Effect of Proximity to the Modiolus for the Cochlear CI532 Slim Modiolar Electrode Array on Evoked Compound Action Potentials and Programming Levels.

BACKGROUND: The first surgeries with CI532 showed an effect of the proximity of the electrode to the modiolus on the Evoked Compound Action Potentials (ECAPs). OBJECTIVES: Objectives of the study were to investigate the effect of the "pullback" procedure on intraoperative ECAP responses in three different electrode array positions and additionally to compare behavioral thresholds with the thresholds obtained in a group of patients using the standard insertion. The hypothesis of this study is that pullback will cause lower ECAPs and behavioral thresholds. PATIENTS: The study included 40 patients, 20 in the pullback insertion group and 20 in the standard insertion group (without pullback). METHOD: During insertion of the CI532 electrode array, ECAP was performed in three different positions for the pullback group: at initial insertion, at over-insertion, and after pullback. Insertion was monitored by fluoroscopy. In the standard group, ECAP was performed at the initial position, which is also the final position. ECAP thresholds (T-ECAPs) were compared within subjects at the initial and the final position in the pullback group and between groups in the final positions of the pullback and standard groups. Programming levels (C- and T-levels) were compared between the two groups 1 year after switch-on. RESULTS: Intraoperative measurements pullback shows lower average T-ECAPs after pullback compared to thresholds in initial position. Comparison of intraoperative T-ECAPs at the final positions showed no statistically significant difference between the pullback group and the standard insertion group. Furthermore, 1 year after switch-on there was no statistically significant difference in C- and T-levels between the two groups. CONCLUSION: The pullback maneuver of the CI532 electrode array after an over-insertion gave significantly lower T-ECAPs compared to the thresholds at the initial position. However, the between-groups analysis of pullback and standard insertion showed neither significantly different T-ECAPs nor different programming levels. Because T-ECAPs and programming levels vary considerably between subjects, large groups are required to detect differences between groups. Additionally, the effect pullback technique to preserving the residual hearing is not known yet.

Long-term ototoxicity in women after cisplatin treatment for ovarian germ cell cancer.

OBJECTIVE: Evaluate long-term cisplatin-induced ototoxicity in women treated for malignant ovarian germ cell tumors (MOGCT). METHODS: Seventy-four women treated for MOGCT in Norway (1980-2009) were analyzed: 41 had received cisplatin-based chemotherapy (CBCT) ("Cases") and 33 had no CBCT ("Controls"). Median follow-up was 15years. Hearing was assessed by pure tone audiometry and by the SCIN questionnaire. Air conduction thresholds were reported as absolute hearing thresholds and age-adjusted thresholds. Absolute and age-adjusted hearing loss were defined as thresholds of >20dB at any frequency. Tinnitus was evaluated using the Tinnitus Handicap Inventory. Serum Platinum Concentration (SPC) was determined. RESULTS: Absolute hearing loss was identified in 21 Cases (51%) and 24 Controls (73%). After adjusting for age, only 9 Cases (22%) and 5 Controls (15%) remained. Age-adjusted hearing thresholds at 4, 6 and 8kHz were slightly but significantly higher in Cases compared to Controls. Subjective hearing loss was reported by 27% of Cases and 21% of Controls, who were significantly older. Elevated SPC values were detected up to 20years after CBCT, but SPC did not correlate significantly with age-adjusted hearing loss. The rate of tinnitus was similar in Cases and Controls. CONCLUSION: Long-term MOGCT survivors treated with CBCT have small but significant reductions in age-adjusted hearing thresholds at 4, 6 and 8kHz versus Controls. Approximately one in four women experienced subjective hearing loss. To avoid overestimation of clinically relevant cisplatin-induced ototoxicity, absolute hearing thresholds should be age-adjusted and compared to an age-matched control group.

Superior semicircular canal dehiscence syndrome. / Buegangsdehiscens.

