Tinnitus Patient Management

1. Tinnitus Patient Management Robert W. Sweetow, Ph.D. University of California San Francisco, California

2. Some questions you need to consider • How much time does it take? • Is this worth my time? • How much will it cost me? (time, money, emotions) • How do I get reimbursed? • My very first case

3. Possible Mechanisms • Abnormal rhythm or rate of spontaneous 8th nerve discharges • Increased spontaneous activity in dorsal cochlear nucleus resulting from reduced peripheral input • Unlike spontaneous activity, these neural discharges may become phasic and correlated

4. Proposed Mechanisms • Imbalance of afferent and efferent: • Deficient afferent neurotransmitter glutamate at the cochlear-8th nerve synapse • “disinhibition” from neurotransmitters such as gamma aminobutyric acid (GABA) • Toxicity or imbalance of other of neurotransmitters • Ion channel regulation for excitability of neurons • Calcium induced changes in intra or extracellular processes (noise) • Vitamin B , zinc deficiencies?

5. Correlated activity across nerves by phase locking - ephaptic transmission Extralemniscal neurons, particularly in dorsal cochlear nucleus and AII area, receiving input from somasthetic system over-representation of edge-frequencies (neural plasticity) Increased activity in one hemisphere (right OR left, but not both, as occurs during sound stimulation) Enhanced perception hypothesis Sensory remodeling in the central auditory system Association with fear and threat (limbic system) Proposed mechanisms

6. Tinnitus and Hearing Loss

7. Correlation between tinnitus severity and auditory threshold Tsai, Cheung, and Sweetow, 2007

8. Models based on peripheral measures fail to predict completely percept laterality • Role of CNS adaptation (neural plasticity) • Absolute and relative depth of hearing loss are uncorrelated to percept severity • Role of CNS emotional binding (limbic system) Tsai, Cheung, and Sweetow, 2007

9. Subjective tinnitus idiopathic sensory neural central • Objective tinnitus vascular muscular

10. Some outer and middle ear pathologies associated with tinnitus cholesteotoma mastoiditis otosclerosis otitis media impacted cerumen allergies palatal myoclonus head/ear trauma patulous eustachian tube glomus jugulare tumor abnormal middle ear resonance

11. Some inner ear pathologies associated with tinnitus acoustic trauma presbycusis noise exposure meniere’s disease labyrinthitis acoustic neuroma head/ear trauma ototoxicity meningitis perilymph fistula autoimmune inner ear disease vestibular schwannoma sudden hearing loss

12. Some central auditory nervous system pathologies associated with tinnitus • vascular • dementia • cardiovascular disease/hypertension • blood disease /anemia • multiple sclerosis

13. Other factors associated with tinnitus • temporomandibular disorders • cervical misalignment • menses/menopause • renal disease / Alport’s / kidney transplants • lyme disease • zinc deficiency • poor circulation • Hypothyroid/ hyperthyroid disorders

14. Results Summary • Tinnitus Percept Laterality is captured by Model 2 ( maximum difference for two consecutive frequencies) for 63% cases. • Tinnitus Percept Severity is uncorrelated to absolute or relative hearing loss. Tsai, Cheung, and Sweetow, 2007

15. Models based on peripheral measures fail to predict completely percept laterality • Role of CNS adaptation (neural plasticity) • Absolute and relative depth of hearing loss are uncorrelated to percept severity • Role of CNS emotional binding (limbic system) Tsai, Cheung, and Sweetow, 2007

16. Mechanisms & Etiologies • Para-auditory structures (Tyler,1993) • Body continually produces noise that can be transmitted to the base of the skull. • Usually these noises are inaudible but the patient becomes aware of these noises for a variety of reasons • Vascular neoplasms, arteriovenous malformations, venous hum, TMJ syndrome, and muscle contraction tinnitus.

17. Para-auditory generation • Vascular neoplasms • Cochlea detects blood flow • Potentially serious prognosis--symptom of vascular disease • Management--generally surgical

18. Para-auditory generation • Arteriovenous malformations • Developmental abnormalities--microscopic channels may canalize rapidly and result in rapid enlargement with increasing tinnitus • Often characterized by pulsatile tinnitus and may include distortion of the face or neck and discoloration of the skin. • Pulsatile rate increases with increased heart rate

19. Para-auditory Generation • Venous hum • Low pitched hum arising from the neck of many children and some adults, particularly young women. • Has been attributed to the transverse process of the second cervical vertebra impinging on the jugular vein. • Turning head away from involved side decreases it

20. Para-auditory generation • Venous Hum • Benign Intracranial Hypertension • Obesity • Menstrual irregularities • Hypothyroidism • Hyperthyroidism • Anemia • Vitamin A or D Deficiency • Side Effect of Oral Contraceptives • Pregnancy and Postpartum

