Dizziness

Question 1

Definite Meniere’s Disease - Definition

Answer 1

1) Two or more spontaneous vertigo episodes, lasting 20 minutes to 12 hours
2) Audiometrically documented low-mid frequency Hearing Loss* in one ear on one occasion, before/during/after one episode of vertigo
3) Fluctuating aural symptoms (hearing, tinnitus, or fullness) in the affected ear
4) Not better accounted for by another vestibular diagnosis

• > 30 dB in 2 contiguous frequencies below 2000Hz

Question 2

Probable Meniere’s Disease - Definition

Answer 2

1) Two or more spontaneous vertigo episodes, lasting 20 minutes to 24 hours
2) Fluctuating aural symptoms (hearing, tinnitus, or fullness) in the affected ear
3) Not better accounted for by another vestibular diagnosis

Question 3

Management of Meniere’s Disease

Answer 3

Reaching the ‘good enough’ point with shared decision making
1) Medical therapy (90% of patients are managed medically):
- Diet
- Diuretics
- Betahistine
- Steroids (Injectable)
- Migraine meds
- Chemical labyrinthectomy
2) Surgical Therapy (10% of patient’s are managed surgically):
- Endolymphatic sac surgery
- Vestibular nerve section
- Surgical labyrinthectomy

Question 4

Meniere’s Disease Overview

Answer 4

1) Named after French physician, Prosper Meniere, who in 1861 highlighted that vertigo originated from inner ear damage
2) Theory of Reissner’s membrane
- Leading theory that MD occurs from endolymphatic duct distention secondary to Reissner’s membrane rupture
- Microtears increase exposure to harmful levels of K+ rich endolymph to surround cochlear apparatus and CN VIII
- Endolymphatic hydrops (ELH) pathologically confirmed post-mortem and associated with hearing loss > 40dB
3) Meniere’s Disease (primary ELH producing classic triad of symptoms) vs Meniere’s Syndrome (ELH secondary to underlying otologic disease)
4) Diagnosis: diagnosis is clinical and is based on patient-reported symptoms and audiometric data often made over a period of years to decades
- Formal vestibular function testing or ECochG not needed to establish the diagnosis of MD

Question 5

Epidemiology of Meniere’s Disease

Answer 5

1) Age:
- Most prevalent age 40-60 years with peak onset 40-50
- MS is seen in children with congenital malformations of the inner ear
- Increased risk with age
- Almost exclusively occurs in adults with < 3% occurring in patient’s < 18 years old
2) Gender:
- Male = Female
- Some studies say F>M due to inner ear autoimmune pathologies
3) Incidence: 8.2/100,000 adults
4) Prevalence:
- 50-200 affected per 100,000 adults
- Lack of widely accepted criteria explains wide range

Question 6

Meniere’s Disease - Associations

Answer 6

1) There is an increased association in patient’s suffering from migraine HA and autoimmune conditions
- Ankylosing spondylitis, SLE, and RA
2) Bilateral Meniere’s disease
- Simultaneous bilateral presentation extremely rare
- Disease progression to bilateral MD is more common and often seen after 2 decades of disease onset
- One etiology supports immunologic mechanism of autoantibodies to endolymphatic sac

Question 7

Definition of Definite Meniere’s Disease

Answer 7

1) >/= Two spontaneous episodes of vertigo lasting 20 minutes to 12 hours
2) Low to mid-frequency SNHL supported by audiometry in the affected ear before, during, or after an episode of vertigo
3) Additional fluctuating aural symptoms in the affected ear (i.e. fullness, tinnitus)
4) Other etiologies excluded

Question 8

Definition of Probable Meniere’s Disease.

