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Item 12 guiding premises of pediatric cochlear implant habilitation(ScienceDirect, 2018-02-28) Robbins, Amy McConkey; Otolaryngology -- Head and Neck Surgery, School of MedicineItem Cochlear Implantation in US Military Veterans: A Single Institution Study(Sage, 2023-05-12) Totten, Douglas J.; Saltagi, Abdul; Libich, Karen; Pisoni, David B.; Nelson, Rick F.; Otolaryngology -- Head and Neck Surgery, School of MedicineObjective: Military veterans have high rates of noise-induced hearing loss (NIHL) which is associated with more significant spiral ganglion neuronal loss. This study explores the relationship between NIHL and cochlear implant (CI) outcomes in veterans. Study design: Retrospective case series of veterans who underwent CI between 2019 and 2021. Setting: Veterans Health Administration hospital. Methods: AzBio Sentence Test, Consonant-Nucleus-Consonant (CNC) scores, and Speech, Spatial, and Qualities of Hearing Scale (SSQ) were measured pre- and postoperatively. Linear regression assessed relationships between outcomes and noise exposure history, etiology of hearing loss, duration of hearing loss, and Self-Administered Gerocognitive Exam (SAGE) scores. Results: Fifty-two male veterans were implanted at an average (standard deviation) age of 75.0 (9.2) years without major complications. The average duration of hearing loss was 36.0 (18.4) years. The average time of hearing aid use was 21.2 (15.4) years. Noise exposure was reported in 51.3% of patients. Objectively, AzBio and CNC scores 6 months postoperatively showed significant improvement of 48% and 39%, respectively. Subjectively, average 6-month SSQ scores showed significant improvement by 34 points (p < .0001). Younger age, SAGE score ≥17, and shorter duration of amplification were associated with higher postoperative AzBio scores. Greater improvement in AzBio and CNC scores was associated with lower preoperative scores. Noise exposure was not associated with any difference in CI performance. Conclusion: Despite high levels of noise exposure and advanced age, veterans derive substantial benefits from cochlear implantation. SAGE score ≥17 may be predictive of overall CI outcomes. Noise exposure does not impact CI outcomes.Item Family Environment in Children With Hearing Aids and Cochlear Implants: Associations With Spoken Language, Psychosocial Functioning, and Cognitive Development(Wolters Kluwer, 2020) Frush Holt, Rachael; Beer, Jessica; Kronenberger, William G.; Pisoni, David B.; Lalonde, Kaylah; Mulinaro, Lindsay; Otolaryngology -- Head and Neck Surgery, School of MedicineObjectives: To examine differences in family environment and associations between family environment and key speech, language, and cognitive outcomes in samples of children with normal hearing and deaf and hard-of-hearing (DHH) children who use hearing aids and cochlear implants. Design: Thirty families of children with normal hearing (n = 10), hearing aids (n = 10), or cochlear implants (n = 10) completed questionnaires evaluating executive function, social skills, and problem behaviors. Children's language and receptive vocabulary were evaluated using standardized measures in the children's homes. In addition, families were administered a standardized in-home questionnaire and observational assessment regarding the home environment. Results: Family environment overall was similar across hearing level and sensory aid, although some differences were found on parental responsivity and physical environment. The level of supportiveness and enrichment within family relationships accounted for much of the relations between family environment and the psychosocial and neurocognitive development of DHH children. In contrast, the availability of objects and experiences to stimulate learning in the home was related to the development of spoken language. Conclusions: Whereas broad characteristics of the family environments of DHH children may not differ from those of hearing children, variability in family functioning is related to DHH children's at-risk speech, language, and cognitive outcomes. Results support the importance of further research to clarify and explain these relations, which might suggest novel methods and targets of family-based interventions to improve developmental outcomes.Item Functional Hearing Quality in Prelingually Deaf School-Age Children and Adolescents with Cochlear Implants(Taylor & Francis, 2021) Kronenberger, William G.; Bozell, Hannah; Henning, Shirley C.; Montgomery, Caitlin J.; Ditmars, Allison M.; Pisoni, David B.; Psychiatry, School of MedicineObjective: This study investigated differences in functional hearing quality between youth with cochlear implants (CIs) and normal hearing (NH) peers, as well as associations between functional hearing quality and audiological measures, speech perception, language and executive functioning (EF). Design: Youth with CIs and NH peers completed measures of audiological functioning, speech perception, language and EF. Parents completed the Quality of Hearing Scale (QHS), a questionnaire measure of functional hearing quality. Study sample: Participants were 43 prelingually-deaf, early-implanted, long-term CI users and 43 NH peers aged 7-17 years. Results: Compared to NH peers, youth with CIs showed poorer functional hearing quality on the QHS Speech, Localization, and Sounds subscales and more hearing effort on the QHS Effort subscale. QHS scores did not correlate significantly with audiological/hearing history measures but were significantly correlated with most speech perception, language and EF scores in the CI sample. In the NH sample, QHS scores were uncorrelated with speech perception and language and were inconsistently