Misplaced Cochlear Implant Electrodes Outside the Cochlea: A Literature Review and Presentation of Radiological and Electrophysiological Findings.

HYPOTHESIS: It is possible to detect when misplacement and malposition of the cochlear implant (CI) electrode array has occurred intraoperatively through different investigations. We aim to explore the literature surrounding cochlear implant misplacements and share our personal experience with such cases to formulate a quick-reference guide that may be able to help cochlear implant teams detect misplacements early. BACKGROUND: Misplacement and malposition of a cochlear implant array can lead to poor hearing outcomes. Where misplacements go undetected during the primary surgery, patients may undergo further surgery to replace the implant array into the correct intracochlear position. METHODS: Systematic literature review on cochlear implant misplacements and malpositions and a retrospective review of our program's cases in over 6,000 CI procedures. RESULTS: Twenty-nine cases of CI misplacements are reported in the English literature. Sixteen cases of cochlear implant misplacements are reported from our institution with a rate of 0.28%. A further 12 cases of intracochlear malpositions are presented. The electrophysiological (CI electrically evoked auditory brainstem response, transimpedance matrix) and radiological (X-ray and computed tomography scan) findings from our experience are displayed in a tabulated quick-reference guide to show the possible characteristics of misplaced and malpositioned cochlear implant electrode arrays. CONCLUSION: Both intraoperative electrophysiological and radiological tests can show when the array has been misplaced or if there is an intracochlear malposition, to prompt timely intra-operative reinsertion to yield better outcomes for patients.

Risk Factors for Facial Nerve and Other Nonauditory Side Effects Following Cochlear Implantation.

OBJECTIVE: The purpose of this study was to characterize a cohort of patients with nonauditory side-effects (NASx) following cochlear implant (CI) surgery. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: One hundred twenty three multichannel CI recipients with intraoperative facial nerve stimulation (FNS). INTERVENTIONS: Intraoperative electrical auditory brainstem responses (eABR) during CI surgery. MAIN OUTCOME MEASURES: Nonauditory side effects post-CI activation. RESULTS: Intraoperative FNS was identified in 2.26% of patients (123/5441), of whom, 34% (42/123) experienced VII stimulation on CI activation. Pain was experienced by 22% (27/123) and vestibular dysfunction was experienced by 4% (5/123) of cases. All case who experienced pain and/or vestibular NASx also experienced VII stimulation. The majority of cases were managed by CI remapping or observation and habituation.Significant relationships were found between etiology of hearing loss and presence of FNS upon initial activation (p < 0.05). No significance was found between FNS intraoperatively and at initial activation for all assumed mechanisms of hearing loss (p > 0.05) with the exceptions of acquired hearing loss of undetermined etiology and toxic etiology group (p < 0.05).There was no significant impact of implant array design (p > 0.05). CONCLUSIONS: This study has characterized patients with NASx in a large cohort of CI patients. One third of cases identified with FNS intraoperatively, developed NASx post-CI activation. Risk factors for NASx postactivation include high-risk etiologies and intraoperative objective measures (i.e., eABR). This may assist surgeons and audiologists to identify at-risk patients who may need modifications in CI program planning.

Intraoperative Cochlear Implant Reinsertion Effects Evaluated by Electrode Impedance.

OBJECTIVES: To assess the effect on impedance levels of intraoperative reinsertion of a cochlear implant (CI) array compared with matched controls. STUDY DESIGN: Retrospective patient review. SETTING: Cochlear implant center. PATIENTS: CI recipients in the Sydney Cochlear Implant Centre (SCIC) database who required intraoperative array reinsertion and matched controls. Exclusion criteria; known preceding meningitis or labyrinthitis ossificans; electrode array buckling; incomplete "final" insertion. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Impedance values measured intraoperatively, at switch on, 3 months, 6 months, and 12 months postoperatively were analyzed. The Generalized Estimating Equation (GEE) Model was used to compare cases with controls for each device, at each time point, and for each channel. RESULTS: Thirty-one reinsertion cases identified; six CI 422 arrays; 14 CI 24RE (ST) arrays, and 11 CI 512 arrays. No increase in impedance levels was found in the reinsertion cases when compared with their matched controls. The only statistical difference in impedance was seen in the CI 422 cohort at switch on with the reinsertion cases having lower impedances (p = 0.03). CONCLUSION: This is the first study to examine impedance values in patients who underwent intraoperative CI array reinsertion and to compare them with the impedances of matched controls. No significant increase found in impedances between our reinsertion cases and matched controls, suggesting the reinsertion did not result in any additional trauma or inflammation. This has implications for surgery both in routine cases such as a faulty electrode and also for future design of mechanisms for delivery of intracochlear therapies.

