Tinnitus PDF
Tinnitus PDF
Tinnitus PDF
DOI 10.1007/s40134-017-0199-7
Frederick J. A. Meijer1
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5 Page 2 of 10 Curr Radiol Rep (2017) 5:5
somatomotor, and visual-motor circuits, e.g., by pressure disease. The value of imaging modalities for the detection
on myofacial trigger points, specific eye-movements, or and characterization of different pathologies, which can
powerful muscle contractions. cause pulsatile tinnitus, is presented in Table 1.
When the auditory perception is only perceived by the For screening for underlying pathology and for the evalu-
patient and cannot be heard by the clinician by auscultation, ation of a possible soft tissue mass or intracranial pathology,
it is called subjective tinnitus. In case of objective tinnitus, initial evaluation with MRI and MR angiography (MRA) is
which can be heard by the clinician, more frequently the recommended with reported high diagnostic accuracy [17•].
etiology can be found by ancillary investigations and there An appropriate MRI protocol for the evaluation of cochlear
is usually a genuine physical source of sound in contrary to and retro-cochlear pathology includes at least T1-W and 3D
subjective tinnitus [10]. Subjective tinnitus is more preva- T2-W sequences of the skull base and the posterior fossa.
lent than objective tinnitus. The etiology of subjective tin- Intravenous administration of a contrast agent, gadolinium,
nitus often lies in otologic disorders that also lead to should be considered for the detection of possible labyrinth or
conductive or sensorineural hearing loss [10, 11]. Con- cranial nerve enhancement, and for the detection of a soft
ductive hearing loss may be caused by impaction of ceru- tissue mass. A subsequent contrast-enhanced or time of flight
men, external or internal otitis, cholesteatoma, ossicular MRA is advised, in case a paraganglioma or vascular mal-
chain abnormalities, or tympanic membrane perforation formation is suspected. The inclusion of a sequence covering
[12]. Sensorineural hearing loss is caused by disease or the whole head, a T1-W, T2-W, or FLAIR sequence, needs to
abnormality at the level of the inner ear or eighth cranial be considered for the evaluation of intracranial space-occu-
nerve, and etiologies include noise-induced hearing loss, pying or vascular pathology.
Meniere’s disease, or acoustic neurinoma [13–15]. For the evaluation of osseous pathology of the temporal
Identification of the underlying cause of pulsatile tinnitus is bone, a limited scanning range of thin-sliced (submilli-
important for adequate treatment and for prognosis estima- metric) CT is sufficient. Multiplanar reconstruction (MPR)
tion. Different guidelines are available for the diagnostic of the acquisition is crucial for adequate evaluation, such as
work-up of pulsatile tinnitus [3, 10, 11, 16]. Complete and the identification of bony dehiscence of vascular canals or
detailed history taking is essential, which includes question- the skull base.
ing for possible accompanying complaints like vertigo, Multi-detector CTA or CT venography (CTV) of the
hearing loss, otorrhoea, and otalgia as well as the course of head and neck region can be performed for the evaluation
symptoms. In addition, one needs to be aware of possible of vascular pathology. Bone window images of the skull
accompanying neurological deficits. Next, an otologic phys- and temporal bone can be reconstructed from a multi-de-
ical examination needs to be performed. Using otoscopy, one tector CTA or CTV acquisition, obviating the need for a
can already evaluate the presence of a tympanic cavity mass. separate acquisition, which reduces radiation exposure.
