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Pediatric cochlear implantation in cochlear malformations.

OBJECTIVE: This study aimed to present relevant information about pediatric cochlear implantation in malformed cochleas based on the experience gathered with 12 implanted children. STUDY DESIGN: A retrospective analysis was performed. SETTING: All patients were diagnosed and implanted at the Medical University of Hannover. Medical check-ups were performed regularly. The rehabilitation concept was developed by the Cochlear Implant Center of Hannover. PATIENTS: All children were female and were between 2 and 13 years of age at the time of implantation, with the average age being 4 years and 2 months. Only patients who were younger than 14 years of age and implanted between September 1992 and October 1995 were evaluated. INTERVENTION: Diagnostic computed tomographic scans including three-dimensional reconstructions and magnetic resonance imaging images were performed. In all cases, Nucleus devices (Mini 22 or 20 + 2) were implanted. Medical University of Hannover standard surgical technique was used, although in most cases, facial nerve monitoring and electrically evoked auditory brain stem responses were additionally recorded. Total or partial obliteration of the middle ear had occurred in two cases. An anteroposterior approach was used four times. The implantation was followed by the standard rehabilitation procedure for children. RESULTS: No serious complications occurred. All children responded to acoustic stimuli and showed improvement in their speech production. However, one autistic child performed poorly, and for another child suffering from a CHARGE syndrome, results still are pending. CONCLUSIONS: Given suitable preconditions, cochlear implantation is feasible with an acceptable risk of complications. Implantation appears to be beneficial in most cases with cochlear malformations provided that eighth nerve and cochlear lumen are present.

Adolescent↗

Development and application of a health-related quality-of-life instrument for adults with cochlear implants: the Nijmegen cochlear implant questionnaire.

OBJECTIVE: The goal was to develop a quantifiable, self-assessment health-related quality of life (QoL) instrument for use in cochlear implant (CI) users. DESIGN: Three principal domains were distinguished: physical, psychological, and social. Forty-five postlingually deaf adult multichannel CI users and 46 deaf candidates on the waiting list for CIs (control group) participated in the study. RESULTS: Retrospective scores for the CI group corresponded very well with the scores for the control group. Current QoL scores were substantially higher for all 6 subdomains. Internal consistency and testretest reliability coefficients proved to be satisfactory, and the ability to detect clinical changes with the Nijmegen Cochlear Implantation Questionnaire (NCIQ) proved to be good. CONCLUSIONS: The psychometric characteristics of the NCIQ proved to be reliable and probably valid and sensitive to clinical changes. The data obtained with the NCIQ reflected that the instrument was able to detect that a CI had significant effects on several health-related QoL aspects, including the social and psychological domains.

Activities of Daily Living↗

[Multi-channel cochlear implant in cochlear ossification].

Cochlear ossification, considered until only a few years ago as a contraindication for cochlear implants (C.I.), may now be managed by means of a wide variety of surgical techniques. In cases with massive ossification, the drill-out circummodiolar technique described by Gantz et al. in 1988 and successively modified by Balkany et al. in 1997 may be adopted. The technique of electrode insertion in the scala vestibuli, perfected by Steenerson et al. in 1990, may be used when cochlear ossification has spread no further than the scala tympani. Other methods call for a groove to be drilled along the proximal tip of the basal turn of the cochlea (Cohen and Waltzman, 1993), the insertion of electrodes through the middle cranial fossa (Colletti et al., 2000), or the utilization of a double electrode array (Bredberg et al., 1997, Lenarz et al., 2001). This study reports the experience conducted at the Cochlear Implants Centre of the Otorhinolaryngoiatrics, Otological and Otoneurological Microsurgery Section of the University of Parma in a group of 15 patients who underwent C.I. in the presence of varying degrees of ossification. In 3 cases the ossification was limited to the region of the round window and a few millimetres of the scala tympani; cochleostomy was performed anteriorly and inferiorly to the anterior niche of the round window. In 11 cases (of which 3 of pediatric age), the ossification had spread to the horizontal portion of the scala tympani; in these cases, the electrodes were inserted in the scala vestibuli. The scala vestibuli was opened by drilling anteriorly to the round window and superiorly to the spiral ligament. In the only case of massively ossified cochlea, it was possible to partially insert the electrodes in a circum-modiolar tunnel. In the 12-month follow-up hearing test, the 3 patients with ossification of the round window region and the first millimetres of the scala tympani respectively averaged 61.6% in recognizing 2-syllable words and 59% in recognizing words embedded in phrases. The averages on the 12-month follow-up hearing test in the 8 adult patients who received the implant in the scala vestibuli were 80.6% in recognizing 2-syllable words and 89.1% in recognizing words in phrases. The 3 pediatric patients were classified on the Geers and Moog scale, which situated 2 of them in the 6th category of perception and 1 of them in the 4th category of perception. As regards the only case of massive cochlear ossification, the patient underwent surgery recently, and the sole follow-up available is the one conducted after only 3 months; the vowel identification average was 55%; the average on the VCV test was 31%; and the 2-syllable word recognition average was 20%.

