Faculty Bibliography

OBJECTIVE:To evaluate the performance changes after sequential bilateral cochlear implantation in a pediatric population of bimodal cochlear implant (CI) users. To evaluate the factors which influence the parental and recipient decision to discontinue hearing aid use and seek a second implant. STUDY DESIGN/METHODS:Retrospective case review, within-subject comparison. SETTING/METHODS:Tertiary referral center. PATIENTS/METHODS:Thirty-one pediatric (<18 yr) bimodal CI users who underwent sequential bilateral CI. INTERVENTIONS/METHODS:Sequential bilateral CI. MAIN OUTCOME MEASURES/METHODS:Parental and/or recipient's reasons for discontinuing their hearing aid and pursuing a second implant, device usage from datalogs, speech understanding in the bimodal and bilateral CI condition. RESULTS:Parents/patients were motivated to pursue sequential bilateral CI based on their positive performance with CI1, the expectation of further improvement with a second CI, and the prospect of having a second independently functional ear. In the bimodal condition, mean word recognition score (WRS), sentence recognition in quiet (SIQ), and sentence recognition in noise (SIN) scores were 87.4, 97.3, and 92.9% respectively. At 1-year post-sequential bilateral CI, the mean WRS, SIQ, and SIN score were 92.7, 98.7, and 97.7%, respectively. The improvement in bilateral CI speech scores compared with bimodal scores was statistically significant for WRS (p = 0.015). A ceiling effect limited the ability to detect further meaningful differences on speech perception testing. CONCLUSIONS:The bilateral CI condition demonstrates equivalent or slightly superior performance compared with the bimodal condition. Several non-speech benefits were elicited from parents as reasons for pursuing a second implant. Close monitoring of the residual acoustic hearing, inquiring about the perceived benefits provided by the HA, and early counseling regarding the potential for sequential bilateral CI are important aspects in determining if and when a second implant is indicated.

OBJECTIVES/OBJECTIVE:The auditory experience of early deafened pediatric cochlear implant (CI) users is different from that of postlingually deafened adult CI users due to disparities in the developing auditory system. It is therefore expected that the auditory psychophysical capabilities between these two groups would differ. In this study, temporal resolving ability was investigated using a temporal modulation detection task to compare the performance outcomes between these two groups. DESIGN/METHODS:The minimum detectable modulation depth of amplitude modulated broadband noise at 100 Hz was measured for 11 early deafened children with a CI and 16 postlingually deafened adult CI users. RESULTS:Amplitude modulation detection thresholds were significantly lower (i.e., better) for the pediatric CI users than for the adult CI users. Within each group, modulation detection thresholds were not significantly associated with chronologic age, age at implantation, or years of CI experience. CONCLUSIONS:Early implanted children whose auditory systems develop in response to electric stimulation demonstrate better temporal resolving abilities than postlingually deafened adult CI users. This finding provides evidence to suggest that early implanted children might benefit from sound coding strategies emphasizing temporal information.

OBJECTIVES: To examine the variables affecting outcomes for sequential bilateral cochlear implantation patients in the adolescent population. STUDY DESIGN: Retrospective chart review at tertiary care center. METHODS: Main outcome measures were open set speech recognition tests at the word (Consonant-Nucleus-Consonant/Phonetically Balanced Kindergarten List Test [CNC/PBK]) and sentence levels in noise (Hearing in Noise Test-Noise [HINT-N]) in different test conditions with respect to the age at first and sequential implantation, as well as the interval between implants. RESULTS: Despite a mean age at sequential implantation of 13.5 years, sequential bilateral implanted adolescents revealed significant improvement in the sequential cochlear implant (CI2) ear. The mean time interval between implants was 8.2 years. A wide range of performance was noted, and age at implantation and interval between first cochlear implant (CI1) and CI2 did not predict outcome. Mean CNC/PBK score with CI1 alone was 83.0%, with the CI2 alone was 56.5%, and with bilateral implants was 86.8%. Sentence scores (HINT-N) were 89.5% for CI1, 74.2% for CI2, and 94.4% for bilateral CI condition. The clinical relevance of these enhanced perception abilities requires attention to individual device use, performance with the first implant, and subjective benefits reported by patients. CONCLUSIONS: Bilateral sequential cochlear implantation leads to improved speech perception in the adolescent population and should be considered in this population, even after a long period of deafness and despite a prolonged interval between implants. Numerous factors affect the ability to predict performance, but age at implantation and interimplant interval were not correlated with performance measures. Extensive preoperative counseling and individualized evaluation are critical to ensure that patients and families understand the range of possible outcomes. LEVEL OF EVIDENCE: 6. Laryngoscope, 2015.

