Evaluation of the Adult Dizzy Patient Elizabeth Kelly, MD Boys Town National Research Hospital ENT Institute Neurotology/Otology September 27, 2018

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Evaluation of the Adult Dizzy Patient Elizabeth Kelly, MD Boys Town National Research Hospital ENT Institute Neurotology/Otology September 27, 2018 No disclosures Objectives Describe different presentations and etiologies for dizziness in the adult patient Discuss medical and surgical management strategies for the adult dizzy patient Review the impact of aging on the vestibular system and clinical implications of these changes Discuss case presentations of patients with dizziness 1

Vestibular symptoms 10 30% of population May be debilitating missed work, social isolation, depression. Common presenting symptom in primary care offices and specialty clinics Includes: vertigo, dizziness, unsteadiness, oscillopsia, presyncope, syncope. Agrawal J Vestib Res 2013 Health impact of dizziness NHANEs data 35.4 % prevalence of vestibular dysfunction > 40 years Based on posturography. Increased in those with HTN, diabetes Those with vestibular dysfunction, more likely to report dizziness and complain of history of falls Increasing age associated with vestibular dysfunction Agrawal Arch Intern Med 2009 Health impact of dizziness Complex physiology multiple provider visits, delay in diagnosis and management. Significant consumption of ED services, multispecialty consultation, and imaging Cost burden on patient and provider Kaylie Arch Otolaryngol Head Neck Surg 2012 Agrawal J Vestib Res 2013 2

Etiology for Brain dizziness Inner ear Vision Cardiovascular Lower extremities, Proprioception Etiology for dizziness Metabolic Trauma Inner ear Psychiatric Medications Central vestibular disorder Cardiovascular Visual Infection Peripheral vestibular disorders More frequent Men, age 60 69 years old Most common in otolaryngology clinic BPPV Meniere s disease Vestibular neuritis/labyrinthitis Vestibular migraine 3

Anatomy of the vestibular system Bony labyrinth Membranous labyrinth Perilymph (CSF) Endolymph (intracellular) Semicircular canals Superior Posterior Horizontal Otolith organs Utricle and saccule Anatomy of the vestibular system Semicircular canals Endolymph moves in response to head angular acceleration Fluid movement stimulates/inhibits hair cells Otolith organs Linear acceleration and gravitational changes Glasscock Shambaugh Surgery of the ear Anatomy of the vestibular system Superior vestibular nerve Superior SCC Horizontal SCC Utricle Inferior vestibular nerve Posterior SCC Saccule Shruti Otology Neurotology 2015 4

Evaluation History and Physical Exam: most valuable information for correct diagnosis Audiogram Vestibular Testing Imaging Referrals Tell me about your dizziness... Vertigo Spinning Lightheaded Off balance Brain fog Disequilibrium Unsteadiness Floating History First attack or recurrent vertigo Spontaneous or Positional Duration of each spell (seconds, minutes, hours, days, continuous?) Associated symptoms Any recent events that contribute Welgampola Neurol Clin 2015 5

Physical exam Detailed head and neck, neurologic exam Assessment of vestibular function and cerebellar function Often normal exam findings Vestibular disorders Benign paroxysmal positional vertigo Meniere s disease Vestibular migraine Labyrinthitis/vestibular neuritis Superior semicircular canal dehiscence Bilateral vestibule hypofunction Benign Paroxysmal Positional Vertigo (BPPV) Disorder of inner ear with repeated episodes of positional vertigo Displaced otolith particles Most common cause of peripheral vertigo Variants Posterior canal more common Horizontal canal Anterior canal 1% AAO HNS Clinical Practice Guideline 6

BPPV Diagnostic criteria Posterior canal BPPV Upbeating torsional nystagmus Repeated episodes of vertigo with changes in head position Vertigo associated with nystagmus provoked by Dix Hallpike Latency period of 5 20 sec Crescendo decrescendo provoked vertigo and nystagmus up to 60 sec Dix Hallpike BPPV Limitations of Dix Hallpike Considered gold standard to diagnose posterior canal BPPV Sensitivity 82%, specificity 71% in specialty clinics Speed of head movements Angle during maneuver Time of day Lopez Escamez Acta Otorhinolaringol Esp 2000 BPPV Limitations of Dix Hallpike Careful consideration: Vascular disease Cervical stenosis or radiculopathy Decreased neck ROM Down s syndrome Obesity 7

