitus (pulsatile [PT] and nonpulsatile [NPT]) were assessed for the presence of dural venous sinuses AG. During the same interval, patients with AGs found incidentally on all magnetic resonance imagings ordered without an indication of tinnitus were reviewed. Demographic variables recorded included patient age, sex, race, body mass index, and a history of idiopathic intracranial hypertension (IIH) or obstructive sleep apnea. Location of AGs, when present, were recorded. Results: A total of 651 (PT 250, NPT 401) were found to have AGs. AGs had a higher prevalence in PT patients (10.4% [n = 26]) versus NPT patients (0.3% [n = 1]; odds ratio, 31.0; confidence interval 4.1-234; p < 0.001). Of the 77,607 patients who had an indication for imaging other than tinnitus, 230 patients (0.30%) were found to have incidental AGs, suggesting that the NPT cohort was an adequate control. Patients with PT were more likely to have a higher body mass index, be female, be non-White, and have an existing diagnosis of IIH. For all patients with AGs, AGs were more likely to be found in the lateral sinuses (i.e., sigmoid, transverse) in the PT group (odds ratio, 8.1; confidence interval, 1.1-61.1; p = 0.0218). Conclusions: This study evaluates the association between AG and PT, finding higher rates of AG in patients with PT than in NPT. However, despite the increased prevalence of AG in patients with IIH, these data combined with existing literature would suggest that AGs are not necessarily the missing link to explain PT pathophysiology in IIH.
Article
Objective To investigate the diagnosis of dynamic volume computed tomography (CT) for pulsatile tinnitus caused by sigmoid sinus diverticulum (SSD) and bone defects. Methods Data obtained by dynamic volume CT from 35 patients with SSD were retrospectively collected. Then the ear morphological parameters, including bone defect, transverse sinus stenosis, position of the jugular bulb, jugular bulb diverticulum, defect of the jugular bulb wall, gross venous sinus thrombosis and SSD, and blood perfusion parameters, including cerebral blood flow (CBF), cerebral blood volume (CBV), and mean transit time (MTT), were evaluated and compared between the tinnitus side and the asymptomatic side of the ear. Results The maximum diameters of the bone defects on the tinnitus side were greater than those on the asymptomatic side (Horizontal 6.36±2.35mm vs. 1.12±0.78mm;Longitudinal 4.87±1.25 vs. 0.88±0.06mm). Dynamic volume CT visually displayed the SSD herniated into the adjacent mastoid via the bone defect. Transverse sinus stenosis, high position of the jugular bulb, jugular bulb diverticulum, defect of the jugular bulb wall, and gross venous sinus thrombosis were present more frequently on the tinnitus side than on the asymptomatic side (P < 0.05). Moreover, CBF, CBV, and MTT were significantly greater on the tinnitus side than on the asymptomatic side (P < 0.05). Conclusion Dynamic volume CT examination is an effective method for the diagnosis of pulsatile tinnitus caused by SSD with bone defects.
Article
Objective: Idiopathic pulsatile tinnitus (IPT) is associated with high patient morbidity although treatment methods remain unsatisfactory. In the present study, the transtemporal sigmoid sinus decompression is used in the treatment of idiopathic pulsatile tinnitus. Study design: Retrospective case study. Setting: Tertiary referral center. Patients: From 2005 to 2020, 287 patients presented with a complaint of pulsatile tinnitus. After exclusion criteria, 25 patients were diagnosed with IPT. Those patients underwent treatment and were included in a retrospective study. Interventions: Following failed conservative therapies, the primary author performed a transtemporal sigmoid sinus decompression surgery on the patients under general anesthesia. Main outcome measures: Long-term resolution of IPT was measured using the Tinnitus Handicap Inventory (THI). Outcome measurements were taken preoperatively, immediately postoperatively, three months postoperatively, and the status of all 25 patients is known at the time of this study. Results: Transtemporal sigmoid sinus decompression was performed on 25 patients (mean age: 51.7 years, 80.0% female). Out of the 25 patients, 23 (92.0%) patients experienced complete resolution of their IPT. Statistically significant differences based on preoperative THI (mean THI: 4.19) were evident immediately after surgery (mean THI: 1.31; p < 0.001), at 3 months postoperatively (mean THI: 1.19; p < 0.001), and over a mean follow-up time of 68.7 months (range, 3-168 months) (mean THI: 1.38; p < 0.001). Out of the two patients considered unsuccessful, Case 21 experienced a partial resolution. No major postoperative complications occurred. Conclusions: Transtemporal sigmoid sinus decompression is a safe and effective surgical procedure demonstrated to give near total resolution in properly selected patients and provides long-term relief for patients with IPT.
Article
There has been a growing interest in the investigation of hydroacoustic characteristics of pulsatile tinnitus (PT). However, a proper technique for computational fluid dynamics (CFD) simulation has yet to be discussed. The primary goal of this paper was to investigate the intrasinus hydroacoustic characteristics of PT at the transverse-sigmoid junction (TSJ) using Doppler ultrasound and examine the validity of CFD techniques in simultaneity. The preoperative and intraoperative Doppler ultrasound were performed on a patient with PT at upper jugular vein and TSJ, respectively. Canonical CFD techniques were applied to solve the computational transverse-sigmoid sinus flow domain and compared with the Doppler’s measurements. In addition, the spectro-temporal analysis was performed for the sonification of PT. PT was associated with the recirculating flows at the TSJ according to ultrasonographic detection. This pathogenic region was characterized by a sudden deceleration of flow velocity and inverse increase of flow static pressure, which large eddy simulation (LES) resulted in the smallest 7.4% velocity difference compared to the measured Doppler data, albeit with little differences compared to other solvers. Therefore, based on this case study, the transient LES approach is an optimal CFD method for the computational simulation of the complex hemodynamics at the TSJ. Further numerical studies with large case series are warrranted.
Article
Objective:To probe the clinical characteristics of diagnosis and therapy of vascular pulsatile tinnitus(PT) associated with sigmoid sinus-mastoid. Methods:Retrospectively analyzed the clinical data of the hospitalized 45 PT patients of an ear surgeon in one hospital between January 2013 to January 2020, and observed the effectiveness with surgery and non-surgery therapy. Surgical procedures include reconstruction the bone wall of sigmoid sinus by transmastoid approach and ligation of mastoid emissary vein. Non-surgery therapy includes anti-anemia therapy and observation. All patients have been followed-up in ENT outpatient. Results: Of 45 cases, female:male was 43:2, the mean age was 42.7 years old. The other PT patients were the subjective tinnitus except two females were the objective tinnitus. Of 40 cases, 38 patients underwent transmastoid approach to reconstructed sigmoid sinus bone wall, including 6 patients with the ligated mastoid emissary vein at the same period.The other 2 cases with the ligated mastoid emissary vein only.Five cases were treated by non-surgery therapies, including 2 cases anti-anemia therapy and 3 cases observation. The longest follow-up period was seven and a half years, the shortest was six months. One case was lost to follow up. The total cure rate was 80.0%(36/45),the surgery cure rate was 82.5%(33/40), the non-surgery cure rate was 60.0% (3/5). Conclusion:The pathophysiologic mechanism of the PT is still complex and unclear until now. However, the following conditions probably play an important role in the etiology: female, common features of anatomy anomalies, hemodynamic variations. It is a key point to confirm the responsible site or the main cause of the PT . Although the surgery is relatively simple, the effect is remarkable and no major postoperative complications,surgery could not be a only choice.
