Pulsatile tinnitus contemporary assessment and management.pdf

Pulsatile tinnitus: contemporary assessment and management Aristides Sismanisa,b a Department of Otorhinolaryngology – Head and Neck Purpose of review Surgery, University of Athens and bA’ ORL Clinic, Ippokration Hospital, Athens, Greece...

Pulsatile tinnitus: contemporary assessment and management Aristides Sismanisa,b a Department of Otorhinolaryngology – Head and Neck Purpose of review Surgery, University of Athens and bA’ ORL Clinic, Ippokration Hospital, Athens, Greece Pulsatile tinnitus is an uncommon otologic symptom, which often presents a diagnostic and management dilemma to the otolaryngologist. The majority of patients with pulsatile Correspondence to Aristides Sismanis, MD, FACS, Professor of Otorhinolaryngology – Head and Neck tinnitus have a treatable cause. Failure to establish correct diagnosis may have Surgery, University of Athens, Athens, Greece disastrous consequences, because a potentially life-threatening, underlying disorder E-mail: [email protected] may be present. The purpose of this review is to familiarize the otolaryngologist with the Current Opinion in Otolaryngology & Head and most common causes, evaluation, and management of pulsatile tinnitus. Neck Surgery 2011, 19:348–357 Recent findings The pathophysiology, classification, various causes, evaluation, and management of the most common causes of pulsatile tinnitus are presented in this review. Summary 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. Keywords arteriovenous fistulae, arteriovenous malformation, bruit, cause, idiopathic intracranial hypertension, management, pulsatile tinnitus Curr Opin Otolaryngol Head Neck Surg 19:348–357 ß 2011 Wolters Kluwer Health | Lippincott Williams & Wilkins 1068-9508 The following are the most common causes of pulsatile Introduction tinnitus. Pulsatile tinnitus is an uncommon otologic symptom, which often presents a diagnostic and management Arterial causes dilemma to the otolaryngologist. The purpose of this The most common causes of arterial pulsatile tinnitus are review is to familiarize the otolaryngologist with the most as follows. common causes, evaluation, and management of pulsatile tinnitus. Atherosclerotic carotid artery disease Atherosclerotic carotid artery disease (ACAD) is a common cause of pulsatile tinnitus in patients older Pathophysiology and classification than 50 years, and often associated risk factors such Pulsating tinnitus originates from vascular structures as hypertension, angina, hyperlipidemia, diabetes within the cranial cavity, head and neck region, and mellitus, and smoking are present. Pulsatile tinnitus thoracic cavity, and it is transmitted to the cochlea by may be the first manifestation of ACAD, and, therefore, bony or vascular structures. Pulsatile tinnitus arises from the otolaryngologist may be the first to be consulted either increased blood flow or stenosis of a vascular lumen. Pulsatile tinnitus is secondary to bruit(s) produced and can be classified as arterial or venous according to the by turbulent blood flow at stenotic segment(s) of vessel of origin. Pulsatile tinnitus can be further classified the carotid system. In a series of 12 patients with as objective or subjective according to whether it is audible pulsatile tinnitus secondary to ACAD, ipsilateral to both patient and examiner or to the patient only. carotid bruit was present in all of them. Diagnosis can be confirmed in most patients by duplex ultrasound Rarely, objective tinnitus originates from other structures studies. In patients with ACAD at the skull base and is classified as nonvascular. High-pitched tinnitus or the cavernous sinus, duplex ultrasound studies may with a pulsatile component, often bilateral and associated not be revealing and MRI should be considered. with high-frequency sensorineural hearing loss (SNHL), Recently, MRI has been shown to accurately identify should not be confused with arterial pulsatile tinnitus. carotid plaque features, including intraplaque hemor- This type of tinnitus is subjective and is considered to be rhage, neovasculature, and vascular wall inflammation related to the SNHL. [2
]. 1068-9508 ß 2011 Wolters Kluwer Health | Lippincott Williams & Wilkins DOI:10.1097/MOO.0b013e3283493fd8 Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.  Pulsatile tinnitus Sismanis 349 Intracranial vascular abnormalities Key points Intracranial vascular abnormalities are uncommon causes of pulsatile tinnitus; however, a high index of suspicion
Pulsatile tinnitus is an uncommon otologic symp- and proper evaluation are required to avoid misdiagnosis tom, which always deserves thorough investigation. and catastrophic consequences. The majority of these
Most patients have a treatable underlying cause. lesions are dural arteriovenous fistulae (AVFs) and arter-
Detailed history and auscultation of the head and iovenous malformations (AVMs), and pulsatile tinnitus is neck are of utmost importance.