Answer 8

1) >/= Two spontaneous episodes of vertigo lasting 20 minutes to 24 hours
2) Lacks audiometric support required for definite MD
3) Additional fluctuating aural symptoms in the affected ear (i.e. fullness, tinnitus)
4) Other etiologies excluded

Question 9

Meniere’s Disease Staging

Answer 9

1) Active MD: MD attacks weekly or monthly
2) Chronic MD: MD attacks few times annually, good control
3) Burned Out MD: Severe to profound HL with minimal function from diseased ear

Question 10

Severity of Meniere’s Disease

Answer 10

1) Mild MD: Occasional attacks, episodes lasting minutes
2) Moderate MD: Occasional attacks with few debilitating episodes
3) Severe MD: Frequent attacks, often debilitating

Question 11

Failure of Meniere’s Disease Treatment

Answer 11

1) Conservative Treatment: Poor response to triggers (Salt, Diet)
2) Medical Management: Failed response to diuretics, betahistine, steroids, etc.)
3) Surgical Management: Refractory to destructive therapies (surgical labyrinthectomy, vestibular neurectomy)

Question 12

What is the Differential Diagnosis of Meniere’s Disease?

Answer 12

1) Autoimmune etiologies
2) Basilar migraine
3) Vestibular migraine
4) BPPV
5) Ischemia/Stroke
6) Infectious etiologies
7) Labyrinthitis
8) Medication toxicity (aminoglycosides, loop diuretics)
9) Otosyphilis
10) Vestibular Neuritis
11) Vestibular Schwannoma

Question 13

Presentation of Meniere’s Disease

Answer 13

1) Natural course of MD is progressive and unpredictable
2) Important to differentiate vertigo from vestibular disturbance or vague dizziness
- Vertigo: False sensation of self-motion with surroundings that seem to spin or flow; Rotational in nature
- Dizziness: impaired spatial orientation without distortion in motion

Question 14

You should differentiate between central, peripheral, and cardiovascular etiologies of vertigo

Answer 14

1) Central origin of vertigo with neurologic signs, new onset headache, nystagmus, and acute deafness raise concerns.
2) Key Questions:
- Overlapping otologic symptoms prior, during and after the attack
- Duration of active vertigo
- Triggers (i.e. light sensitivity, motion sickness)
- Spontaneous or provoked onset
- Aggravated or alleviated by head position
- Falls
- Otologic history (prior ear surgery, otorrhea/chronic ear infections, otalgia, facial numbness, CHL/SNHL
- Medication history
- Family and social history

Question 15

What is serviceable hearing?

Answer 15

Levels of adequate hearing perception often defined by the patient: may be audiometrically defined based on level of hearing loss (HL), pure tone average (PTA), and word recognition/discrimination scores (WRS) from the vestibular schwannoma literature:
1) AAO-HNS Scale:
- Class A: Discrimination 70-100%; PTA < 30 dB
- Class B: Discrimination 50-69%; PTA 31-50 dB
- Class C: Discrimination 50-69%; PTA > 50 dB
- Class D: Discrimination < 50%; any PTA
2) Most clinicians consider Class A and B/C to be useable/serviceable hearing
- Class D not considered serviceable hearing

Question 16

What testing should be done for suspected Meniere’s Disease?

Answer 16

1) Full otologic examination:
- Facial nerve testing
- Weber and Rinne (SNHL)
- Vestibular testing: Dix-Hallpike, Head-thrust
2) Audiogram
- Unilateral HL should undergo MRI of the IACs to r/o retrocochlear disease processes; high resolution MRI may visualize endolymph hydrops
3) Video or electronystagmogram
4) Electrocochleography
5) Brainstem Evoked Response Audiometry
6) Vestibular evoked myogenic potentials (cVEMPs, oVEMPs)

Question 17

Treatment of Meniere’s Disease - what are the conservative/noninvasive options?

Answer 17

Conservative/Noninvasive:
1) Dietary/Lifestyle:
- Sodium restriction (1500-2300mg/day per AHA)
- Limit EtOH
- Limit Caffeine consumption
- Stress reduction (higher levels of vasopressin in plasma of MD patients)

Trigger Management:
1) Vestibular rehabilitation for chronic imbalance - Grade A
- Not recommended for acute vertigo

Hearing aids or amplification technology

Recommendation against positive pressure devices (i.e. Meniette - Grade B)

Question 18

Treatment of Meniere’s Disease - what are the options?

