Cochlear Implantation in Children With Autism Spectrum Disorder.

OBJECTIVE: To assess the outcome of cochlear implantation in children with autism spectrum disorder (ASD). STUDY DESIGN: Retrospective case review and survey. SETTING: Tertiary referral center. PATIENTS: Children who meet criteria for cochlear implantation and diagnosis of ASD. MAIN OUTCOME MEASURES: Receptive and expressive language scores and parental survey data. RESULTS: Fifteen patients with history of ASD and cochlear implantation were analyzed and compared with 15 patients who received cochlear implant and have no other disability. Postoperatively, more than 67% of children with ASD significantly improved their speech perception skills, and 60% significantly improved their speech expression skills, whereas all patients in the control group showed significant improvement in both aspects. The top 3 reported improvements after cochlear implantation were name recognition, response to verbal requests, and enjoyment of music. Of all behavioral aspects, the use of eye contact was the least improved. Survey results in regard to improvements in patient interaction were more subtle when compared with those related to sound and speech perception. The most improved aspects in the ASD patients' lives after cochlear implantation seemed to be attending to other people's requests and conforming to family routines. Of note, awareness of the child's environment is the most highly ranked improvement attributed to the cochlear implant. CONCLUSION: Cochlear implants are effective and beneficial for hearing impaired members of the ASD population, although development of language may lag behind that of implanted children with no additional disabilities. Significant speech perception and overall behavior improvement are noted.

Cochlear implant surgery in patients more than seventy-nine years old.

OBJECTIVES/HYPOTHESIS: To evaluate the surgical complications, auditory performance, and hearing handicap following cochlear implantation in patients greater than 79 years of age. STUDY DESIGN: Retrospective trial, tertiary referral center. METHODS: The study group was comprised of 21 patients implanted after 79 years of age from 1996 through 2006 with follow-ups past their 8th decade. Pre-op evaluation consisted of pure-tone audiometry and speech discrimination scores (Hearing in Noise Test and City University of New York sentence test). The results of these tests were compared to similar tests taken post-op. A validated hearing handicap questionnaire was used to evaluate the outcome. RESULTS: There were no permanent medical or surgical complications. However, two patients developed exacerbations of previous comorbid conditions (i.e., urinary retention and acute delirium). Implanted patients experienced a significant improvement in audiologic performance, post-op pure tone average, and post-op speech scores (P < .001). A majority of them were able to use the phone and reported that the cochlear implant was of great benefit to them. The post-op hearing handicap inventory for the elderly demonstrated a significant decrease of hearing handicap scores. CONCLUSIONS: This is the first study to focus on a patient group this advanced in age. With increasing life expectancy, we should begin to stratify risk versus benefit of cochlear implantation in this age group. Cochlear implantation improved audiologic performance and the quality of life in patients older than 79 years old. There were no permanent medical or surgical complications. Chronic pain and temporary vertigo were the most common complications reported in this elderly group. Laryngoscope, 2009.

Cochlear implantation in individuals with Usher type 1 syndrome.

