Pulsatile tinnitus (PT) is characterized by the auditory perception of rhythmic, pulse-synchronous sounds. Until recently, little was known about this condition, frequently confused by many practitioners to be an uncommon variant of primary idiopathic tinnitus.1,2 However, PT is a separate and distinct symptom that can be the sole symptom of underlying pathology. The differential diagnosis of conditions that trigger PT is lengthy, and determining an accurate diagnosis is critical to developing an appropriate treatment plan.
Several schemas have been proposed to categorize tinnitus, most commonly either by audibility (subjective vs objective) or origin (vascular vs nonvascular). However, given the advances in understanding of PT, such historical classifications fall short. Many cases of PT cannot be objectively heard by the examiner. Similarly, many nonvascular sources can cause pulse-synchronous tinnitus. The authors prefer a different taxonomy, first divided by pulse-phase rhythmicity (arterial vs venous) (Table 1). This review details the most common treatable neurosurgical entities that can present with pulse-synchronous tinnitus (Table 2).
TABLE 1. -
Classification of Tinnitus
| Primary idiopathic tinnitus
||• Frequently associated with SNHL
||• Unilateral or bilateral
| Pulse synchronous
||• Less common
||• Louder/more bothersome
||• Detectable bruit or thrill
||• Change in position or manual compression generally no effect
||• More common
||• Mild, intermittent, or less bothersome
||• Change in position or manual compression can cause reduction or cessation
SNHL, sensorineural hearing loss.
TABLE 2. -
Classification of Neurosurgically Treatable Conditions Associated With Pulsatile Tinnitus, by Phase
| Cervical carotid stenosis
| Petrous carotid dissection/aneurysm
| Cavernous carotid dissection/aneurysm
| Middle meningeal
| Carotid cavernous
| Super sagittal sinus
| Glomus tumors
| Paget disease
| Superior semicircular canal dehiscence
| Dural venous sinus thrombosis
| Sagittal sinus stenosis
| Transverse/sigmoid stenosis
| Jugular bulb abnormalities
| Emissary vein dilation
| Condylar vein dilation
| Intracranial hypertension
| Chiari malformation
AVF, arteriovenous fistulae; AVM, arteriovenous malformation; FMD, fibromuscular dysplasia; ICAD, ICA dissection.
ARTERIAL (SYSTOLIC) PULSE-SYNCHRONOUS TINNITUS
Arterial PT is pulse-synchronous tinnitus that is noted by the patient or clinician during the systolic phase. These tend to be relatively louder and more bothersome to patients than venous PT. Typically, a bruit or thrill can be appreciated. Auscultation of the neck and cranium can be helpful and should be performed with a stethoscope in a quiet room. In the absence of a bruit, the clinician can feel for the cervical carotid pulse and ask the patient to indicate whether the pulsations they are hearing are “on the beat” (systolic) or “off the beat” (diastolic). Unlike venous PT, changes in neck position or compression of the cervical vasculature does not usually result in a significant change in loudness or character. In general, arterial PT is less common than venous PT; successful management is expected to result in a complete or near-complete resolution of PT.