Article
The diverse etiopathogenesis of pulsatile tinnitus (PT) makes it a difficult condition to diagnose and treat. To describe the clinical features, investigations and diagnosis of patients presenting with pulsatile tinnitus (PT). Retrospective chart review in an otology unit of a tertiary care referral centre. All medical records of patients who had a complaint of pulsatile tinnitus during the period 1st January 2014–1st May 2020 were included in the study. Data regarding history, characteristics of tinnitus, examination findings, investigations and diagnosis were collected and analyzed. Sixty-four patients with complaints of PT presented to our clinic during this time period and were included in the study giving a prevalence of 0.09%. Definite diagnosis was made in 62 (96.8%) cases with a detailed history, clinical examination and tailored investigations. Pathologies diagnosed were paraganglioma (25%), superior semicircular canal dehiscence (20.3%), anterior inferior cerebellar artery loop (7.8%), sigmoid sinus wall dehiscence (10.9%), sigmoid sinus diverticulum (6.25%), jugular bulb anomalies (7.8%) and hyperpneumatised petrous apex (3.1%) among others. Rare causes encountered were IgG4 disease, far advanced otosclerosis, vestibular aqueduct dehiscence and idiopathic intracranial hypertension. Pulsatile tinnitus is a rare complaint in the Otology clinic. Almost all cases of PT can be diagnosed correctly and appropriate treatment initiated with a logical approach to investigations.
Article
Purpose of review: This article reviews the causes of tinnitus, hyperacusis, and otalgia, as well as hearing loss relevant for clinicians in the field of neurology. Recent findings: Important causes of unilateral and bilateral tinnitus are discussed, including those that are treatable or caused by serious structural or vascular causes. Concepts of hyperacusis and misophonia are covered, along with various types of neurologic disorders that can lead to pain in the ear. Hearing loss is common but not always purely otologic. Summary: Tinnitus and hearing loss are common symptoms that are sometimes related to a primary neurologic disorder. This review, tailored to neurologists who care for patients who may be referred to or encountered in neurology practice, provides information on hearing disorders, how to recognize when a neurologic process may be involved, and when to refer to otolaryngology or other specialists.
Article
Background Sigmoid sinus dehiscence (SSD) is an important etiology of pulsatile tinnitus (PT) though there is currently no consensus on the prevalence of SSD in non-PT populations. This study establishes a grading system of SSD and analyzes a non-PT cohort for prevalence of SSD. Methods In this retrospective study temporal bone CT scans of 91 patients without PT were analyzed for SSD. The dehiscence was divided into three grades: Grade 1 indicating a micro dehiscence of <3.5 mm with an opening to the mastoid air cells, Grade 2 indicating a major dehiscence of >3.5 mm with an opening to the mastoid air cells, and Grade 3 indicating a sigmoid sinus wall dehiscence opening directly to the underlying tissue. Results In patients without PT, SSD occurred in 34% of the cohort. Of these, 75% were Grade 1 and 25% were Grade 2. The range of dehiscence measurements for Grade 1 dehiscences was 0.9–3.4 mm. The range of dehiscence measurements for Grade 2 was 4–7.5 mm. There were no cases of Grade 3 dehiscence among this cohort. Conclusions SSD occurred in over a third of our non-symptomatic cohort. While all grades of SSD may currently be treated surgically, a large portion of non-PT patients may have these sigmoid sinus anomalies asymptomatically. This grading system allows for the standardization of SSD definition and severity in future studies. Grade 3 dehiscences were completely absent in this cohort of non-PT patients.
Article
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.
Article
Objective: To evaluate the prevalence, surgical management, and audiologic impact of pulsatile tinnitus caused by sigmoid sinus dehiscence. Study design and setting: Retrospective chart review at a tertiary care hospital. Patients: Adults with unilateral pulsatile tinnitus attributable to sigmoid sinus dehiscence who underwent resurfacing between January 2010 and January 2020. Interventions: Transmastoid sigmoid resurfacing. Main outcome measures: Resolution of pulsatile tinnitus; audiologic outcomes; complications; tinnitus etiologies. Results: Nineteen patients (89.4% women) had surgery for suspected sigmoid sinus dehiscence. The mean dehiscence size was 6.1 mm (range, 1-10.7 mm). Eight patients had concurrent sigmoid sinus diverticulum and one patient also had jugular bulb dehiscence. Only two patients (10.5%) had the defect identified by radiology. Low-frequency pure-tone average, measured at frequencies of 250 and 500 Hz, showed a significant median improvement of 8.8 dB following resurfacing (18.8 dB versus 10.0 dB, p = 0.02). The majority of patients had complete resolution of pulsatile tinnitus (16/19, 84.2%). Of those without complete resolution, two patients had partial response and one patient had no improvement. There were no significant complications. Of 41 consecutively tracked patients with a pulsatile tinnitus chief complaint, sigmoid pathology represented 32% of cases. Conclusions: Sigmoid sinus dehiscence represents a common vascular cause of pulsatile tinnitus that, if properly assessed, may be amenable to surgical intervention. Sigmoid sinus resurfacing is safe, does not require decompression, and may improve low-frequency hearing. Radiographic findings of dehiscence are often overlooked without a high index of clinical suspicion. Its relationship with transverse sinus pathology and idiopathic intracranial hypertension remain unclear.
Chapter
Professional musicians are at high risk of developing tinnitus due to their over-exposure to music at both occupational and recreational level. This is a 5-year long prospective case series study performed in the Musicians/Performing Arts Medicine Clinic of the 1st Otorhinolaryngology Department of the National and Kapodistrian University of Athens. A total of 274 professional musicians underwent thorough medical history, history of music exposure, assessment of the impact their hearing status has on their professional life (Musicians Hearing Handicap Index), behavioral (Pure Tone Audiometry, standard and extended high frequency) and objective audiometric tests (TEOAE and DPOAE). Standard pure tone audiometry thresholds were correlated with the presence of tinnitus only at high frequencies. Musicians with tinnitus had a clinical and significant higher MHHI score and the incidence of tinnitus was significantly higher in participants suffering from musculoskeletal disease and those with abnormal PTA. Participants' hours of practice were similar in those with tinnitus and those without. The tinnitus group (and in order of descending effect size) had significantly worse thresholds in high frequency audiometry (≥ 3000 Hz) as well as lower signal to noise ratios in DPOAE at almost all frequencies and in TEOAE at high frequencies (2.8 and 4 kHz). A subgroup analysis of the musicians with normal PTA, showed that those with tinnitus showed elevated thresholds in the extended high frequency. In conclusion, tinnitus occurrence in musicians with normal audiogram is potentially correlated with high frequency hearing loss and impaired otoacoustic emissions and these two examinations should be considered in this group.
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Idiopathic intracranial hypertension (IIH) is a clinical condition characterized by elevated intracranial pressure and absence of clinical, laboratory or radiographic evidence of central nervous system infection, vascular malformation, intracranial space occupying lesion or hydrocephalus. In the last years the raising understanding of pediatric IIH, especially concerning its demographics and epidemiology, has brought up to a redefinition of diagnostic criteria and reevaluation of pathogenesis and treatment. The authors reviewed the records of nineteen consecutive children with newly diagnosed IIH in order to compare demographic characteristics, clinical pictures and ophthalmologic aspects as optic disc evaluation and visual field evaluation, as well as treatment modalities and follow up. Beside obesity and female gender, potential alternative risk factors remains to be investigated, which need a good collaboration between neuro-ophthalmologists and pediatric neurologists.