Answer 18

Non-Destructive:
1) Medical
- Oral: Diuretics, Betahistine, Steroids, Vestibular suppressants and antiemetics: antihistamines, benzodiazepines, anticholinergics)
- Intratympanic: Dexamethasone, Methylprednisolone
2) Surgical
- Endolymphatic sac surgery - Hearing sparing: endolymphatic sac incision, endolymphatic subarachnoid shunting, endolymphatic mastoid shunting and endolymphatic decompression

Destructive:
1) Medical:
- Intratympanic gentamycin
2) Surgical:
- Vestibular nerve section - hearing sparing
- Labyrinthectomy - ablative approach in non-serviceable hearing

Question 19

Meniere’s Disease - Goals of Treatment

Answer 19

1) No clear standardization exists in clinically defining, diagnosing and managing Meniere’s disease
2) Aim of treatment of MD is not curative, but to improve quality of life
3) Audiogram plays a pivotal role in initial work-up after a thorough history and physical exam
4) Treatments range from conservative to medical and surgical non-destructive and destructive options

Question 20

What is the pathophysiology of Meniere’s Disease?

Answer 20

1) Endolymphatic hydrops was observed in temporal bone specimens in Meniere’s patients
2) Obliteration of endolymphatic duct in guinea pig produced hydrops and leads to hearing loss
Source: Kimura and Schuknecht, 1965

Question 21

What is the Genetic makeup of Meniere’s Disease?

Answer 21

1) Meniere’s Disease does run in some families
2) Whole exome sequencing studies have identified several genes in autosomal dominant familial MD: COCH, FAM136A, DTNA, PRKCB, DPT, SEMA3D
Sources: Martin-Sierra et al, 2016 & 2017, Requena et al, 2015

Question 22

How do allergies affect patients with Meniere’s Disease?

Answer 22

1) Exposure of environmental antigen can lead to endolymphatic hydrops
- Takeda et al, 2012
2) Allergy treatment can lead to improvement in Meniere’s symptoms
- Weinreich and Agrawal, 2014

Question 23

What are the diagnostic criteria for Meniere’s Disease?

Answer 23

1) Definite MD
- Two or more spontaneous episodes of vertigo, each lasting 20 minutes to 12 hours
- Audiometrically documented low-to medium frequency sensorineural hearing loss in one ear, defining the affected ear on at least one occasion before, during, or after one of the episodes of vertigo
- Fluctuating aural symptoms (hearing, tinnitus, or fullness) in the affected ear
- Not better accounted for by another vestibular diagnosis
2) Probable MD
- Two or more episodes of vertigo or dizziness, each lasting 20 minutes to 24 hours
- Fluctuating aural symptoms (hearing, tinnitus, or fullness) in the affected ear
- Not better accounted for by another vestibular diagnosis

Question 24

What is the differential diagnosis of Meniere’s disease?

Answer 24

1) Autosomal dominant sensorineural hearing loss type 9 (DFNA9) caused by COCH gene
2) Autosomal dominant SNHL type 6/14 (DFNA6/14) caused by WSF1 gene
3) Autoimmune inner ear disease
4) Cerebrovascular disease (stroke/TIA in the vertebrobasilar system/bleeding)
5) Cogan’s syndrome - some cases have recurrences
6) Endolymphatic sac tumor
7) Meningiomas and other masses of the CP angle
8) Neurofibromatosis
9) Otosyphilis
10) Susac syndrome
11) Third window syndromes (Perilymph fistula, canal dehiscence, enlarged vestibular aqueduct)
12) Vestibular migraine
13) Vestibular paroxysmia (neurovascular compression syndrome)
14) Vestibular schwannoma
15) Vogt-Koyanagi-Harada syndrome

Question 25

What is the clinical course of Meniere’s disease?

Answer 25

1) Meniere’s disease can present in phases with vertigo being the most common initial symptom
2) The classic triad includes
- Fluctuating hearing loss
- Vertigo
- Tinnitus
- Aural fullness is common
3) Less than 30% of patients present with the classic triad at once. It is not unusual to experience latency from the onset of auditory symptoms to full blown disease process

Question 26

Meniere’s Disease - what is the clinical course of the auditory symptoms?

