Nucleus 21-Channel Auditory Brainstem Implant in Patients with Previous Tumour Removal: El implante auditivo de tallo cerebral Nucleus de 21 canales en pacientes con reseccion tumoral previa

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.     

Multichannel auditory brainstem implantation: the Australian experience

The multichannel auditory brainstem implant (ABI) provides the potential for hearing restoration in patients with neurofibromatosis type 2 (NF2). Programmes for auditory brainstem implantation have been established in two Australian centres. Eight patients have been implanted under the protocol of an international multi-centre clinical trial. Three patients had ABI insertion at the time of first side tumour removal, four at second side tumour removal and one after previous bilateral surgery where there was some residual tumour. The translabyrinthine approach was used in all cases. Successful positioning of the electrode array was achieved in seven of eight patients, all of whom achieved auditory perception with electrical stimulation. Intra-operative electrically evoked auditory brainstem response testing was successful in four patients and was useful in confirming correct electrode position. In six cases post-operative psychophysical and auditory perception testing demonstrated that useful auditory sensations were achieved. Five of these patients regularly used the implant. In one patient electrode placement was unsuccessful and only non-auditory sensations occurred on stimulation. In the remaining patients non-auditory sensations were minimal and avoidable by selective electrode programming. Auditory brainstem implantation should be considered in patients with NF2. The greatest benefit is seen in patients without debilitating disease who have non-aidable hearing in the contralateral ear.     

Hearing habilitation with auditory brainstem implantation in two children with cochlear nerve aplasia

Patients with aplasia and hypoplasia of the cochlear nerve have no chance of having their hearing restored by stimulating the periphery of the auditory system using the traditional cochlear implant. A possible approach to auditory rehabilitation may be direct electrical stimulation of the cochlear nuclei with an auditory brainstem implant (ABI). Recently, two children, aged 4 and 3 years, respectively, with bilateral severe cochlear malformations and cochlear nerve aplasia received an ABI. The present paper reports the technique and the preliminary results of this experience. The classic retrosigmoid approach was used. The correct position of the electrodes was estimated with the aid of EABRs and neural response telemetry (NRT). No postoperative complications were observed. High-resolution CT scans with a bone algorithm reconstruction technique were taken postoperatively to evaluate electrode placement before discharge. The ABI was activated 30 days after implantation in both patients. To date 16 and 13 electrodes, respectively, have been activated in the two children. Three months after activation the first patient had achieved good environmental sound awareness, good speech detection and some speech discrimination. The second child, 1 month after activation, had achieved good environmental sound awareness and moderate speech detection. To the best of our knowledge this is the first report of patients with hypoplasia of the cochlea and aplasia of the cochlear nerve, aged below 5 years and treated with an ABI.     

Auditory brainstem implants: current neurosurgical experiences and perspective

The objective of this study was to present aspects of the current treatment protocol, such as patient evaluation and selection for therapy, multimodality monitoring for optimal auditory brainstem implant (ABI) positioning and radiological evaluation, that might have an impact on the functional results of ABI. Out of a series of 145 patients with bilateral vestibular schwannomas 10 patients received an ABI, eight of which are reported here. Patient selection was based on disease course, clinical and radiological criteria (according to the Hannover evaluation and prognosis scaling of neurofibromatosis type 2 (NF2)), extensive otological test battery and psycho-social factors. ABI placement was controlled by multimodality electrophysiological monitoring in order to activate the auditory pathway and to prevent false stimulation of the cranial nerve nuclei or long sensory or motor tracts. Results of hearing function were correlated with patients' ages, duration of deafness, tumour extension, tumour-induced compression or deformation of the brainstem, and numbers of activated electrodes without any side-effects. Out of 59 patients with pre-operative deafness eight patients received an ABI of the Nucleus 22 type. All these patients became continuous users without any side effects and experienced improved quality of life. Speech reception in combination with lip-reading was markedly improved, with further improvement over a long period. A short duration of deafness may be favourable for achieving good results, while age was not a relevant factor. Lateral recess obstruction may necessitate a more meticulous dissection, but did not prevent good placement of the ABI in the lateral recess. Pre-existing brainstem compression did not prevent good results, but brainstem deformation and ipsi- and contralateral distortion were followed by a less favourable outcome. Among the factors that can be influenced by the therapy management are the selection of patients with a slow progressing NF2 disease, a short duration of deafness, a careful analysis of brainstem deformation and consideration of either side for implantation. Long-standing brainstem deformation might not lead to recovery, but instead lead to a low number of active electrodes and possibly only moderate results. ABI treatment is a safe procedure that can increase a patient's quality of life considerably. ABI placement along with neurophysiological control helps to prevent side effects and to improve acoustic activation. Further studies on structural and functional changes of the brainstem after previous tumour compression and distortion should increase our understanding and facilitate a decision on the best side for ABI implantation.     

