Ossicular abnormality in Larsen's syndrome: a case report.
Larsen's syndrome consists of a skeletal dysplasia with multiple joint abnormalities and a characteristic facies. No reports of this syndrome have documented hearing loss due to ossicular joint abnormalities. A 6-year-old girl is presented with the characteristic abnormalities of Larsen's syndrome, a normal otoscopic examination, and significant bilateral conductive hearing loss. Exploratory tympanotomy demonstrated an incudostapedial joint abnormality, as well as a fixed stapes footplate. The possibility of ossicular joint abnormalities, in addition to the other joint abnormalities associated with this syndrome, must be considered.
Maack
R W
RW
Department of Otolaryngology--Head and Neck Surgery, Washington University, St Louis, MO.
Muntz
H R
HR
D002648:Child; D002972:Cleft Palate; D004429:Ear Ossicles / Q000002:abnormalities*; D005260:Female; D005530:Foot Deformities; D006226:Hand Deformities; D006312:Hearing Loss, Bilateral; D006314:Hearing Loss, Conductive; D006801:Humans; D007592:Joint Diseases / Q000151:congenital*; D004204:Joint Dislocations / Q000151:congenital*; D013577:Syndrome