21. Para-auditory Generation • TMJ syndrome • The petro-tympanic fissure is traversed by the mandibular ligament (Pinto’s ligament), the chorda tympani, and the anterior tympanic artery...disruption of these structures with a change or misalignment of the jaw can cause tinnitus and other ear symptoms • Diagnosis • Management

22. Para-auditory Generation • Muscle contraction tinnitus • Synchronous contraction of many or most of the fibers of one or more middle ear or palatal muscles (palatal myoclonus), either voluntary or involuntary • Pulsatile (clicking) tinnitus that does not change with changes in heart rate during exercise • Management (botox injections)

23. Diagnostic Issues in Tinnitus:a Neuro-otological Perspective Robert Aaron Levine, MD

24. Tinnitus - unilateral • Coarse intermittent sounds coincident with jaw or head movements • Typical of a foreign body (i.e. cerumen, water or other liquids, or a hair resting against the tympanic membrane) • Fluttering

25. Fluttering • Stapedius muscle contractions • If associated with facial movements, then stapedial contractions are likely cause • Commonly seen after recovery from Bell’s palsy • When affected side of face contracts, ipsilateral stapedius muscle also contracts (synkinesis) due to aberrant facial nerve regeneration.

26. Tinnitus - unilateral or non-lateralized • Pulsatile • Clicking • Autophony (echoing of the voice), or blowing tinnitus • Hallucinations (non-verbal, stereotyped repetitive)

27. Pulsatile • Determine whether it is related to cardiac cycle by comparing your silent count of patient’s cardiac pulse while patient is silently counting the pulsations of his/her tinnitus

28. Pulsatile - history • An association with headaches, blurring of vision, and menstrual irregularities in an obese woman is suspicious for benign intracranial hypertension. • Abrupt onset with unilateral neck or head pains suggests a carotid dissection. • Changes in tinnitus intensity with head turning suggests a venous source for the tinnitus – from a source ipsilateral to the direction that decreases the tinnitus • If the patient can obliterate the tinnitus with localized pressure in the periauricular region then an emissary vein is probably accounting for the tinnitus. • An associated fluctuating hearing loss raises the possibility of microvascular compression of the auditory nerve causing the pulsatile tinnitus. (Ohashi, Yasumura et al., 1992; Waldvogel, Mattle et al., 1998)

29. Pulsatile • MRI studies of patients with unilateral tinnitus can detect vascular compression of the auditory nerve on the asymptomatic side as frequently as on the symptomatic side (Makins, Nikolopoulos et al., 1998)

30. Pulsatile – physical exam • A crescent purple coloration to the tympanic membrane is diagnostic of a glomus jugulare tumor. • Otoscopic observation of a red mass behind the tympanic membrane is suggestive of an aberrant carotid artery, dehiscent jugular bulb, or vascular tumor. • A unilateral conductive hearing loss in association with ipsilateral pulsatile tinnitus and an otherwise normal exam may suggest otosclerosis, as does Schwartze’s sign (red hue behind the tympanic membrane on otoscopy). • Detection of a bruit ipsilateral to the pulsatile tinnitus suggests that the tinnitus is from the same source as the bruit. The source of the bruit then must be sought.

31. Pulsatile – physical exam • Obliteration or reduction in the intensity of the pulsatile tinnitus with ipsilateral jugular compression (light or moderate pressure below the angle of the jaw) implicates a venous source of the tinnitus; whereas a decrease in the tinnitus with ipsilateral carotid compression implicates an arterial source arising from the carotid system.

32. Pulsatile - Diagnostic studies • Because high cardiac output states such as anemia or hyperthyroidism can cause pulsatile tinnitus (usually bilateral), all patients should have a thyroid profile and a hematocrit. • If a carotid lesion is suspected then either a duplex ultrasound study of the carotid or MRA should be performed. • If a retrotympanic mass is suspected, then a high-resolution contrast-enhanced CT scan of the temporal bones should be obtained. Otherwise, a contrast-enhanced MRI scan of the temporal bone and cranium should be obtained. • If all non-invasive imaging studies have been unremarkable and raised intracranial pressure has been ruled out, then cerebral angiography should be considered, because a dural arteriovenous malformation can sometimes go undetected by another diagnostic study. • Because significant, but rare, morbidity can occur with angiography, careful deliberation must be given to the decision to proceed with angiography.

33. Clicking • Relatively rare • Appears to be due to contractions of tensor tympani or the nasopharyngeal muscles controlling the patency of the Eustachian tube • It can sometimes be bilateral, in which case, it is usually associated with palatal myoclonus. • Often the clicking can be heard by the examiner • Sometimes acoustic impedance measurements have abnormalities that correspond

34. Autophony (echoing of the voice), or blowing tinnitus • Patulous ipsilateral Eustachian tube • Confirmatory features include disappearance of their complaints when their head is in a dependent position and abnormally large changes in the tympanic membrane acoustic impedance with respirations.

