Safety and Early Outcomes of Cochlear Implantation of Nucleus Devices in Infants: A Multi-Centre Study.

This multi-center study examined the safety and effectiveness of cochlear implantation of children between 9 and 11 months of age. The intended impact was to support practice regarding candidacy assessment and prognostic counseling of pediatric cochlear implant candidates. Data in the clinical chart of children implanted at 9-11 months of age with Cochlear Ltd devices at five cochlear implant centers in the United States and Canada were included in analyses. The study included data from two cohorts implanted with one or two Nucleus devices during the periods of January 1, 2012-December 31, 2017 (Cohort 1, n = 83) or between January 1, 2018 and May 15, 2020 (Cohort 2, n = 50). Major adverse events (requiring another procedure/hospitalization) and minor adverse events (managed with medication alone or underwent an expected course of treatment that did not require surgery or hospitalization) out to 2 years post-implant were monitored and outcomes measured by audiometric thresholds and parent-reports on the IT-MAIS and LittlEARS questionnaires were collected. Results revealed 60 adverse events in 41 children and 227 ears implanted (26%) of which 14 major events occurred in 11 children; all were transitory and resolved. Improved hearing with cochlear implant use was shown in all outcome measures. Findings reveal that the procedure is safe for infants and that they show clear benefits of cochlear implantation including increased audibility and hearing development.

Invasive Pneumococcal Disease in High-risk Children: A 10-Year Retrospective Study.

BACKGROUND: Despite the availability of conjugate pneumococcal vaccines, children with high-risk conditions remain vulnerable to invasive pneumococcal disease (IPD). This study sought to describe IPD prevalence, vaccination and outcomes among high-risk children. METHODS: We used International Classification of Disease10 discharge and microbiology codes to identify patients hospitalized for IPD at a large pediatric hospital from January 1, 2009, to December 31, 2018. Patients were considered high-risk if they had: primary immunodeficiency, asplenia, transplant, active malignancy, sickle cell disease, cochlear implant, nephrotic syndrome, chronic lung disease, cerebrospinal fluid leak, HIV or used immunosuppressive therapy. RESULTS: In total 94 high-risk patients were hospitalized for IPD. The most common high-risk conditions included malignancy (n = 33, 35%), solid-organ or bone marrow transplant (n = 17, 18%) and sickle cell disease (n = 14, 15%). Bacteremia was the most common presentation (n = 81, 86%) followed by pneumonia (n = 23, 25%) and meningitis (n = 9, 10%). No deaths occurred. Of 66 patients with known pneumococcal vaccination status, 15 (23%) were unvaccinated, and 51 (77%) received at least one dose of a pneumococcal vaccine; 20 received all four recommended pneumococcal conjugate vaccine (PCV) doses. Only three children received PPSV23. Of 20 children with no or partial (<3 doses) immunization, 70% (14) of IPD episodes were due to vaccine-preventable serotypes. Of 66 known IPD serotypes, 17% (n = 11) were covered by PCV13, 39% (n = 26) were covered by PPSV23 and 39% (n = 26) were nonvaccine serotype. CONCLUSIONS: Despite the availability of effective pneumococcal vaccines, IPD persists among children with high-risk conditions. Improving PCV13 and PPSV23 vaccination could significantly reduce IPD; most episodes were due to vaccine-preventable serotypes in incompletely immunized patients.

Longitudinal Effects of Simultaneous and Sequential Bilateral Cochlear Implantation on Cortical Auditory-Evoked Potentials Recorded at Cz in a Large Cohort of Children.