Radiologic evaluation should be individualized the most common manifestation. The transverse and according to the type of pulsatile tinnitus (arterial sigmoid dural sinuses are the most commonly involved, or venous), presence of retrotympanic pathology, followed by the cavernous sinus [3
,4
]. Dural AVFs and audible bruit. comprise approximately 15% of intracranial AVMs, which
Treatment is directed toward the correction of the usually become symptomatic during the fifth or sixth underlying cause. decades of life [5,6]. In contrast to AVMs, AVFs are usually acquired and thought to result from dural sinuses, spontaneous or traumatic thrombosis, obstructing neo- vement is frequently associated with pulsatile tinnitus plasms, surgery, and infection. As the thrombosed seg- [14
], these patients may first consult the otolaryngolo- ments recanalize, ingrowths of dural arteries take place gist. Cases with carotid or vertebral arteries involvement and arterial to sinus anastomoses are formed. Pulsatile may develop dissection and/or aneurysms and present tinnitus in these patients is of the arterial type and is with symptoms of transient ischemic attacks or stroke associated with a loud bruit over the involved dural sinus [15,16]. The typical angiographic finding is that of a (usually the retroauricular area) as well. ‘string of beads’. According to a recent report, the most critical anatomical Tortuous internal carotid artery feature is the presence of cortical venous drainage as a Pulsatile tinnitus secondary to a tortuous internal carotid finding, which identifies lesions at a high risk for future artery is more common in older individuals and is often hemorrhage or ischemic neurological injury [7

]. accompanied by an audible bruit. Rarely this entity may Patients who present with pulsatile tinnitus have a less present as an abnormal sensation in the throat associated aggressive clinical course with an estimated annual rate with a pulsating pharyngeal or cervical mass [17,18]. It is of intracerebral hemorrhage of 1.4–1.5% [7

]. The likely that carotid vessels become tortuous with aging, mortality from hemorrhage of dural AVFs has been and turbulent blood flow and pulsatile tinnitus are pro- reported between 10 and 20%. duced. Atherosclerosis and FMD have been reported in association with tortuous internal carotid artery. In Aneurysms of anterior inferior cerebellar artery and dis- the author’s experience, this is a benign condition and secting aneurysms of the internal carotid and vertebral pulsatile tinnitus usually subsides spontaneously with arteries are less often associated with pulsatile tinnitus time. Symptoms of cerebral ischemia warrant consul- [8,9]. Other manifestations of dissecting aneurysms tation with a vascular surgeon. Diagnosis is made with include pain, transient ischemic attacks, cranial neuro- computed tomography angiography (CTA) or magnetic pathies, Horner’s syndrome, and subarachnoid hemor- resonance angiography (MRA). rhage [5,9–11]. Sudden head rotation, especially when accompanied by extension (i.e. the tennis ‘ace serve’), The arterial causes of pulsatile tinnitus are as follows: can be a precipitating event. Fibromuscular dysplasia (FMD), various arteriopathies such as Marfan syndrome, (1) Dural, skull base, and cervical region AVMs/AVFs and osteogenesis imperfecta are predisposing factors. [3
,4
,21–25]. (2) Atherosclerotic carotid and subclavian artery disease Glomus tumors of the jugular foramen and middle ear [1,26–29]. Subjective pulsatile tinnitus and conductive hearing loss (3) Glomus tumors of jugular foramen and middle ear are the most common manifestations of glomus jugulare [30,31]. and tympanicum tumors. Diagnosis is based upon (4) Tortuous internal carotid artery. otoscopic findings and imaging studies of the temporal (5) Dehiscence of the superior semicircular canal. bone and skull base. (6) FMD of the carotid artery [15,33,34]. (7) Increased cardiac output (anemia, thrombocythe- Fibromuscular dysplasia mia, thyrotoxicosis, and pregnancy) [35,36]. FMD is a nonatherosclerotic, noninflammatory stenosing (8) Extracranial carotid artery dissection [37,38]. vascular disease, which primarily affects women of age (9) Intrapetrous carotid artery dissection and aneurysm 20–60 years. This entity most commonly involves the [39,40]. renal and internal carotid arteries. As carotid artery invol- (10) Brachiocephalic artery stenosis. Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.  350 Otology and neuro-otology (11) External carotid artery stenosis. CSF hypersecretion induced by elevated estrogen levels, (12) Vascular anomalies of the middle ear [43–47]. which are produced in excess by fat tissues in obese (13) Aberrant artery in the stria vascularis. patients, have been reported as pathophysiologic mech- (14) Vascular compression of the eighth nerve [49–51]. anisms as well. (15) Aortic murmurs. (16) Paget’s disease [53–55]. Pulsatile tinnitus in IIH syndrome is believed to result (17) Otosclerosis. from the systolic pulsations of the CSF, which originate (18) Hypertension – antihypertensive agents. mainly from the arteries of the Circle of Willis. These pulsations, which are increased in magnitude in the Venous causes presence of intracranial hypertension, are transmitted The most common causes of venous pulsatile tinnitus are to the exposed medial aspects of the dural venous sinuses as follows. (transverse and sigmoid), compressing their walls syn- chronously with the arterial pulsations [57,78]. The ensu- Idiopathic intracranial hypertension syndrome ing periodic narrowing of the dural venous sinuses lumen In the author’s experience, idiopathic intracranial hyper- converts the laminar blood flow to turbulent and produces tension (IIH) syndrome is one of the most common the pulsatile tinnitus. The low-frequency SNHL causes of venous pulsatile tinnitus, which may be the seen in many of these patients is believed to result from first or only manifestation of this syndrome [57,58]. Other the masking effect of the pulsatile tinnitus. This is associated otologic symptoms may include hearing loss, supported by the fact that light digital compression over dizziness, and aural fullness. For this reason, the the ipsilateral internal jugular vein (IJV) results in cessa- otolaryngologist may be the first to be consulted. The tion of the tinnitus and immediate improvement or exact pathophysiology of this entity remains unclear; it normalization of hearing. Stretching or compression affects obese women of childbearing age in more than of the cochlear nerve and brain stem, caused by the 90% of cases and is associated with increased intracranial intracranial hypertension, can also play a role in the hearing pressure (ICP) without focal signs for neurological dys- loss and dizziness. This is supported by the abnormal function except for occasional fifth, sixth, and seventh auditory-evoked response (ABR) present in one-third of cranial nerve palsies [60,61,62
