Legent F, Prost A, Beauvillain C, Wallez B, Gallois J R
Ann Otolaryngol Chir Cervicofac. 1976 Jun;93(6):355-65.
Typical of the syndrome described by Muckle and Wells is a combination of progressive perceptive deafness appearing at various ages in a family, but usually at the same age in the same family, arthralgia, urticarious eruption and renal amyloidosis. The existence of incomplete forms, either without deafness or without renal amyloidosis should be known. This perceptive deafness is very probably due to lesion of the internal ear but the histological characteristics do not seem to be very clear at the present time. Finally, as certain of our observations show, in a few cases, conduction may be impaired, either in isolation, or associated with impairment of perception. Only an analysis of a large number of cases will enable the audiological characteristics of this curious syndrome to be defined.
穆克勒（Muckle）和韦尔斯（Wells）所描述的综合征的典型特征是，在一个家族中不同年龄出现进行性感音神经性耳聋（但通常在同一家族中为相同年龄）、关节痛、荨麻疹样皮疹和肾淀粉样变性。应了解不完全形式的存在，即无耳聋或无肾淀粉样变性的情况。这种感音神经性耳聋很可能是由于内耳病变所致，但目前组织学特征似乎不太明确。最后，正如我们的某些观察结果所示，在少数情况下，传导可能单独受损，或与感知障碍相关。只有对大量病例进行分析，才能明确这种奇特综合征的听力学特征。
