[Transregional tracking in newborn hearing screening]. / Regionenübergreifendes Tracking im Neugeborenen-Hörscreening.

BACKGROUND AND OBJECTIVE: When patients in the universal newborn hearing screening program move from one geographical area to another between initial screening and medical follow-up, the responsibility for their tracking also moves from one screening center to another. As a result, these patients are lost to follow-up according to the center which had initial responsibility. In cooperation with the Association of German Hearing Screening Centers ("Verband Deutscher Hörscreening-Zentralen e. V.," VDHZ) as an offer to the developers of tracking software, a concept for nationwide tracking including a reference implementation and evaluation is described. METHODS: On the basis of error analysis of real screening data, techniques for preprocessing data, the technical background of the interface, and details regarding integration of the interface into tracking software are presented. Data from a stress test are shown. RESULTS: In a simulation stress test with six hearing screening centers and 54,551 children, all requests were answered within an average response time of 637 ms (standard deviation, SD = 266 ms; median 613 ms). Anonymized surnames (n = 675/1.24%) and duplicate entries in the database (n = 49/0.01%) were detected. CONCLUSION: A transregional tracking procedure using heterogeneous tracking software is possible without the use of a standardized screening ID. The presented approach seems conceptually and technically suitable.

[Quality of universal newborn hearing screening results : Multicenter analysis of data recorded between 2009 and 2012 in four German states]. / Ergebnisqualität im universellen Neugeborenen-Hörscreening : Multizentrische Analyse von Daten der Jahre 2009 bis 2012 aus 4 Bundesländern.

BACKGROUND: Bearing in mind the impending evaluation of newborn hearing screening in Germany, this study investigated whether multicenter analysis of the screening results from four German states is possible and to what extent the results meet national quality and outcome criteria. MATERIALS AND METHODS: The screening data from 170 hospitals and a total of 533,150 newborns (21 % of all German newborns) from 2009 to 2012 were evaluated according to definite rules and analyzed in terms of averages, as well as over time. RESULTS: During the investigated period and averaged over the hospitals, the quality criteria "percentage of screened newborns" (91.4 %) and "percentage requiring further follow-up" (5.0 %), the "day of screening" (day 4), as well as the target parameter "age at diagnosis" (4.8 months) were not met. Steady improvements were observed over time: in the last year of the evaluation, 95.3 % of children were examined; only 4.8 % required follow-up and the age at diagnosis decreased to 4.2 months. On average, 83 % of the babies were screened before day 4. The steady reduction in variance of most of the variables from the participating hospitals indicates continual improvement. CONCLUSION: A multicenter analysis of screening data is possible and valid in the case of good quality data.

[Universal newborn hearing screening : Definition of uniform parameters by the Association of German Hearing Screening Centers as a requirement for nationwide evaluation with valid results]. / Universelles Neugeborenen-Hörscreening : Definition einheitlicher Parameter durch den Verband Deutscher Hörscreening-Zentralen (VDHZ) als Voraussetzung für eine flächendeckende Evaluation mit validen Ergebnissen.

BACKGROUND: Since 2009, all newborns in Germany have been entitled to universal neonatal hearing screening (UNHS). UNHS with tracking of test results leads to earlier detection of hearing disorders. The Association of German Hearing Screening Centers (Verband Deutscher Hörscreening-Zentralen, VDHZ) was founded to promote nationwide tracking, validity and quality control of UNHS results. OBJECTIVES: A comparable data structure in the different screening centers, with uniform definitions of primary parameters is essential for the nationwide evaluation of UNHS results. To address the question of whether a data structure with comparable definitions already exists or still has to be created, the existing structures and primary parameter definitions in the hearing screening centers should be investigated and compared. METHODS: A survey was conducted in all hearing screening centers to assess how data on the primary UNHS parameters defined in pediatric guidelines was gathered. In the case of discrepancies, uniform definitions were created. Finally, the practicability of these definitions was evaluated. RESULTS: Due to differing definitions of primary parameters, some of the data were not comparable between the individual centers. Therefore, uniform definitions were created in a consensus process. In the centers, the screening method, the two-step first screening and the result of the first screening now correspond to these uniform definitions. Other parameters, e.g. the total number of newborns, still vary widely, rendering the comparison of screening rates almost impossible. CONCLUSION: Valid evaluation of UNHS not only requires nationwide establishment of hearing screening centers, but also unified data structures and parameter definitions.

[Using an employee survey as a means of quality assurance in newborn hearing screening]. / Mitarbeiterbefragung als instrument der qualitätssicherung im neugeborenen-hörscreening.