OBJECTIVES: Auditory development after bilateral cochlear implantation in children has been measured using source localization of multi-channel late latency responses. It is not clear, however, whether this development can be tracked using a more clinically feasible method of recording from one active recording electrode placed at mid-line center of the head (Cz). DESIGN: In this prospective cohort study, cortical auditory-evoked potential responses (CAEPs) were recorded from Cz referenced to each earlobe (Cz-CAEP) from 222 children with bilateral cochlear implant (CI); 128 (mean ± SD age: 2.78 ± 3.30 years) received both CIs in the same surgery (simultaneous group) and 94 (aged 7.72 ± 4.45 years) received a second CI after 4.21 ± 2.98 years of unilateral CI use. We sought to (1) identify cortical development over the first couple of years of bilateral CI use; (2) measure known asymmetries in auditory development between the CIs; and (3) detect the effects of bilateral rather than unilateral CI use. 4556 Cz-CAEPs were recorded across the cohort over 33.50 ± 7.67 months duration of bilateral CI use. Given concerns related to peak picking, amplitude areas were measured across two response time windows (50 to 199 ms and 200 to 400 ms). RESULTS: Results indicated that small response amplitudes occur at initial CI use and amplitudes increase in the negative or positive direction rapidly over the first months of CI use in both time windows. Asymmetries between Cz-CAEPs evoked by each CI were found in the sequential group and reduced with bilateral CI use, particularly in the first time window; these differences increased with longer inter-implant delay. Bilaterally evoked Cz-CAEPs were larger in amplitude than unilateral responses from either CI in the simultaneous group. In the sequential group, bilateral responses were similar to responses from the first implanted side but increased in relative amplitude with bilateral CI use. The Cz-CAEP measures were not able to predict asymmetries or bilateral benefits in speech perception measures. CONCLUSIONS: The Cz-CAEP was able to indicate cortical detection of CI input and showed gross morphological changes with bilateral CI use. Findings indicate Cz-CAEPs can be used to identify gross changes in auditory development in children with bilateral CIs, but they are less sensitive to tracking the remaining abnormalities that are measured by multi-channel CAEPs and speech perception testing.

Endoscopic Repair of Round Window Cochleocele.

OBJECTIVE: To describe the novel presentation, implications, and endoscopic management of a congenital round window cochleocele. PATIENT: A 16-month old girl with profound hearing loss from bilateral incomplete partition type 1 (IP1) anomaly of the cochlea plus left-sided cochlear nerve aplasia and cochleocele. INTERVENTION: Anomalies were identified with computed tomographic scanning and magnetic resonance imaging during cochlear implant candidacy assessment. While under general anesthesia for right-sided cochlear implantation, the cochleocele was removed and packed with temporalis fascia using transcanal endoscopic ear surgery. The endoscope was held by an assistant while the surgeon packed the round window using a two-handed technique to counter the gush of cerebrospinal fluid. RESULTS: The child made an uneventful recovery with no cerebrospinal fluid leak or meningitis. CONCLUSIONS: Cochleocele can protrude through the round window of a cochlea with incomplete partition type 1 anomaly. An increased risk of meningitis secondary to acute otitis media is expected given the known risk from cochleocele arising through a stapes footplate fistula. Care should be taken to check for the presence of a cochleocele in hearing loss from congenital malformations involving the basal turn of the cochlea on imaging and also at the time of cochlear implant surgery. Repair should be considered at the first opportunity to prevent meningitis. If early cochlear implant surgery is not feasible or appropriate (as, for example, with cochlear nerve aplasia), transcanal endoscopic ear surgery provides good access for a low-morbidity approach.

Combination mTOR and SHP2 inhibitor treatment of lymphatic malformation endothelial cells.

Mammalian target of rapamycin (mTOR) inhibitors are clinically effective at treating some complex lymphatic malformations (LM). The mTOR inhibitor rapamycin blocks the phosphoinositide 3-kinase (PI3K) pathway, which is commonly mutated in this condition. Although rapamycin is effective at controlling symptoms of LM, treatment courses are long, not all LMs respond to treatment, and many patients relapse after treatment has stopped. Concurrent rat sarcoma virus (RAS) pathway abnormalities have been identified in LM, which may limit the effectiveness of rapamycin. Protein tyrosine phosphatase-2 (SHP2) controls the RAS pathway upstream, and SHP2 inhibitors are being investigated for treatment of various tumors. The objective of this study was to determine the impact of SHP2 inhibition in combination with rapamycin on LM growth in vitro. Using primary patient cells isolated from a surgically resected LM, we found that combination treatment with rapamycin and the SHP2 inhibitor SHP099 caused a synergistic reduction in cell growth, migration and lymphangiogenesis. These results suggest that combination treatment targeting the PI3K and RAS signaling pathways may result in effective treatment of LMs of the head and neck.

First Generation Osseointegrated Steady State Implant Benefits in Children With Hearing Loss.

