Document Detail


Stapedial ankylosis in the Mayer-Rokitansky-Küster-Hauser syndrome.
MedLine Citation:
PMID:  7598861     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We describe a 21-year-old woman with congenital unilateral conductive hearing loss and an atypical form of the Mayer-Rokitansky-Küster-Hauser syndrome. To our knowledge, this is the first reported case of this syndrome in which surgery for congenital stapedial ankylosis was successful. Besides aplasia of the vagina and uterus, the patient also had various other anomalies, such as the Klippel-Feil syndrome, Sprengel's deformity, and congenital stapedial ankylosis. Congenital hearing loss is an associated characteristic of the Mayer-Rokitansky-Küster-Hauser syndrome (10% to 20% of cases), particularly in the atypical form. Against the background of the favorable results of surgery for isolated unilateral congenital stapedial ankylosis and other unilateral congenital anomalies of the middle ear that have been described in the literature and the significant advantages of bilateral hearing, we used stapedectomy to successfully treat this case of Mayer-Rokitansky-Küster-Hauser syndrome with unilateral congenital stapedial ankylosis.
Authors:
C W Cremers; E H Strübbe; W N Willemsen
Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Archives of otolaryngology--head & neck surgery     Volume:  121     ISSN:  0886-4470     ISO Abbreviation:  Arch. Otolaryngol. Head Neck Surg.     Publication Date:  1995 Jul 
Date Detail:
Created Date:  1995-08-10     Completed Date:  1995-08-10     Revised Date:  2006-03-28    
Medline Journal Info:
Nlm Unique ID:  8603209     Medline TA:  Arch Otolaryngol Head Neck Surg     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  800-3     Citation Subset:  AIM; IM    
Affiliation:
Department of Otolaryngology, University Hospital Nijmegen, The Netherlands.
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / diagnosis*
Adult
Ankylosis / congenital*,  diagnosis,  surgery
Ear Diseases / congenital,  diagnosis,  surgery
Female
Genitalia, Female / abnormalities*
Hearing Loss, Conductive / congenital,  diagnosis,  surgery
Humans
Stapes*
Stapes Surgery
Syndrome

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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