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Communication and language in severely deaf adolescents.

One of the most important psychological effects of early severe or profound deafness is an impairment in natural language-processing ability, with a consequent reduction in communicative skill secondary to the sensory deficit. Prelingual damage blocks the development of certain sequential/syntactical skills necessary for the acquisition of normal linguistic competence. Educators of the deaf in the UK have typically attempted to improve linguistic ability using amplification and intensive training in lip-reading, but evidence from several psychological studies suggests that communication systems based upon manual signing are more productive of both linguistic and basic cognitive skills. This paper reports an experimental study of the communicative characteristics of both oral and total communication systems as a function of language structure. For almost all types of structure investigated, the total system was found to be the more effective method of communication.

Adolescent↗

McGurk effect in non-English listeners: few visual effects for Japanese subjects hearing Japanese syllables of high auditory intelligibility.

The McGurk effect is a phenomenon that demonstrates a perceptual fusion between auditory and visual (lip-read) information in speech perception under the condition of audio-visual discrepancy, created by dubbed video tapes. This paper investigated whether or not the McGurk effect could be extended to Japanese subjects listening to Japanese syllables of different auditory intelligibility. The audio and video signal of a female talker's speech for ten Japanese syllables (/ba/, /pa/, /ma/, /wa/, /da/, /ta/, /na/, /ra/, /ga/, /ka/) was combined on videotapes, giving 100 audio-visual stimuli. These stimuli were presented to ten Japanese subjects who were required to identify the stimuli as heard speech in both noise-added and noise-free conditions. For both conditions, the intelligibility of the auditory stimuli was measured, by presenting the audio-alone stimuli. The results showed that, in the noise-free condition, the McGurk effect was small and almost limited to auditory stimuli of which the intelligibility was less than 100%. In the noise-added condition, the McGurk effect was very strong and widespread. These results indicate that the "Japanese McGurk effect" is less easily induced than the English one, and that it depends on the auditory intelligibility of the speech signal.

Adult↗

Use of a multichannel auditory brainstem implant for neurofibromatosis type 2.

Neurofibromatosis type 2 (NF 2) typically results in deafness due to disruption of the cochlear nerves, making peripheral devices such as cochlear implants ineffective. Auditory brainstem implants (ABIs), for direct electrical stimulation of the cochlear nucleus, have been used to provide auditory stimulation in this group of patients. Currently, 141 patients have been implanted in our institution, most recently using an advanced multichannel device. We report results of a recent series of 86 patients who received ABIs. Of this group, 60 had successful implantation, recovered from surgery, responded successfully to stimulation and underwent a full course of device programming and audiologic testing. This group had significant improvement in scores on several audiologic tests compared to baseline. When used to augment lip reading, improvement was also seen. The degree of improvement varied considerably among patients. ABI is a useful device for deaf patients with NF 2. As measured by audiologic testing, many patients receive substantial benefit with regard to sound and speech comprehension.

Brain Stem↗

Auditory brainstem implantation to rehabilitate profound hearing loss with totally ossified cochleae induced by pneumococcal meningitis.

Hearing rehabilitation by cochlear implantation is not always possible in case of total ossification after pneumococcal meningitis. We report 3 cases of postmeningitis profound hearing loss with total cochlear ossification in adults who underwent auditory brainstem implantation (Nucleus 22, Cochlear Inc., Lane Cove, Australia) between 1999 and 2004. The postoperative follow-up period ranged from 1 to 6 years. Eleven to 15 out of 22 electrodes were activated. All patients had significant speech discrimination in the sound-only mode and an enhanced lip-reading performance with the implant. Auditory brainstem implants are an efficient means of auditory rehabilitation and may be considered in selected cases of bilateral profound hearing loss with the impossibility of cochlear implantation.

Adult↗

Speech performance by patients with multichannel cochlear implants.

Thirteen patients were implanted with multichannel cochlear implants between April 1, 1984 and February 25, 1985. The patients are fitted with the sound processor unit 4 to 6 weeks postsurgery. One patient had a single-channel unit replaced with a multichannel implant. She reported improved sound quality. All patients use their implants daily and all patients would undergo implantation again (based on their current performance). Evidence exists that continued use leads to improved performance. Patient S.S. progressed from auditory-only scores of 12% and 24% to 100% in 4 months. The average open set auditory-only performance of six patients in the first 4 months on a spondee word list was 22%. The average of eight patients--more than 4 months post sound processor--is 44%, indicating improvement with use. Patients perform better when combining auditory input with visual input than they do with lip reading alone. The multichannel implant is well tolerated and accepted, and results in speech performance improvement.