Acoustic Stimulation↗

Superiority of bilateral cochlear implantation over unilateral cochlear implantation in tone discrimination in chinese patients.

PURPOSE: The benefits of bilateral cochlear implants (CIs) versus unilateral CIs were evaluated by comparing the Cantonese lexical tone discrimination scores conducted in a quiet environment and against a background noise. MATERIALS AND METHODS: Four postlingually deafened Cantonese-speaking adults (2 men and 2 women) with bilateral CIs were included in this study. The subjects were their own control in the monaural hearing condition. Both the Cantonese lexical tone stimuli and the speech-weighted background noise were presented at 0(0) azimuth and at a distance of 1 m from the subject. The speech stimuli, which were maintained at 65 dB sound pressure level, were presented in both a quiet environment and against a background noise at signal to noise ratios (SNRs) of +15, +10, +5, 0, -5, -10, and -15. RESULTS: Against a background noise, the bilateral CIs required +5 dB SNR only to obtain significant scores in discriminating Cantonese lexical tones and to achieve discrimination scores that were comparable to the optimal discrimination scores obtained in quiet. No significant difference in the discrimination scores was observed between binaural and monaural hearing conditions when the tests were conducted in quiet. CONCLUSIONS: Our study showed that in the presence of background noise, bilateral CIs were better than unilateral CIs in discriminating Cantonese lexical tones.

Adult↗

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↗

Recognition of speech presented at soft to loud levels by adult cochlear implant recipients of three cochlear implant systems.

OBJECTIVE: The purpose of this study was to conduct a large-scale investigation with adult recipients of the Clarion, Med-El, and Nucleus cochlear implant systems to (1) determine average scores and ranges of performance for word and sentence stimuli presented at three intensity levels (70, 60, and 50 dB SPL); (2) provide information on the variability of scores for each subject by obtaining test-retest measures for all test conditions; and (3) further evaluate the potential use of lower speech presentation levels (i.e., 60 and/or 50 dB SPL) in cochlear implant candidacy assessment. DESIGN: Seventy-eight adult cochlear implant recipients, 26 with each of the three cochlear implant systems, participated in the study. To ensure that the data collected reflect the range of performance of adult recipients using recent technology for the three implant systems (Clarion HiFocus I or II, Med-El Combi 40+, Nucleus 24M or 24R), a composite range and distribution of consonant-nucleus-consonant (CNC) monosyllabic word scores was determined. Subjects using each device were selected to closely represent this range and distribution of CNC performance. During test sessions, subjects were administered the Hearing in Noise Test (HINT) sentence test and the CNC word test at three presentation levels (70, 60, and 50 dB SPL). HINT sentences also were administered at 60 dB SPL with a signal-to-noise ratio (SNR) of +8 dB. Warble tones were used to determine sound-field threshold levels from 250 to 4000 Hz. Test-retest measures were obtained for each of the speech recognition tests as well as for warble-tone sound-field thresholds. RESULTS: Cochlear implant recipients using the Clarion, Med-El, or Nucleus devices performed on average equally as well at 60 compared with 70 dB SPL when listening for words and sentences. Additionally, subjects had substantial open-set speech perception performance at the softer level of 50 dB SPL for the same stimuli; however, subjects' ability to understand speech was poorer when listening in noise to signals of greater intensity (60 dB SPL + 8 SNR) than when listening to signals presented at a soft presentation level (50 dB SPL) in quiet. A significant correlation was found between sound-field thresholds and speech recognition scores for presentation levels below 70 dB SPL. The results demonstrated a high test-retest reliability with cochlear implant users for these presentation levels and stimuli. Average sound-field thresholds were between 24 and 29 dB HL for frequencies of 250 to 4000 Hz, and results across sessions were essentially the same. CONCLUSIONS: Speech perception measures used with cochlear implant candidates and recipients should reflect the listening challenges that individuals encounter in natural communication situations. These data provide the basis for recommending new candidacy criteria based on speech recognition tests presented at 60 and/or 50 dB SPL, intensity levels that reflect real-life listening, rather than 70 dB SPL.

