Pediatric Cochlear Implants: Additional Disabilities Prevalence, Risk Factors, and Effect on Language Outcomes
ABSTRACT To determine the prevalence of additional disabilities in a pediatric cochlear population, to identify medical and radiologic conditions associated with additional disabilities, and to identify the effect of additional disabilities on speech perception and language at 12 months postoperatively.
Retrospective case review.
Tertiary referral center and cochlear implant program.
Records were reviewed for children 0 to 16 years old inclusive, who had cochlear implant-related operations over a 12-month period.
Diagnostic and rehabilitative.
Additional disabilities prevalence; medical history and radiologic abnormalities; and the effect on Categories of Auditory Performance (CAP) score at 12 months postoperatively.
Eighty-eight children having 96 operations were identified. The overall prevalence of additional disabilities (including developmental delay, cerebral palsy, visual impairment, autism and attention deficit disorder) was 33%. The main conditions associated with additional disabilities were syndromes and chromosomal abnormalities (87%), jaundice (86%), prematurity (62%), cytomegalovirus (60%), and inner ear abnormalities including cochlea nerve hypoplasia or aplasia (75%) and semicircular canal anomalies (56%). At 12 months postoperatively, almost all (96%) of the children without additional disabilities had a CAP score of 5 or greater (speech), compared with 52% of children with additional disabilities. Children with developmental delay had a median CAP score of 4, at 12 months compared with 6 for those without developmental delay.
Additional disabilities are prevalent in approximately a third of pediatric cochlear implant patients. Additional disabilities significantly affect the outcomes of cochlear implants.
- SourceAvailable from: Jane Black
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- "Previously, few studies have analysed cases with multiple suspect factors, particularly in multi-handicapped children. Birman et al. (2012), for example, undertook a retrospective study of 88 children to determine the prevalence of additional disabilities in a PCI population, to identify medical and radiologic conditions associated with additional disabilities and to identify the effect of additional disabilities on speech perception and language at 12 months postoperatively. The numbers of conditions, such as rubella, cytomegalovirus, Connexin 26, and auditory nerve hypoplasia, were very small and could not be generalized – a common small sample difficulty. "
ABSTRACT: Identification and evaluation of prognostic factors that are associated with paediatric cochlear implantation (PCI) outcomes was the aim of this study. A retrospective review of 174 charts was performed at the Royal Children's Hospital and the Hear and Say Centre, Brisbane. This examined the possible influence of a number of variables (including age at implant, family, additional disabilities, surgical complications, gender, GJB2 mutations, meningitis, inner ear malformations, and prematurity) on outcome measures: receptive, expressive, and total language, receptive and expressive vocabulary, speech articulation and categories of auditory performance at 18-24 months post-implant. Multiple regression analysis was used to identify variables related to language and vocabulary outcomes. The findings suggest that inner ear malformations and family concern are negatively associated with receptive and expressive language and receptive vocabulary scores. There was marginal evidence to suggest that increasing age at implantation was associated with lower receptive and expressive language scores. Prognostic factors that have been adequately validated statistically include inner ear malformations, the influence of family and late age at implantation. However, this study identified a need to define better the impact of the various degrees of inner ear malformations, to particularly emphasize the role of family as a strong predictor of PCI outcomes, and to confine the study of 'age at implantation' to pre-lingually deafened children. Evaluation of prognostic factors is a key element in PCI. This study confirmed several factors that are strongly associated with outcomes. For better research, there is a need for universal standardized outcome measures and development of a standardized framework for recording patient data.Cochlear implants international 08/2013; 15(2). DOI:10.1179/1754762813Y.0000000045
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ABSTRACT: Objective To compare the long-term speech perception and production outcomes after cochlear implantation (CI) in children deafened by congenital cytomegalovirus (cCMV) with a matched group of Cx26-CI children by controlling for chronological age and magnetic resonance imaging (MRI) findings. Methods Retrospective review of 12 cCMV-CI children and matched Cx26-CI children for speech perception and speech production outcomes. Results Two trends were seen in our data. First, cCMV-CI children with normal MRI scans perform equally or even slightly better on speech perception tests compared to their Cx26-CI peers during the first three years. The majority of cCMV-CI children with normal MRI scans (5 out of 7), suffered from a delayed-onset SNHL. Their mean age at first implantation (2yr9m, range 15m-82m) was higher compared to their matched Cx26 peers (9m, range 7m-12m). Before being implanted, the majority of these delayed-onset hearing impaired children had benefitted from a certain period of normal hearing (with or without amplification of a hearing aid). Possibly, this input might have led to an advantage the first three years after CI. Second, results between cCMV-CI children with and cCMV-CI children without MRI abnormalities and their matched Cx26-CI counterparts tentatively suggest that, over a 5-yr follow-up period, cCMV-CI children with abnormalities on MRI scans catch up for speech perception, but lag behind for speech production. Conclusion cCMV-CI children with normal MRI scans perform equally or even slightly better on speech perception tests compared to their Cx26-CI peers during the first three years, whereas results between cCMV-CI children with and cCMV-CI children without MRI abnormalities and their matched Cx26-CI counterparts tentatively suggest that, over a 5-yr follow-up period, cCMV-CI children with abnormal MRI scans catch up for speech perception, but lag behind for speech production. In future, the inclusion of MRI results may assist in improved counseling of parents with cCMV deafened children seeking CI.International journal of pediatric otorhinolaryngology 01/2013; 78(3). DOI:10.1016/j.ijporl.2013.11.009 · 1.32 Impact Factor
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ABSTRACT: Die Cochleaimplantat(CI)-Versorgung hat sich als Standard bei der Versorgung von prälingual, postlingual sowie perilingual ertaubten (gehörlosen) und resthörigen Kindern etabliert. Bei beidseitiger Indikation wird heute standardmäßig eine beidseitige Versorgung empfohlen. Die CI-Versorgung ermöglicht das Hören und damit eine Sprachentwicklung, Sprachverständlichkeit und Sprachproduktion, deren Ergebnisse am besten sind, wenn die CI-Versorgung so frühzeitig wie möglich nach der sicheren Diagnosestellung erfolgt. Aufgrund der Weiterentwicklungen bei präoperativer Diagnostik, Elektrodendesign, Sprachkodierungsstrategien und chirurgischen Techniken wurden Indikationserweiterungen möglich. Heutzutage können mit Ausnahme einer cochleären Aplasie und/oder einer Aplasie des Hörnervs nahezu alle Fehlbildungen mit einem CI versorgt werden. Neue Indikationen betreffen die partielle sowie die einseitige Taubheit. CI-Versorgung ist ein ausgesprochener Teamprozess. Neben der medizinischen Nachbetreuung der Kinder ist die Einbeziehung von Eltern und Bezugspersonen in die Kooperation von implantierender Klinik, Audiologen, Pädagogen und der angeschlossenen Fördereinrichtungen für den Erfolg der Rehabilitation von Bedeutung.HNO 05/2013; 61(5). DOI:10.1007/s00106-013-2699-y · 0.54 Impact Factor