Middle ear deformity in arthrogryposis multiplex congenita

Arthrogryposis multiplex congenita (AMC) is a rare congenital syndrome consisting of two or more joint contractures in different parts of the body. Otolaryngological abnormalities have been previously described in a significant proportion of cases. However, to date, the only frequent otologic deform...

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Bibliographic Details
Published inAnnals of otology, rhinology & laryngology Vol. 103; no. 9; p. 729
Main Authors Quinn, S J, Bleach, N R, Richards, A E
Format Journal Article
LanguageEnglish
Published United States 01.09.1994
Subjects
Adult
Arthrogryposis - pathology
Ear Ossicles - abnormalities
Ear, Middle - abnormalities
Female
Hearing Loss, Bilateral - etiology
Hearing Loss, Conductive - etiology
Humans
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Summary:Arthrogryposis multiplex congenita (AMC) is a rare congenital syndrome consisting of two or more joint contractures in different parts of the body. Otolaryngological abnormalities have been previously described in a significant proportion of cases. However, to date, the only frequent otologic deformity recorded in the published literature has been low-set ears. We present a case with a combination of AMC and a bilateral conductive hearing loss due to an unusual middle ear deformity. To our knowledge, no such case has been previously reported.
ISSN:0003-4894
DOI:10.1177/000348949410300913