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Otology > Pulsatile Tinnitus > Flashcards

Pulsatile Tinnitus Flashcards

1
Q

Pulsatile Tinnitus - Definition

A

1) Abnormal perception of rhythmic sound of internal origin
2) Can be subjective or objective
- can be pulse synchronous or non-synchronous
- Unilateral or bilateral
3) Etiology can be
- Venous
- Arterial
- Non-Vascular source
4) Caused by abnormal blood flow pattern transmitted to the cochlea

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2
Q

Epidemiology of Pulsatile Tinnitus

A

1) 10% of the population has tinnitus
2) 4-10% of tinnitus patients report pulsatile tinnitus
3) Associated with anxiety, depression, suicide
4) There is an identifiable cause in 70% of patients

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3
Q

Clinical Presentation of Pulsatile Tinnitus

A

1) History
- Onset, progression, severity, P/P factors, mitigating positions or movements
- Associated symptoms, Hearing loss, autophony, Tullio phenomenon, aural pressure, Headaches, visual changes, vertigo, anxiety, depression
- Headaches: if there is a positional component, it is suspicious for Idiopathic Intracranial Hypertension
- Is the Tinnitus pulse synchronous or asynchronous: abnormal blood flow vs mechanical sounds (within the body)
-

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4
Q

Headache and Pulsatile Tinnitus

A

If there are positional changes associated with the headaches or pulsatile tinnitus, this is suspicious for Idiopathic Intracranial Hypertension

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5
Q

What is the significance of Pulse synchronous tinnitus vs asynchronous tinnitus?

A

1) Pulse synchronous: abnormal vascular flow
2) Pulse asynchronous: mechanical sounds within the body

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6
Q

Where do ‘drum beat’ or ‘suction cup’ like sounds in the ear come from?

A

Palatal or middle ear myoclonus

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7
Q

What is the significance of the frequency of pulsatile tinnitus?

A

1) Low frequency buzzing or humming sound = Venous origin
2) High frequency ‘whooshing,’ ‘hissing,’ or ‘fetal heartbeat’ sounds = Arterial origin
3) Very high frequency - ringing or continuous high frequency with breaks = not likely vascular

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8
Q

Physical Exam for Pulsatile Tinnitus

A

1) Complete H/N exam
- Detailed neurotologic exam: microotoscopy, neuro/CN exam
- Cranial nerve exam
2) Auscultation: behind the ear/around the ear
3) Neck: Carotid bruits
4) Cardiac: Heart murmurs
Note: if you can hear a bruit around the ear without a stethoscope - has to be an arteriovenous fistula. No other condition can cause this finding.

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9
Q

If you can hear a bruit around the ear without a stethoscope, what does this signify?

A

This has to be an arteriovenous fistula - no other condition can cause this finding.

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10
Q

Pulsatile Tinnitus - Definition

A

Must be pulse synchronous

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11
Q

Ipsilateral Internal Jugular Vein Compression and pulsatile tinnitus

A

If the tinnitus resolves with ipsilateral internal jugular vein compression, it is venous in origin. Put the patient’s head in different positions to see how it affects the pulsatile tinnitus.

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12
Q

Ipsilateral Head Turning and pulsatile tinnitus

A

If the pulsatile tinnitus improves with ipsilateral head turning, it is from an ipsilateral venous cause

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13
Q

If contralateral neck compression worsens the pulsatile tinnitus

A

The PT is from an ipsilateral venous cause due to increased transtorcular flow

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14
Q

If ipsilateral neck compression worsens the pulsatile tinnitus?

A

Is from ipsilateral condylar vein flow or an arterial cause due to partial narrowing.

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15
Q

If occipital artery compression improves pulsatile tinnitus, what is the likely cause of the PT?

A

a dural AVF supplied by the occipital artery.

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16
Q

What audiometric testing should be done to evaluate pulsatile tinnitus?

