Ear chondritis is more typical and is mostly referred to as otolaryngitis by ENT physicians. It is the first occurrence in about one-third of patients and is present in a single 90 percent of patients during the course of the disease. The auricle is red, swollen, and hot, does not involve the earlobe, occurs unilaterally or bilaterally, lasts for days or weeks, and is not treated with antibiotics. Arthritis is more common, single or multiple joints, with pain in the thoracic ribs, wandering or fluid accumulation. Rhinocochondritis, with a presentation similar to otichondritis, is acute and may include nasal bleeding; repeated attacks may lead to collapse of the nasal bone. All parts of the eye can be involved, mostly manifesting as sclerositis, keratitis, conjunctivitis, and anterior and posterior uveitis. Respiratory tract involvement is a serious type of the disease and can occur from the vocal cords to the secondary trachea; in severe cases, the trachea is collapsed and narrowed. It manifests as refractory cough, hoarseness, shortness of breath or wheezing, and even pressure pain in the tracheal area. Inner ear damage can be unilateral or bilateral, with tinnitus, various types of deafness, and vertigo. Cardiovascular system involvement can present with heart valve damage, conduction block, arrhythmia, aortic aneurysm, or pericarditis. Neurological damage can involve the 2nd (optic), 6th (abducens), 7th (facial), and 8th (auditory) cranial nerves. A variety of skin manifestations may be associated with the disease, such as erythema nodosum, depigmentation, sweet’s, vasculitis-like rash, etc. Other rheumatic diseases, such as rheumatoid arthritis, lupus erythematosus, thyroid disease, ulcerative colitis, etc. Thus, the clinical manifestations of the disease are complex and can be easily misdiagnosed or missed. Therefore, necessary investigations are needed for suspected patients with unexplained, prolonged and recurrent ear, nose, throat and eye diseases.