Superior semicircular canal dehiscence syndrome is a condition with troubling ear symptoms and vertigo caused by a defect in the bone between the inner ear and the middle cranial fossa. The disease is not dangerous, and for many patients it is sufficient to provide a thorough explanation for the symptoms and advice about coping strategies, balance exercises and, if necessary, use of assisted hearing devices. Surgical treatment may be appropriate for patients with severe symptoms.

Insertion Depth in Cochlear Implantation and Outcome in Residual Hearing and Vestibular Function.

OBJECTIVES: It has long been known that cochlear implantation may cause loss of residual hearing and vestibular function. Different insertion depths may cause varying degrees of intracochlear trauma in the apical region of the cochlea. The present study investigated the correlation between the insertion depth and postoperative loss of residual hearing and vestibular function. DESIGN: Thirty-nine adults underwent unilateral cochlear implantation. One group received a Med-El +Flex electrode array (24 mm; n = 4), 1 group received a Med-El +Flex electrode array (28 mm; n = 18), and 1 group received a Med-El +Flex electrode array (31.5 mm; n = 17). Residual hearing, cervical vestibular-evoked myogenic potentials, videonystagmography, and subjective visual vertical/horizontal were explored before and after surgery. The electrode insertion depth and scalar position were examined with high-resolution rotational tomography after implantation in 29 subjects. RESULTS: There was no observed relationship between the angular insertion depth (405° to 708°) and loss of low-frequency pure-tone average. Frequency-specific analysis revealed a weak relationship between the angular insertion depth and loss of hearing at 250 Hz (R= 0.20; p = 0.02). There was no statistically significant difference in the residual hearing and vestibular function between the +Flex and the +Flex electrode array. Eight percent of the cases had vertigo after surgery. The electrode arrays were positioned inside the scala tympani and not scala vestibuli in all subjects. In 18% of the cases, the +Flex electrode array was not fully inserted. CONCLUSIONS: The final outcome in residual hearing correlates very weakly with the angular insertion depth for depths above 405°. Postoperative loss of vestibular function did not correlate with the angular insertion depth or age at implantation. The surgical protocol used in this study seems to minimize the risk of postoperative vertigo symptoms.

Deep round window insertion versus standard approach in cochlear implant surgery.

The aim of this study was to compare the outcomes of vestibular tests and the residual hearing of patients who have undergone full insertion cochlear implant surgery using the round window approach with a hearing preservation protocol (RW-HP) or the standard cochleostomy approach (SCA) without hearing preservation. A prospective study of 34 adults who underwent unilateral cochlear implantation was carried out. One group was operated using the RW-HP (n = 17) approach with Med-El +Flex(SOFT) electrode array with full insertion, while the control group underwent a more conventional SCA surgery (n = 17) with shorter perimodiolar electrodes. Assessments of residual hearing, cervical vestibular-evoked myogenic potentials (cVEMP), videonystagmography, subjective visual vertical/horizontal (SVH/SVV) were performed before and after surgery. There was a significantly (p < 0.05) greater number of subjects who exhibited complete or partial hearing preservation in the deep insertion RW-HP group (9/17) compared to the SCA group (2/15). A higher degree of vestibular loss but a lower degree of vertigo symptoms could be seen in the RW-HP group, but the differences were not statistically significant. It is possible to preserve residual hearing to a certain extent also with deep insertion. Full insertion with hearing preservation was less harmful to residual hearing particularly at 125 Hz (p < 0.05), than was the standard cochleostomy approach.

Cochlear-facial dehiscence - the most common cause of facial nerve stimulation from a cochlear implant? A case-control study.