A

1) Air/Bone conduction thresholds
2) Tympanometry
3) Acoustic reflex testing
4) Speech audiometry
5) Transcanal sound recordings

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17
Q

What is the differential diagnosis for a patient that has asymmetric SNHL and ipsilateral pulsatile tinnitus?

A

1) Paraganglioma
2) Schwannoma
3) Neoplasm
4) Meniere’s disease

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18
Q

What are the audiometric findings in a patient with superior semicircular canal dehiscence syndrome?

A

1) Suprathreshold low frequency bone conduction
2) Decreased air conduction thresholds
3) Normal acoustic reflexes

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19
Q

What imaging/tests should be obtained to evaluate patients with pulsatile tinnitus/pulse synchronous tinnitus?

A

1) MRI
- MRI of the Brain with IV Contrast
- MRA + MRV
2) High resolution temporal bone CT scan
3) CT angiogram of the head
- Has a lower sensitivity than MRI for Intracranial Hypertension and dural Arteriovenous Fistulae
4) Diagnostic cerebral angiogram
5) Image guided lumbar puncture with recording of opening pressure

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20
Q

What should the initial radiologic study/studies be in the evaluation of Pulse Synchronous Tinnitus?

A

1) CT Angiogram of the head
or
2) MRI/MRA/MRV of the head
Note: You should tailor the w/u to the physical exam findings

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21
Q

What are the different types of Pulsatile Tinnitus?

A

1) Structural
2) Metabolic
3) Vascular

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22
Q

What are the structural causes of pulsatile tinnitus?

A

1) Neoplasm
- Paraganglioma
- Schwannoma
- Skull base meningioma
- Endolymphatic sac neoplasm
- Skull base vascular metastasis
2) Temporal bone pathologic abnormality
- Semicircular canal dehiscence
- Otospongiosis
- Carotid canal dehiscence

23
Q

What are the metabolic causes of pulsatile tinnitus?

A

1) Ototoxic medications
- Aminoglycosides
- Cisplatinum
2) Vitamin toxic effects
- Vitamin B6
3) Myoclonus
- Tensor tympani muscle
- Stapedius muscle
- Soft palate
4) High cardiac output
- Hyperthyroidism
- Anemia
- Valvular heart disease

24
Q

What are the vascular causes of pulsatile tinnitus?

A

1) Venous causes
- Idiopathic intracranial hypertension
- Dural venous sinus stenosis
- Jugular vein stenosis
- Dural venous sinus diverticulum
- Jugular bulb diverticulum
- High riding jugular bulb
2) Arterial causes
- Dural Arteriovenous fistula
- Carotid-Cavernous fistula
- A-V Malformation
- Aneurysm
- Arterial dissection
- Fibromuscular dysplasia
- Carotid stenosis
- Aberrant arterial course
- Dolichoectasia = dilated and elongated (when arteries deteriorate their tunica intima/media, it weakens the vessel walls causing the artery to elongate and distend)

25
Q

What are the structural causes of pulsatile tinnitus?

A

Neoplasms and Temporal Bone pathologic abnormalities - detectable on MRI or CT scans
1) Paraglangliomas
- Neuroendocrine tumors are derived from the embryonic neural crest
- Pathogenic variants in genes encoding succinate dehydrogenase enzyme complex
- Parasympathetic paraganglia associated with the auricular branch of the vagus nerve or the tympanic branch of the glossopharyngeal nerve
- Marginal sinus dural AV fistula can be misdiagnosed as a jugular paraganglioma on MRI - angiography may be needed
2) Vestibular schwannomas can present with pulsatile tinnitus
- These are benign tumors of the Schwann cells arising from the vestibular nerve, or less frequently intralabyrinthine
- More than 60% of patients with vestibular schwannomas have tinnitus (Matthies - 1997)
- Asymmetric SNHL and pulsatile tinnitus
3) Superior Semi-Circular Canal Dehiscence Syndrome - Major cause of pulsatile tinnitus
- Thinning or absence of bone overlying the superior semicircular canal
- This leads to the increased audibility of any internal sound
- Symptoms include: aural pressure, conductive hearing loss, autophony, Tullio phenomenon
- Diagnosis - Imaging: CT scan with Stenver view and Poschl views (you need to get these special views), CT scan slice thickness should be no thicker than 0.6mm contiguous cuts
- Diagnosis: 1) Must have classic symptoms - must hear tuning fork on ankle, 2) Audiogram, 3) VEMP testing, 4) High resolution CT with Stenver and Poschl views, 5)

