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Pathophysiology of endolymphatic hydrops

Summary

Endolymphatic hydrops of the nonprogressive type occurs in response to a single traumatic or toxic insult of limited duration and although it may result in permanent deficits in sensorineural function, there is total subsidence of vestibular symptoms. Endolymphatic hydrops of the progressive type, on the other hand, appears to be the result of permanent impairment of endolymph resorption and is caused principally by disorders of the endolymphatic sac. It occurs in Menière's disease, syphilitic labyrinthitis and the delayed hydrops syndrome. In addition to deafness of varying extent, it is characterized by episodic vertigo and sometimes by Hennebert's sign. Histological studies suggest that the acute vertiginous episodes are caused by potassium intoxication following ruptures of the membranous labyrinth and that Hennebert's sign is caused by vestibular fibrosis.

Zusammenfassung

Der endolymphatische Hydrops vorn nicht fortschreitenden Typ entsteht nach einmaligem Trauma oder kurzzeitiger Intoxikation. Obwohl ein bleibender Verlust der Sinnesfunktion eintreten kann, fehlen vestibuläre Symptome. Der endolymphatische Hydrops vom fortschreitenden Typ scheint durch ständige Störung der Endolymphresorption zu entstehen und wird vor allem durch Erkrankungen des Saccus endolymphaticus verursacht. Diese Form findet sich bei der Menièreschen Erkrankung, der syphilitischen Labyrinthitis und beim „Delayed hydrops syndrome“. Neben verschiedengradigen Hörstö rungen findet man hier anfallsartigen Schwindel und manchmal das Henne bertsche Zeichen. Histologische Untersuchungen lassen vermuten, daß die Schwindelanfälle durch Kaliumintoxikation nach Rupturen im membranösen Labyrinth verursacht sind, während das Hennebertsche Zeichen durch eine Fibröse des Vestibulums bedingt wird.

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Schuknecht, H.F. Pathophysiology of endolymphatic hydrops. Arch Otorhinolaryngol 212, 253–262 (1976). https://doi.org/10.1007/BF00453673

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Key words

  • Hydrops
  • Membranous labyrinth
  • Menière's disease
  • Biochemistry
  • Fluid physiology