OBJECTIVE: To assess outcomes of a new Osseointegrated Steady State Implant (OSSI) for bone conduction in adolescents. METHOD: In an initial trial, 14 adolescents (14.5 years of age, SD = 2.22) were provided with an OSSI; unilateral OSSI (n = 13), bilateral OSSI in sequential surgeries (n = 1). Outcomes measured were surgical duration, complications, hearing thresholds, speech perception and self-reported hearing benefits using the Speech and Spatial Quality of Hearing Questionnaire. RESULTS: The surgical times were mean 93.6 minutes (SD = 33.3). Surgery was slightly longer in three adolescents who required skin flap reduction (n = 1) or significant bone polishing (n = 2) (121.33 minutes, SD = 8.14). Adverse events occurred in two adolescents post-implant poor external device retention in one child requiring revision flap reduction and inflammation at the incision site due to magnet overuse in another. The "Digital Link Calibration" measure was a good proxy predictor of the strength of magnet required for external device adherence (p = 0.002). The OSSI increased audibility in the implanted ear by mean 31.48 dB HL (SE = 1.58). Aided thresholds were best at 1 kHz (mean 25.33 dB HL, SD = 22.60) and only slightly poorer at 3000 and 4000 Hz (estimate decrease = 8.33 dB HL, SE = 3.54), reflecting good auditory sensitivity even at high frequencies. Speech perception when using the new device alone was good (89.67%, SD = 7.84%) and self-reported hearing by participants and parents improved in all domains assessed by the Speech and Spatial Quality of Hearing Questionnaire (estimate = 1.90 points, SE = 0.25, p < 0.0001). CONCLUSION: The OSSI provides hearing benefits with surgical safety in a carefully selected cohort of adolescents.

Cochlear Implantation in Infants: Why and How.

In children with congenital deafness, cochlear implantation (CI) prior to 12 months of age offers the opportunity to foster more typical auditory development during late infancy and early childhood. Recent studies have found a positive association between early implantation and expressive and receptive language outcomes, with some children able to achieve normal language skills by the time of school entry. Universal newborn hearing screening improved early detection and diagnosis of congenital hearing loss, allowing for earlier intervention, including decision-making regarding cochlear implant (CI) candidacy. It can be more challenging to confirm CI candidacy in infants; therefore, a multidisciplinary approach, including objective audiometric testing, is recommended to not only confirm the diagnosis but also to counsel families regarding expectations and long-term management. Surgeons performing CI surgery in young children should consider both the anesthetic risks of surgery in infancy and the ways in which mastoid anatomy may differ between infants and older children or adults. Multiple studies have found CI surgery in infants can be performed safely and effectively. This article reviews current evidence regarding indications for implantation in children younger than 12 months of age and discusses perioperative considerations and surgical technique.

Cochlear Implantation in Infants: Evidence of Safety.

The aim of this study was to investigate surgical, anesthetic, and device-related complications associated with cochlear implantation (CI) in children younger than 1 year of age. This was a multicenter, retrospective chart review of all children with severe-to-profound sensorineural hearing loss who underwent cochlear implantation with a Cochlear Nucleus Implant System before 1 year of age. Endpoints included perioperative course, major and minor surgical, anesthetic and device-related complications, and 30-day readmission rates. One hundred thirty-six infants (242 ears) met criteria. The mean age at implantation was 9.4 months (standard deviation 1.8). Six-month follow-up was reported in all patients. There were no major anesthetic or device-related complications. Adverse events were reported in 34 of implanted ears (14%; 7 major, 27 minor). Sixteen adverse events occurred ≤30 days of surgery, and 18 occurred >30 days of surgery. The 30-day readmission rate was 1.5%. The rate of adverse events did not correlate with preexisting medical comorbidities or duration under anesthesia. There was no significant difference detected in complication rate for patients younger than 9 months of age versus those 9 to 11 months of age. This study demonstrates the safety of CI surgery in infants and supports reducing the indication for cochlear implantation to younger than 1 year of age for children with bilateral, profound sensorineural hearing loss obtaining a Cochlear Nucleus Implant System.

RNA Sequencing and Pathways Analyses of Middle Ear Epithelia From Patients With Otitis Media.

