Ménière’s DiseaseSam Maleki, Jordan Braun, Alex Wohl, Rob Whittaker

Etiology

Female Caucasians most prone to disorder. 157/100k in England

46/100k in France

Peak incidence 40-60 y.o. (1.3:1 female to male ratio) 2-50% of symptoms arise in opposite ear

Prevalence rates caused by differences in environment, genetics, or diagnostic criteria is unclear

Familial occurrence reported in 10-20% cases (autosomal dominant mode of inheritance)

Genetics – human leukocyte antigens B8/DR3 & Cw7 have been associated

Anatomy/Physiology

Vestibular System Detects forces from gravity & movement, maintains clear vision

during head motion (VOR) by head positioning

Semicircular canals – ring-shaped, fluid filled (endolymph) oriented in 3D provides sensory input to velocity & angular acceleration (ampulla deflected away from direction of head movement by endolymph)

Speed & direction of deflection of hair cells of ampulla determines the rate of firing of the vestibular nerve

Ends of semicircular duct open into otolith (utricle & saccule) – contained hair cells covered in otolithic membrane (otoconia produce shear force)

Signals carried by vestibular nerve - If lesion in vestibular nerve, brain can possibly adapt from intact opposite nerve & recalibrate

Motor output through vesibulospinal reflexes (VSRs) – automatic control of postural muscles in trunk & limbs

Anatomy/Physiology Cont’d

Audition Tympanic membrane Ossicles cochlea via oval windows

Scala vestibula & scala tympani (perilymph), Scala media (endolymph)

Pressure waves travels through scala vestibuli, helicotrema, & scala tympani pressure changes onto basilar membrane & into Organ of Corti exits round window at end of scala tympani

Inner ear (cochlea – fluid filled tube dived by organ of Corti)

Fluid incompressible & bony wall rigid, important to maintain fluid volume

Sound through ossicles oval window scala vestibuli (perilymph) scala tympani round window

Endolymph in scala media

Pathology

Endolymphatic hydrops – over accumulation of endolymph compromising perilymphatic space Lack of absorption of endolymph in endolymphatic duct &

fluid backs up into system

Characterized by episodic vertigo; fluctuating, sensorineural hearing loss; sensation of fullness in the ears; & tinnitus

Vertigo most debilitating symptom with intervals of hours to days

Simultaneous hearing deterioration of involved ear

Reduction in responsiveness of involved peripheral vestibular system can occur

Pathology cont’d

Multifactorial causes Fibrosis, atrophy of the sac, obstruction of the

endolymphatic duct, infection, or the vascularity in the region in the inner ear.

Otosyphilis (involvement of the inner ear in collagen vascular disease)

Immune responses likely within the complex related to allergic reactions & histamine

Viral infection – more susceptible to changes in thyroid, Na+ or hormone dysfunction

Overproduction of endolymph by stria vasularis

Blow to head, a fall, or flexion/extension injury

Pathology cont’d

Pathogenesis of symptoms uncertain

Deficits related to volume/pressure changes within closed fluid system Membranous labyrinth progressively dilate until the wall

makes contact with the stapes footplate & the cochlear duct fills the entire scala vestibula vestibular & cochlear dysfunction

Distension of otoliths can put pressure on the ampulla, creating sensation of spinning that is characteristic of acute unilateral dysfunction

Membrane rupture leak of K+ into endolymph Nerve palsy

Pathology cont’d

Typical attack of hydrops – initial sensation of fullness of the ear, reduction in hearing, & tinnitus Followed by rotational vertigo (30 min – 24 hours), postural

imbalance, nystagmus, & nausea

Permanent loss of hearing over time

Tinnitus is commonly described as low-pitched roaring or seashell like

History

General Questions: Age, date of onset, previous history of falls

Triad of Associated Symptoms: Vertigo

Tinnitus

Fluctuating Hearing Loss

Family History 7-10% affected

Employment: Current work, community, & leisure actions, tasks, or activities

History Cont’d

Functional status & activity level: current/prior functional status in self care/home & in work

Medications

Other clinical tests: lab & diagnostic tests, review of available records, review of other clinical findings

Employment: Current work, community, & leisure actions, tasks, or activities

General health status: general health perception, physical function, psychological function, role function, social function

Other clinical tests: lab & diagnostic tests, review of available records, review of other clinical findings

Systems Review

CV: BP, edema, HR, RR

Integumentary: pliability, scar formation, skin color/integrity

Musculoskeletal: ROM, strength, symmetry, height/weight

Neuromuscular: coordination (balance, gait, locomotion, transfers, transitions), motor function

Cranial Nerve Testing Nystagmus testing

Communication, affect, cognition, language, & learning style Ability to make needs known, consciousness, expected

emotional/behavior responses, learning preferences, orientation (person, place, time)