Adult↗

Prognosticating speech performance in multichannel cochlear implant patients.

Twenty patients received multichannel cochlear implants between April 1984 and May 1986 at the University of Utah Medical Center. All patients have been followed for at least 1 year postimplant. Preimplant screening included audiometric testing, electronystagmogram (ENG), promontory stimulation, computed tomography (CT) scanning, and psychological evaluation. Based on postimplant audio-only CID sentence discrimination scores, these patients were divided into three groups: good (CID greater than 79%), intermediate (CID, 21% to 79%), and poor (CID less than 21%). Preimplant factors that correlated with CID scores were hearing loss duration, previous use of hearing aids, lip-reading ability, tinnitus, positive ENG calorics, preimplant pure-tone average, promontory stimulation threshold, and understanding of the project. Only previous hearing aid usage approached statistical significance (p = 0.05). A larger patient sample is needed to verify these results.

Adult↗

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.

Adult↗

First Croatian auditory brainstem implantation.

A deaf female patient was diagnosed with bilateral acoustic neurinomas. Diagnosis incorporated the standard audiological battery for sensorineural hearing loss, computed tomography and magnetic resonance imaging. The left side had been operated on four years previously in another clinic using the suboccipital approach. The auditory brainstem implant surgery was performed on the 'second side' using the same approach. It was an uneventful operation with good anatomy and no serious post-operative complications. Post-operatively, the patient performed exceptionally well, with up to 50 per cent of words recognized in the opened set and 85 per cent in the closed set, both without lip-reading.

Adult↗

Multichannel auditory brainstem implant: US clinical trial results.

Since 1994, a US Food and Drug Administration clinical trial evaluated the multichannel auditory brainstem implant (ABI) on 92 subjects with neurofibromatosis type 2 (NF2). The trial has shown that 85 per cent of patients receive auditory sensations. A small number of patients demonstrate a clinically significant degree of open-set sentence recognition in the sound-alone condition; however, when the ABI is combined with lip-reading cues, 93 per cent of patients demonstrate improved sentence understanding at three to six months. In addition, the majority of recipients report daily use of their devices, and satisfaction with the decision to receive the ABI.

Adolescent↗

First auditory brainstem implant in the Czech Republic.

In the Czech Republic, the first implantation of a stimulation electrode into the brainstem was performed on 11 January 1999 in the Department of ORL, Head and Neck Surgery, The First Medical Faculty, Charles University in Prague, University Hospital Motol. The selected patient was a 40-year-old woman with neurofibromatosis type 2 (NF2) who had previously undergone bilateral vestibular schwannoma surgery. Both tumours had been radically removed, the left-sided tumour in 1987, the right-sided one in 1988. She had been completely deaf since the last operation, i.e., for 11 years. The surgery was realized by the international cooperation of three teams. Placement of the electrode pad of the Nucleus CI21 + 1M system on the ventral and dorsal cochlear nuclei was performed. Electrically evoked auditory brainstem responses (EABRs) proved the correct position of the electrode array. The post-operative course was uneventful. Six weeks after the surgery the patient received her speech processor. Since that time, the patient already absolved several sessions of a speech processor tune-up. She uses the device as an aid in lip-reading. No adverse or pathological side effects have been observed. The patient was the 45th person in Europe to receive an ABI and the first in the Czech Republic.

Adult↗

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.

Adolescent↗

Surgical experiences in 58 cases using the Nucleus 22 multichannel auditory brainstem implant.

Patients with bilateral total deafness due to lesions of the vestibulocochlear nerve can be treated by electrical stimulation of the second auditory neuron. A 22-channel multi-electrode implant with transcutaneous transmission was developed that allows the selection of the most useful electrodes. Acoustic neuromas were removed from 49 out of 58 patients by ENT surgeons and neurosurgeons working in collaboration and using either a translabyrinthine or suboccipital approach. The central electroauditory prosthesis was implanted in the same procedure. Six patients were deaf after previous acoustic neuroma surgery without recurrence, three had diagnoses other than neurofibromatosis type 2 (NF2). There were no complications due to the implantation procedure. Side effects could be excluded by stimulation of the auditory electrodes alone. Most of the patients used their ABI daily. They reported perception of different sounds and frequencies, enhancement of lip-reading ability, and three of the patients were able to use the telephone.