OBJECTIVE: To assess whether recombinant growth factor (hGH) therapy has an effect on cochlear implant (CI) performance. PATIENTS: Two pediatric CI recipients (S1, S2) who underwent treatment with hGH for short stature were identified for review. S1 has bilateral labyrinthine dysplasia and received implants at ages 10 months (right) and 4 years 3 months (left). S2 was diagnosed with severe to progressive sensorineural hearing loss bilaterally and received a CI at age 9 years 10 months (left). INTERVENTION(S): Case series. MAIN OUTCOME MEASURE(S): Cochlear implant, hGH, and speech perception data were collected. Phonetically Balanced Kindergarten (PBK) and Consonant Nucleus Consonant (CNC) word recognition scores were reviewed to assess auditory perception. Electrode impedances, threshold levels, and comfort levels were also reviewed. RESULTS: After 4 months of hGH, word recognition scores for S1 were observed to decrease from 90 to 72% (right) and were stable at 40% (left). Despite troubleshooting, performance continued to decline bilaterally to 52% (right) and 28% (left), and the decision was made to discontinue hGH. One month after cessation of hGH, word recognition scores began improving to 74% (right) and 68% (left). Word recognition scores for S2 were observed to have decreased from 92% the previous year to 82% after taking hGH for 2 months. Given both our previous experience with S1 and discussions with S2's parents, hGH was discontinued after 10 months of therapy. Two months after cessation of hGH, S2's word recognition had improved to 86% (left). CONCLUSIONS: Our case studies illustrate that implanted children undergoing treatment with hGH may experience a decrease in speech perception, which recovers after the cessation of treatment. Since hGH use has become more prevalent in recent years, it is important to inquire whether children undergoing, or who have undergone, implantation are receiving hGH so that they may be appropriately monitored.

OBJECTIVES: To determine which demographic or performance variables are associated with inconsistent use of a second implant in pediatric recipients of sequential bilateral cochlear implants (CIs). METHODS: A retrospective chart review was conducted on pediatric recipients of sequential bilateral CIs. Children were divided into two age groups, 5-9 and 10-17 years of age. For each group, we examined whether inconsistent use of the second implant (CI-2) was associated with a variety of demographic variables, or speech-perception scores. RESULTS: In children aged 5-9 years, inconsistent use of CI-2 was not significantly associated with any demographic variable, but was related to both the word-recognition score with CI-2, and the difference in word-recognition scores between the first implant (CI-1) and CI-2. In children aged 10-17 years, these relationships were not significant due to smaller number of subjects. Finally, CI-2 word-recognition scores across all children were significantly correlated with the age of implantation for both CI-1 and CI-2, and the time between CI-1 and CI-2 surgeries. DISCUSSION: Speech-recognition scores obtained with CI-2, and the extent to which it differs from CI-1, are most closely related with inconsistent use of CI-2 in pediatric sequential implantees. These results are consistent with similar data previously reported by other investigators. While children implanted with CI-2 at a later age generally perform more poorly, most children still use both implants, and benefit from CI-2 even when receiving the implant as an adolescent. CONCLUSION: In pediatric recipients of sequential bilateral CIs, inconsistent use of CI-2 is related to the speech recognition scores with CI-2, and the difference in speech-recognition scores between CI-1 and CI-2. In addition, speech-recognition scores with CI-2 are related to the amount of time between CI-1 and CI-2 surgeries, and the age of implantation for both CI-1 and CI-2.

OBJECTIVES: To determine the efficacy of cochlear implantation (CI) in prelingually deafened adolescent children and to evaluate predictive variables for successful outcomes. DESIGN: Retrospective medical record review. PARTICIPANTS: Children aged 10 to 17 years with prelingual hearing loss (mean length of deafness, 11.5 years) who received a unilateral CI (mean age at CI, 12.9 years). Intervention Unilateral CI. MAIN OUTCOME MEASURES: Standard speech perception testing (Consonant-Nucleus-Consonant [CNC] monosyllabic word test and Hearing in Noise [HINT] sentence test) was performed preoperatively, 1 year postoperatively (year 1), and at the last follow-up/end of the study (EOS). RESULTS: There was a highly significant improvement in speech perception scores for both HINT sentence and CNC word testing from the preoperative testing to year 1 (mean change score, 51.10% and 32.23%, respectively; P < .001) and from the preoperative testing to EOS (mean change score, 60.02% and 38.73%, respectively; P < .001), with a significantly greater increase during the first year (P < .001). In addition, there was a highly significant correlation between improvements in performance scores on the CNC word and HINT sentence speech perception tests and both age at CI and length of deafness at the year 1 testing (P </=.009) but not from the year 1 testing to EOS testing. Adolescents with progressive deafness and those using oral communication before CI performed significantly better than age-matched peers. CONCLUSIONS: Adolescents with prelingual deafness undergoing unilateral CI show significant improvement in objective hearing outcome measures. Patients with shorter lengths of deafness and earlier age at CI tend to outperform their peers. In addition, patients with progressive deafness and those using oral communication have significantly better objective outcomes than their peers