Adult↗

Using the CLARION cochlear implant in cochlear ossification.

This paper is a retrospective review of 5 patients with various degrees of cochlear ossification who were implanted with the CLARION Multi-Strategy Cochlear Implant. Preoperative computed tomography scans, intraoperative findings, surgical technique, and hearing outcomes are discussed in a case report format. Full implantation was achieved in all cases by a systematic approach that included drill-through of proximal obstruction (2 cases), scala vestibuli insertion (2 cases), and complete drill-out (1 case). The only complication was delayed wound healing in a patient with sickle cell disease, chronic active hepatitis, and steroid dependency on antimetabolite therapy. Early results show that the 4 patients with at least 3 months of experience have a mean open-set sentence recognition score of 55% and a mean open-set word recognition score of 24%. The conclusion is that implantation of the Clarion device in ossified cochleas can be successful in all degrees of ossification and can provide significant hearing benefit.

Adolescent↗

Cochlear implantation in cochlear otosclerosis.

OBJECTIVES/HYPOTHESIS: The objective was to correlate implant performance in cochlear otosclerosis to 1) matched control samples, 2) severity of otic capsule involvement, 3) prior ipsilateral surgery, and 4) programming issues. STUDY DESIGN: Retrospective case controlled study. METHODS: Study cohort comprised 30 individuals. Diagnosis was based on prior ear surgery (stapedectomy [n = 18] or fenestration [n = 2]) and/or pathognomonic radiological findings. High-resolution computed tomography images of the temporal bones were assessed by two radiologists and graded (range, 0-3) for the extent of otosclerosis. Operative records were reviewed. Performance, programming visits, and the number of electrode deactivations at 6 months and at 1 year after implantation were determined for the individuals with otosclerosis and compared with a group of matched control subjects. A within-group comparison correlating severity of otosclerosis to the above was carried out. RESULTS: Implant performance in individuals with cochlear otosclerosis was not significantly different from those without. Previous surgery on the side of implantation did not alter performance. Programming difficulty as reflected in the number of visits and electrode deactivation for sound quality reasons were comparable. Deactivation for facial nerve stimulation occurred exclusively in otosclerotics with the most severe radiological disease (grade 3) and was only with non-modiolar hugging electrodes (n = 5). There was no observed difference between the radiological extent of otosclerosis and implant performance. CONCLUSION: Individuals with severe otosclerosis considering cochlear implantation can be counseled to expect similar benefit to those without, regardless of whether prior surgery occurred on the side of implantation or of severity of otic capsule involvement. There is a significant risk of facial nerve stimulation in otosclerotics with grade 3 disease.

Case-Control Studies↗

A prospective, randomized study of cochlear implants. The Department of Veterans Affairs Cochlear Implant Study Group.

BACKGROUND: Cochlear implants restore some degree of hearing in patients with severe hearing impairment, but the efficacy of different implants has not been compared. We conducted a prospective trial to compare several cochlear implants. METHODS: We studied 82 patients who were randomly assigned to receive one of three cochlear implants: the Ineraid multichannel implant (implant 1), the Nucleus multichannel implant (implant 2), and the 3M/Vienna single-channel implant (implant 3). All the patients had profound deafness, and none had derived benefit from hearing aids. The assigned device was successfully implanted in 80 patients. Twenty-four hearing tests were used to assess the patients' performance before implantation and 12 and 24 months after implantation. The tests were grouped into five categories according to their content, and a weighted composite index was developed to provide a single numerical indicator of the overall auditory response. RESULTS: All the patients were able to hear with their implants. Nineteen of the 30 patients (63 percent) who received implant 2, 18 of the 30 patients (60 percent) who received implant 1, and 1 of the 20 patients (5 percent) who received implant 3 were able to distinguish some words and sentences. The scores for the composite index were similar in the patients who received implant 1 and those who received implant 2, and were higher in both these groups than in the patients who received implant 3 (P = 0.02). When 24 patients with implant 2 were given an improved speech processor, their composite index increased significantly within 3 months (P < 0.001); their score at that time was also significantly higher (P = 0.04) than the score of the patients with implant 1 at 24 months. Age at implantation, lip-reading ability, and IQ were prognostic indicators of the patients' performance with a cochlear implant. CONCLUSIONS: Multichannel cochlear implants are superior to single-channel implants, especially for understanding speech. Changes in speech processing can improve patients' performance.

Aged↗

[Pedagogic aspects of rehabilitation of children with a cochlear implant. Report from the Hannover Cochlear Implant Center].

