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The role of a graded profile analysis in determining candidacy and outcome for cochlear implantation in children.

Assessment of candidacy for cochlear implantation in children continues to present a challenge to cochlear implant programs. The efficacy of implantation depends on a number of factors and as a result a multidisciplinary approach has been adopted. At the Cochlear Implant Program at The Hospital for Sick Children, Toronto, we use a graded profile analysis (GPA) which has been adapted from the Children's Implant Profile (CHIP) developed by Hellman (S.A. Hellman, P.M. Chute, R.E. Kretschmer, M.E., Nevins, S.C. Parisier, L.C. Thurston, The development of a Children's Implant Profile, Am. Ann. Deaf. 136 (1991) 77-81). This structured assessment allows each potential candidate to be 'scored' in each category of assessment giving a potential range of -14 to +14. In this retrospective study of 109 candidates we examine the relationship between GPA scores and decision to implant. For those patients who were implanted, the relationship between GPA score and speech perception outcomes was also evaluated. Three distinct groups of children emerged from the analysis. The first group had scores less than 5 and were not considered to be suitable for implantation. Within the second group who scored between 5 and 8, there was no clear relationship between decision to implant and score. The last group, who scored 9-14, was considered to be generally suitable for implantation provided that there were no medical contraindications and the parents consented to implantation. There was a significant association between rate of improvement of speech perception and GPA score. Those patients with scores of 9 to +14 improved at a faster rate than group 5-8 (P < 0.05). The implications of these findings are discussed.

Adolescent↗

Pediatric cochlear implantation: the parents' perspective.

OBJECTIVE: To analyze parental views on cochlear implantation, before and in the years following implantation, to determine whether the results from the intervention met their expectations. DESIGN: Prospective longitudinal study to assess parental perspectives of an unselected group of children with cochlear implantation. SETTING: Tertiary referral pediatric cochlear implant center in the United Kingdom. SUBJECTS: Forty-three parents of children with cochlear implantation. INTERVENTION: A specifically designed questionnaire was administered to assess preimplant expectations and observed changes and concerns at 1, 2, and 3 years following implantation. Three key domains were evaluated: (1) communication with others, (2) listening to speech without lipreading, and (3) the development of speech and language. RESULTS: Preoperative expectations were met or surpassed at each of the follow-up intervals. In the area of communication, 35 (81%) parents expected a definite improvement preoperatively, and 3 years following implantation, 42 (98%) actually saw such an improvement. The respective numbers in the area of listening to speech were 15 (35%) and 38 (88%), and for speech development, 37 (86%) and 37 (86%). Speech development was the major area of concern at all intervals. CONCLUSION: This study demonstrates the ability of cochlear implantation to meet or surpass parental expectations in 3 important outcome domains: communication, listening to speech, and the development of speech and language.

Child↗

Cochlear implantation in adults and children.

Cochlear implants are electronic devices that are introduced surgically into the inner ear and directly stimulate the auditory nerve in response to sound. They are of benefit to profoundly or totally deaf patients who derive no material benefit from optimally fitted hearing aids.

Adolescent↗

Speech-evoked cortical potentials and speech recognition in cochlear implant users.

Processing in the auditory cortex may play a role in the unexplained variability in cochlear implant benefit. P300 and N1/P2 were elicited in post-lingually deaf cochlear implant users wearing a Nucleus multichannel cochlear implant. Four sound contrasts were presented (500-1,000 Hz, /ba/-/da/, /ba/-/pa/ and /i/-/a/). N1 and P2 were present in all subjects for all conditions. Prolonged N1, P2 and P300 latencies were found in the cochlear implant group compared to a control group of subjects with normal hearing. Cochlear implant users show smaller amplitudes of N1 for all the speech signals as well as smaller amplitudes of P2 for the consonants compared to the controls. P300 results of the cochlear implant users were compared to behavioural results of speech recognition testing. A relation was found between P300 amplitude and magnitude for the 500-1,000 Hz and /i/-/a/ contrasts and behavioural speech recognition in cochlear implant users. The results suggest that P300 measurements are useful and have additional value to speech recognition evaluations in cochlear implant users.

Adolescent↗

Cost-utility analysis of the cochlear implant in children.