,63,64]. IIH syndrome may these patients. also present mainly with posture-dependent headaches and visual changes (blurred vision, transient visual MRI and magnetic resonance venography (MRV) are obscurations, retrobulbar pain, and diplopia) because of necessary to rule out a neoplastic lesion and dural sinus papilledema , which prompt patients to seek con- thrombosis. MRI is normal in most patients, although sulation with a neurologist or ophthalmologist. Although an empty sella, small ventricles, and flattening of the papilledema is common in these patients, its absence posterior aspect of the globe are suggestive of this entity does not exclude this entity [66–68]. Optical coherence [56,80]. Anatomic obstruction of the transverse venous tomography (OCT) has recently been used to differen- sinuses has been reported in IIH syndrome. Direct tiate papilledema secondary to increased ICP from optic retrograde cerebral venography with manometry has disc swelling secondary to optic neuropathy [65,69]. been recommended in order to establish diagnosis and Children are affected as well, although less often. treatment with stents [81–83]. In another study, MRV Diagnosis is established by lumbar puncture and docu- identified bilateral dural venous sinus stenosis in 27 of mentation of elevated cerebrospinal fluid (CSF) pressure 29 patients with IIH syndrome and only in four of 59 (>200 mm of water) with normal CSF constituents. controls. It was not clear whether stenosis was a cause or In most patients, IIH syndrome has a benign and effect of the intracranial hypertension, and this has been self-limiting course; however, in 25% of patients it may reported by others [84,85]. become chronic. Although many causes have been alleged for this syn- Idiopathic or essential pulsatile tinnitus drome, few besides obesity, hypervitaminosis A and Idiopathic or essential pulsatile tinnitus and venous hum related compounds, steroid withdrawal, and female sex are terms used interchangeably in the literature to have been proven. It has been reported that describe patients with pulsatile tinnitus of unclear cause increased ICP in obese patients is secondary to associated [86,87]. Diagnosis of this condition should be made only elevated intra-abdominal, pleural, cardiac filling, and after appropriate evaluation and elimination of other cerebral venous pressures [73–75]. This pathophysiolo- disorders. As the majority of studies on idiopathic pulsa- gic mechanism is further supported by an animal study tile tinnitus were reported prior to the introduction of demonstrating increased ICP upon acute elevation of MRI/MRV/MRA and the overall better understanding of intra-abdominal pressure. Increased cerebral blood the various causes of pulsatile tinnitus, it is possible that flow secondary to cerebrovascular resistance changes and some of these patients may have had IIH syndrome or Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.  Pulsatile tinnitus Sismanis 351 other pathologies such as AVFs, AVMs, or abnormalities palate, or pharynx can be present in patients with tensor of the dural venous sinuses. tympani myoclonus. Wide opening of the oral cavity during examination may eliminate the soft palate myo- The venous causes of pulsatile tinnitus are as follows: clonic contractions. Transnasal fiberoptic inspection of the soft palate and pharynx is more revealing in (1) IIH syndrome. these patients. (2) Jugular bulb abnormalities: high location, dehis- cence, and diverticula [88–92,93

,94]. Auscultation of the ear canal, periauricular region, orbits, (3) Transverse-sigmoid sinus stenosis and aneurysms cervical region, and chest is the most important aspect of [95
,96–98]. the examination. This should preferably be performed (4) Abnormal condylar and mastoid emissary veins with a modified electronic stethoscope in an audio [99,100]. booth [110,111]. Should objective pulsatile tinnitus be (5) Increased ICP associated with Arnold–Chiari syn- detected, its rate should be compared with the patient’s drome and stenosis of the sylvian aqueduct. pulse rate and the effect of light digital pressure over the (6) Idiopathic or essential tinnitus [24,86,87,102]. ipsilateral IJV should be checked. Pulsatile tinnitus of venous origin, such as in patients with IIH syndrome, Nonvascular causes decreases or completely subsides with this maneuver Myoclonic contractions of the tensor veli palatini, levator [57,59]. In patients with arterial pulsatile tinnitus, how- veli palatini, salpingopharyngeus, and superior constric- ever, this maneuver is ineffective. The effect of head tor muscles are the most common nonvascular causes, rotation on tinnitus intensity should also be checked. which can result in objective pulsating sounds. These Venous pulsatile tinnitus decreases or completely sub- contractions can range between 10 and 240/min and may sides with head rotation toward the ipsilateral side, be confused with the arterial pulse. probably because of compression of the IJV between the contracting sternocleidomastoid muscle and the This disorder is often seen in young patients, usually transverse process of the atlas. A complete neurologic within the first three decades of life, although it may be examination should be performed. seen in older individuals as well [103,104,105
,106]. Associated neurologic disorders such as brain stem infarc- tions, multiple sclerosis, trauma, and syphilis have also Audiologic and electrophysiologic testing been reported. Involvement of the olivary tracts, Pure tone (air and bone conduction) and speech audio- posterior longitudinal bundle, dentate nucleus, and reti- metry should be performed in all patients. When hearing cular formation has been identified in these patients loss of 20 dB or more is detected in the low frequencies, a [86,107]. repeat audiogram should be obtained while the patient is applying light digital pressure over the ipsilateral IJV. Myoclonic contractions of the stapedial muscle have also This maneuver typically results in improvement or nor- been reported as a cause of pulsatile tinnitus. malization of pure tones in patients with venous pulsatile tinnitus, such as in IIH syndrome, because of elimination Evaluation of the masking effect of the tinnitus. Discrimination The history, otoscopic examination, and auscultation are is typically excellent in these patients. of utmost importance in evaluating patients with pulsatile tinnitus. Older patients with previous history of cerebro- Impedance audiometry can be useful in patients sus- vascular accident, transient ischemic attacks, hyperlipi- pected of tensor tympani myoclonus. demia, hypertension, diabetes mellitus, and smoking are suspicious for