Ear Structures of importanceimportanceEar Otologic DizzinessOtologic Dizziness (Dizziness from Ear)Ear)(Dizziness

Timothy C. Hain, MDMDTimothy

Northwestern University, ChicagoChicagoNorthwestern [email protected]@northwestern.edu

T h e ea r is an inertia l nav iga tion The ear is an inertial navigation Vestibular ReflexesVestibular Reflexes devicedevice nn Semicircular Canals nn VOR: Vestibulo--VestibuloVOR: are rate sensors.are sensors. ocular reflexocular reflex nn Otoliths (utricle and nn VSR: Vestibulospinal saccule) are linear saccule) are linear reflexreflex accelerometersaccelerometers nn Bilateral symmetry means redundant design.design.

Positional VertigoPositional Vertigo Otologic (Ear) DizzinessOtologic Dizziness The most common syndromesyndromeThe nn BPPV (benign paroxysmal nn positional vertigo) positional vertigo) ----aboutabout Benign Paroxysmal 50% of otologicotologic, 20% allall, Otologic Dizziness nn Meniere’s disease ----aboutabout Fistula SCD 20%20% Bilateral Other Positional Vertigo nn Vestibular neuritis and related conditions (15%)related (15%) Neuritis BPPV (BPPV) ----bed spinsbed spins nn Bilateral vestibular loss nn Menieres Orthostatic hypotension (dizzy upright)upright)Orthostatic (about 1%)(about 1%) nn nn SCD and Fistula (rare but Central positional nystagmus (dizzy everywhere)everywhere)Central worth knowing)knowing)worth nn Low CSF pressure syndrome (dizzy upright)upright)Low

1 Benign Paroxysmal Positional Positional VertigoPositional Vertigo Vertigo (BPPV)Vertigo (BPPV) DixDix--Hallpike ManeuverManeuverHallpike

61 Y/O man slipped on wet floor. LOC for 20 minutes. In ER, unable to sit up because of dizziness Hallpike Maneuver: Positive

Benign Paroxysmal Positional BPPV Mechanism: Utricular debris migrates to Vertigo (BPPV)Vertigo (BPPV) posterior canal nn 20% of allall vertigovertigo nn Brief and strongstrongBrief nn Provoked by change of head positionpositionProvoked nn Definitively diagnosed by Hallpike testDefinitively test

BPPV treatmentBPPV treatment Unilateral VestibularUnilateral Vestibular nn Medication (e.g. antivertantivert)) –– nn Vestibular Neuritis/LabyrinthitisLabyrinthitisNeuritis/Vestibular (common)(common) minor benefitminor benefit nn Meniere’s disease (unusual, 1/2000 ––MayMay avoid vomiting by pretreatingpretreating prevalence)prevalence) nn nn Excellent response to PTExcellent PT Acoustic Neuroma (very rare)rare)Acoustic nn Surgery ––canalcanal plugging if nn Vestibular paroxysmiaparoxysmia(not sure how rehab fails (need more common)common) rehab after plug). Rarely rehab after plug). Rarely done.done.

2 Vestibular Spontaneous Nystagmus Vestibular Neuritis: CaseVestibular Case seen with video Frenzel Goggles

56 y/o woman began to become dizzy after lunch. Dizziness increased over hours, and consisted of a spinning “merri-go-round” sensation, combined with unsteadiness. Vomiting ensued 2 hours later, and she was brought by family members to the ER.

Vestibular Spontaneous Nystagmus recorded on ENG Aside : how to examine for SNAside SN (Electronystagmography)

nn Frenzel Goggles (best)Frenzel (best) nn Ophthalmoscope (good ––butbut backwards)backwards) nn GazeGaze--evokedevoked nystagmus (pretty good)(pretty good) nn Sheet of white paper (neat)Sheet (neat)