CONTEXT: Barriers to the use of cochlear implants in children with profound deafness include device costs, difficulty assessing benefit, and lack of data to compare the implant with other medical interventions. OBJECTIVE: To determine the quality of life and cost consequences for deaf children who receive a cochlear implant. DESIGN: Cost-utility analysis using preintervention, postintervention, and cross-sectional surveys conducted from July 1998 to May 2000. SETTING: Hearing clinic at a US academic medical center. PARTICIPANTS: Parents of 78 profoundly deaf children (average age, 7.5 years) who received cochlear implants. MAIN OUTCOME MEASURES: Direct and total cost to society per quality-adjusted life-year (QALY) using the time-trade-off (TTO), visual analog scale (VAS), and Health Utilities Index-Mark III (HUI), discounting costs and benefits 3% annually. Parents rated their child's health state at the time of the survey and immediately before and 1 year before implantation. RESULTS: Recipients had an average of 1.9 years of implant use. Mean VAS scores increased by 0. 27, from 0.59 before implantation to 0.86 at survey. In a subset of participants, TTO scores increased by 0.22, from 0.75 to 0.97 (n = 40) and HUI scores increased by 0.39, from 0.25 to 0.64 (n = 22). Quality-of-life scores were no different 1 year before and immediately before implantation. Discounted direct costs were $60,228, yielding $9,029 per QALY using the TTO, $7,500 per QALY using the VAS, and $5,197 per QALY using the HUI. Including indirect costs such as reduced educational expenses, the cochlear implant provided a savings of $53,198 per child. CONCLUSIONS: Cochlear implants in profoundly deaf children have a positive effect on quality of life at reasonable direct costs and appear to result in a net savings to society. JAMA. 2000;284:850-856

Child↗

Outcome analysis of cochlear implant reimplantation in children.

OBJECTIVES: We review our experience gained in performing cochlear reimplantation in 25 children who have had multichannel cochlear implant device failure at the Cochlear Implant Center of the Manhattan Eye, Ear and Throat Hospital and Lenox Hill Hospital (New York, NY), to assess the feasibility of cochlear reimplantation in children and the effect of reinsertion on audiological performance. STUDY DESIGN: We retrospectively analyzed the outcome of 27 consecutive multichannel cochlear implant reinsertions performed in 25 children at the Manhattan Eye, Ear and Throat Hospital and Lenox Hill Hospital. These reimplantations were performed between 1990 and 1999, with a minimum follow-up of 6 months for both surgical and audiological performance. METHODS: Charts were reviewed for patient factors, findings at the time of initial operation and repeat operation, and results of audiological testing both before and after reimplantation. RESULTS: Surgical complications of reimplantation included two intraoperative cerebral spinal fluid leaks and two late postoperative flap breakdowns with implant extrusions. For the most part, depth of electrode insertion was unchanged. Open-set speech recognition scores and speech perception abilities remained stable or improved compared with results before reimplantation. CONCLUSION: Our results confirm that cochlear implant reimplantation is technically feasible and allows for continued auditory development for the child who has a cochlear implant device failure.

Cerebrospinal Fluid↗

Tinnitus suppression following cochlear implantation. A multifactorial investigation.

The effects of cochlear implant on loudness, annoyance, daily duration, location, and residual inhibition of tinnitus were evaluated by a closed-ended, quantifiable questionnaire in 33 postlingually deafened patients who had received implants at the University of Michigan, Ann Arbor, between 1986 and 1990. Preoperative tinnitus was present in 85% of patients. A statistical comparison of preoperative vs postoperative loudness and annoyance indicated a significant reduction in both of these complaints postoperatively. Loudness and annoyance were significantly correlated, both preoperatively and postoperatively. Fifteen patients (54%) with preoperative tinnitus demonstrated a loudness decrease of 30% or more; 43% demonstrated an annoyance decrease of 30% or more; and 48% demonstrated a decrease of 30% or more in daily tinnitus duration. Patients who experienced a loudness or annoyance decrease of 30% or more after implantation demonstrated significantly higher preoperative levels of these complaints, suggesting that degree of tinnitus reduction after implantation may be related to preoperative loudness and annoyance levels. Contralateral tinnitus suppression was reported by 42% of patients. Residual inhibition ranging from 60 seconds to several hours was reported by 50% of patients, predominantly in the ear with the implant. Age, gender, cause of hearing loss, duration of tinnitus, cochlear implant usage, and time after implantation were not predictive of tinnitus suppression. Overall, the majority of the patients (74%) thought that their cochlear implant was helpful in tinnitus suppression, especially in the ear with the implant. Contralateral residual inhibition and tinnitus suppression suggest a central mechanism contributing to these phenomena.

