progressive deafness

Conclusion The authors suggested that the triad of blue sclera, osteopsathyrosis and progressive deafness is the characteristic manifestations of the disease, especially blue sclera, which may be regarded as an initial and suggestible physical sign, and an essential criterion for diagnosis.

结论蓝巩膜、骨脆、进行性耳聋为遗传性成骨不全的特征性表现，尤其蓝巩膜为本病首现及易显体征，为诊断必备条件。

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