Vestibular Grand Rounds

Vestibular Grand Rounds Jamie M. Bogle, AuD, PhD Christopher Zalewski, MA Darcy Strong, AuD Anne Mull, AuD Symptomatic Superior Canal Dehiscence Following Head Trauma Jamie M. Bogle, AuD, PhD Bogle.Jamie@mayo.edu eaudiology Web Seminar Vestibular Grand Rounds 26 July 2012 2012 MFMER slide-2 Superior Canal Dehiscence Auditory / vestibular dysfunction Symptomatic vs Anatomic Surgical intervention Surfacing / plugging; cartilage cap Resolution of symptoms Lundy et al, 2011 Minor et al, 1998; Minor, 2005; Zhou et al, 2007; Chi et al, 2010; Minor, 2000; Brantberg et al, 2001; Mikulec et al, 2005; Hillman et al, 2006; Crane et al, 2008; Lundy et al, 2011 2012 MFMER slide-3 1

Cause of Dizziness? Sound pressure / increased labyrinthine pressure relative to the middle cranial fossa Third mobile window Vestibular sensor deflection http://www.tchain.com/otoneurology/disorders/unilat/fistula.html; Minor et al, 1998; Brantberg et al, 1999 2012 MFMER slide-4 Common Symptoms Autophonia Dizziness / vertigo / chronic dysequilibrium Tullio phenomenon Hennebert phenomenon Physical stressevoked dizziness Pulse-synchronous oscillopsia Hyperacusis Fullness Pulsatile tinnitus Brain fog Fatigue 2012 MFMER slide-5 Common Signs Tullio-, Hennebert-evoked nystagmus Enlarged cervical, ocular VEMPs Paradoxical conductive hearing loss with present acoustic reflexes 2012 MFMER slide-6 2

Case 1 2012 MFMER slide-7 Case 1 73 year old, male Upper respiratory infection Fell, hit head on bathtub, LOC 5 minutes Hx of significant anxiety / depression CC: Dizziness Following head trauma Lightheaded, spacey, unsteady, off-balanced Denies vertigo, Tullio, autophony, aural fullness 2012 MFMER slide-8 Case 1 Assessment PE normal Word recognition (88% AD, 80% AS WNL) Tympanometry / Reflexes WNL AU 2012 MFMER slide-9 3

Case 1 Vestibular Study Additional oculomotor testing WNL No positional / positioning / hyperventilation-induced nystagmus Rotational chair testing non-significant 2012 MFMER slide-10 Case 1 Vestibular Study Calorics 2012 MFMER slide-11 Case 1 - cvemp 100 db nhl 270.44 V 90 db nhl 80 db nhl 75 db nhl Amplitude larger than expected for age / sex (p=0.10) Threshold lower than expected for age / sex (p=0.24) 70 db nhl 2012 MFMER slide-12 4

Case 1 - ovemp 10.58 V Current ovemp Literature Reduced presence / amplitude > 50 years More reliable parameters than cvemp 100 db nhl n10 Considerably larger (n1-p1) amplitude than expected for age. Piker et al, 2011 Nguyen et al, 2010; Piker et al, 2011 2012 MFMER slide-13 Case 1 Impressions Post concussion? Central involvement? Large cvemp, ovemp Abnormal ocular motor presentation 2012 MFMER slide-14 Case 1 AD: Thin plate AS: Dehiscence 2012 MFMER slide-15 5

Case 1 Cartilage cap occlusion Lundy et al. 2011 2012 MFMER slide-16 Case 1 Return visit (1 month) Reports improvement, still some occasional imbalance Remaining lightheadedness to be explored Final Impression Post-traumatic SCD treated with cartilage cap technique To return in 2 months for follow up, possible vestibular battery, physical therapy 2012 MFMER slide-17 Case 2 2012 MFMER slide-18 6

