KEGG   DISEASE: Delayed endolymphatic hydrops
Entry
H01706                      Disease                                
Name
Delayed endolymphatic hydrops
Description
Delayed endolymphatic hydrops (DEH) is a disorder, characterized by episodic vertigo that develops some time after the onset of a profound, typically unilateral sensorineural hearing loss. DEH can be differentiated from Meniere's disease. The age of onset of symptoms is typically in later childhood and adolescence or between 40 and 60 years of age. DEH probably is caused by atrophy or fibrous obliteration of the endolymphatic resorptive system of the membranous labyrinth. It has been reported that genetic factors may sometimes be the cause of this disease. Two types of DEH exist: 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. DEH requires conservative treatment, such as that typically used for Meniere's disease, including the appropriate concomitant use of diuretics, such as isosorbide or steroids for at least 3-6 months. When recurrent episodic vertigo cannot be remedied through conservative treatment, labyrinthectomy and vestibular neurectomy on the deaf ear are curative for ipsilateral DEH. However, no such surgical treatment is available for the contralateral type.
Category
Nervous system disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
 10 Diseases of the ear or mastoid process
  Diseases of inner ear
   AB31  Episodic vestibular syndrome
    H01706  Delayed endolymphatic hydrops
Comment
See also H01495 Meniere disease.
Other DBs
ICD-11: AB31.0
ICD-10: H81.0
MeSH: D018159
OMIM: 612097
Reference
  Authors
Dodson KM, Kamei T, Sismanis A, Nance WE
  Title
Familial unilateral deafness and delayed endolymphatic hydrops.
  Journal
Am J Med Genet A 143A:1661-5 (2007)
DOI:10.1002/ajmg.a.31741
Reference
  Authors
Kamei T
  Title
Delayed endolymphatic hydrops as a clinical entity.
  Journal
Int Tinnitus J 10:137-43 (2004)
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