Non-classical presentation of congenital cholesteatoma

Abstract

Congenital cholesteatoma is a rare entity. It arises from aberrant epithelial remnants left at the time of closure of the neural groove, between the 3rd and 5th week of embryonic life. Otoscopy typically shows a pearly white mass, medial to the intact tympanic membrane, primarily in the anterosuperior quadrant, without a preceding history of otorrhoea, tympanic membrane perforation or surgery. It can present at any age from birth to early adulthood. The most common presenting symptom is hearing loss or facial nerve palsy. We present a non-classical presentation of congenital cholesteatoma with loss of taste sensation and numbness on the anterior two-thirds of the tongue associated with a bulging posterosuperior quadrant of the ipsilateral tympanic membrane in a 52-year-old male. After investigation, the patient underwent surgical management resulting in improvement of his symptoms.

Congenital Intralabyrinthine Cholesteatoma

“…Many theories have been popularized to explain the genesis of congenital cholesteatoma. Incomplete involution or persistence of the epidermoid formation in the middle ear cleft is the most widely accepted theory [ 1 , 7 – 9 ]. Levenson et al [ 10 ] postulate that congenital cholesteatoma results from metaplastic transformation of chronically inflamed middle ear mucosa to keratinizing squamous epithelium.…”

Management of Congenital Cholesteatoma with Otoendoscopic Surgery: Case Report

“…1 It mostly appears in yo ung chil dren, 2,3-5 mostly in boys. 2 It is usu ally a cap su lar cho les te a to ma in the an te ro supe ri or part of the tympa nic ca vity.…”

Clinical aspects and surgical outcomes of pediatric cholesteatoma in a tertiary care teaching hospital

“…16 (45.00%) cases were discovered asymptomatic in a study done by Park, et al 12 CC is usually asymptomatic in the initial stages of growth. 20 Cholesteatomas often exist in a nonaggressive state and asymptomatic, remaining undetected for years before potentially dangerous presentations manifest. 21 According to symptomatology, patients with CC represented with more commonly with hearing impairment followed by tinnitus, otalgia, ear fullness, and facial palsy in this study.…”