ACAD. Obese females associated ABRs may be considered in selected cases only. Abnorm- with hearing loss, headaches, aural fullness, dizziness, alities of ABR, consisting mainly of prolonged interpeak and visual disturbances are highly suspicious for IIH latencies, have been detected in one-third of patients syndrome [57,59]. Sudden onset of pulsatile tinnitus with IIH syndrome, which normalize following successful in association with cervical or facial pain, headache, management. Electronystagmography may be con- and symptoms of cerebral ischemia is compatible with sidered in patients with associated dizziness. extracranial or intrapetrous carotid artery dissection [37,39,109]. Metabolic workup Otoscopy is essential as it may reveal a retrotympanic Metabolic workup is rarely revealing in these patients; lesion such as a high or exposed jugular bulb, aberrant however, complete blood count and thyroid function carotid artery, glomus tumor, and Schwartze’s sign. tests should be obtained in patients with increased Rhythmic movements of the tympanic membrane, soft cardiac output syndrome to exclude anemia and Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.  352 Otology and neuro-otology hyperthyroidism. Serum lipid profile and fasting blood retroauricular bruits, and for prospective surgical candi- sugar should be considered in patients suspicious of dates in order to evaluate the collateral circulation of the ACAD. brain (arterial and venous) in anticipation of possible vessel ligation and/or preoperative tumor embolization. Ultrasound studies Duplex carotid ultrasound (including the subclavian Figure 1 a and b depict algorithms for the evaluation of arteries) and echocardiogram studies should be obtained patients with pulsatile tinnitus. in patients suspected of ACAD and valvular disease. These studies should be performed prior to any radiologic evaluation, as they may be the only tests required to Management establish diagnosis. Management should be directed toward treating the underlying cause. The following describes management of the most common pulsatile tinnitus causes. Radiologic evaluation The radiologic evaluation is individualized according to Patients with IIH syndrome will often present to the the otoscopic findings and pulsatile tinnitus character- otolaryngologist first because of disturbing pulsatile tin- istics (arterial/venous type). nitus. Obese patients should understand the connection between body weight and pulsatile tinnitus. Associated Normal otoscopy comorbidities such as hypertension, diabetes mellitus, Patients with venous pulsatile tinnitus are scheduled for a gastroesophageal reflux, and obstructive sleep apnea are brain MRI/MRV as initial evaluation. Abnormalities of common in these patients and appropriate referral to the jugular bulb/dural venous sinuses and findings pre- other specialists should be advised. Weight reduction sent in IIH syndrome (empty sella, small ventricles, and is the most important aspect of management and will flattening of posterior aspect of globe) can be detected reduce or even eliminate pulsatile tinnitus in the majority with these studies. Other rare congenital central nervous of patients [62
,119
]. Administration of acetazolamide system abnormalities such as Chiari I malformation and (Diamox, Lederle Parenterals, Inc.) is thought to reduce stenosis of the sylvian aqueduct can be detected with CSF production and can be helpful in decreasing tinnitus brain MRI as well. intensity, although it rarely eliminates this symptom. Lumbar–peritoneal shunt should be considered Patients with arterial pulsatile tinnitus are considered for for patients with progressive deterioration of vision, a CTA as initial evaluation. Experience with CTA persistent headaches, and disabling pulsatile tinnitus suggests this study to be satisfactory in evaluating intra- [56,57,59]. In morbidly obese patients, however, this cranial vascular lesions [112–116]. This is a fast imaging procedure is often complicated by the occlusion of the technique and, as the upper neck is included, cervical shunt secondary to increased intraabdominal pressure vascular pathology, such as a carotid body tumor, can be. Weight reduction surgery in morbidly obese detected as well. Tortuous carotid vessels, AVF/AVMs, patients with pulsatile tinnitus is very effective in elim- carotid artery dissections/aneurysms, cervical/intracranial inating this symptom. Thirteen out of 16 patients who ACAD, and FMD can be diagnosed with this study. underwent this procedure experienced complete resol- ution of their pulsatile tinnitus. Evaluation and Patients with isolated cervical carotid bruits are considered close follow-up by an opthalmologist and neurologist for a carotid duplex ultrasound prior to CTA. If ACAD is are of outmost importance. Optic nerve sheath fenestra- confirmed, no other imaging study is necessary. tion should be considered for patients with progressive visual loss [120,123]. Abnormal otoscopy/retrotympanic pathology These patients are considered for a CTA of the temporal Patients with ACAD and disturbing pulsatile tinnitus bones and neck at the initial evaluation. For patients with should be considered for surgical intervention. glomus jugulare/tympanicum tumors, the presence of Carotid endarterectomy (CEA) and carotid angioplasty synchronous carotid body tumor(s) can easily be detected with stenting (CAS) are the two procedures for treating in the same study. Lesions of the temporal bones such as ACAD. Randomized controlled trials comparing the effi- glomus tympanicum, ectopic carotid artery, and jugular cacy of CEA vs. medical therapy have shown a clear bulb/ dural venous sinuses abnormalities are easily benefit for CEA in patients with symptomatic stenosis detected as well. of greater than 70% and a lesser benefit in patients with 50–69% stenosis. More recent studies comparing CAS Carotid angiography is indicated only in cases with strong with CEA failed to reach conclusions regarding a clear suspicion of an AVF/AVM, such as patients with loud neurologic outcome advantage of one method over the Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.  Pulsatile tinnitus Sismanis 353 Figure 1 Pulsatile tinnitus evaluation algorithm (a) Normal otoscopy Venous PT Arterial PT MRI/MRV Duplex Ultrasound CTA (Isolated neck bruit) Empty Sella Normal Small ventricles (Papilledema, Cervical/Intracranial ACAD Normal Flattening of Obese female) ACAD posterior aspect AVF/AVM of globe Tortuous carotid vessels LP Dissection/Aneurysms Fibromuscular Dysplasia Conventional angiogram? IIH Syndrome LP IIH Syndrome (b) Retrotympanic pathology CTA Glomus Tumors (Tympanicum/Jugulare) Jugular bulb/Dural venous sinuses abnormalities (Mega bulb, High location, Diverticulum) Aberrant internal carotid artery Carotid body tumor ACAD, atherosclerotic carotid artery disease; AVF, arteriovenous fistula; AVM, arteriovenous malformation; CTA, computed tomography angiography; IIH, idiopathic intracranial hypertension; LP, lumbar puncture; MRV, magnetic resonance venography; PT, pulsatile tinnitus. other [125
