Utility of Intraoperative Radiographs in Pediatric Cochlear Implant Surgery.

OBJECTIVE: To evaluate the role of intraoperative radiographs to confirm electrode position following pediatric cochlear implantation (CI). STUDY DESIGN: Retrospective chart review. SETTING: Single tertiary care pediatric center. METHODS: A retrospective chart review was conducted, including all pediatric patients undergoing CI at UPMC Children's Hospital of Pittsburgh over a 13-year period. RESULTS: We identified 326 patients undergoing 492 procedures. Across the cohort, there were 7 cases that required intraoperative electrode reinsertion due to malposition or presumed malposition. For 6 of the 7 cases, intraoperative X-ray identified electrode malposition. Neural response telemetry (NRT) testing was also abnormal for 4 of these cases prior to reinsertion. Implantation of Cochlear's Slim Modiolar electrode was associated with an abnormal perioperative X-ray (odds ratio [OR]: 9.2, p = 0.03) and increased change in management (OR: 9.2, p = 0.03) compared to Cochlear's Contour Advance (CA). Incidence of abnormal X-rays was 1.24% overall, 4% in the Slim Modiolar group, and 0.3% in the CA group. The Slim Modiolar electrode accounted for 4 of 7 cases requiring reinsertion, and in all 4 of these cases, electrode fold-over was identified on the X-ray. NRT was normal in 1 of these 4 cases. CONCLUSION: The use of Cochlear's Slim Modiolar electrode was associated with a significantly increased risk of abnormal intraoperative X-ray compared to the CA electrode. Given the risk of fold-over with routine insertion and normal electrical testing using the Slim Modiolar electrode, we recommend routine use of intraoperative skull X-ray to confirm electrode position.

Cochlear implant indications: a review of third-party payers' policies for standard and expanded indications.

As cochlear implant (CI) candidacy has expanded, commercial payers in the United States have varied in their adoption of new indications, potentially confusing providers' knowledge about appropriate patients for referral. We reviewed how third-party payers classify the medical necessity of cochlear implants for a variety of indications across the lifespan.We compared policies of the six largest commercial payers in our region, focusing on clinical scenarios for which many centers experience difficulty obtaining pre-authorization. These include: (1) CI in children under 12 months, (2) audiometric and speech perception criteria in children, (3) sequential bilateral CI, (4) electro-acoustic stimulation, (5) impending cochlear ossification, and (6) single-sided deafness (SSD).Of the more notable findings for the clinical scenarios half of commercial payers have a pediatric age requirement of greater than 12 months. Generally, audiologic and speech perception criteria are more stringent for children than adults across all policies. SSD is considered investigational by most policies.Third-party payers employ variable criteria regarding the medical necessity of CI, many of which are not contemporaneous with clinical knowledge and best practices. This may impact referral patterns among audiologists. More methodologically rigorous clinical trials may help shift such restrictive policies to benefit a greater number of patients.

Adverse events in pediatric cochlear implant patients undergoing magnetic resonance imaging.

OBJECTIVE: To investigate the prevalence and nature of adverse events in magnetic resonance imaging (MRI) of pediatric cochlear implant (CI) patients. METHODS: Retrospective chart review at a tertiary pediatric hospital. CI patients who underwent MRI from 2004 through 2019 were identified via our internal radiology database. Comorbidities, CI model, age at MRI, number of MRIs, type of MRIs, indication for MRIs, precautions taken for MRIs, quality of MRIs, anesthesia during MRIs, patient language abilities, and adverse events were recorded from the electronic medical record. The literature was reviewed, and our results were compared to those of previous similar series. RESULTS: From 2004 to 2019, 12 pediatric patients (17 ears) with CIs underwent 22 MRIs. 12 MRIs were performed in CI patients with retained internal magnet. 4/22 MRIs resulted in morbidity; 2 patients experienced pain requiring MRI abortion, 1 experienced magnet rotation requiring surgical replacement, and 1 underwent operative removal of the magnet prior to the scan with surgical replacement thereafter. 19/22 MRIs were performed to evaluate the brain; 17/22 of the radiologic reports noted limitation of evaluation due to artifact. 18/22 MRIs required the administration of anesthesia. 9 of the 22 MRI events involved 2 patients whose CIs had been without internal magnet in anticipation of future MRI requirement. CONCLUSIONS: Adverse events affecting pediatric patients with CI can occur as a result of MRI, despite appropriate precautions. Safety requires consideration of factors unique to a pediatric hearing-impaired population. Clinicians must remain informed on best practices and manufacturer recommendations.

