The present paper describes a rare case of a unilateral congenital ossicular abnormality that presented in adulthood. A unilateral conductive hearing loss was revealed during a routine examination for a professional driving license. Differential diagnosis included otosclerosis and ossicular anomalies. Our patient, a 40-year-old man, had no history of otitis media or trauma to the head and he was not aware of his hearing loss. Exploratory tympanotomy revealed absence of the long process of the incus and of the stapes. A remnant of the underdeveloped stapes footplate was removed and reconstruction of the defect using a 5.0-mm malleus-oval window prosthesis had an excellent result with complete restoration of the air-bone gap. The patient experienced binaural hearing with great excitement.

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