title: Auditory brainstem implantation improves speech recognition in neurofibromatosis type II patients.
abstract: This prospective study aimed to determine speech understanding in neurofibromatosis type II (NF2) patients following implantation of a MED-EL COMBI 40+ auditory brainstem implant (ABI). Patients (n = 32) were enrolled postsurgically. Nonauditory side effects were evaluated at fitting and audiological performance was determined using the Sound Effects Recognition Test (SERT), Monosyllable-Trochee-Polysyllable (MTP) test and open-set sentence tests. Subjective benefits were determined by questionnaire. ABI activation was documented in 27 patients, 2 patients were too ill for testing and 3 patients were without any auditory perception. SERT and MTP outcomes under auditory-only conditions improved significantly between first fitting and 12-month follow-up. Open-set sentence recognition improved from 5% at first fitting to 37% after 12 months. The number of active electrodes had no significant effect on performance. All questionnaire respondents were 'satisfied' to 'very satisfied' with their ABI. An ABI is an effective treatment option in NF2 patients with the potential to provide open-set speech recognition and subjective benefits. To our knowledge, the data presented herein is exceptional in terms of the open-set speech perception achieved in NF2 patients.
authors.affiliation: Department of Neurosurgery, University of Würzburg, Würzburg, Germany.
authors.forename: Cordula
authors.initials: C
authors.lastname: Matthies
authors.forename: Stefan
authors.initials: S
authors.lastname: Brill
authors.forename: Kimitaka
authors.initials: K
authors.lastname: Kaga
authors.forename: Akio
authors.initials: A
authors.lastname: Morita
authors.forename: Kozo
authors.initials: K
authors.lastname: Kumakawa
authors.forename: Henryk
authors.initials: H
authors.lastname: Skarzynski
authors.forename: Andre
authors.initials: A
authors.lastname: Claassen
authors.forename: Yau
authors.initials: Y
authors.lastname: Hui
authors.forename: Charlotte
authors.initials: C
authors.lastname: Chiong
authors.forename: Joachim
authors.initials: J
authors.lastname: Müller
authors.forename: Robert
authors.initials: R
authors.lastname: Behr
mesh_terms: D000328:Adult; D038263:Auditory Brain Stem Implantation / Q000009:adverse effects / Q000379:methods*; D038262:Auditory Brain Stem Implants; D005260:Female; D006801:Humans; D008085:Lipreading; D008297:Male; D008875:Middle Aged; D016518:Neurofibromatosis 2 / Q000150:complications / Q000601:surgery*; D009464:Neuroma, Acoustic / Q000209:etiology / Q000601:surgery*; D010700:Phonetics; D011446:Prospective Studies; D017755:Prosthesis Fitting; D013063:Speech Discrimination Tests; D013067:Speech Perception; D016896:Treatment Outcome; D055815:Young Adult