correlated with EF. Conclusions: The QHS is a valid measure of functional hearing quality that is distinct from office-based audiometric or hearing history measures. Functional hearing outcomes are associated with speech-language and EF outcomes in CI users.Item Information theoretic evaluation of a noiseband-based cochlear implant simulator(Elsevier, 2016-03) Aguiar, Daniel E.; Taylor, N. Ellen; Li, Jung; Gazanfari, Daniel K.; Talavage, Thomas M.; Laflen, J. Brandon; Neuberger, Heidi; Svirsky, Mario A.; Department of Otolaryngology--Head & Neck Surgery, School of MedicineNoise-band vocoders are often used to simulate the signal processing algorithms used in cochlear implants (CIs), producing acoustic stimuli that may be presented to normal hearing (NH) subjects. Such evaluations may obviate the heterogeneity of CI user populations, achieving greater experimental control than when testing on CI subjects. However, it remains an open question whether advancements in algorithms developed on NH subjects using a simulator will necessarily improve performance in CI users. This study assessed the similarity in vowel identification of CI subjects and NH subjects using an 8-channel noise-band vocoder simulator configured to match input and output frequencies or to mimic output after a basalward shift of input frequencies. Under each stimulus condition, NH subjects performed the task both with and without feedback/training. Similarity of NH subjects to CI users was evaluated using correct identification rates and information theoretic approaches. Feedback/training produced higher rates of correct identification, as expected, but also resulted in error patterns that were closer to those of the CI users. Further evaluation remains necessary to determine how patterns of confusion at the token level are affected by the various parameters in CI simulators, providing insight into how a true CI simulation may be developed to facilitate more rapid prototyping and testing of novel CI signal processing and electrical stimulation strategies.Item Language processing fluency and verbal working memory in prelingually deaf long-term cochlear implant users: A pilot study(Taylor & Francis, 2018-11) Kronenberger, William G.; Henning, Shirley C.; Ditmars, Allison M.; Pisoni, David B.; Psychiatry, School of MedicineOBJECTIVE: Verbal working memory (WM) is more strongly correlated with spoken language skills in prelingually deaf, early-implanted cochlear implant (CI) users than in normal-hearing (NH) peers, suggesting that CI users access WM in order to support and compensate for their slower, more effortful spoken language processing. This pilot study tested the feasibility and validity of a dual-task method for establishing the causal role of WM in basic language processing (lexical access speed) in samples of 9 CI users (ages 8-26 years) and 9 NH peers. METHODS: Participants completed tests of lexical access speed (rapid automatized picture naming test and lexical decision test) under two administration conditions: a standard condition and a dual-task WM condition requiring participants to hold numerals in WM during completion of the lexical access speed tests. RESULTS: CI users showed more dual-task interference (decline in speed during the WM condition compared to the standard condition) than NH peers, indicating that their lexical access speed was more dependent on engagement of WM resources. Furthermore, dual-task interference scores were significantly correlated with several measures of speed-based executive functioning (EF), consistent with the hypothesis that the dual-task method reflects the involvement of EF in language processing. CONCLUSION: These pilot study results support the feasibility and validity of the dual-task WM method for investigating the influence of WM in the basic language processing of CI users. Preliminary findings indicate that CI users are more dependent on the use of WM as a compensatory strategy during slow-effortful basic language processing than NH peers.Item Long-Term Speech and Language Outcomes in Prelingually Deaf Children, Adolescents and Young Adults Who Received Cochlear Implants in Childhood(Karger, 2013) Ruffin, Chad V.; Kronenberger, William G.; Colson, Bethany G.; Henning, Shirley C.; Pisoni, David B.; Otolaryngology -- Head and Neck Surgery, School of MedicineThis study investigated long-term speech and language outcomes in 51 prelingually deaf children, adolescents and young adults who received cochlear implants (CIs) prior to 7 years of age and had used their implants for at least 7 years. Average speech perception scores were similar to those found in prior research with other samples of experienced CI users. Mean language test scores were lower than norm-referenced scores from nationally representative normal-hearing, typically developing samples, although a majority of the CI users scored within 1 standard deviation of the normative mean or higher on the Peabody Picture Vocabulary Test, Fourth Edition (63%), and the Clinical Evaluation of Language Fundamentals, Fourth Edition (69%). Speech perception scores were negatively associated with a meningitic etiology of hearing loss, older age at implantation, poorer preimplant unaided pure-tone average thresholds, lower family income and the use of 'total communication'. Subjects who had used CIs for 15 years or more were more likely to have these characteristics and were more likely to score lower on measures of speech perception compared to those who had used CIs for 14 years or less. The aggregation of these risk factors in the >15 years of CI use subgroup accounts for their lower speech perception scores and may stem from more conservative CI candidacy criteria in use at the beginning of pediatric