Cochlear implantation in a patient with osteogenesis imperfecta and otospongiosis

Tsun Sheng Huang*, Pen Tung Yen, Shu Yu Liu

*Corresponding author for this work

Research output: Contribution to journalJournal Article peer-review

16 Scopus citations

Abstract

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.

Original languageEnglish
Pages (from-to)209-212
Number of pages4
JournalAmerican Journal of Otolaryngology - Head and Neck Medicine and Surgery
Volume19
Issue number3
DOIs
StatePublished - 1998
Externally publishedYes

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