As we see it cochlear implant for deaf born and for children who became deaf is only justified if the cochlear implant center can belay an interdisciplinary teamwork preoperatively and the securing of habilitation respectively rehabilitation. The cooperation between parents and teachers of the children is absolutely necessary. As the neurophysiologists see it the cochlear implant is a good chance for young deaf born children to use sensitive phases for development of hearing and speech abilities. In these phases the children needs adequate stimuli for development. The changes for developing open speech abilities are slight, if the children are older.

Child↗

Intracochlear misdirected implantation of a cochlear implant.

This paper discusses a rare complication of an intracochlear misdirection of the electrode of a cochlear implant in a 55-year-old male. The patient received a cochlear implant using the mastoid-saving surgical approach. Intraoperative measurements showed impedance and NRT reactions suggesting a reasonable function of the inner ear and the implant. Postoperatively our patient suffered from passing vertigo. Postoperative CT scans revealed a misdirection implantation of the cochlear implant into the vestibular part of the inner ear. A deformed implant was removed and a reimplantation was successfully performed after enlarging and reshaping the cochleostomy. Our patient now enjoys all the benefits of an optimal functioning cochlear implant. Intracochlear misdirection of the electrode can occur even when intraoperative measurements seem to be normal. When a patient suffers from unexplained vertigo after cochlear implantation with a poor function of the implant a misdirection of the active electrode must be considered. New high resolution peroperative three-dimensional imaging techniques can probably help to avoid such insertion failures.

Cochlea↗

Facial nerve stimulation with cochlear implantation. VA Cooperative Study Group on Cochlear Implantation.

The course of the facial nerve may place it within the current field generated by an activated cochlear implant to produce incidental facial movement. We investigated the presence of facial nerve stimulation associated with cochlear implants in the VA Cooperative Study of Advanced Cochlear implants. Twelve of 82 patients enrolled in this study demonstrated facial nerve stimulation within 2 years of implant activation. Facial nerve stimulation in six patients with multiple channel implants (Nucleus or ineraid devices) either resolved spontaneously (n = 2), or was eliminated by deactivating basal (n = 2) or apical (n = 2) electrodes. Two of six patients with single-channel electrodes (3-M/Vienna devices) demonstrated facial nerve stimulation that resolved spontaneously (n = 2), resolved with lowering current output (n = 2), or was refractory to processor adjustment (n = 2). Intraoperative assessment in one of the refractory cases indicated that facial nerve stimulation resulted from current spread through the modiolus to activate the facial nerve. A variety of factors, including implant design, stimulus parameters, and local tissue impedances, may interact to produce incidental facial stimulation. Low-impedance pathways between the scala tympani and the modiolus may deserve increased recognition as an interactive factor in cochlear implant performance.

Adult↗

Effects of stimulation by cochlear implant on the cochlear nerve.

Degeneration of the cochlear nerve before and after placement of the cochlear implant might influence the efficacy of the device. We examined histological characteristics, including the caliber of the cochlear nerve fibers of the central segment proximal to the porus acusticus, in three profoundly deaf patients. Two of them used a cochlear implant for many years longer in one ear than in the other, and one used an implant in one ear only. No qualitative or quantitative differences between the two sides were found. However, in all three cases we found that the cochlear nerves on both sides were substantially degenerated. These results indicated no noticeable effects of stimulation by the cochlear implant on the central portion of the cochlear nerve.

Adult↗

Initial experience with the cochlear implant in children.

The decision to implant cochlear prostheses in children came after positive findings with deaf adults and the belief that the benefits of receiving sound far outweigh the potential risks involved. As of December 1981, 12 profoundly deaf children have received the single-electrode cochlear implant. Their ages range from 3.5 to 17 years. Test results are consistent with those of adults. Mean thresholds across the frequency range fall between 59-64 dB SPL. With the implant, these children can perform specific timing and intensity differentiations which could not be done with hearing aids. Their voices are quieter and more modulated, and the children are not quite as noisy with objects in the environment (eg, do not slam doors any more). This preliminary data demonstrates that implanting subjects under the age of 18 years is feasible.

Acoustic Stimulation↗

Electrically evoked brainstem responses in cochlear implant recipients.

Electrical stimulation of the auditory nerve in the profoundly deaf population through implanted cochlear prostheses has increased the need for reliable electrophysiologic assessment tools. We have recorded electrically evoked brainstem responses (EABRs) in 21 subjects who have received a 3M/House cochlear implant. Recordings have been made, both intraoperatively and postoperatively, in the laboratory setting. The recording technique, methods of stimulus artifact suppression, and results of our measurements are described. Clinical applications of this technology are suggested.

Adolescent↗