cochlear implantation.Item Speed of Information Processing and Verbal Working Memory in Children and Adolescents With Cochlear Implants(Wolters Kluwer, 2023) Herran, Reid M.; Montgomery, Caitlin J.; Henning, Shirley C.; Herbert, Carolyn J.; Ditmars, Allison M.; Yates, Catherine J.; Pisoni, David B.; Kronenberger, William G.; Otolaryngology -- Head and Neck Surgery, School of MedicineBackground: Verbal working memory delays are found in many deaf children with cochlear implants compared with normal-hearing peers, but the factors contributing to these delays are not well understood. This study investigated differences between cochlear implant users and normal-hearing peers in memory scanning speed during a challenging verbal working memory task. To better understand variability in verbal working memory capacity within each sample, associations between memory scanning speed, speech recognition, and language were also investigated. Methods: Twenty-five prelingually deaf, early implanted children (age, 8-17 yr) with cochlear implants and 25 normal-hearing peers completed the Wechsler Intelligence Scale for Children, Fifth Edition, Letter-Number Sequencing (LNS) working memory task. Timing measures were made for response latency and average pause duration between letters/numbers recalled during the task. Participants also completed measures of speech recognition, vocabulary, and language comprehension. Results: Children with cochlear implants had longer pause durations than normal-hearing peers during three-span LNS sequences, but the groups did not differ in response latencies or in pause durations during two-span LNS sequences. In the sample of cochlear implant users, poorer speech recognition was correlated with longer pause durations during two-span sequences, whereas poorer vocabulary and weaker language comprehension were correlated with longer response latencies during two-span sequences. Response latencies and pause durations were unrelated to language in the normal-hearing sample. Conclusion: Children with cochlear implants have slower verbal working memory scanning speed than children with normal hearing. More robust phonological-lexical representations of language in memory may facilitate faster memory scanning speed and better working memory in cochlear implant users.Item TMPRSS3 expression is limited in spiral ganglion neurons: implication for successful cochlear implantation(BMJ, 2022) Chen, Yuan-Siao; Cabrera, Ernesto; Tucker, Brady J.; Shin, Timothy J.; Moawad, Jasmine V.; Totten, Douglas J.; Booth, Kevin T.; Nelson, Rick F.; Otolaryngology -- Head and Neck Surgery, School of MedicineBackground: It is well established that biallelic mutations in transmembrane protease, serine 3 (TMPRSS3) cause hearing loss. Currently, there is controversy regarding the audiological outcomes after cochlear implantation (CI) for TMPRSS3-associated hearing loss. This controversy creates confusion among healthcare providers regarding the best treatment options for individuals with TMPRSS3-related hearing loss. Methods: A literature review was performed to identify all published cases of patients with TMPRSS3-associated hearing loss who received a CI. CI outcomes of this cohort were compared with published adult CI cohorts using postoperative consonant-nucleus-consonant (CNC) word performance. TMPRSS3 expression in mouse cochlea and human auditory nerves (HAN) was determined by using hybridisation chain reaction and single-cell RNA-sequencing analysis. Results: In aggregate, 27 patients (30 total CI ears) with TMPRSS3-associated hearing loss treated with CI, and 85% of patients reported favourable outcomes. Postoperative CNC word scores in patients with TMPRSS3-associated hearing loss were not significantly different than those seen in adult CI cohorts (8 studies). Robust Tmprss3 expression occurs throughout the mouse organ of Corti, the spindle and root cells of the lateral wall and faint staining within <5% of the HAN, representing type II spiral ganglion neurons. Adult HAN express negligible levels of TMPRSS3. Conclusion: The clinical features after CI and physiological expression of TMPRSS3 suggest against a major role of TMPRSS3 in auditory neurons.Item Verbal learning and memory in prelingually deaf children with cochlear implants(Taylor & Francis, 2018-10) Kronenberger, William G.; Henning, Shirley C.; Ditmars, Allison M.; Roman, Adrienne S.; Pisoni, David B.; Psychiatry, School of MedicineOBJECTIVE: Deaf children with cochlear implants (CIs) show poorer verbal working memory compared to normal-hearing (NH) peers, but little is known about their verbal learning and memory (VLM) processes involving multi-trial free recall. DESIGN: Children with CIs were compared to NH peers using the California Verbal Learning Test for Children (CVLT-C). STUDY SAMPLE: Participants were 21 deaf (before age 6 months) children (6-16 years old) implanted prior to age 3 years, and 21 age-IQ matched NH peers. RESULTS: Results revealed no differences between groups in number of words recalled. However, CI users showed a pattern of increasing use of serial clustering strategies across learning trials, whereas NH peers decreased their use of serial clustering strategies. In the CI sample (but not in the NH sample), verbal working memory test scores were related to resistance to the build-up of proactive interference, and sentence recognition was associated with performance on the first exposure to the word list and to the use of recency recall strategies. CONCLUSIONS: Children with CIs showed robust evidence of VLM comparable to NH peers. However, their VLM processing (especially recency and proactive interference) was related to speech perception outcomes and verbal WM in different ways from NH peers.