Cochlear implantation for auditory rehabilitation in Camurati-Engelmann disease. Ann Otol Rhinol Laryngol 2000 Feb;109(2):160-2
Date
02/24/2000Pubmed ID
10685567DOI
10.1177/000348940010900209Scopus ID
2-s2.0-0343484975 (requires institutional sign-in at Scopus site) 11 CitationsAbstract
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 MSAuthors
David R. Friedland MD Associate Director, Director, Chief, Professor in the Otolaryngology department at Medical College of WisconsinJohn 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
AdultAged
Camurati-Engelmann Syndrome
Cochlear Implantation
Contraindications
Female
Hearing Loss, Sensorineural
Humans
Male