The retrosigmoid approach for auditory brainstem implantation

OBJECTIVE: To describe our experience with the retrosigmoid-transmeatal (RS-TM) approach in auditory brainstem implantation (ABI) as well as the anatomosurgical guidelines for this route. STUDY DESIGN: Retrospective case review. SETTING: Ear, Nose, and Throat Department of the University of Verona. PATIENTS: Five patients with neurofibromatosis type 2 (NF2) were operated on for vestibular schwannoma removal with ABI implantation from April 1997 to June 1999. The patients were four men and one woman, whose ages ranged from 22 to 37 years. The tumor sizes ranged from 12 to 30 mm. The records of a total of 179 patients operated on for vestibular schwannoma (VS) removal via the RS-TM approach from January 1990 to June 1999 were also evaluated. Their ages ranged from 18 to 88 years (average 54 years). The tumor sizes ranged from 4 to 50 mm. Five patients had a solitary VS in the only hearing ear. INTERVENTION: The classic RS-TM approach was used in all patients. After tumor excision, for ABI implantation, the landmarks (seventh, eighth, and ninth cranial nerves, choroid plexus) for the foramen of Luschka were carefully identified. The choroid plexus was then partially removed, and the tela choroidea was divided and bent back. The floor of the lateral recess of the fourth ventricle and the convolution of the dorsal cochlear nucleus became visible. The electrode array was then inserted into the lateral recess and correctly positioned with the aid of electrically evoked auditory brainstem responses (EABRs). MAIN OUTCOME MEASURES: Intraoperative EABR and postoperative speech perception evaluation. RESULTS: Auditory sensations were induced in all patients with various numbers of electrodes. Different pitch sensations could be identified with different electrode stimulation. CONCLUSIONS: In the authors' experience, the RS-TM approach is the route of choice for patients who are candidates for ABI when there is a chance of hearing preservation during surgery. If auditory function is lost during surgery, anatomical preservation of the cochlear nerve may allow hearing restoration with a cochlear implant. Direct intraoperative recording of cochlear nerve action potentials (CNAPs) and round window electrical stimulation are mandatory for these purposes. In addition, decompression of the intrameatal portion of the vestibular schwannoma and planned partial tumor resection with hearing preservation are also possible with the RS-TM approach.     

Results from a European clinical investigation of the Nucleus multichannel auditory brainstem implant

OBJECTIVE: This study was designed to investigate the perceptual benefits and potential risks of implanting the Nucleus(R) multichannel auditory brainstem implant. DESIGN: Between September 1992 and October 1997 a total of 27 subjects received a Nucleus 20- or 21-channel Auditory Brainstem Implant (ABI). All subjects involved in the trial had bilateral acoustic tumour as a result of neurofibromatosis type 2 (NF2) resulting in complete dysfunction of the VIIIth nerve. The study used each subject as their own control without a preoperative baseline because residual hearing, if existing, was destroyed at surgery by tumour removal. A battery of speech tests was conducted to evaluate each patient's performance and communication abilities. Tests were conducted, where possible, in the auditory-only, visual-only, and auditory-visual conditions at 3 days postoperatively (baseline), at 3-mo intervals for the first year and every 12 mo thereafter. A subjective performance questionnaire was administered together with an extensive neurological examination at each test interval. RESULTS: 27 subjects involved in this trial were successfully implanted with a Nucleus ABI. One subject died 2 days postoperatively due to a lung embolism unrelated to the device. Twenty-six subjects underwent device activation and all but one patient received auditory sensation at initial stimulation (96.2%). On average 8.6 (+/-4.2) of the available 21 electrodes were used in the patients' MAPs. Performance evaluation measures showed that the majority of users had access to auditory information such as environmental sound awareness together with stress and rhythm cues in speech that assist with lipreading. Although most subjects did not achieve any functional auditory-alone, open-set speech understanding, two subjects from this series (7.4%) did receive sufficient benefit to be able to use the ABI in conversation without lipreading. CONCLUSIONS: Although the medical risks and surgical complexity associated with ABI device implantation are far greater than those for a cochlear implant, the clinical results from this trial show that the Nucleus multichannel ABI is capable of providing a significant patient benefit over risk ratio for subjects suffering loss of hearing due to bilateral retrocochlear lesions.     