Case 2 53 year old, male Tailgate of dump truck hit just behind / above the left ear Bleeding from left ear, vomiting CC: Dizziness, head / neck pain Stooped over Feels tumbling when gets up too quickly Felt jittery, jello head 2012 MFMER slide-19 Case 2 Assessment PE normal Normal MRI Word recognition WNL Tympanograms WNL AU; Reflexes elevated 2012 MFMER slide-20 Case 2 2012 MFMER slide-21 7

Case 2 - cvemp 270.02 V 180.08 V 2012 MFMER slide-22 Case 2 - Positionals 2012 MFMER slide-23 Case 2 - Impressions BPPV not currently symptomatic? Post concussion? Large cvemp and oblique (down beating component) positional nystagmus 2012 MFMER slide-24 8

Case 2 Return visit (6 months) Transient dizziness Normal PE CT 2012 MFMER slide-25 Case 2 AS: Dehiscence 2012 MFMER slide-26 Case 2 Return visit (24 months) Loud sounds: falls suddenly like he has been shot Tympanometry: felt like someone was pushing him 2012 MFMER slide-27 9

Case 2 Return visit (36 months) Noise: knees buckle Talking inside my head Lifting, strain = dizzy 2012 MFMER slide-28 Case 2 Final Impression Post-traumatic SCD with progressive auditory / vestibular symptoms, signs 2012 MFMER slide-29 Take Home 1. SCD may present following acute incident not always gradually developing. Minor head injury, blast injury, concussion, etc 2. Appropriate diagnosis? Presenting signs and symptoms But! Signs, symptoms may evolve over time you see a snapshot! Imperfect relationship between signs, symptoms, test results, and anatomy. Stimmer et al, 2012; Williamson et al, 2003; Cloutier et al, 2008; Crovetto et al, 2009; Strupp et al, 2000; Mehlenbacher et al, 2012; Minor, 2005; Zhou et al, 2007; Hegemann & Carey, 2011 2012 MFMER slide-30 10

Questions? To ask a question, please type your question into the chat box in the lower left corner of the screen and click on the Send button located right below the box. 2012 MFMER slide-31 Chris Zalewski National Institutes of Health, NIDCD Clinical Determination for Vestibular Schwanomma Surgical Resection on a Patient with NF2 a four year journey History 12 year old female with Neurofibromatosis Type II Known to NIH Audiology since her baseline assessment in June of 2009 when she was 8 years of age Recent history of two right ear middle ear surgeries: First in March of 2011, for middle exploration and subsequent removal of a facial neuroma within the middle ear cavity A second right ear postauricular canal wall up mastoidectomy, ossiculoplatsy with TORP, placement of a pressure equalization tube, and sural nerve cable grafting was performed in May of 2011 Iatrogenic right facial palsy secondary to facial neuroma resection Aggressive growth of vestibular schwanommas with her left VS being significantly larger (.does this matter?) Patient reports zero difficulties with balance and is an active pre-teen who snow skis, is a cheerleader, swims, loves to ride a bike, and enjoys long distance running 11

Neurofibromatosis Type 2 Autosomal dominant genetic syndrome caused by a mutation in the NF2 gene on chromosome 22 responsible for human merlin, a protein responsible for tumor suppression. Merlin is commonly found in nervous tissues, some fetal tissue and in adherens junctions (protein complexes that occur at cell cell junctions in epithelial tissues) Hallmark finding of NF2 is bilateral vestibular schwanommas Other cranial nerves and meninges Spinal tumors Cataracts Skin neurofibrommas The principal treatments consist of neurosurgical removal of the tumors and surgical treatment of the eye lesions. There is no therapy for the underlying disorder of cell function caused by the genetic mutation. Audiogram 2012 Left ear Right ear Vestibular Assessment CDPP VEMP VNG Spontaneous Testing Positional Testing Caloric Irrigations Rotational Testing Ocular motor testing SHA 60 0 & 240 0 Step Testing 12

Computerized Dynamic Posturography Sensory Organization Test (SOT) SOT - 2009 SOT - 2010 SOT - 2011 SOT - 2012 Vestibular Evoked Myogenic Potentials VEMP - 2010 VEMP - 2011 VEMP - 2012 Spontaneous Testing Vision Denied 2009 2010 13