An underlying vascular etiology can be suspected when pul- While the anatomic evaluation of multi-detector CTA/CTV
satile tinnitus is influenced by vascular compression or when a is excellent, the evaluation of flow dynamics is limited
vascular bruit is heard by auscultation. Audiometric evalua- because only a single time point is obtained during the
tion may also reveal a possible cause of tinnitus, such as noise- passage of a contrast bolus. Dynamic CTA, also referred to
induced hearing loss or otosclerosis. as 4D-CTA, is a technique that combines the non-invasive
In this review, we will discuss imaging strategies for the nature of CTA with the dynamic acquisition of digital
diagnostic work-up of pulsatile tinnitus. Furthermore, the subtraction angiography (DSA) [18•]. 4D-CTA enables the
differential diagnosis of pulsatile tinnitus is discussed and evaluation of flow dynamics of vasculature by multiple
imaging findings of different diseases are presented, both subsequent CT acquisitions, or a continuous volume CT
for CT and MRI. We differentiate vascular, neoplastic, and acquisition, for a period of time. The coverage and tem-
osseous etiologies. poral resolution of 4D-CTA depend on the width of the CT
detector. Detector configurations that cover the whole head
Radiological Work-Up: What Imaging Strategy with 16-cm coverage are available from 2 major vendors,
to Choose? either as 320 9 0.5 mm or 256 9 0.625 mm collimations
[18•]. A temporal resolution up to 20 frames/sec can be
Considering the broad differential diagnosis of pulsatile achieved from a continuous volume acquisition. Scanners
tinnitus, the optimal diagnostic imaging strategy depends with 4- to 8-cm coverage acquire smaller portions of the
on the initial clinical evaluation. Both CT and MRI can be vascular system. An advantage of 4D-CTA over dynamic
useful, and in general, these modalities are complementary. MRA is that 4D-CTA is not limited by the trade-off
The scanning protocol can be optimized based on the between temporal and spatial resolution [19]. The radiation
estimated a priori chance for finding specific pathology, or dose of 4D-CTA should however be kept as low as pos-
the need to rule out more rare but clinical significant sible, which can be achieved by implementing adequate
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Table 1 Value of imaging modalities for the detection and characterization of different pathologies which can cause pulsatile tinnitus
Pathology Non- Conventional 4D- Conventional MRA/ DSA Duplex
enhanced CTA/CTVa CTAa MRI study MRV ultrasound
CT
filtering and image registration techniques. 4D-CTA gen- occurs between arteries and veins without the presence of a
erates a large amount of data, which requires powerful normal intervening arteriole-capillary bed. Typically, an
workstations and optimized data processing. In case a AVM develops in adolescence or young adulthood but can
vascular malformation such as a dAVF is considered as a remain occult for a long period [20]. An AVM located in
possible cause of pulsatile tinnitus, 4D-CTA can be a non- the head and neck region can be the cause of pulsatile
invasive alternative to DSA. tinnitus.
The role of DSA in the diagnostic work-up of pulsatile An AVF is an, usually acquired, abnormal connection
tinnitus has been minimized, and should be reserved for the between an artery and a vein without an intervening nidus.
indication to rule out vascular pathology in case MRI/MRA Located along the dura or within a dural sinus, these are
and CT/(4D-)CTA have not revealed the cause of pulsatile called dural AVF (dAVF). A direct arteriovenous shunt
tinnitus. DSA can also be performed for the purpose of between a cerebral artery and a cortical draining vein is
treatment planning, e.g., for determining the exact called a pial AVF, and occur within the brain or along the
angioarchitecture of a vascular malformation or in preop- brain surface. An AVF can have a simple (single AVF) or
erative tumor embolization. more complex (multihole AVF) angioarchitecture.
The role of duplex ultrasound in the diagnostic work-up The pathophysiology of dAVFs is controversial, but
of pulsatile tinnitus is limited, although duplex ultrasound mostly considered a result of local hypoperfusion within a
is an effective screening tool for the evaluation of super- thrombosed dural sinus [21]. Progressive angiogenesis is
ficial tissue structures or superficial vascular malformations triggered in the dural sinus wall and proliferation of
and for the evaluation of vessel wall pathology of the microvascular networks develops into a plexus of venous
carotid arteries. channels, leading to an AVF. Pulsatile tinnitus results from
high-flow passage through the sigmoid and petrosal sinus.
AVF is more frequent in pulsatile tinnitus than AVM,
Differential Diagnosis and Imaging Findings with reported prevalence numbers varying from 2 to 27%
[6, 22, 23].
Vascular Etiologies The nidus and tortuous vessels of an AVM can be
detected either by MRI/MRA or CTA (Fig. 1), although the
Arteriovenous Malformation and Dural Arteriovenous evaluation of the precise angioarchitecture of an AVM or
Fistula micro-AVM is better depicted on DSA or 4D-CTA [24••,
25]. The detection of a dAVF is challenging on MRI/MRA
An AVM consists of a network of tortuous dilated arteries and conventional CTA because frequently only indirect
and veins, referred to as a nidus, through which shunting signs of a dAVF are visible, such as dilated vessels,
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cerebral edema, or (micro)hemorrhage. The evaluation of of tinnitus still need to be further addressed in larger cohort
flow dynamics is limited on MRI/MRA and conventional studies.