OBJECTIVES: To investigate the prevalence of cochlear-facial dehiscence (CFD) and other radiographical pathologies in ears with facial nerve stimulation (FNS) from a cochlear implant (CI). METHODS: Retrospective case-control study of 27 patients with CI and FNS on either ear (study group) and 27 patients without FNS, matched for age, sex and type of electrode array (control group). Preoperative CT scans of all 108 ears were re-evaluated. Subanalyses included comparisons between the study and control groups and associations between FNS and radiographic pathologies. RESULTS: CFDs were detected in 20 of 54 ears (37%) in the study group and in 3 of 54 ears (6%) in the control group (P < 0.001). The corresponding numbers of otosclerosis were 10 (18%) and 0 (P = 0.011) and of developmental anomalies 16 (30%) and 8 (15%) (not significant). FNS was present in 33 ears in the study group, of which 14 (42%) had a CFD. FNS was absent in six ears with CFD and CI, four of which contralateral to an ear with FNS. Eight of 14 ears with FNS and CFD had a lateral electrode array and six had a perimodiolar electrode array. We found no association between the presence of CFD and stimulation thresholds for FNS. The adjusted odds ratio for developing FNS in the presence of a CFD was 9.9 (95% CI 2.7-36.0). CONCLUSIONS: CFD was the most common radiographic pathology in ears with FNS, with a 10-fold increased risk of FNS. To avoid CI-related FNS, preoperative CT scan and awareness of typical dehiscence symptoms are strongly recommended.

Intraoperative Measured Electrocochleography and Fluoroscopy Video to Detect Cochlea Trauma.

HYPOTHESIS: Gross electrode movements detected with intraoperative, real-time X-ray fluoroscopy will correlate with fluctuations in cochlear output, as measured with intraoperative electrocochleography (ECochG). BACKGROUND: Indications for cochlear implantation (CI) are expanding to include patients with residual hearing; however, implant recipients often lose residual hearing after CI. The objective of this study was to identify probable traumatic events during implantation by combining electrophysiological monitoring of cochlear function with simultaneous X-ray monitoring. The surgical timing of these apparently traumatic events was then investigated. METHODS: For 19 adult patients (21 surgeries, 2 bilateral), the ECochG responses were measured during implantation of a cochlear nucleus slim modiolar electrode (CI532/CI632, Cochlear Ltd Australia Nucleus slim modiolar). Simultaneous fluoroscopy was performed, as well as a postoperative cone-beam computed tomography (CT) scan. For all patients, pre- and postoperative audiograms were recorded up to 1 year after surgery to record the loss of residual hearing. RESULTS: Electrode insertions for 21 surgeries were successfully monitored. A drop in ECochG response was significantly correlated with reduced hearing preservation compared with patients with preserved responses throughout. Drops in the ECochG response were measured to occur during insertion, because of movement of the array after insertion was complete, including while sealing of the electrode array at the round window or coiling of the array lead within the mastoid cavity. In some patients, a reduction in cochlear output, resulting in poor ECochG response, was inferred to occur before the beginning of implantation. CONCLUSION: The combination of perioperative ECochG measurements, microscope video, fluoroscopy, and postoperative CT scan may inform on what causes the loss of residual hearing after implantation. These findings will be used to improve the surgical procedure in future.

Long-Term Outcomes of Round Window Reinforcement for Superior Semicircular Canal Dehiscence Syndrome.