Global outcomes

Functional Limitations – Nottingham health profile, SF – 12/36, Quality of Well being (self administered), dizziness handicap inventory1

Visual analogue scale (VAS), dizziness handicap inventory (DHI), functional disability scale, motion sensitivity quotient (MSQ)2

Gait, locomotion, & balance Elderly mobility scale, Fugl-Meyer assessment scale, functional

standing test, hop tests, obstacle course, seated postural control measure, TUG, trunk control

Berg balance scale, Romberg Tests, sit to stand tests, tilt board balance tests, Tinetti performance-oriented mobility scale

Functional ambulation profile, gait abnormality rating scale, gait speed, Rivermead visual gait assessment

PT tests

Smooth pursuits (nystagmus)

Saccadic eye movements (look back/forth 2 objects)

VOR (focus on an object while turning head)

Head thrusts (quick passive movements by PT)

Head shaking (pt. actively move head quickly)

Dix-Hallpike maneuver (BPPV test)

Lab (by physician) Caloric (air/water injected - alter temp)

Rotational (Barany test, rotate in chair, watch eyes, balance master)

Special Tests

Dix-hallpike Dx of BPPV http://youtu.be/kEM9p4EX1jk

Sensitivity – 79% [95% CI: 65-94%]

Specificity – 75% [95% CI: 33-100%]

LR+ – 3.14 [95% CI: 0.58-17.58]

LR- – 0.28 [95% CI: 0.11-.0.69]

Sidelying Test Sensitivity – 90% [95% CI: 79-100%]

Specificity – 75% [95% CI: 33-100%]

LR+ – 3.59 [95% CI 0.65-19.67]

LR- – 0.14 [95% CI: 0.04-0.46]

Establishing a Diagnosis of Benign Paroxysmal Positional Vertigo Through the Dix-Hallpike & Side-Lying Maneuvers (2008)

Special Tests Cont’d

Vestibular evoked myogenic potentials (VEMPs) Sensitivity – 50.0%

Specificity – 48.9%

LR+ – 1.04

LR- – 1.00

Caloric Test Sensitivity – 37.7%

Specificity – 51.2%

LR+ – 0.75

LR- – 0.72

No significant difference in hearing level between patients appropriately or inappropriately identified by VEMPs, whereas significant difference in those of the caloric test.

Combined VEMP & caloric test increased sensitivity to 65.8%

The diagnostic value of vestibular evoked myogenic potentials in patients with Meniere’s disease (2013)

Evaluation

Rule out differential diagnosis

Potential referral for diagnosis

Describe frequency & duration of symptoms

Refer to previous slides for other testing

Differential Diagnosis

Pathology Implications for the Physical Therapist Presence of neurologic signs or symptoms such as syncope, visual aura,

& motor weakness suggest another diagnosis

Disorders that present with similar symptoms include: migraine, acoustic neuroma, perilmyphatic fistula, dehiscence of the superior semicircular canal, labyrinthitis, autoimmune inner ear disorder, & MS

Vertigo – a feeling of spinning & loss of balance, caused by disease affecting the inner ear or the vestibular nerve

Migraines – a regular aching/throbbing headache that usually affects one side of the head usually goes along with nausea & troubled vision.

Vestibular Neuronitis – Can be a series/single attack of vertigo or a persistent condition that decreases over 6 weeks

Postural muscle weakness, reflex integrity/peripheral nerve

BPPV

Differential Diagnosis Cont’d

Factors that may differentiate Ménière’s disease from benign recurrent vertigo Based on case-control study of 112 patients with Ménière’s

disease & 63 patients with benign recurrent vertigo

Vertigo attacks with unilateral tinnitus & unilateral hearing loss more likely to be Ménière’s disease in multivariate analysis

Earlier age at onset & shorter duration of vertigo attacks, female preponderance, & presence of migraine more common in benign recurrent vertigo

Differential Diagnosis (Reference)

Other problems to be considered include the following: Trauma, Endocrine abnormalities, Thyroid dysfunction, Hyperlipidemia,

Diabetes, Congenital anomalies, Autoimmune problems/inner ear inflammation,

Differential Diagnosis Anterior Circulation Stroke, AVM, Basilar Artery Thrombosis, Benign

