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Background: In order to optimise the support of children with cochlear implants (CI), it is very important to detect slow developmental processes as early as possible. Data from the LittlEARS® Auditory Questionnaire (LEAQ) from children with early bilateral CI are evaluated and presented in relation to age and hearing age and compared with language development data recorded later., Materials and Methods: This retrospective multicentre study included data from a total of 554 children for whom at least one LEAQ was completed during the course of CI rehabilitation. Children without additional disabilities who received bilateral simultaneous or sequential CI treatment were included., Results: As expected, there are high correlations between hearing age (HA) and the overall LEAQ total score. When analysed according to chronological age (CA), development runs roughly parallel to the development of children with normal hearing, albeit at a lower level. Children implanted early up to an age of ≤ 12 months consistently achieve approximately 7-8 raw points more. Only the LEAQ results of the later test times (from the age of 18 months) correlate with some areas of the speech development test for children (SETK; areas 3-5)., Conclusion: The earliest possible detection of critical developmental processes in children with CI is extremely important. In the case of very early CI fitting, the CA should be used as a reference measure in diagnostics. The LEAQ values determined for the group of children with CI are suitable to a limited extent as generally valid reference values for children with early bilateral CI. Further studies should continue to work out the correlations between early preverbal development and later speech development., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Background: Very early bilateral cochlear implant (CI) provision is today's established standard for children. Therefore, the assessment of preverbal and verbal performance in very early stages of development is becoming increasingly important. Performance data from cohorts of children were evaluated and presented based on diagnostic assessment using chronological age (CA) and hearing age (HA)., Methods: The present study, as part of a retrospective multicentre study, included 4 cohorts (N = 72-233) of children with bilateral CI without additional disabilities. Their results in the German parent questionnaires Elternfragebögen zur Früherkennung von Risikokindern(ELFRA‑1 and ELFRA-2) subdivided for CA and HA were statistically analysed. The data were also analysed in terms of mono-/bilingualism and age at CI provision., Results: Overall, verbal performance in relation to CA was lower than in relation to HA. Preverbal skills were largely CA appropriate. Children with bi-/multilingual language acquisition performed significantly lower. Verbal performance in ELFRA‑2 referenced to CA was negatively correlated with age at CI provision., Conclusion: In the case of early CI provision, CA should be the preferred reference mark in preverbal and verbal assessment in order to obtain exact individual performance levels and avoid bias in results. The percentiles determined are of limited use as generally valid reference values to which the individual performance of bilaterally implanted children could be compared. Further multicentre studies should be initiated., (© 2024. The Author(s).)

Background: Normal-hearing children show signs of various phonological processes during language development. These processes represent simplifications of articulation, which are overcome at different time points. For the German language, there are currently no reliable data regarding whether these developmental stages also apply to deaf children with cochlear implants (CI)., Materials and Methods: Phonological development in deaf children with CI was examined and evaluated with the PLAKSS ("Psycholinguistischen Analyse kindlicher Sprechstörungen"). The results of this analysis (time of test 1 = T1, n = 33) were compared to those of a PLAKSS diagnostic evaluation performed 1 year previously (time of test 0 = T0, n = 31)., Results: At T1, 76 % of the whole group showed a phonological development that did not correspond to their hearing age (as measured from the time of the first CI implantation). The most frequently observed phonological processes were the reduction of consonant clusters and fronting. However, 83 % of the group had fewer phonological processes inappropriate to their hearing age at T1 than they did at T0., Conclusion: The phonological development of children with CI is not equivalent to their hearing age and is structured differently to that of normal-hearing children.

Background: After cochlear implantation, most parents expect a normal speech and general development of their child. However, it remains unclear how quickly after early cochlear implantation these children can compensate for their deficits compared to normal-hearing children., Methods: This study retrospectively analyzed ELFRA-1 questionnaire data from 40 children with borderline deafness or high-grade hearing loss (without other known impairments) who had undergone cochlear implantation at a university medical center before reaching 2 years of age. ELFRA-1 questionnaires were filled out parents assisted by specialists 12 months after implantation. Questions assessed the children's speech production and comprehension, as well as their use of gestures and fine motoric skills., Results: At an average hearing-age of 12 months, the children achieved normal values in all of the subgroups that were comparable to those of 12-month-old children without hearing impairments. A significant correlation (p = 0.01) between the individual subgroups of the ELFRA-1 (speech production, speech comprehension, gestures and fine motor skills) was observed. Unilingual educated children performed significantly better overall., Conclusion: Within 12 months of receiving a cochlear implant, all children passed the four categories of the ELFRA-1. This demonstrates a rapid compensation of deficits in speech, motor skills and gesture development by children undergoing early cochlear implantation.

