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Objective: Surgery for hearing restoration with auditory brainstem implantation (ABI) in patients with neurofibromatosis type 2 (NF2) is considered safe, as no increase in postoperative complications related to surgery or device implantation has been observed. Over the last 10 years, we have extended the use of ABI to nontumor (NT) adults and children with cochlear or cochlear nerve malfunctions who would not or did not benefit from a cochlear implant (CI). This article examines the complications encountered in ABI surgery in all patient groups., Study Design: Retrospective case review., Setting: Tertiary referral center., Patients: : One hundred fourteen ABI operations were performed in Verona (Italy) from 1997 to 2008 in 83 adults and 31 children. Thirty-six had NF2 (34 adults and 2 children), and 78 (49 adults and 29 children) had NT cochlear and cochlear nerve disorders., Intervention: A retrosigmoid-transmeatal approach was used in the NF2 cases, and a simple retrosigmoid approach was used in the NT patients., Main Outcome Measures: Surgical complications are tabulated according to standard reporting categories and are presented separately and analyzed statistically for NF2, NT adults, and NT children. Complication rates are compared with those of CI and microvascular decompression surgery., Results: ABI surgery has a very low major complication rate, particularly in non-NF2 patients. Minor complications were easily controlled with complete resolution in all cases. Although the potential complications of intradural ABI implantation are more severe than those of a transmastoid approach for CIs, the actual observed complication rates in ABI candidates without NF2 tumors are comparable to those of CI surgery in both adults and children., Conclusion: This study demonstrates that the ABI is a safe procedure when performed by an experienced surgical and rehabilitation team, and its benefits can be extended to a larger population of deaf patients.

Objectives: Recently, open-set speech recognition performance has been observed with electric stimulation of the brainstem in some nontumor (NT) patients. These outcomes require that we reevaluate the criteria for patient selection and the rationale for expanding the application for the auditory brainstem implant (ABI) to NT adult patients with profound hearing loss. This study presents results from up to 10 years in adults, with analysis based on cause., Materials and Methods: In our Department, from April 1997 to September 2007, ABIs have been implanted in 112 patients (83 adults and 29 children) with tumor (T) and NT disorders. Of the 112 patients, 15 have previously had a cochlear implant elsewhere with no sound detection. This article presents speech recognition results from the 80 patients (32 neurofibromatosis type 2 and 48 NT) who had achieved at least 1-year follow-up. The retrosigmoid approach was used in all NT patients, and the retrosigmoid-transmeatal approach was used in all T patients., Results: All patients had a functioning implantation, and no complications were observed during the operation, activation, or long-term use of the ABI. At the most recent follow-up, NT adults scored from 10 to 100% in open-set speech perception tests (average, 59%), and T patients scored from 5 to 31% (average, 10%). The differences between these results are statistically significant (p = 0.0007). The best performance was observed in patients who lost their nerve VIII from head trauma or severe ossification. Lowest performance (although still highly beneficial to the patient) was observed in patients with neurologic disorders, neuropathy, and cochlear malformations., Conclusion: Our experience clearly indicates that the ABI is an effective tool for hearing rehabilitation in patients with profound hearing loss who cannot be fitted with cochlear implants.

Hypothesis: Electrical stimulation of the inferior colliculus in the midbrain can provide a safe and efficacious alternative to auditory brainstem implants (ABIs)., Background: Patients with neurofibromatosis type 2 (NF2) receive limited speech recognition with ABIs. Some ABI patients without NF2 can achieve excellent speech understanding, suggesting that the limited NF2 performance is due to brainstem damage from the tumor and its removal., Methods: An array of electrodes (Med-El ABI) was placed on the dorsal surface of the inferior colliculus in the midbrain of a human volunteer as an auditory prosthesis via an infratentorial supracerebellar median surgical approach. Electrophysiological responses, psychophysical responses, and speech recognition were measured., Results: Electrical stimulation produced auditory sensations on all 12 electrodes with no nonauditory sensations. Auditory threshold levels indicated the stability of the electrode array over time. Electrophysiological measures showed activation in the contralateral auditory cortex but none in ipsilateral cortex. All electrodes demonstrated a full range of loudness sensation and electrode-specific pitch sensations. Speech recognition was significant, but limited in the first month after surgery., Conclusion: This approach may provide advantages for patients with brainstem damage.

Unlabelled: Previous studies have considered only patients with neurofibromatosis type 2 (NF2) older than 12 years as candidates for an auditory brainstem implant (ABI). Our study expands the potential criteria to include both children and adult subjects with other cochlear or cochlear nerve malfunctions who either would not benefit at all from a cochlear implant (eg, cochlear nerve aplasia or avulsion) or whose benefit was or would be severely compromised (eg, cochlear ossification, cochlear fracture)., Study Design: In our department, over the period from April 1997 to September 2002, 29 patients, 20 adults and 9 children, were fitted with ABIs. Their ages ranged from 14 months to 70 years. Thirteen subjects had tumors, 10 NF2 and 3 solitary vestibular schwannoma, and 16 patients had a variety of nontumor (NT) cochlear or cochlear nerve diseases. A retrosigmoid-transmeatal approach was used in T and a retrosigmoid approach in NT patients. The electrode array was inserted into the lateral recess of the fourth ventricle and correct electrode positioning was monitored with the aid of electrically evoked auditory brainstem responses (EABRs)., Results: Correct implantation was achieved in all patients. No complications were observed due to implantation surgery or related to ABI activation or long-term use. Auditory sensations were induced in all patients with various numbers of electrodes (from 5 to 15). Different pitch sensations were identifiable with different electrode stimulation. Closed-set word recognition, open-set sentence recognition, and speech tracking scores achieved by the patients are reported in detail. The auditory performance of the patients showed significantly better outcomes than controls (Multicentric European clinical investigations on ABI with NF2)., Conclusion: We have shown that the indications for the ABI can be extended to include NT patients with severe cochlear and/or cochlear nerve abnormalities. The degree of auditory benefit varies as a function of the underlying pathological conditions, with NT subjects exhibiting significantly better outcomes than the T patients.

Objective: To investigate the auditory rehabilitative results achieved in five patients with cochlear implants (CIs) who subsequently received, due to poor results, auditory brainstem implants (ABIs)., Material and Methods: Between April 1997 and March 2003, 37 patients (age range 14 months to 70 years) were fitted with ABIs in our ENT Department. Fourteen subjects had neurofibromatosis type 2 and 23 were non-tumor patients who had cochlea or cochlear nerve disease. Five subjects had previously been treated with a CI and received an ABI owing to the poor results achieved. One child had bilateral undiagnosed cochlear nerve aplasia and one was suffering from auditory neuropathy; three adults had total cochlear ossification., Results: The open-set sentence recognition score (auditory-only mode) 6-8 months after ABI activation ranged from 0% to 100% in adults. In 1 subject the speech-tracking score was 56 words/min with the ABI. The two children who had achieved no hearing ability with their CI were able to detect sounds and words as early as 3 months after activation of the ABI., Conclusion: CI failure as a result of anatomical abnormalities can be remedied by an ABI.