Vestibular Paroxysmia

1. Vestibular Paroxysmia
Ade Wijaya, MD – February 2020

2. Outline:
• Introduction
• Pathogenesis
• Diagnostic Criteria
• Diagnosis
• Differential Diagnosis
• Management
• Summary

3. Introduction
• In 1975 Jannetta and colleagues described a “neurovascular cross-compression in
patients with hyperactive dysfunction symptoms of the eighth cranial nerve”
• The term “vestibular paroxysmia” (VP) was introduced by Brandt and Dieterich in
1994
• A rare disease (<1 in 2000 people)
• Prevalence: 4% of all vertigo and dizziness
• The mean years of age: 48-51 years old
• Can occur in children and appears to have often a good long term prognosis with
spontaneous remission with age
Strupp M, Lopez-Escamez JA, Kim JS, Straumann D, Jen JC, Carey J, Bisdorff A, Brandt T. Vestibular paroxysmia: diagnostic criteria. Journal of Vestibular Research. 2016 Jan 1;26(5-6):409-15.

4. Pathogenesis
• ephaptic discharges, i.e. pathological paroxysmal interaxonal transmissions
between neighboring, partially demyelinated axons.
• The likely site of the lesion is the central (oligodendroglia) myelin proximal
to the “transition zone”. This corresponds to the first 15mm after the nerve
exit.
• Potential causes for nerve injury are focal irritation by a blood vessel, tumor
or cyst compression, demyelination, trauma and unidentified causes.
Strupp M, Lopez-Escamez JA, Kim JS, Straumann D, Jen JC, Carey J, Bisdorff A, Brandt T. Vestibular paroxysmia: diagnostic criteria. Journal of Vestibular Research. 2016 Jan 1;26(5-6):409-15.

5. Diagnostic Criteria
• A) At least ten attacks of spontaneous spinning or non-spinning vertigo
• B) Duration less than 1 minute
• C) Stereotyped phenomenology in a particular patient
• D) Response to a treatment with carbamazepine/oxcarbazepine
• E) Not better accounted for by another diagnosis.
Strupp M, Lopez-Escamez JA, Kim JS, Straumann D, Jen JC, Carey J, Bisdorff A, Brandt T. Vestibular paroxysmia: diagnostic criteria. Journal of Vestibular Research. 2016 Jan 1;26(5-6):409-15.

6. Diagnostic Criteria (probable)
• A) At least five attacks of spinning or non-spinning vertigo
• B) Duration less than 5 minutes
• C) Spontaneous occurrence or provoked by certain head-movements
• D) Stereotyped phenomenology in a particular patient
• E) Not better accounted for by another diagnosis.
Strupp M, Lopez-Escamez JA, Kim JS, Straumann D, Jen JC, Carey J, Bisdorff A, Brandt T. Vestibular paroxysmia: diagnostic criteria. Journal of Vestibular Research. 2016 Jan 1;26(5-6):409-15.

7. Diagnosis
• Audiometry: mild to moderate unilateral hypofunction
• A high-resolution MRI with CISS/FIESTA sequences of the brainstem
Chang T.P. , Wu Y.C. and Hsu Y.C. , Vestibular paroxysmia associated with paroxysmal pulsatile tinnitus: A case report and review of the literature, Acta Neurol Taiwan 22 (2013), 72–75.
Hufner K. , Barresi D. , Glaser M. , Linn J. , Adrion C. , Mansmann U. , Brandt T. and Strupp M. , Vestibular paroxysmia: Diagnostic features and medical treatment, Neurology 71 (2008), 1006–1014.

8. Differential Diagnosis
• Menière’s disease
• Tumarkin’s otolithic crisis (“vestibular
drop attacks”)
• Paroxysmal brainstem attacks with vertigo,
dysarthria or ataxia
• Vestibular migraine
• Vertebrobasilar transient ischemic attacks
• Panic attacks
• Perilymph fistula
• Episodic ataxia type 2
• Epilepsy with vestibular aura
• BPPV
• Central positional vertigo/nystagmus,
• “Rotational vertebral artery occlusion
syndrome” (RVAOS)
• Orthostatic hypotension
• Cysts or tumors in the cerebello-pontine
angle
Strupp M, Lopez-Escamez JA, Kim JS, Straumann D, Jen JC, Carey J, Bisdorff A, Brandt T. Vestibular paroxysmia: diagnostic criteria. Journal of Vestibular Research. 2016 Jan 1;26(5-6):409-15.

9. Management
• Carbamazepine (200–800mg/day) or oxcarbazepine (300–900mg/day)
• Phenytoin or valproic acid are possible alternatives
• operative microvascular decompression should be reserved for cases with VP
who respond but do not tolerate the treatment with the above mentioned
drugs and in whom the affected side could be clearly identified because of
the risk of a brainstem infarction due to intra- or post-operative vasospasm.
Strupp M, Lopez-Escamez JA, Kim JS, Straumann D, Jen JC, Carey J, Bisdorff A, Brandt T. Vestibular paroxysmia: diagnostic criteria. Journal of Vestibular Research. 2016 Jan 1;26(5-6):409-15.

10. Summary
• Rare
• Ephaptic discharges
• A high-resolution MRI with CISS/FIESTA sequences of the brainstem
• Carbamazepine (200–800mg/day) or oxcarbazepine (300–900mg/day)

11. THANK YOU