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Pulsatile tinnitus is a relatively common, potentially incapacitating condition that is often vascular in origin. We present a case of disabling pulsatile tinnitus caused by a transverse-sigmoid sinus aneurysm that was surgically treated with self-tying U-clips (Medtronic, Inc., Memphis, TN). We also review the literature and discuss other described interventions. A 48-year-old woman presented with a 5-year history of progressive pulsatile tinnitus involving the right ear. Her physical examination was consistent with a lesion that was venous in origin. Angiography demonstrated a wide-necked venous aneurysm of the transverse-sigmoid sinus that had eroded the mastoid bone. The patient underwent a retromastoid suboccipital craniectomy to expose the aneurysm and surrounding anatomy. The aneurysm dome was tamponaded and the aneurysm neck was coagulated until the dome had shrunk to a small remnant. The linear defect in the transverse sigmoid junction was then reconstructed with a series of U-clips and covered with Gelfoam hemostatic sponge (Pfizer, Inc., New York, NY). The patient awakened without neurological deficit and with immediate resolution of her tinnitus. A postoperative angiogram demonstrated obliteration of the aneurysm, with minimal stenosis in the region of the repair and good flow through the dominant right transverse-sigmoid junction. This technical case report describes a novel definitive surgical treatment of venous sinus aneurysms. This technique does not necessitate long-term anticoagulation, has a low likelihood of reintervention, and provides immediate resolution of pulsatile tinnitus.
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To compare the characteristics of idiopathic intracranial hypertension (IIH) in men vs women in a multicenter study. Medical records of all consecutive patients with definite IIH seen at three university hospitals were reviewed. Demographics, associated factors, and visual function at presentation and follow-up were collected. Patients were divided into two groups based on sex for statistical comparisons. We included 721 consecutive patients, including 66 men (9%) and 655 women (91%). Men were more likely to have sleep apnea (24% vs 4%, p < 0.001) and were older (37 vs 28 years, p = 0.02). As their first symptom of IIH, men were less likely to report headache (55% vs 75%, p < 0.001) but more likely to report visual disturbances (35% vs 20%, p = 0.005). Men continued to have less headache (79% vs 89%, p = 0.01) at initial neuro-ophthalmologic assessment. Visual acuity and visual fields at presentation and last follow-up were significantly worse among men. The relative risk of severe visual loss for men compared with women was 2.1 (95% CI 1.4-3.3, p = 0.002) for at least one eye and 2.1 (95% CI 1.1-3.7, p = 0.03) for both eyes. Logistic regression supported sex as an independent risk factor for severe visual loss. Men with idiopathic intracranial hypertension (IIH) are twice as likely as women to develop severe visual loss. Men and women have different symptom profiles, which could represent differences in symptom expression or symptom thresholds between the sexes. Men with IIH likely need to be followed more closely regarding visual function because they may not reliably experience or report other symptoms of increased intracranial pressure.
Article
Objective To observe intracranial pressure in women with idiopathic intracranial hypertension who follow a low energy diet. Design Prospective cohort study. Setting Outpatient department and the clinical research facility based at two separate hospitals within the United Kingdom. Participants 25 women with body mass index (BMI) >25, with active (papilloedema and intracranial pressure >25 cm H2O), chronic (over three months) idiopathic intracranial hypertension. Women who had undergone surgery to treat idiopathic intracranial hypertension were excluded. Intervention Stage 1: no new intervention; stage 2: nutritionally complete low energy (calorie) diet (1777 kJ/day (425 kcal/day)); stage 3: follow-up period after the diet. Each stage lasted three months. Main outcome measure The primary outcome was reduction in intracranial pressure after the diet. Secondary measures included score on headache impact test-6, papilloedema (as measured by ultrasonography of the elevation of the optic disc and diameter of the nerve sheath, together with thickness of the peripapillary retina measured by optical coherence tomography), mean deviation of Humphrey visual field, LogMAR visual acuity, and symptoms. Outcome measures were assessed at baseline and three, six, and nine months. Lumbar puncture, to quantify intracranial pressure, was measured at baseline and three and six months. Results All variables remained stable over stage 1. During stage 2, there were significant reductions in weight (mean 15.7 (SD 8.0) kg, P<0.001), intracranial pressure (mean 8.0 (SD 4.2) cm H2O, P<0.001), score on headache impact test (7.6 (SD 10.1), P=0.004), and papilloedema (optic disc elevation (mean 0.15 (SD 0.23) mm, P=0.002), diameter of the nerve sheath (mean 0.7 (SD 0.8) mm, P=0.004), and thickness of the peripapillary retina (mean 25.7 (SD 36.1) µ, P=0.001)). Mean deviation of the Humphrey visual field remained stable, and in only five patients, the LogMAR visual acuity improved by one line. Fewer women reported symptoms including tinnitus, diplopia, and obscurations (10 v 4, P=0.004; 7 v 0, P=0.008; and 4 v 0, P=0.025, respectively). Re-evaluation at three months after the diet showed no significant change in weight (0.21 (SD 6.8) kg), and all outcome measures were maintained. Conclusion Women with idiopathic intracranial hypertension who followed a low energy diet for three months had significantly reduced intracranial pressure compared with pressure measured in the three months before the diet, as well as improved symptoms and reduced papilloedema. These reductions persisted for three months after they stopped the diet.
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Educational objectives: To better understand the various etiologies and pathophysical mechanisms of pulsatile tinnitus and to perform a thorough and cost-effective workup and effectively treat most of these patients.
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: The natural course of fibromuscular dysplasia (FMD) of the internal carotid artery (ICA). a stenosing angiopathy associated with cerebrovascular insufficiency, has not been described. A search of medical records located 16 female patients with angiographically demonstrated FMD of the ICA. The identical twin of 1 patient was included in the registry on the basis of noninvasive studies consistent with FMD of the ICA. The mean age at diagnosis was 58 years. Follow-up examinations were performed an average of 3.8 years after diagnosis (range, 1 to 9 years); the evaluation included clinical, angiographic, and Doppler studies. Fifteen patients showed no evidence of progression of FMD, whereas 2 patients with coincident atherosclerotic disease had suffered strokes. One patient had undergone surgical dilatation of the ICA. 3 had received oral anticoagulants, and 13 had received either aspirin or no specific therapy. In light of the apparently benign clinical course of uncomplicated FMD of the ICA, it is concluded that dilatation is rarely warranted. (Neurosurgery 10:39-43. 1982) Copyright (C) by the Congress of Neurological Surgeons
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Bilateral optic disc swelling requires following a number of steps from discovery to causal diagnosis. First, it is necessary to differentiate between true optic disc swelling and disc elevation without true swelling. Then fundus examination, visual acuity and visual field, fluorescein angiography, and optical coherence tomography are performed in order to differentiate papilledema secondary to increased intracranial pressure from optic disc swelling secondary to optic neuropathy. Even if the most frequent etiology is idiopathic intracranial hypertension, the clinician must check for the absence of any signs or symptoms related to hypertension secondary to a cerebral tumor or to cerebral venous thrombosis. Fortunately, modern imaging techniques have facilitated the differential diagnoses of optic disc swelling, and the combination of magnetic resonance imaging (MRI) and magnetic resonance venography appears to be necessary each time the diagnosis of idiopathic hypertension is suggested.
