KEGG   DISEASE: 遅発性内リンパ水腫
エントリ  
H01706                                                             
名称    
遅発性内リンパ水腫
概要    
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.
カテゴリ  
神経系疾患
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 10 耳・乳様突起の疾患
  内耳疾患
   AB31  発作性前庭症候群
    H01706  遅発性内リンパ水腫
指定難病 [jp08407.html]
 H01706
コメント  
See also H01495 Meniere disease.
リンク   
ICD-11: AB31.0
ICD-10: H81.0
MeSH: D018159
OMIM: 612097
文献    
  著者
Dodson KM, Kamei T, Sismanis A, Nance WE
  タイトル
Familial unilateral deafness and delayed endolymphatic hydrops.
  雑誌
Am J Med Genet A 143A:1661-5 (2007)
DOI:10.1002/ajmg.a.31741
文献    
  著者
Kamei T
  タイトル
Delayed endolymphatic hydrops as a clinical entity.
  雑誌
Int Tinnitus J 10:137-43 (2004)
LinkDB    

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