Korean J Otolaryngol-Head Neck Surg.  1998 Aug;41(8):1082-1086.

Polyostotic Fibrous Dysplasia with Cholesteatoma on External Auditory Canal and Mastoid

• Shin SO
• Yang SK
• Yoo IS

Abstract

Fibrous dysplasia of temporal bone is a rare disease that may lead to progressive stenosis of external auditory canal. This stenosis leads to trapping of skin and development of cholesteatoma. Most cases with fibrous dysplasia of temporal bone are monostotic, and the polyostotic type is far more rare. We have experienced a recurred polyostotic fibrous dysplasia with cholesteatoma on the external auditory canal and the mastoid. Our purpose of this paper is to discuss properties and management of this rare disease. A twenty-one-year-old woman who had undergone canaloplasty for polyostotic fibrous dysplasia of right temporal bone and occipital bone 5 years ago returned complaining of intermittent right-sided otorrhea for one year. She underwent canal down mastoidectomy with tympanoplasty type 3 for recurred canal obstruction and developing cholesteatoma. The patient has been followed up regularly for one year. There is no evidence of the recurrence of the disease or associated symptoms. We emphasize the need for a long term follow-up of patients with this disease as cholesteatoma or restenosis may develop insidiously.

Keyword

Polyostotic fibrous dysplasia; Temporal and occipital bones; Cholesteatoma

MeSH Terms

Cholesteatoma*
Constriction, Pathologic
Ear Canal*
Female
Fibrous Dysplasia, Polyostotic*
Follow-Up Studies
Humans
Mastoid*
Occipital Bone
Rare Diseases
Recurrence
Skin
Temporal Bone
Tympanoplasty