Cochlear Implant Outcomes in Patients With Severe Compared With Profound Hearing Loss.

OBJECTIVE: To compare the speech perception outcomes for patients with preoperative severe versus profound hearing loss with a cochlear implant (CI). STUDY DESIGN: Retrospective patient review. SETTING: Cochlear implant program. PATIENTS: Cochlear implant adult recipients (16 yr and above) having surgery between 2008 and 2015 with speech perception results and four frequency averaged severe (70-89 dBHL) or profound (90 dBHL and above) hearing loss. Prelingual deaf adults were included in the data. INTERVENTION: Cochlear implant. MAIN OUTCOME MEASURES: Speech perception scores with CUNY sentences and monosyllabic (CNC/CVC) word scores at preoperative and 3, 6, and 12 months postoperatively testing. Mann-Whitney U test was performed to compare outcomes of the two groups. Interquartile comparisons were also made. RESULTS: The severe group had significantly better speech perception than the profound hearing loss group for CUNY sentences and CNC/CVC word scores preoperatively (p < 0.001) (p < 0.001), at 6 months (p < 0.001) (p < 0.001), and at 12 months (p < 0.01) (p < 0.001), respectively. At 3 months there was no significant difference. The number of patients in each severe or profound group at the different time points ranged from 92 to 367 patients for CUNY sentences and from 52 to 216 patients for the word scores. The 12 months' lower quartile score for CUNY sentences for severe and profound groups was 83% and 75% respectively. The lower quartile score for words was 32% and 26% respectively. CONCLUSION: Adult CI recipients showed marked improvements in speech perception with a CI. Those with severe hearing loss have significantly better outcomes compared with profound hearing loss patients. These outcomes can inform CI candidacy evaluation criteria.

Cochlear Implant Outcomes in Large Vestibular Aqueduct Syndrome-Should We Provide Cochlear Implants Earlier?

OBJECTIVE: Examine postoperative speech perception outcomes in a large vestibular aqueduct syndrome (LVAS) patients at a major cochlear implantation center. STUDY DESIGN: Retrospective analysis of the Sydney Cochlear Implant Centre (SCIC) database and medical records from January 1994 to December 2015 was performed. SETTING: Tertiary referral center. PATIENTS: Patients with a diagnosis of LVAS who received a cochlear implant (CI). Only those with speech perception outcomes recorded at least 12 months post implant were included in our analysis. INTERVENTION(S): Therapeutic. MAIN OUTCOME MEASURE(S): Postoperative speech perception scores. RESULTS: Between 1994 and 2015, 176 adult and pediatric patients with a diagnosis of LVAS underwent cochlear implantation at SCIC. Postoperative Bamford-Kowal Bench (BKB) sentence test scores were obtained for 97 patients. The postoperative median BKB score was 93% with a lower quartile score of 85% and an upper quartile score of 98%. Smaller numbers were available for post-CI City University of New York (CUNY) and Consonant-Nucleus-Consonant (CNC) word scores yet similar excellent results were seen. CONCLUSIONS: Our study results suggest the CI should be considered when BKB scores have dropped to 85%. We suggest that rather than LVAS cases representing a challenge to cochlear implantation, they are amongst the best candidates for surgery, and should receive a CI at an earlier stage in hearing loss, when they have better speech perception. This allows stable hearing to be established earlier along with excellent speech perception outcomes.

Impedance, neural response telemetry, and speech perception outcomes after reimplantation of cochlear implants in children.

