Connective tissue disorders may present with various otolaryngologic symptoms. The manifestations may represent vasculopathy, arthopathy, neuropathy or a combination of pathological processes. Rheumatoid arthritis may involve the middle ear, temporomandibular joints and larynx with mild symptoms or may be life-threatening in instances of cricoarytenoid arthritis. Dermatomyositis with its cutaneous and muscular symptoms prevalent in the head and neck is frequently associated with malignancy in patients over 40. Scleroderma not only affects the gastrointestinal tract but can also produce significant limitations of respiratory function. Systemic lupus erythematosus, polyarteritis modosa, Wegener's granulomatosis and giant cell arteritis produce numerous vascular changes in structures of otolaryngologic significance. In this puzzling group of disorders, protean symptoms may precede the development of a fulminant, widespread and fatal disease.