The results in patients implanted with the nucleus double array cochlear implant: pitch discrimination and auditory performance.

OBJECTIVE: In patients with total or surgically inaccessible cochlear obliteration, only a reduced number of active electrodes can be inserted with standard cochlear implants, resulting in below average auditory performance. Therefore, a special implant with two electrode arrays was developed on the basis of the Nucleus 22 cochlear implant, the socalled Double Array. One electrode array with 11 active electrodes is inserted into the basal turn of the cochlea, while the second array with 10 active electrodes is inserted into the second turn. The Double Array is now available on the basis of the more advanced Nucleus 24 with 11 active electrodes on each array and two reference electrodes, one at the case and the second one an additional ball electrode, which is placed under the temporalis muscle. For device description and surgical technique see Lenarz et al. (2001). This paper presents psychophysical data on pitch discrimination and auditory performance of patients implanted with a Double Array on the basis of the Nucleus 22. STUDY DESIGN: A prospective intra-individual study using a Latin square paradigm was performed in six adult patients with obliterated cochlea who received the Nucleus 22 Double Array. After appropriate fitting and loudness balancing, patients were tested either with the basal, the apical or both electrode arrays. Apart from auditory performance tests including numbers and monosyllable word tests, pitch discrimination was determined with a defined procedure. RESULTS: When activating each array alone, auditory performance was better with the basal array than with the apical array. Both arrays together showed marked improvement compared with the basal array, indicating an additional effect of the second array. Pitch discrimination was significantly better for the electrodes in the basal turn than in the second turn, indicating differences in electrical excitation of the auditory nerve fibers. Pitch discrimination was positively correlated with auditory performance data. CONCLUSION: The additional apical array leads to significant improvement in auditory performance in patients with obliterated cochleae by increasing the number of intracochlear electrodes. Despite reduced pitch discrimination, the apical array provides important information for speech recognition. For this reason the Double Array provides a profound advantage for patients with obliterated or surgically inaccessible cochleae.

Auditory brainstem implant: part I. Auditory performance and its evolution over time.

OBJECTIVE: Evaluation of auditory performance and its evolution over time in patients with the auditory brainstem implant. STUDY DESIGN: Prospective study. SETTING: Tertiary referral center. PATIENTS AND METHODS: Between May 1996 and April 2000, 14 patients with neurofibromatosis type 2 underwent implantation with a multichannel auditory brainstem implant. Auditory performance data were obtained in 13 patients who had used their device on a regular daily basis for 1 to 41 months (average 19 months). Hearing evaluation was based on the results of four tests (vowel confusion, consonant confusion, Freiburger numbers, and speech-tracking test), which were performed with and without lip-reading at regular intervals after device activation. RESULTS: 12 patients received auditory sensation through the auditory brainstem implant immediately after device activation. In one patient, because of postoperative electrode migration, device activation was not successful. In this case, after the electrode array was repositioned, activation was successful. The results of the audiovisual mode 2 weeks after device activation revealed a lip-reading enhancement above the chance level in about 50% of the patients in the vowel confusion and speech-tracking tests and in 70% of the patients in the consonant confusion test. Lip-reading enhancement improved within the first 6 months and then entered a plateau phase, which was more prominent in the monosyllabic vowel and consonant tests. In the auditory alone mode, more than half of the patients showed their first positive result in the vowel test 3 months after device activation, but it took about 6 months until half of the patients revealed a result above the chance level in the consonant and Freiburger numbers tests. Open set speech recognition in the auditory alone mode (in the speech-tracking test) was not common and happened relatively late (within 1 year or later). DISCUSSION AND CONCLUSION: Although auditory sensation appeared immediately after device activation, a period of 6 months was necessary for relearning and adaptation of the central auditory system to the altered form of auditory information presented by the auditory brainstem implant.

[Subjective deafness in case of peri-synaptic audiopathy. Isolated defects of the inner haircells?]. / Funktionelle Taubheit bei peri-synaptischer Audiopathie - Isolierte Störungen der inneren Haarzellen?