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Tinnitus is a frequent presenting symptom. Tinnitus that is rhythmic and synchronous with the patient's heartbeat is rare. Pulsatile tinnitus may be the only symptom of life-threatening and treatable diseases. The presence of hearing loss or vertigo focuses the diagnostic evaluation. The cause of pulsatile tinnitus may be found on otoscopic examination. Audiologic assessment and enhanced computed tomography often contribute to the diagnosis. Increased intracranial pressure should be excluded with a fundoscopic examination. Arteriography is required to diagnose life-threatening and treatable lesions in the presence of normal otoscopy, audiologic assessment, and enhanced computed tomography.
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Anatomically, the superior boundary of the jugular bulb lies below the floor of the hypotympanum of the middle ear space. In a rare instance, it can extend upward, elevate the floor of the hypotympanum and present in the middle ear. This elevation is an anomaly well known but rarely witnessed or documented in the literature. This may result in serious hemorrhage at the time of surgery if unrecognized by the surgeon and if it is injured during temporal bone surgery. Two hundred fifty-seven histologically prepared temporal bones were examined microscopically for anomalous positions of the jugular bulb. This survey represents 189 patients. Thirteen temporal bones were found in which the jugular bulb extended into the middle ear space above the inferior rim of the bony annulus. Although this anatomical variation occurs infrequently in the general population, a 6 percent incidence of high placed jugular bulb in the middle ear was found in the temporal bones studied in this series.
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Wir präsentieren einen Fall eines 43 jährigen Patienten mit einseitiger sensorineuraler Schwerhörigkeit und Zufallsbefund einer aberrierende A.carotis interna im linken Mittelohr, welche einen pulsierenden Tinnitus verursacht. Das aberrierende Gefäß war inital bei einer Magnetresonanztomographie (MRT) nicht zu sehen und konnte erst mittels Computertomographie und MR-Angiographie (MRA) nachgewiesen werden. Um den aberrierenden Verlauf einer A.carotis interna zu erkennen, ist häufig neben einer konventionellen MRT eine MRA nötig, um die Diagnose zu sichern und andere Differenzialdiagnosen auszuschließen. We present the case of a 43-year-old patient with sensorineural hearing loss and the finding of an aberrant internal carotid artery in the left tympanic cavity that was causing pulsatile tinnitus. The aberrant vessel was initially invisible on magnetic resonance imaging (MRI) and was confirmed by high-resolution computed tomography and MR angiography (MRA). Recognition of an aberrant course of an internal carotid artery often requires a combination of MRI and MRA to establish the diagnosis and rule out other differential diagnoses.
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Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a disorder of elevated intracranial pressure of unknown cause. Patients present with daily headache, pulse-synchronous tinnitus, transient visual obscurations papilledema with its associated visual loss, and diplopia from sixth nerve paresis. Many disease associations have been alleged, but few besides obesity, hypervitaminosis A and related compounds, steroid withdrawal, and female gender have been proven. Although absorption of cerebrospinal fluid (CSF) occurs through arachnoid granulations and extracranial lymphatics, outflow resistance is increased in IIH; therefore intracranial pressure must increase for CSF to be absorbed. The mainstays of medical treatment are a reduced-sodium weight-reduction program and acetazolamide. If patients fail medical therapy, surgical procedures, most commonly optic never sheath fenestration and CSF shunting, are employed. The main morbidity of IIH is visual loss. This is present in most patients and can usually be reversed if recognized early in the course of the disease and treated.
Article
Pulsatile tinnitus is an uncommon otologic symptom, which may be the presenting complaint of a potentially devastating pathology. Understanding this manifestation as a possible symptom of a significant vascular abnormality is crucial to guide management and treatment. We describe a 38-year-old woman with sudden-onset right-sided pulsatile tinnitus. A right extracranial internal carotid artery (ICA) dissection was diagnosed with MRI/magnetic resonance angiography (MRA) and treated with anticoagulation. Follow-up MRI/MRA demonstrated complete resolution. Two months later, left-sided pulsatile tinnitus evolved. An MRI/MRA of the neck demonstrated left-sided extracranial ICA dissection. She was treated in a similar fashion and a repeat MRI/MRA demonstrated its resolution. Spontaneous extracranial ICA dissection may present with pulsatile tinnitus as the only symptom in 4% to 50% of patients. Subsequent evolution of a contralateral dissection is even more uncommon. Generally, treatment of this phenomenon is conservative utilizing anticoagulation or aspirin; however, surgical intervention may be necessary.
Article
Fibromuscular dysplasia (FMD) is a nonatherosclerotic noninflammatory vascular disease that primarily affects women from age 20 to 60, but may also occur in infants and children, men, and the elderly. It most commonly affects the renal and carotid arteries but has been observed in almost every artery in the body. FMD has been considered rare and thus is often underdiagnosed and poorly understood by many health care providers. There are, however, data to suggest that FMD is much more common than previously thought, perhaps affecting as many as 4% of adult women. When it affects the renal arteries, the most common presentation is hypertension. When it affects the carotid or vertebral arteries, the patient may present with transient ischemic attack or stroke, or dissection. An increasing number of patients are asymptomatic and are only discovered incidentally when imaging is performed for some other reason or by the detection of an asymptomatic bruit. FMD should be considered in the differential diagnosis of a young person with a cervical bruit; a "swishing" sound in the ear(s); transient ischemic attack, stroke, or dissection of an artery; or in individuals aged ≤ 35 years with onset hypertension. Treatment consists of antiplatelet therapy for asymptomatic individuals and percutaneous balloon angioplasty for patients with indications for intervention. Patients with aneurysms should be treated with a covered stent or open surgical repair. Little new information has been published about FMD in the last 40 years. The recently instituted International Registry for Fibromuscular Dysplasia will remedy that situation and provide observational data on a large numbers of patients with FMD.
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Carotid artery stenosis is a major risk factor for stroke, and treatments for this condition to decrease the risk of stroke include medical therapy, carotid endarterectomy (CEA), and, more recently, carotid angioplasty and stenting (CAS). Randomized controlled trials comparing the efficacy of CEA vs medical therapy showed a clear benefit for CEA in patients with symptomatic carotid artery stenosis of greater than 70% and a lesser benefit in patients with 50% to 69% stenosis. Treatments have evolved in the ensuing 20 years, and a new method, CAS, has emerged as a possible alternative to CEA. In early results, CAS proved feasible but did not compare favorably with CEA. Later and larger-scale studies comparing CAS to CEA failed to reach conclusions regarding a clear neurologic outcome advantage of one method over the other. This subject was of sufficient interest that 2 larger-scale randomized controlled trials comparing CAS and CEA, the Carotid Revascularization Endarterectomy vs Stenting Trial and the International Carotid Stenting Study, were undertaken to further explore this issue. This brief review places the new data arising from these studies in the context of prior efforts to address the problem of carotid artery stenosis and explores further opportunities for improvement and patient recommendations in light of these new findings.
Article
Idiopathic intracranial hypertension (IIH), or pseudotumor cerebri, describes a condition of elevated intracranial pressure (ICP) that typically presents in obese women of childbearing age with symptoms and signs of posture-dependent headaches, pulsatile tinnitus, visual changes, and papilledema. Optical coherence tomography (OCT) has begun to be utilized as an adjunctive, quantitative tool in the evaluation of patients with IIH to help distinguish between true optic nerve head edema and pseudopapilledema, and to contribute to our understanding of the consequences of prolonged optic nerve edema. Although few longitudinal studies of patients with IIH have been published to date, it appears that there may be a correlation between retinal nerve fiber layer (RNFL) thickness and visual function. With the new spectral domain OCT, additional parameters of the optic nerve imaging, including volume and height measurements, might provide greater sensitivity of the response to treatment and the long-term visual outcome in patients with IIH.