CTA, and therefore DSA is considered the gold standard
for the detection and evaluation of a dAVF. As DSA is a Vascular Stenoses
invasive procedure, it bears a small but non-ne-
glectable risk of neurological complications [26]. There is In the elderly population, atherosclerotic disease of the
increasing evidence that 4D-CTA has added value over carotid or vertebral arteries is thought to be the most
conventional CTA for the diagnosis, treatment planning, common cause of pulsatile tinnitus [34]. In a significant
and follow-up of dAVF [27••, 28]. Abnormal venous stenosed or occluded artery, increased vascular flow on the
drainage is the hallmark for classifying and treatment contralateral side could lead to pulsatile tinnitus as a
decision making of a dAVF. The Borden and Cognard symptom.
classifications are most commonly used for dAVFs Fibromuscular dysplasia (FMD) is a segmental non-
[29, 30]. Retrograde venous flow in cortical veins, which is atheromatous, non-inflammatory vascular disease of
associated with increased risk of intracranial hemorrhage, unknown etiology. Often it is a disease of the young
can be adequately visualized by 4D-CTA [31]. An example leading to vascular stenosis and cerebral ischemia. In
of a multihole dAVF located in the sigmoid sinus as medium-sized arteries, like the vertebral and carotid
identified by 4D-CTA and DSA is provided in Fig. 2. arteries, fibroblast-like changes of the smooth muscle cells
The main arterial feeders and the patterns of venous cause narrowing of the arteries and seem to cause pulsitale
drainage of a dAVF or AVM as detected by 4D-CTA or tinnitus more frequently than in atherosclerotic disease.
MRA seem to be sufficient in most cases to correctly This is probably due to the location of arterial stenosis in
identify and classify an AVM or AVF, which could save FMD. In FMD, stenosis of the carotid artery is frequently
the patient a pre-treatment invasive DSA [27, 28, 32]. In located at the upper cervical level, resulting in easily
addition, 4D-CTA could replace invasive DSA in the fol- transmitted vascular turbulence to the temporal bone. The
low-up of head and neck vascular malformations. The classical imaging appearance of FMD is the so-called
decrease in spatial resolution in comparison to DSA does ‘‘string of beads’’ pattern shown on angiographic studies.
not seem to change clinical management for most patients. Vascular loops and elongated arteries are occasionally
Recently, carotid duplex ultrasound focusing on low described as a possible cause of pulsatile tinnitus [35, 36].
resistance indexes of the external carotid and occipital Considering the presence of these vascular loops and
arteries has been reported as a possible screening tool for elongations also in asymptomatic patients, other possible
dAVF in patients with pulsatile tinnitus [33]. The role of causes of pulsatile tinnitus need to be ruled out in those
4D-CTA and duplex ultrasound in the diagnostic work-up subjects.
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Fig. 2 Dural arteriovenous fistula (dAVF) located in the right coded as red-orange, delayed contrast enhancement is coded as
sigmoid sinus as identified by 4D-CTA and DSA. Left 4D-CTA yellow-green. Notice the red-colored, hypertrophic occipital artery on
lateral subtracted MIP demonstrating abnormal early contrast filling the right side serving as arterial feeders of the dAVF. Right DSA,
of the sigmoid sinus (white arrow) consistent with dAVF. Hyper- selective contrast injection of the external carotid artery showing a
trophic occipital artery identified as arterial feeder (black arrow). hypertrophic tortuous occipital artery (black arrows). Venous
Anterograde venous drainage in the jugular vein. Middle Color-coded drainage of the sigmoid sinus into the jugular vein (white arrows)
processing of 4D-CTA. Early contrast enhancement (arterial flow) is (Color figure online)
Persistence of the Stapedial Artery Rotating the patient’s head away from the involved side
may relieve the murmur. Symptoms may increase by
An aberrant course of the internal carotid artery and per- rotating the head toward the involved side. There seems to
sistence of the stapedial artery are congenital variants that be an association with congenital variants such as a high-
need to be recognized on imaging studies. An aberrant riding, enlarged, or diverticulum of the jugular bulb, which
course of the internal carotid artery in the middle ear may can be best depicted on thin-sliced high-resolution CT [38].
mimic a soft tissue mass or paraganglioma at otoscopy. Prevalence of sigmoid sinus diverticulum and dehiscence
The aberrant carotid artery enters the tympanic cavity via has been reported to be significantly higher in pulsatile
an enlarged tympanic canaliculus and then runs though the tinnitus than in the general population [39•].
