Sánchez-Alcón M D, Pérez Garrigues H, Faus J, Muñoz A, Morera C
Servicio de Otorrinolaringología, Hospital Universitario La Fe.
An Otorrinolaringol Ibero Am. 1993;20(4):403-14.
This study deals with 8 cases of progressive deafness in patients with abnormalities in growth and skeletal disease, suffering from Osteogenesis imperfecta (5 of them are a familial case). The work is both and audiological and radiological research of the temporal bone, done in order to uncover the reasons of the patient's deafness. This curses just like otosclerosis with a conductive component (fixation of the stapes) and a sensitive one (cochlear demineralization), starting up in adolescence as bilateral and symmetric process. Because of its progressive nature the AA, recommend as treatment the ear prostheses and refuse the surgery of the stapes.
本研究涉及8例患有生长异常和骨骼疾病的进行性耳聋患者，这些患者患有成骨不全症（其中5例为家族性病例）。这项工作是对颞骨进行听力学和放射学研究，旨在找出患者耳聋的原因。这种疾病的病程类似于耳硬化症，有传导性成分（镫骨固定）和感音性成分（耳蜗脱矿质），在青春期开始时为双侧对称过程。由于其进行性本质，作者建议采用耳部假体治疗，而拒绝进行镫骨手术。