Retrosigmoid approach for auditory brainstem implant

The present paper reports our experience with the surgical retrosigmoid-transmastoid (RS-TM) technique for implanting auditory brainstem implants (ABIs). From April 1997 to August 1998, four patients with neurofibromatosis type 2 (NF2) were operated on for vestibular schwannoma removal with ABI implantation. The subjects (three men and one women) ranged in age from 22 to 31 years. Tumour size ranged from 12 to 30 mm. A classical RS-TM approach was performed. After tumour excision, identification of landmarks (VIIth, VIIIth and IXth cranial nerves, choroid plexus) to the foramen of Luschka was carefully carried out. The choroid plexus was partially removed and the tela choroidea divided and deflected. The floor of the lateral recess of the fourth ventricle and the convolution of the dorsal cochlear nucleus became visible. The electrode array was then inserted into the lateral recess and placed in the correct position with the help of electrically-evoked auditory brain stem responses. Auditory sensations were induced in all patients with various numbers of electrodes. Different pitch sensations could be identified with different electrode stimulation. Details of the results are presented. In our series, the RS-TM approach represents the elective route for ABI insertion.     

Stimulation of the cochlear nucleus with multichannel auditory brainstem implants and long-term results: Freiburg patients

Since 1992 18 patients with bilateral retrocochlear deafness have been provided with a multichannel auditory brainstem implant (ABI). The surgical procedure implies tumour removal and ABI implantation in one stage. Most implantations were via the translabyrinthine approach. The long-term follow-up varied between nine and 80 months. In one case auditory perception could not be achieved and in a second case post-operative stimulation was not possible as the subject died due to lung emboli. In all the other cases auditory perception was achieved and only two subjects became non-users during the follow-up period. The presented long-term results suggest that deaf neurofibromatosis type 2 patients regain acoustic contact with the environment, enlarge their communication skills and improve their quality of life by using a multichannel auditory brainstem prosthesis.     

Evaluation of the non-auditory responses in individuals with auditory brainstem implant

ObjectivesAfter auditory brainstem implant (ABI) surgery, stimulation of certain cranial nerves may result in a non-auditory response, and the electrodes that stimulate these nerves may be deactivated. The goals of this study are to compare the number of active electrodes in the initial activation and the last fitting, to investigate non-auditory response types and their frequency as a result of non-auditory stimulation, to compare the placements of deactivated electrodes as a result of non-auditory stimulation in the initial activation and the last fitting.MethodsThe computer software system was used to perform a retrospective analysis of the fitting data of 69 ABI users who underwent auditory brainstem implant surgery between January 1997 and January 2019. The non-auditory response types, deactive electrodes, and the positioning of the deactive electrodes horizontally and vertically were recorded in these users during the initial activation and the last fitting.ResultsThere was no statistically significant difference between the number of active electrodes in the initial activation and the last fitting. The proportion of the users with deactive electrodes in the initial activation and the last fitting was not statistically significant different. In the horizontal and vertical placement classification, the placement of the deactive electrodes was not statistically different between initial activation and last fitting. The most common type of non-auditory response was facial nerve stimulation at the initial activation and no auditory perception at the last fitting. According to the difference between the number of active and deactive electrodes in the initial activation and the last fitting, as well as the auditory and non-auditory responses, it was found that the ABI users were statistically different between the initial activation and the last fitting.ConclusionThe results of this study show that not only auditory but also non-auditory responses occur in most ABI users. In addition, to the best of our knowledge, this study is the first to examine the frequencies of non-auditory response types, and the placement of the electrodes that cause these responses according to horizontal and vertical classifications.     

听觉脑干置入用于双侧听神经瘤全聋患者的听力康复(附1例报告)

目的：探讨听觉脑干置入（ABI）用于双侧听神经瘤全聋患者的听力康复。方法：对1例双侧听神经瘤全聋患者，在经乙状窦枕下径路切除第2侧听神经瘤时，同期将12道听觉脑干装置的电极阵置入第4脑室的侧隐窝内，术中行第Ⅶ、Ⅸ脑神经监测，并且记录电刺激脑干诱发电位，以确定和校正电极位置。术后2个月开通电极并作调试。结果：术后头颅X线侧位片示电极位置正确，术后开通调试发现电刺激12个电极均能引起听觉反应，无一电极引起非听觉反应。结论：多道ABI能让双侧听神经瘤全聋患者产生有意义的听觉。术中电极阵准确地置入到脑干耳蜗核是手术成功的关键。     

Electrically evoked auditory brainstem response monitoring of auditory brainstem implant integrity during facial nerve tumor surgery.