Vestibular Neuritis ---- rxrx Meniere’s DiseaseDiseaseMeniere’s nn Disturbance of unknown cause (Viral ? Vascular) cause (Viral ? Vascular) nn involving vestibular nerve Prosper MeniereMeniere or ganglionor ganglion ––Fluctuating hearinghearingFluctuating nn Disability typically lasts 2 weeks.weeks. ––Episodic VertigoEpisodic Vertigo nn Steroids if dxdx first 2 daysfirst days ––Fluctuating (roaring) TinnitusTinnitusFluctuating nn Symptomatic Rx Symptomatic Rx ––Aural FullnessAural Fullness (meclizine, (meclizine, phenerganphenergan,, benzodiazepine)benzodiazepine) nn About 1/2000 people in populationpopulationAbout nn Rehab if still symptomatic Rehab if still symptomatic nn after 2 months.months.after Chronic condition ––lasts lifetimelifetimelasts nn These patients can still get BPPV !BPPV !

3 Etiology of Meniere’s (Dogma)Etiology (Dogma) Meniere’s disease ––symptomssymptoms nn Dilation and episodic rupture of inner ear membranes (Endolymphatic Hydrops)Hydrops)(Endolymphatic nn Progressive hearing loss ----sometimessometimes go nn As endolymph volume and pressure increases, the As endolymph volume and pressure increases, the deafdeaf utricular/saccular and Reissner’s membranes rupture, releasing potassiumreleasing potassium--richrich endolymph into the perilymph nn Episodic vertigo ––outout of commission for causing cochlear/vestibular paralysisparalysiscausing several daysdaysseveral nn Ataxia ––gradually increases over yearsyearsgradually nn Visual sensitivity àà

Visual Sensitivity is commonVisual common Otolithic Crises of TumarkinTumarkinOtolithic nn Sensory integration nn Drop attacksDrop attacks disorder ––upweightupweight nn Go from upright to vision, downweight on floor in fraction everything elseelseeverything of secondof second nn Grocery store, nn No LOCNo LOC Omnimax, Target, etcetcOmnimax, nn Very dangerousVery dangerous nn Typical of disorders nn with intermittent Destructive vestibular problemsproblemsvestibular treatment is besttreatment best

Treatments of MenieresTreatments Menieres Acoustic NeuromaAcoustic Neuroma nn Medical management –– ––Usually ineffectiveineffectiveUsually nn Bad rehab candidate while fluctuatingBad fluctuating nn SurgerySurgery –– Low dose gentamicin treatment works nicelynicelyLow ––High dose gentamicin treatment (overkill)(overkill)High nn Rehab useful post destructive treatmentRehab treatment

Hain TC, Ostrowski T. Unsteady Influence. Menieres disease. Advances for directors in rehabilitation October 2007, 51-51

4 Acoustic NeuromaAcoustic Neuroma Treatment of Acoustic NeuromaTreatment Neuroma nn Cause of unilateral nn Watchful waiting (about 25%)25%)Watchful vestibular losslossvestibular nn Operative removal (about 50%) ––losinglosing nn Rare cause of Rare cause of groundground unilateral losslossunilateral nn nn Generally also deaf on Gamma Knife (about 25%) ––gaininggaining one sidesideone ground because effective and noninvasivenoninvasiveground nn Slowly progressive –– nn Good rehab candidateGood candidate little or no vertigolittle vertigo

Vestibular Paroxysmia (AKA Clinical Diagnosis of MVCClinical MVC microvascular compression)compression)microvascular

nn Quick spinsQuick spins nn Irritation of vestibular nervenerveIrritation nn May have nystagmus on hyperventilationhyperventilationMay nn Quick spins, tilts, dipsdipsQuick nn Response to anticonvulsantResponse anticonvulsant nn Motion sensitivitysensitivityMotion nn No rehab potentialpotentialNo nn May follow 88May thth nerve surgery, Gamma knife treatment, acoustic neuromaneuromatreatment, nn Wastebasket syndrome in some cases ?Wastebasket ?

Bilateral Vestibular LossLossBilateral SYMPTOMS OF BILATERAL VESTIBULAR LOSSVESTIBULAR LOSS A stewardess developed a toe-nail infection. She ll OSCILLOPSIAOSCILLOPSIA underwent course of gentamicin and vancomycin. 12 days after starting therapy she developed imbalance. 21 days after starting, she was “staggering like a drunk person”. Meclizine was prescribed. Gentamicin was stopped on day 29. One year later, the patient had persistent imbalance, visual symptoms, and had not returned to work. Hearing is normal. She unsuccessfully sued her doctor for malpractice.