OBJECTIVE: To compare mean impedance levels, neural response telemetry (NRT), and auditory perception after initial and explant-reimplant pediatric cochlear implants. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral hospital and cochlear implant program. PATIENTS: Children 0 to 16 years inclusive who have undergone explant-reimplant of their cochlear implant. INTERVENTION: Impedance levels, NRT, and speech perception performance. MAIN OUTCOME MEASURES: Impedance, NRT, and auditory perception at switch on, 3 months, 12 months, 3 years, and 5 years after initial cochlear implant and reimplantation. RESULTS: The explant-reimplant group receiving Cochlear contour array had significantly (p < 0.001) raised impedance at switch on, 3 months, 12 months, and 3 years, compared with their initial implant. The explant-reimplant group receiving Cochlear straight array had marginally significant (p = 0.045) raised impedance at switch on, 3 months, 12 months, and 3 and 5 years. Infection was associated with greater increases in impedance in the reimplant Contour group. NRT was increased in the explant-reimplant group but not significantly (p = 0.06). Auditory perception returned to preexplant levels within 6 months in 61% of children. CONCLUSION: Impedance is higher after explant-reimplant and remains increased for years after explant-reimplant with Cochlear contour and to a lesser degree the straight array device.

Preliminary results and technique for electrophysiological intra-operative monitoring of residual hearing during cochlear implantation.

AIMS: To assess the feasibility of intra-operative monitoring of residual hearing using electrocochleography (ECOG) during cochlear implantation. We present our methods and preliminary results. METHODS: We attempted to monitor 21 consecutive paediatric and adult subjects during cochlear implantation. All subjects were implanted via a promontory cochleostomy with full electrode insertions of Cochlear(TM) Nucleus® (Cochlear Nucleus, Cochlear Ltd, Australia) devices: 8 with straight electrodes and 13 with contoured electrodes. Baseline recordings were made after the completion of the posterior tympanotomy, and subsequently at stages of the cochleostomy and electrode insertion. RESULTS: We were unable to obtain recordings on five patients because the ear-insert tubing kinked, which disrupted transmission of the stimulus. Five had insufficient cochlear function to be able to record a baseline ECOG. No subjects in whom we were able to obtain a satisfactory baseline ECOG (n=11) had a reduction in ECOG action potential (AP) amplitude, morphology or threshold up to completion of the cochleostomy. Seven maintained an unchanged ECOG throughout the procedure to full insertion. An implant had to be replaced because of a faulty straight electrode in one patient. The amplitude significantly reduced during insertion of the replacement array. Two had a reduction in amplitude, threshold, and shape of the wave related to inadvertent suction of the perilymph. Subsequently, one maintained this changed ECOG to the end of the procedure, but the other progressed to complete loss of the ECOG during insertion of the array. One other subject had a significant reduction in the amplitude during insertion of the electrode from a depth of approximately 18mm to full insertion. CONCLUSIONS: Intra-operative monitoring of residual hearing may be possible in most patients undergoing cochlear implantation. This pilot study suggests that cochleostomy is not associated with intra-operative loss of residual hearing; ECOG can be preserved during the procedure in most patients; intra-operative loss of hearing is most likely to occur when the tip of the array reaches the basal turn of the cochlea. This risk may increase if the array has to be removed and re-inserted. Suction of perilymph causes immediate changes, which may not recover.

The use of intra-operative electrical auditory brainstem responses to predict the speech perception outcome after cochlear implantation.

Electrically evoked auditory brainstem responses (EABR) were obtained intra-operatively from children receiving full insertion of Cochlear CI24 implant. The EABR waveform was quantified according to the presence or absence of waves (eII-eV). Each trace was scored from 0-3 providing a maximum score for all 22 electrodes of 66 in the monopolar mode. Speech perception scores were measured after one year in 245 children and after two years of cochlear implant use in 148 children. Significant differences in the EABR waveforms were found between those recipients who achieved scores of 4 or greater on the Melbourne scale and those recipients who only had a modest outcome. After two years, the outcome showed even greater differences. The group of 103 children who had outcomes of 4 or greater at one year improved from a mean score of 4.7 to 5.7. The group of 45 children who had outcomes of 3 or less at one year only improved from a mean score of 2.9 to 3.15. In this latter group, only four children showed any significant improvement.