Cervical Carotid Stenosis
Internal carotid artery (ICA) stenosis due to either hematoma of the cervical artery or atherosclerotic ICA disease (ACAD) is the most common cause of arterial PT.3 When symptomatic, patients may present with the classic triad of partial Horner syndrome, unusual neck pain or headache, and cerebral or retinal ischemia.3,4 However, PT can be the first or only symptom of ACAD. In addition, ipsilateral bruit unaffected by pressure or head rotation may be heard on auscultation in the case of ACAD.3
PT in cervical ICA dissection (ICAD) develops after a spontaneous or traumatic tear in the arterial intima that leads to diverted turbulent arterial blood flow.5 This condition can cause PT in elder patients; however, cervical manipulative therapy or traumatic injuries can cause ICAD and subsequent PT in younger patients.6,7 Patients with ICAD can also present unilateral headaches, posterior cervical pain, cranial nerve palsies, oculosympathetic palsy, and cerebral or retinal ischemia. Objective bruits may be detected on arterial auscultation.7 PT has been reported in 5% to 15% of patients with ICAD but is infrequently the sole presenting symptom.8 ICADs are typically identified on MRI/magnetic resonance angiography (MRA) or computed tomography (CT) angiography, or conventional angiography (Figure 1).9 Many cases of ICAD are self-limiting, with stenosis resolving in approximately 46% to 90% of patients over a 3- to 6-mo interval.10 Invasive options such as angioplasty and stenting are reserved for cases of hemodynamically significant stenosis or expanding lesions with progressive stenosis.9 In the majority of cases of PT secondary to ICAD, PT spontaneously improves together with the arterial injury over the course of 2 to 6 months.6,8
Fibromuscular dysplasia (FMD) is a form of nonatherosclerotic angiopathy that can cause arterial tortuosity, stenosis, aneurysms, and dissections.11 Patients with FMD may present with headache, vertigo, cerebral infarct, or transient ischemic attack.5 PT has been reported in an estimated 27% to 53% of patients with FMD.12,13 FMD can be identified on CT angiography or catheter angiography, with arteries showing a characteristic “string of beads” appearance.14 Limited results are available about the natural history of PT in patients with FMD, although there are reports of patients whose PT resolved after stenting of dissections associated with FMD.15 Typically, asymptomatic patients are successfully treated with antiplatelet therapy alone.5,13 Percutaneous angioplasty, with or without stent placement, can be performed in patients who have recurrent ischemic events while on medical therapy or have expanding pseudoaneurysms.16
Petrous Carotid Stenosis
The petrous segment of the ICA courses through the temporal bone, in close proximity to the cochlea.17 PT can be caused by turbulent flow in this region associated with petrous ICA atherosclerotic disease.18 Atherosclerotic disease in the petrous carotid commonly presents with transient ischemic attack or stroke; however, PT can be the sole presenting symptom of stenosis in this region.19,20 Similar to other arterial-phase PT etiologies, tinnitus caused by ICA stenosis does not improve on digital pressure or head rotation.21 Atherosclerotic disease in the petrous carotid can be managed medically, with antiplatelet medications, or with angioplasty and stenting for patients with severe or symptomatic stenosis.22 Improvement in PT has been reported after successful stenting of the petrous ICA.19,20,23
Petrous Carotid Dissection
Intrapetrous ICAD can be congenital, traumatic, or mycotic and is prone to develop at the cervico–petrous junction, where the more mobile cervical carotid becomes fixed to the skull base.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.18 As in the cervical carotid, treatment of spontaneous and asymptomatic dissections is typically medical management with antiplatelet medications. Endovascular treatment can be given for patients with increasing pseudoaneurysm size or recurrent ischemia, despite medical management.28 Improvement in PT symptoms has been reported after both medical treatment and endovascular treatment of petrous segment dissections.8,29,30
Petrous Carotid Aneurysm
Aneurysms of the petrous segment carotid are rare.29 Presenting symptoms for aneurysms in this region include conductive and sensorineural hearing loss, epistaxis (due to extradural arterial rupture), headache, and PT.31 PT is believed to result from lateral extension of the aneurysm affecting the tympanic cavity.32 Resolution of PT after treatment has been reported.29,31 Because the carotid is encased in bone as it passes through the carotid canal in this region, endovascular treatment with flow diversion or coil embolization is preferred if treatment is required.33 Traumatic pseudoaneurysm of the petrous carotid is very rare and has been documented after middle ear surgery. Both conservative and surgical treatments have demonstrated complete resolution.34,35
Cavernous Carotid Dissection
Intradural intracranial ICADs can occur at the carotid cavern. 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. Intracranial dissections are often associated with subarachnoid hemorrhage due to rupture of the thinner intracranial adventitia. Anticoagulants are contraindicated in cases of subarachnoid hemorrhage because they may worsen the condition; otherwise, anticoagulants have been shown to demonstrate successful resolution of the dissection with infrequent incidents of renewed symptoms. Surgery is suggested only in cases of progressing dissection.38,39
Middle Meningeal Arteriovenous Fistula
Middle meningeal arteriovenous fistulas are a rare entity of an aberrant connection between the middle meningeal artery (MMA) and neighboring veins or dural venous sinuses, either spontaneously or because of trauma.40 MMA fistulas are a known cause of PT, and PT can be the sole presenting symptom.41 In addition to PT, symptoms can include headache, exophthalmos, chemosis, diplopia, and fatal intracranial hemorrhage.42 Regardless of etiology, there is a risk of catastrophic intracranial hemorrhage associated with these fistulae.40 MMA arteriovenous fistulae (AVFs) can be detected with CT angiography or MRA, but the diagnostic workup requires catheter angiography to characterize the arteriovenous connection and flow.40 In cases in which patients presented with PT, immediate and complete resolution of PT has been reported after fistula embolization.41,43
