TY - JOUR
T1 - Cochlear implantation in a patient with osteogenesis imperfecta and otospongiosis
AU - Huang, Tsun Sheng
AU - Yen, Pen Tung
AU - Liu, Shu Yu
PY - 1998
Y1 - 1998
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=0031950585&partnerID=8YFLogxK
U2 - 10.1016/S0196-0709(98)90090-4
DO - 10.1016/S0196-0709(98)90090-4
M3 - 文章
C2 - 9617935
AN - SCOPUS:0031950585
SN - 0196-0709
VL - 19
SP - 209
EP - 212
JO - American Journal of Otolaryngology - Head and Neck Medicine and Surgery
JF - American Journal of Otolaryngology - Head and Neck Medicine and Surgery
IS - 3
ER -