A Study to Determine Various Factors Influencing Auditory Outcomes in Paediatric Cochlear Implantation.

To evaluate various factors that affects the auditory outcomes in pediatric patients with bilateral profound sensorineural hearing loss who underwent unilateral cochlear implantation. 50 prelingually deaf pediatric patients aged between 1 and 6 years with bilateral profound sensorineural hearing loss who underwent unilateral cochlear implantation between January 2016 and June 2018 at our tertiary centre were included in this study. Auditory performance was measured using revised categories of auditory performance score at 1 year post cochlear implantation. A Statistical analysis of several factors was performed to reveal any significant relation with outcomes of cochlear implantation. The results of the study showed that patients with younger age at implantation, patient who used hearing aid prior to surgery and patients with normal cochlear morphology had better auditory outcomes post cochlear implantation than their counter group and are the factors which have positive effect on the cochlear implantation outcomes, whereas factors which did not showed any significant relation with the auditory outcomes of cochlear implantation are gender, consanguinity and birth hypoxia. Age at implantation, use of hearing aid prior to surgery and cochlear morphology are the factors with direct impact on the outcomes of cochlear implant.

Music perception and training for pediatric cochlear implant users.

INTRODUCTION: Cochlear implants (CIs) are biomedical devices that restore sound perception for people with severe-to-profound sensorineural hearing loss. Most postlingually deafened CI users are able to achieve excellent speech recognition in quiet environments. However, current CI sound processors remain limited in their ability to deliver fine spectrotemporal information, making it difficult for CI users to perceive complex sounds. Limited access to complex acoustic cues such as music, environmental sounds, lexical tones, and voice emotion may have significant ramifications on quality of life, social development, and community interactions. AREAS COVERED: The purpose of this review article is to summarize the literature on CIs and music perception, with an emphasis on music training in pediatric CI recipients. The findings have implications on our understanding of noninvasive, accessible methods for improving auditory processing and may help advance our ability to improve sound quality and performance for implantees. EXPERT OPINION: Music training, particularly in the pediatric population, may be able to continue to enhance auditory processing even after performance plateaus. The effects of these training programs appear generalizable to non-trained musical tasks, speech prosody and, emotion perception. Future studies should employ rigorous control groups involving a non-musical acoustic intervention, standardized auditory stimuli, and the provision of feedback.

Simultaneous bilateral cochlear implantation in children aged 12‒18 months is safe and can be performed using standard cochlear implant surgical techniques.

PURPOSE: Simultaneous bilateral cochlear implantation is often seen as the gold-standard intervention for many people with bilateral deafness. However, this intervention is not as commonly performed in China. This paper presents the surgical technique and the peri- and postoperative safety results from simultaneous bilateral implantation and compares them with those from sequential bilateral implantation. METHODS: Twenty nine children (aged 12‒18 months) met the inclusion/exclusion criteria and took part in this study. 10 participants received a unilateral cochlear implantation; 19 participants received simultaneous bilateral cochlear implantation. The same standard surgical technique (transmastoid facial recess approach with round window insertion) was used in all implantations. Safety was assessed via monitoring peri- and postoperative adverse events. The helpfulness of select surgical tools was assessed via questionnaire. RESULTS: No adverse events were reported for any participants. All queried surgical tools were helpful in all 244 recorded responses. CONCLUSION: Simultaneous bilateral cochlear implantation can be performed using the same surgical technique as unilateral implantation and poses no increased safety risk for children aged 1‒2 years.

