Best Doctors Physician Webinars. Case Studies in Diagnostic Errors: Our Errors in Diagnosing Dizziness

Best Doctors Physician Webinars Case Studies in Diagnostic Errors: Our Errors in Diagnosing Dizziness

Moderator and Panel Martin Samuels, MD, MSc, FAAN, MACP, FRCP Chairman, Department of Neurology, Brigham and Women s Hospital Professor of Neurology, Harvard Medical School Hugh Calkins, MD Nicholas J. Fortuin M.D. Professor of Cardiology Professor of Medicine Director, Cardiac Arrhythmia Services; Electrophysiology Laboratory; Johns Hopkins ARVD/C Program; Johns Hopkins AF Center President, Heart Rhythm Society Jennifer Derebery, MD Associate, House Ear Clinic, Inc. Clinical Professor, Department of Otolaryngology University of Southern California School of Medicine Cliff A Megerian, MD, FACS Professor and Chairman Otolaryngology-Head and Neck Surgery Case Western Reserve University School of Medicine Director Ear, Nose and Throat Institute Richard and Patrica Pogue Endowed Chair in Auditory Surgery and Hearing Sciences University Hospitals Case Medical Center, Cleveland, Ohio

ACCME Information Our Errors in Diagnosing Dizziness Best Doctors is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide Continuing Medical Education (CME) for physicians. To view this CME activity (webinar), you will need a Windows or Mac operating system and to download the WebEx conferencing software. To claim your CME credit, you will be provided with a brief CME questionnaire. Any information you provide in this questionnaire is confidential though may be used for reporting purposes to the ACCME. If you have questions about this webinar as an ACCME activity, please email physicians@bestdoctors.com.

Disclosure Information Our Errors in Diagnosing Dizziness The panelists on today s webinar have the following financial relationships to disclose: Dr. Hugh Calkins has no relevant financial relationships to disclose Dr. Jennifer Derebery has disclosed that she has the following financial relationships with: Epic Hearing Healthcare Board of Directors Alcon Laboratories Speakers Bureau Sonitus Medical Inc. Board of Directors; Member of Scientific Advisory Board SRxA Advisory board; Speakers Bureau Sunovion Inc. Advisory Board; Speakers Bureau Teva Advisory Board Merck Speakers Bureau Janssen Pharmaceutical Companies Research Support Dr. Cliff Megerian has no relevant financial relationships to disclose Dr. Martin A. Samuels has no relevant financial relationships to disclose None of the Best Doctors staff who assisted in preparing the content of this webinar have relevant financial relationships to disclose No reference will be made to off label use and/or investigational use of pharmaceuticals/devices in this webinar

Dr. Samuels Case 34 year old woman with dizziness, by which she means a sense of impending faint, only occurring in the upright posture. The problem has been present for a couple of years but is clearly worsening in the past few months. Meclizine yields no benefit. She has been told by an autonomic specialist that she has postural orthostatic tachycardia syndrome (POTS). Midodrine causes hypertension but no benefit. Leg crossing with thigh clenching maneuvers have modest benefit but less so in the past three months.

Examination Blood pressure Lying: 130/75 Sitting: 150/90 Standing: 110/65 with symptoms Heart Rate Lying: 72 Sitting: 84 Standing: 110 with symptoms Cardiac examination is normal Mental state is normal Neurological examination is normal

Diagnosis? Pheochromocytoma of the adrenal Postural Orthostatic Tachycardia Syndrome (POTS) Asymmetric septal hypertrophy Takotsubo-like cardiomyopathy Anxiety Paraganglioma of the carotid bulb

Diagnosis? Pheochromocytoma of the adrenal Postural Orthostatic Tachycardia Syndrome (POTS) Asymmetric septal hypertrophy Takotsubo-like cardiomyopathy Anxiety Paraganglioma of the carotid bulb

Principles from Case: Adrenal Pheochromocytoma POTS is a syndrome; not a diagnosis Long term exposure to catecholamines leads to down-regulation of receptors in resistance vasculature (splanchnic and muscle) Highest blood pressure in sitting position suggests a catecholamine secreting tumor in the abdomen Paragangliomas are chromaffin cell tumors outside of the adrenal

Dizziness Panel Jennifer Derebery MD FACS House Clinic Los Angeles, CA

The Balance System Brain Vestibular system Proprioceptive system Visual system

Dizziness - Differential Diagnosis Peripheral Central Systemic Meniere s Disease Acoustic neuroma Cardiac arrhythmia Acute otitis media Brainstem CVA Cardiac valvular dz. Perilymphatic fistula CNS trauma Carotid stenosis Cholesteatoma CNS neoplasm Orthostatic hypoten. Viral labyrinthitis Multiple sclerosis Alcohol intoxication Bacterial labyrinthitis Vertebrobasilar insuff. Sleep deprivation Vestibular neuronitis Motion sickness Med. overdose Ototoxicity Presbystasis Toxin exposure Otologic surgery Psychogenic disorders Hypoglycemia Otologic injury/trauma Arnold-Chiari malform. Autonomic dysf. Otosyphilis CNS infection Hyperventilation BPPV Seizure disorder Panic Migraine

Differential Diagnosis of Dizziness in the Elderly Presbystasis Vestibular loss Polypharmacy CVA/TIA Cardiac Multifactorial Labile BP/Orthostasis BPPV Meniere s Disease Vertebrobasilar insufficiency Duplex Ultrasound study Vestibular system Brain Visual system Proprioceptive system

Urgent Cases CNS (brainstem/cerebellar) infarct CNS (brainstem/cerebellar) hemorrhage CNS infection Complicated otitis media Acute purulent otitis media Chronic otitis media with cholesteatoma

