Cholesteatoma: Congenital and Acquired (original) (raw)

CHOLESTEATOMA, a progressively enlarging accumulation of keratinizing squamous epithelium within the temporal bone, is a disease process which is of common interest to the pediatrician, family practitioner, and otologist. These lesions may be classified into congenital and acquired varieties,1,4,9 as noted in Table I. While the acquired types are relatively common, congenital cholesteatomas remain asymptomatic until erosion into important structures in the temporal bone, or secondary infection arouses suspicion of their presence. Those arising in the middle ear can frequently be diagnosed by otoscopy prior to the onset of significant destruction. Four cases of congenital cholesteatoma are reported. The diagnosis, pathophysiology, and management of this condition is reviewed in an effort to stimulate renewed awareness of congenital cholesteatoma, and facilitate early diagnosis and treatment prior to the development of extensive otologic disease. R~port of Cases Case J. A two year old male was referred for evaluation of recurrent episodes of bilateral, acute otitis media. There was no past history of otorrhea or hearing loss. Examination showed bilateral pearly white masses in the anterior-superior quadrant of each mesotympanum (Fig. 1). The tympanic membranes were intact, translucent and slightly retracted. Surgical exploration of the right middle ear disclosed an easily removable three to four mm spherical mass attached to the anterior portion of the neck of the malleus (Figs. 2 and 3). Six weeks later, surgical exploration of the left middle ear showed a similar lesion. Histological examination of both masses was compatible with the diagnosis of cholesteatoma. Case 2. A 26 month old male was referred because of an abnormal otologic examination. There were no symptoms of otologic disease. Examination revealed a white mass in the anterior-superior quadrant of the right mesotympanum. Surgical exploration disclosed a three to four mm mass attached to the undersurface of the tympanic membrane which was removed without difficulty. By histologic examination, the diagnosis was cholesteatoma. Case 3. A five year old male was referred in 1971 for an evaluation of serous otitis media. The tympanic membranes were intact, dull, and retracted. Pure tone audiometry showed a 25dB conductive loss in the right ear and 15dB loss in the left. Myringotomies were performed. Ten months later, hearing in the left ear deteriorated to a 53dB conductive loss. The tympanic membranes were opaque and tympanosclerotic, and myringotomies were again performed. Two years later, due to persistant conductive loss and "thickening" of the posterior-superior quadrant of the left tympanic mem-From the