Deafness in cryoglobulinemia.
We present a histopathological study of 44-year-old female with essential cryoglobulinemia. She had suffered from purpura and ulcer in winter, bilateral tinnitus and progressive hearing loss. An audiogram taken a week before her death showed bilateral total deafness. In the cochlea of the left temporal bone, the organ of Corti was either missing or present as a mound. The stria vascularis was atrophic throughout the cochlea. The tectorial membrane showed drooping and encapsulation. Reissner membrane was in the normal position. Eosinophilic precipitate was noted in the scala media at the site where Reissner membrane bulged. The spiral ganglion cells were well preserved. In the lower basal turn, there was fibrosis and ossification intermingled in the scala tympani. Ossification was most marked near the basal end. The semicircular canals and vestibule were almost totally ossified and fused with surrounding bone. There was a small, cyst-like structure in the vestibule containing eosinophilic fluid. Our findings indicate that the deafness was the result of circulatory disturbance due to cryoglobulinemia. To our knowledge, this is the first cryoglobulinemia case in which temporal bone findings are reported.
D000328:Adult; D003051:Cochlea / Q000473:pathology; D003449:Cryoglobulinemia / Q000150:complications / Q000473:pathology*; D003638:Deafness / Q000209:etiology / Q000473:pathology*; D005260:Female; D006801:Humans; D010265:Paraproteinemias / Q000473:pathology*; D013701:Temporal Bone / Q000473:pathology