Article
Opinion statement: Idiopathic intracranial hypertension (IIH) is a disorder of raised intracranial pressure of unknown etiology. For overweight or obese patients with IIH, weight reduction of 5% to 10% of total body weight at diagnosis is a long-term treatment strategy. Though not proven, the initiation of acetazolamide can assist in symptom reduction and resolution. In patients with either fulminant IIH or those on maximal medical management with progressive vision loss, intravenous steroids and acetazolamide can be initiated while surgical options are urgently arranged. Because of its lower complication rate, I prefer to use optic nerve sheath fenestration in settings of precipitous visual decline, but I have used cerebrospinal fluid diversion surgery in settings of vision loss with severe, intractable headache. Often, the choice of surgical intervention is individualized for the patient and the available expertise. In the future, results from the ongoing multicenter, double-blind, placebo-controlled Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) will provide important data regarding the efficacy of acetazolamide and the utility of diet and exercise.
Article
Stroke is a leading cause of mortality and long-term morbidity. As a means for stroke prevention, an estimated 99,000 carotid endarterectomy procedures were performed in the USA in 2006. Traditionally, the degree of luminal stenosis has been used as a marker of the stage of atherosclerosis and as an indication for surgical intervention. However, prospective clinical trials have shown that the majority of patients with a history of recent transient ischemic attack or stroke have mild-to-moderate carotid stenosis. Using stenosis criteria, many of these symptomatic individuals would be considered to have early-stage carotid atherosclerosis. It is evident that improved criteria are needed for identifying the high-risk carotid plaque across a range of stenoses. Histological studies have led to the hypothesis that plaques with larger lipid-rich necrotic cores, thin fibrous cap rupture, intraplaque hemorrhage, plaque neovasculature and vessel wall inflammation are characteristics of the high-risk, 'vulnerable plaque'. Despite the widespread consensus on the importance of these plaque features, testing the vulnerable plaque hypothesis in prospective clinical studies has been hindered by the lack of reliable imaging tools for in vivo plaque characterization. MRI has been shown to accurately identify key carotid plaque features, including the fibrous cap, lipid-rich necrotic core, intraplaque hemorrhage, neovasculature and vascular wall inflammation. Thus, MRI is a histologically validated technique that will permit prospective testing of the vulnerable plaque hypothesis. This article will provide a summary of the histological validation of carotid MRI, and highlight its application in prospective clinical studies aimed at early identification of the high-risk atherosclerotic carotid plaque.
Article
Idiopathic intracranial hypertension (IIH) is a disorder of raised intracranial pressure (ICP) in the absence of identifiable pathology. The purpose of this study was to evaluate the clinical presentation and monitor a 3-month course using frequent optical coherence tomography (OCT) evaluations, visual field testings and lumbar opening pressure measurements. A longitudinal study of 17 patients with newly diagnosed IIH and 20 healthy overweight controls were included in the study. Peripapillary retinal nerve fiber layer thickness (RNFLT) and retinal thickness (RT) measurements (Stratus OCT-3, fast RNFL 3.4 protocol), and Humphrey visual field testing were evaluated at regular intervals. Repeat lumbar puncture was performed at final visit (n = 13). The diagnostic delay was 3 months and initial symptoms were headache (94%), visual blurring (82%) and pulsatile tinnitus (65%). Complete clinical remission was achieved in 65%, partial in 29% and unchanged symptoms in 6%. Total average RNFLT and RT decreased significantly during the follow-up period (p < 0.0001 and p < 0.0001, respectively). Changes in RNFLT and RT correlated with improvements in visual field mean deviation (MD) (RNFLT: p = 0.006; RT: p = 0.03) and pattern standard deviation (PSD) (RNFLT: p = 0.002; RT: p = 0.003). In patients with weight-loss >3.5% of BMI, ICP decreased significantly (p = 0.0003). In patients with weight-loss <3.5% of BMI, changes in ICP were insignificant (p = 0.6). OCT combined with visual field testing may be a valuable objective tool to monitor IIH patients and the short term IIH outcome is positive. Weight-loss is the main predictor of a favorable outcome with respect to CSF pressure.
Article
The cervical course of the internal carotid artery is almost straight in contrast to the intracranial portions which are highly tortuous. The incidence of variations in the cervical course of the internal carotid artery of the population is approximately 10-40 percent. In this case report, a 76-year-old female patient with a pulsatile mass at the posterior oropharyngeal wall and anterior neck was presented. Physical examination revealed a pulsatile anterior neck mass, and a pulsatile mass at the right posterior wall of the oropharynx. Imaging revealed a bilateral tortuous internal carotid artery and segmental left internal carotid arterectomy and distal internal carotid artery - lateral common carotid artery anastamosis were performed with no postoperative complications.
Article
Aneurysms of the petrous portion of the internal carotid artery (ICA) are rare. Their etiology is usually congenital, traumatic, or mycotic. Depending on the size and location of the aneurysm, the direction of its growth, and the specific adjacent structures involved, patients may or may not present with signs and symptoms. When signs and symptoms do manifest, they may include headaches, epistaxis, a vascular retrotympanic mass with hemotympanum and/or otorrhagia, pulsatile tinnitus, hearing loss, vertigo, and Horner syndrome or Raeder paratrigeminal neuralgia. We describe the imaging aspects of the case of a 27-year-old man who presented with a 5-day history of unilateral symptoms secondary to a lesion located in the area of the right foramen lacerum. The lesion proved to be an aneurysm of the petrous portion of the ICA. We discuss the anatomic, imaging, and otologic aspects of ICA aneurysms in this location.
Article
Surgical resection, preoperative embolization, radiation therapy, and stereotactic radiosurgery have been used to treat glomus jugulare tumors (GJT). However, the optimal treatment of these tumors remains unclear. The authors report their data on treatment of GJTs with gamma knife radiosurgery (GKS). Retrospective review and pooled analysis. Fifteen patients (nine female, six male) were treated with GKS at a single tertiary care institution for GJTs over a 14-year period. Criteria for selection included GKS followed by at least one posttreatment radiographic image, and volumetric analysis was performed. A required 15% change in tumor volume was considered real. Pooled analysis was performed to compare outcomes with other series. The mean total radiologic follow-up was 43.2 months. The mean dose-to-the tumor margin was 14.6 Gy. The mean tumor size at treatment was 7.3 cc and 6.3 cc at last follow-up. After treatment, seven tumors decreased (46.7%), five remained unchanged (33.3%), and three (20%) grew on imaging. Treatment failures received a mean marginal dose of 13.2 Gy compared with 15.1 Gy for treatment successes (P =.08). Overall tumor control rate after GKS in the existing literature with inclusion of the present study is 90.5%. GKS is an effective treatment option for patients with GJTs, including those with prior surgical resection. Marginal radiation doses greater than 13 Gy may be optimal for tumor control. Longer follow-up will better define the benefits and risks of stereotactic radiosurgery in treating patients with GJT.
