There are numerous potential causes of conductive hearing loss (HL). It is important to obtain a thorough history and perform a complete examination, including audiometric testing and radiographic evaluation when necessary. In this report, we present a patient with an intact tympanic membrane, no history of ear disease or trauma who as an adult developed progressive, conductive HL because of an anomalous course of a dehiscent facial nerve. In the patient with a conductive HL and at least partially intact reflexes, superior semicircular canal dehiscence, fracture of the stapes superstructure proximal to the tendon, other third window phenomena, and now dehiscence of the facial nerve resulting in decreased mobility of the ossicular chain must be considered.