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Abstract
Three patients with neurofibromatosis type 2 who had undergone previous cochlear nerve tumour removal were implanted with the Nucleus 21-channel auditory brainstem implant (ABI). The time intervals between tumour removal and implantation were 4, 5, and 7 years, respectively. Total bilateral deafness was confirmed before implantation. One patient was also blind after acute intracranial hypertension. The translabyrinthine approach was used in all cases. The choice of side for implantation depended on pre-operative magnetic resonance imaging study, the facial nerve function, the presence of recurrent and/or other lesions, and the patient's preference. Although the scarring of prior surgical procedure largely changed the anatomical structures in the cerebello-pontine angle, various landmarks could be found to locate the foramen of Luschka, where the ABI electrode was inserted into the lateral recess of the fourth ventricle. During surgery, the electrically evoked auditory brainstem responses were recorded to confirm that the ABI stimulation activated the auditory system; the electromyogram of the 7th and 9th nerves was helpful in finding the landmarks and minimising the triggering of the cranial nerves with ABI stimulation. The number of active electrodes was 21, 7, and 4 in the three patients. All obtained meaningful pitch scaling and useful auditory sensations. One patient, with 21 activated electrodes, has achieved functional open-set speech understanding. The second patient, with 7 activated electrodes, has benefited from environmental sound awareness and improved lipreading. The last, blind, patient, with 4 electrodes activated, achieved only perception of environmental sounds.