5 S Y M P T O M S OF B ILA T E RA L SYMPTOMS OF BILATERAL Bilateral Vestibular LossLossBilateral VESTIBULAR LOSSVESTIBULAR LOSS Causes:Causes:

nn Ototoxicity !Ototoxicity ! ll ATAXIAATAXIA nn Congenital Bilateral forms of 2%Solvent unilateral disorders (e.g. unilateral disorders (e.g. 5% Unknown bilateral vestib neuritis)neuritis)bilateral 26% nn Congenital (e.g. Gentamicin Mondini malformation)malformation)Mondini V Neuritis 58% 9% nn idiopathicidiopathic

N=43, NMH 1990-1998

Dynamic Illegible ‘E’ testDynamic test DIAGNOSIS IS EASYEASYDIAGNOSIS (DIE test)(DIE test) ll History of recent IV antibiotic medicationmedicationHistory nn ll Distance vision with head Eyes closed tandem Romberg is positivepositiveEyes stillstill ll Dynamic illegible ‘E’ test (DIE) failed nn Distance vision with head movingmoving nn Normal: 0Normal: 0--2 lines change.change.2 nn Abnormal: 4Abnormal: 4--77 lines ------>> changechange

LABORATORY DIAGNOSISLABORATORY DIAGNOSIS Rapid Dolls failedRapid failed Everything should be “dead”“dead”Everything nn VOR: Vestibulo--VestibuloVOR: ocular reflexocular reflex ll ENGENG ll RotatoryRotatory chairchair ll VEMPVEMP

6 DIAGNOSIS ContinuedDIAGNOSIS Continued DIAGNOSIS ContinuedDIAGNOSIS Continued ll Rotatory chair confirms diagnosis but requires cooperationrequires cooperation ll ENG shows little or no responseresponse

P er ilym ph F istu la a nd S C D (s u pe rio r Perilymph Fistula and SCD (superior Treatment BilateralTreatment Bilateral canal dehiscence)dehiscence)canal

Fluctuating conditions nn No medical management (other than avoiding more damage)avoiding damage) No rehab until after surgery nn Outstanding rehab candidateOutstanding candidate nn Superior Canal nn Be prepared for a depositiondepositionBe DehiscenceDehiscence

Case: WSCase: WS Tullio in SCDSCDTullio

Retired plastic surgeon, with impaired hearing related to war injuries, found that when he went to church, when organ was playing, certain notes made him stagger. His otolaryngologist noted that during audiometry (with hearing aid in), certain tones reliably induced dizziness and a mixed vertical/torsional nystagmus. This “Tullio’s phenomenon” could be easily reproduced experimentally. MRI scan was normal.

7 ValsalvaValsalva in SCDin SCD

Superior Canal DehiscenceDehiscenceSuperior Diagnosis of SCDDiagnosis SCD nn Etiology:Etiology: ––CongenitalCongenital bone defect nn (2% ?)(2% ?) History of sound and pressure sensitivityHistory sensitivity ––TraumaTrauma may nn ValsalvaValsalva test is easiest bedside testtesttest exacerbateexacerbate nn nn Treatment:Treatment: Temporal Bone CT scan (high resolution)resolution)Temporal ––SurgicalSurgical nn VEMP: Vestibular evoked myogenicmyogenic potentialspotentials »»PlugPlug »»ResurfaceResurface

Case: KFCase: KF A large round window fistula was found and symptoms completely resolved after a second surgery. •After SCUBA diving, a young woman developed vertigo, aural fullness and tinnitus for 1 year. •Symptoms were worsened by tragal pressure and straining. Surgery was performed.

8 Summary of Otologic VertigoVertigoSummary More detailsMore details

Hain, T.C. Approach to the patient Otologic Fistula with Dizziness and Vertigo. Practical Other Dizziness SCD Neurology (Ed. Biller), 2002. Bilateral LippincottLippincott--RavenRaven Neuritis BPPV More moviesMore movies Menieres www.dizzinesswww.dizziness--andand--hearing.comhearing.com

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