BACKGROUND AND OBJECTIVES: Studies drawing information not only from technical data but also from surveying human resources behind the universal newborn hearing screening (UNHS) appear to be a rarity. This study aims at showing how the state of both knowledge and practical skills among the screening staff are essential aspects in future quality management. MATERIALS AND METHODS: A self-developed questionnaire was sent to hospital staff addressing a total of 710 nurses who were registered as having undertaken a UNHS training course. Questions were aimed at aspects of organization, personal practical skills, current problems and improvement possibilities. RESULTS: High rates of occupancy, lack of trained personnel, technical issues and background noise disturbances were considered to be factors that increased time pressure and slowed down procedures. Of the participants 16 % considered communicating a "refer" result to parents a difficult step and 8 % felt insecure when explaining the aims and procedures to parents. There was a high interest in further training sessions. CONCLUSIONS: This survey served well to reveal aspects of improvement in screening procedures and meeting staff needs. The training sessions should outline practical aspects of conducting screening and also professional, sensitive communication to parents.

[Pedaudiological diagnostics in the first year of life . Clinical follow-up, risk factors, and middle ear function]. / Pädaudiologische Diagnostik im ersten Lebensjahr. Klinische Verläufe, Risikofaktoren und Mittelohrfunktion.

The universal neonatal hearing screening (UNHS) program demands detection of hearing loss within the first 3 months of life. Practicability and different screening methods should be evaluated. Thus, 617 patients (329 m., 288 w.) were analyzed; 246 children were referred in the UNHS, 389 with risk factors. In 459 children (74%), automated auditory brainstem response (ABR) screening in our department excluded hearing loss, thereof 129 (21%) underwent diagnostic auditory brainstem-evoked audiometry responses: 20 (16%) showed normal and 109 (84%) elevated ABR thresholds. A total of 91 children (83%) received hearing aids and 11 children (10%) treatment of middle ear effusion. Hearing loss was diagnosed in 18% of all children, 24% with UNHS referral and 34% with both referral and risk factors. Craniofacial anomalies, premature birth < 32 weeks of pregnancy, and syndromes were the most frequent risk factors. Reevaluation by ABR showed an improvement to normal hearing in 3 (of 14) children. The 226 Hz compared to 1,000 Hz-tympanometry showed different specificity (95.5 vs. 85.5%) and sensitivity (32.5 vs. 57.1%). Diagnosis within 3 months is possible, but very challenging in children with risk factors.

[Identifying language and communication disorders as part of the medical curriculum]. / Erkennen von Sprach- und Sprechstörungen als Lerninhalt des Medizinstudiums.

BACKGROUND: Like hearing loss, language and speech disorders can also lead to impaired communication. Speech and language disorders can occur at any age, for example as a specific language impairment, stuttering, dysarthria, and aphasia. Hence, irrespective of their speciality, there is a high probability that physicians will at some point encounter communication-impaired patients, be required to classify their disorder and refer them for appropriate therapy. METHODS: A new module entitled 'communication disorders' consisting of two teaching units was integrated in the practical course 'ENT--phoniatrics--pedaudiology' for undergraduate clinical students in 2008. The learning objective of the first unit was to identify and classify communication disorders, presented using sound and video data, by means of simple criteria. The module was evaluated on the basis of questionnaires completed by 164 students. RESULTS: On a scale of 1-7, the evaluation showed overall positive results with 6.54 (highest score) for professional competence and 5.32 (lowest score) for discussion. The overall score was 12.28 out of a possible maximum of 15 points. CONCLUSION: The ability to identify communication impairments is an important medical skill. Since communication disorders are common diseases we suggest that this skill be incorporated in the medical curriculum. Thus we have introduced a module for communication disorders; its evaluation by students shows a high level of satisfaction in each category. After developing specific diagnostic indicators students were able to independently describe and identify communication disorders.

TCERG1L allelic variation is associated with cisplatin-induced hearing loss in childhood cancer, a PanCareLIFE study.

In children with cancer, the heterogeneity in ototoxicity occurrence after similar treatment suggests a role for genetic susceptibility. Using a genome-wide association study (GWAS) approach, we identified a genetic variant in TCERG1L (rs893507) to be associated with hearing loss in 390 non-cranial irradiated, cisplatin-treated children with cancer. These results were replicated in two independent, similarly treated cohorts (n = 192 and 188, respectively) (combined cohort: P = 5.3 × 10-10, OR 3.11, 95% CI 2.2-4.5). Modulating TCERG1L expression in cultured human cells revealed significantly altered cellular responses to cisplatin-induced cytokine secretion and toxicity. These results contribute to insights into the genetic and pathophysiological basis of cisplatin-induced ototoxicity.

[Do children with cochlear implants read or write differently?: literacy acquisition after cochlear implantation]. / Lesen und schreiben CI-Kinder anders?: Schriftspracherwerb nach Versorgung mit einem Cochlear implant.