Positional Vertigo in Emergency Medicine, Benign Skull Tumors, Brainstem Gliomas, Cerumen Impaction Removal, Ear Foreign Body Removal in Emergency Medicine, HIV-1 Associated CNS Conditions – Meningitis, Hypothyroidism & Myxedema Coma in Emergency Medicine, Inner Ear Labyrinthitis, Intracranial Hemorrhage, Ischemic Stroke in Emergency Medicine, Lyme Disease, Migraine Headache, Multiple Sclerosis, Neurosyphilis, Otitis Media in Emergency Medicine, Polyarteritis Nodosa, Posterior Cerebral Artery Stroke, Primary Malignant Skull Tumors, Rheumatoid Arthritis, Salicylate Toxicity in Emergency Medicine, Subarachnoid Hemorrhage in Emergency Medicine, Syncope & Related Paroxysmal Spells, Temporal Lobe Epilepsy, Transient Ischemic Attack, Vestibular Neuronitis, Viral Encephalitis, Viral Meningitis, Wernicke Encephalopathy

Diagnosis

Practice Pattern 5A: Primary Prevention/Risk Reduction for Loss of Balance & Falling

ICD-9-CM Code – 386.0

Pathology Implications for the Physical Therapist 2 or more definitive episodes of spontaneous rotation

vertigo lasting at least 20 minutes (nausea & vomiting abates by 24 hours)

Low frequency sensorineural hearing loss documented by audiometry

Tinnitus or aural fullness in the affected ear

Exclusion of other causes for the symptoms

Prognosis

Expected Range of Number of Visits Per Episode of Care

2-18 visits

Range affected by: accessibility/availability of resources, adherence, age, cognitive status, co-morbidities, concurrent medical interventions, level of impairment/physical function, living environment, nutritional status/overall health status, psychological & socioeconomic factors, social support, stability of condition

Prognosis Cont’d

Highly variable, Attacks increase in frequency in first years then decrease

Clusters of attacks may be separated by periods of long remission – balance function between attacks can be normal, although a sense of disequilibrium often persists later in the disorder

2-6% of patients experience “drop attacks” (otolithic crisis of Tumarkin) – abruptly thrown to the ground without LOC & with little/no vertigo

Initially 1 ear – bilateral disease ranges from 2-78% with an average incidence of 45%

If bilateral involvement has not occurred within 5 years of onset of first ear, then unlikely will occur

Hearing loss fluctuating, low-frequency sensorineural loss early becomes irreversible often progressing in severity with higher frequencies & loss of speech discrimination

Problem list/Symptoms

Inner ear condition of vestibular & cochlear systems Recurrent vertigo

Hearing loss & tinnitus in one year

Feeling of pressure differences in ears

Nausea

Balance deficits

Risk of fall

Goals

Patient will reduce the risk of falling through therapeutic exercise, balance training, & lifestyle modification within 4-6 weeks to improve quality of life.

Reduce nystagmus

Improve dizziness

Increase smooth pursuit

Independent HEP

Increase balance

Improvement via functional test (Berg, MiniBEST, ect.)

Surgical Intervention

Cochlear implant Improved hearing reported with cochlear implantation in case

series of 9 patients (mean age 61 years) with Ménière’s disease for at least 10 years & severe sensorineural hearing loss

Vertigo may decrease with/without surgical intervention

Endolymphatic sac surgery does not appear effective for Ménière’s disease

Endolymphatic sac shunt & ventilating tube insertion appear similarly effective both treatments associated with significant reductions in dizzy spells at 6 & 12 months, but no significant differences between groups

Middle ear injections Gentamicin

Steroids

Post-Surgical Timeline

Goal: 30 days before return to work

Physician will assess need for continued interventions, or possible use of medications

PT may be utilized to address any functional limitations Neuromuscular Reeducation

Strengthening

Aerobic Conditioning

PT will continue to monitor for signs/symptoms that indicate referral back to a physician is necessary

Intervention Cont’d

Patient will need referral from Physician PT alone cannot diagnose Ménière’s Disease

Precautions/Contraindications Sudden loss of hearing

Increased feeling of pressure or fullness to discomfort in ears

Severe ringing in ears

Severe increase in symptoms

Severe nausea

Intervention

Addressing required functions, collaboration & coordination with agencies (equipment, payers, home care), communication (education/documentation), data collection/analysis, documentation

Therapeutic exercise – aerobic/endurance training, balance/coordination/agility, body mechanics/postural stabilization, flexibility, gait training Treatment

Diuretics can control vertigo & stabilize hearing in more than ½ of individuals

Restricting salt, caffeine, alcohol, & nicotine (reduces endolymph volume by fluid removal)

Antivertiginous meds, antiemetic, sedatives, antidepressants, & psychiatric treatment

Corticosteroid infusion of the middle ear via a transtympanic route – autoimmune & inflammatory injury

Intratympanic gentamicin used for chronic unrelenting unilateral hydrops

Surgery for endolymphatic decompression

Inpatient/Outpatient Care (nonsurgical) Balance/Vestibular training program progressions

Gaze stabilization exercises

Hip, knee, & ankle strategies

Therapeutic Exercises Aerobic

Strength

Assess Vertigo if needed

Gait analysis

Patient Education Home analysis

HEP

The use of different devices (hearing, AD)