Evoked potentials identified as electrically evoked auditory brainstem responses (EABRs) have been recorded from a patient in response to electrical stimulation of the cochlear nucleus via an auditory brainstem implant. Recording such EABRs during surgery for removal of an ipsilateral facial nerve tumor provided a means to monitor the integrity of the implant. The presence of stable EABRs similar to those obtained before surgery indicated that the lead wires had not been severed and that the implanted electrodes had not been dislodged. EABR recording may also be useful for assisting with positioning the stimulating electrodes during initial implantation surgery, by verifying that stimulation can activate the auditory system.     

多道听觉脑干植入的临床应用

目的　探讨多道听觉脑干植入的手术方法及其对恢复双侧听神经瘤所致全聋患者听力的效果。方法　 7例双侧听神经瘤患者 ,在肿瘤摘除后将 2 1道听觉脑干植入 (auditorybrainstemimplant,ABI)电极植入于第四脑室外侧隐窝内 ,直接刺激脑干耳蜗核 ,术中以面神经电图、舌咽神经肌电图及电诱发听觉脑干反应 (electricallyevokedauditorybrainstemresponses,EABR)确定电极的正确位置。术后 6周开通装置并调试 ,定期行言语识别能力测试。结果　 6例患者术中均能准确定位外侧隐窝 ,并能记录到典型的EABR ;术后均能获得不同程度的言语识别能力 ,其中 2例获得开放句识别能力。另 1例患者术中解剖定位困难 ,术中未能记录到EABR。术后电极刺激 1例无非听觉反应、5例部分电极产生非听觉反应、1例全部电极均产生非听觉反应 ,有非听觉反应的相应电极被关闭。结论　多道听觉脑干植入能使因双侧听神经瘤而全聋的患者产生有意义的听觉 ,术中正确定位脑干耳蜗核为手术成功的关键。     

Auditory brainstem implant in auditory rehabilitation of patients with neurofibromatosis type 2: Hannover programme

An auditory brainstem implant (ABI) is indicated for patients suffering from bilateral neural deafness. The most affected patients are those with neurofibromatosis type 2 (NF2). An implantation is possible either at the same time as, or after, surgical removal of an acoustic neuroma. This paper demonstrates the results of eight out of 11 patients with NF2, seven of whom received an ABI after tumour removal. Pre-operatively, all of them were deaf. Post-operatively, the first fitting served to determine the individual stimulation parameters for each electrode. The stimulation-dependent side-effects were eliminated by reducing the stimulus intensity without causing negative effects on the hearing with the ABI. Only in one case was an open set understanding achieved within the first year. However, all patients had a better speech understanding when they combined their hearing with the ABI and their lip-reading abilities. There is no correlation between the performance with ABI and the tumour size or the duration of deafness.     

Auditory rehabilitation with cochlear implantation in patients with neurofibromatosis type 2

Conclusions: New technological developments will most probably improve the efficiency of auditory brainstem implantation (ABI). Meanwhile, cochlear implantation in patients who have undergone prior reductive surgery, and who have maintained a positive electric stimulation, is an excellent alternative for rehabilitating complete and bilateral hearing loss in patients with neurofibromatosis type 2 (NF2). Auditory results are far better than those reported after ABI. Long-term follow-up will be necessary to demonstrate the validity of this strategy. Objectives: ABIs restore some degree of auditory perception in NF2 patients with bilateral and complete hearing loss, but results are often inadequate for maintaining social and professional activities. The aim of this study was to report the results of auditory rehabilitation by cochlear implantation in three cases of NF2. Patients and methods: This was a retrospective study undertaken in a tertiary referral center. The first patient had undergone previous surgery for a left grade III vestibular schwannoma (VS) and then underwent irradiation for a right grade I VS. Two years after irradiation, he suddenly lost his remaining hearing. Electric promontory stimulation was positive and cochlear implantation was performed. The second patient had undergone surgery for a left grade III VS and followed for a right grade II VS. She suddenly lost her remaining hearing. A cytoreductive surgery was performed and the cochlear nerve was preserved. Postoperative electric stimulation was positive. She was then implanted with a cochlear implant. The third patient presented with a right stage III and a left stage I VS. She first underwent a subtotal removal of the left VS with immediate cochlear implantation. She then underwent removal of the right VS stage III with no possible preservation of the cochlear nerve. Results: All three patients had excellent postoperative speech performance and were back to work 3 months after implantation. Imaging follow-ups at 4, 2, and 1 year, respectively, do not show any evolution of the tumor.     