BACKGROUND: Despite the fact that literacy acquisition in hearing impaired children is frequently hampered, reading and writing competences continue not to be regularly evaluated and documented in children fitted with cochlear implants (CI). In this 2-year longitudinal study literacy acquisition in children fitted with CI was investigated. PATIENTS AND METHODS: In total, 18 pre- and primary school children fitted with CI who had suffered prelingual deafness were examined. Subjects' ages at CI fitting ranged from 0.9 to 5.9 years; they were raised orally and monolingual German and showed normal intellectual achievement. Familial risk of developing dyslexia was ruled out. To assess subjects' literacy acquisition precursor and partial abilities in reading and writing according to dual route and developmental models were examined three times within 2 years. Precursor abilities included development of vocabulary and phonological awareness. Partial abilities were mastery in sublexical and lexical word processing in reading and writing as well as auditory and visual working memory. RESULTS: Subjects showed a broad range in performance regarding vocabulary development as well as literacy. Discrepant results in terms of age equivalent visual and underachievement in auditory working memory as well as good achievement in implicit phonological awareness and weakness in explicit demands on phoneme analysis and manipulation of phonemes can be described. Indications were that subjects tended towards lip reading the instructor's item presentation. Performance in the administered writing test reveals a preference for lexical word processing, whereas sublexical word processing seems to make relatively higher demands on subjects. CONCLUSIONS: Easier processing of visual information in partial and precursor abilities are consistent with a tendency to prefer a visual-lexical processing strategy. The presented study stresses the importance of generally assessing reading and writing skills when evaluating language development in children supplied with cochlear implants. Partial and precursor abilities should be included in order to identify any delay in development promptly. Thus, individual qualitative characteristics can be addressed in therapeutic intervention.

[Deviant language development following cochlear implantation? Applicability of the parent questionnaire ELFRA-2]. / Abweichende Sprachentwicklung nach Cochleaimplantation? Zur Anwendbarkeit des Elternfragebogens ELFRA-2.

BACKGROUND: The parent questionnaire ELFRA-2 is considered a valid tool for early detection of delayed language development in 2-year-old children. Applicability for children treated with cochlear implants (CI) is to be investigated. METHODS: By means of the ELFRA-2 we documented longitudinally for up to 24 months post implantation language development in 27 children treated before 3 years of age. The critical developmental criteria (related to age) were applied to CI children (related to duration of CI use) and gender-related normative data were taken as a reference. RESULTS: Only two boys were identified as showing a language delay after 2 years of CI use. However, using normative data 11-44% of the children performed below average. Development in girls was faster than in boys. The influence of preoperative hearing experience declined over time. CONCLUSION: The critical developmental criteria of ELFRA-2 have proved to be unreliable for the identification of varying development after CI. Modified and gender-related evaluation is necessary.

Atypical organisation of the auditory cortex in dyslexia as revealed by MEG.

Neuroanatomical and -radiological studies have converged to suggest an atypical organisation in the temporal bank of the left-hemispheric Sylvian fissure for dyslexia. Against the background of this finding, we applied high temporal resolution magnetoencephalography (MEG) to investigate functional aspects of the left-hemispheric auditory cortex in 11 right-handed dyslexic children (aged 8-13 years) and nine matched normal subjects (aged 8-14 years). Event-related field components during a passive oddball paradigm with pure tones and consonant-vowel syllables were evaluated. The first major peak of the auditory evoked response, the M80, showed identical topographical distributions in both groups. In contrast, the generating brain structures of the later M210 component were located more anterior to the earlier response in children with dyslexia only. Control children exhibited the expected activation of more posterior source locations of the component that appeared later in the processing stream. Since the group difference in the relative location of the M210 source seemed to be independent of stimulus category, it is concluded that dyslexics and normally literate children differ as to the organisation of their left-hemispheric auditory cortex.

Melatonin is a useful alternative to sedation in children undergoing brainstem audiometry with an age dependent success rate--a field report of 250 investigations.

Though brainstem audiometry is one of the most important investigations in pediatric audiology, it often necessitates sedation or general anaesthetics, especially in newborns and infants. Melatonin, inducing natural sleep without the risks of sedation, has been successfully used to induce sleep prior to EEG investigations. 250 children (142 male, 108 female) with suspected hearing loss underwent ABR (auditory brainstem responses) tests in melatonin-induced sleep. Click-induced and notched-noise ABR tests were performed. Click tests were successfully performed in 216 of 249 children or 86.7% (123 male, 93 female), notched-noise tests in 115 of 155 children or 74.2%. Failure rates showed an age dependence increasing from 4% in children <1 year to 25%>3 years, but no gender difference. In conclusion, melatonin-induced sleep is a good alternative to sedation, especially in children younger than 3 years. This method is widely accepted by parents and permits earlier diagnosis of hearing impairment in a routine clinical setting. The number of children undergoing general anaesthesia for ABR investigation was reduced from over 60 per year in 2000-2002 to 12 in 2005, which means >80% less general anaesthesia.

