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J Neurol Surg Rep 2014; 75(01): e113-e116
DOI: 10.1055/s-0034-1376200
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Delayed Presentation of a Congenital Cholesteatoma in a 64-year-old Man: Case Report and Review of the Literature

Nishant Davidoss
1   Department of Ear, Nose and Throat, Royal Perth Hospital, Perth, Western Australia, Australia
,
Jennifer Ha
2   Department of Otolaryngology, Princess Margaret Hospital, Perth, Western Australia, Australia
,
Rupan Banga
3   Department of Otolaryngology, Head and Neck Surgery, Fremantle Hospital, Perth, Western Australia, Australia
,
Gunesh Rajan
4   Department of Otolaryngology, University of Western Australia, Crawley, Western Australia, Australia
› Author Affiliations
Further Information

Publication History

23 August 2013

15 March 2014

Publication Date:
24 June 2014 (online)

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Abstract

Introduction Congenital cholesteatomas of the temporal bone are epidermoid cysts of embryologic origin that result in progressive desquamation and trapping of squamous epithelium behind an intact tympanic membrane. They are benign, slowly progressive lesions that can be found in various areas of the temporal bone. We report a case of a patient with a massive cholesteatoma first detected at the age of 64 years, causing significant destruction of the mastoid and petrous temporal bones, and adjacent occipital bone.

Methods We reviewed the literature and a case report of a patient seen in our institution recently. The Medline database was used to search multiple terms including “congenital” and “cholesteatoma.”

Results The patient's congenital cholesteatoma was detected incidentally on a computed tomography scan when the patient's only symptoms were unilateral conductive hearing loss with a family history of hearing loss. It was subsequently successfully operated on with minimal postoperative complications.

Conclusions Congenital cholesteatomas of mastoid origin can often exist for many years in a subclinical state and develop into a massive size before causing symptoms. A high index of suspicion is necessary to detect congenital cholesteatomas in patients with unilateral conductive hearing loss who are otherwise asymptomatic and have a normal tympanic membrane.

Keywords

cholesteatoma - congenital - mastoid - massive - large - incidental - hearing loss - management - review
 

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