In fact, we have found a mere 7 reports including more than 5 cases. ĭue to the sparse occurrence of EECC many studies only report on a few typical cases. Whereas these conditions to some extent can be explained by their causes of origin, the aetiology in primary EECC is unknown. In addition, radiation therapy incorporating the ear canal can also lead to EECC. Secondary EECC is related to a variety of conditions mainly postoperative, although factors like recurrent inflammation as well as postinflammatory and posttraumatic stenosis or atresia with ear canal obstruction also occur. One classification has been suggested by Tos: 1) primary EECC, 2) secondary EECC, and 3) cholesteatoma associated with congenital atresia of the ear canal. Hence, EECC may be an insidious entity concealing serious destruction with few or no symptoms.Ĭlassifications of the EECC can be based on pathogenetic theories. Symptoms like otorrhea and pain are often reported, but many cases can be remarkably silent or even asymptomatic. Involvement of the sigmoid sinus and the dura of the tegmen can also be found. These properties characterising the EECC also explain the extension of bony erosion seen in some cases with subsequent invasion of the adjacent structures of the temporal bone, like the mastoid, middle ear cavity and exposure of the temporomandibular joint, and in rare cases dehiscence of the facial nerve and the labyrinth. Their only conclusion was that there are still no reliable consistent symptoms or clinical signs that can differentiate between the two conditions however, the most useful finding confirming an EECC is focal osteonecrosis or sequestration of bone lacking an epithelial covering. reviewed the literature in attempt to define clearer distinctions. While these studies have clarified definitions to some extent, a number of common features also remain, and most recently, Persaud et al. in 1980, and a histopathological study by Naiberg et al. Present definitions have mainly been based on a review by Piepergerdes et al. In fact, the two terms have previously been used interchangeably, but since treatment strategies are different, the distinction between the two conditions is important. While these cases have appeared as EECC, they may also have represented cases of keratosis obturans, which has similar characteristics. EECC presents itself by an accumulation of epithelial debris in the ear canal, and early reports on such manifestations have been made in 1850 by Toynbee and later in 1893 by Scholefield. Whereas the aetiology of secondary EECC can be explained, the origin of primary EECC remains uncertain smoking and minor trauma of the ear canal may predispose.Įxternal ear canal cholesteatoma (EECC) is a rare condition with an estimated incidence of 1.2 per 1,000 new otological patients. Otalgia was the predominant symptom and often related to extension into nearby structures. ConclusionĮECC is a rare condition with inconsistent and silent symptoms, whereas the extent of destruction may be pronounced. In primary EECC 48% of cases reported mechanical trauma. In one primary case the facial nerve was exposed and in a posttraumatic case the atticus and antrum were invaded. In total the temporomandibular joint was exposed in 11 cases, while the mastoid and middle ear was invaded in six and three cases, respectively. Similar symptoms were found in secondary EECC, but less pronounced. Primary EECC showed a right/left ratio of 12/13 and presented with otalgia (n = 15), itching (n = 5), occlusion (n = 4), hearing loss (n = 3), fullness (n = 2), and otorrhea (n = 1). Twenty-five cases were primary, while 23 cases were secondary: postoperative (n = 9), postinflammatory (n = 5), postirradiatory (n = 7), and posttraumatic (n = 2). Overall incidence rate was 0.30 cases per year per 100,000 inhabitants. Resultsįorty-five patients were identified with 48 EECC. Main outcome measures were incidence rates, classification according to causes, symptoms, extensions in the ear canal including adjacent structures, and possible etiological factors. Retrospective evaluation of clinical records of all consecutive patients with EECC in the period 1979 to 2005 in a tertiary referral centre. To evaluate symptoms, clinical findings, and etiological factors in external ear canal cholesteatoma (EECC).
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