Frequency-specific electrocochleography indicates that presynaptic and postsynaptic mechanisms of auditory neuropathy exist.

OBJECTIVES: The physiological mechanisms underlying auditory neuropathy (AN) remain unclear and it is likely that the multiple disruptions are classified under the broadly defined term. Cochlear implantation is being more widely used in this population to bypass the suspected site-of-lesion although a number of cases have been identified within the Sydney Cochlear Implant Centre where this management strategy has been unsuccessful. It is likely that this relates to the different physiological mechanisms underlying AN. DESIGN: To investigate the site-of-lesion in AN, frequency-specific round window electrocochleography (ECochG) was used to assess local hair-cell, dendritic, and axonal currents generated within the cochlea in 14 subjects with AN and compared with responses from two normally hearing subjects. ECochG results were then compared with electrically evoked auditory brain stem response (EABR) measured after cochlear implantation. RESULTS: The results of this study demonstrate that two dominant patterns of ECochG waveforms (produced by a high-frequency alternating tone burst) can be identified in this population of AN subjects: (a) gross waveform showing a prolonged summating potential (SP) latency that, in most cases, is followed by a small compound action potential; and (b) gross waveform showing a normal latency SP waveform followed by a broad negative potential [assumed to reflect the dendritic potential (DP) identified in anaesthetized guinea-pigs]. This study demonstrates that in most subjects (n = 7) with a prolonged latency SP but no DP, normal morphology EABR waveforms were elicited for all electrode channels. On the other hand, all subjects (n = 7) who showed a normal latency SP followed by a broad negative DP, showed EABR waveforms that were absent or having poor wave V morphology. The authors' interpretation of this is that ECochG results may provide a classification of AN into pre- and postsynaptic lesions. CONCLUSIONS: We suggest that a presynaptic and postsynaptic type of AN exist that may have implications for the fitting of cochlear implants.

Predicting cochlear implant outcomes in children with auditory neuropathy.

OBJECTIVE: To examine the outcome of cochlear implantation in children with auditory neuropathy (AN) and cochlear nerve deficiency (Group A). Results are compared with a cohort of children with AN and normal cochlear nerves (Group B). STUDY DESIGN: Retrospective cohort study. SETTING: The Sydney Cochlear Implant Centre and the Children's Hospital at Westmead. PATIENTS: Children younger than 15 years with bilateral profound sensorineural hearing loss and the diagnosis of AN confirmed on electrophysiologic testing. All children underwent cochlear implantation with Nucleus 24 cochlear implants from 1997 to 2006. INTERVENTIONS: Magnetic resonance imaging was examined for deficiency of the vestibulocochlear nerve. Brain and inner ear abnormalities were recorded. Cochlear implant outcomes and demographic variables were compared. MAIN OUTCOME MEASURES: Melbourne speech perception score (MSPS) at 1 year and implant evoked electric auditory brainstem response (EABR). RESULTS: Group A performed significantly worse on both parameters than Group B. In Group A, median MSPS was 1, compared with a median score of 4 in Group B (z = -3.010; p = 0.003). EABR was abnormal in 13 of 15 (87%) children in Group A, compared with 9 of 39 (23%) in Group B. Children in both groups with abnormal EABR had significantly worse MSPS (z = -2.780; p = 0.005). Fourteen of 15 children with cochlear nerve deficiency had associated inner ear abnormalities. CONCLUSION: Children with AN can have associated cochlear nerve deficiency. These patients have worse speech perception scores at 1 year post cochlear implantation, higher rates of abnormal EABR, and more associated inner ear abnormalities than children with AN and normal cochlear nerves.

Meningitis resulting in hearing loss and labyrinthitis ossificans - does the causative organism matter?