26
Q

Structural Causes of Pulsatile Tinnitus: Superior Semi-Circular Canal Dehiscence Syndrome and Pulsatile Tinnitus.

A

1) SSCDS is a major cause of pulsatile tinnitus is caused by thinning or absence of bone overlying the superior semicircular canal
2) Thinning of bone over the superior semicircular canal leads to the increased audibility of any internal sound
3) Symptoms include:
- aural pressure
- conductive hearing loss
- autophony
- Tullio phenomenon
4) Diagnosis
- Imaging: High resolution CT scan of the TBone with Stenver and Poschl views with CT scan slice thickness no thicker than 0.6mm contiguous cuts
- To make the diagnosis of SSCDS, you must have: 1) Must have classic symptoms - must hear tuning fork on ankle, 2) Audiogram, 3) VEMP testing, 4) High resolution CT scan with Stenver and Poschl views
5) Note: just bcs there is SSC dehiscence on CT scan doesn’t mean that the patient has SSCD syndrome - the patient must have symptoms
6) Many other things can mimic SSCD syndrome: vestibular migraine
- VEMP testing (cVEMP and oVEMP)
- Tuning fork on ankle testing
- Can they hear their eyeballs move?

27
Q

Structural Causes of Pulsatile Tinnitus: Osseous Pathology

A

1) Paget’s disease
2) Otosclerosis
3) Vascularized bone lesions
- Hemangioma
- Basal meningioma
- Langerhan’s histiocytosis
- Bone metastasis
4) Boney dehiscence
- Dehiscence of the Tegmen or Sigmoid sinus: may be related to prior surgery or chronic otitis media
- Dehiscence of the cortical plate overlying the sigmoid often coexists with sinus diverticula and intracranial venous hypertension

28
Q

Metabolic Causes of Pulsatile Tinnitus

A

1) Ototoxic medications
2) Hypervitaminosis B6
3) Myoclonus
- Tensor tympani muscle
- Stapedius muscle
- Soft palate
4) Anemia
5) Hyperthyroidism
6) Valvular heart disease

29
Q

Vascular Causes of Pulsatile Tinnitus

A

1) Carotid stenosis
2) Carotid artery dissection
3) Fibromuscular dysplasia
4) Aneurysm
5) Aberrant internal carotid artery
6) Arteriovenous fistula
7) Arteriovenous malformation
8) Persistent stapedial artery

30
Q

Fibromuscular dysplasia and pulsatile tinnitus

A

1) Definition: segmental, non-atheromatous, non-inflammatory disease
2) Unknown etiology
3) Pulsatile tinnitus occurs in 37% of patients with FMD
4) Prevalence of Stroke: 9.8%
5) Prevalence of TIA: 13.4%

31
Q

Arteriovenous Malformations and pulsatile tinnitus

A

1) Definition: network of tortuous, dilated arteries and veins. These serve as a nidus through which shunting occurs without an arteriole/capillary bed.
2) Diagnosis: MRI/MRA or CT Angiogram can detect
- Precise architecture is better seen on angiography
3) Retrograde venous flow in cortical veins is associated with an increased risk of intracranial hemorrhage

32
Q

Arteriovenous Fistulae and pulsatile tinnitus

A

1) Definition: acquired abnormal connection between an artery and a vein without an intervening nidus
- Type I, Type II, and Type III
2) These are thought to be caused by local hypoperfusion within a thrombosed dural sinus, triggering progressive angiogenesis
3) Indirect signs of dAVF visible on MRI/MRA or CT Angiography as dilated vessels, cerebral edema, and microhemorrhage
4) Angiography is considered the ‘Gold Standard’ for detection and evaluation. 4-D CT also provides some benefit
5) Retrograde venous flow in cortical veins is associated with increased risk of intracranial hemorrhage.