BPPV Diagnostic criteria Supine roll test Horizontal canal BPPV Less common Possible after treatment Horizontal nystagmus Toward ground or away (geotropic or ageotropic) Most intense side is affected ear BPPV Management Should NOT obtain radiographic imaging, vestibular testing, unless uncertain May offer observation as initial management May offer vestibular rehab for initial treatment for BPPV Should NOT routinely treat with vestibular suppressants BPPV Management Should treat patients with posterior canal BPPV with particle repositioning maneuver Epley Maneuver (right) 8

BPPV Management Barbecue roll maneuver (right) BPPV Outcome Should reassess within 1 month after an initial period of observation or treatment to confirm symptom resolution Should counsel patients regarding the impact of BPPV on safety, potential for disease recurrence and importance of follow up. Positive predictors of BPPV Lying down/rolling over Vertigo Negative predictor Dizziness lasting more than 20 minutes 9

Meniere s Disease (MD) Etiology unclear Changes in inner ear fluid volumes endolymphatic hydrops (ELH) Supported in cadaver temporal bone studies All with MD had ELH 65% with ELH had concurrent MD Lopez Escamez J Vest Res 2015 Merchant 2005 MD Diagnostic criteria Definite MD 2 or more spontaneous episodes of vertigo lasting 20 min to 12 hours Audiometrically documented low to medium frequency sensorineural hearing loss in one ear before, during or after an episode of vertigo Fluctuating aural symptoms (hearing, tinnitus or fullness) in affected ear Probable MD 2 or more episodes of vertigo or dizziness, each lasting 20 min to 24 hours Fluctuating aural symptoms (hearing, tinnitus or fullness) in affected ear MD Disease progression Progressive with unpredictable fluctuations Early stage disease Acute vertigo attacks increase over first few years Late stage disease Near complete cessation of vertigo Hearing loss persistent and worse Episodic nature Challenges to distinguish periodic disease resolution vs treatment effects 10

MD Diagnostic Testing Serial audiograms Vestibular testing consideration Imaging MD Management Goals: Reduce severity/frequency of vertigo attacks Relieve or prevent auditory symptoms Improve QOL Non Ablative Least Invasive Ablative Invasive MD Management Dietary modifications, lifestyle changes Oral pharmacotherapy Meniett Device Intratympanic steroids Endolymphatic sac surgery Non ablative Ablative Intratympanic gentamicin Labyrinthectomy Vestibular nerve section 11

MD Symptom reduction Dietary modifications Environmental triggers allergy, weather Behavioral triggers stress, smoking, etoh MD Pharmacotherapy Acute medications Meclizine Valium Maintenance medications Diuretics Betahistine Steroids MD Positive pressure therapy Meniett device Controlled small pressure pulses to inner ear Need tympanostomy tube placement Conflicting evidence 12

MD Intratympanic therapies Intratympanic steroids (non ablative) Improves cochlear blood flow through anti inflammatory effects Improve vestibular symptoms, less risk on hearing Intratympanic gentamicin (ablative) Chemical ablation of vestibular system Risk for hearing loss/cochlear damage Dosing and frequency is not standardized Initially daily titration of medications > high rates of hearing loss Changed to as needed or monthly injections Int Arch Otorhinolaryngol 2018 MD Endolymphatic sac procedure Non ablative option for preserved hearing Controversial Cochrane review 2 RCT comparing with those receiving tubes/mastoid > no difference in outcomes Other SR 75% patients with symptom improvement after surgery Pullens. Cochrane Database Syst Rev 2013 Lambert. Otol Neurotol. 2014. Glasscock Shambaugh Surgery of the ear 13

MD Ablative surgical procedures Labyrinthectomy Persistent symptoms refractory to less definitive treatment Non serviceable hearing in affected ear Surgical risks: CSF leak Facial nerve injury Dizziness Glasscock Shambaugh Surgery of the ear MD Ablative surgical procedures Vestibular nerve section Persistent symptoms Serviceable hearing Intradural procedure Transection of vestibular nerve Glasscock Shambaugh Surgery of the ear MD Rehabilitation Progressive disease Counsel patients on hearing loss Discuss hearing aids or assistive technology Vestibular rehabilitation 14