Adult↗

Cochlear implant performance after reimplantation: a multicenter study.

OBJECTIVE: This study compares auditory performance between original and replacement cochlear implants. STUDY DESIGN AND SETTING: Data from 18 U.S. cochlear implant programs were obtained by retrospective chart review. Patients received and returned subjective questionnaires. PATIENTS: Twenty-eight adults with once-functioning Nucleus 22 cochlear implants that failed received replacement Nucleus 22 cochlear implants in the same ears. MAIN OUTCOME MEASURES: Objective measures included sentence (CID Everyday Sentences or Iowa Sentences) and monosyllabic word (NU-6 Words or CNC Words) speech discrimination scores. Patients also rated and compared performance using subjective scales. RESULTS: Thirty-seven percent of patients had significantly higher sentence or word scores with their replacement cochlear implants than with their original implants, 26% had no significant change, and 37% had significantly poorer scores. Subjectively, 57% of patients reported that the performance of their replacement device was better or the same and 43% reported that it was poorer. There was no correlation between performance with the replacement cochlear implant and cause of the original device failure, duration of original device use before failure, surgical complications with either implantation, changes in electrode insertion depths, or preoperative variables, such as age, etiology, or duration of deafness. CONCLUSIONS: Speech recognition ability with a replacement cochlear implant may significantly increase or decrease from that with the original implant. Experienced cochlear implant patients facing reimplantation must be counseled regarding the possibility of differences in sound quality and speech recognition performance with their replacement device.

Adult↗

Speech intelligibility in children after cochlear implantation.

OBJECTIVE: This study aimed to evaluate the long-term speech intelligibility of young deaf children after cochlear implantation. STUDY DESIGN: The study design was a prospective study following a large group of consecutively implanted deaf children with up to 5 years' cochlear implant use. SETTING: The study was conducted at a pediatric tertiary referral center for cochlear implantation. PATIENTS: All children in the study were congenitally deaf or deafened before 3 years of age. They each received a Nucleus multichannel cochlear implant before the age of 7 years. Eighty-four subjects were evaluated up to 5 years after cochlear implantation. INTERVENTION: Cochlear implantation followed by an intensive program of local and center-based assessment and rehabilitation was performed. MAIN OUTCOME MEASURES: A speech intelligibility rating scale evaluated the spontaneous speech of each child before and at yearly intervals for 5 years after implantation. RESULTS: After cochlear implantation, the difference between the speech intelligibility ratings increased significantly each year for 4 years (Mann-Whitney U-test). For the first 2 years, the average rating remained "prerecognizable words" or "unintelligible speech." It was not until the 3-year interval that the average intelligibility rating became category 3 (intelligible speech if someone concentrates and lip-reads). At the 4-year interval, 85% of children had some intelligible connected speech. This improvement continued, and at the 5-year interval, the median speech intelligibility was category 4 (intelligible speech to a listener with a little experience of deaf speech) and the mode was category 5 (intelligible speech to all listeners). CONCLUSION: Congenital and prelingually deaf children gradually develop intelligible speech that does not plateau 5 years after implantation.

Cochlear Implantation↗

MED-EL Cochlear implants: state of the art and a glimpse into the future.

Cochlear implantation is an accepted treatment method for adults and children with severe to profound hearing loss. Confidence in technology has led to changes in individuals who can receive a cochlear implant and changes in expected benefit with a cochlear implant. This article describes the research and development activities at MED-EL, which make possible the implementation of new speech-coding strategies as well as the application of acoustic and electric stimulation via a combined speech processor in MED-EL devices. Research on benefits from bilateral cochlear implantation and electric-acoustic stimulation are also reviewed. Finally, the potential of drug delivery systems is considered as a way to improve cochlear implant outcomes, and results from preliminary evaluations of a hybrid cochlear implant system with drug delivery capabilities are reported.

Acoustic Stimulation↗

Cochlear implantation in adults with prelingual deafness. Part II. Underlying constraints that affect audiological outcomes.