Answer 26

1) Hearing loss typically affects the low frequency first
2) Tinnitus may become louder over time and eventually become permanent
3) An increase in tinnitus and aural fullness may be associated with vertigo episodes
4) An association between hearing loss and vertigo episode is sometimes reported
5) Lermoyez syndrome: a rare Meniere’s disease variant where during a vertigo crisis, the patient reports hearing improvement

Question 27

Meneire’s Disease - what is the clinical course of the vestibular symptoms?

Answer 27

1) Vertigo is defined as the sensation of self-motion when no self-motion is occurring. Episodic dizziness and unsteadiness are not considered as criteria for definite Meniere’s disease.
2) Dietary triggers of vertigo are sometimes reported
3) Vertigo crises usually persist, and then decrease with remission periods up to months/years
4) In the final phase of the disease, most Meniere’s patient’s experience imbalance instead of overt vertigo attacks.
5) Drop attacks (Tumarkin crisis): acute episodes of sudden collapse without loss of consciousness. More commonly observed in bilateral Meniere’s disease.

Question 28

What is the Prognosis of Meniere’s Disease?

Answer 28

1) Hearing loss and tinnitus can become permanent over time
2) The long-term prognosis of vertigo attacks is good:
- Green et al (1991) showed that 50% of patients have complete resolution, and 28% have partial resolution
- Uemura et all (1977) showed 74% of patients have resolution of vertigo
3) Patients may experience imbalance instead of vertigo as the disease advances

Question 29

What are the challenges associated with making the diagnosis of Meniere’s disease?

Answer 29

1) Clinical heterogeneity of Meniere’s disease presentation: some patients present with auditory symptoms initially, whereas others present with vestibular symptoms first
2) Difficulty in establishing a diagnosis: multiple disease entities with similar clinical presentations may lead to diagnostic delay
3) Lack of diagnostic tests which can monitor the disease process in real time (biomarkers, imaging modalities)

Question 30

What are the symptoms of Meniere’s disease?

Answer 30

Constellation of four symptoms:
1) Vertigo: 20 minutes to 2 hours
2) Low frequency tinnitus typically described as humming
3) Hearing loss
4) Aural fullness

Question 31

What studies can assist in the diagnosis of Meniere’s disease?

Answer 31

1) Audiogram: pure tone and speech
2) Videonystagmography (VNG) typically not necessary
- Gives information on hypofunction and/or info on compensation
3) Rotary chair information on state of compensation
4) Electrocochleography (ECoG): SP/AP ratio indicating hyrdrops

Question 32

What are the AAO-HNSF 1995 Diagnostic Criteria for Meniere’s disease?

Answer 32

1) Possible MD: episodic vertigo w/o documented HL or SNHL w/disequilibrium but w/o episodes
2) Probable MD: One definitive vertigo episode & audiometrically documented HL
3) Definite MD: > 2 definitive spontaneous vertigo > 20 minutes with audiometrically documented HL, tinnitus, aural fullness
4) Certain MD: definitive & histopathologic confirmation

Question 33

What is the differential diagnosis of Meniere’s symptoms?

Answer 33

1) Episodic dizziness/vertigo
2) Migraine vestibulopathy including cases with overlap, typically no hearing loss
3) Meniere’s syndrome
- Endolymphatic sac neoplasm
4) BPPV
- Lasts seconds to minutes
- Positional
- No hearing loss
5) Retrocochlear pathologies

Question 34

Vestibular Migraines

Answer 34

1) Periods typically days/weeks
2) Aural symptoms may occur in VM
- Tinnitus
- Aural fullness
3) Can have mild hearing loss - HL should definitely not progress
4) Motion intolerance
- Personal and family h/o migraines
5) > 50% Comorbid psychiatric disorders
6) Response to medications is not a diagnostic criterion

Question 35

Diagnosis of Meniere’s disease

Answer 35

1) Complex diagnostic process
2) There is often a level of uncertainty, especially early on
3) Utilize
- H&P
- Audiogram
- MRI
- Consider VNG, ECochG

Question 36

Treatment algorithm for Meniere’s disease

Answer 36

1) Step One
- Lifestyle counseling
- Low salt diet
- Betahistine
- Diuretics
2) Step Two - if step one ineffective:
- Local conservative medical tx: intratympanic steroid injections
3) Step Three - if step two ineffective:
- Useful Hearing present: 1) Conservative surgical tx (endolymphatic sac surgery); 2) Ablative surgical tx (vestibular nerve section)
- No useful hearing present: 1) Local ablative medical treatment (intratympanic gentamycin injections); 2) Ablative surgical treatment (vestibular nerve section, labynthectomy +/- cochlear implant)

Question 37

Which treatments are used for treating Meniere’s disease?