Our aim was to demonstrate whether one causative agent of meningitis is more likely to cause profound hearing loss and labyrinthitis ossificans. We obtained data from the New South Wales health department for cases of meningitis between 1995 and 2005 (1568 cases) and the Sydney Cochlear Implant Centre for cochlear implant patients with hearing loss secondary to meningitis from 1984 to 2005 (70 ears in 59 patients). The aetiological agents were compared with regard to their ability to cause profound hearing loss and cochlear ossification. Neisseria meningitidis resulted in 56.9% of cases of meningitis and 11.4% of the cases of profound hearing loss resulting in cochlear implantation (incidence of profound hearing loss of 0.4%). Streptococcus pneumoniae, however, caused 41.1% of meningitis but 85.7% of cochlear implantation (incidence of 4.6%). Labyrinthitis ossificans was more common after infection with Streptococcus pneumoniae but there was no statistically significant difference between Streptococcus pneumoniae, Haemophilus influenzae or Neisseria meningitidis for labyrinthitis ossificans (p = 0.45, chi-squared test). In conclusion Neisseria meningitidis meningitis carries a very low risk of profound hearing loss but Streptococcus pneumoniae meningitis a significantly higher risk.

Auditory neuropathy: an update.

OBJECTIVES: To describe the round window electrocochleography (RWECochG) and electric auditory brainstem responses (EABR) in ears affected by auditory neuropathy (AN), and to determine if these electrophysiological tests can predict the outcome following cochlear implant surgery. METHODS: A longitudinal study of all pediatric cochlear implant patients between 1994 and 2005 was undertaken. Speech perception outcomes after cochlear implantation and electrophysiological data were collected prospectively and analyzed. Some otoacoustic emissions (OAE) data were collected retrospectively during the neonatal period. All subjects were tested using round window electrocochleography (RWEcochG), auditory brainstem responses (ABR), and implant-evoked electric auditory brainstem responses (EABR). The auditory neuropathy (AN) group consisted of 39 children (78 ears) which had present OAE and absent or grossly abnormal ABR (a broad N1 component only). RESULTS: All 78 ears from the 39 AN children showed large cochlear microphonics (CM) and an abnormal positive potential (APP) using RW ECochG. A further 21 children showed large CM and APP but had not been tested for OAE. In total, 60 children were discovered to have APP among 435 pediatric patients who received a cochlear implant. Electrically evoked ABR (EABR) from the implanted ear were normal in 45 and abnormal in 15. 46 age matched patients without large CM and APP were used as a control group. Two year postimplant scores (Melbourne categories) were: 6.27 (APP and normal EABR), 2.25 (APP and abnormal EABR) and 5.37 (control group). Mann-Whitney U Test for nonparametric data was used to test for significant difference at significance level p < 0.005 (two tailed). The APP ears which provided normal EABR had significantly better outcomes after cochlear implantation than APP ears which had abnormal EABR. Furthermore, the APP ears which provided normal EABR performed significantly better after cochlear implant surgery than the control group of patients with no OAE, appropriate ABR results and normal EABR. CONCLUSIONS: Ears affected by AN provide large CM and APP on RW ECochG. The presence of normal EABR may indicate a significantly better outcome after cochlear implant surgery than for those APP ears which had abnormal or absent EABR. Based on these findings it is suggested that the presence of APP and/ or OAE in 75% of the ears which have absent or abnormal ABR may not indicate a pathological condition affecting the auditory nerve or synapse but only survival of outer hair cells despite extensive loss of inner hair cells.

Trans-tympanic electric auditory brainstem response (TT-EABR): the importance of the positioning of the stimulating electrode.

Trans-tympanic electric auditory brainstem response (TT-EABR) is an established pre-operative investigation in cochlear implantation surgery. Various techniques have been employed to obtain electrical responses but there has been no universal agreement on the exact positioning of the stimulating electrode on the medial wall of the mesotympanum. The authors investigate the relationship of the positioning of the electrode and the brainstem response.

Cochlear implantations in children with Waardenburg syndrome: an electrophysiological and psychophysical review.

