Cochlear implantation in a patient with osteogenesis imperfecta and otospongiosis

Purpose: We describe the findings in a patient with osteogenesis imperfecta (van der Hoeve syndrome) with otospongiosis who received Nucleus 22-channel (Cochlear Pty, Limited, Australia) cochlear implantation. Cochlear implantation in cases of osteogenesis imperfecta with otospongiosis appears to be...

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Bibliographic Details
Published in:	American journal of otolaryngology 1998-05, Vol.19 (3), p.209-212
Main Authors:	Huang, Tsun-Sheng, Yen, Pen-Tung, Liu, Shu-Yu
Format:	Article
Language:	eng
Subjects:	Adult Biological and medical sciences Cochlear Implantation Cochlear Implants Deafness - diagnosis Deafness - etiology Deafness - rehabilitation Female Head and neck surgery. Maxillofacial surgery. Dental surgery. Orthodontics Humans Medical sciences Osteogenesis Imperfecta - complications Otosclerosis - complications 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:	Purpose: We describe the findings in a patient with osteogenesis imperfecta (van der Hoeve syndrome) with otospongiosis who received Nucleus 22-channel (Cochlear Pty, Limited, Australia) cochlear implantation. Cochlear implantation in cases of osteogenesis imperfecta with otospongiosis appears to be extremely rare. The syndrome itself and otospongiosis are both associated with debilitating hearing impairment. Methods: A female patient who had a triad of brittle bones, blue sclera, and hearing impairment and underwent cochlear implantation was selected for this study. Results: During surgery, otospongiotic feature of hypervascular, reddish middle-ear mucosa was found. While performing cochleostomy, very soft and brittle cochlear bone was noted, which may be the effect of both osteogenesis imperfecta and otospongiosis. Conclusion: Although cochlear implantation is not always indicated in cases such as described herein, these patients with profound deafness may benefit from it.
ISSN:	0196-0709 1532-818X