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Cerebrospinal fluid (CSF) leak is the most frequently reported significant complication in pediatric ABI surgery, occurring in up to 8.5% of patients. Surgical protocols frequently call for a watertight dural closure followed by use of a mastoid/temporal pressure dressing to minimize the risk of CSF fistula and fluid collections. Evidence from cerebellopontine angle surgery confirms that intracranial CSF pressure rises immediately after posterior fossa surgery and normalizes after 48 hours, with impaired absorption the believed cause. This can result in CSF egress around the tract of the ABI electrode cable into the subgaleal space. Complications of pseudomeningocele include skin wound breakdown, meningitis, and need for further surgery. Multiple case reports describe revision surgery on pediatric ABI patients to place fat and other tissue grafts and plating systems to address these issues. Pressure dressing use is also problematic and may provide a false sense of assurance to the operating surgeon. Surgeons have been shown to be unable to accurately assess pressure to prevent magnet migration with dressings during MRI. It is therefore difficult to support the routine use of tight pressure dressings over an implanted device in a thin-scalped child when this could result in a pressure ulcer and devastating wound complication. At our pediatric ABI program, we have begun to perform lumbar subarachnoid drainage routinely for 48 hours postoperatively after retrosigmoid (RS) ABI placement in children as we routinely do in adults having acoustic neuroma surgery. We use dressings for only 24 hours to provide initial counterpressure to prevent pseudomeningocele as the lumbar drain is begun. There have been no complications related to lumbar drain use, and at our pediatric hospital lumbar drains are managed on the regular ward. We believe the combination of lumbar drainage and short pressure dressing use allows for the best risk-benefit profile in these patients. [ABSTRACT FROM AUTHOR]

Objective: To determine the safety and feasibility of the auditory brainstem implant (ABI) in congenitally deaf children with cochlear aplasia and/or cochlear nerve deficiency. Study Design: National Institutes of Health-Funded Phase I feasibility clinical trial of surgery in 10 children, ages 2 to 5 years, over a 3-year period. Intervention(s): ABI implantation and postsurgical programming. Main Outcome Measure(s): The primary outcome measure is the number and type of adverse events during ABI surgery and postsurgical follow-up, including behavioral mapping of the device. The secondary outcome measure is access to and early integration of sound. Results: To date, 6 children have successfully undergone ABI surgery and postoperative behavioral programming. Three of the implanted children have been followed up for 36 months, 1 for 24 months and another for 12 months, and one has just been activated. Expected adverse events have been documented in three of the six children who received the ABI. One child experienced a cerebral spinal fluid leak, which resolved with lumbar drainage. One child demonstrated vestibular side effects during device programming, which resolved by deactivating one electrode. One child experienced postoperative vomiting resulting in an abdominal radiograph. The first five children all have speech detection threshold of 40dB or less, with 4 at 25dB or less. Scores on the IT-MAIS/MAIS range from 10 to 32 (out of a total of 40), and the children are demonstrating some ability to discriminate between closed-set words that differ by number of syllables (pattern perception), with one child able to discriminate with open-set ability with auditory stimulation only. Conclusion: ABI surgery and device activation seem to be safe and feasible in this preliminary cohort. Speech detection, particularly pattern perception and word discrimination, develops slowly and long-term followup and rehabilitation is necessary with an experienced team. [ABSTRACT FROM AUTHOR]

Objective: This study sought to determine which variables contribute to attainment of age-appropriate phoneme production skills in pediatric cochlear implant (CI) users between 4 and 8 years post-CI. Methods: Participants include 161 children with CIs enrolled in a longitudinal, multi-center project, the Childhood Development after Cochlear Implantation study. All received a CI by 5 years of age (M=2.4 years). Children completed the Goldman-Fristoe Test of Articulation (GFTA) between 4 and 8 years post-CI. Participants were grouped by attainment of age-appropriate production skills (GFTA<85: Below normal range; GFTA≥85: Within normal range) at 4 and 8 years post-CI. Error scores were computed for each child. Data were analyzed using a mixed-effects linear model utilizing all available GFTA data 4-8 years post-CI. Results: One-half of participants had normal phoneme production at 4 years post-CI. Number of errors across participant groups decreased from 21.3 at 4 years post- CI to 10.2 at 8 years post-CI. Presence of residual hearing at baseline (p<.01) and better language scores at baseline (p<.05) and 36 months post-CI (p<.0001) significantly related to better GFTA scores over the 4-8 year followup period. Children who scored <85 on GFTA at both 4 and 8 years post-CI had significantly less residual hearing, lower speech perception scores, and poorer language outcomes at baseline and 3 years post-CI relative to other participant groups. Conclusions: Pediatric CI users who attain age-appropriate speech production scores at 4 and 8 years post-CI likely derived benefit from hearing aids pre-CI because of useable residual hearing, with a subsequent language advantage from the outset. Children with CI without usable residual hearing or adequate language skills by 3 years post-CI have greater risk of persistently delayed speech production skills after implantation, and may require more therapeutic intervention and support to maximize communication outcomes. [ABSTRACT FROM AUTHOR]

Objectives: Most hearing parents of children born with severe to profound SNHL wish their children to acquire spoken language. A key question parents face after the HL diagnosis is to identify a habilitation program that will facilitate subsequent language development while contemplating treatment options such as CI. We studied this question using longitudinal data from a multi-center study. Methods: CI candidates (mean age 26m) were classified into 5 subgroups based on parent-reported communication modes in habilitation settings before CI: 1:American Sign Language (ASL) only (N=42); 2:auditory-verbal, auditory- oral, or cued speech only (ORAL, N=63); 3:concurrent ORAL and ASL (N=47); 4:ORAL and non-ASL signs or total communication (N=19); and 5:no reported habilitation or use of gestures only (N=16). Spoken language developments were tracked via the Reynell Developmental Language Scales from baseline to the 3rd post- CI annual follow-up, and the Comprehensive Assessment of Spoken Language annually thereafter. Core composite SS based on age appropriate core tests through the 8th year follow-up were analyzed using mixed-effects regression accounting for age at CI, family income, and cognitive screening, speech perception, and maternal sensitivity scores. Pragmatic language SS between 4- and 8 years post CI were analyzed similarly. Results : There was significantly different longitudinal trajectories in overall spoken language development between groups (p=0.02). Group 2 gained 1.2 points/year faster (p=0.0037) and group 4 gained 1.5 points/year faster (p=0.0105) in core composite SS over 8 years, while groups 3 and 5 were not statistically different, when compared to the group 1 (ASL only). Although similar between-group patterns were seen in pragmatic language SS 4-8 years post CI, the differences were not statistically significant. Conclusion: Parental report of enrollment into an ASL only habilitation setting before CI was associated with a slower rate of overall spoken language development post CI in comparison to other communication settings. [ABSTRACT FROM AUTHOR]