Auditory brainstem implant in posttraumatic cochlear nerve avulsion

Patients aged over 12 years with neurofibromatosis type 2 are considered candidates for an auditory brainstem implant (ABI). This study extends the indication criteria of ABI to subjects with profound hearing loss due to damaged cochleas and/or cochlear nerves (CNs) following head injuries. In our department, over the period from April 1997 to November 2002, 32 patients, 23 adults and 9 children, were fitted with ABIs. Their ages ranged from 14 months to 70 years. These patients were suffering from a variety of tumor (13 subjects) and nontumor CN or cochlear diseases (19 subjects). Six patients, 5 adults and 1 child, had profound hearing loss following head injury. Their mean age was 25 years (range: 16-48 years). Five were male and 1 female. The retrosigmoid approach was used in all 6 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 and neural response telemetry. Correct implantation was achieved in all patients. No complications were observed due to implantation surgery or related to ABI activation and stimulation of the cochlear nuclei. At activation, an average of 9.8 electrodes (range 5-13) were switched on without side effects. One to 6 electrodes were activated in the following sessions after time periods ranging from 2 to 16 months. All patients achieved auditory-alone-mode closed-set word recognition scores ranging from 40 to 100%; 3 had auditory-alone-mode open-set sentence recognition scores of 60-100%; 2 of these even had speech-tracking performance scores of 38 and 43 words, respectively, showing an ability to engage in normal conversation and converse over the phone. The present study demonstrates that the ABI is a useful rehabilitation instrument in subjects with damaged cochleas and/or CN avulsion following head injury who are unamenable or poorly responsive to auditory rehabilitation using cochlear implants.     

Clinical application of the multichannel auditory brainstem implant]

OBJECTIVE: To investigate the surgical techniques and speech performance of multichannel auditory brainstem implant (ABI) in patients with bilateral acoustic neuromas (neurofibromatosis type 2). METHODS: The nucleus 21 channel auditory brainstem implant was implanted into the lateral recess of the fourth ventricle through the translabyrinthine approach in 7 patients after removal of the tumor. The accurate placement of electrode array was ensured by the electromyogram monitoring of the 7th and 9th nerves and the electrically evoked auditory brainstem responses (EABR). Initial switch-on occurred six weeks postoperatively. Speech evaluation was performed every 3 months for the first year and annually thereafter. RESULTS: During the surgery, the lateral recess could be found and the typical EABR could be recorded in 6 cases. They later reported a significant benefit from the device. Two of the cases have achieved functional open-set speech understanding. In contrast, one patient with no EABR because of difficulty of the anatomic location during the surgery had no sensations postoperatively. CONCLUSION: The multichannel ABI could effectively restore auditory sensations in patients deafened by bilateral acoustic neuromas. The accurate location of the cochlear nucleus complex during surgery was the key factor for the success of the operation.     

Speech perception ability in a patient with a 8-channel auditory brainstem implant]

Auditory brainstem implant (ABI) is a central prosthesis that directly stimulates the cochlear nucleus in the brainstem for those who have interrupted auditory nerves and cannot benefit from the cochlear implantation. Speech perception in a recipient of the Nuclues 8 channel ABI, the first in Japan, is reported. A 25-year-old man with bilateral acoustic nerve tumors postlingually deafened due to tumor resection received auditory sensations with 5 channels. The correct answer using a coding strategy, SPEAK, was 35% for 5 vowels and 36% for 5 monosyllables. The use of ABI also improved his lip-reading ability on monosyllables and open-set words. This indicated that he benefited from ABI, although it was limited. Even after 1 year and 3 months of follow-up, he had no serious side effects such an infection or implant rejection.     