Abnormal communication between the ICA and the cavernous sinus can result in PT if the fistula occurs in the posterior portion of the sinus. This anastomosis can occur spontaneously (typically in elder patients) or after trauma without concomitant venous injury (Figure 2).44,45 Fistulas are classified as direct or indirect, depending on whether the aberrant connection is from the ICA or from ICA or external carotid artery meningeal branches.46,47 Direct fistulas are generally post-traumatic and are believed to present with more severe symptoms; however, PT has been reported with both direct and indirect fistulas. Other ophthalmic symptoms can include proptosis, diplopia, chemosis, and blurred vision.45,48,49 A transarterial approach can be used for embolization of direct fistulas, with transvenous approaches preferred for indirect fistulas.50 Complete resolution of symptoms, including PT, has been reported with both observation and intervention.44,47
Superior Sagittal Sinus Fistula
Not commonly encountered, fistulas of the sagittal sinus present a problematic origin of PT because treatment success is less certain.51 These fistulae can be congenital or acquired after trauma, surgery, or venous sinus thrombosis and can manifest in both progressive and nonprogressive forms.52,53 Embolization and stereotactic radiosurgery have demonstrated moderate success alone and in combination.52-54
AVFs of the Sigmoid/Transverse Sinus
Dural AV fistulas are the second most common cause of arterial pulse-synchronous tinnitus.46,49 PT can be the sole presenting symptom in 13% of patients with sigmoid/transverse fistulas (Figure 3).46 Such dural AVFs constitute approximately 15% of intracranial arteriovenous malformations and are believed to arise spontaneously or after trauma, thrombosis, obstructive neoplasm, surgery, or infection.55,56 Commonly, dural AVFs manifest in the fifth and sixth decade of life.57,58 On the Borden scale, both types I and II have been highly associated with PT.59 Often, PT causing AVFs may be diagnosed by an audible pulsatile bruit in the postauricular region and symptom resolution after manual venous compression.58,60 Improvement or complete resolution of PT has been reported in up to 92% of patients after endovascular treatment of dural AVFs.46
Carotid Body Tumor
Paragangliomas are neuroendocrine neoplasms that originate from paraganglia of the autonomic nervous system, most commonly in the head and neck (Figure 4).61 The carotid body is the most common location for a paraganglioma, and lesions in this area can infrequently present with PT.62,63 Carotid body paragangliomas may be diagnosed in association with a family history because they are believed to have a genetic basis.64,65 Surgical resection, embolization, radiotherapy, and even pharmacotherapy have proven successful in controlling these tumors with no metastasis because of their benign character and slow pattern of progression.62,63,66
Glomus Tumors (Tympanicum/Jugulare)
Paraganglioma tumors presenting in the jugular bulb and middle ear are a common cause for PT.61 In both cases of paragangliomas arising from the jugular bulb and neoplasms along the tympanic segment of Jacobson (IX) and Arnold (X) nerves, PT is the most common presenting symptom.67-69 Both tumors are typically slow-growing, benign, and nonsecreting and are found predominately in women between their fourth and sixth decades. These neoplasms have also been known to generate cranial nerve palsies, sensorineural hearing loss, ear pain, bleeding, and dizziness.67,70,71 Bright red retro-tympanic nests of tumor are frequently seen in the inferior middle ear. Glomus tumors are easily differentiated from the white, smooth appearance of an aberrant ICA and the dark blue hue of a high/dehiscent jugular bulb. However, in larger tumors, it can be difficult to differentiate glomus tympanicum from glomus jugulare clinically, and therefore, MRI and CT can be helpful to better define the origin (Figure 5). Traditionally, surgery and stereotactic radiosurgery are the gold standards for treatment because they have high levels of success and low levels of recurrence or complication.71-74 Studies demonstrate up to 100% resolution of PT after surgical intervention.75,76
Other Causes of Arterial PT
Paget disease is a progressive disorder involving bony remodeling secondary to increased osteoclast bone resorption.77 In the skull, this remodeling can affect the architecture and vasculature of the petrous pyramid, the external canal, the middle ear, and the inner ear capsule, thus causing PT.78 Most patients are affected bilaterally in their sixth decade and often show associated symptoms such as headaches, skull enlargement, bowing of the tibiae, and hearing loss. Treatment is almost always nonsurgical and includes pharmacotherapies such as bisphosphonates, calcitonin, and nonsteroidal anti-inflammatory agents.79
VENOUS (DIASTOLIC) PULSE-SYNCHRONOUS TINNITUS
Venous PT is pulse-synchronous tinnitus that is noted during the diastolic phase. These tend to be relatively quieter and less bothersome to patients than arterial PT. Often the PT is mild and intermittent. Unlike arterial PT, changes in neck position or gentle palpation of the neck can result in temporary reduction or resolution. As with arterial PT, the clinician can feel for the cervical carotid pulse and ask the patient to indicate whether the pulsations they are hearing are “on the beat” (systolic) or “off the beat” (diastolic). Auscultation of the neck and cranium can be helpful and should be performed with a stethoscope in a quiet room. Unlike arterial PT, many etiologies causing venous PT are more benign.
