Immune mediated sensorineual hearing loss in a myositis patient

Hippokratia 2005, 9(3):132-133

P Boura, P Skendros, S Papadopoulos, J Kountouras
Division of Clinical Immunology, 2nd Dpt Internal Medicine, Hippokratio General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece


An unusual case of an immune mediated sensorineural hearing loss in a myositis patient is described. ?24-year-o1d male deve1oped progressive sensorineural hearing loss (SNHL) and subsequently clinical p??ture compatible to acute myositis. He was ??positive. The diagnosis of myositis is based on clinical picture, aldolase levels and electromyogram (EMG) findings although, muscle biopsy was not performed due to the patient’s refusal. Laboratory investigation for infectious myopathies, sarcoidosis as well as systematic vasculitides, Cogan syndrome or secondary myositis due to connective tissue disorders like systemic lupus or Sjogren syndrome was negative. He received high doses of steroids (1.5 mg/kg BW) and clinical status, CRP, aldolase levels and EMG findings were normalized, except for the SNHL where there was no improvement. During the follow-up period the patient had steady transaminasaemia. Although definite autoimmune liver disease was not confirmed by 1iver histology, azathioprine 100 mg daily was added, and transaminases declined to normal values. We conclude that, patients with inflammatory muscle disease and hearing disturbance should be considered to and investigated for immune mediated inner ear disease.