OBJECTIVES/HYPOTHESIS: To discuss the underlying physiological and anatomical constraints on audiological performance of late-implanted prelingually deafened adult cochlear implant patients. STUDY DESIGN: Retrospective review. METHODS: Published literature on the topic of auditory pathway responses to prolonged congenital deafness was reviewed. In particular, the authors sought to identify the anatomical and physiological changes that take place in both the peripheral and central auditory pathways in response to prolonged deafness, as well as how they are altered by chronic electrical stimulation. RESULTS: The currently available evidence suggests that the colonization of the auditory cortex by other sensory modalities is the main limiting factor in postimplantation performance, not the pathological degenerative changes of the auditory nerve, cochlear nucleus, or auditory midbrain. CONCLUSION: The reviewed evidence, although circumstantial, suggests that emphasizing aurally based educational programs before (with hearing aids) and after cochlear implantation could reduce the cortical colonization phenomenon and potentially improve postimplantation audiological performance of patients with long-term prelingual deafness.

Adult↗

[The cochlear implant--evolution of hearing and language with an artificial inner ear].

One of the most spectacular progresses in modern medicine is the possibility to replace a deaf ear, a sensory organ in total by an implantable electronic prosthesis, a so-called cochlear implant (CI). The CI stimulates the auditory nerve by electrical pulses and thus generates the sensation of hearing along the auditory pathway. One of the most impressive aspects of cochlear implantation is the fact that small children with profound deafness who were able a few years ago to learn spoken language only to very limited extent may achieve nowadays an almost normal language development. Duration and intensity of the training of listening and spoken language vary considerably as a function of etiology and time of deafness. Most important for the development of language is sufficient stimulation of the auditory pathway during early childhood. Early diagnosis of a severe to profound deafness is most important in order to fit hearing aids or a cochlear implant without a time delay. Affected children need intensive training by professionals specialized in education and speech therapy. Adults and adolescents who lost their hearing when language acquisition was established may understand spoken language only a few weeks after receiving a cochlear implant. Several individuals are able to use the telephone. Preliminary results after bilateral cochlear implantation of children and adults show advantages. Not only do these subjects report "better hearing with two CI as compared to hearing with one CI," but some of them developed directional hearing in a rather short time lag after fitting the second implant. And in addition they achieve better speech discrimination in environmental noise.

Adolescent↗

Cochlear implantation: a review of the literature and the Nijmegen results.

The field of cochlear implantation is developing rapidly. In subjects with bilateral profound deafness who gain no benefit from conventional hearing aids the aim of cochlear implantation is to provide a means for them to receive auditory sensations. Throughout the world, most cochlear implant centres are still continuing their research efforts to improve the results with this technique. Although it is still difficult to predict how an individual will perform with a cochlear implant, the success of cochlear implantation can no longer be denied. In this paper, we review some recent papers and reports, and the results of the various Nijmegen cochlear implant studies. Data about subject selection, examinations, surgery and the outcome are discussed. Our results were in good agreement with those of other authors. It can be concluded once again that cochlear implantation is an effective treatment for postlingually deaf adults and children, and for prelingually (congenital or acquired) deaf children with profound bilateral sensorineural deafness.

Adolescent↗

Cochlear implantation after labyrinthectomy.

OBJECTIVE: The goal was to report a method used for intraoperative ear selection for cochlear implantation using electrical brainstem response. Initial patient response and the longer-term results of cochlear implantation after labyrinthectomy were compared. STUDY DESIGN: This was a specific retrospective review of a single case of cochlear implantation after labyrinthectomy. SETTING: The study involved a tertiary referral center in both an ambulatory and a hospital setting. PATIENT: The study involved a report of a single patient who was evaluated for a possible cochlear implant and successfully underwent cochlear implantation. INTERVENTIONS: A case study of a profoundly deaf individual is presented, including the diagnostic measures used to determine the candidacy for cochlear implantation, the ear selected, and the rehabilitation. MAIN OUTCOME MEASURES: Both early (3 months) and later (14 months) postoperative results clearly demonstrate that a cochlear implant in a patient with a previous labyrinthectomy can be beneficial. RESULTS: The early and later results after cochlear implantation are compared in a single case study. CONCLUSION: This case study demonstrates that there is improvement in sound awareness, speech recognition, and communication after cochlear implantation in a previously labyrinthectomized ear.