Dural Venous Sinus Thrombosis
Thrombosis of the dural sinuses presents most commonly in women of childbearing age and predominantly affects the transverse and sigmoid sinuses.80 Up to 85% of patients who present with sinus thrombosis have an underlying prothrombotic risk factor.81 Symptoms may include headaches, seizures, cranial nerve palsies, visual impairment, or hemispheric stroke symptoms.81 Patients may present with PT either ipsilateral or contralateral to the occlusion.80 Contralateral PT is believed to result from increased and turbulent venous outflow on the patient side.82 Typically, anticoagulation results in resolution of PT over several months as the thrombus resolves, with endovascular thrombectomy or decompressive surgery indicated in patients with acute presentation of severe disease.80,82,83
Sagittal Sinus Stenosis
Sagittal sinus stenosis (SSS) can also cause PT. SSS has been shown to develop spontaneously and also after transverse-sigmoid sinus stenting procedures and traumatic injuries, particularly with skull fractures overlying the sinus.84 Stenosis of the sinus can cause venous hypertension and increased intracranial pressure.85 Headache and visual obscurations may accompany PT in clinical presentation. In the case of traumatic injury, surgical elevation of the fracture can relieve SSS compression and resolve symptoms.85 In idiopathic cases, sagittal sinus stenting yielded improvement in a majority of patients.86
Transverse and Sigmoid Sinus Dehiscence and Diverticula
PT resulting from transverse sinus stenosis (TSS) is believed to arise from systolic pulsations of the CSF, most commonly identified in patients with intracranial hypertension (IIH).87,88 These pulsations are believed to compress the walls of the dural sinuses; however, whether TSS is a cause or effect of IIH is unknown.89,90 In addition, TSS is associated with sigmoid sinus diverticulum and dehiscence, especially in cases of stenosis of the distal transverse sinus. The turbulent flow from the transverse segment is believed to cause erosion and then weakens the unprotected sinus wall, forming a diverticulum.89 PT in cases of TSS has also been demonstrated in cases without IIH.91 Often TSS ceases on light digital pressure over the ipsilateral internal jugular vein (IJV).92 MRI and MRV are recommended diagnostic pathways to rule out neoplastic lesions and dural sinus thrombosis.93,94 Dural venous sinus stenting has shown high levels of success in TSS both with and without IIH, with low rates of complication and revision.91 Treatment has been found to result in complete resolution of PT in up to 95% of patients.95
Abnormalities of the Jugular Bulb and Internal Jugular Vein
Owing to the close proximity of the jugular bulb to the cochlea, vascular abnormalities causing abnormal flow through the bulb and proximal IJV can cause PT.96 In cases of an anterior-lying sigmoid sinus, an acutely bending IJV has been observed herniating through the dural layer just beyond the jugular foramen, creating a diverticulum of the middle ear.97-99 In rare cases, this diverticulum has been noted to extend to the inner ear.100 However, PT has been detected in cases of dehiscent bulbs both with and without diverticulum.97 PT is the one of the most common presenting symptoms of a high or dehiscent jugular bulb.101 Patients will often have a history of tympanic trauma, chronic otitis media, infection, or lack of ossification. A bluish middle ear mass has been observed in the posterior quadrant of the tympanic membrane and may present with loss of sensorineural hearing loss, loss of membrane mobility, and pulsatile bruits over the temporal bone. To differentiate the middle ear mass created by a dehiscent jugular bulb from a glomus tumor, MRA and diagnostic angiogram are recommended.27,102 Among various interventions, venous sinus stenting, endovascular coiling of jugular bulb diverticula, and middle ear reconstruction are effective treatment options.103,104 Complete resolution of PT generally occurs after successful treatment of the underlying venous irregularity.101,103
Dilated Condylar Vein