Article
Bilateral optic disc swelling requires following a number of steps from discovery to causal diagnosis. First, it is necessary to differentiate between true optic disc swelling and disc elevation without true swelling. Then fundus examination, visual acuity and visual field, fluorescein angiography, and optical coherence tomography are performed in order to differentiate papilledema secondary to increased intracranial pressure from optic disc swelling secondary to optic neuropathy. Even if the most frequent etiology is idiopathic intracranial hypertension, the clinician must check for the absence of any signs or symptoms related to hypertension secondary to a cerebral tumor or to cerebral venous thrombosis. Fortunately, modern imaging techniques have facilitated the differential diagnoses of optic disc swelling, and the combination of magnetic resonance imaging (MRI) and magnetic resonance venography appears to be necessary each time the diagnosis of idiopathic hypertension is suggested. Copyright (c) 2010 Elsevier Masson SAS. All rights reserved.
Article
We describe the case of a 48-year-old woman who presented with a sigmoid sinus aneurysm. These rare entities have only recently been described in the literature and the ideal treatment approach has not been elucidated. This report represents additional evidence in a growing body of literature that suggests that endovascular therapy is a safe and effective therapeutic alternative to surgical reconstruction of the sigmoid sinus in selected cases of intractable pulsatile tinnitus.
Article
To assess the effectiveness of middle ear floor reconstruction in management of vascular tinnitus due to high jugular bulb with dehiscent middle ear floor. Case series with chart review. Tertiary academic medical center. We reviewed the medical records of seven patients with high dehiscent jugular bulb, presenting with incapacitating pulsatile roaring tinnitus that was abolished by digital compression of the ipsilateral jugular vein, from January 2002 to December 2006. The diagnosis was confirmed by CT scan of the temporal bone (bone window, coronal views). The seven patients were surgically explored, five under local anesthesia (to monitor the results with possible intraoperative revision) and two under general endotracheal anesthesia, for middle ear floor reconstruction that was done using bone dust, perichondrium, and tragal cartilage (mean follow-up 28 months). Of the seven patients, tinnitus disappeared in four (57%) and decreased in one. The overall improvement was five of seven (71%). One patient had postoperative increased intracranial pressure. The preliminary results suggest that surgical reconstruction of the middle ear floor under local anesthesia offers valuable treatment for patients with incapacitating tinnitus due to dehiscent middle ear floor. However, the risk of sigmoid sinus thrombosis should be considered. To our knowledge, this is the first trial of multilayer reconstruction of the middle ear floor dehiscence to manage high jugular bulb causing tinnitus.
Article
Cranial nerve palsies are regularly observed in patients with arteriovenous fistulas of the cavernous sinus. The purpose of our study was to determine the long-term clinical outcome-with a special focus on extra-ocular muscular dysfunctions-in patients who had undergone endovascular treatment of a cavernous sinus fistula with detachable coils. Sixteen patients were recalled for an ophthalmoneurologic control examination (mean interval of 4.4 years). The mRS and the EQ-5D questionnaire were used for the description of general outcome. Age, duration of symptoms, character of the fistula (direct, dural), and coil volume were tested to assess their relevance for persistent symptoms. All patients displayed complete regression of chemosis, exophthalmus, and pulsating tinnitus with no evidence of recurrences. Oculomotor disturbances persisted in 9 of 13 patients and caused permanent diplopia in 7 patients. In 15 patients a mRS score of 1 or 2 was achieved; however, 7 patients reported some limitations in life quality (EQ-5D). A significant correlation was found between coil volume and persistent diplopia (P = .032) and persistent cranial nerve VI paresis (P = .037). Coil embolization of the cavernous sinus led to durable closure of AVF and reliable regression of acute symptoms. However, long-term follow-up showed a 44% rate of persistent cranial nerve deficits with disturbances of oculomotor and visual functions. This may be explained by the underlying fistula size itself and/or the space-occupying effect of the coils. As neuro-ophthalmologic outcome is crucial for control of therapeutic success, patients should be routinely examined by ophthalmologists.
Article
Deformities of the carotid artery are rare. Tortuosity, kinking and coiling of the internal carotid artery may be observed with advancing age. A tortuous internal carotid artery may cause an abnormal sensation in the throat. In the early twentieth century, there were several reported cases of fatal haemorrhage during pharyngeal surgical procedures, because this condition went undetected. We present two cases of tortuosity of the right internal carotid artery. Both women complained of abnormal throat sensations. Endoscopic studies and radiological examinations revealed tortuous right internal carotid arteries presenting as pulsatile masses. A literature review revealed that, in most reported cases, this deformity occurred on the right side. We believe that the defect and its right-sided predominance can be attributed to anatomical influences and factors affecting blood pressure. In most reported cases of tortuous internal carotid artery, the defect occurred on the right side and patients complained of an abnormal sensation in the throat. This information is useful in the diagnosis of this condition. It is important for otolaryngologists to recognise this anomaly, because fatal haemorrhage can occur in patients with this condition during surgical procedures on the pharynx.
Article
To demonstrate the feasibility of treatment and early outcomes for patients treated with gamma knife radiosurgery (GKR), with or without surgical resection, for glomus jugulare tumours. Between January 2007 and November 2008, 10 patients with glomus jugulare tumours were treated with GKR. Eight had prior surgical resection, seven subtotal resection and one total resection. In two cases GKR was the only definitive therapy. Baseline neurological deficits were prospectively recorded and present in 90% prior to GKR. The median tumour size and volume were 4 cc (0.7-10.9 cc). The median marginal tumour dose was 14 Gy (12-16 Gy). Clinical and radiographic outcomes are reported with a median follow-up of 9.7 months. Stereotactic frame placement allowed treatment of all 10 lesions, although 3-point fixation was sometimes required to avoid collisions. No patients developed worsening of symptoms or new neurological complaints after GKR; symptom relief was achieved in 50% of cases. No cases of clinical or radiographic progression were identified. Radiographically, 80% of lesions were stable and 20% showed significant shrinkage. GKR is an excellent option for patients with glomus jugulare tumours after complete or subtotal resection or at recurrence. Appropriately planned frame placement allows successful treatment delivery without difficulty. GKR improved symptoms, prevented neurological progression and achieved radiographic stability or regression in all cases.
Article
Internal carotid artery dissections (ICADs) with occlusion present with a high morbidity and mortality. No specific medical treatment has proven to be effective in this setting. In selected cases of ICAD with occlusion, stent-assisted angioplasty has been shown to be effective in restoring the perfusion. Spontaneous ICAD causing occlusion successfully recanalized with multiple telescoped stents extending intracranially has only been reported exceptionally. We report cases of symptomatic acute carotid occlusion after spontaneous dissection extending from the cervical to the petrocavernous ICA segments. Imaging studies revealed the presence of an extensive penumbra area in every case. Patients were treated by means of multiple stents deployed in a telescoped fashion with the aid of a delayed double-contrast road map. Post-procedural angiography demonstrated restitution of the carotid lumen with no signs of residual dissection or intracranial emboli. The patients improved rapidly, showing no residual neurological deficit after a week. At follow-up, patients are clinically asymptomatic and the vessel is patent with no radiological signs of myointimal hyperplasia. The successful angiographic and clinical results observed in our cases of extraintracranial stenting of a long carotid dissection causing occlusion contribute to the literature of carotid dissection treated with multiple stents.