Surgical Intervention

Vestibular nerve section: Selective vestibular nerve section (AKA vestibular

neurectomy)

Goal is to disconnect diseased labyrinth from brainstem while preserving hearing

Complications may include hearing loss, facial nerve injury, CSF leak, & headache

Retrosigmoid approach of vestibular nerve section reported to control vertigo in patients with Meniere's disease

Translabyrinthine vestibular nerve section may be superior to labyrinthectomy for improving balance, but appears to have similar efficacy for vertigo

Home Program

Hearing Aid

Meniette device-positive pressure device administer 3x/day for five min/session. Equalizes pressure in patient with persistent problems

Home assessment

Continue inpatient/outpatient care

Dietary Changes Limit Caffeine & sodium

Lifestyle changes Stop smoking

Manage stress/anxiety

Eat regularly

Patient Education

Who’s affected/prevalence

What causes the disease

How the disease impacts function (hearing/balance?)

Identifying signs/symptoms

Options for treatment (refer/balance training)

HEP

Potential of extra help

Secondary complications in life

Three systems that control balance Somatosensory, visual, vestibular

Discharge

Occurs when anticipated goal & expected outcomes have been achieved Patient has met goals

Improved functional ability

Improved quality of life

Dependent on medical/psychosocial status

Significant improvement on functional assessments

PT determines pt. will no longer benefit

References

1. Guide to physical therapy practice. 2nd ed. APTA; 2003.

2. O'Sullivan SB, Schmitz TJ. Physical rehabilitation. F a Davis Company; 2007. 3. Kisner C, Colby LA. Therapeutic exercise: Foundations and techniques. F a Davis Company; 2007.

3. Goodman CC, Fuller KS. Pathology: Implications for the physical therapist. SAUNDERS W B Company; 2009.

4. Alexander TH, Harris JP. Current epidemiology of meniere's syndrome. Otolaryngol Clin North Am. 2010;43(5):965-970. doi: 10.1016/j.otc.2010.05.001; 10.1016/j.otc.2010.05.001.

5. Egami N, Ushio M, Yamasoba T, Yamaguchi T, Murofushi T, Iwasaki S. The diagnostic value of vestibular evoked myogenic potentials in patients with meniere's disease. J Vestib Res. 2013;23(4-5):249-257. doi: 10.3233/VES-130484; 10.3233/VES-130484.

6. Guidetti G, Monzani D, Rovatti V. Clinical examination of labyrinthine-defective patients out of the vertigo attack: Sensitivity and specificity of three low-cost methods. Acta Otorhinolaryngol Ital. 2006;26(2):96-101.

7. Saeed SR. Fortnightly review. Diagnosis and treatment of Meniere's disease. BMJ. 1998 Jan 31;316(7128):368-72 

8. Vassiliou A, Vlastarakos PV, Maragoudakis P, Candiloros D, Nikolopoulos TP. Meniere's disease: Still a mystery disease with difficult differential diagnosis. Ann Indian Acad Neurol. 2011;14(1):12-18. doi: 10.4103/0972-2327.78043; 10.4103/0972-2327.78043.

9. Meniere's society. Meniere's Society Web site. http://www.menieres.org.uk/. Updated 2013. Accessed February 26, 2014.

10. Li J, Lorenzo N. Meniere disease (idiopathic endolymphatic hydrops) Differential diagnoses. Medscape Web site. http://emedicine.medscape.com/article/1159069-differential. Updated 2014. Accessed February 26, 2014.

11. Meniere's disease. Mayo Clinic Web site. http://www.mayoclinic.org/diseases-conditions/menieres-disease/basics/definition/CON-20028251. Updated 2012. Accessed February 24, 2014.

References

12. Rauch, SD. Clinical hints and precipitating factors in patient’s suffering from Meniere’s disease. Otolaryngol Clin North Am. 2010 Oct; 43(5): 1011-7

13. Minor LB, Schessel DA, Carey JP. Meniere's disease. Curr Opin Neurol. 2004 Feb;17(1):9-16

14. Committee on Hearing and Equilibrium guidelines for the diagnosis and evaluation of therapy in Menière's disease. American Academy of Otolaryngology-Head and Neck Foundation, Inc. Otolaryngol Head Neck Surg. 1995 Sep;113(3):181-5, commentary can be found in Otolaryngol Head Neck Surg 1996 Jun;114(6):835

15. Syed I, Aldren C. Meniere's disease: an evidence based approach to assessment and management. Int J Clin Pract. 2012 Feb;66(2):166-70

16. Enticott JC, O'leary SJ, Briggs RJ. Effects of vestibulo-ocular reflex exercises on vestibular compensation after vestibular schwannoma surgery. Otol Neurotol. 2005;26(2):265-269.