![]() The eroded area is usually filled by bone sequestrum. On physical examination, this tumor often extends to the floor of the and the posterior wall near the osteochondral junction of the canal. In spontaneous cases, patients do not have any history of otologic symptoms and there is wide variation in frequency and quantity of Otorrhea, otalgia, and facial palsy. The matrix of keratin begins to reshape and form recesses that invade the mastoid and other middle ear structures. It is common to find epidermal epithelial cells surrounding bone sequestrum. ![]() 11 Histopathological studies confirm the presence of periostitis located in the eroded area, periosteitis and bone sequestrums, and granulation tissue. 9, 10Īudiometric state is usually variable it can be found from normal hearing to moderate conductive hearing loss. 3, 6 According to available literature, this disease most often affects elderly patients, is mostly one-sided presentation and there is no gender predominance. ![]() 4Īll these theories converge on the idea that maybe ischemia and secondary bone infection due the keratin accumulation eventually lead to necrosis and reactive periostitis of the bony EAC. The etiology and pathogenesis are still poorly understood, but there are several theories about its origin, including: localized periostitis, chronic inflammation of the EAC, failure in the epithelial cells clearance mechanisms or the dehiscence of the petrotimpanic fissure. 1, 2, 9 It is a diagnostic challenge because of its invasive nature and the wide range of possibilities before the clinician faces against a mass of such characteristics. Case presentationĬEAC is an accumulation of keratin with osteitic and osteloytic capacity. Here, we present a case of spontaneous CEAC with a literature review in order to spread the existence of this rare entity, presenting clinical features and emphasizing proper diagnosis and treatment. Usually accompanied by performing a canaloplasty, mastoidectomy or obliteration of the residual defect, as the case warrants, being sure to remove all bone matrix and necrotic tissue. Treatment involves removal of granulation tissue. 3, 4 Also it must be differentiated from necrotizing otitis externa and malignant tumors such as squamous cell carcinoma of the EAC. For this reason, the pattern of bone erosion, detection of necrosis and sequestrum of the underlying bone is essential to carry out an accurate diagnosis.ĭue to advances in imaging methods, the CEAC can be differentiated from other diseases and inflammatory or EAC tumors processes. ![]() 8īecause the CEAC has no pathognomonic signs or symptoms, often confused with keroatosis obturans during clinical evaluation. 7 Spontaneous CEAC is slightly less frequent, constituting approximately 48% of cases. Its origin may be spontaneous or arise from a previous ear condition or surgical procedure. It is estimated that the CEAC is responsible for 0.1-0.5% of otologic pathology 1, 3 with an incidence of 0.3 cases per year per 100,000 inhabitants that is, sixty times less than its nearest equivalent, ear cholesteatoma of the middle ear. The CEAC is a rare disease, its diagnosis is mainly clinical and is based on imaging studies however, it is often not diagnosed promptly. 5 It differs from the keratosis obturans because it produces a circumferential widening of the EAC, chronic Otorrhea and pain, but no concomitant bone lysis. Cholesteatoma of the External Auditory Canal Cholesteatoma of (CEAC) is defined as an accumulation of keratin that produces periosteitis and bone erosion of its walls 1-4 (usually in their lower portions or above) the eardrum and middle ear sometimes not affected. ![]()
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