Spontaneous Testing Vision Denied Spontaneous 2011 2-3 0 2-3 0 Spontaneous Testing Vision Denied Spontaneous 2012 2-3 0 Summary thus far. Date Impression Localizing Pathology Posturography Normal from 2009 through 2012 Normal Asymmetry amplitude 2010 (L>R) with absence of right VEMP in 2011 & 2012 2 0 to iatrogenic CHL Normal Left; Indeterminate Right VEMP Spontaneous Not present in 2009 & 2010; Ipsiversive look spontaneous vision denied in 2011; slight RB 2012 Central vs. Left Peripheral Ocular Motor Normal from 2009 through 2012 (data not shown) Normal Caloric Patient deferred in 2009 & 2010; Deferred in 2011 & 2012 for their non-contributory nature (CHL)? Bottom Line Summary ( thus far): Normal physiology through 2010 Starting 2011, non-localizing subtle spontaneous nystagmus (not uncharacteristic of NF2). VEMP: non-contributory right ear and an intact LEFT saccular pathway. 14

Sinusoidal Harmonic Acceleration SHA - 2009 SHA - 2010 SHA - 2011 SHA - 2012 Rotational Vestibular Testing 60 0 Step Testing time decay 37% VOR decay time constant (normal >10 sec) Rotational Vestibular Testing 60 0 Step Testing 2009 NORMAL (>10 seconds) 15

Rotational Vestibular Testing 60 0 Step Testing 2012 ABNORMAL (>10 seconds) What has rotational testing added.? The increased low frequency phase lead in the presence of normal VOR gain (although borderline low), suggests the presence of a compensated vestibular lesion but remains non-lateralizing without caloric irrigations The 60 0 step test has essentially confirmed the presence of a nonlocalizing pathology although the diagnosis of NF2 suggests a peripheral SOL and what are we STILL missing? Velocity Step Testing - 240 0 379.03 LEFT 203.06 RIGHT Per RIGHT 16

Rotational Vestibular Testing 240 0 Step Testing 2012 140.80 + 134.80 = 275.28 66.68 + 58.12 = 124.80 37.66% Right > Left Differential Physiology. Date Impression Localizing Pathology 2009 2010 2011 2012 Essentially normal physiology; isolated slight low frequency VOR phase lead Essentially unchanged; Novel asymmetrical VEMP finding ( right) Essentially unchanged with the exception of novel absence of VEMP right ear (due to iatrogenic CHL); Ipsiversive look spontaneous vision denied Slight decreasing VOR gain with slight increase in VOR phase lead; slight RB 2012 Deferred Caloric Irrigations; Nonlocalizing SOL Which ear? Both? Normal LEFT saccular pathway; Abnormal VEMP right could this be the culprit ear? Non-Localizing SOL; Right VEMP is no longer useful; Non-localizing spontaneous nystagmus Is the undetected pathology getting worse? The addition of 240 0 step test finally ends the dispute for peripheral dysfunction. Despite the normal left ear VEMP, the subtle indices for peripheral dysfunction lateralize to the left ear (likely the superior branch of the vestibular nerve. The right ear is likely the primary contributor to the overall objective vestibular response. Conclusions The left ear present VEMP suggests an intact inferior vestibular branch, however, the significant high velocity asymmetrical step testing suggests a primary dysfunction localizing to the h-scc and/or the superior branch of the vestibular nerve. The right ear absent VEMP is non-contributory, however, the previously noted reduced right ear VEMP amplitude in 2010 may suggest some involvement ( would/could this suggestion inappropriately guide our assumptions for any future vestibular weaknesses?) Rotational testing suggests a well-compensated vestibulopathy but earlier studies up until 2012 lacked any lateralizing value. The high velocity step test suggests a robust right ear h-scc response / a significantly weaker left ear h-scc and/or superior vestibular nerve. Is the right ear vestibular schwannoma confined to the facial nerve? In the absence of high velocity step testing, what is the suggestion for lateralizing any loss of function? The slight RB spontaneous nystagmus in 2012? Despite the historical surgery to the right ear, the robust right ear vestibular response would argue against any vestibular schwanomma tumor resection in this ear.unless medically indicated. 17