Waardenburg syndrome presents with dystopia canthorum, pigmentary abnormalities of hair, iris and skin (often a white forelock and heterochromia iridis) and sensorineural deafness. The authors review the electrophysiological and psychophysical findings of implanted children with Waardenburg syndrome at the Sydney Cochlear Implant Centre. Twenty children with Waardenburg syndrome received cochlear implants between 1985 and 2001. Electrical auditory brainstem response (EABR) was performed in all of these patients intra-operatively as part of the routine investigations. Only 13 of these patients were assessed one year or more post-operatively by means of the Melbourne Categories (0-7). Four patients (20%) were found to have abnormal EABR recordings. The mode of Melbourne Categories in this group (n = 3) was 1 at one year post-operation. The other 16 patients were found to have normal EABR and the mode of Melbourne Categories in this group (n = 10) was 7. A poor outcome after cochlear implantation was associated with abnormal EABR recordings (a 'true' auditory neuropathy) and was found in a significant proportion of patients with Waardenburg syndrome.

Displacement of electrodes of a cochlear implant into the vestibular system: intra- and postoperative electrophysiological analyses.

A cochlear implant device is normally inserted into the scala tympani via a posterior tympanotomy and cochleostomy. There has been no previous report of displacement of the array into the vestibular part of the labyrinth. The authors present and discuss the audiological and electrophysiological measurements of a case in which part of the array herniated through into the vestibule.

Role of auditory stimulation in maturation of the auditory pathway.

OBJECTIVE: To compare the maturation of the auditory pathway, as shown by electrical brainstem auditory potentials (EABRs), in ears with and without prior auditory stimulation. MATERIAL AND METHODS: Electrophysiological data were collected prospectively from ears which had received cochlear implants. Implant-evoked (Imp)EABRs were recorded. Thirty children, implanted after January 2000, were selected according to a strict inclusion/exclusion protocol. All the children had received a 22-channel Nucleus cochlear implant (CI24 series). Intraoperatively, ImpEABRs were recorded using the Medelec Synergy Evoked Response system in conjunction with Nucleus Neural Response Telemetry software. The ImpEABR latencies of waves eII, eIII and eV and the morphology of wave eV were assessed. RESULTS: ImpEABRs alter during the first 12 months of life. The latency becomes shorter during this period and the morphology of wave eV alters from a broad shape to a more distinct waveform. This appears to occur independently, even in the absence of auditory stimulation. CONCLUSION: The development of electrical brainstem auditory potentials is not dependent on auditory stimulation.

Surgical complications of 844 consecutive cochlear implantations and observations on large versus small incisions.

OBJECTIVES: To list the complications encountered in a series of 844 consecutive patients and to evaluate the long-term difference in wound and flap problems between large and small incisions. PATIENTS: 844 consecutive patients underwent cochlear implantation at the Sydney Cochlear Implant Centre. 212 cases were operated on prior to October 1994 using the retroauricular 'C'-shaped incision or a postauricular incision with a horizontal posterior limb. After October 1994 a new, small vertical postaural incision was used in all patients. Postoperative problems were analysed. STUDY DESIGN: Prospective longitudinal study of cochlear implant recipients from1984 to 2003. SETTING: Tertiary care referral centre. INTERVENTION CHANGE IN INCISION FOR COCHLEAR IMPLANTATION. MAIN OUTCOME MEASURE: Causes of postoperative problems, need for reimplatation, and wound and flap problems. RESULTS: 80 out of the total 844 patients underwent revision procedures for various reasons. The commonest cause of reimplantation was device failure (2.01%) or suboptimum performance of the device (2.37%). Wound and flap problems were encountered by 5 patients out of 212 (2.3%) in the first group. In comparison, 7 out of 632 patients (1.10%) from the later group experienced wound and flap problems using the new incision. CONCLUSION: There is a reduced incidence of wound and flap problems with small skin incisions and minimal scalp mobilization. Device failure, wound and flap problems are still the commonest causes of explantation. Performance of the replacement device usually was similar to the original device and was not related to the aetiology of deafness or to the cause of explantation. These data will be useful in counselling patients for reimplantation/revision surgery.