]. Angioplasty has been reported to relieve carotid artery dissection with occlusion, stent-assisted pulsatile tinnitus secondary to atherosclerotic obstruction angioplasty is effective [131,132]. of the subclavian and intracranial carotid arteries [27,126]. Repair of symptomatic high/dehisced jugular bulbs and Glomus tympanicum tumors are amenable to surgical diverticulae has been reported by using bone dust, peri- extirpation with excellent long-term results [127,128]. chondrium, tragal cartilage, conchal cartilage, and mas- Treatment of glomus jugulare tumors should be indivi- toid cortical bone [44,93

,133,134]. Surgical repair of dualized, and, although the traditional management has transverse and sigmoid sinus aneurysms is effective in been surgical removal, stereotactic radiosurgery has eliminating pulsatile tinnitus [95
,135]. recently been revealed to be very effective [129
,130
]. Sectioning of the levator veli palatini muscle has been Although there have been no prospective, randomized reported for treating pulsatile tinnitus in cases with therapeutic trials for patients with carotid artery dissec- palatal myoclonus. However, botulinum toxin injec- tion, experience shows that anticoagulants such as tion seems to be a more appropriate treatment heparin followed by coumadin are effective in preventing [105
,136,137]. Tensor tympani and stapedial myoclonus further artery-to-artery emboli. In selected cases of may respond to sectioning of the respective muscles via Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.  354 Otology and neuro-otology tympanotomy [138,139]. The author’s experience with 4 Bink A, Goller K, Luchtenberg M, et al. Long-term outcome after coil
embolization of cavernous sinus arteriovenous fistulas. AJNR Am J Neuro- two patients (three ears) has been very satisfactory. radiol 2010; 31:1216–1221. 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 Dural AVFs can be treated very effectively with combined of persistent cranial nerve deficits with disturbances of oculomotor and visual transvenous coil occlusion of the venous sinus segment 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 and transarterial occlusion of supplying arteries. for control of therapeutic success, patients should be routinely examined by ophthalmologists. Finally, ligation of the ipsilateral to the tinnitus IJV has 5 Carmody RF. Vascular malformations. In: Zimmerman RA, Gibby WA, Carmody RF, editors. Neuroimaging clinical and physical principles. New been recommended in the literature for patients with York: Springer-Verlag, Inc.; 2000. pp. 833–862. idiopathic pulsatile tinnitus. The results of this procedure 6 Hoang TA, Hasso AN. Intracranial vascular malformations. Neuroimaging have been very inconsistent and poor overall. In a series Clin North Am 1994; 4:823–847. 7 Zipfel GJ, Shah MN, Refai D, et al. Cranial dural arteriovenous fistulas: of 13 patients with essential tinnitus, three underwent

modification of angiographic classification scales based on new natural ligation of the ipsilateral IJV and only 1 benefited per- history data. Neurosurg Focus 2009; 26:E14. This article presents a modification to the existing classification scales of intra- manently. The other two patients experienced return cranial dural arteriovenous fistulas based on newly published research regarding of their pulsatile tinnitus within a few days. This the relationship of clinical symptoms and outcome. Patients who present inciden- tally or with symptoms of pulsatile tinnitus or ophthalmological phenomena have a procedure should rarely, if ever, be performed for alle- less aggressive clinical course. The authors have defined this subgroup as viating pulsatile tinnitus. asymptomatic cranial venous drainage (CVD). On the basis of 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. 8 Zager EL, Shaver EG, Hurst RW, Flamm ES. Distal anterior inferior cerebellar Conclusion artery aneurysms. Report of four cases. J Neurosurg 2002; 97:692–696. Pulsatile tinnitus deserves thorough evaluation. Failure to 9 Vories A, Liening D. Spontaneous dissection of the internal carotid artery presenting with pulsatile tinnitus. Am J Otolaryngol 1998; 19:213–215. establish correct diagnosis may have disastrous con- 10 Depauw P, Defreyne L, Dewaele F, Caemaert J. Endovascular treatment of a sequences, as potentially life-threatening pathologies such giant petrous internal carotid artery aneurysm. Case report and review of the as an AVF may be present. In the majority of cases there is a literature. Minim Invasive Neurosurg 2003; 46:250–253. treatable cause. Detailed history and auscultation of the 11 Dehaene I, Meeus L. Vertebral artery dissection without ischemic events in the vertebro-basilar system. Acta Neurol Belg 1989; 89:366–369. head and neck are of utmost importance. Radiologic 12 Medina DM, Carmody RF. Stroke. In: Zimmerman RA, Gibby WA, Carmody evaluation should be individualized according to the type RF, editors. Neuroimaging clinical and physical principles. New York: of pulsatile tinnitus (arterial or venous), presence of retro- Springer-Verlag Inc.; 2000. pp. 833–863. 13 Fayad JN, Keles B, Brackmann DE. Jugular foramen tumors: clinical char- tympanic pathology, and audible bruit. Treatment is acteristics and treatment outcomes. Otol Neurotol 2010; 31:299–305. directed toward the correction of the underlying cause. 14 Olin JW, Sealove BA. Diagnosis, management, and future developments of Ligation of the IJV for treatment should rarely, if ever, be
fibromuscular dysplasia. J Vasc Surg 2011; 53:826–836. Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular performed for alleviating pulsatile tinnitus. 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. An increasing number of patients are asymptomatic and are only discovered inciden- Acknowledgements tally when imaging is performed for some other reason or by the detection of an Conflicts of interest asymptomatic bruit. FMD should be considered in the differential diagnosis of a There are no conflicts of interest. 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 or less with onset of hypertension. Treatment consists of antiplatelet therapy for asymptomatic individuals and percutaneous balloon angioplasty for patients with References and recommended reading indications for intervention. Papers of particular interest, published within the annual period of review, have 15 Dufour JJ, Lavigne F, Plante R, Caouette H. Pulsatile tinnitus and fibromus- been highlighted as: cular dysplasia of the internal carotid. J Otolaryngol 1985; 14:293–295.