33
Q

Persistent Stapedial Artery and Pulsatile Tinnitus

A

1) Aberrant course of the Internal Carotid Artery
2) Failure of regression of the Stapedial Artery
3) The proximal course of the middle meningeal artery does not develop
- There is an absent foramen spinosum
4) Associated with subtle enlargement of the tympanic segment of the facial nerve
5) CTA or Angiography can be confirmatory

34
Q

Venous Causes of Pulsatile Tinnitus

A

1) Diverticula of the sigmoid sinus or jugular bulb
2) Stenosis of the transverse sinus, sigmoid sinus, and internal jugular vein
3) High riding jugular bulb
4) Enlarged condylar veins

35
Q

Idiopathic Intracranial Hypertension as a cause of Pulsatile Tinnitus

A

1) Symptoms:
- Headaches: worse in the dependent position
- Transient or gradual vision loss
- Low frequency, pulse synchronous tinnitus
2) Diagnosis:
- Modified Dandy criteria
- Lumbar puncture opening pressure > 25cm water
- MRV or objective assessment of tinnitus before and after removal of 20mL of CSF can be a good prognostic factor for therapy
3) Associations:
- Stenosis of the Transverse or Sigmoid Sinus
- Optic nerve sheath distension or tortuosity
- Empty sella
- Enlarged trigeminal cisterns
- Meningoceles
- Superior Semicircular Canal Dehiscence

36
Q

Idiopathic Intracranial Hypertension

A

1) The patient hears the pulsatile tinnitus in their head, not their ears
2) Associated with
- Empty sella
- Arachnoid pits
3) If suspicion is high for IIHTN, then obtain an LP with opening pressure - this is diagnostic
4) Treatment: VP or LP shunt

37
Q

Decision Tree for Pulse Synchronous Tinnitus (1)

A

1) Does pulse synchronous Tinnitus disappear with neck compression?
- If yes, then it is of vascular origin
- If no, then it is of non vascular origin

38
Q

Decision Tree for Pulse Synchronous Tinnitus (2) - Vascular causes

A

1) Arterial causes of pulsatile tinnitus
- A-V shunt, AVM, AVF, Padget’s disease
- Internal carotid artery
- Aneurysm
- Glomus tumors
2) Venous causes of pulsatile tinnitus
- Idiopathic Intracranial HTN
- Sigmoid sinus issues

39
Q

Decision Tree for Pulse Synchronous Tinnitus (3) - Nonvascular causes

A

1) Conductive hearing loss
2) Padget’s disease
3) Otosclerosis
4) Superior semicircular canal dehiscence syndrome
5) Meniere’s disease
6) Migraine headaches

40
Q

If pulsatile tinnitus disappears reliably with Internal Jugular Vein compression - what are the potential causes of the pulsatile tinnitus?

A

There are only two causes - this is venous phase pulsatile tinnitus:
1) Intracranial hypertension
- Has there been any recent weight gain?
2) Sigmoid sinus disease

41
Q

Pulsatile tinnitus and idiopathic intracranial hypertension

A

1) Probably underdiagnosed
2) Definitive diagnosis requires lumbar puncture with opening pressures
3) Affects 1:100,000 people
- 19 to 20: 100,000 in obese middle-aged women (20X more common in obese middle-aged females)
- However, a young thin man with intracranial hypertension can be easily overlooked
4) These patients can lose vision and hearing because of intracranial hypertension

42
Q

What are the causes of increased intracranial hypertension?

A

1) Obesity causing intracranial hypertension
2) CSF overproduction
3) CSF under absorption

43
Q

What are the known causes of increased intracranial hypertension?

