Cochlear Implantation for Auditory Rehabilitation in Camurati-Engelmann Disease

Camurati-Engelmann disease (progressive hereditary diaphyseal dysplasia) is a rare sclerotic bone disease involving the diaphyses of the long bones, skull base, and clavicles. Progressive sclerosis of cranial nerve foramina has been implicated in cranial nerve deficits, including facial nerve palsy,...

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Bibliographic Details
Published in:Annals of otology, rhinology & laryngology rhinology & laryngology, 2000-02, Vol.109 (2), p.160-162
Main Authors: Friedland, David R., Rhee, John S., Wackym, Phillip A., Finn, Mary S.
Format: Article
Language:English
Subjects:
Adult
Aged
Biological and medical sciences
Camurati-Engelmann Syndrome - complications
Cochlear Implantation
Contraindications
Ear, auditive nerve, cochleovestibular tract, facial nerve: diseases, semeiology
Female
Head and neck surgery. Maxillofacial surgery. Dental surgery. Orthodontics
Hearing Loss, Sensorineural - etiology
Hearing Loss, Sensorineural - rehabilitation
Humans
Male
Medical sciences
Non tumoral diseases
Otorhinolaryngology. Stomatology
Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases
Surgery of the ear, the auditive nerve and the facial nerve
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Summary:Camurati-Engelmann disease (progressive hereditary diaphyseal dysplasia) is a rare sclerotic bone disease involving the diaphyses of the long bones, skull base, and clavicles. Progressive sclerosis of cranial nerve foramina has been implicated in cranial nerve deficits, including facial nerve palsy, vestibular disturbances, and hearing loss. Two patients with Camurati-Engelmann disease and concomitant sensorineural hearing loss are presented. Both patients were evaluated for cochlear implantation. One patient was successfully implanted after preoperative imaging revealed no involvement of the internal auditory canals. The porous nature of the affected bone, however, necessitated the inactivation of 1 electrode to prevent facial nerve stimulation. A second patient was rejected as a potential implant recipient due, in part, to narrow internal auditory canals and rapidly progressive disease. The otologic manifestations of Camurati-Engelmann disease are reviewed, and issues related to cochlear implantation in this rare disease are discussed.
ISSN:0003-4894
1943-572X
DOI:10.1177/000348940010900209