Audiological findings after multichannel cochlear implantation in patients with Mondini dysplasia

Abstract

Mondini dysplasia is a congenital malformation of the inner ear. To date, five individuals with this malformation have received cochlear implants at the South of England Cochlear Implant Centre. The aim of this study was to review the audiological findings of these individuals after implantation. The soundfield thresholds after implantation are in the region of 30-40 dB (A). The results of suprathreshold speech recognition tasks show substantial variability in performance but this is no greater than that obtained from implant users with no malformation. All individuals were able to detect and recognize a variety of environmental sounds that would previously have been inaudible. These findings, along with the reported improvement in quality of life, mean that Mondini dysplasia is not a contra-indication for multichannel cochlear implantation. This information will be useful to other centres when considering implantation in similar patients.

Radiological diagnosis of incomplete partition type I versus type II: significance for cochlear implantation

“…Previous studies have already shown that cochlear implantation in such cases is possible [1][2][3][10][11][12][13][14][15][16][17][18][19]. However, the diagnosis of the precise malformation of the cochlea is essential, as factors such as selection of electrode type, programming of surgical approach and high risk of cerebrospinal fluid gushing have to be clarified before any intervention [1][2][3].…”

“…Previous studies also suggested cochlear implantation for malformed cases. However, they either used other classifications examining all IP patients under the same group [1,11,[13][14][15]19] or referred to small cohorts or even case reports [3,12,16,18]. Thus, the precise outcome and benefits of the patients in cases of IP-I could not be shown.…”

“…Based on these classifications, numerous studies tried to evaluate the outcome of cochlear implantation in the malformed inner ear, showing that cochlear implantation in such cases is surgically challenging but possible, resulting in most patients in sound and/ or speech perception [1][2][3][10][11][12][13][14][15][16][17][18][19].…”

Cochlear Implantation in Mondini Dysplasia

“…Various authors have reported improvement of auditory perception in several patients with inner ear malformation after cochlear implantation [2,[9][10][11]. In contrast, other authors report that patients with severe inner ear malformation are not suitable candidates for cochlear implantation as there may be an increased risk of accidental penetration of the electrode array through the fundus in the internal auditory canal [12].…”

Audiological performance after cochlear implantation in children with inner ear malformations

“…Although inner ear malformation was once regarded as a contraindication for cochlear implantation [8], studies at different centers [10][11][12][13][14][15][16][17] have proved this treatment not only to be feasible surgically, but also to be beneficial for the children from an audiological point of view.…”