Failure rate in pediatric cochlear implantation and hearing results following revision surgery.

INTRODUCTION: In a discussion of the risks and benefits of pediatric cochlear implantation, device failure and the need for revision surgery is often overlooked. The failure rate has not been investigated extensively for this population of patients. Hearing results are under-reported following revision surgery as well. We will review our experience with cochlear implant failure, revision, and hearing results when available to better guide the preoperative counseling of families considering cochlear implantation. METHODS: Retrospective chart review of all children undergoing cochlear implantation from 2004 to 2014. RESULTS: In this review of 579 cases of pediatric cochlear implantation, a 4.7% device failure rate was identified. Additionally, there was a 0.3% device infection rate, as well as a 0.3% electrode extrusion rate. 10 patients had audiometric data prior to and following revision surgery. These data demonstrate similar pre-failure and post revision surgery pure tone average. CONCLUSION: Cochlear implant device failure is the most common long-term complication of surgery; fortunately, hearing outcomes following revision surgery, evaluated with pure tone average, revealed no decline in auditory performance.

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.

Otologic characteristics of individuals with deletions of distal 18q.

OBJECTIVES/HYPOTHESIS: To fully describe the otologic features seen in individuals with deletions of the distal long arm of chromosome 18 (distal 18q-). STUDY DESIGN: Cross-sectional/observational. METHODS: More than 200 individuals with deletions of the long arm of chromosome 18 underwent a complete otologic and audiologic examination. In addition, chromosome microarray analysis to determine the chromosome copy number was completed for all participants. Overall, 113 subjects had clinical and audiological data to be reported here. RESULTS: Sixty-six percent of this population had aural stenosis or atresia. No subject had microtia. In the 53 individuals for whom serial data was available, enlargement of ear canal diameter was seen in 48% of ears with stenosis, examined over time. Abnormalities of the palate were seen in nearly 18% of patients and included complete or incomplete clefts of the palate, submucous clefts, and velopharyngeal insufficiency. A conductive hearing impairment was identified in 112 ears (49.5%), and sensorineural hearing loss was identified in 28%. Eustachian tube dysfunction was common and found in 78% of ears examined. CONCLUSIONS: The otologic phenotype varies widely among individuals with distal 18q-. External auditory canal stenosis without microtia is a hallmark of the disease. Hearing impairment is also very common, with both sensorineural losses and conductive losses contributing to morbidity. Moreover, the critical region for sensorineural hearing loss will aid in the identification of the gene responsible for this aspect of the distal 18q- phenotype. LEVEL OF EVIDENCE: 4.

Sensorineural hearing loss in people with deletions of 18q: hearing in 18q-.

OBJECTIVE: The objective of this study was to characterize hearing loss in individuals with deletions of distal chromsome18q and to identify the smallest region of overlap of their deletions, thereby identifying potential causative genes. STUDY DESIGN: The clinical data were collected via a retrospective case study. Molecular data were obtained via high-resolution chromosome microarray analysis. SETTING: The study was conducted as a component of the ongoing research protocols at the Chromosome 18 Clinical Research Center at the University of Texas Health Science Center at San Antonio. PATIENTS: Thirty-eight participants with a deletion of the distal portion of the long arm of chromosome 18 were recruited to this study. INTERVENTIONS: The participants underwent an otologic examination as well as a basic audiometry evaluation. Blood samples were obtained, and high-resolution chromosome microarray analysis was performed. MAIN OUTCOMES MEASURES: Pure tone averages and speech discrimination scores were determined for each participant. The region of hemizygosity for each participant was determined to within 2 Kb each of their breakpoints. RESULTS: Twenty-four participants (63%) had high-frequency hearing loss, similar to the pattern seen in presbycusis. Comparison of microarray results allowed identification of eight genes, including the candidate gene for dysmyelination (MBP). CONCLUSION: Individuals with a deletion of a 2.8 Mb region of 18q23 have a high probability (83%) of high-frequency sensorineural hearing loss.

Complication rate of minimally invasive cochlear implantation.

OBJECTIVES: To assess the complication rate of minimally invasive cochlear implantation (MICI). STUDY DESIGN AND SETTING: Data for this study were obtained via a retrospective analysis of records at the Ear Medical Group, San Antonio, TX, after IRB approval at the University of Texas Health Science Center at San Antonio. The surgical complications of MICI were recorded in a spreadsheet format; 176 patients were included in the study. RESULTS: A total of 22 (12.5%) complications were noted in the study. There were 0 life-threatening, 7 major, and 15 minor complications. Of the 7 major complications, 3 were device failures, 2 developed delayed mastoiditis, 1 required receiver/stimulator repositioning, and 1 involved facial paralysis. CONCLUSIONS: MICI is as safe as standard cochlear implantation (SCI) and affords with it other benefits. Eliminating the scalp flap avoids devascularization and minimizes the opportunity of flap infection or necrosis. Complications not related to the flap are similar to SCI. EBM RATING: C-4.