Questions. To ask a question, please type your question into the chat box in the lower left corner of the screen and click on the Send button located right below the box. Vestibular Grand Rounds AAA Webinar July 2012 Darcy A Strong, AuD 28 year old male Unsteady vision when playing sports Difficulty driving Poor balance when climbing Quick head movements cause blurry vision Denied vertigo 18

Audiometric Assessments cvemp LEFT RIGHT Oculomotor Testing 19

Positional Testing Caloric Irrigations Initial Spontaneous Right Warm Right Cool Left Cool Left Warm Rotary Chair 20

Vestibular Assessment Results Bilateral vestibular hypofunction Bilateral Vestibular Hypofunction Common causes: Vestibulotoxicity Meningitis Bilateral ear surgery Congenital disorders/malformations Trauma Elderly Bilateral vestibular neuritis Progressive neuropathy Meniere s Migraine Jen (2009) Diagnosis Medical case history revealed paternal history of Charcot Marie Tooth Disease Diagnosis: Progressive Bilateral VIII Nerve Neuropathy, likely Charcot Marie Tooth Disease 21

Charcot Marie Tooth Disease Chronic, progressive neuropathy affecting both motor and sensory nerves Most common inherited neurological disease Prevalence: 40/100,000 Reilly, et al. (2011) CMT: Classifications Type 1 Autosomal Dominant Affects myelin Type 2 Affects axons Greater range in onset and severity of symptoms Type 3 Originally called Dejerine-Sottas Severe de-myelination Often begins in infancy Type 4 Autosomal Recessive Very Rare Often results in severe sensory symptoms (cataracts, deafness) Yiu, et al. (2011) CMT: Genetics 22

CMT: Diagnosis Family history Clinical exam/symptoms Nerve conduction studies Genetic testing CMT: Onset Typical is within first two decades of life Rate of progression varies usually very slow Almost never affects brain function Is not life threatening CMT: Common Signs and Symptoms Weakness and sensory loss of lower and then upper limbs Difficulty running and walking Tripping Twisting of the ankle Foot drop Muscle cramps Hand tremors Reilly, et al. (2011) 23

CMT: Effects to Audio-Vestibular Function Auditory Neuropathy Vestibular function is rarely mentioned Gradual loss - compensation Poor overall motor function Not referred for vestibular testing Dubourg, et al. (2001) CMT: Treatment There is no effective drug treatment. Supportive treatment is limited to rehabilitation therapy Surgical treatment based on symptoms (less common) 28 year old male Diamox Vestibular Rehabilitation Counseling Bilateral vestibular loss Hearing loss 24

References Bahr M, Andres F, Timmerman V, Nelis ME, Van Broeckhoven C, Dichgans (1999). J. Central visual, acoustic, and motor pathway involvement in a Charcot-Marie-Tooth family with an Asn205Ser mutation in the connexin 32 gene (in process citation). J Neurol Neurosurg Psychiatry; 66:202 6. Bird, T.D. (1998). Charcot Marie Tooth Neuropathy X Type 1. Gene Reviews. Dubourg, O. Tardieu, S. Birouk, N. Gouider, R. Leger, J.M. et al. (2001). Clinical, electrophysiological and molecular genetic characteristics of 93 patients with x-linked charcot marie tooth disease. Brain. 124, 1958-1967. Jen, J.C. (2009) Bilateral vestibulopathy: clinical, diagnostic and genetic considerations. Semin Neurol, 29(5):528-33. Perez H, Vilchez J, Sevilla T, Martinez L. (1988). Audiologic evaluation in Charcot-Marie-Tooth disease. Scand Audiol Suppl; 30:211 13. Reilly, M. M., Murphy, S. M., & Laurá, M. (2011). Charcot-Marie-Tooth disease. Journal Of The Peripheral Nervous System, 16(1), 1-14. doi:10.1111/j.1529-8027.2011.00324.x Yiu, E., Geevasinga, N., Nicholson, G., Fagan, E., Ryan, M., & Ouvrier, R. (2011). A retrospective review of X-linked Charcot-Marie-Tooth disease in childhood. Neurology, 76(5), 461-466. Questions To ask a question, please type your question into the chat box in the lower left corner of the screen and click on the Send button located right below the box. Atypical Fluctuating Hearing Loss and Vertigo: A Pediatric Vestibular Case Anne Mull, Au.D. Nemours Children s Clinic Jacksonville, FL 25

