Bilaterale Vestibulopathie

Zusammenfassung

Die bilaterale Vestibulopathie (BV) ist eine oft übersehene vestibuläre Erkrankung, die durch einen kompletten Ausfall oder ein inkomplettes Defizit beider Labyrinthe und/oder der Vestibularisnerven charakterisiert ist. Zur Bestätigung der Diagnose dient in erster Linie der Kopfimpulstest nach Halmagyi und das Elektronystagmogramm mit kalorischer Prüfung. Die Diagnostik sollte durch audiologische Untersuchungen, akustischund vestibulär evozierte myogene Potenziale und gegebenenfalls durch spezifische Laborund Liquoruntersuchungen ergänzt werden. Die drei häufigsten Ursachen der BV sind ototoxische Aminoglykoside, Morbus Menière und Meningitis/ Enzephalitis. Bei der Hälfte der Patienten bleibt die Ursache ungeklärt, idiopathische BV. Bei einem Viertel aller BV-Patienten besteht auch ein zerebelläres Syndrom. Bei etwa 30% dieser Subgruppe finden sich zusätzlich Zeichen einer peripheren Polyneuropathie. Diese Symptomtrias könnte für ein bislang nicht bekanntes neurodegeneratives Syndrom sprechen. Bei mehr als 80% der Patienten kommt es im Verlauf der Erkrankung zu keiner signifikanten Besserung der vestibulären Defizite. Daher sind präventive Maßnahmen, eine schnelle Diagnosestellung und Therapieeinleitung sowie intensive Physiotherapie mit Gangund Gleichgewichtstraining essenziell.

Summary

Bilateral vestibulopathy (BV) is a quite common vestibular disorder which is characterized by an impairment or loss of function of both peripheral labyrinths or of the eighth nerves. When BV is suspected, the diagnosis can be confirmed by the head impulse test and electronystagmography with caloric irrigation. Further, an audiogram and acoustic and vestibular evoked myogenic potentials should be performed. Other specific investigations and laboratory tests have to be initiated according to the suspected cause. The most common causes are ototoxic aminoglycosides, Menière’s disease and meningitis.The etiology of BV remains unclear in 50% of the patients (so-called idiopathic BV). Strikingly, 25% of all patients exhibit a cerebellar syndrome which is associated with a peripheral polyneuropathy in about 30% of this subgroup. This suggests a new syndrome which may be caused by neurodegenerative processes. More than 80% of the patients with BV do not improve during the course of the disease. This emphasis the need for prevention, early diagnosis and rapid initiation of a specific and appropriate therapy as well as intensive physiotherapy with gait and balance training.

Schlüsselwörter

Bilaterale Vestibulopathie - zerebelläres Syndrom - Prognose

Keywords

Bilateral vestibulopathy - cerebellar syndrome - prognosis

• Literatur

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