Auditory synaptopathy/neuropathy (AS/AN) is a special subtype of sensorineural hearing disorders with heterogeneous phenotypes and underestimated incidence. AS/AN generally develops in infancy, occasionally in adulthood. Symptoms include fluctuating, mostly bilateral hearing loss and abnormally reduced speech comprehension, especially in noisy environments. Within audiological assessments, patients with AS/AN present otoacoustic emissions (TEOAE; DPOAE) and cochlear microphonics (CM), absence of stapedius reflexes (SR) as well as absent or pathologically altered auditory evoked brainstem potentials (ABR). Children with AS/AN cannot be identified within OAE-based newborn hearing screening programs. Clinical findings, transtympanic electrocochleography (ECoG) and further diagnostic tools permit further identification of individual characteristics. In individual cases conventional amplification and the use of FM systems may improve hearing and communication skills. If these interventions, accompanied by intensive hearing, speech and language therapy are unsuccessful, cochlear implants (CI) or alternative forms of communication may be useful options for rehabilitation.

Background: The Functioning After Pediatric Cochlear Implantation (FAPCI) instrument was recently developed to determine the communicative performance of 2-5-year-old prelingually deafened, cochlear-implanted children. Because of its high reliability and validity, as well as possible additional information compared with existing questionnaires, the 23-item parent-proxy questionnaire was translated from U.S. English to German prior to validation., Material and Methods: Initially, the German inventory was qualitatively developed by experts in audiology and speech pathology in collaboration with a professional American translator. Based on a sample of parents' responses, the outcome was quantitatively validated using psychometric methods (Cronbach's alpha, principal components analysis). Finally, the nomological validity was verified by correlating the overall FAPCI value with an external criterion (i.e., hearing age)., Results: For almost all age groups, Cronbach's alpha exceeded the minimum value of the original study (0.86). Principal components analysis revealed a two-factor solution (speech perception/production). The fitting of a nonparametric regression line to the data points showed that the total FAPCI score was positively associated with the time of implant use., Conclusion: The results show concordance between the German and the English versions of the FAPCI. The two instruments agree in reliability as well as in validity. The suitability of the German version in the clinical and therapeutic routine needs to be confirmed in further investigations.

Background and Objective: This study compares the results of the outpatient-based program of the Cochlear Implant Center Ruhr with inpatient-based rehabilitation, which is almost exclusively performed in Germany., Patients/methods: The Department of Otorhinolaryngology at the University of Essen in Germany provided 52 patients with either 22- or 24-channel Nucleus cochlear implants from March 1996 to July 1999. Almost all patients (n = 49) were rehabilitated on an outpatient basis, which is the standard in many cochlear implant centers outside Germany., Results: The longest follow-up period at the University of Essen Department of Otorhinolaryngology was 36 months. Minor complications occurred in 10% of the patients. After 24 months, the first three implanted patients were able to discriminate 100% of numbers and over 60% of syllables in the Freiburg speech discrimination test. The patients who developed an understanding of open speech were able to discriminate 31 words per minute with cochlear implant and without lipreading after 24 months. Children were seen to double their Schmid-Giovannini scores at 6 months postimplantation., Conclusions: The Essen outpatient-based cochlear implant program demonstrates results in speech development and speech understanding equal to those of centers providing inpatient rehabilitation. A special advantage is continuous rehabilitation with professionals known to the child for several years. In children especially, exhaustive commuting reduces school attendance and is a burden on the accompanying guardians. As an inpatient, however, the child is torn from his familiar environment. Parents with several children have particular difficulties in accompanying their child and indeed this may not always be possible.