Vestibular paroxysmia: a treatable neurovascular cross-compression syndrome
J Neurol
Vestibular paroxysmia: a treatable neurovascular cross-compression syndrome
Thomas Brandt 0 1 2 3 4
Michael Strupp 0 1 2 3 4
Marianne Dieterich 0 1 2 3 4
0 Department of Neurology, Ludwig-Maximilians University , Munich , Germany
1 Institute for Clinical Neurosciences, Ludwig-Maximilians University , Munich , Germany
2 & Thomas Brandt
3 Munich Cluster for Systems Neurology (SyNergy) , Munich , Germany
4 German Center for Vertigo and Balance Disorders, Ludwig- Maximilians University , Munich , Germany
The leading symptoms of vestibular paroxysmia (VP) are recurrent, spontaneous, short attacks of spinning or non-spinning vertigo that generally last less than one minute and occur in a series of up to 30 or more per day. VP may manifest when arteries in the cerebellar pontine angle cause a segmental, pressure-induced dysfunction of the eighth nerve. The symptoms are usually triggered by direct pulsatile compression with ephaptic discharges, less often by conduction blocks. MR imaging reveals the neurovascular compression of the eighth nerve (3D constructive interference in steady state and 3D timeof-flight sequences) in more than 95 % of cases. A loop of the anterior inferior cerebellar artery seems to be most often involved, less so the posterior inferior cerebellar artery, the vertebral artery, or a vein. The frequent attacks of vertigo respond to carbamazepine or oxcarbazepine, even in low dosages (200-600 mg/d or 300-900 mg/d, respectively), which have been shown to also be effective in children. Alternative drugs to try are lamotrigine, phenytoin, gabapentin, topiramate or baclofen or other
Vestibular paroxysmia; Neurovascular cross- compression; Vestibular nerve; Episodic vertigo; Carbamazepine; Review
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This manuscript is part of a supplement sponsored by the German
Federal Ministry of Education and Research within the funding
initiative for integrated research and treatment centers.
non-antiepileptic drugs used in trigeminal neuralgia. The
results of ongoing randomized placebo-controlled
treatment studies, however, are not yet available. Surgical
microvascular decompression of the eighth nerve is the
‘‘ultima ratio’’ for medically intractable cases or in
exceptional cases of non-vascular compression of the
eighth nerve by a tumor or cyst. The International Barany
Society for Neuro-Otology is currently working on a
consensus document on the clinical criteria for establishing a
diagnosis of VP as a clinical entity.
Introduction
The main symptoms of vestibular paroxysmia (VP) are
brief attacks of spinning or non-spinning vertigo which
lasts a fraction of a second to a few minutes and occurs
with or without ear symptoms (tinnitus and hypo- or
hyperacusis). Arteries or rarely veins in the cerebellar
pontine angle are the pathophysiological cause of a
segmental, pressure-induced dysfunction of the eighth nerve.
As in trigeminal neuralgia, the symptoms are triggered by
direct pulsatile compression and ephaptic pathological
paroxysmal interaxonal transmission between
neighbouring and possibly in part demyelinated axons [
1, 2
]. VP
accounts for 3.7 % of 17,718 consecutive outpatients of the
German Center for Vertigo and Balance Disorders (Fig. 1).
The syndrome of neurovascular cross-compression of
the eighth nerve was previously connected with so-called
‘‘disabling positional vertigo’’ [
3
], a very heterogeneous
syndrome of vertigo with symptoms of various durations
Fig. 1 Common peripheral vertigo and balance disorders of the
vestibular labyrinth (right) and the vestibular nerve (left) make up
about 47 % of more than 17,000 outpatients in a multidisciplinary
dizziness unit (Brandt et al. 2014). Labyrinthine disorders include
benign paroxysmal positional vertigo due to canalo- or
cupulolithiasis, superior canal dehiscence syndrome, and Menie`re’s disease with
endolymphatic hydrops. Vestibular nerve disorders include superior
(from seconds to days), various characteristic features
(rotatory or postural vertigo, light-headedness or gait
instability without vertigo), and varying accompanying
symptoms. As these vague descriptions also applied to
patients with benign paroxysmal positional vertigo,
Menie`re’s disease, bilateral vestibulopathy, or somatoform
phobic postural vertigo, the clinical definition was
subsequently made more precise and then the term ‘vestibular
paroxysmia’ was introduced [
1
]. Whereas Møller and
Jannetta [
4
] recommended microvascular surgical
decompression with various degrees of success, we introduced the
use of anticonvulsants such as carbamazepine as the
medical therapy of first choice [
1
].
Clinical syndrome
VP is suspected if brief and frequent attacks of vertigo are
accompanied by the following features [
1
]:
• Short attacks of rotatory or postural vertigo last for
seconds to minutes with instability of posture and gait.
• Attacks may often be triggered by particular head
positions or hyperventilation, and may be influenced by
changing the head posi (...truncated)