Polyostotic fibrous dysplasia in an adult with middle ear cholesteatoma and ipsilateral visual impairment

• We present an adult female with polyostotic fibrous dysplasia (FD). • Facial deformities and decreased vision were the main symptoms observed. • We describe the natural course of FD, including its clinical manifestations, imaging features and treatments. Fibrous dysplasia (FD) is a rare benign fibro-osseous disorder that typically onsets in childhood or adolescence. After puberty, the progression of the skeletal lesions tends to be suspended. Unlike other reported FD patients, we describe an adult female with middle ear cholesteatoma, who gradually experienced a vision decline in right eye at the age of 46, accompanied by facial asymmetry and deformity mainly located around the zygomatic arch, and oculomotor paralysis in right. Besides the involvement of craniofacial bones, another asymptomatic lesion had been detected incidentally in left femur by whole-body bone scintigraphy. The patient has no osteodynia, fracture, cutaneous or endocrine findings which are the common manifestations of FD. A surgical procedure had been subsequently performed to relieve neurologic compression. The specimens from skull base further confirmed the diagnosis of FD histopathologically.