Diagnosis Based on the Temporal Pattern of Symptoms Seconds Minutes Hours Days Constant BPPV, postural, central TIA s, central Meniere s Viral labyrinthitis Metabolic, psychogenic, toxic, central

The Vertigo matrix Vertigo Episodic Continuous Hearing + Meniere s Labyrinthitis loss - BPPV Vestibular neuritis

Recurrent BPPV?Migraine-associated Vertigo Spontaneous or positional vertigo Head motion intolerance Visual vertigo Episodic secs (10%) to minutes (30%) to hours (30%) to several days (30%) Headache Photo, phonophobia

Migraine-associated vertigo The diagnosis of vestibular migraine is based on recurrent vestibular symptoms, a history of migraine, a temporal association between vestibular symptoms and migraine symptoms and exclusion of other causes of vestibular symptoms. Symptoms that qualify for a diagnosis of vestibular migraine include various types of vertigo as well as head motion-induced dizziness with nausea. Symptoms must be of moderate or severe intensity. Duration of acute episodes is limited to a window of between 5 minutes and 72 hours. Lempert T, et al. Vestibular migraine: diagnostic criteria. J Vestib Research. 2012;22(4):167-72.

Migraine-associated Vertigo Any age F > M Family history common Migraine HA s often replaced by vertigo spells in women around menopause

Migraine Associated Vertigo Treatment Beta-blocker Propranolol 10mg po bid TCA s Amitriptyline 25mg po qhs Topiramate 25mg po qhs Acetazolamide Triptans acute Rx Vestibular suppressants Referral to Neurology

My Mistake in Diagnosis Jennifer Derebery MD FACS

Patient 1 89 yo active male is seen in consultation at the request of his primary care MD for evaluation of dizziness and hearing loss.

History Poor daily balance Walks unassisted Has not fallen Some hearing loss (no fluctuation) Family complains No aural symptoms with dizziness C/o fatigue, increase sleepiness last 4 months

PMHx HTN No cardiac history Non smoker No history migraine Parents had hearing loss with age Some occupational noise exposure in distant past

Physical exam Healthy appearing man; arrives with cane and family Falls asleep during exam Weber mid; AC > BC, AU No spont. or gaze-evoked nystagmus Ears normal CN intact Head tilt test negative Romberg/tandem Romberg unable to do tandem gait, wide based stance Orthostatics negative Audiologist had to awaken repeatedly to obtain audiogram

Audiogram

What Next?

My Diagnosis Presbycusis Presbystasis

My Treatment Hearing aid evaluation Niacin Vestibular rehab/ use cane No further evaluation

Patient Outcome Fell 2 weeks later in home Emergency Room MRI: 6 metastatic brain lesions; Primary found to be lung Died in 2 weeks of disease

Note to Self Even with age, unusual to fall asleep so much in exam and audio.

Dr. Calkins Case 25 yo woman Complains of intermittent dizziness She describes the episodes as a sense of imbalance. Occur while standing. Denies syncope

Dr. Calkins Case Prior evaluation: Physical examination BP 110/70, pulse 60, no change on standing no abnormalities present ECG normal Event monitor no arrhythmias seen Echocardiogram - normal

Dr. Calkins Case Tilt Table Test Developed severe presyncope 20 minutes into tilt. SBP fell from 110 to 70, HR fell from 86 to 40 Symptoms of lightheadedness and imbalance reproduced. Treatment Increase salt and fluid Education Sleep with head higher than feet Clinical course - symptoms resolved

Dr. Megerian s Case 37 year old female with VHL syndrome (history of renal cysts, retinal angiomas and pheochromocytoma). 2 years history of fluctuating left sided hearing loss, recent onset of intractable vertigo and left sided tinnitus. Received diagnosis of Meniere s disease.

Dr. Megerian s Case Audiogram Left ear with 55dB SNHL in the low frequencies SRT 50, Discrimination 72% Right ear with normal hearing SRT 5, Discrimination 100% ENG 33% left caloric weakness

Dr. Megerian s Case

Intraductal In-Situ Papillary Hyperplasia Megerian et al., Laryngoscope, 1995, 105: 801-8

Mechanisms of Cochleo-Vestibular Symptoms Lonser et al., NEJM, 2004, 350:2481-6

Dr. Megerian s Case Underwent post auricular mastoidectomy with excision of endolymphatic sac tumor.

Retrolabyrinthine-Transdural Megerian, Haynes et al., Otol & Neurotol, 2002,23:378-87

Transmastoid Retrolabyrinthine Jeffrey et al., J.Neurosurg, 2005, 102:503-12

Transmastoid Retrolabyrinthine Jeffrey et al., J.Neurosurg, 2005, 102:503-12

Transmastoid Retrolabyrinthine Jeffrey et al., J.Neurosurg, 2005, 102:503-12

VHL Autosomal dominant Germline mutation of VHL gene (chromosome 3) Prevalence 1/39,000 Predisposition to benign/malignant visceral and CNS lesions

VHL Visceral neoplasms Renal cell carcinoma and cysts Pheochromocytoma Pancreatic neuroendocrine tumors Reproductive adnexal cystadenoma CNS neoplasms Hemangioblastoma (cerebellum, brainstem, spine) Retinal angioma ELST

VHL and ELST Incidence 11-16% Bilateral 30% ELST associated to Online Mendelian Inheritence in Man VHL disease (No. 193300)

Grade II 40 yo male with ataxia, vertigo, and hearing loss Staged post-fossa and transmastoid resection

Grade IV Presented 6 years later with multiple cranial nerve palsies Tumor involved clivus, cavernous sinus, and sphenoid sinus

Future Issues Early identification in VHL Early excision Role of Gamma Knife Meniere s remains diagnosis of exclusion Sporadic cases outnumber syndromic cases

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