Temporal Bone Fibrous Dysplasia Complicated by Cholesteatoma of the Middle Ear: A Case Report

Introduction: The temporal bone fibrous dysplasia is a rare osteodystrophy of the temporal bone that can cause obstruction of the external auditory canal and even constriction of the endolymphatic organs and cochlear nerve. The blockage of the external auditory canal can lead to the formation of cholesteatomas in 40% of the patients with temporal bone involvement.

Objective: The objective of this study is to describe a case of the temporal bone with fibrous dysplasia complicated with middle ear cholesteatoma.

Resumed Report: A 29-year-old, black, female patient (R.A.S) presented with a history of chronic otitis media in the right ear since 2005, with several unsuccessful different treatments. In 2008, she reported hearing loss, tinnitus, and dizziness in standing position; and after 5 years, retroauricular pain and serosanguinous otorrhea occasionally were reported. No associated diseases or positive family history for bone fibrous dysplasia or hearing loss was noted. Right ear otoscopy with stenosis of the external auditory meatus and an audiogram showed a severe conductive hearing loss in the right ear. A canal wall down tympanomastoidectomy was performed with complete removal of the cholesteatoma in September 2013. During surgery dehiscent facial nerve canal and sigmoid sinus were observed, but no facial palsy or other complications were observed in the postoperative period.

Conclusion: Fibrous dysplasia of the temporal bone complicated with cholesteatoma of the middle ear is a rare condition and its treatment is based on the cholesteatoma surgical removal. Despite surgical difficulties due to the bone structure, the cholesteatoma removal is necessary, in order to decrease morbidity.