35. Musical hallucinations • Occur in elderly patients (more commonly in women) with a longstanding progressive moderate to severe bilateral hearing loss. • The tunes can be vocal and/or instrumental. • While usually bilateral, can be unilateral even with a bilateral hearing loss. • Can be precipitated by new medication, and resolved when medication is stopped.

36. Auditory hallucinations • Differ from musical hallucinations in several respects • Usually abrupt in onset and associated with focal neurological findings due to a brainstem stroke or space occupying lesion • Brain imaging is required

37. Tinnitus quality • 85% of subjects reported having experienced another type of “normal” tinnitus; namely transient (typically less than a minute). • Transient tinnitus follows exposure to loud sound. • About 55% of population report such tinnitus lasting minutes to days.

38. Always unilateral, may be with vestibular symptoms • Meniere’s Syndrome • Perilymphatic fistula • Herpes Zoster Oticus (Ramsay-Hunt Syndrome) • Cerebellopontine angle tumors • Central nervous system – caudal to trapezoid body • Sudden idiopathic hearing loss

39. Meniere’s Syndrome • While the tinnitus is often described as roaring early in the illness, with more advanced stages of the syndrome, the tinnitus tends to become more variable in its description.

40. Perilymphatic fistula • Tends to be high frequency (hissing, crickets, etc.) with no recovery. • The defect can be caused by barotrauma, head trauma, valsalva, or erosion of the bony labyrinth due to an inflammatory or neoplastic process, or following middle ear surgery, such as stapedectomy. • Diagnosis can be suggested by the “fistula test” – the induction of nystagmus by positive or negative pressure applied to the external auditory canal.

41. Herpes Zoster Oticus (Ramsay-Hunt Syndrome) • Intense ear pain followed by ipsilateral tinnitus, hearing loss, vertigo and facial paralysis

42. Sudden idiopathic hearing loss • Always unilateral

43. Always with hearing loss,may be unilateral or non-lateralized • Acute acoustic trauma • Chronic progressive hearing loss (presbycusis, chronic acoustic trauma, hereditary hearing loss) • Autoimmune inner ear disease

44. May be no hearing loss, may be unilateral, or non-lateralized • Somatic (head or upper cervical) • Trauma • Post-infectious • Medication-related (including withdrawal syndromes)

45. Somatic (head or upper cervical) • Higher incidence of tinnitus in normal hearing subjects with temporomandibular joint syndrome than in controls (Chole and Parker, 1992) • The same is true regarding whiplash (Tjell, Tenenbaum et al., 1999) • Tinnitus temporally associated with unilateral somatic disorders are localized to the ipsilateral ear (Levine, 1999a) • The physical examination should include: • Inspection of the teeth for evidence of bruxism, such as excessive wear of the bottom incisors • Palpation of the head and neck musculature for tender muscles under increased tension • Forceful systematic isometric concentration of muscle groups of the head and neck for their effects upon the patient’s tinnitus

46. Properties suggesting somatic component • Intermittency • Large fluctuations in loudness • Variability of location • Diurnal pattern • No hearing loss but head or neck trauma

47. Somatic – Diurnal fluctuations • Suggest somatic modulation is operative • Tinnitus louder upon awakening raises the possibility that somatic factors (such as bruxism) are active during sleep and are causing an increase in tinnitus loudness. • Tinnitus vanished by awakening and then returning a few hours into the day suggests that during the day they are re-activating their tinnitus through somatic mechanisms, such as the tonic muscle contractions required to support the head in an upright position or clenching related to the stress of daily activities. • Tinnitus that is louder after awakening from a nap in a chair may related to somatic factors such as stretching of the neck muscles when their head passively falls forward while dozing in a sitting position.

48. Otoacoustic emissions • While spontaneous emissions are common (75% of female and 45% of male normal or near normal ears), tinnitus due to spontaneous otoacoustic emissions is uncommon. • said to account for tinnitus of 1-2% of the patients of one British tinnitus clinic • The diagnosis is made by measuring an emission and showing that its suppression abolishes the tinnitus. • The emission can be suppressed in either of two ways: • Presentation of a low-level tone near the emission frequency • The use of aspirin (Penner and Coles, 1992)

49. SOAE-Associated Tinnitus • Accounts for 1-9 % of cases • May be hum or tonal • Generally in younger patients and localized in better ear • Responds to drug induced suppression • Case

50. Sensorineural Generation • Auditory deprivation and conductive hearing loss • SOAE-associated tinnitus • Cochlear disorders (SNHL) • Central processing effects