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Melnick和Needles氏所描述的骨发育异常是一种少见的遗传性疾病。表现为前额突出,错(牙合),颌小,脊柱后侧突,膝外翻,前臂呈弓形,和指端缩短。X线片可见肋骨、长骨呈不规则条状缩窄,严重的髋外翻和骨盆畸形,颅底骨及乳突硬化,鼻窦呈原基状(未气化),长骨的骨皮质有斑片状硬化。作者报告一例12岁男孩。除有骨发育异常外,尚有小耳、耳廓变形、耳道狭窄、双侧传导性聋、500~4000赫的镫骨反射消失,鼓室顺应性减低,中耳有积液。右鼓膜穿刺有渗液,手术探查见听小骨正常,但无蜗窗。左耳鼓膜切开有渗液,未作进一步探查,术后纯音听力无改变。
Bone dysplasia, described by Melnick and Needles, is a rare genetic disorder. The performance of prominent forehead, wrong (occlusion), jaw small, spinal dorsal, knee valgus, arched forearm, and the shortening of the thumb. X-ray film shows ribs, long ribs showed irregular strip narrowing, severe hip valgus and pelvic deformity, skull base and mastoid sclerosis, sinus primitive primordial (non-gasification), long bone cortical plaque Hardened. The author reports a 12-year-old boy. In addition to bone dysplasia, there are small ears, auricle deformation, narrowing of the ear canal, bilateral conductive deafness, tarsal reflex 500-4000 Hz disappeared, tympanic compliance decreased, the middle ear effusion. Right tympanic membrane perforation, surgical exploration to see the normal bone, but no snail. Left ear tympanic membrane incision with exudate, no further exploration, postoperative pure tone hearing no change.