The long-term outcome of cases with cochlear implantation due to advanced otosclerosis and van der Hoeve syndrome

Abstract

Both of advanced cochlear otosclerosis and van der Hoeve syndrome (osteogenesis imperfecta) show similar progressive profound deafness and demineralization of the temporal bone surrounding the cochlear capsule. It has recently been reported that the association with COL1A1 in these two diseases may cause similar demineralization. For those patients, cochlear implantation (CI) is the only promising treatment option. However, such Japanese patients are very rare and only a case study on CI insists of relatively poor speech perception result and complications of facial nerve stimulation or unstable T/C level.
Here we reported a long-term outcome in 12 patients with advanced cochlear otosclerosis and van der Hoeve syndrome. We evaluated the speech performance by using Fukuda VTR before 2003 and using by CI-2004 since 2004. As a result, the mean sentence perception score (91%) of 12 cases was better than that (78%) of CI cases with deafness by other causes. Facial nerve stimulation was elicited in only one of 12 cases. Therefore, we concluded that CI can be considered as a treatment option for deaf patients with advanced cochlear otosclerosis and van der Hoeve syndrome.
Furthermore, according to this conclusion, we proposed the strategy flow chart how to select the hearing aid, stapes surgery or CI for Japanese patients with advanced otosclerosis and van der Hoeve syndrome, based on the speech discrimination score, computed tomography classification and the air-bone gap.