middle ear where it, due to a dehiscence in the carotid
plate, enters the horizontal carotid canal (Fig. 3). The Paraganglioma
ascending carotid canal on the affected side has not
developed and is therefore absent on CT or MRI. Paraganglioma, also known as glomus tumor, is the most
A persistent stapedial artery fails to regress in early fetal frequent neoplastic cause of pulsatile tinnitus [40, 41].
development. As a result, the proximal course of the Most paragangliomas are sporadic, about 7–10% are
middle meningeal artery will not develop and the foramen familial and usually autosomal dominant in inheritance
spinosum will be absent (Fig. 3). In addition, CT may show [42]. In case of familial paragangliomas, they are fre-
subtle enlargement of the tympanic segment of the facial quently multicentric (35–50%) and can be associated with
nerve canal in the coronal plane. These findings are multiple endocrine neoplasia (MEN IIa and IIb) or
therefore indirect signs for possible persistence of the phakomatoses [42].
stapedial artery, especially because the persistent stapedial Being a highly vascularized lesion, it is one of the most
artery itself is usually hardly visible on MRI/MRA. How- common causes of pulsatile tinnitus. Involving only the
ever, one should consider that in about 3% of the cases, the jugular bulb (glomus jugulare), the middle ear or mastoid
foramen spinosum is absent on CT [37]. Both CTA or DSA (glomus tympanicum) or both (glomus jugulotympan-
can be used for confirmation. icum), most of the paragangliomas located in the temporal
bone will present with pulsatile tinnitus [41]. Bilateral
Venous Tinnitus pulsatile tinnitus is described in about 10% of cases due to
a possible bilateral localization of paraganglioma [40, 43].
Venous tinnitus is heard as a continuous murmer that In contrary, pulsatile tinnitus is generally not a presenting
exaggerates in systole. By light pressure on the ipsilateral symptom in a vagal paraganglioma (located along the vagal
jugular vein, the murmur decreases. Light pressure on the nerve) or carotid body paraganglioma (located at the car-
contralateral jugular vein will increase the murmur. otid bifurcation).
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A tympanic paraganglioma can be detected by otoscopic early contrast enhancement in the arterial contrast phase. In
inspection as a red, pulsatile tympanic mass and can be most instances, a hypertrophic ascending pharengeal artery
very small at presentation measuring only a few millime- can be identified as the main arterial feeder of the para-
ters. Paragangliomas grow along planes of least resistance ganglioma. DSA is not required for diagnosis, but can be
following existing pathways, through bony canals or along performed in case preoperative tumor embolization is
the vessels and nerves. A tympanic paraganglioma can considered.
arise from glomus bodies anywhere along the Jacobsen
nerve, a tympanic branch of the glossopharyngeal nerve. Osseous Pathology
Both CT and MRI can be used for the detection and
evaluation of a paraganglioma. The majority of tympanic Paget Disease
paragangliomas are located on the promontory as a small
well-defined tympanic mass with soft tissue mass. Usually, Paget disease is a primary bone disorder, which can be
there is no or little surrounding bone erosion. These small located in different parts of the skeleton, including the skull
tumors are best evaluated using thin-sliced CT with a bone and temporal bone. The disease is characterized by osteo-
algorithm (Fig. 4). clastic resorption, osteoblastic regeneration, and mosaic
There is an anatomic close relation between the tym- bone replacement, seen as areas of abnormal lytic or
panic cavity and the jugular foramen. As a result, a large sclerotic changes of bony structures on CT or MRI.
percentage of the paragangliomas will present as the Increase in the number and size of local vessels in Paget
jugulotympanic variant due to a tumor located in the disease could be the cause of pulsatile tinnitus [45].
jugular foramen with extension through the jugular plate
into the hypotympanum. MRI can be used to evaluate bony Otosclerosis
invasion, although this is usually better appreciated on CT.