BACKGROUND: Damage to or functional impair of the inner hair cells, synapsis or dendrites of the ganglion cells of the auditory nerve result in specific audiometric findings. Due to the normal function of the outer hair cells otoacoustic emissions can be registered, ABR and ECochG show at least elevated thresholds or are absent. PATIENTS: We demonstrate 5 cases with these audiological findings described in the literature as Auditory Neuropathy. RESULTS: All patients have profound to severe hearing loss with poor speech understanding under best aided conditions with conventional hearing aids. 3 patients, which were implanted with a cochlear implant have speech understanding but one prelingually adult, also implanted, has only sound identification. CONCLUSIONS: Hypoxia, carboplatin, ototoxicity and metabolic disorders are possible etiologies for damage to the inner hair cells or synapsis. The results will be discussed with reference to the localisation of the pathology and the definition as Auditory Neuropathy.

The nucleus double array cochlear implant: a new concept for the obliterated cochlea.

OBJECTIVE: To increase the number of intracochlear electrodes that may be inserted into a totally obliterated cochlea, a special implant has been developed in collaboration with Cochlear Limited. This implant features two separate electrode carriers containing 11 and 10 active electrodes, respectively, as well as a reference electrode located on the receiver-stimulator package. The potential stimulation modes available with this device therefore include monopolar and bipolar stimulation, and stimulation between both arrays. SURGICAL TECHNIQUE: A cochleostomy anterior to the round window provides access to the basal turn (both the scala tympani and the scala vestibuli), and new built connective tissue and bone can be removed until the anterior wall of the basal turn is approached. A second cochleostomy is performed at the second turn caudal of the cochleariform process and 2 mm anterior of the oval window after removal of the incus. New tissue should also be removed if necessary. The two electrode carriers are then placed into the scala tympani of the basal and the scala vestibuli of the second turn, respectively. The remaining surgical procedure is identical with that used for cochlear implantation in patients without obliterated cochleas. PATIENTS: In this clinical study, 10 patients aged 32 to 66 years with an obliterated cochlea each received a double array cochlear implant. All patients had total obliteration of the basal turn either on preoperative imaging or during surgery. Intraoperatively, the second turn was not obliterated in only 4 of 10 patients. Postoperatively, a standard audiologic test battery was used to determine auditory improvement over time. POSTOPERATIVE RESULTS: All patients achieved significantly improved speech understanding when the additional apical electrode array was used, compared with the use of each electrode array independently. No complications occurred. CONCLUSION: In patients with a totally obliterated cochlea, the number of intracochlear electrodes can be increased by use of the Nucleus double array implant. As a result, patients achieve significantly better auditory results.

Extensive monitoring during auditory brainstem implant surgery.

In patients with reduced auditory nerve function, for example due to tumour removal or an accident, hearing rehabilitation can be elicited by an auditory brainstem implant (ABI). The electrode array of the ABI manufactured by Cochlear Ltd., Sydney, consists of 21 circled contacts in a silicon carrier. This is inserted in the lateral recess of the fourth ventricle. Since 1996, in Hannover eight patients have been implanted with a cochlear ABI Nucleus 21 + 1. All of them were profoundly deaf on both sides due to neurofibromatosis type 2 (NF2). To find the optimal electrode position during surgery, a multimodal monitoring by auditory evoked potentials (AEP), electromyography (EMG) and somatosensory evoked potentials (SEP) was performed. When monitoring AEPs, the function of the implant can be checked first by the stimulus artefact. By analysing the AEPs in more detail, the optimal positioning of the electrode on the cochlear nucleus can be found. If systems other than the auditory system are stimulated this will be revealed in one or more of the AEP, EMG and SEP recordings. According to the literature, AEPs stimulated by an ABI consist of three vertex positive peaks with latencies shorter than 4 ms. Typical AEPs are correlated with good post-operative hearing sensation. Comparing these AEPs with AEPs stimulated acoustically or electrically at different sites of the auditory system, it can be assumed that the first peak corresponds to J3, the second to J4 and the last to J5. From this comparison it can also be concluded that no potentials should occur later than 5 ms. This corresponds to our findings. Post-operatively, side-effects occurred when areas of the electrode array were stimulated that showed potentials with latencies longer than 5 ms intra-operatively. Our results indicate that monitoring is an essential aid for the surgeon in finding the optimal electrode position. Positioning solely with reference to anatomical landmarks may not be enough to find the optimal functional position.