Answer 37

Most common procedures performed:
1) Intratympanic steroid or gentamicin injections (90%)
2) Endolymphatic sac decompression (8%)
3) Transmastoid labyrinthectomy (2%)
4) Vestibular nerve section (0.4%)

Question 38

If a Meniere’s patient has failed diet/diuretic therapy and has continued attacks and serviceable hearing, what would be the reasonable next treatment options?

Answer 38

1) IT steroids are a reasonable option
2) IT Gentamicin would be reasonable if the patient is willing to accept the prolonged recovery (compared to IT steroids) and slightly higher risk of hearing loss, in exchange for the most definitive in-office option
3) Endolymphatic sac decompression is a consideration, but lower on the list given conflicting reports of benefit and higher potential risks.

Question 39

What should you do for a MD patient who has failed diet and diuretic treatment, but who has continued attacks and non-serviceable hearing?

Answer 39

As with all MD treatments, there is a significant role for patient preference given the generally poor data:
1) IT Gentamicin is the most definitive in-office option and does not require surgery
2) Labyrinthectomy (with consideration of cochlear implant) is the most definitive option. The patient must accept the loss of acoustical hearing.

Question 40

What are the goals for the treatment of Meniere’s Disease?

Answer 40

1) To prevent or at least reduce the severity and frequency of vertigo attacks
2) To relieve or prevent hearing loss, tinnitus, and aural fullness and improve overall quality of life

Question 41

What are the treatments for MD from simple to most invasive to include non-ablative and ablative options?

Answer 41

1) Traditional treatment approaches for Meniere’s disease include:
- Dietary/lifestyle and/or trigger management approaches
- Medical, surgical, complementary/alternative, allergy, immunomodulatory
- Vestibular, and aural therapy; and oral IT medications
- All of the above have variable results
2) For those MD patients with persistent, disabling attacks after failing conservative therapy, other more invasive or involved treatments can be considered
3) One of the main considerations in choosing a form or treatment is the status of the patient’s residual hearing

Question 42

Treatment options for MD and hearing loss

Answer 42

1) One main consideration about the choice of treatment is the hearing status and whether it is usable or not
- Patients with usable hearing, nonablative procedures have been advocated: 1) IT steroids, 2) Endolymphatic sac decompression
- Patients with no meaningful/useful hearing, surgical or chemical inner ear ablative treatments are often implemented: 1) IT Gentamycin, 2) Vestibular nerve section, 3) Labyrinthectomy
2) The rational for ablative approaches is to attempt to convert a dynamic fluctuating inner ear lesion to a static state through destruction of the inner ear

Question 43

Abortive Medical Treatment for Meniere’s Disease

Answer 43

1) Vestibular suppressants
2) Vestibular suppressants primarily appear to act by suppressing central vestibular neural activity at the level of the brainstem and concomitantly suppressing nausea
3) These medications should be used only to suppress acute vertiginous events
4) Chronic use of these drugs is undesirable, as these agents can suppress central adaptation/compensation to vestibular loss and can thus perpetuate symptoms of chronic imbalance
- First-Generation antihistamines
- Benzodiazepines
- Anticholinergics

Question 44

Abortive medical treatment for MD attacks - Antihistamines

Answer 44

1) Antihistamines
- First generation antihistamines cross the blood brain barrier
- They bind to several neurotransmitter receptors, including histamine and muscarinic acetylcholine receptors
- They suppress a variety of symptoms, including vertigo and nausea
2) Examples:
- Dimenhyrdrinate (25-50mg Q6 hours)
- Meclizine (12.5-25mg Q8 hours)
- Diphenhydramine (25-50mg Q6 hours)