OBJECTIVE: To report long-term patient reported outcome measures (PROMs) after standardized round window rein-forcement (RWR) with thin cartilage and perichondrium for superior semicircular canal dehiscence syndrome (SSCDS). METHODS: Cross-sectional survey in 2020 and retrospective longitudinal study of outcomes at 3 months, 1 year, and last follow-up after 28 consecutive RWRs. Endpoints were the last follow-up for PROMs and 1 year postoperatively for audiometry and cVEMP. RESULTS: At the last follow-up (mean 2.4 years postoperatively), improvement was reported for 19/28 ears (68%) in symptoms overall; for 17/27 ears (63%) in auditory symptoms, and for 12/24 (50%) in vestibular symptoms. Nine ears (32%) required further surgery. No major complications occurred. On the group level, postoperative improvement at 3 months declined significantly for auditory and vestibular symptoms over time, and stayed stable beyond 1 year. Improved ears had on average 2.9 years follow-up. Improvement correlated significantly with SSCD length, and was associated with contralateral dehiscence. Thirteen ears (46%) had another potential cause of vestibular symptoms. This group showed significantly less improvement. Audiometric test results did not predict PROMs. CONCLUSIONS: Since long-term outcomes do not compare with those reported for plugging and repair, RWR is not suggested as a first line intervention for SSCDS. Considering the stable rates of improvement on average 2.9 years postoperatively and the absence of major complications, RWR may be an alternative to plugging or repair in fragile patients who do not accept the risk of complications with more invasive surgery, and who accept that results may deteriorate over time.

Cochlear Implantation for Patients with a Vestibular Schwannoma: Effect on Tinnitus Handicap.

BACKGROUND: Tinnitus is a common symptom among individuals with a vestibular schwannoma. In recent years, cochlear implantation, often combined with tumor resection, is an increasingly used option in the management of these patients. The existing literature does not account well for the effect of treatment on tinnitus burden. Thus, this paper reports the effect of cochlear implantation on tinnitus in a cohort of vestibular schwannoma patients. METHODS: Individuals with vestibular schwannoma undergoing cochlear implantation were retrospectively reviewed for tinnitus burden, as evaluated by the Tinnitus Handicap Inventory, administered before and after implantation. The outcome measures were total Tinnitus Handicap Inventory score and scores from each of the Tinnitus Handicap Inventory subdomains (functional, emotional, and catastrophic). In addition, the existing literature on tinnitus in cochlear implanted vestibular schwannoma patients was reviewed. RESULTS: Tumor management consisted of simultaneous resection (77%), previous resection (9%), observation (9%) and radiation (5%). Complete Tinnitus Handicap Inventory evaluation was available for 17 patients. After implantation, the median THItotal changed from 18 to 10 (P = .0006), the subdomain THIfunctional from 10 to 3 (P = .006), the THIemotional from 3 to 0 (P = .023) and the THIcatastrophic from 6 to 1 (P = .004). In the scarcely reported tinnitus outcomes in the literature, most but not all cases experienced a decrease in tinnitus. CONCLUSIONS: The tinnitus burden is significantly reduced by cochlear implantation in individuals with a vestibular schwannoma. This agrees with findings for other etiologies indicating cochlear implantation and supports the eligibility for hearing rehabilitation with a cochlear implant for this specific group of patients.

Fusion of Technology in Cochlear Implantation Surgery: Investigation of Fluoroscopically Assisted Robotic Electrode Insertion.

The HEARO cochlear implantation surgery aims to replace the conventional wide mastoidectomy approach with a minimally invasive direct cochlear access. The main advantage of the HEARO access would be that the trajectory accommodates the optimal and individualized insertion parameters such as type of cochlear access and trajectory angles into the cochlea. To investigate the quality of electrode insertion with the HEARO procedure, the insertion process was inspected under fluoroscopy in 16 human cadaver temporal bones. Prior to the insertion, the robotic middle and inner ear access were performed through the HEARO procedures. The status of the insertion was analyzed on the post-operative image with Siemens Artis Pheno (Siemens AG, Munich, Germany). The completion of the full HEARO procedure, including the robotic inner ear access and fluoroscopy electrode insertion, was possible in all 16 cases. It was possible to insert the electrode in all 16 cases through the drilled tunnel. However, one case in which the full cochlea was not visible on the post-operative image for analysis was excluded. The post-operative analysis of the electrode insertion showed an average insertion angle of 507°, which is equivalent to 1.4 turns of the cochlea, and minimal and maximal insertion angles were recorded as 373° (1 cochlear turn) and 645° (1.8 cochlear turn), respectively. The fluoroscopy inspection indicated no sign of complications during the insertion.

