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Abstract
The authors have observed 12 cases of Paget's disease with deafness. The form in which it is most frequently found is of a mixed type. The other types of deafness found are all of the perceptive type. Pure transmission deafness in Paget's disease does exist, but is extremely rare. The pathogenesis is complex: stapedo-vestibular ankylosis; involvement of the organ of Corti due to changes in the petrous bone; compression of the nerve in the internal auditory canal; elongation of the auditory nerve in the posterior fossa; compression of the cochlear-vestibular nuclei due to a bending of the cerebral trunk, and finally vascular troubles in particular, at the level of the organ of Corti.
The indications for copho-surgery depend upon the extent of the transmission deafness and the degree of stabilisation in the process of Paget's disease. Moatly labyrinthisation destroys the improvement obtained by surgery.