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 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.