Unusual cases of congenital cholesteatoma of the ear

Abstract

Congenital cholesteatoma may originate at various sites in the temporal bone. For example, in the petrous apex, the cerebellopontine angle, the middle ear cavity, the mastoid process or the external auditory canal. The least common site being the mastoid process. We present two cases of congenital cholesteatoma of the mastoid process, each presenting with different symptoms and at different ages. Both patients underwent surgical treatment, which confirmed the diagnosis and radiological findings.

Congenital cholesteatoma isolated to the mastoid presenting as stricture of the external auditory canal

“…In previous reports [2,[4][5][6][7]9,10], symptoms including neck pain, retroauricular swelling and pain, otorrhea, and dizziness have been reported. Of the five lesions isolated to the mastoid reported by Warren et al [8], two were incidental findings during radiologic evaluations, one presented as ear pain, one as hearing loss, and one as neck mass.…”

“…The most common site of origin is the middle ear cavity, and the most rare is the mastoid process [2][3][4][5][6][7][8][9]. Moreover, congenital cholesteatoma isolated to the mastoid, not involving the middle ear, is very rare.…”

“…Moreover, congenital cholesteatoma isolated to the mastoid, not involving the middle ear, is very rare. From the worldwide literature [2][3][4][5][6][7][8][9][10], we were able to identify only 14 cases of congenital cholesteatoma isolated to the mastoid, including the present case. Warren et al [8] evaluated a series with mastoid congenital cholesteatoma and reported that 5 of 9 cases were isolated to the mastoid.…”

“…Congenital cholesteatoma is an epidermoid cyst arising from congenital remnants of keratinizing squamous epithelium in the temporal bone, although the etiology remains controversial [2][3][4]. The most common site of origin is the middle ear cavity, and the most rare is the mastoid process [2][3][4][5][6][7][8][9]. We report herein an extremely rare case of congenital cholesteatoma that was isolated to the mastoid and presented as stricture of the external auditory canal and provide a review of the literature regarding congenital cholesteatoma isolated to the mastoid.…”

Congenital Cholesteatoma of Temporal Bone with Bezold’s Abscess: Case Report

“…1 Absence of medial wall of mastoid antrum in the region of Trautman's triangle and sinus plate bony deficiency in the posterior meatal wall Fig. 2 Intra operative photo clip showing delineated cholesteatomatous sac from dura limited to mastoid antrum, which is considered the least common site of CC [10]. Here the patient was unaware of possible lethal nature of the disease because of the mild symptoms like dull pain in the temporal region.…”