OBJECTIVE: To analyze the occurrence of the Usher type 1 (USH1) gene mutations in cochlear implant recipients with deaf-blind Usher syndrome, and to assess the potential effect of these genes and other factors on the therapeutic outcome. STUDY DESIGN: Case series study of nine patients with the phenotypic diagnosis of USH1. METHODS AND SUBJECTS: Mutation analysis of four USH1 genes (MYO7A, USH1C, CDH23, and PCDH15) by single strand conformational polymorphism (SSCP) and direct sequencing methods. Pre- and post-implantation audiologic tests including pure tone audiometry, speech perception measures, and qualitative assessment of auditory performance. Nine USH1 patients who received their cochlear implants at the University of Miami Ear Institute, Miami, FL, USA, and at the Department of Cochlear Implants, Great Ormond Street Hospital for Children, London, UK. RESULTS: DNA samples from five of the nine patients were available for mutation analysis. Three of the five patients were found to carry USH1 mutations including two with a truncated mutation in CDH23 and one being a digenic inheritance with mutations in CDH23 and PCDH15. We may have failed to detect mutations in the amplicons analyzed, as neither SSCP nor direct sequencing, even combined, detects all mutations present. Our failure to detect mutations in all five patients may also confirm the genetic heterogeneity of USH1 and additional USH1 loci remain to be mapped. Pre-implantation assessment indicated that all of the subjects were pre-linguistically profoundly deaf, had no consistent response to sound, had varying degrees of auditory-oral habilitation. Age at implantation ranged from 2 to 11 years. There was post-implantation improvement in sound detection and speech recognition measures in closed-set format in all patients. Children implanted at an age of 3 years or less showed good open-set speech perception with lip-reading. All patients are implant users. Those patients who do not show open-set perception still use the cochlear implant as an adjunct of lip-reading or total communication. CONCLUSION: Testing for mutations in the USH1 genes allows early identification and intervention of children with USH1; timely intervention is important to maximize the development of useful auditory-oral communication skills prior to the onset of the visual impairment.

Cochlear implantation in organ transplantation.

OBJECTIVES/HYPOTHESIS: Compared to immunocompetent patients, organ transplant recipients receiving immunosuppressant medications may experience higher rates of postoperative complications. This study was designed to retrospectively review the outcomes of cochlear implantation among organ transplant patients. STUDY DESIGN: Retrospective case series. METHODS: Five organ transplant patients received seven cochlear implantations at the University of Miami Ear Institute from January 1, 1992, until August 31, 2005. Inpatient and outpatient records were analyzed to identify healing problems, wound infections, and speech recognition after implantation. RESULTS: There were no healing problems, wound infections, or other complications noted among cochlear implant recipients who had also received organ transplantation. Open-set sentence discrimination as measured by the Hearing in Noise Test (HINT), City University of New York (CUNY) test, and/or Central Institute for the Deaf (CID) tests ranged from 46% to 89%. Open-set word recognition as measured by the Consonant Nucleus Consonant (CNC) or the Northwestern University number 6 (NU-6) test ranged from 26% to 64%. CONCLUSIONS: This series suggests that cochlear implantation can be safe and effective for solid organ transplant recipients on immunosuppressants, with discrimination performance within the expected ranges for cochlear implant patients

Electrode migration after cochlear implantation.

OBJECTIVE: To review the occurrence of electrode migration after cochlear implantation. STUDY DESIGN: Review of public database and retrospective case series. SETTING: Tertiary academic referral center, ambulatory. PATIENTS: Retrospective review of electrode migration reported in association with the US Food and Drug Administration Manufacturer and User Facility Device Experience database and a single-institution case series between 1996 and 2006. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURE: Occurrence of electrode migration. RESULTS: During the period 1996 to 2006, 151 reports of electrode migration were filed in the Manufacturer and User Facility Device Experience database. The most common association with migration was cochlear ossification, although 127 of 151 cases had no known association. During an equivalent period, 637 cochlear implant (CI) procedures were performed at the University of Miami. Of 580 primary CI operations, 2 cases of electrode migration were identified, both associated with reossification of a cochlea that was fully ossified at the first procedure. CONCLUSION: Electrode migration is an underrecognized complication of CI surgery and may be associated with cochlear ossification. Electrode stabilization techniques may reduce the incidence of CI electrode migration in nonossified or incompletely ossified cases.

Performance after cochlear implantation in DFNB1 patients.