Question 45

Abortive medical treatment for MD attacks - Antihistamines

Answer 45

1) Antihistamines
- First generation antihistamines cross the blood brain barrier
- They bind to several neurotransmitter receptors, including histamine and muscarinic acetylcholine receptors
- They suppress a variety of symptoms, including vertigo and nausea
2) Examples:
- Dimenhyrdrinate (25-50mg Q6 hours)
- Meclizine (12.5-25mg Q8 hours)
- Diphenhydramine (25-50mg Q6 hjours)

Question 46

Abortive Medical Treatment - Benzodiazepines

Answer 46

1) Benzodiazepines are gamma aminobutyric acid (GABA) receptor agonists, are also effective at suppressing vertigo, and can thus secondarily mitigate vertigo-associated nausea
2) Benzodiazepines carry significant risk for drug dependence
3) Examples:
- Diazepam (2=10mg Q8 hours)
- Lorazepam (1-2 mg Q8 hours) due to its rapid onset of action and shorter duration
- Clonazepam (0.5-1.0 mg Q8 hours)

Question 47

Abortive Medical Treatment - Anticholinergic Drugs

Answer 47

1) Anticholinergics block muscarinic receptors and can suppress acute vertigo attacks
2) Side effects:
- Blurred vision
- Dry mouth
- dilated pupils
- Urinary retention
- Sedation
3) Because of their side-effect profile and potential for significant toxicity and withdrawal effects when used for more than several days, they are not commonly prescribed for acute vertigo control associated with Meniere’s disease
4) Examples:
- Scopalamine
- Glycopyrrolate

Question 48

Meniere’s Disease - what are the recommended lifestyle changes for the treatment of Meniere’s disease?

Answer 48

Triggers
1) Dietary sodium:
- No specific guideline exists that can recommend a specific daily sodium intake to prevent Meniere’s disease attacks
- Americal Heart Association recommends no more than 2300 mg of sodium/day and an ideal limit of no more than 1500 mg/day for most adults (Cogswell ME 2012)
- Most utilize the AHA’s endorsement as a reasonable parameter of a sodium-restricted diet in adults
2) Allergic triggers
3) Stress

Question 49

Maintenance Medications in the treatment of Meniere’s disease - Diuretics

Answer 49

1) Diuretics are believed to alter the electrolyte balance in endolymph, subsequently reducing endolymph volume
2) Thiazides: HCTZ, Chlorthalidone, and Indapamide
- Thiazides are contraindicated in patients with Gout
3) Potassium sparing: Triamterine, Spironolactone
- Potassium-sparing diuretics are contraindicated in patients with acute or severe renal failure
4) Cochrane review:
- Due to a lack of high-quality studies, Cochrane reviews have determined that the effect of diuretics on the frequency and severity of attacks effects in Meniere’s disease could not be rigorously evaluated
- Some studies in the Cochrane review did report improvement in patients’ vertigo with the use of diuretics

Question 50

Maintenance Medications for the treatment of Meniere’s disease - Betahistine dihyrochloride

Answer 50

1) Side Effects: Headache, Imbalance, Nausea, Feeling hot, eye irritation, palpitations, upper GI symptoms
2) Cautions: Should be used with caution in patients with asthma and h/o peptic ulcer disease; avoid in patients with pheochromocytoma

Question 51

Meniett device - should this be used in the treatment of Meniere’s disease?

Answer 51

1) Positive pressure therapy: a PE tube is placed in the ear drum which allows micropulses of pressure to enter the middle ear space, the pressure is transferred to the inner ear, resulting in a displacement of the excess inner ear fluid (endolymph), theoretically resulting in ‘normal’ inner ear pressure.
2) The AAO-HNS clinical practice guideline for Meniere’s disease recommends against positive pressure therapy. Based on a systematic review and randomized trials showing ineffectiveness of devices like the Meniett device.

Question 52

Intratympanic (IT) Steroid Injection - effectiveness in Meniere’s disease.