A

1) Medications
- Acne meds: Accutane, cycline antibiotics
- Lithium
- Oral contraceptives, Growth hormone, oxytocin
- Phenytoin, Levodopa
2) Venous anomalies

44
Q

Intracranial hypertension and Accutane or ‘Cycline’ Antibiotics

A

Note: if a patient has been treated with Accutane or a ‘cycline’ antibiotic and they start to hear their pulse, this is almost certainly intracranial hypertension.

45
Q

How is idiopathic intracranial hypertension diagnosed?

A

1) Papilledema: send to ophthalmology for a dilated eye exam
- Note: not every patient with intracranial hypertension has papilledema
2) Imaging: flattening of the posterior aspect of the eye/globe
3) Lumbar Puncture with opening pressures - Gold Standard method of diagnosis
4) MRI/MRA/MRV or CTA/CTV of the head

46
Q

How to treat idiopathic intracranial hypertension

A

1) Stop medications responsible if possible
2) Give
- Diamox
- Topamax
- Lasix
3) VP or LP shunt placement
4) Weight loss

47
Q

Papilledema and idiopathic intracranial hypertension

A

1) If the patient has papilledema, then they almost certainly have intracranial hypertension.
2) If the fundoscopic exam does not show papilledema, this does not r/o intracranial hypertension - need to perform a lumbar puncture with opening pressure measurement to definitively diagnose IIHTN

48
Q

Lumbar Puncture in the diagnosis of idiopathic intracranial hypertension

A

1) LP is to diagnostic gold standard
2) If the opening pressure is 22 or 23, ask the patient if the tinnitus disappears when fluid is drained off during the LP
- If the pulsatile tinnitus disappears when fluid is drained during the LP, then the patient has intracranial HTN.
- If the tinnitus disappears as above, then treat the patient with Diamox until the tinnitus disappears.
- If the patient has an increased opening pressure on LP (35 or 40), unless they are massively obese and can lose a lot of weight with bariatric surgery or medications, then they will most likely require a VP or LP shunt. Diamox will not reduce a severely increased intracranial pressure down to the normal range.

49
Q

If you see intracranial venous constriction on your scans in the setting of idiopathic intracranial hypertension

A

1) This is not the cause of IIHTN, but is rather the sequalae of the increased intracranial pressure. When you drain off CSF, this resolves.
2) The constriction/stenosis of the venous system is from the enlargement of arachnoid granulations that have grown into the venous vessel.
3) The more arachnoid granulations that you have, the higher the likelihood is that you have increased intracranial hypertension.

50
Q

Treatment of Pulsatile Tinnitus

A

1) Medications: Diamox
- Diamox is the only medication that works, but will not control severely elevated idiopathic intracranial hypertension
- Diamox will only treat mildly elevated intracranial hypertension
2) Other Treatments
- Gastric bypass
- Biologics

51
Q

Intracranial hypertension and papilledema

A

1) Many patients with intracranial hypertension do not have papilledema.
2) You can ask a Neurophthalmologist to do an OCT test - this measures the optic nerve and the nerve sheath. This is more reliable than a fundoscopic exam. This test is not as reliable as a lumbar puncture with opening pressure measurement.

52
Q

Pulsatile Tinnitus and Venous Anomalies

A

1) Venous anomalies are much less frequent as the cause of pulsatile tinnitus.
- Patients with a dehiscent sigmoid sinus almost never have pulsatile tinnitus
2) Sigmoid sinus diverticulum - may cause pulsatile tinnitus
- Treatment: expose the diverticulum with a drill and then cover with bone cement

53
Q

Causes of Arterial Pulsatile Tinnitus

A

Petrosal dural fistula:
1) Diagnosis:
- Angiography - best
- Won’t show up on MRI
2) Treatment: embolization

Jugular Paragangliomas:
1) Diagnosis
- MRI
- Temporal Bone CT scan
- Angiography
- PET CT
2) Look for concurrent, distant disease

54
Q
A

Otology (9 decks)

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