[Model oriented assessment of literacy performance in children with cochlear implants]. / Vorschläge zur modellgeleiteten Erhebung der Schriftsprachkompetenzen bei Schulkindern nach Cochlea Implantation - eine Pilotstudie.

BACKGROUND: Although most hearing-impaired children lag behind normally hearing children in literacy acquisition, this aspect has hardly been addressed in the evaluation of language acquisition after cochlear implantation. The present study investigated written language abilities in 8 school-age children with cochlear implants. Neurolinguistic dual-route-models of written language processing indicate that literacy acquisition leads to the establishment of two distinct reading and writing strategies: a lexical one for the quick processing of known words and a sublexical one for decoding unfamiliar words or nonwords letter by letter. PATIENTS: 8 school-aged children were investigated, a very heterogeneous group concerning age of onset of hearing impairment, educational placement, and competences in sign language. However, this range is typical of the group of CI-children. METHODS: The aim was to investigate if children with cochlear implants are able to establish both strategies or if they need to find a differential and individual access to written language. Performance within the Salzburger Lese-Rechtschreib-Test was evaluated. Individual performance of each subject was analysed. RESULTS: Performance varied substantially ranging from only rudimentary spoken and written language abilities in two children to age-equivalent performance in three of them. Severe qualitative differences in written language processing were shown in the remaining three subjects. Suggestions for remediation were made and a re-test was carried out after 12 months. Their individual profiles of performance are described in detail. CONCLUSIONS: The present study stresses the importance of a thorough investigation of written language performance in the evaluation of language acquisition after cochlear implantation. The results draw a very heterogeneous picture of performance. Model-oriented testing and analysis of performance prove to be sensible in at least a subpopulation of children with cochlear implants. Based on a better understanding of their acquired word-processing strategies, remediation programs meeting the needs of each individual child can be derived.

[The use of melatonin as an alternative to sedation in children undergoing brainstem audiometry]. / Melatonin als Alternative zur Sedierung bei Durchführung einer Hirnstammaudiometrie bei Kindern.

BACKGROUND: Though one of the most important investigations in paediatric audiology, brainstem evoked response audiometry (BERA) often necessitates sedation or general anaesthetics, especially in newborn and infants. In paediatric neurology, melatonin has been successfully used for some years to induce sleep prior to EEG investigations. Melatonin as a hormone regulating the circadian rhythm induces natural sleep without the risks of sedation. Side effects are not known. METHODS: Click-induced BERA was first performed in 10 adults with normal hearing with and without previous melatonin administration, and click thresholds and latencies of evoked potentials were compared. 50 children then underwent BERA in melatonin-induced sleep. RESULTS: Click thresholds in adults were mostly identical (r = 0,88), while the mean latencies of evoked potentials seemed to be minimally prolonged (r from 0,82 to 0,95). Click-induced BERA was successful in 45 of the 50 children, and notched-noise BERA in at least 2 frequencies in 38 of 43 children. CONCLUSIONS: Offering a high success rate with no side effects, melatonin-induced sleep seems to be a good alternative to sedation. This method is widely accepted by parents and permits earlier diagnosis of hearing impairment in the routine clinical setting.

[Test set for the evaluation of hearing and speech development after cochlear implantation in children]. / Evaluationsset zur Hör-/Sprachentwicklung nach Cochlea-Implantation bei Kindern.

BACKGROUND: Since autumn 1998 the multicenter interdisciplinary study group "Test Materials for CI Children" has been compiling a uniform examination tool for evaluation of speech and hearing development after cochlear implantation in childhood. METHODS USED: After studying the relevant literature, suitable materials were checked for practical applicability, modified and provided with criteria for execution and break-off. For data acquisition, observation forms for preparation of a PC-version were developed. RESULTS: The evaluation set contains forms for master data with supplements relating to postoperative processes. The hearing tests check supra-threshold hearing with loudness scaling for children, speech comprehension in silence (Mainz and Göttingen Test for Speech Comprehension in Childhood) and phonemic differentiation (Oldenburg Rhyme Test for Children), the central auditory processes of detection, discrimination, identification and recognition (modification of the "Frankfurt Functional Hearing Test for Children") and audiovisual speech perception (Open Paragraph Tracking, Kiel Speech Track Program). The materials for speech and language development comprise phonetics-phonology, lexicon and semantics (LOGO Pronunciation Test), syntax and morphology (analysis of spontaneous speech), language comprehension (Reynell Scales), communication and pragmatics (observation forms). The MAIS and MUSS modified questionnaires are integrated. CONCLUSIONS: The evaluation set serves quality assurance and permits factor analysis as well as controls for regularity through the multicenter comparison of long-term developmental trends after cochlear implantation.