A CT scan with bone algorithm may show irregular ero- Otosclerosis, also known as otospongiosis, is an idiopathic
sions of the adjacent bone (Fig. 5). These bony changes infiltrative process of the petrous bone. It causes both
can have a ‘‘moth eaten’’ appearance. The characteristics sensorineural and conductive hearing loss, and can be the
on CT are being used for the Fisch classification of cause of pulsatile tinnitus [46, 47]. Two types of otoscle-
(jugulo)-tympanic paragangliomas, based on which treat- rosis are differentiated based on the primary region of
ment decisions are made [44]. involvement, fenestral otosclerosis and cochlear otoscle-
The classical MRI characteristics of a paraganglioma rosis. The latter is also referred to as retrofenestral oto-
include a mixture of hypo- and hyper-intensity on T1-W sclerosis. High-resolution, thin-sliced CT typically shows
and T2-W sequences, also referred to as a ‘salt-and-pepper’ abnormal hypoattenuated bone in the region of the fissula
pattern, based on multiple vascular flow voids within the antefenestram in fenestral otosclerosis (Fig. 6 left).
lesion. Due to the hypervascular nature, a paraganglioma Cochlear otosclerosis appears as a hypoattenuated halo
enhances avidly (Fig. 5). Dynamic contrast-enhanced surrounding the cochlea on CT (Fig. 6 right). MRI is
MRA can differentiate a paraganglioma from lymph nodes considered not sensitive for the diagnosis of otosclerosis,
or other soft tissue lesions, as a paraganglioma will show although inhomogeneous high T2 signal, low T1 signal, or
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Fig. 5 Glomus jugulotympanicum on CT and MRI. Left CT demon- voids, which makes up the ‘salt and pepper’ appearance. Right Axial
strating erosive changes at the jugular bulb (arrows). Notice extension contrast-enhanced T1-W with fat suppression demonstrates avid
of the soft tissue into the middle ear (arrowhead). Middle Axial T1-W contrast enhancement of the tumor
image shows a mixture of signal intensities due to vascular flow
subtle contrast enhancement can be seen in the affected trabecular thickening is seen on CT with preservation of
petrous bone. the inner and outer cortex. MRI will show heterogeneous
hyper-intense signal on T1-W and T2-W sequences, and
Other Osseous Pathology less frequently hypo- or iso-intense signal intensity. A
hemangioma enhances diffusely after contrast
Highly vascularized bone lesions, like osseous heman- administration.
gioma, basal meningeoma, Langerhans cell histiocytosis, A meningioma, a tumor originating from the meninges,
or bone metastases, have been described as possible causes can show permeative changes and sclerosis of the adjacent
of pulsatile tinnitus [48–50]. bone but can also have a significant intraosseous localiza-
An osseous hemangioma is a sharply demarcated tion. A meningeoma is characterized by homogeneous
expansile intraosseous lesion. Typically, aggravated
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Fig. 7 Meningeoma on MRI. Axial contrast-enhanced T1-W images. Enhancing mass located in the left cerebellar-pontine angle with extension
into hypoglossal canal (arrow), jugular plate (encircled), and the middle ear (arrowhead)
contrast enhancement and typically shows a dural tail Examples of typical highly vascularized bone metas-
configuration (Fig. 7). tases include renal cell carcinoma and thyroid carcinoma.
Langerhans cell histiocytosis can occur anywhere in the
skeleton, and in the head and neck region, the most fre- Idiopathic Intracranial Hypertension
quent localizations include the mastoid and the squamous
portion of the temporal bone. The affected bone shows Idiopathic intracranial hypertension (IIH), which predom-
lytic, punched-out changes on CT. The affected bone inantly affects young obese women, may cause pulsatile
marrow will show signal alterations on MRI. tinnitus, although IIH is primarily characterized by
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symptoms of headache and blurred vision due to increased use, distribution, and reproduction in any medium, provided you give
cerebrospinal fluid pressure [51••]. Brain MRI typically appropriate credit to the original author(s) and the source, provide a
link to the Creative Commons license, and indicate if changes were
shows an empty sella and increased cerebrospinal fluid made.
around the optic nerves. The exact pathophysiology of IIH
is unknown but can develop in patients with a history of
dural sinus trombosis. Dural sinus stenosis or compression
can also be observed in IHH. It is therefore advised to References
perform MRV or CTV in a patient with pulsatile tinnitus
Papers of particular interest, published recently, have been
and suspicion of IIH.
highlighted as:
• of importance and
•• of major importance
Conclusion
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Open Access This article is distributed under the terms of the first line diagnostic imaging modality in pulsatile tinnitus, as it
Creative Commons Attribution 4.0 International License (http:// can accurately detect underlying vascular or neoplastic
creativecommons.org/licenses/by/4.0/), which permits unrestricted pathology.
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