OBJECTIVE: To evaluate the speech perception and language development with cochlear implants (CI) of DFNB1 children in comparison with non-DFNB1 deaf children. STUDY DESIGN: Retrospective case series. SETTING: Academic tertiary center. RESULTS: Thirty-one congenitally deafened children, screened for GJB2 allele variants, were followed for an average 32 months after CI. With the use of age-appropriate testing, 75% of DFNB1 and 53% of non-DFNB1 children achieved open set word recognition (speech perception category [SPC] level 6). Multivariate analysis showed that SPC was primarily dependent on duration of CI use, but not on the cause of hearing loss. In Reynell language tests, DFNB1 children showed more consistent and quicker gains than non-DFNB1 children. CONCLUSION: Although children with CI with DFNB1 show faster gains in Reynell scores, duration of CI use appears to have a greater effect on speech perception than DFNB1 status. SIGNIFICANCE: Identification of DFNB1 children is useful in counseling of CI outcomes.

Nucleus Freedom North American clinical trial.

OBJECTIVE: To evaluate hearing outcomes and effects of stimulation rate on performance with the Nucleus Freedom cochlear implant (Cochlear Americas, Denver, CO). STUDY DESIGN AND SETTING: Randomized, controlled, prospective, single-blind clinical study using single-subject repeated measures (A-B-A-B) design at 14 academic centers in the United States and Canada and comparison with outcomes of a prior device by the same manufacturer. PATIENTS: Seventy-one severely/profoundly hearing impaired adults. RESULTS: Seventy-one adult recipients were randomly programmed in two different sets of rate: ACE or higher rate ACE RE. Mean scores for Consonant Nucleus Consonant words is 57%, Hearing in Noise Test (HINT) sentences in quiet 78%, and HINT sentences in noise 64%. Sixty-seven percent of subjects preferred slower rates of stimulation, and performance did not improve with higher rates of stimulation using this device. CONCLUSIONS: Subjects performed well, and there was no advantage to higher stimulation rates with this device. SIGNIFICANCE: Higher stimulation rates do not necessarily result in improved performance.

Conservation of residual acoustic hearing after cochlear implantation.

OBJECTIVE: This study was designed to test the hypothesis that partial hearing conservation is attainable after cochlear implantation with a long perimodiolar electrode. Surgical strategies for hearing conservation during cochlear implantation are described. STUDY DESIGN: Prospective, single-subject, repeated-measures design. SETTING: Academic tertiary care center. PATIENTS: Twenty-eight severely to profoundly hearing-impaired adult cochlear implant recipients who had some measurable hearing preoperatively. INTERVENTION: Cochlear implantation using Nucleus Freedom Contour Advance electrode. MAIN OUTCOME MEASURES: Preimplant and postimplant pure-tone thresholds and speech recognition scores were obtained to determine the incidence and degree of conserved hearing at a mean interval of 9 (+/-3.9) months. RESULTS: Thirty-two percent of subjects experienced complete conservation of hearing (0- to 10-dB loss), and 57% experienced partial conservation of hearing (>11 dB) after implantation. However, open-set speech recognition was partially conserved in only one subject. Cochlear implant performance was not better in patients with conservation of residual hearing. CONCLUSION: Conservation of pure-tone hearing was possible in 89% of implanted patients; however, residual speech perception was not conserved with this long perimodiolar electrode. A ceiling effect tends to inflate the prevalence of hearing conservation in implantation studies of severely to profoundly hearing-impaired patients.

Use of neural response telemetry measures to objectively set the comfort levels in the Nucleus 24 cochlear implant.

Cochlear implant programming necessitates accurate setting of programming levels, including maximum stimulation levels, of all active electrodes. Frequently, clinical techniques are adequate for setting these levels; however, they are sometimes insufficient (e.g., very young children). In the Nucleus 24, several methods have been suggested for estimation of comfort levels (C levels) from neural response telemetry (NRT); however, many require co-application of clinical measurements. Data was obtained from 21 adult Nucleus 24 recipients to develop reliable predictions of C levels. Multiple regression analysis was performed on NRT threshold, slope of the NRT growth function, age, length of deafness, length of cochlear implant use and electrode impedance to examine predictive ability. Only the NRT threshold and slope of the growth function measures were significant predictors yielding R2 values from 0.391 to 0.769. Results demonstrated that these measures may provide an alternative means of estimating C levels when other clinical measures are unavailable.