OBJECTIVE:: To determine whether intraoperative neural response telemetry (tNRT) is predictive of postoperative speech perception. STUDY DESIGN:: Retrospective review. SETTING:: Tertiary referral center. PATIENTS:: Children (n = 24) aged between 5 and 17 years and adults 18 years and older (n = 73) with severe-to-profound hearing loss and implanted with the Nucleus Freedom device between 2005 and 2008 and observed at least 1 year were included. INTERVENTION:: Intraoperative neural response telemetry after insertion of the electrode array. MAIN OUTCOME MEASURE:: Measures included 1) intraoperative tNRT measurements and 2) preoperative and 1-year postoperative open-set word recognition scores using age-appropriate open-set tests for children and adults. Intraoperative neural response telemetry levels for electrodes E20, E15, E10, and E5 in each patient were correlated to performance at the 1-year evaluation interval. RESULTS:: No correlation existed between tNRT responses and open-set speech performance at the 1-year evaluation. Several patients had absent tNRT in the OR but developed speech recognition abilities, whereas the remaining patients had intraoperative responses with levels of postoperative performance ranging from 0% to 100%. CONCLUSION:: This study suggests that there is no significant correlation between intraoperative tNRT and speech perception performance at 1 year. At the time of surgery, tNRT provides valuable information regarding the electrical output of the implant and the response of the auditory system to electrical stimulation and preliminary device programming data; however, it is not a valuable predictor of postoperative performance. Furthermore, the absence of tNRT does not necessarily indicate a lack of stimulation

OBJECTIVES: Ear anomalies and deafness are associated with CHARGE syndrome, which also presents with a cluster of features including coloboma of the eye, heart defects, atresia of the choanae, developmental retardation, and genitourinary abnormalities. The aim of this study is to explore the viability of cochlear implantation in children with CHARGE syndrome and to assess the outcome. STUDY DESIGN: Retrospective chart review. METHODS: Eleven children presenting with severe to profound sensorineural hearing loss associated with CHARGE syndrome were the subjects of this study. Routine audiometric measurements and the Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS) were performed pre- and postoperatively. In addition, the degree of the subjects' cochlear deformity were measured and correlated to outcome. RESULTS: All patients had varying degrees of ear anomalies, seven patients suffered from coloboma of the eyes, two had heart defects, five exhibited choanal atresia, eleven showed developmental retardation, and six had genitourinary abnormalities. Ten of the children underwent cochlear implantation with complete insertion of the electrode array without complication and were followed over a 3-month to a 7-year period. The eleventh child was not implanted because of severe retardation. All of the implanted children showed varying, but limited degrees, of auditory benefit as measured by routine audiometry and the IT-MAIS. CONCLUSIONS: Careful treatment planning for children with sensorineural hearing loss and CHARGE syndrome can lead to varying, but limited degrees, of auditory benefit with no increase in surgical complications. Although the implant enhanced the children's 'connectivity' to the environment, it did not promote the development of oral language skills in this population

OBJECTIVE: To document oral language proficiency in a group of prelingually deaf bilingual children with a cochlear implant. DESIGN: Using a repeated-measures paradigm, oral language skills in the first and second language were evaluated at 2 yearly intervals after implantation. Language data were compared with normative data from children with normal hearing. SUBJECTS: Twelve deaf children between the ages of 20 months and 15 years who had received a cochlear implant before the age of 3 years. OUTCOME MEASURE: First-language skills were assessed using 1 of 2 standardized tests, either the Oral and Written Language Scales or the Reynell Developmental Language Scales, depending on the child's age. Second-language proficiency was assessed using the Student Oral Language Observation Matrix. RESULTS: Average standard scores in the first language fell solidly within the average range of normal-hearing peers. Second-language skills showed steady improvement from year 1 to year 2, along a continuum that reflected the amount and intensity of exposure of the child to the second language and the length of experience with the implant. CONCLUSION: A cochlear implant can make oral proficiency in more than 1 language possible for prelingually deaf children