Atypical presentation of congenital cholesteatoma in an adult case with good hearing result

Abstract

IntroductionCongenital cholesteatoma is thought to be caused by inadequate folding of the epidermoid formation inside the middle ear cleft. During development of the middle ear mucosa, stratified squamous epithelium accumulates in the embryonic life. Its typical appearance is a “pearl” beneath the anterosuperior quadrant of the tympanic membrane.Presentation of caseWe report 28 years-old case with congenital cholesteatoma in the posterosuperior quadrant of middle ear cavity. The main complaint was the hearing loss which had developed slowly over several years.DiscussionThe case was surgically treated. Postoperative hearing result was satisfactory.ConclusionCongenital cholesteatoma may occur in atypical locations and ages. Many authors prefer canal wall down tympanomastoidectomy. But it can also be treated successfully by intact canal wall tympanomastoidectomy with good hearing results.

Isolated Incudostapedial Cholesteatomas: Unique Radiologic and Surgical Features

“…One case report describes a presumed IIC diagnosis in a 28-year-old with similar imaging and clinical features to those in our study. 16 The 5 patients described here presented between 5.5 and 16.0 years (average of 10.7 years), showing that IICs may have a slightly later age of presentation with a wide age range that includes young adults. This can be due to the paucity of symptoms and normal examination, which can delay the diagnosis.…”

“…Newly diagnosed isolated hearing loss in children with unremarkable audiologic history and normal otoscopy, combined with a CT scan showing abnormalities and discontinuity of the ossicular chain, should alert physicians to the possibility of this diagnosis. 16 The CT scans were critical in guiding the diagnosis for all patients in this series. Despite the subtlety in identifying ossicular abnormalities, CT scans remain the imaging study of choice for investigating conductive hearing loss when other etiologies have been ruled out.…”

Periotic congenital cholesteatoma – A rare case report

“…The disease can involve an unusual location in the adults and elderly producing conductive hearing loss and complications. 6 Even though congenital cholesteatoma has a destructive nature, most of them being asymptomatic and appear to be an innocuous keratin pearl. This makes the diagnosis difficult.…”