Jill Chinnici

Effects of Cytomegalovirus (CMV) Related Deafness on Pediatric Cochlear Implant Outcomes

OBJECTIVE: Human cytomegalovirus (CMV) is a commonly recognized viral cause of perinatal sensorineural hearing loss. CMV-infected infants are also at risk for developmental neurological deficits. This retrospective study assesses the impact of CMV-induced deafness on pediatric cochlear implant outcomes. STUDY DESIGN AND SETTING: Thirteen patients from the Johns Hopkins pediatric cochlear implant database were identified with CMV-related deafness. A retrospective review of the medical records of the Johns Hopkins Hospital was performed. RESULTS: The mean age at implantation was 5.6 years. Follow-up audiometric data ranged from 6 to 48 months postoperatively. Mean speech perception scores were 4.5 (out of 6) following implantation. CONCLUSION: We have shown that cochlear implants can provide useful speech comprehension to patients with CMV-related deafness. Speech recognition scores were within the range established by our overall pediatric implant population. SIGNIFICANCE: This observation underscores the importance of a multidisciplinary rehabilitation program following implantation in these patients at risk for cognitive delay. EBM RATING: C

Revision cochlear implantation surgery in adults: indications and results.

Objective: To assess the efficacy, risks, and indications of revision cochlear implantation (RCI) and to identify the clinical, audiologic, and device-related characteristics that predict outcome. Study Design: Retrospective case series. Setting: Academic tertiary referral center. Patients: Adults (≥18 yr) who underwent RCI at Johns Hopkins University. Intervention: Revision cochlear implant surgery. Main Outcome Measures: Speech perception by open-set testing and patient report and patient report of symptom resolution. Results: During the 16-year period of this study, 4.8% of all adults implanted at our center have required 1 or more RCI surgeries. A total of 48 RCIs have been performed. The indications for RCI included infection (12%), electrode extrusion (15%), hard failure (23%), suspected device failure (42%), and isolated facial nerve stimulation (8%). Overall, successful resolution of the implant-related or medical condition was achieved with RCI in 83% of cases. Speech perception was lower in only 1 (2.1%) of 48 cases. Satisfactory preoperative speech recognition was preserved or surpassed in 5 of 6 infection cases and 8 cases with progressive symptoms of tinnitus and facial nerve stimulation. All cases of hard failure regained or surpassed previous peak performance. Improved speech recognition was experienced by 75% of cases with suspected device failure. Of cases in which RCI failed to restore previous functional benefit, there was a significant association with advanced age (>70 yr). Whereas an abnormal integrity test was predictive of favorable outcome after RCI, a negative test was not predictive of outcome. A similar pattern of results was observed with respect to ex vivo device analysis. Conclusion: Revision cochlear implantation can be safely performed to restore lost benefit in appropriately selected cases. When properly performed after medical and audiologic options have been exhausted, RCI rarely compromises previous function and, in most cases, can resolve functional complaints and distracting symptoms. When positive, integrity testing is a useful screen for the presence of a device defect. In cases in which device integrity is uncertain, clinical judgment guided by longitudinal assessment can help determine whether RCI is likely to be beneficial.

Revision cochlear implant surgery in children: The johns hopkins experience

Objective: To assess the efficacy of revision cochlear implantation (RCI) and to identify clinical and device-related factors predictive of RCI outcome. Study Design: Retrospective case series with attempted correlation to ex vivo device analysis. Setting: Academic tertiary referral center. Patients: Children (<18 yr) who underwent RCI in a tertiary-care center. Intervention: Revision cochlear implantation with multichannel cochlear implant. Main Outcome Measures: Open-set speech perception testing and subjective report by child, family, teacher, or therapist; patient report of symptom resolution. Results: During the 15-year period, 13% of pediatric CI surgeries were revised (at an average of 3.4 yr after initial operation). Hard failures (42%), suspected device failure (29%), and extracochlear electrodes (16%) were the most common indications for RCI. Most hard and suspected device failure cases (n = 37; 84%) reported a return to previous peak performance within 18 months of RCI. Previous peak performance was more likely to be achieved or exceeded in younger than in older children. A decline in speech perception was a stronger predictor of successful outcome (100%) than chronic underperformance (57%) among children with suspected device failure. Preoperative integrity testing was only moderately predictive of functional results or findings at ex vivo device analysis. Conclusion: Revision cochlear implantation should be considered when longitudinal clinical assessment reveals an unfavorable trajectory in the development of receptive communication skills. Device testing provides important, but not definitive, supporting data. Revision cochlear implantation as guided by device and patient factors can exert a positive impact on access to verbal language and therefore its development. A delay to act can have lasting negative consequences because benefits seem to diminish with age.

Improvements in visual attention in deaf infants and toddlers after cochlear implantation

The aims of this study were to examine the development of visual attention in deaf and hearing infants and toddlers, and assess whether improvements in visual attention were observed in the deaf sample after 12 months of cochlear implantation. A novel puppet task, based on a measure of attention developed with normally hearing infants, was administered to 88 deaf and 42 normal-hearing children at three time points: baseline, six and 12 months post-implantation for the deaf sample. At baseline, deaf children demonstrated significantly more inattentive looks during the puppet skits than hearing children, and these looks were of longer duration, confirming the results of prior studies which have documented deficits in visual attention in deaf children. Longitudinal analyses showed significant decreases in the frequency of inattentive looks for both groups, with a significant decrease in the duration of inattentive looks only for the cochlear implant group. The largest decrease in duration of off-task looks occurred at six months post-implantation, indicating that improvements occurred rapidly after restoration of auditory input. These results provided support for the ‘division of labor’ hypothesis which suggests that deaf children with no access or limited access to sound must monitor their environment visually, making it difficult for them to focus and attend to specific tasks. Cochlear implantation appeared to alter the developmental trajectory of visual attention in a positive manner. The clinical implications of visual attention for the development of early language, reading and social skills are discussed.

Electric charge requirements of pediatric cochlear implant recipients enrolled in the childhood development after cochlear implantation study

Objective: To evaluate mapping characteristics of children with cochlear implants who are enrolled in the Childhood Development After Cochlear Implantation (CDACI) multicenter study. Study Design: Longitudinal evaluation during 24 months of speech processor maps of children with cochlear implants prospectively enrolled in the study. Setting: Six tertiary referral centers. Subjects: One hundred eighty-eight children enrolled in the CDACI study who were 5 years old or younger at the time of enrollment. Of these children, 184 received unilateral implants, and 4 received simultaneous bilateral implants. Intervention: Children attended regular mapping sessions at their implant clinic as part of the study protocol. Maps were examined for each subject at 4 different time intervals: at device activation and 6, 12, and 24 months postactivation. Main Outcome Measures: Mean C/M levels (in charge per phase) were compared for 4 different time intervals, for 3 different devices, for 6 different implant centers, and for children with normal and abnormal cochleae. Results: All 3 types of implant devices demonstrate significant increases in C/M levels between device activation and the 24-month appointment. Significant differences in mean C/M levels were noted between devices. Children with cochlear anomalies demonstrate significantly greater C/M levels than children with normal cochleae. Conclusion: The CDACI study has enabled us to evaluate the mapping characteristics of pediatric patients who use 3 different devices and were implanted at a variety of implant centers. Analysis of such data enables us to better understand the mapping characteristics of children with cochlear implants.