Cochlear nucleus auditory prostheses

Persons who lack an auditory nerve cannot benefit from cochlear implants, but a prosthesis utilizing an electrode array implanted on the surface of the cochlear nucleus can restore some hearing. Worldwide, more than 500 persons have received these "auditory brainstem implants," most commonly after removal of the tumors that occur with Type 2 Neurofibromatosis (NF2). Typically, the ABIs provide these individuals with improved speech perception when combined with lip-reading and useful perception of environmental sounds, but little open-set speech recognition. The feasibility of supplementing the array of surface electrodes with penetrating microstimulating electrodes has been investigated in animal studies, and 10 persons with NF2 have received implants that include a surface array and an array of penetrating microelectrodes. Their speech perception is not significantly better than that of the NF2 patients who have only the surface arrays, but the findings do validate the concept of intranuclear stimulation and suggest how such prostheses might be improved by modifying the microstimulating array and also by optimizing the sound processing strategies. Recent publications have described ABI patients with deafness of etiologies other than NF2 who have achieved open-set speech recognition. This suggests that the cochlear nuclei of the NF2 patients are damaged by the disease process or during surgical removal of the tumor.