Objective Selection of diagnostic tests for children with sensorineural hearing loss (SNHL) is influenced by clinical suspicion. Testing results reported in the literature are similarly biased. We evaluate the usefulness of a comprehensive diagnostic battery for each child. Study Design Retrospective review. Setting Tertiary care university hospital. Patients A total of 270 children referred for severe to profound SNHL between January 2002 and June 2009. Interventions Results of the following were reviewed: magnetic resonance imaging, computed tomography, renal ultrasound, electrocardiography, fluorescent treponemal antibody absorption test, connexin 26 sequencing, genetic consultation, and ophthalmologic consultation. Main Outcome Measure Diagnostic yield of each test was determined. Results Each diagnostic test or consultation was completed by at least 95% of patients for whom it was ordered. Magnetic resonance imaging revealed abnormalities explaining SNHL in 24% of patients. Computed tomography showed inner ear anomalies in 18% of patients. Biallelic connexin 26 mutations were found in 15%. Renal ultrasound found anomalies in 4% of patients. Electrocardiography found 1% of patients with prolonged QT intervals. Fluorescent treponemal antibody absorption test result was positive in 0.5%. Genetic consultation found a genetic cause for hearing loss in 25%. Ophthalmologic consultation found abnormalities associated with hearing loss in 8%. Conclusion Diagnostic radiologic imaging is the highest yielding test for evaluating children with SNHL. Connexin 26 sequencing identifies a nearly nonoverlapping subset of children compared with imaging. Specialty consultations, particularly from a clinical geneticist, can improve diagnostic yield. Other tests, although of lower diagnostic yield for SNHL, can identify important diseases that significantly affect patient health.

Objective The benefits of cochlear implantation for children with developmental delays (DD) are often unclear. We compared cognition, adaptive behavior, familial stress, and communication in children with and without DD. Study Design Retrospective review Setting Two tertiary care pediatric hospitals Patients 204 children who underwent cochlear implantation assessed before and >1 year after implantation Main Outcome Measures The Mullen Scales of Early Learning (MSEL), Vineland Adaptive Behavior Scales (VABS), Parental Stress Index (PSI), and Preschool Language Scale (PLS). Results We developed a specific definition of DD for hearing-impaired children based upon DSM-IV criteria for mental retardation; 60 children met the criteria for DD and 144 children did not. Prior to implantation, multiple linear regression demonstrated that children with DD had lower scores in every domain of the MSEL and VABS (p<0.05) but no differences in any domains of the PSI and PLS (p>0.1) compared to children without DD. After implantation, children without DD demonstrated significant improvements in intelligence as measured by the MSEL, age-appropriate improvements in adaptive behavior as evaluated by the VABS, and their familial stress levels were not increased after cochlear implantation. In contrast, children with DD underwent implantation at a later age and demonstrated less comprehensive developmental improvements after cochlear implantation and higher stress levels. However, when the age differences were taken into account using multiple linear regression analyses, the differences between two cohorts were reduced. Conclusions These data indicate that our definition of DD is a reliable method of stratifying deaf children. While children with DD have a normal developmental rate of adaptive behavior after cochlear implantation, their developmental rate of intelligence is lower and they have higher stress levels than children without DD. However, our data suggest that if children with DD could be implanted as early as children without DD, their intelligence and stress outcomes would be improved.

Objectives/Hypothesis To examine the characteristics of pediatric cochlear implant channel malfunction preceding device failure. Study Design Retrospective review. Methods All pediatric patients who underwent cochlear implantation at a tertiary academic medical center were reviewed regarding device type, reason for replacement, time to replacement, and timing and pattern of channel faults in failed versus nonfailed devices. Results Between 1993 and 2008, 264 pediatric cochlear implantations were performed. With an average 894-day follow-up, the replacement rate was 9.5% (25/264). Reasons for replacement were device failure (6.4%), medical/surgical failure (2.3%), and obsolescence (0.8%). Replacement rates were comparable among Advanced Bionics (13.3%), Cochlear Corporation (6.3%), and MED-EL (10.3%) devices. Fifty-two cochlear implants developed at least one channel fault, and 13 eventually progressed to failure requiring replacement. MED-EL devices comprised 12 of these 13 failures. At the 12-month follow-up interval, one, three, and five channel faults predicted 40%, 75%, and 100% probabilities of eventual electrode failure, respectively. Channels destined to fail demonstrated small, yet statistically significant, impedance elevations 12 months before failure and large elevations 3 months before failure. Conclusions Replacement of cochlear implants in pediatric patients is common and is due to device malfunction about one half of the time. Earlier initial channel fault, earlier subsequent channel faults, adjacent channel faults, and a greater total number of channel faults were associated with the need for replacement surgery. Elevations in a channel’s impedance should raise the concern for an impending failure. These predictors can help the cochlear implant team when considering surgery to replace the device.

The objective of this study was to determine if intra-operative auditory monitoring is feasible during cochlear implantation and whether this can be used as feedback to the surgeon to improve the preservation of residual hearing. This prospective non-randomised study was set in a paediatric tertiary referral hospital. Thirty eight consecutive paediatric patients undergoing cochlear implantation who had measurable auditory thresholds pre-operatively were divided into two cohorts. The unmonitored cohort included the fi rst 22 patients and the monitored cohort included the last 16 patients. The main outcome measure(s) were pre-operative, intra-operative and more than one month post-operative average auditory thresholds at 500, 1000 and 2000 Hz measured using auditory steady-state response audiometry. The average pre-operative thresholds were 103.5 dB HL and 99.7 dB HL in the unmonitored and monitored cohorts, respectively. These were not statistically different (p > 0.3). In the monitored cohort, we measured auditory thresholds to assess cochlear function at multiple time points during the operation. Compared to baseline, thresholds were increased 0.7 dB after drilling the mastoidectomy and well, 0.2 dB after opening the cochlea and 4.6 dB after inserting the electrode array. One month post-operatively, the average thresholds were 114.0 dB HL in the unmoni-tored cohort but only 98.8 dB HL in the monitored cohort (p < 0.001). Both the use of intra-operative auditory monitoring and higher pre-operative thresholds were associated with improved preservation of residual hearing (p < = 0.001). Intra-operative auditory monitoring is a viable tool that can provide real-time feedback to the surgeon during cochlear implant surgery. These data suggest that this can lead the surgeon to modify his or her surgical technique in ways that can improve the rate of long-term hearing preservation.