Article
Palatal tremor is a rare neurotological disorder responsible for objective tinnitus in children. Palatal tremor may be symptomatic of an underlying neurological disease or essential when a cause cannot be identified. We report a case of an essential palatal tremor in a 10-year-old girl complaining of clicking tinnitus. No treatment was undergone as she was not obviously bothered by the ear-clicking sound. Different treatment modalities have been used for distressing tinnitus related to palatal myoclonus. Recently several publications reported satisfactory results with botulinum toxin injection, which seems to be the treatment of choice.
Article
It has been postulated that cerebral venous outflow "obstruction" is a precipitating factor for many cases of idiopathic intracranial hypertension (IIH). We describe a 17-year-old woman with IIH, and "venous obstruction" repeatedly demonstrated on magnetic resonance venography (MRV) that within minutes resolved partially when the cerebrospinal fluid (CSF) pressure was reduced to 11 cmH(2)O and completely when the pressure was reduced to 8cmH(2)O. These findings further support the view that raised pressure is the cause of the "obstruction", rather than the obstruction being the primary cause of the IIH. It also raises questions about how low the CSF pressure should be reduced at therapeutic lumbar puncture.
Article
To describe the diagnosis, management, and treatment outcome of jugular foramen (JF) tumors. Retrospective chart review. Charts of the 83 patients diagnosed with JF tumors between January 1997 and May 2008 were reviewed. Presenting symptoms, otologic and neurotologic examination, audiologic thresholds, treatment procedure, surgical technique, tumor size and classification, and postoperative complications were recorded. Facial nerve function was graded using the House-Brackmann scale. Extent of tumor removal was determined at time of surgery, followed by routine radiographic follow-up. The mean age of patients with JF tumors was 48.5 years (standard deviation, 16.3 yr), and women (79.5%) outnumbered men (20.5%). Most had glomus jugulare (GJ) tumors (n = 67, 80.7%); 9 patients had lower cranial nerve schwannomas (10.8%), and 7 patients had meningiomas (8.4%). The most frequent initial symptoms included pulsatile tinnitus (84.3%), conductive hearing loss (75.9%), and hoarseness (34.9%). Sixty-one patients (73.5%) underwent surgery, 18.1% had radiotherapy, and 8.4% were observed. Total tumor removal was achieved in 81% of surgery cases. New lower cranial nerve (CN) deficits occurred after surgery in 18.9% of GJ, 22.2% of schwannoma, and 50% of the 4 meningiomas. At last follow-up, 88.1% of surgical patients had normal or near-normal (House-Brackmann I or II) facial function. Total resection of GJ tumors, meningiomas, and lower CN schwannomas can be a curative treatment. However, subtotal removal may be required to preserve CN function, vital vascular structures, and the brainstem. Postoperative radiotherapy is used to control residual tumor. When postoperative complications develop in patients, early rehabilitation is important to decrease mortality and morbidity. Therefore, patients should be closely followed.
Article
We present the case of a 43-year-old patient with sensorineural hearing loss and the finding of an aberrant internal carotid artery in the left tympanic cavity that was causing pulsatile tinnitus. The aberrant vessel was initially invisible on magnetic resonance imaging (MRI) and was confirmed by high-resolution computed tomography and MR angiography (MRA). Recognition of an aberrant course of an internal carotid artery often requires a combination of MRI and MRA to establish the diagnosis and rule out other differential diagnoses.
Article
Atherosclerotic carotid artery disease (ACAD) is a rare but recognized cause of pulsatile tinnitus. Existing literature of reported cure for pulsatile tinnitus is reviewed. We found: (1) a male preponderance exists; (2) ipsilateral carotid endarterectomy (CEA) for tinnitus is 92% (12 of 13) effective; (3) proximal lesions lend themselves to CEA whereas distal lesions have been treated by stenting; (4) overall 68% (15 of 22) are cured by intervention; and (5) 89% (17 of 19) can expect immediate relief. We now present a case of bilateral pulsatile tinnitus relieved by bilateral carotid endarterectomy.
Article
This article presents a modification to the existing classification scales of intracranial dural arteriovenous fistulas based on newly published research regarding the relationship of clinical symptoms and outcome. The 2 commonly used scales, the Borden-Shucart and Cognard scales, rely entirely on angiographic features for categorization. The most critical anatomical feature is the identification of cortical venous drainage (CVD; Borden-Shucart Types II and III and Cognard Types IIb, IIa + b, III, IV, and V), as this feature identifies lesions at high risk for future hemorrhage or ischemic neurological injury. Yet recent data has emerged indicating that within these high-risk groups, most of the risk for future injury is in the subgroup presenting with intracerebral hemorrhage or nonhemorrhagic neurological deficits. The authors have defined this subgroup as symptomatic CVD. Patients who present incidentally or with symptoms of pulsatile tinnitus or ophthalmological phenomena have a less aggressive clinical course. The authors have defined this subgroup as asymptomatic CVD. Based on recent data the annual rate of intracerebral hemorrhage is 7.4-7.6% for patients with symptomatic CVD compared with 1.4-1.5% for those with asymptomatic CVD. The addition of asymptomatic CVD or symptomatic CVD as modifiers to the Borden-Shucart and Cognard systems improves their accuracy for risk stratification of patients with high-grade dural arteriovenous fistulas.
Article
Jugular bulb diverticulum is a rare diagnosis, as fewer than 50 cases have been reported in the literature. It has been reported that unilateral auditory symptoms may accompany this entity, although some patients are asymptomatic. We present a case series of 3 patients who were referred to our tertiary care neurotology center with a unilateral jugular bulb diverticulum along with unilateral sensorineural hearing loss and tinnitus. These patients were evaluated clinically and radiographically. This case series (1) adds further documentation of the presence of unilateral auditory symptoms in patients with a jugular bulb diverticulum and (2) demonstrates the value of computed tomographic venography in the diagnosis of jugular bulb diverticulum.
Article
This study aimed to evaluate the safety and efficiency of the endovascular treatment of transverse-sigmoid sinus dural arteriovenous fistulas (TS_dAVF). A total of 150 consecutive patients and 348 procedures were evaluated. Pulsatile tinnitus (81%), headache (15%), and intracranial hemorrhage (10%) were the most frequent manifestations of the TS_dAVFs. More than half of the affected sinuses were partially or completely thrombosed. Access-wise treatment was performed transarterial (n = 33), transvenous (n = 21), or a combination thereof (n = 96). A mean of 2.4 procedures per patient was required. Immediate postprocedural occlusion rate after transarterial embolization was 30% only. Transvenous treatment alone resulted in an early occlusion rate of 81%, with delayed complete obliteration of half of the remaining fistulas. After combined transarterial/transvenous treatment, the angiographic cure rate was 54%. At follow-up, 88% of patients with residual shunt after the treatment showed complete occlusion. The cumulative complication rate was 9% (n = 13), with minor adverse events in ten patients (7%) and major complications in three patients (2%). Transvenous coil occlusion of the sinus segment with the adjacent dAVF site, eventually combined with transarterial occlusion of supplying arteries, is a very effective and well-tolerated treatment method. In selected patients, variations of these methods (e.g., sinus stenting, compartmental sinus occlusion) can be useful.
