[Deafness in the Muckle-Wells syndrome]

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.