Maack R W, Muntz H R
Department of Otolaryngology--Head and Neck Surgery, Washington University, St Louis, MO.
Am J Otolaryngol. 1991 Jan-Feb;12(1):51-3. doi: 10.1016/0196-0709(91)90073-o.
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.
拉森综合征是一种伴有多关节异常和特征性面容的骨骼发育不良疾病。尚无关于该综合征因听小骨关节异常导致听力损失的报道。本文报告一名6岁女孩,具有拉森综合征的特征性异常表现,耳镜检查正常,但存在显著的双侧传导性听力损失。中耳探查术显示砧镫关节异常以及镫骨足板固定。除了与该综合征相关的其他关节异常外,还必须考虑听小骨关节异常的可能性。