Nemours Children s Clinic Jacksonville Outpatient pediatric specialty clinic Specialties include: Otolaryngology and Neuro Otology Audiology Ophthalmology Neurology Established vestibular laboratory in 2008 Patient Introduction 8 year old male Presents with sudden hearing loss and tinnitus in the right ear Denies vertigo or imbalance Medical History Born full term, unremarkable birth history Passed newborn hearing screening Met early speech and motor milestones on time Dyslexia and dysgraphia Diagnosed in kindergarten Mother has fluctuating unilateral sensorineural hearing loss and episodic vertigo Began at age 47 Attributed to rheumatoid arthritis 26

History, contd No significant history of: Ophthalmologic problems Middle ear disease Head trauma Ototoxic medication exposure Allergies Headache Initial Audiogram Tympanometry (226 Hz): normal bilaterally Word Recognition Scores: 80% AD, 80% AS Ipsilateral Acoustic Reflexes: present at 1000 Hz bilaterally DPOAEs: Present 2 3 khz only AD, Present 2 6 khz AS ENT Consultation Normal otoscopic examination CT scan of temporal bones normal Referral to genetics Thyroid, metabolic, and Connexin 26 labs all yielded normal results 27

Patient Returns 3 Months Later Tympanometry (226 Hz): Type C tympanograms bilaterally Word Recognition Scores: 100% left ear, 100% right ear DPOAEs present 2 6 khz bilaterally Initial Evaluation Three Months Later WD: 80% right, 100% left Tympanometry (226 Hz): Type A bilaterally DPOAEs: Present 2 3 khz only right ear, Present 2 6 khz only left ear Tympanometry (226 Hz): Type C bilaterally DPOAEs: Present 2 6 khz bilaterally Patient returns 3 years later New complaint of episodes of true vertigo, lasting 8 12 hours Accompanied by hearing loss, aural fullness and tinnitus in the left ear No right sided symptoms during the episodes No headaches 28

3 Years After Initial Presentation Tympanometry (226 Hz): normal bilaterally Word Recognition Scores: 100% left ear, 100% right ear DPOAEs present 2 6 khz bilaterally Initial Evaluation Three Months Later Three Years Later Vestibular Evaluation Ocular motor testing: Slight horizontal, left beating gaze evoked nystagmus on gaze left Saccades, smooth pursuit, and OPK nystagmus WNL Slow Harmonic Acceleration (.02.64 Hz): Gain and symmetry values fell within adult norms Phase lead for low frequency rotations Poor visual fixation suppression Not significant able to suppress well during calorics 29

Vestibular Eval, cont d Dix Hallpike maneuvers and head rolls were negative No significant spontaneous/positional nystagmus Normal, symmetrical caloric responses Absent VEMP on the left Summary of Vestibular Evaluation Abnormal findings: Gazed evoked nystagmus on gaze left Phase lead on SHA, despite normal gain and symmetry values Absent VEMP on the left Suggests transient peripheral vestibulopathy Involving at least the cochlea, saccule, and horizontal canal VEMP implicates the left ear, however no other finding is localizing 30