The Relationship Between Cisplatin-related and Age-related Hearing Loss During an Extended Follow-up.

OBJECTIVES: Cisplatin-related hearing loss (HL) is claimed to progress after treatment. This controlled longitudinal study with extended follow-up investigates HL in testicular cancer survivors (TCSs) after cisplatin-based chemotherapy (CBCT). STUDY DESIGN: Controlled longitudinal study. METHODS: Eighty-two TCSs treated with CBCT between 1980 and 1994 in Norway participated in two surveys (S1/S3), including pure-tone audiograms (0.125-8 kHz) and self-reported HL, 12 and 31 years after treatment, respectively. Hearing thresholds were age-adjusted based on age-matched hearing thresholds from the general population (controls). Hearing loss was defined as thresholds >20 dB at any frequency. RESULTS: Between the two surveys, the prevalence of high-frequency HL (4, 6, and 8 kHz) increased from 73% to 94% but approached those of the aging general population after age adjustment. In TCSs aged >40 years at first survey, HL at the subsequent survey equaled that of controls. Self-reported HL increased from seven (9%) at S1 to 20 (26%) at S3. At S1, age-adjusted HL was identified in all (seven) TCSs reporting decreased hearing whereas at S3, hearing thresholds did not differ from controls in seven out of 20 patients reporting HL. CONCLUSION: CBCT-related ototoxicity causes high-frequency HL, but in contrast to reports from follow-up studies from the first post-treatment decade, no major progression was found beyond the first post-treatment decade for frequencies 0.125-8 kHz. Importantly, with extended follow-up, hearing thresholds of patients approach those of the general population, possibly due to a less-than-additive effect with age-related hearing loss (ARHL) in CBCT-treated patients. Age-and sex-matching is strongly advised in long-term follow-up of CBCT-related ototoxicity. Specificity for detecting ototoxicity with self-reported questionnaires decreases with extended follow-up. LEVEL OF EVIDENCE: 3 Laryngoscope, 130:E515-E523, 2020.

Cochlear implants in eight children with Down Syndrome - Auditory performance and challenges in assessment.

OBJECTIVES: A small proportion of children with Down Syndrome (DS) have severe to profound hearing loss and may potentially benefit from a cochlear implant (CI). Evidence on outcomes in DS is very limited, and there is a need for further investigation to provide a basis for clinical evaluation of candidates and outcomes. This study aims to explore outcomes of CI in children with DS in Norway. METHODS: Data on all children with DS and CI in Norway were collected from the CI registry and patients' records at the national pediatric CI center. Main outcome measures were: use of CI, Category of Auditory Performance (CAP), Speech Intelligibility Rate (SIR), and parents' and caregivers' views of the benefits of CI. RESULTS: Eight children with DS have received CI in Norway, all bilaterally. The outcomes varied greatly. All children used their CIs, and all of them responded to environmental sounds. Four children reached CAP score 5 after several years of use, (i.e., they understand phrases without lip reading). All children scored at least 2, (i.e., responds to speech sounds). One child reached a SIR score of 3, (i.e. connected speech is intelligible to experienced listeners). The rest of the children reached SIR scores of 1 or 1-2, (i.e., connected speech is unintelligible). Without exception, parents had an entirely positive attitude to their children using a CI. Co-morbidity and middle ear disease frequently delayed implantation. CONCLUSIONS: Our experience with CI in children with DS is positive. However, CI cannot replace other types of communication for these children, and it is important to give parents realistic expectations prior to surgery. Outcomes might be considered limited when evaluated with instruments for testing auditory performance and speech intelligibility constructed for children without additional disabilities. We do not believe that such outcomes reflect the benefit in real life.

The impact of ethnicity on cochlear implantation in Norwegian children.

