Experiences from Auditory Brainstem Implantation (ABIs) in four paediatric patients.

INTRODUCTION: Indications for auditory brainstem implants (ABIs) have been widened from patients with neurofibromatosis type 2 (NF2) to paediatric patients with congenital cochlear malformations, cochlear nerve hypoplasia/aplasia, or cochlear ossification after meningitis. We present four ABI surgeries performed in children at Uppsala University Hospital in Sweden since 2009. METHODS: Three children were implanted with implants from Cochlear Ltd. (Lane Cove, Australia) and one child with an implant from MedEl GMBH (Innsbruck, Austria). A boy with Goldenhar syndrome was implanted with a Cochlear Nucleus ABI24M at age 2 years (patient 1). Another boy with CHARGE syndrome was implanted with a Cochlear Nucleus ABI541 at age 2.5 years (patient 2). Another boy with post-ossification meningitis was implanted with a Cochlear Nucleus ABI24M at age 4 years (patient 3). A girl with cochlear aplasia was implanted with a MedEl Synchrony ABI at age 3 years (patient 4). In patients 1, 2, and 3, the trans-labyrinthine approach was used, and in patient 4 the retro-sigmoid approach was used. RESULTS: Three of the four children benefited from their ABIs and use it full time. Two of the full time users had categories of auditory performance (CAP) score of 4 at their last follow up visit (6 and 2.5 years postoperative) which means they can discriminate consistently any combination of two of Ling's sounds. One child has not been fully evaluated yet, but is a full time user and had CAP 2 (responds to speech sounds) after 3 months of ABI use. No severe side or unpleasant stimulation effects have been observed so far. There was one case of immediate electrode migration and one case of implant device failure after 6.5 years. CONCLUSION: ABI should be considered as an option in the rehabilitation of children with similar diagnoses.

Self-reported benefit, sound perception, and quality-of-life in patients with auditory brainstem implants (ABIs).

CONCLUSION: The majority of the patients used their auditory brainstem implants (ABIs) all the time, reporting that he/she would make the decision to receive an implant again if the decision were reconsidered. The findings support that the ABI is a valuable treatment in patients with type 2 neurofibromatosis (NF2) and in children with congenital inner ear and nerve anomalies or cochlear ossification. OBJECTIVE: To evaluate the patients who underwent ABI implantation in Uppsala during 1993-2013. This study analyzed patients' implant use, perception of environmental sounds, perceived benefit from the implant, and quality-of-life (QoL). METHOD: The NF2-patients (n = 20) comprised the majority of the patients, and there were a few non-NF2 pediatric patients (n = 4). The exclusion criteria included deceased patients (n = 4) and patients with no hearing sensations from the implant, or those with an inactivated ABI (n = 2). The data were collected from a questionnaire survey. RESULTS: Eleven adult patients and two pediatric patients answered the questionnaires. Eight of the adult patients used their implants 'always'. The two children always used their implants. Hearing problems had the largest negative effect on the QoL. The non-users and the users scored equally on the NFTI-QoL.

Prognostic value of electrically evoked auditory brainstem responses in cochlear implantation.

OBJECTIVES: The aim of this study was to investigate whether electrical auditory brainstem responses (eABRs) obtained during cochlear implantation (CI) can predict CI outcomes. We also aimed to assess whether eABR can be used to select patients for auditory brainstem implantation (ABI). METHODS: This was a retrospective study. The latencies and quality of the eABR waveforms from adult patients implanted with CI in Uppsala from 2011 to 2013 (n = 74) and four children with severe cochlear abnormalities were analyzed. Speech perception was assessed through postoperative monosyllabic word (MS-word) recognition. A score was constructed for each patient based on wave II, III, and V patency. RESULTS: eABR latencies increased towards base stimulation of the cochlea. Wave V for the mid- and low-frequency regions was the most robust. Significant latency shifts occurred in wave V from the low- to high-frequency regions (**P < 0.01) and from the mid- to high-frequency regions (**P < 0.01). No correlations were found between waveform score, wave V-III interval, wave V latency, and MS-word scores. A negative eABR always predicted a negative outcome. Among the patients with negative outcomes, 75% had eABRs. DISCUSSION: Implant electrical stimulation and brain stem recordings can be used (eABRs wave V) to predict a negative functional outcome. Low-frequency waves V were observed in all patients with successful CI outcomes. Patients for whom eABR waveforms were completely absent had unsuccessful CI outcomes.

Auditory brainstem implants (ABIs)--20 years of clinical experience in Uppsala, Sweden.

CONCLUSIONS: Even though sound perception may be limited after treatment with an auditory brainstem implant (ABI), it provides benefits and should be selectively offered to patients. Importantly the patients must be motivated, given reasonable expectations of outcome and offered long-term rehabilitation with a considerable 'learn to listen' period with the implant device. OBJECTIVES: To describe the clinical experiences and results of 24 ABI surgeries performed in Uppsala University Hospital between 1993 and 2013. METHODS: Most patients (n = 20) suffered from neurofibromatosis type 2 (NF2); a few patients (n = 4) were paediatric non-NF2 patients. The files were searched for information on the presurgery size of the vestibular schwannoma, whether the patient had undergone gamma knife treatment, the surgical approach, the side effects of the surgery and of the use of the implant, the electrode activation pattern and implant use, and categories of auditory performance (CAP) score. RESULTS: Our results show that many patients greatly benefited from an ABI, and most of the patients used their implants even though the hearing improvements usually consisted of awareness of surrounding sounds and improved lip-reading. No severe side effects were observed from implant stimulation.