[Exostosis of the internal auditory canal in a patient with myotonic dystrophy].

A 53-year-old patient with myotonic dystrophy presented to our clinic with progressive bilateral hearing loss. The ENT status and particularly the otological examination were without pathological signs. Pure tone audiograms showed a bilateral moderate to severe sensorineural hearing loss. Routinely performed computed tomography of the temporal bones revealed the rare picture of exostosis of the internal auditory canals and the medial surface of the petrous bones. To our knowledge, this is the first report describing exostosis of the internal auditory canal in a patient with myotonic dystrophy, although at present it remains unclear in how far there is a causal connection between these two pathologies.