Vestibular Migraine (VM) Migraine is 3 rd most common disease worldwide Lifetime prevalence 16% Underappreciated cause of dizziness VM Diagnostic criteria Definite VM At least 5 episodes of vestibular symptoms of moderate to severe intensity lasting 5 min to 72 hours Current or previous history of migraine 1 or more migraine features with at least 50% of vestibular episodes Probable VM At least 5 episodes with vestibular symptoms of moderate or severe intensity lasting 5 min to 72 h Current or previous history of migraine OR 1 or more migraine features with at least 50% of vestibular episodes VM Other symptoms to consider Vestibular symptoms with sinus pressure, neck pain, ear pressure referred pain within trigemino cervical complex. May not have had migraine for years prior to vestibular symptoms Duration of symptoms varies continuous for months or multiple times per day Extreme motion sensitivity Symptoms with head position changes Uncommon to have hearing loss, tinnitus 15

VM Clinical presentation Risk factors Young age (40 yo) Female History of anxiety or depression Prior head trauma Comorbid with BPPV and MD Impacts QOL 60% unable to go to school or work VM No diagnostic test to confirm Can have abnormalities on vestibular testing Review of MD vs VM Caloric weakness more likely in MD, 2 studies there was no difference VEMPs no difference but tuning frequency may be beneficial to distinguish Saliba J Clin Med Res 2017 VM Management Dietary modifications/ Identification of triggers Medications Abortive treatment rarely helpful Prophylactic treatment elevate migraine threshold to prevent attacks Not one gold standard medication Depended on current medications, comorbidities May have mild side effects Trial for several months and then wean to smallest effective dose 16

VM Prophylactic medications Tricyclic antidepressants (nortriptyline, amitriptyline) Dry mouth, sedation Anticonvulsants (topiramate, gabapentin) Weight loss, cognitive side effects Calcium channel blockers (verapamil) Hypotension Beta blockers (propranolol) Caution in asthmatics Labyrinthitis Inflammation of membranous labyrinth Etiology: infection, autoimmune, systemic, trauma Serous: mild to moderate hearing loss with mild vestibular dysfunction Suppurative: profound SNHL, otalgia, fever, emesis, vertigo. Labyrinthitis Exam Audio Vestibular testing Blood cx, LP CT Treatment: Abx Treat cholesteatoma Myringotomy Vestibular suppressants Vestibular rehab 17

Vestibular neuritis Reactivation of latent HSV 1 unilateral vestibular deafferentiation Hsv 1 DNA on nerve fibers in autopsy Atrophy and inflammation of nerve Ischemic damage to labyrinth Superior nerve most common Longer course in bone and more narrow Inferior nerve Complete vestibular nerve Vestibular neuritis Diagnosis Clinical presentation Spontaneous vertigo severe for several days N/V Unsteadiness Physical Exam Nystagmus Fast phase away from lesion Follows Alexanders law Remainder of neurologic exam Vestibular neuritis vestibular testing Localize superior, inferior, or total nerve involvement Superior vestibular nerve Abnormal superior, horizontal canal vhit Abnormal caloric Abnormal ovemp Inferior vestibular nerve Abnormal posterior canal Abnormal cvemp 18

Vestibular neuritis Management Vestibular rehab Prognostic information BPPV risk Recovery 2 11% with recurrence Superior canal dehiscence syndrome (SCDS) Absence of bone overlying superior semicircular canal 3 rd mobile window Oval window Round window Sound and/or pressure induced vertigo https://www.brighamandwomensfaulkner.org/programs andservices/otolaryngology/ear/superior semicircular canal dehiscence SCDS Clinical presentation Auditory symptoms Hearing loss Aural fullness Autophony Hyperacusis Tinnitus Vestibular symptoms Vertigo Imbalance Sound, pressure, exercise provoked dizziness Oscillopsia 19

SCDS Physical exam Normal ear exam Tullio phenomenon Vertigo, eye movement with loud sound Hennebert s sign Vertigo, nystagmus induced by pressure in middle ear. Shuman et al. Laryngoscope. 2012 SCDS Diagnostic testing Audiogram Normal acoustic reflexes VEMP No correlation with threshold, ABG and symptoms SCDS Imaging High resolution CT scan 9% prevalence of radiographic dehiscence Rare SCDS Lower rates with histopath confirmation Williamson Otolaryngolology Head and Neck Surgery 2003 20