The posterior condylar vein joins the superior bulb of the internal jugular with the deep cervical venous system.105 Dilation of the condylar emissary vein alone has been observed to generate PT; however, in other cases, enlarged condylar veins were found in conjunction with IJV narrowing.106,107 Although rare occurrences, the presence of dilated posterior condylar vessels has been diagnosed using digital subtraction angiography, CT, and MRI.108 In addition, manual pressure over the IJV that resolves the tinnitus can also be used as evidence of a venous etiology. Surgical and endovascular treatments and conservative observation have led to successful outcomes with resolution of tinnitus noted after treatment.106
Dilated Mastoid Vein
Similar to the condylar emissary, dilation of the mastoid emissary vein may also lead to PT. These cases have been documented both with and without abnormal mastoid foramina and jugular fossa. Endovascular embolization is the preferred treatment modality, and resolution of tinnitus has been reported after successful treatment.107,109,110
Idiopathic IIH is the most common cause of venous PT and one of the most common causes of PT overall.92,111 IIH is associated with PT; headache; papilledema; visual obscurities; palsies of the fifth, sixth, and seventh nerves; and pseudosensorineural hearing loss.21,112 Approximately 50% of patients with IIH will have PT, typically presenting with unilateral PT.113 The exact pathophysiology connecting IIH and PT remains unknown, but the proposed mechanisms include turbulent flow through venous sinuses that are compressed by increased intracranial pressure (Figure 6).114 In addition, recent studies suggest a correlation between sigmoid sinus wall abnormalities and PT in patients with IIH.115-117 Along with diuretics and carbonic anhydrase inhibitors, bariatric surgery and intracranial venous stenting have been recommended as successful treatment regimens.117-120 Of note, an estimated 25% of patients with IIH will have non-PT, and tinnitus in these patients typically does not improve after treatment.113
Nonidiopathic Causes of IIH
Type I Arnold–Chiari malformation has been reported as a rare cause of PT. Similarly, congenital stenosis of the Sylvian aqueduct has been a documented cause of PT. Stenosis of the sylvian duct is believed to create turbulent blood flow in the dural venous sinuses and an elevated intracranial pressure.121 PT arising from type I Chiari malformations typically presents in patients from 25 to 35 years with symptoms such as occipital or generalized headaches, dizziness, sensory deficits, nystagmus, neck pain, extremity weakness, and vertigo. In addition, sensorineural hearing loss is believed to be a result of the masking effect of PT.121,122 Decompressive surgery exhibited 75% success rates in cases of Chiari and congenital sylvian stenosis derived PT and sensorineural hearing loss.123
Superior Semicircular Canal Dehiscence
PT arising from dehiscence of the superior semicircular canal can be either unilateral or bilateral and is associated with Tullio phenomenon, chronic disequilibrium, and noise and pressure evoked vertigo.124,125 Caution should be exercised to avoid misdiagnosis of other similar diseases such as otosclerosis, Meniere disease, benign paroxysmal positional vertigo, and perilymph fistulae.125,126 Treatment is surgical (plugging and/or resurfacing of the dehiscent superior semicircular canal) and can be performed through a subtemporal craniotomy or transmastoid approach.127 Surgical interventions offer high rates of complete symptom resolution.
PT presents a diagnostic challenge because it can be caused by a wide array of pathologies. Although the majority of underlying etiologies are benign and fall within the purview of the otolaryngologist, PT can be the first and sole presenting symptom for a variety of life-threatening pathologies requiring neurosurgical diagnosis and management. Careful history, examination, and workup are critical to the successful treatment any patient presenting with PT.
This study did not receive any funding or financial support.
The authors have no personal, financial, or institutional interest in any of the drugs, materials, or devices described in this article.
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