OBJECTIVES: Otitis media (OM) is the most common pediatric diagnosis in the United States. However, our understanding of the molecular pathogenesis of OM remains relatively poor. Investigation of molecular pathways involved in OM may improve the understanding of this disease process and elucidate novel therapeutic targets. In this study, RNA sequencing (RNA-Seq) was used to discern cellular changes associated with OME compared to healthy middle ear epithelium (MEE). STUDY DESIGN: Ex vivo case-control translational. METHODS: Middle ear epithelia was collected from five pediatric patients diagnosed with OME undergoing tympanostomy tube placement and five otherwise healthy pediatric patients undergoing cochlear implantation. Specimens underwent RNA-Seq and pathways analyses. RESULTS: A total of 1,292 genes exhibited differential expression in MEE from OME patients compared to controls including genes involved in inflammation, immune response to bacterial OM pathogens, mucociliary clearance, regulation of proliferation and transformation, and auditory cell differentiation. Top networks identified in OME were organismal injury and abnormalities, cell morphology, and auditory disease. Top Ingenuity canonical pathways identified were axonal guidance signaling, which contains genes associated with auditory development and disease and nicotine degradation II and III pathways. Associated upstream regulators included ß-estradiol, dexamethasone, and G-protein-coupled estrogen receptor-1 (GPER1), which are associated with otoprotection or inflammation during insult. CONCLUSIONS: RNA-Seq demonstrates differential gene expression in MEE from patients with OME compared to healthy controls with important implications for infection susceptibility, hearing loss, and a role for tobacco exposure in the development and/or severity of OME in pediatric patients. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:2590-2597, 2021.

Exposure to Spoken Communication in Children With Cochlear Implants During the COVID-19 Lockdown.

Importance: The coronavirus disease 2019 (COVID-19) lockdowns in Ontario, Canada in the spring of 2020 created unprecedented changes in the lives of all children, including children with hearing loss. Objective: To quantify how these lockdowns changed the spoken communication environments of children with cochlear implants by comparing the sounds they were exposed to before the Ontario provincial state of emergency in March 2020 and during the resulting closures of schools and nonessential businesses. Design, Setting, and Participants: This experimental cohort study comprised children with hearing loss who used cochlear implants to hear. These children were chosen because (1) their devices monitor and catalog levels and types of sounds during hourly use per day (datalogs), and (2) this group is particularly vulnerable to reduced sound exposure. Children were recruited from the Cochlear Implant Program at a tertiary pediatric hospital in Ontario, Canada. Children whose cochlear implant datalogs were captured between February 1 and March 16, 2020, shortly before lockdown (pre-COVID-19), were identified. Repeated measures were collected in 45 children during initial easing of lockdown restrictions (stages 1-2 of the provincial recovery plan); resulting datalogs encompassed the lockdown period (peri-COVID-19). Main Outcomes and Measures: Hours of sound captured by the Cochlear Nucleus datalogging system (Cochlear Corporation) in 6 categories of input levels (<40, 40-49, 50-59, 60-69, 70-79, ≥80 A-weighted dB sound pressure levels [dBA]) and 6 auditory scene categories (quiet, speech, speech-in-noise, music, noise, and other). Mixed-model regression analyses revealed main effects with post hoc adjustment of confidence intervals using the Satterthwaite method. Results: A total of 45 children (mean [SD] age, 7.7 [5.0] years; 23 girls [51.1%]) participated in this cohort study. Results showed similar daily use of cochlear implants during the pre- and peri-COVID-19 periods (9.80 mean hours pre-COVID-19 and 9.34 mean hours peri-COVID-19). Despite consistent device use, these children experienced significant quieting of input sound levels peri-COVID-19 by 0.49 hour (95% CI, 0.21-0.80 hour) at 60 to 69 dBA and 1.70 hours (95% CI, 1.42-1.99 hours) at 70 to 79 dBA with clear reductions in speech exposure by 0.98 hour (95% CI, 0.49-1.47 hours). This outcome translated into a reduction of speech:quiet from 1.6:1.0 pre-COVID-19 to 0.9:1.0 during lockdowns. The greatest reductions in percentage of daily speech occurred in school-aged children (elementary, 12.32% [95% CI, 7.15%-17.49%]; middle school, 11.76% [95% CI, 5.00%-18.52%]; and high school, 9.60% [95% CI, 3.27%-15.93%]). Increased daily percentage of quiet (7.00% [95% CI, 4.27%-9.74%]) was most prevalent for children who had fewer numbers of people in their household (estimate [SE] = -1.12% [0.50%] per person; Cohen f = 0.31). Conclusions and Relevance: The findings of this cohort study indicate a clear association of COVID-19 lockdowns with a reduction in children's access to spoken communication.

