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Cochlear implantation for auditory rehabilitation in Camurati-Engelmann disease. Ann Otol Rhinol Laryngol 2000 Feb;109(2):160-2

Date

02/24/2000

Pubmed ID

10685567

DOI

10.1177/000348940010900209

Scopus ID

2-s2.0-0343484975   11 Citations

Abstract

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.

Author List

Friedland DR, Wackym PA, Rhee JS, Finn MS

Authors

David R. Friedland MD Chief, Professor in the Otolaryngology department at Medical College of Wisconsin
John S. Rhee MD Chair, Professor in the Otolaryngology department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Adult
Aged
Camurati-Engelmann Syndrome
Cochlear Implantation
Contraindications
Female
Hearing Loss, Sensorineural
Humans
Male
jenkins-FCD Prod-478 d1509cf07a111124a2d122fd3df854cc0b993c00