Delayed endolymphatic hydrops as a clinical entity

Authors

Professor em. Dr Tamio Kamei (Maebashi, Japan)

Proceedings

ASN - Volume 2 - 2004

Topics

Neurootology
Equilibrium
Diseases
Neurootology
Equilibrium
Neurootology

Keywords

Contralateral type, Delayed endolymphatic hydrops, Ipsilateral type, Juvenile unilateral deafness, Labyrinthectomy, Recurrent vertigo.

Download

PDF file, 85kB

Abstract

Delayed endolymphatic hydrops (DEH) is a clinical entity which can be differentiated from Ménière’s disease and is typically observed in patients who have been suffering from longstanding unilateral profound inner ear hearing loss. DEH is probably caused by delayed atrophy or fibrous obliteration of the endolymphatic resorptive system of the membranous labyrinth. The time that elapses between the occurrence of hearing loss and the onset of DEH can range from 1 to 74 years. The most common cause of hearing loss preceding DEH is juvenile (-onset) unilateral profound deafness (early childhood unilateral profound sensorineural hearing loss of unknown etiology), followed by labyrinthitis of various causes as well as physical and acoustic traumas to the inner ear. There are two types of DEH: the ipsilateral type, in which the ear with profound hearing loss suffers progressive endolymphatic hydrops; and the contralateral type, in which the formation of progressive endolymphatic hydrops takes place in the ear opposite to the previously deafened ear. The incidence rate of the ipsilateral type is higher than that of the contralateral type, and the contralateral type is more common in older patients. When recurrent episodic vertigo cannot be remedied through conservative treatment, labyrinthectomy and vestibular neurectomy on the deaf ear are curative for ipsilateral DEH. However, there is no such surgical treatment available for the contralateral type.