Measurement Properties of the Hearing Environments and Reflection of Quality of Life (HEAR-QL) 28-item Questionnaire in Cholesteatoma.

OBJECTIVE: We sought to assess the reliability and construct validity of the Hearing Environments and Reflection on Quality of Life Adolescent (HEAR-QL 28) quality of life measure (QoL) in cholesteatoma. STUDY DESIGN: Observational. SETTING: Tertiary referral center. PATIENTS: One hundred seventeen patients with a diagnosis or history of cholesteatoma completed HEAR-QL 28. In addition to patients within the age range recommended for HEAR-QL 28 (13-18 yr), patients under 13 years old who were able to complete HEAR-QL 28 without parental assistance were included. INTERVENTION S: Completion of HEAR-QL 28 QoL measure. MAIN OUTCOME MEASURE S: HEAR-QL 28 score, four tone average pure tone audiogram hearing threshold and categorical classification of hearing loss as mild, moderate, and severe. RESULTS: HEAR-QL 28 did not demonstrate discriminative ability on the basis of audiometric threshold, but did discriminate between participants hearing normally (four tone average pure tone audiogram <30 dB HL) (HEAR-QL 86/100) after cholesteatoma surgery from those with unilateral hearing loss (HEAR-QL 73/100) (p < 0.001). Those with unilateral loss could in turn be differentiated from those with bilateral loss (HEAR-QL 60/100) (p < 0.006). CONCLUSION: HEAR-QL 28 is valid measure of QoL in cholesteatoma with no evidence of redundancy and excellent internal consistency. The importance of considering QoL impact of cholesteatoma is highlighted by 17% of participants reporting the normality or abnormality of their hearing differently from their audiometric threshold. The HEAR-QL 28 provides insight into the ability to cope with their hearing environment in a specific environment.

Pediatric laryngoscopy and bronchoscopy during the COVID-19 pandemic: A four-center collaborative protocol to improve safety with perioperative management strategies and creation of a surgical tent with disposable drapes.

Aerosolization procedures during the COVID-19 pandemic place all operating room personnel at risk for exposure. We offer detailed perioperative management strategies and present a specific protocol designed to improve safety during pediatric laryngoscopy and bronchoscopy. Several methods of using disposable drapes for various procedures are described, with the goal of constructing a tent around the patient to decrease widespread contamination of dispersed droplets and generated aerosol. The concepts presented herein are translatable to future situations where aerosol generating procedures increase risk for any pathogenic exposure. This protocol is a collaborative effort based on knowledge gleaned from clinical and simulation experience from Children's Hospital Colorado, Children's Hospital of Philadelphia, The Hospital for Sick Children in Toronto, and Boston Children's Hospital.

International Pediatric Otolaryngology Group (IPOG) Consensus Recommendations: Congenital Cholesteatoma.

OBJECTIVE: To provide recommendations to otolaryngologists and allied physicians for the comprehensive management of children who present with signs and symptoms of congenital cholesteatoma. METHODS: A two-iterative Delphi method questionnaire was used to establish expert recommendations by the members of the International Pediatric Otolaryngology Group, on the preoperative work-up, the perioperative considerations, and follow-up. RESULTS: Twenty-two members completed the survey, in 14 tertiary-care center departments representing 5 countries. The main consensual recommendations were: a precise otoscopic description of the quadrants involved, extensive audiological workup (bilateral tonal, vocal audiometry, and BERA), and a CT scan are required. Facial nerve monitoring and a combination of microscope and telescope are recommended for surgical removal. Clinical and audiological follow-up should be pursued yearly for at least 5 years. First MRI follow-up should be done at 18 months postoperatively if the removal violated the matrix. MRI follow-up duration depends on the initial extent of the cholesteatoma. CONCLUSION: The goal of preoperative and follow-up consensus from International Pediatric Otolaryngology Group participants is to help manage infants and children with congenital cholesteatoma. The operative techniques may vary, and experienced surgeons must perform these procedures.

Ectopic cervical thymus in children: Clinical and radiographic features.