SCDS Management Observation Reassurance Discuss studies of hearing stability Progression of dizziness Decision for surgery Subjective symptoms Functional impairment Auditory symptoms greater improvement vs vestibular symptoms after surgical repair Surgery Goal: eliminate 3 rd mobile window Approaches Transmastoid vs middle fossa Canal plugging and/or resurfacing SCDS Surgical Management Transmastoid approach Middle fossa approach Bilateral vestibular hypofunction (BVH) Bilateral reduced or absent function of vestibular organs. Clinical Presentation Oscillopsia Imbalance Cognitive deficits Autonomic symptoms Exam Abnormal head thrust Vestibular testing 21

BVH Subtypes Recurrent vertigo and BVH Rapidly progressive BVH Slowly progressive BVH BVH with neurological deficits Episodes of vertigo, then development of BVH Sudden onset or rapid progression Clinical symptoms of BVH develop gradually, mostly without episodes of vertigo Clinical symptoms of BVH with neuro deficits Peripheral polyneuropathy, cerebellar ataxia. BVH Cause Etiology often challenging Definite Probable Idiopathic migraine? Lucieer. Front Neurol. 2016 Bilateral vestibular hypofunction Management Vestibular rehab Improves gaze and postural stability Future... vestibular implants? 22

Dizziness in the older adult Common, 30% elderly have balance disorder Rate increases with age Debilitating Impact QOL, risk for falls Challenging no diagnostic alogrithm for dizziness, often mixed with nonvestibular causes. Should we modify our approach in this population? Dizziness in the older adult Impact on vestibular system Temporal bone studies in animals and humans Depletion of vestibular hair cells and otoliths Dysfunction of remaining hair cells and loss of vestibular ganglion cells Decline in vestibular processing Decreased ability to process visual and vestibular signals Decreased ability to compensate Dizziness in the older adult Evaluation Consider common etiologies BPPV Multisensory deficits Multifactorial etiology Microvascular disease > disequilibrium of aging Migraine and other psychological factors are less common 23

Older adult Prebyastasis Disequilibrium of aging Multifactorial Eye disorders (glaucoma, cataracts, macular degeneration) Disorders of peripheral sensory organs (peripheral vascular disease) Arthritic disorders (cervical spine, knee, hips) Multisensory disorders (diabetes) Significant negative influence on vestibular function Vestibulotoxic microangiopathic effects Impaired glucose metabolism alters metabolism of inner ear fluids labyrinthine dysfunction Cardiovascular Polypharmacy Wetmore 2011 Presbystasis Clinical presentation Gradual difficulty in walking, need to hold onto objects for steadiness Increased falls No dizziness with sitting still or lying down Dizziness in the older adult Evaluation Physical exam Orthostatics Dix hallpike Evaluate mini mental status exam Vestibular testing Subtle changes noted with age in calorics and rotary chair VEMPS some age changes (increase thresholds and decreased amplitude response) 24

Dizziness in the older adult BPPV Episodic, sporadic, short bursts of spinning sensation with head movements, bending over, rolling over in bed Migration of loosened otoconia from otolith organs Some with just symptoms in specific head positions others with persistent instability between spells Perform Dix hallpike in older dizzy patients if able. Treatment: trendelenburg position, foam wedge as neck kyphosis Mimics: vertebrobasilar disease or cervical spin osteoarthritis Symptoms in head hanging position, no nystagmus (or purely vertical nystagmus) Risk factors of HTN, cardiac disease, Diabetes More likely to benefit from balance therapy in addition to maneuvers. Wetmore 2011 Dizziness in the older adult Vestibular physical therapy Beneficial for older adults Gait Body balance control Activities of daily living Jung et al: 240 patients older than 70 helpful even without specific diagnosis Ricci. Contemporary Clinical Trials. 2015 Dizziness in the older adult Polypharmacy Fall risk increasing drugs Antihypertensives Psychotropic (CNS acting meds) Narcotics In one review of neurotology clinic 40% on FRID, 1/3 on 2 or more (30% in other studies) Multidisciplinary approach to determine tradeoff with medical condition. Study shown if discontinue FRID...less risk of falls 25

Fall risk increasing drugs Systematic review and meta analysis Central nervous system active drugs increase risk of falling Huang. Drugs & Aging. 2012 Fall risk increasing drugs Huang. Drugs & Aging. 2012 Reducing morbidity Reduce orthostatic hypotension Reduce mediations that worsen balance Improve visual acuity with appropriate glasses Refer patients with history of falls, instability, or balance issues to vestibular therapy Home safety nightlights, no loose rugs Assistive devices (canes, walker?) Exercise Walking speed increase Sturdy shoes 26