Article
To compare clinical features, visual characteristics, and treatment of idiopathic intracranial hypertension patients with and without papilledema. Idiopathic intracranial hypertension does not often occur without papilledema. This study estimates the prevalence and compares the clinical characteristics of idiopathic intracranial hypertension patients with and without papilledema. We performed a cross-sectional analysis of all idiopathic intracranial hypertension patients diagnosed at the University of Utah Neuro-Ophthalmology Unit between 1990 and 2003. Patient records were reviewed for presence of papilledema and other signs, symptoms, and treatment characteristics. Each patient without papilledema was matched to the patient with papilledema who was closest to his/her age and sex. McNemar's and Wilcoxon-signed rank sum tests were used to compare characteristics between matched pairs. Among all patients (n = 353), the prevalence of those without papilledema was 5.7% (n = 20). Patients without papilledema reported photopsias (20%), and were found to have spontaneous venous pulsations (75%) and non-physiologic visual field constriction (20%) more often than did those with papilledema. Mean opening pressure, although above normal, was lower in patients without papilledema (mean = 309 mm cerebrospinal fluid) compared with those with papilledema (mean = 373 mm cerebrospinal fluid, P = .031). Idiopathic intracranial hypertension patients without papilledema had more frequent diagnostic lumbar punctures than did patients with papilledema. Visual acuities and treatment were similar between groups. The clinical presentation of idiopathic intracranial hypertension without papilledema is only somewhat different from that of idiopathic intracranial hypertension with papilledema. The lower opening pressure in patients without papilledema may explain variations in symptoms and signs between the 2 groups. When there are visual field changes in idiopathic intracranial hypertension without papilledema, non-physiologic visual loss should be considered.
Article
To provide evidence for the endovascular repair of patients with extracranial carotid artery dissection. A comprehensive literature review was performed whereby all studies that reported on the results of endoluminal repair of extracranial carotid artery dissection and provided information about primary technical and clinical success were identified. The Pubmed, Embase, and Medline databases were searched between January 1997 and February 2008 by two independent observers by using combinations of search terms "endovascular repair," "extracranial carotid artery," and "carotid dissection." After studies were selected according to the given criteria, 13 studies were included in our statistical analysis. The number of reported patients was 62, with a total of 63 extracranial carotid artery dissections. The mean patient age was 43.3 years. The mean follow-up period was 15.7 months +/- 8.7. Various causes were responsible for the disease, including a blunt neck injury in 28 patients (45%), spontaneous dissection in 21 (37%), and iatrogenic trauma during invasive radiologic procedure in 17.7% patients. The technical success rate was 100% (63 of 63 procedures). The primary and 1-year patency rate of the stents and/or stent-grafts was 100%. The overall major adverse cardiovascular events rate was 11% (seven strokes). The total follow-up mortality rate was 0%. The current status of the reported cases in the literature regarding the treatment of carotid artery dissection by means of stent placement shows excellent early and 1-year patency rates and a low major adverse cardiovascular event rate. However, further evaluation is necessary to draw robust conclusions.
Article
Internal carotid artery morphologic abnormalities mainly consist of tortuosities and coilings and can present with pulsatile tinnitus (PT). The purpose of this presentation is to report 3 representative cases and propose clinical and radiologic diagnostic criteria. Three patients presenting with PT. Clinical evaluation including auscultation of the ear canal and head and neck. All patients underwent computed tomography angiography of the head and neck. Clinical evaluation, computed tomography angiography of the head and neck. Head bruit or objective tinnitus were detected in 2 patients. Symptoms of cerebral ischemia were absent. All patients were found to have tortuosities of the internal carotid arteries below the cranium base. One patient, in addition to tortuosity, had coiling as well. Morphologic abnormalities of the internal carotid artery may be associated with PT. Proper clinical evaluation coupled with computed tomography angiography of the head and neck can differentiate these abnormalities from other more serious vascular etiologies. Symptoms of cerebral ischemia warrant consultation with a vascular surgeon.
Article
Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a disorder of elevated intracranial pressure of unknown cause. Patients present with daily headache, pulse-synchronous tinnitus, transient visual obscurations, papilledema with its associated visual loss, and diplopia from sixth nerve paresis. Many disease associations have been alleged, but few besides obesity, hypervitaminosis A and related compounds, steroid withdrawal, and female gender have been proven. Although absorption of cerebrospinal fluid (CSF) occurs through arachnoid granulations and extracranial lymphatics, outflow resistance is increased in IIH; therefore, intracranial pressure must increase for CSF to be absorbed. The mainstays of medical treatment are a reduced-sodium weight-reduction program and acetazolamide. If patients fail medical therapy, surgical procedures, most commonly optic nerve sheath fenestration and CSF shunting, are employed. The main morbidity of IIH is visual loss. This is present in most patients and can usually be reversed if recognized early in the course of the disease and treated.
Article
Seventy-five patients with glomus tumors in the head and neck had a 37% incidence of cranial nerve paralysis and a 14.6% incidence of intracranial extension. Jugular foramen syndrome is associated with 50% and hypoglossal nerve involvement with 75% posterior fossa tumor invasion. Horner's syndrome is associated with 50% middle cranial fossa tumor invasion. The incidence of central nervous system (CNS) involvement with cranial NERVE PARALYSIS (NOT INCLUDING VII nerve) is 52%. Otologic findings and VII nerve paralysis did not correlate with tumor resectability, CNS extension, and prognosis.
Article
Few conditions are seen as commonly by the otologist and are more poorly understood than subjective tinnitus. Tinnitus has been reported in as high as 80% of patients seen in an otolaryngology practice. This symptom is especially marked in patients with a hearing problem and can be so severe that it becomes incapacitating. Careful diagnosis and classification of tinnitus is important for understanding of the problem. Identification of the frequency and intensity of masking, using a tinnitus analyzer, is useful in selecting the form of treatment. Analysis of the history, physical findings and the use of special electrocochleography and brain stem evoked response audiometry help to identify the site of lesion, which may be within the cochlea, cochlear nerve, cochlear nucleus, brain stem, midbrain or auditory cortex. Specific disease entities should be identified and treated. Lesions of the end-organ or cochlear nerve can be treated when necessary by translabyrinthine or middle cranial fossa section of the cochlear nerve. Tinnitus from cervical nerve lesions can be treated by rhizotomy. The use of a hearing aid or introduction of a sound with a tinnitus masker has been found to be 82% effective in suppressing tinnitus. Maskers can be combined with a hearing aid in some cases. The pathogenesis of tinnitus is discussed, but the method of action of tinnitus relief by auditory stimulation is still unclear. A thoughtful and complete examination with our new diagnostic tools and the judicious selection of therapy now makes it possible to give relief to the majority of patients suffering with disturbing tinnitus.
Article
An unusual case presenting to the otolaryngologist as pulsatile tinnitus is discussed. Fowler points out that the circulatory response to anemia is increased cardiac output. There is associated tachycardia and increased arterial pulse pressure. Because of this increased flow state and turbulence, systolic bruits, venous hums, and "capillary" pulsations are found. This increased flow state is perceived in the ear as a transmitted pulsatile tinnitus. The successful treatment of this patient's pernicious anemia corrected the hyperdynamic circulatory state, and resulted in disappearance of her tinnitus.
Article
Carotid arteriograms on three patients with unilateral pulsatile tinnitus demonstrated an ipsilateral atypical trigeminal artery extending from the cavernous portion of the internal carotid artery to form the posterior inferior cerebellar artery. Illustrations and a dissection of a human fetus with a similar finding show this artery crossing the cochlear nerve near its insertion in the pons. Evidence is presented suggesting that neurovascular compression of the eighth nerve is the source of pulsatile tinnitus in these patients.