OBJECTIVES: Ectopic thymus is rare and can be a diagnostic challenge. This study evaluated the management of children radiographically diagnosed with ectopic cervical thymus. METHODS: A retrospective review of 100 patients was performed. Data related to clinical presentation, radiological imaging, pathology, and management were collected. Changes in lesion volume were tracked over time. Clinical characteristics were compared based on lesion location in the neck using analysis of variance modelling. RESULTS: There were 115 lesions with radiographic features of ectopic cervical thymus (15 children had bilateral lesions). Diagnosis was based on ultrasound in 98% of patients, magnetic resonance imaging in 18%, and computed tomography in 11%. Mean (SD) follow-up duration was 2 (2.2) years. Forty-four percent (51/115) of lesions involved the thyroid gland, 29% (33/115) were in the central neck but separate from the thyroid, 18% (21/115) had mediastinal extension, and 8% (9/115) involved the submandibular region. Location was unclear for two patients. Submandibular lesions were on average 12.4 cm3 larger (95% CI, 8.2, 16.6) than mediastinal lesions at diagnosis, P ≤ .001. Volume of thymic tissue decreased over time, from a mean (standard deviation [SD]) volume of 4.3 cm3 (9.2) at initial ultrasound to 2.7 cm3 (6.1) at final ultrasound (paired t-test, P = .008). Only two patients required surgery: one for compressive symptoms, and the other to rule out malignancy. CONCLUSION: Ninety-eight percent of children with ectopic cervical thymus were managed conservatively without issues. We propose a classification system based on location to ease communication among clinicians and to help follow these lesions over time. LEVEL OF EVIDENCE: 4, case series Laryngoscope, 130:1577-1582, 2020.

Electrical Vestibular Stimulation in Humans: A Narrative Review.

BACKGROUND: In patients with bilateral vestibulopathy, the regular treatment options, such as medication, surgery, and/or vestibular rehabilitation, do not always suffice. Therefore, the focus in this field of vestibular research shifted to electrical vestibular stimulation (EVS) and the development of a system capable of artificially restoring the vestibular function. Key Message: Currently, three approaches are being investigated: vestibular co-stimulation with a cochlear implant (CI), EVS with a vestibular implant (VI), and galvanic vestibular stimulation (GVS). All three applications show promising results but due to conceptual differences and the experimental state, a consensus on which application is the most ideal for which type of patient is still missing. SUMMARY: Vestibular co-stimulation with a CI is based on "spread of excitation," which is a phenomenon that occurs when the currents from the CI spread to the surrounding structures and stimulate them. It has been shown that CI activation can indeed result in stimulation of the vestibular structures. Therefore, the question was raised whether vestibular co-stimulation can be functionally used in patients with bilateral vestibulopathy. A more direct vestibular stimulation method can be accomplished by implantation and activation of a VI. The concept of the VI is based on the technology and principles of the CI. Different VI prototypes are currently being evaluated regarding feasibility and functionality. So far, all of them were capable of activating different types of vestibular reflexes. A third stimulation method is GVS, which requires the use of surface electrodes instead of an implanted electrode array. However, as the currents are sent through the skull from one mastoid to the other, GVS is rather unspecific. It should be mentioned though, that the reported spread of excitation in both CI and VI use also seems to induce a more unspecific stimulation. Although all three applications of EVS were shown to be effective, it has yet to be defined which option is more desirable based on applicability and efficiency. It is possible and even likely that there is a place for all three approaches, given the diversity of the patient population who serves to gain from such technologies.

Management of Traumatic Injury and Osseointegration Failure in Children With Percutaneous Bone Conduction Implants.

OBJECTIVE: This study examines the incidence and management of traumatic loss or osseointegration failure of percutaneous bone conduction implants in children. STUDY DESIGN: Case series. SETTING: Pediatric tertiary care institution. PATIENTS: Children who underwent percutaneous osseointegrated implant placement from 1996 to 2016. INTERVENTIONS: Clinical evaluation and revision surgery after implant loss. MAIN OUTCOME MEASURES: This study compares the characteristics of children who experienced traumatic loss of implant to those who did not to calculate odds ratios (ORs) describing the risk of injury and investigate device utilization after implant failure. RESULTS: One hundred forty-seven children received percutaneous bone conduction devices; 129 were followed for at least 1 year. Trauma occurred in 19 of 129 cases (15%). Among children with traumatic injury, mean age at initial surgery was 5 years (SD = ±3.3), and 42% had a developmental delay. Among children without traumatic injury, mean age at initial surgery was 6.5 years (SD = ±4.4), and 28% had a developmental delay. Multivariate logistic regression found no significant differences in age, sex, or developmental delay associated with implant loss. In five of 19 traumatic cases (26%), the implant remained in situ due to either skull fracture or abutment loss. In the remaining 14 of 19 cases (74%), there was osseointegration failure with extrusion of the implant. Seventeen children underwent revision surgery utilizing previously placed "sleeper," or backup, osseointegrated implant, and 14 (82%) of these continued to use their device. Two patients with extruded implants did not undergo revision surgery. CONCLUSION: Traumatic injury or osseointegration failure leads to loss of percutaneous bone conduction implants in approximately 15% of children. Revision surgery is often successful.