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 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 Nucleus Double Array Cochlear Implant: a new concept in obliterated cochlea]. / Das Nucleus Double Array Cochlear Implant: Ein neues Konzept bei obliterierter Cochlea.

AIM: In order to increase the number of intracochlear electrodes to be inserted into a totally obliterated cochlea a special implant has been developed in collaboration with Cochlear Ltd. The implant features two separate electrode carriers containing 11 and 10 active electrodes, respectively, and a reference electrode on the receiver stimulator package. The potential stimulation modes include monopolar and bipolar stimulation as well as stimulation between both arrays. SURGICAL TECHNIQUE: A cochleostomy at the round window provides access to the scala tympani. Newly formed bone is removed as far as the anterior portion of the basal turn. Care is taken to identify and preserve the osseous border of the cochlea. A second cochleostomy is performed immediately caudal to the cochleariform process after removal of the incus. New tissue can be removed here in the same way. The two electrode carriers are then placed into the first and the second turn respectively. The remaining procedure corresponds to the procedure for cochlear implantation in cases in which the cochlea is not obliterated. PATIENTS: For the purpose of a clinical study n = 10 patients aged between 32-66 years with an obliterated cochlea were fitted with a double array cochlear implant. All patients showed signs of total obliteration of the basal turn either in preoperative imaging or during surgery. Intraoperative inspection revealed that the second turn was not obliterated in 4 of 10 patients. POSTOPERATIVE RESULTS: Postoperatively, a standard test battery was used to determine auditory performance over a period of time. All patients achieved significantly better speech understanding due to the additional apical electrode array. No complications occurred. CONCLUSION: In cases involving an obliterated cochlea, the number of intracochlear electrodes can be increased with the double array implant. As a result, the patients achieve significantly better auditory results.

Intraoperative test of auditory nerve function.

OBJECTIVE: To develop a preoperative objective test of auditory nerve function for the assessment of cochlear implant candidacy, especially in children. PATIENTS AND METHODS: We stimulated electrically with a ball electrode before insertion of the implant. First the stimulus was applied bipolar between the promontory and the round window. To record auditory brain stem responses (EABRs) very high stimulus intensities were needed, which was not possible in all patients. RESULTS: By stimulation in the basal turn of the cochlea, evoked potentials could be derived. Although in some patients the facial nerve was stimulated as a side effect, auditory evoked potentials could be recorded. A facial muscle artifact can be differentiated from the EABRs by latency and the slope of the input-output function. CONCLUSION: For the present, the only reliable test seems to be the EABR recording stimulated within the cochlea.

Monitoring the electrode position during acoustic neuroma surgery.

OBJECTIVE: To obtain information about the auditory brain stem responses during auditory brain stem implantation. SETTING: Operating room during acoustic neuroma surgery. METHODS: Electrical stimulation of the auditory system during acoustic neuroma surgery, by placement of a monopolar or bipolar electrode on the nerve or nerve entry zone of the brain stem, and monitoring of the evoked auditory brain stem responses (EABR) recorded from the scalp. In some patients, a multichannel silicon electrode array was placed at the foramen of Luschka. Biphasic rectangular current pulses were applied, and EABRs were recorded. RESULTS: Usually the derived potentials consisted of three peaks with a latency below 4 ms. Sometimes we got a complex of two or more peaks. The interpeak interval between the first and second peak was about 0.7 to 1.0 ms, independently of the stimulating electrode position, but the absolute latency of the first peak increased from a minimum of 0.7 ms stimulated at the foramen of Luschka to a maximum of 1.3 ms stimulated at the nerve.