of special interest 16 Van Damme H, Sakalihasan N, Limet R. Fibromuscular dysplasia of the

of outstanding interest internal carotid artery. Personal experience with 13 cases and literature Additional references related to this topic can also be found in the Current review. Acta Chir Belg 1999; 99:163–168. World Literature section in this issue (p. 411). 17 Hosokawa S, Mineta H. Tortuous internal carotid artery presenting as a pharyngeal mass. J Laryngol Otol 2010; 124:1033–1036. 1 Sismanis A, Stamm MA, Sobel M. Objective tinnitus in patients with athero- sclerotic carotid artery disease. Am J Otol 1994; 15:404–407. 18 Beriat GK, Ezerarslan H, Kocaturk S, et al. Pulsatile oropharyngeal and neck mass caused by bilateral tortuous internal carotid artery: a case report. Kulak 2 Hatsukami TS, Yuan C. MRI in the early identification and classification of Burun Bogaz Ihtis Derg 2010; 20:260–263.
high-risk atherosclerotic carotid plaques. Imaging Med 2010; 2:63–75. Despite the widespread consensus on the importance of these plaque features, 19 Aleksic M, Schutz G, Gerth S, Mulch J. Surgical approach to kinking and testing the vulnerable plaque hypothesis in prospective clinical studies has been coiling of the internal carotid artery. J Cardiovasc Surg 2004; 45:43–48. hindered by the lack of reliable imaging tools for in-vivo plaque characterization. 20 Sismanis A, Girevendoulis A. Pulsatile tinnitus associated with internal This article provides a summary of the histological validation of carotid MRI and carotid artery morphologic abnormalities. Otol Neurotol 2008; 29:1032– highlights its application in prospective clinical studies aimed at early identification 1036. of the high-risk atherosclerotic carotid plaque. 21 Woo HJ, Song SY, Kim YD, Bai CH. Arteriovenous malformation of the 3 Bink A, Berkefeld J, Kraus L, et al. Long-term outcome in patients treated for external ear: a case report. Auris Nasus Larynx 2008; 35:556–558.
benign dural arteriovenous fistulas of the posterior fossa. Neuroradiology 2010. [Epub ahead of print] 22 Mahmood A, Malik GM. Dural arteriovenous malformations of the skull base. Partial treatment did not resolve the clinical symptoms of patients with ‘benign’ Neurol Res 2003; 25:860–864. dural AVF in the follow-up and was not clearly superior to conservative manage- 23 Tylern RS, Babin RW. Tinnitus. In: Cummings CW, Frederickson JM, Harker ment. These results suggest that embolization should be offered only if there is a LA, et al., editors. Head and neck surgery. St. Louis: CV Mosby Co.; 1986. possibility of a complete cure without major peri-interventional risks. pp. 3201–3217. Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.  Pulsatile tinnitus Sismanis 355 24 Ward PH, Babin R, Calcaterra TC, Konrad HR. Operative treatment of 52 Remley KB, Harnsberger HR, Jacobs JM, Smoker WR. The radiologic surgical lesions with objective tinnitus. Ann Otol Rhinol Laryngol 1975; evaluation of pulsatile tinnitus and the vascular tympanic membrane. Semin 84:473–482. Ultrasound CT MR 1989; 10:236–250. 25 Chen YJ, How CK, Chern CH. Cerebral dural arteriovenous fistulas present- 53 Levine SB, Snow JB Jr. Pulsatile tinnitus. Laryngoscope 1987; 97:401– ing as pulsatile tinnitus. Intern Med J 2007; 37:503. 406. 26 Nishikawa M, Handa H, Hirai O, et al. Intolerable pulse-synchronous tinnitus 54 Gibson R. Tinnitus in Paget’s disease with external carotid ligation. J Laryngol caused by occlusion of the contralateral common carotid artery. A successful Otol 1973; 87:299–301. treatment by aorto-carotid bypass surgery. Acta Neurochir (Wien) 1989; 101:80–83. 55 Davies DG. Paget’s disease of the temporal bone. A clinical and histopatho- logical survey. Acta Otolaryngol 1968; Suppl 242:3þ. 27 Donald JJ, Raphael MJ. Pulsatile tinnitus relieved by angioplasty. Clin Radiol 1991; 43:132–134. 56 Sismanis A, Smoker WR. Pulsatile tinnitus: recent advances in diagnosis. Laryngoscope 1994; 104:681–688. 28 Sonmez G, Basekim CC, Ozturk E, et al. Imaging of pulsatile tinnitus: a review of 74 patients. Clin Imaging 2007; 31:102–108. 57 Sismanis A. Otologic manifestations of benign intracranial hypertension syndrome: diagnosis and management. Laryngoscope 1987; 97:1–17. 29 Daneshi A, Hadizadeh H, Mahmoudian S, et al. Pulsatile tinnitus and carotid artery atherosclerosis. Int Tinnitus J 2004; 10:161–164. 58 Sismanis A. Pulsatile tinnitus. A 15-year experience. Am J Otol 1998; 19:472–477. 