Background: Fibrous dysplasia is a benign disease characterized as a progressive replacement of bone by an abnormal fibrous tissue. The temporal bone is involved in 20% of cases where it causes deformity an dysfunction. We present a case of progressive hearing loss due to fibrous dysplasia of the temporal bone.Case Report: A 45-tear-old man who presents with progressive hearing loss over 7 years is reported. Clinical examination showed an extensive stenosis of the right external auditory meatus. The rest of the clinical examination and laboratory tests were within normal limits. Audiometry revealed a mixed hearing loss in the right ear, the pure-tone average was 70 dB. Hearing in the left ear was normal. High resolution Compute tomography (CT) of the skull base demonstrated a bony overgrowth with sclerosis of the right temporal bone. It produced a stenosisof right external auditory meatus.Conclusions: Fibrous dysplasia of temporal bone should be one of the differential diagnosis in the case of a young patient with progressive hearing loss and external auditory canal stenosis.

Keywords: fibrous dysplasia, Temporal Bone, Hearing Loss