High Electrode Impedance Values in Pediatric Cochlear Implant Recipients May Imply Insufficient Auditory and Language Skills Development.

BACKGROUND: Measurements of electrode impedance values are routinely performed after cochlear implantation. The primary objective of the study was to determine if pediatric, prelingually deafened patients with different postoperative performances showed significantly different impedance values one year after implantation. METHODS: This study comprised 42 pediatric cochlear implant recipients provided with the device in a single academic tertiary referral center between 1 January 2000, and 31 December 2016. Medical chart analysis was performed in order to assess evolution of impedance values during the first postoperative year on a monthly basis. Electrode impedance values measurements one year postoperatively were compared between children with successful and unsuccessful auditory and language skills development assessed using the EARS protocol (a name of a performance test). Furthermore, values were compared among recipients of different implant types and among different cochlear segments. RESULTS: A gradual rise of average impedance values was found during the first months of implant use (1st month, 7.32 kΩ; 3rd month, 7.86 kΩ) with the peak at the 4th postoperative month (7.96 kΩ), followed by a gradual decrease towards the 12th month (6th month, 7.62 kΩ; 12th month, 6.86 kΩ). Lower values at the 12th postoperative month were observed in recipients with successful development compared to patients presented with unsuccessful development (6.22 kΩ vs. 7.82 kΩ; p = 0.001). Mean impedance values were different when compared among cochlear segments and among different implant types. CONCLUSION: High electrode impedance values one year after implantation in pediatric patients may imply insufficient auditory and language skills development. Further studies are needed in order to validate our results.

Hipoacusia neurosensorial no sindrómica: caracterización molecular, desempeño auditivo y tratamiento en pacientes con mutaciones en las conexinas 26 y 30 / Non-syndromic sensorineural hearing loss: molecular characterization, auditory performance, and treatment in patients with connexin 26 and 30 mutations

La hipoacusia congénita o de aparición temprana es un trastorno sensorial muy frecuente en niños. Las causas son diversas, pueden intervenir factores genéticos y/o ambientales. El 80% de la sordera hereditaria es no sindrómica y de herencia autosómica recesiva. Hasta un 50% de estos casos se deben a mutaciones en el locus DFNB1 donde están localizados los genes GJB2 y GJB6, que codifican las conexinas 26 y 30, dos proteínas que se expresan predominantemente en la cóclea. Se han reportado más de 100 mutaciones en el gen GJB2, con una mutación muy frecuente, 35delG, que representa hasta un 85% de los alelos mutados. Una deleción en el gen GJB6, (delGJB6-D13S1830), surge como la segunda mutación más frecuente. La hipoacusia debida a mutaciones en estos genes es de inicio prelocutivo, con un grado de severidad que varía de moderado a profundo, existiendo casos leves en menor proporción, con variaciones inter e intrafamiliares. Es generalmente estable, bilateral, y afecta a todas las frecuencias. El conocimiento de las causas genéticas de la hipoacusia ha permitido contar con nuevas herramientas para el diagnóstico, y como consecuencia, se ha optimizado el asesoramiento genético y facilitado el diagnóstico precoz de los pacientes, incluso en el período prenatal. La detección precoz tiene un impacto inmediato en la implementación de terapias que permiten una estimulación auditiva temprana. En esta revisión se describe el papel de las conexinas en la fisiología auditiva, así como también las características moleculares y audiológicas y el desempeño auditivo con audífonos e implante coclear en pacientes que presentan mutaciones en las conexinas 26 y 30.

