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

2. 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
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
Jennifer Derebery, MD
Associate, House Ear Clinic, Inc.
Clinical Professor, Department of Otolaryngology
University of Southern California School of Medicine
Martin Samuels, MD, MSc, FAAN, MACP, FRCP
Chairman, Department of Neurology, Brigham and Women’s Hospital
Professor of Neurology, Harvard Medical School
Moderator and Panel

3. ACCME Information
Our Errors in Diagnosing Dizziness
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4. 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

5. 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.

6. 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

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

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

9. 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

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

11. “The Balance System”
Brain
Vestibular
system
Visual system
Proprioceptive
system

12. 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 neuronitisMotion 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

13. 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
Brain
Vestibular
system
Visual system
Proprioceptive
system

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

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

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

17. 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

18. 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

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

20. 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

21. My Mistake in Diagnosis
Jennifer Derebery MD FACS

22. 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.

23. 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

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

25. 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

26. Audiogram

27. What Next?

28. My Diagnosis
• Presbycusis
• Presbystasis

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

30. 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

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

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

33. 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

34. 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

35. 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.

36. 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

37. Dr. Megerian’s Case

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

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

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

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

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

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

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

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

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

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

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

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

50. 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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