Deafness in Cryoglobulinemia

We present a histopathological study of a 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.

[1]  U. Fisch,et al.  Polyarteritis nodosa as a cause of sudden deafness. A human temporal bone study. , 1981, American journal of otolaryngology.

[2]  A. Belal.  Pathology of vascular sensorineural hearing impairment , 1980, The Laryngoscope.

[3]  K. Kitamura,et al.  Abrupt (sharp cut) type sensorineural hearing loss--a human temporal bone study--. , 1979, Auris, nasus, larynx.

[4]  R. Gussen,et al.  Sudden Deafnfess of Vascular Origin: A Human Temporal Bone Study , 1976, The Annals of otology, rhinology, and laryngology.

[5]  J. Clauvel,et al.  Biologic and clinical significance of cryoglobulins. A report of 86 cases. , 1974, The American journal of medicine.

[6]  H. Schuknecht,et al.  The pathology of sudden deafness. , 1973, Acta oto-laryngologica.

[7]  P. Verroust,et al.  Glomerular lesions in monoclonal gammopathies and mixed essential cryoglobulinemias IgG-IgM. , 1971, Advances in nephrology from the Necker Hospital.

[8]  A. Cracchiolo,et al.  Cryoglobulinemia and disease. , 1970, Annals of internal medicine.

[9]  H. Schuknecht,et al.  The spiral ganglion in profound deafness. , 1968, Acta oto-laryngologica.

[10]  J. Lindsay,et al.  Inner ear pathology of sudden deafness. Histopathology of acquired deafness in the adult coincident with viral infection. , 1967, Archives of otolaryngology.

[11]  M. Meltzer,et al.  Cryoglobulinemia--a clinical and laboratory study. II. Cryoglobulins with rheumatoid factor activity. , 1966, The American journal of medicine.

[12]  C. Rojer,et al.  Otologic manifestations of waldenstrom's macroglobulinemia , 1966 .

[13]  H. Schuknecht,et al.  Light Microscopic Observations on the Pathology of Endolymph , 1966, The Journal of Laryngology & Otology.

[14]  J. Coe,et al.  Neurologic aspects of Waldenstrom's macroglobulinemia; report of a case. , 1960, Archives of neurology.

[15]  J. Lindsay,et al.  LXVII Inner Ear Pathology in Deafness Due to Mumps , 1960, The Annals of otology, rhinology, and laryngology.

[16]  C. Fernández,et al.  EXPERIMENTS ON TEMPORARY OBSTRUCTION OF THE INTERNAL AUDITORY ARTERY. , 1959, The Laryngoscope.

[17]  J. Lindsay.  Sudden deafness due to virus infection. , 1959, A.M.A. archives of otolaryngology.

[18]  R. Kimura,et al.  I Arterial Obstruction of the Labyrinth , 1958 .

[19]  R. Kimura,et al.  Arterial obstruction of the labyrinth. I. Cochlear changes. , 1958, The Annals of otology, rhinology, and laryngology.

[20]  R. Kimura,et al.  Arterial obstruction of the labyrinth. II. Vestibular changes. , 1958, The Annals of otology, rhinology, and laryngology.

[21]  R. Kimura,et al.  XXVII Extensive Venous Obstruction of the Labyrinth , 1956 .

[22]  R. Kimura,et al.  Extensive venous obstruction of the labyrinth. A. Cochlear changes. , 1956, The Annals of otology, rhinology, and laryngology.

[23]  J. Lindsay,et al.  LIV Inner Ear Pathology Due to Measles , 1954 .

[24]  J. Lindsay,et al.  Inner ear pathology due to measles. , 1954, The Annals of otology, rhinology, and laryngology.

[25]  J. Hutchinson,et al.  Cryoglobulinemia: report of a case associated with gangrene of the digits. , 1953, Annals of internal medicine.

[26]  J. Marrack,et al.  Study of a case of purpura associated with bone changes and formation of a gel in the serum on cooling. , 1950, British journal of experimental pathology.

[27]  G. Reader,et al.  Cryoglobulinemia; report of two cases with discussion of clinical manifestations, incidence and significance. , 1950, Annals of internal medicine.