Extrusion of straight cochlear implant electrodes May be diminished by proximal fixation.

OBJECTIVES: To determine if proximal fixation of the cochlear implant helps reduce the rate of straight electrode array extrusion/migration. METHODS: Records of 278 children implanted with a straight electrode at a tertiary pediatric hospital from 1990 to 2017 were reviewed. Demographics, operative reports, electrode type, radiological studies and audiological assessments including impedance and stimulation parameters were collected. Significant abnormalities were defined by >75% change in impedance relative to 1 month post-implant values. The main outcome is the incidence of electrode extrusion based on significant changes in impedance and comparative radiological imaging looking for electrode extrusion. RESULTS: During this period, straight electrodes were chosen infrequently (298/1781 devices implanted (16.73%) in 278 children). They were used prior to the introduction of pre-curved electrodes (153/298) and subsequently for hearing preservation (33/298) and in anomalous cochleae (48/298). Most recently they were used in a comparative study of children receiving one perimodiolar and one straight array (64/298). All electrodes were proximally fixated with a suture around the incus buttress (Ned's knot). None of the 298 electrodes required repositioning. Comparative radiographs after long duration implant use were available in 72 of 298 devices (24.2%) revealing no changes in electrode position. Significant impedance changes (>75%), indicative of open circuits, were noted in 6/298 devices (2%); ≤2 electrodes at various array positions were affected in each child. In one case, repositioning of the receiver/stimulator may have damaged the electrodes as they exited the device body but no changes in electrode position were evident on the post-repositioning radiograph. CONCLUSIONS: Extrusion of straight electrodes is a recognized complication of cochlear implant surgery but was not observed in our cohort with proximal electrode fixation.

Transmastoid access in branchio-oto-renal syndrome: A reappraisal of computed tomography imaging.

OBJECTIVE: To evaluate for temporal bone abnormalities that might affect transmastoid surgery such as cochlear implantation in cases of branchio-oto-renal syndrome (BOR). STUDY DESIGN: Retrospective review. METHODS: Qualitative assessment of temporal bone computed tomography imaging was performed by a neuroradiologist for 30 individuals with BOR (60 ears) and 20 controls with normal hearing (20 ears). Transmastoid access was assessed categorically across 4 features: tip development, cortex pneumatization, tegmen height, and facial recess pneumatization. The appearance of 4 standard landmarks (Koerner's septum, antrum, prominence of the horizontal semicircular canal, incudal short process) was also dichotomized as normal or abnormal. Data were compared using Fisher's exact testing. RESULTS: Mastoid height differed between the groups with tip underdevelopment noted in 72% of BOR ears vs. 40% of controls (p = 0.02), and a low tegmen was seen in 68% of BOR ears and 25% of controls (p < 0.01). Significant differences in pneumatization were also found for the mastoid cortex (28% non-pneumatized in BOR vs. 5% in controls; p = 0.03) and the facial recess (27% non-pneumatized in BOR vs. 0% in controls; p = 0.01). Standard landmarks were easily identified in all of the control mastoids. In the BOR group, Koerner's septum was abnormally located or absent in 45%, and the antrum was severely hypoplastic or absent in 50%. Similarly, the prominence of the horizontal semicircular canal and the short process of the incus were dysplastic in 73% (44/60) and 62% (37/60), respectively. CONCLUSIONS: Mastoid abnormalities are common in BOR syndrome. Restricted transmastoid access and abnormal or absent mastoid landmarks should be anticipated in those patients with BOR who become cochlear implant candidates. LEVEL OF EVIDENCE: 4.