Adolescent↗

Iowa cochlear implant clinical project: results with two single-channel cochlear implants and one multi-channel cochlear implant.

Cochlear implants have become a realistic alternative for the management of profoundly deaf patients. A variety of implants with differing electrode designs and coding strategies have been developed by nine major implant centers around the world. Each center has their "star" patient, but objective comparisons between these different implant designs are unavailable. In order to determine the performance characteristics of the present generation of cochlear implants, comparison data are vital. We have developed an independent center where uniform objective comparisons of different cochlear implants can be performed longitudinally. This report will present results of nine patients implanted with three different cochlear implant prostheses. Four patients have been implanted with the Los Angeles (House) single-channel implant, three patients have received the Vienna (Hochmair) single-channel intracochlear device, and two patients have been implanted with the Melbourne (Clark) 21-channel unit. All patients have had 11 months or more of experience with their cochlear prostheses. The results of a comprehensive audiologic battery which includes audiovisual and environmental tasks are presented. All implants provide significant improvement in speechreading and sound awareness. The findings to date suggest that there is strong correlation between top-down cognitive processing (as reflected by lip reading skills) and performance with cochlear implants.

Adult↗

Neuroprosthetic hearing with auditory brainstem implants.

The auditory brainstem implant (ABI) does provide auditory sensations, recognition of environmental sounds and aid in spoken communication in about 300 patients worldwide. It is no more an investigative device but widely accepted for the treatment of patients who have lost hearing due to bilateral tumors of the hearing nerve who transmits the acoustic information from the cochlea to the brain. Most of the implanted patients are completely deaf when the implant is switched off. In contrast to cochlear implants, only few of the implanted patients achieve open-set speech recognition without the help of visual cues. On average, the ABI improves communicative functions like speech recognition at about 30% when compared to lip-reading only. The task for the next years is to improve the outcome of ABI further by developing new less invasive operative approaches as well as new hardware and software for the ABI device.

Auditory Brain Stem Implants↗

Does cause of deafness influence outcome after cochlear implantation in children?

OBJECTIVES: The objective of this study was to evaluate long-term speech perception abilities of comparable groups of postmeningitic and congenitally deaf children after cochlear implantation. METHODS: This prospective longitudinal study comprised 46 postmeningitic deaf children and 83 congenitally deaf children with age at implantation of < or = 5.6 years. Both groups were comparable with respect to educational setting and mode of communication and included children with additional disabilities. RESULTS: Both postmeningitic and congenitally deaf children showed significant progress after implantation. Most (73% and 77%, respectively) could understand conversation without lip-reading or use the telephone with a known speaker 5 years after implantation, whereas none could do so before implantation. At the same interval, the postmeningitic and congenitally deaf children scored a mean open-set speech perception score of 47 (range: 0-91) and 46 (range: 0-107) words per minute, respectively, on connected discourse tracking. The respective mean scores at the 3-year interval were 22 and 29 correct words per minute, respectively. None of these children could score a single correct word per minute before implantation. The progress in both groups was statistically significant. When the 2 groups were compared, there was no statistically significant difference. CONCLUSION: Postmeningitic and congenitally deaf children showed significant improvement in their auditory receptive abilities at the 3- and 5-year intervals after cochlear implantation. There was no statistically significant difference between the outcomes of the 2 groups, suggesting that, provided that children receive an implant early, cause of deafness has little influence on outcome. Although the prevalence of other disabilities was similar in both groups, for individual children, their presence may have profound impact. The study supports the concept of implantation early in life, irrespective of the cause of deafness.

Child↗

Communication and reasoning skills of deaf and hearing signers.

Six tests of intellectual performance were administered to 25 deaf and 21 hearing adult signers. The two groups were not significantly different on tests of Lip Reading and Reception of Signed English but the hearing subjects were superior on tests of non-verbal reasoning and perceptual speed. The hearing were greatly superior on a test of verbal reasoning.

Adult↗

Indications and results of the multichannel cochlear implant.

In the case of total deafness, the multichannel cochlear implant prosthesis constitutes a substantial improvement. It allows known words to be recognized without lip reading, with a great percentage of good responses. Clinical and phoniatric results are reported in 21 cases of patients with implants.

Acoustic Stimulation↗