Aged↗

The binaural digisonic cochlear implant: surgical technique.

The surgical technique of cochlear implantation is currently well established. It is sure and efficient. The results of the cochlear implant concerning speech perception are good and have a favorable cost/efficiency ratio. However, some points remain to be researched. Bilateral implantation allows one to obtain a binaural perception and especially to increase, theoretically, the possibilities of frequency discrimination by reducing interactions between electrodes and therefore improving the patient's performance. Nevertheless, it seems important to caution against placing two implants in one patient, especially for economic reasons. Thus, we have developed, with the MXM Company, a concept of a unique implant able to stimulate both cochleas with a single processor and a single receiver: the Binaural Digisonic cochlear implant. This article describes the Binaural Digisonic system, the surgical technique as developed by postmortem dissection, and the first two implantations in patients.

Acoustic Stimulation↗

Cochlear implantation concurrent with translabyrinthine acoustic neuroma resection.

OBJECTIVES/HYPOTHESIS: Cochlear implants provide successful auditory rehabilitation for patients with profound sensorineural hearing loss who do not derive at least marginal benefit from conventional hearing aids. Patients with neurofibromatosis type 2 can present with bilateral profound sensorineural hearing loss caused by bilateral vestibular schwannomas. Auditory rehabilitation in these patients can be challenging. We present the case of one such patient who underwent a concurrent translabyrinthine vestibular schwannoma resection and cochlear implantation in the same ear. STUDY DESIGN: A case report and review of the literature. METHODS: Review the patient's medical record and MEDLINE literature search. RESULTS: The patient presented with a relatively small tumor that was situated in the fundus of the internal auditory canal with intralabyrinthine extension. Postoperative performance with implant stimulation was in the higher range of that for other cochlear implant patients. CONCLUSIONS: To the best of our knowledge, this is the first case reported of simultaneous cochlear implant and translabyrinthine acoustic neuroma resection in the same ear of a patient with neurofibromatosis type 2.

Auditory Threshold↗

Cochlear implantation and meningitis.

OBJECTIVE: Study clinical presentation, diagnosis and treatment of cochlear implant patients diagnosed with bacterial meningitis. BACKGROUND: Cochlear implantation in children is necessary for the optimal development of speech in the developing child diagnosed with profound sensorineural hearing loss. Approximately 60,000 devices have been inserted in adults and children worldwide to date. SETTING: The Department of Pediatric Otolaryngology of a tertiary care children's hospital. METHODS: All patients undergoing cochlear implantation from April 1997 were identified. Patients diagnosed with bacterial meningitis after implantation were selected for study. RESULTS: Of 30 children, two developed bacterial meningitis after cochlear implantation. One patient developed Streptococcus pneumoniae meningitis. One patient developed nontypable Haemophilus influenzae bacterial meningitis. Both patients made a complete recovery. CONCLUSIONS: Prompt diagnosis and treatment of meningitis is essential to optimize outcome.

Child, Preschool↗

Frequency-place compression and expansion in cochlear implant listeners.

In multichannel cochlear implants, low frequency information is delivered to apical cochlear locations while high frequency information is delivered to more basal locations, mimicking the normal acoustic tonotopic organization of the auditory nerves. In clinical practice, little attention has been paid to the distribution of acoustic input across the electrodes of an individual patient that might vary in terms of spacing and absolute tonotopic location. In normal-hearing listeners, Başkent and Shannon (J. Acoust. Soc. Am. 113, 2003) simulated implant signal processing conditions in which the frequency range assigned to the array was systematically made wider or narrower than the simulated stimulation range in the cochlea, resulting in frequency-place compression or expansion, respectively. In general, the best speech recognition was obtained when the input acoustic information was delivered to the matching tonotopic place in the cochlea with least frequency-place distortion. The present study measured phoneme and sentence recognition scores with similar frequency-place manipulations in six Med-El Combi 40+ implant subjects. Stimulation locations were estimated using the Greenwood mapping function based on the estimated electrode insertion depth. Results from frequency-place compression and expansion with implants were similar to simulation results, especially for postlingually deafened subjects, despite the uncertainty in the actual stimulation sites of the auditory nerves. The present study shows that frequency-place mapping is an important factor in implant performance and an individual implant patient's map could be optimized with functional tests using frequency-place manipulations.

Acoustic Stimulation↗