OBJECTIVES: To explore the impact of parental ethnicity on cochlear implantation in children in Norway with regard to incidence rates of cochlear implants (CIs), comorbidies, age at onset of profound deafness (AOD), age at first implantation, uni- or bilateral CI, and speech recognition. METHOD: This retrospective cohort study included all children (N = 278) aged <18 years in Norway who received their first CI during the years 2004-2010. RESULTS: 86 children (30.9%) in our study sample had parents of non-Nordic ethnicity, of whom 46 were born in Nordic countries with two non-Nordic parents. Compared with the background population, children with non-Nordic parents were 1.9 times more likely to have received CI than Nordic children (i.e., born in Nordic countries with Nordic parents). When looking at AOD, uni-vs. bilateral CIs, and comorbidities, no significant differences were found between Nordic children and children with a non-Nordic ethnicity. Among children with AOD <1 year (n = 153), those born in non-Nordic countries with two non-Nordic parents (n = 6) and adopted non-Nordic children (n = 6) received their first CI on average 14.9 and 21.1 months later than Nordic children (n = 104), respectively (p = 0.006 and 0.005). Among children with AOD <1 year, those born in Nordic countries with two non-Nordic parents (n = 31) received their CI at an older age than Nordic children, but this difference was not significant after adjusting for calendar year of implantation and excluding comorbidity as a potential cause of delayed implantation. The mean age at implantation for children with AOD <1 year dropped 2.3 months/year over the study period. The mean monosyllable speech recognition score was 84.7% for Nordic children and 76.3% for children born in Norway with two non-Nordic parents (p = 0.002). CONCLUSIONS: The incidence of CI was significantly higher in children with a non-Nordic vs. a Nordic ethnicity, reflecting a higher incidence of profound deafness. Children born in Norway have equal access to CIs regardless of their ethnicity, but despite being born and receiving care in Norway, prelingually deaf children with non-Nordic parents are at risk of receiving CI later than Nordic children. Moreover, prelingually deaf children who arrive in Norway at an older age may be at risk for a worse prognosis after receiving a CI due to lack of auditory stimulation in early childhood, which is critical for language development and late implantation; this is a serious issue with regard to deafness among refugees.

Evaluation of cost-utility in middle ear implantation in the 'Nordic School': a multicenter study in Sweden and Norway.

CONCLUSION: Hearing restoration using an active middle ear implant (AMEI) is a highly cost-effective treatment for a selected group of patients with no other possibilities for auditory rehabilitation. OBJECTIVES: To evaluate the cost-utility of using an AMEI for hearing rehabilitation. METHODS: This was a prospective, multicenter, single-subject repeated study in six tertiary referral centers. Twenty-four patients with sensorineural (SNHL), conductive (CHL), and mixed hearing loss (MHL) were implanted with the AMEI Vibrant Soundbridge® (VSB) for medical reasons. All patients were previously rehabilitated with conventional hearing aids. Multiple validated quality of life patient questionnaires, Health Utilities Index (HUI 2 and 3), and Glasgow Hearing Aid Benefit Profile (GHABP) were used to determine the utility gain and quality adjusted life years (QALY). Directly related treatment costs for the implantation were calculated and related to utility gain and QALY. RESULTS: The cost/QALY for patients with SNHL was estimated at €7260/QALY, and for patients with C/MHL at €12 503/QALY.

European Bilateral Pediatric Cochlear Implant Forum consensus statement.

This is a consensus statement on pediatric cochlear implantation by the European Bilateral Pediatric Cochlear Implant Forum. The consensus statement was determined by review of current scientific literature to identify areas of scientific and clinical agreement of current understanding of bilateral cochlear implantation. The statement is "Currently we feel that the infant or child with unambiguous cochlear implant candidacy should receive bilateral cochlear implants simultaneously as soon as possible after definitive diagnosis of deafness to permit optimal auditory development; an atraumatic surgical technique designed to preserve cochlear function, minimize cochlear damage, and allow easy, possibly repeated re-implantation is recommended."