Union of the European Phoniatricians' position statement on the exit strategy of phoniatric and laryngological services: staying safe and getting back to normal after the peak of coronavirus disease 2019 (issued on 25th May 2020).

BACKGROUND: The following position statement from the Union of the European Phoniatricians, updated on 25th May 2020 (superseding the previous statement issued on 21st April 2020), contains a series of recommendations for phoniatricians and ENT surgeons who provide and/or run voice, swallowing, speech and language, or paediatric audiology services. OBJECTIVES: This material specifically aims to inform clinical practices in countries where clinics and operating theatres are reopening for elective work. It endeavours to present a current European view in relation to common procedures, many of which fall under the aegis of aerosol generating procedures. CONCLUSION: As evidence continues to build, some of the recommended practices will undoubtedly evolve, but it is hoped that the updated position statement will offer clinicians precepts on safe clinical practice.

[Guideline: Auditory processing and perception disorders: Proposal for treatment and management of APD : S1 guideline of the German Society of Phoniatrics and Pediatric Audiology]. / Leitlinie "Auditive Verarbeitungs- und Wahrnehmungsstörungen": Vorschlag für Behandlung und Management bei AVWS : S1-Leitlinie der Deutschen Gesellschaft für Phoniatrie und Pädaudiologie.

Despite normal hearing thresholds in pure-tone audiometry, 0.5-1% of children have difficulty understanding what they hear. An auditory processing disorder (APD) can be assumed, which should be clarified and treated. In patients with hearing loss, this must first be compensated or resolved. Only hereafter can a suspected APD be confirmed or excluded. Diagnosis of APD requires that a clear discrepancy between the child's performance in individual auditory functions and other cognitive abilities be demonstrated. Combination of therapeutical modalities is considered particularly more beneficial in APD patients than a single modality. Treatment modalities should consider linguistic and cognitive processes (top-down), e.g., metacognitive knowledge of learning strategies or vocabulary expansion, but also address underlying auditory deficits (bottom-up). Almost 50% of children with APD also have a language development disorder requiring treatment and/or dyslexia. Therefore, each therapeutic intervention for a child with APD must be individually adapted according to the diagnosed impairments. Musical training can improve phonologic and reading abilities. Changes and adaptations in the classroom are helpful to support the weak auditory system of children with APD. Architectural planning of classrooms can be a means of ensuring that direct sound is masked by as little diffuse sound as possible. For example, acoustic ceiling tiles are suitable for reducing reverberant and diffuse sound.

[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.

[Muenster Parental Programme--Feedback from Parents: How do parents evaluate an early intervention programme for improving the communication with their baby or toddler with hearing impairment?]. / Münsteraner Elternprogramm--Elternfeedback: Wie beurteilen Eltern die Frühintervention zur Kommunikationsförderung von Säuglingen und Kleinkindern mit Hörschädigung?

BACKGROUND: With the implementation of the UNHS, early educational services' existing concepts of early intervention have to be adapted to the situation and needs of families with a preverbal child who is deaf or hard of hearing. The Muenster Parental Programme (MPP), a module in early family-centered intervention, fulfils this requirement. OBJECTIVE: We report feedback from participating parents regarding processes and outcomes of the MPP. The self-developed questionnaire was checked for its suitability as feedback instrument for measuring parental satisfaction with the MPP. METHOD: 29 parents who participated in the MPP assessed the programme by using the standardised FBB and the self-developed questionnaire FB-MEP, which is specific to the MPP. RESULTS: Using the FBB, 96% of parents judged the MPP to be good or very good. With the FB-MEP, parents rated setting, contents and didactics as highly as they did using the FBB (r = 0.7, p < 0.01). In particular, parents judged both the contact and exchange with other affected parents, and the specific individual support for communicating with their child (including video feedback) as especially helpful. CONCLUSIONS: The results reflect parents' high level of satisfaction with the setting, content, didactics and individual benefit gained by their child and themselves from the MPP. The parents are aware of the efficacy of the MPP, which was shown in the controlled intervention study. The self-developed questionnaire FB-MEP was shown to be a suitable instrument for quality assurance measurements of the MPP.

[Auditory processing and perception disorders (APPD): summary and updated overview]. / Auditive Verarbeitungs- und Wahrnehmungsstörungen (AVWS): Zusammenfassung und aktualisierter Überblick.

