Non-classical presentation of congenital cholesteatoma as cerebrospinal fluid rhinorrhea – Case report and systematic review of the literature

Abstract

Objective

Congenital cholesteatoma (CC) becomes clinically apparent as a cholesteatoma usually during childhood. Nontraumatic otogenic cerebrospinal fluid (CSF) rhinorrhea with an intact tympanic membrane is a very rare symptom.

Methods

The review of recent literature and case report of the 60-year old patient – a trumpeter presented with nontraumatic otogenic CSF rhinorrhea, intact tympanic membrane on microotoscopy, and besides colorless fluid in right nasal cavity, normal finding on nasal endoscopy examination.

Results

CSF rhinorrhea was caused by CC in the petrous bone apex. Early diagnosis was facilitated by computed tomography scanning. Complete cholesteatoma removal was accomplished using a middle fossa craniotomy and an open non-radical antromastoidectomy.

Conclusion

The diagnosis of otogenic cerebrospinal fluid (CSF) rhinorrhea is challenging and it can easily be misdiagnosed. Congenital cholesteatoma is a rare entity. We present a non-classical presentation of CC in an adult male, with a previously unreported symptom of CSF rhinorrhea. Symptomatic improvement occurred after surgical treatment of the disease.