Neuro Otology Consult Normal otoscopic examination Orders MRI of IACs Normal Orders autoimmune labs Given bilateral fluctuating HL and family history of rheumatoid arthritis Rheumatoid factor and Erythrocyte sedimentation rate tests normal Summary of Findings 11 year old male with: Bilateral, low frequency fluctuating SNHL Episodic vertigo lasting 8 12 hours Tinnitus and aural pressure in the left ear only Evidence of transient peripheral vestibulopathy Normal CT & MRI scans Negative autoimmune work up Differential Diagnoses Migraine associated vertigo? No headache No phono/photophobia Duration of vertiginous episodes does not fit typical migraine profile (Shepard, 2006) Autoimmune disease? Family history of rheumatoid arthritis, fluctuating hearing loss Bilateral, fluctuating hearing loss however No visual symptoms Autoimmune labs negative 31

Diagnosis: Endolyphatic hydrops Characterized by: Fluctuating, sensorineural hearing loss Tinnitus Aural fullness Recurrent, episodic vertigo Idiopathic Hamid, 2009 Neuro Otologist s Plan Low salt diet Return in 3 months Patient returns 3 months later Feeling better, no new episodes of vertigo Reports improved hearing in the left ear 32

Happy Ending? Patient returns 4 months later Episodic vertigo, accompanied by tinnitus and aural pressure in the left ear have returned Hearing evaluation shows stable hearing bilaterally Neuro otologist recommends 6 month diuretic trial Under supervision of patient s pediatrician Meniere s Disease Marked by recurrent, spontaneous episodes of: sensorineural hearing loss vertigo aural fullness tinnitus (AAO HNS, 1995) Initial onset typically occurs between 30 and 60 years of age (Rodgers and Telischi, 1997) Definitive diagnosis difficult Pathophysiology remains unknown 33

Meniere s Disease in children Rare in children Incidence in children who complain of dizziness is 1.5 2.9% Little known about progression Symptoms appear to develop over longer period Clinicians must be careful to rule out other disorders with similar clinical presentations (O Reilly, Grindle, 2011) (Choung & Park, 2006) Treatment in Children Same as in adults Salt and caffeine restricted diet first line of therapy Diuretics next line of intervention Under careful supervision Surgical intervention reserved for control of refractory, disabling vertigo Confidence of diagnosis and laterality Rodgers and Telischi, 1997 Implications for the classroom Fluctuating hearing loss Teacher awareness Preferential seating FM system Frequent audiograms Higher levels of school absence All school personnel should be aware of the child s condition Hance, 1990 34

Take Home Messages Meniere s disease is rare in children Take care to rule out other more common causes Definitive diagnosis in any Meniere s case is difficult Vestibular testing can add to the clinical picture in Meniere s disease provide additional evidence of peripheral vestibulopathy aid in determining affected side Questions To ask a question, please type your question into the chat box in the lower left corner of the screen and click on the Send button located right below the box. Thank you! 35

References Choung, YH, Park, K, Kim, CH, Kim, JH, Kim, K. (2006). Rare Cases of Meniere's disease in children. J Laryngol Otol, 120(4): 343 52. Committee on Hearing and Equilibrium guidelines for the diagnosis and evaluation of therapy in Meniere s disease. (1995). American Academy of Otolaryngology Head and Neck Foundation, Inc. Otolaryngol Head Neck Surg, 113(3):181. Hamid, M. (2009). Meniere s disease. Pract Neurol, 9: 157 62. Hance, S. (1990). Meniere's disease in childhood. Language Speech, and Hearing Services in Schools, 21: 132 134. O Reilly, R., Grindle, C. Zwicky, E., and Morlet, T (2011). Development of the vestibular system and balance function: differential diagnosis in the pediatric population. Otolaryngol Clin N Am, 44: 251 271. Rodgers, GK, Telischi, FF. (1997). Meniere's disease in children. Otolaryngol Clin North Am, 30(6): 1101 4. Shepard, N. (2006). Differentiation of Meniere s disease and migraineassociated dizziness: a reivew. J Am Acad Audiol, 17(1): 69 80. 36