The APD guideline of 2009 was supplemented by the statements listed here. The addition is based on current knowledge and findings. Otherwise, the Guideline 2009 remains valid. Here, a summary of the updated APD guideline is given, thus proving an overview of the definition of APD, diagnosis, differential diagnosis and recommended for APD management.

[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.

[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.

[Nonorganic (functional) hearing loss in children]. / Nichtorganische (funktionelle) Hörstörungen bei Kindern.

Nonorganic (functional) hearing loss in children is characterized by hearing loss without a detectable corresponding pathology in the auditory system. It is not an uncommon disease in childhood. Typically, there is a discrepancy between elevated pure tone thresholds and normal speech discrimination in everyday life. We evaluated 85 original publications, 27 reviews and 4 textbook articles. Mean age at diagnosis was 11.3 years. Girls were affected twice as often as boys. Patient histories showed a high prevalence of emotional and school problems. Pre-existing organic hearing loss can be worsened by nonorganic causes. A brainstem audiometry should confirm the diagnosis. The differential diagnosis includes auditory processing disorder, elevated thresholds in mental retardation and auditory neuropathy. We recommend taking a personal history including biographical factors, a psychological assessment including intelligence testing and referral to a child psychiatrist. Prognosis seems to be dependent on the severity of the patient's school and/or personal problems. Categorization following the Austen-Lynch model can be a valuable prognostic factor.

[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.

[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.

[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.

[A new gene locus for an autosomal-dominant non-syndromic hearing impairment (DFNA 33) is situated on chromosome 13q34-qter]. / Ein neuer Genort für eine autosomal-dominante, nichtsyndromale Schwerhörigkeit (DFNA33) liegt auf Chromosom 13q34-qter.

By investigation of a German family pedigree with non-syndromic hearing impairment of early onset and autosomal-dominant mode of inheritance, linkage to known DFNA loci was excluded, and the existence of a new locus (DFNA33) was revealed. In a subsequent genomic scan the phenotype was mapped to a 6 cM interval on chromosome 13q34-qter. A maximum two-point lod score of 2.96 was obtained for the marker D13S285 with a maximum lod score in the multipoint analysis of 3.28 at 124.56 cM.

Megalin genetic polymorphisms and individual sensitivity to the ototoxic effect of cisplatin.

Ototoxicity and nephrotoxicity are dose-limiting side effects of cisplatin. Megalin, a member of the low-density lipoprotein receptor family, is highly expressed in renal proximal tubular cells and marginal cells of the stria vascularis of the inner ear - tissues, which accumulate high levels of platinum-DNA adducts. On the assumption that the mechanisms of cisplatin-induced nephro- and ototoxicity involve megalin we analyzed the incidence of the non-synonymous single nucleotide polymorphisms (SNP) rs2075252 and rs4668123 in 25 patients who developed a distinct hearing loss during cisplatin therapy and in 25 patients without hearing impairment after cisplatin therapy. We found no association between cisplatin-induced ototoxicity and any allele of rs4668123 but observed a higher frequency of the A-allele of rs2075252 in the group with hearing impairment than in the group with normal hearing after cisplatin therapy (0.32 versus 0.14) (chi(2)=5.83, P<0.02; odds ratio: 3.45; 95% confidence interval: 1.11-11.2) indicating that SNPs at the megalin gene might impact the individual susceptibility against cisplatin-induced ototoxicity.

[A new locus for an autosomal dominant, non-syndromic hearing impairment (DFNA57) located on chromosome 19p13.2 and overlapping with DFNB15]. / Ein neuer Genort für eine autosomal dominante, nichtsyndromale Hörstörung (DFNA57) kartiert auf Chromosom 19p13.2 und überlappt mit DFNB15.

BACKGROUND: Non-syndromic hearing loss is the most genetically heterogeneous trait known in humans. To date, 54 loci for autosomal dominant non-syndromic sensorineural hearing loss (NSSHL) have been identified by linkage analysis. METHODS: In this study a German pedigree has been identified segregating a progressive bilateral loss of lower and middle frequencies. RESULTS: A genome-wide screening and linkage analysis revealed the existence of a new NSSHL locus (DFNA57). The phenotype was mapped to a 10 degrees Mbp interval on chromosome 19p13.2 from 7.8 to 18.2 degrees Mbp, a maximum 2-point LOD score of 3.08 was obtained for the marker D19S586. The region overlaps with the recessive locus DFNB15. CONCLUSION: The results underline the heterogeneity of hereditary hearing disorders. Identification of genes can help to reach a better understanding of the molecular mechanism of hearing.