30 Spector GJ, Ciralsky RH, Ogura JH. Glomus tumors in the head and neck. III. Analysis of clinical manifestations. Ann Otol Rhinol Laryngol 1975; 84:73– 59 Sismanis A, Butts FM, Hughes GB. Objective tinnitus in benign intracranial 79. hypertension: an update. Laryngoscope 1990; 100:33–36. 31 Lustig LR, Jackler RK. Paraganglioma (glomus tumor). In: Hughes GB, 60 Sorensen PS, Krogsaa B, Gjerris F. Clinical course and prognosis of Pensak ML, editors. Clinical otology. New York: Thieme; 2007. pp. 326– pseudotumor cerebri. A prospective study of 24 patients. Acta Neurol Scand 330. 1988; 77:164–172. 32 Hillman TA, Kertesz TR, Hadley K, Shelton C. Reversible peripheral vesti- 61 Fishman RA. Benign intracranial hypertension. In: Cerebrospinal fluid in dis- bulopathy: the treatment of superior canal dehiscence. Otolaryngol Head ease of the nervous system. Philadelphia: WB Saunders; 1980. pp. 128–139. Neck Surg 2006; 134:431–436. 62 Skau M, Sander B, Milea D, Jensen R. Disease activity in idiopathic intra- 33 Gruber B, Hemmati M. Fibromuscular dysplasia of the vertebral artery: an
cranial hypertension: a 3-month follow-up study. J Neurol 2011; 258:277– unusual cause of pulsatile tinnitus. Otolaryngol Head Neck Surg 1991; 283. 105:113–114. The purpose of this study was to evaluate the clinical presentation and monitor a 3-month course using frequent OCT evaluations, visual field testings and lumbar 34 Wells RP, Smith RR. Fibromuscular dysplasia of the internal carotid artery: a opening pressure measurements. In patients with weight loss greater than 3.5% of long term follow-up. Neurosurgery 1982; 10:39–43. BMI, ICP decreased significantly (P ¼ 0.0003). In patients with weight loss less 35 Cochran JH Jr, Kosmicki PW. Tinnitus as a presenting symptom in pernicious than 3.5% of BMI, changes in ICP were insignificant (P ¼ 0.6). OCT combined with anemia. Ann Otol Rhinol Laryngol 1979; 88:297. 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 36 Cary FH. Symptomatic venous hum. Report of a case. N Engl J Med 1961; outcome with respect to CSF pressure. 264:869–870. 63 Bruce BB, Kedar S, Van Stavern GP, et al. Idiopathic intracranial hyperten- 37 Selim M, Caplan LR. Carotid artery dissection. Curr Treat Options Cardio- sion in men. Neurology 2009; 72:304–309. vasc Med 2004; 6:249–253. 64 Brackmann DE, Doherty JK. Facial palsy and fallopian canal expansion 38 Liess BD, Lollar KW, Christiansen SG, Vaslow D. Pulsatile tinnitus: a associated with idiopathic intracranial hypertension. Otol Neurotol 2007; harbinger of a greater ill? Head Neck 2009; 31:269–273. 28:715–718. 39 Saeed SR, Hinton AE, Ramsden RT, Lye RH. Spontaneous dissection of 65 Heidary G, Rizzo JF III. Use of optical coherence tomography to evaluate the intrapetrous internal carotid artery. J Laryngol Otol 1990; 104:491– papilledema and pseudopapilledema. Semin Ophthalmol 2010; 25:198–205. 493. 66 Marcelis J, Silberstein SD. Idiopathic intracranial hypertension without 40 Palacios E, Gomez J, Alvernia JE, Jacob C. Aneurysm of the petrous portion of papilledema. Arch Neurol 1991; 48:392–399. the internal carotid artery at the foramen lacerum: anatomic, imaging, and otologic findings. Ear Nose Throat J 2010; 89:303–305. 67 Digre KB, Nakamoto BK, Warner JE, et al. A comparison of idiopathic intracranial hypertension with and without papilledema. Headache 2009; 41 Campbell JB, Simons RM. Brachiocephalic artery stenosis presenting with 49:185–193. objective tinnitus. J Laryngol Otol 1987; 101:718–720. 68 Thurtell MJ, Newman NJ, Biousse V. Visual loss without papilledema in 42 Fernandez AO. Objective tinnitus: a case report. Am J Otol 1983; 4:312– idiopathic intracranial hypertension. J Neuroophthalmol 2010; 30:96–98. 314. 69 Rougier MB. Diagnosing bilateral papilledema [in French]. J Fr Ophtalmol 43 Bold EL, Wanamaker HH, Hughes GB, et al. Magnetic resonance angio- 2010; 33:424–429. graphy of vascular anomalies of the middle ear. Laryngoscope 1994; 70 Dessardo NS, Dessardo S, Sasso A, et al. Pediatric idiopathic intracranial 104:1404–1411. hypertension: clinical and demographic features. Coll Antropol 2010; 34 44 Glasscock ME III, Dickins JR, Jackson CG, Wiet RJ. Vascular anomalies of (Suppl 2):217–221. the middle ear. Laryngoscope 1980; 90:77–88. 71 Wall M, George D. Idiopathic intracranial hypertension. A prospective study 45 Steffen TN. Vascular anomalies of the middle ear. Laryngoscope 1968; of 50 patients. Brain 1991; 114 (Pt 1A):155–180. 