Congenital or early appearing hearing loss is a very common sensory disorder in children. The causes for the disorder are diverse and genetic as well as environmental factors may be involved. Overall, 80% of the hereditary deafness is non-syndromic and of autosomal recessive inheritance. Up to 50% of the cases are associated with mutations in the DFNB1 locus that contains the GJB2 and the GJB6 genes encoding connexins 26 and 30, two proteins that are predominantly expressed in the cochlea. More than 100 mutations of the GJB2 have been reported. The 35delG is a common mutation accounting for up to 85% of the mutated alleles. A deletion in the GJB6 gene, (delGJB6-D13S1830), is the second most frequent mutation found. Hearing loss due to mutations in these genes has an onset before speech develops and degree of severity varies from moderate to severe, with a lower incidence of mild cases and inter- and intrafamily variations. The condition is usually stable, bilateral, and affecting all frequencies. Increased knowledge on the genetic causes of hearing loss has allowed for the development of new diagnostic tools and consequently, improvement of genetic counseling and early, even prenatal, diagnosis. Early detection has an immediate impact with implementation of early auditory stimulation therapies. In this review the role of connexins in auditory physiology described, as well as molecular and audiological features and auditory performance with hearing aids and cochlear implants in patients with connexins 26 and 30 mutations.

Cochlear implantation in children with anomalous cochleovestibular anatomy.

OBJECTIVE: The aim of the study is to assess the audiological and surgical outcome after cochlear implantation in children with inner ear malformation and to compare them with a group of congenitally deaf children implantees without inner ear malformation. INTRODUCTION: Children with profound sensorineural deafness with malformations of the inner ear represent a challenge, accounting to 5-15% of congenital sensorineural deafness. These cases were originally regarded as a contraindication for cochlear implant surgery. METHODS: Retrospective study of 26 patients with congenital inner ear malformation, from a total population of 329 cochlear implant patients. Radiological evaluation with high resolution computed tomography and magnet resonance was performed to all patients in order to evaluate all the preoperative conditions. All patients were tested using European Portuguese word discrimination tests (monosyllabic test, number test and sentences test), capacity of auditory performance (CAP) and speech intelligibility rating (SIR). RESULTS: In all 7.9% of deaf children in our center study have inner ear abnormalities. All children underwent successful implantation. CAP yielded an average 7.1 (+/-1.7), SIR 4.3 (+/-1.0). The children without inner ear abnormalities did not achieve statistically significant better scores. Two children had a perilymph gusher, and there were no other complications. CONCLUSION: Cochlear implantation can be successfully performed in children with inner ear malformation. Audiological performance after cochlear implantation in malformed inner ears is comparable to that found in other congenitally deaf patients. The risk of CSF leak is associated with inner ear abnormalities and should be anticipated during surgery.

Consideration for routine outpatient pediatric cochlear implantation: A retrospective chart review of immediate post-operative complications.

INTRODUCTION: Cochlear implantation is well accepted as the treatment of choice for prelingual deafness in children [1]. However, the safety of routinely performing this procedure on an outpatient basis is debated. We aim to assess immediate postoperative complications that would affect a surgeon's decision to perform pediatric cochlear implantation on an outpatient basis. METHODS: A retrospective chart review was conducted which included all children 17 years old or younger who underwent cochlear implantation from 2004 to 2014 in a private neurotology practice. The immediate postoperative complication rates and types of complications were then examined. RESULTS: A total of 579 cochlear implants were placed in children ages 1-17 years old from 2004 to 2014. The most common complications were nausea/vomiting and dizziness/imbalance. The odds ratio of developing complications in the group ages 1-3 years old versus all other age patients was found to be statistically insignificant (OR 0.90, 95% CI 0.61 to 1.32, p = 0.58). The odds ratio of developing a complication after bilateral implantation compared to unilateral implantation was statistically significant (OR 1.96, 95% CI 1.18 to 3.28, p = 0.01). There was no difference in complication rates when comparing lateral wall and perimodiolar insertions. A total of 6 of 579 (1%) cochlear implants resulted in a complication requiring unplanned medical attention. CONCLUSIONS: Overall, this series offers a decade of experience in pediatric cochlear implantation that shows a low incidence of the need for unplanned medical attention in the immediate postoperative period. The most common complication seen is Post-operative nausea and vomiting (PONV) that appears to be amenable to outpatient management even in the youngest populations. This supports providers routinely performing pediatric cochlear implantation on an outpatient basis.