Beneficial auditory and cognitive effects of auditory brainstem implantation in children

CONCLUSION: This preliminary study demonstrates the development of hearing ability and shows that there is a significant improvement in some cognitive parameters related to selective visual/spatial attention and to fluid or multisensory reasoning, in children fitted with auditory brainstem implantation (ABI). The improvement in cognitive paramenters is due to several factors, among which there is certainly, as demonstrated in the literature on a cochlear implants (CIs), the activation of the auditory sensory canal, which was previously absent. The findings of the present study indicate that children with cochlear or cochlear nerve abnormalities with associated cognitive deficits should not be excluded from ABI implantation. OBJECTIVES: The indications for ABI have been extended over the last 10 years to adults with non-tumoral (NT) cochlear or cochlear nerve abnormalities that cannot benefit from CI. We demonstrated that the ABI with surface electrodes may provide sufficient stimulation of the central auditory system in adults for open set speech recognition. These favourable results motivated us to extend ABI indications to children with profound hearing loss who were not candidates for a CI. This study investigated the performances of young deaf children undergoing ABI, in terms of their auditory perceptual development and their non-verbal cognitive abilities. PATIENTS AND METHODS: In our department from 2000 to 2006, 24 children aged 14 months to 16 years received an ABI for different tumour and non-tumour diseases. Two children had NF2 tumours. Eighteen children had bilateral cochlear nerve aplasia. In this group, nine children had associated cochlear malformations, two had unilateral facial nerve agenesia and two had combined microtia, aural atresia and middle ear malformations. Four of these children had previously been fitted elsewhere with a CI with no auditory results. One child had bilateral incomplete cochlear partition (type II); one child, who had previously been fitted unsuccessfully elsewhere with a CI, had auditory neuropathy; one child showed total cochlear ossification bilaterally due to meningitis; and one child had profound hearing loss with cochlear fractures after a head injury. Twelve of these children had multiple associated psychomotor handicaps. The retrosigmoid approach was used in all children. Intraoperative electrical auditory brainstem responses (EABRs) and postoperative EABRs and electrical middle latency responses (EMLRs) were performed. Perceptual auditory abilities were evaluated with the Evaluation of Auditory Responses to Speech (EARS) battery - the Listening Progress Profile (LIP), the Meaningful Auditory Integration Scale (MAIS), the Meaningful Use of Speech Scale (MUSS) - and the Category of Auditory Performance (CAP). Cognitive evaluation was performed on seven children using the Leiter International Performance Scale - Revised (LIPS-R) test with the following subtests: Figure ground, Form completion, Sequential order and Repeated pattern. RESULTS: No postoperative complications were observed. All children consistently used their devices for >75% of waking hours and had environmental sound awareness and utterance of words and simple sentences. Their CAP scores ranged from 1 to 7 (average =4); with MAIS they scored 2-97.5% (average =38%); MUSS scores ranged from 5 to 100% (average =49%) and LIP scores from 5 to 100% (average =45%). Owing to associated disabilities, 12 children were given other therapies (e.g. physical therapy and counselling) in addition to speech and aural rehabilitation therapy. Scores for two of the four subtests of LIPS-R in this study increased significantly during the first year of auditory brainstem implant use in all seven children selected for cognitive evaluation.     

The first successful case of hearing produced by electrical stimulation of the human midbrain.

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.     

Novel method of fitting of children with auditory brainstem implants

ObjectivesTo develop a reliable and objective fitting method for use with young children with an auditory brainstem implant (ABI).Materials and methodsSubjects were 17 young children implanted with an ABI with the mean age 2 years and 4 months (8–64 months). Evoked auditory brainstem response (eABR) measurements were performed intraoperatively and at activation in order to record the auditory response and non-auditory side effects. Each child was tested to observe any subjective responses to the electric stimuli and non-auditory side effects. All children were fitted based on the postoperative eABR. The minimum follow up time was 12 months.ResultsIntraoperatively an eABR could be obtained in all children. The responses were recordable from 75–100% of all electrodes. At initial stimulation eABR were recordable in all children. The eABR was obtained in 79.7% of all electrodes (25–100%) with a mean eABR threshold of 22.3 nC. eABR without any non-auditory stimulation was recorded on all electrodes in 11 children. Mixed eABR and non-auditory responses were recorded on 2–6 electrodes in 6 children. The subjective auditory responses for at least 1 electrode were noted in 15 children. In the 2 remaining cases the auditory response was obtained only when the device was activated. In all children the subjective responses were within the estimated dynamic range for each electrode. Each child was able to accept up to 100% of volume of the created map. The non-auditory response was observed only on children and electrodes with mixed eABR and non-auditory responses. The mean CAP score at 6 months after the activation was 2.4 (1–4).ConclusionseABR seems to be a reliable tool to judge ABI electrode placement and a reliable method for fitting of young children with an ABI. The data suggest that eABR-based fitting helps children to more quickly achieve auditory perception and development.