78:171–197. 72 Wall M. Idiopathic intracranial hypertension (pseudotumor cerebri). Curr Neurol Neurosci Rep 2008; 8:87–93. 46 Soyka MB, Schuknecht B, Huber AM. Aberrant internal carotid artery as a cause of pulsatile tinnitus: a difficult diagnosis in MRI [in German]? HNO 73 Sugerman HJ, DeMaria EJ, Felton WL III, et al. Increased intra-abdominal 2010; 58:151–154. pressure and cardiac filling pressures in obesity-associated pseudotumor cerebri. Neurology 1997; 49:507–511. 47 Pirodda A, Sorrenti G, Marliani AF, Cappello I. Arterial anomalies of the middle ear associated with stapes ankylosis. J Laryngol Otol 1994; 74 Johnston I, Paterson A. Benign intracranial hypertension. I: Diagnosis and 108:237–239. prognosis. Brain 1974; 97:289–300. 48 Gulya AJ, Shuknecht HF. A large artery in the apical region of the cochlea of a 75 Felton WL, Marmarou A, Bandon K. Cerebrospinal fluid pressure dynamics in man with pulsatile tinnitus. Am J Otol 1984; 5:262. pseudotumor cerebri (abstract). Neurology 1991; 41(Suppl 2). 76 Josephs LG, Este-McDonald JR, Birkett DH, Hirsch EF. Diagnostic 49 Lesinski SG, Chambers AA, Komray R, et al. Why not the eighth nerve? laparoscopy increases intracranial pressure. J Trauma 1994; 36:815–818. Neurovascular compression – probable cause for pulsatile tinnitus. Otolar- yngol Head Neck Surg 1979; 87:89–94. 77 Gross CE, Tranmer BI, Adey G, Kohut J. Increased cerebral blood flow in idiopathic pseudotumour cerebri. Neurol Res 1990; 12:226–230. 50 De Ridder D, De Ridder L, Nowe V, et al. Pulsatile tinnitus and the intrameatal vascular loop: why do we not hear our carotids? Neurosurgery 2005; 78 Langfitt TW. Clinical methods for monitoring intracranial pressure and 57:1213–1217. measuring cerebral blood flow. Clin Neurosurg 1975; 22:302–320. 51 Levine RA. Typewriter tinnitus: a carbamazepine-responsive syndrome re- 79 Sismanis A, Callari RH, Slomka WS, Butts FM. Auditory-evoked responses in lated to auditory nerve vascular compression. ORL J Otorhinolaryngol Relat benign intracranial hypertension syndrome. Laryngoscope 1990; 100:1152– Spec 2006; 68:43–46. 1155. Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.  356 Otology and neuro-otology 80 Agid R, Farb RI, Willinsky RA, et al. Idiopathic intracranial hypertension: the 105 Daval M, Cohen M, Mari I, Ayache D. Objective tinnitus and essential palatal validity of cross-sectional neuroimaging signs. Neuroradiology 2006;
tremor in children: report of a case. Rev Laryngol Otol Rhinol 2009; 48:521–527. 130:117–119. 81 Higgins JN, Cousins C, Owler BK, et al. Idiopathic intracranial hypertension: Palatal tremor is a rare neurotological disorder responsible for objective tinnitus in 12 cases treated by venous sinus stenting. J Neurol Neurosurg Psychiatry children. Palatal tremor may be symptomatic of an underlying neurological disease 2003; 74:1662–1666. or essential when a cause cannot be identified. This is a case report of essential palatal tremor in a 10-year-old girl complaining of clicking tinnitus. No treatment 82 Owler BK, Parker G, Halmagyi GM, et al. Pseudotumor cerebri syndrome: was taken, as she was not obviously bothered by the ear-clicking sound. Recently, venous sinus obstruction and its treatment with stent placement. J Neurosurg several publications reported satisfactory results with botulinum toxin injection, 2003; 98:1045–1055. which seems to be the treatment of choice. 83 Donnet A, Metellus P, Levrier O, et al. Endovascular treatment of idiopathic 106 Oliveira CA, Negreiros JJ, Cavalcante IC, et al. Palatal and middle-ear intracranial hypertension: clinical and radiologic outcome of 10 consecutive myoclonus: a cause for objective tinnitus. Int Tinnitus J 2003; 9:37–41. patients. Neurology 2008; 70:641–647. 107 Heller MF. Vibratory tinnitus and palatal myoclonus. Acta Otolaryngol 1962; 84 Farb RI, Vanek I, Scott JN, et al. Idiopathic intracranial hypertension: the 55:292–298. prevalence and morphology of sinovenous stenosis. Neurology 2003; 60:1418–1424. 108 Pulec JL, Hodell SF, Anthony PF. Tinnitus: diagnosis and treatment. Ann Otol Rhinol Laryngol 1978; 87:821–833. 85 Lee SW, Gates P, Morris P, et al. Idiopathic intracranial hypertension; immediate resolution of venous sinus ‘obstruction’ after reducing cerebro- 109 Sila CA, Furlan AJ, Little JR. Pulsatile tinnitus. Stroke 1987; 18:252– spinal fluid pressure to