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.

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.

Revision cochlear implantation for facial nerve stimulation in otosclerosis.

OBJECTIVE: To find if patients experiencing postsurgical facial nerve stimulation caused by underlying disease process (ie, otosclerosis) can improve their hearing performance with their cochlear implant by reimplantation and by an optimal programming strategy. DESIGN: Retrospective analysis. SETTING: Academic tertiary referral center. PATIENTS: Two cochlear otosclerosis patients with resistant facial nerve stimulation (FNS). Both patients were initially implanted with Nucleus 22 devices (Cochlear Corporation, Englewood, Colo) and they developed FNS after a period of use. Owing to the decreasing number of active electrodes, concurrent decreases in speech understanding occurred. INTERVENTIONS: Various programming approaches were used to address the FNS. Both subjects ultimately received Nucleus 24 devices. One was reimplanted in the same ear, and the other was implanted in the opposite ear. Both have been followed up for 8 months following the reimplantation. MAIN OUTCOME MEASURES: Cochlear implant programming levels, cochlear implant performance, and facial nerve stimulation. RESULTS: The FNS was managed for more than 3 years through optimized programming. However, the FNS progressed until performance dropped below acceptable levels. Reimplantation was believed to be the only option for improvement. After reimplantation and programming, both subjects showed immediate improvement in speech discrimination. One user increased his consonant-nucleus-consonant word score from 12% preoperatively to 42%, and the other's performance increased from 0% to 86%. CONCLUSIONS: Our results suggest that having more programming options with newer devices is critical in otosclerotic or ossified users who experience FNS. Also, reimplantation may be a useful tool to improve performance.

Objective methods in postlingually and prelingually deafened adults for programming cochlear implants: ESR and NRT.

This study compared responses of prelingually and postlingually deafened adult Nucleus 24 cochlear implant users on two objective measures employed to predict programming levels: neural response telemetry (NRT) and electrically evoked stapedial reflexes (eSR). Thirty experienced postlingually and prelingually deafened adult implant users underwent standard behavioural judgements of maximum comfortable loudness levels (C levels) and thresholds (Ts) followed by eSR and NRT measurements. Two different programs were created based on both the subjective judgement and the objective estimates of C levels (eSR thresholds) and these were compared. Relationships between the subjective and the objective measures were statistically analysed. Maximum stimulation levels estimated by both eSR and NRT were highly correlated with C levels. Variability of NRT results was higher than for eSR results. Mean NRT thresholds for postlingually deafened patients were higher than for prelingually deafened patients. A number of prelingually deafened users could distinguish no difference between programs; however, the majority of postlingually deafened users were sensitive to the difference and many reported preference for the program with eSR-estimated C levels. Neural response telemetry thresholds and eSRTs obtained in Nucleus 24 patients are highly correlated with C levels and Ts. Results suggest that estimation of C levels and Ts using NRT or eSR requires different correction factors for prelingually versus postlingually deafened adult subjects.

Facial nerve stimulation after cochlear implantation.

OBJECTIVES: This study was designed to compare the incidence and nature of facial nerve stimulation (FNS) in patients receiving cochlear implants (CI) manufactured by Cochlear Corporation, Advanced Bionics Corporation, and MedEl. STUDY DESIGN: Retrospective chart review at a tertiary referral center. METHODS: The charts of 600 patients who received CIs from 1993 to 2003 with at least 1 year of follow-up were reviewed for significant FNS (FNS on at least 1 channel at functional stimulation levels). Data collected included age, sex, etiology of deafness, device type, electrode, FNS onset after initial stimulation, number and location of electrode contacts causing FNS, and loudness level at which FSN occurred. Nucleus straight and perimodiolar electrodes were also compared. RESULTS: Thirty-nine of 600 (6.5%) patients had FNS on at least one channel, (MedEl 3 of 43 [7.0%], Nucleus 29 of 440 [6.6%], and Clarion 7 of 117 [6.0%]). The incidence of FNS in Nucleus perimodiolar electrodes (16 of 250 [6.4%]) was similar to straight electrodes (13 of 190 (6.8%]), as was the mean number of electrodes causing FNS per patient (11 vs. 12). However, straight electrodes caused stimulation at significantly softer perceived loudness levels than perimodiolar electrodes (P < .0001). CONCLUSIONS: In this large series of CI FNS, the overall incidence of FNS is consistent with previous reports. All devices had a similar incidence of FNS, but perimodiolar electrodes produced FNS only at significantly higher loudness levels than straight electrodes, making them preferable for patients at risk for FNS receiving Nucleus devices.

Changes in programming over time in postmeningitis cochlear implant users.

OBJECTIVE: Although successful cochlear implantation of patients with deafness following meningitis is expected, long-term stability of electrical current requirements has not been systematically evaluated. This study evaluated changes in programming for patients deafened by bacterial meningitis and stability of auditory performance over time. STUDY DESIGN AND SETTING: In this retrospective descriptive study, cochlear implant (CI) stimulation mode and performance of 14 patients deafened by meningitis were compared with those of an age-matched control group of patients deafened by other causes. RESULTS: There were no significant differences in mean performance between the meningitis group and control group (P > 0.05). However, the postmeningitis group required progressively higher stimulation levels and higher programming modes over time as compared to the control group. CONCLUSIONS: Even with deafness accompanied by labyrinthine ossification attributed to meningitis, neural elements were present and could be stimulated. Because increasing levels of stimulation were required over time, postmeningitic children with CIs, and those with cochlear ossification in particular, may need frequent programming adjustments to maintain performance. SIGNIFICANCE: These patients need close follow-up of stimulation levels and programming modes postoperatively in order to perform optimally with CIs. EBM RATING: B-3.

Further prospective findings with compound action potentials from Nucleus 24 cochlear implants.

The purpose of this study was to gain greater understanding of compound action potential (CAP) specific characteristics including: slope of the growth function, P1-N1 amplitude, threshold and latencies of P1 and N1 measured in cochlear implant users. Experienced adult subjects underwent behavioral threshold (T) measurement and electrically elicited stapedial reflex (eSR) recording, followed by CAP measurements on six selected electrodes. Based on the electrically elicited stapedial reflex threshold (eSRT), maximum stimulation level for each measured electrode was set. Relationships among the three thresholds of the above measures and maximum CAP P1-N1 amplitude and slope of the growth function were statistically evaluated for each measured electrode. Threshold of the CAP response showed relationships of similar strength with eSRT and T (r=0.69 and 0.61, respectively). For both slope of the growth function and CAP P1-N1 amplitude, a statistically significant relationship with cochlear place was found. Both specific characteristics of CAP measurement for the most apical electrodes were roughly double those for the most basal electrode (alpha=0.05). This may be partially explained by cochlear anatomy and is consistent with prior mammalian and human studies showing increasing density and survival of spiral ganglion cells in the regions corresponding to intracochlear electrode placement from basal to apical electrodes (90-360 degrees ).

Cochlear implants in children--a review.

Over the past two decades, cochlear implantation has become a widely accepted treatment of deafness in children. Over 20,000 children have received cochlear implants worldwide. Hearing, language and social development outcomes have been positive. We review current issues in cochlear implantation, candidacy, evaluation, surgery, habilitation, ethics and outcomes.