兰色巩膜、脆骨、耳聋综合症(VonHoeve综合症,又名Eddowes综合症)是一少见的单基因常染色体显性遗传病。其特征是兰色巩膜、反复多发性骨折、耳聋。1831年由Hengschel首先报导,继而国外文献报导甚多。兰色巩膜可伴有园锥角膜、巨大角膜、小角膜、球形角膜。骨质脆性增加易骨折,韧带松驰致使关节过度延伸。牙本质发育不良。国内邓奋刚、周先贡曾报告一家系。 Blue sclera, brittle bone, deafness syndrome (Von Hoeve syndrome, also known as Eddowes syndrome) is a rare single-gene autosomal dominant genetic disease. It is characterized by blue sclera, repeated multiple fractures, deafness. Hengschel first reported in 1831, followed by a lot of foreign literature. Blue sclera may be associated with keratoconus, huge cornea, small cornea, cornea. Increased bone fragility easy to fracture, ligament relaxation caused by excessive extension of joints. Dentition dysplasia. Deng Fen Gang, Zhou Xian Gong had reported a department.