[Vibrant Soundbridge®: An Alternative Hearing System for Preschool Children with Aural Atresia]. / Die Vibrant Soundbridge®: Ein alternatives Hörsystem bei Vorschulkindern mit Gehörgangsatresie.

BACKGROUND: The audiological treatment of children with aural atresia makes high demands on physicians and acousticians. Conventional hearing systems are often not tolerated by children and therefore do not meet the needs of the early and efficient therapy of hearing disorders. Aim of the present study was the evaluation of the audiological functional gain in children with uni- and bilateral aural atresia provided with the middle ear implant Vibrant Soundbridge(®) (VSB(®)) below the age of 6 years as well as the analysis of parents' satisfaction assessed with questionnaires. MATERIAL AND METHODS: The VSB(®) was implanted in 16 children, 13 with unilateral and 3 with bilateral aural atresia, with the mean age of 2;11±1;6 years. 3 months after the first fitting of the audio processor, pure-tone audiometry via free field testing with and without the hearing system was performed. Furthermore, parents completed a standardized questionnaire to evaluate their satisfaction with the VSB(®) treatment quality. The questionnaire included items on the acceptance by children, handling, listening effort, behavior, satisfaction, quality of life, aesthetics, and the length of daily use. RESULTS: The use of the VSB(®) resulted in a significantly improved hearing level: 20 dB on average (Z=- 3.06; p=0.002; n=12). The analysis of parents' questionnaire demonstrated high or very high satisfaction with VSB(®) in all subjects. Primarily, the length of daily use of the VSB(®) was significantly higher than that of the hearing system used before with 10.0±2.1 vs. 2.7±2.2 h per day (Z=- 3.06; p=0.002; n=14). CONCLUSION: The VSB(®) presented a good alternative for audiological treatment of uni- and bilateral aural atresia at toddler and pre-school age.

[Validation of the German short version of the Murray Secretion Rating Scale]. / Validierung der deutschen Kurzversion der Sekretbeurteilungsskala nach Murray.

OBJECTIVE: Accumulation of secretions in hypopharynx, aditus vestibule, and trachea is often found in cases of severe dysphagia and is considered a cardinal trait of high clinical and therapeutic importance. For the graduation of the severity level of accumulated secretions, a short version of the 4-point Murray secretion scale is available, which is also integrated into the protocol of the fiberoptic endoscopic evaluation of swallowing (FEES) according to the Langmore standard. This study aimed at the validation of the German translation of this short version in order to facilitate a uniform, standardized evaluation of the accumulation of secretions in dysphagic patients in the German language area. MATERIAL AND METHODS: For the examination of reliability and validity, a reference standard was defined by 2 dysphagia experts on the basis of 40 video files of the FEES examination, 10 videos for each of the severity grades. Afterwards, these videos were rated independently by 4 raters and re-rated in a new randomized order 2 weeks later. RESULTS: Both the intra-rater reliability (τ>0,830***) and the inter-rater reliability (Kendalls W>0,890***) were highly significant and can be considered good. The same is valid for the correlation of ratings with the reference standard (τ=0,969***). CONCLUSIONS: The German translation of the short version of the 4-point Murray secretion scale is recommendable as a reliable and valid instrument for the graduation of the cardinal trait of oropharyngeal dysphagia and also as an evidence-based instrument for standardized use in the German language area.

[Quality Assurance in the Endoscopic Evaluation of Swallowing (FEES)]. / Qualitätssicherung der endoskopischen Schluckdiagnostik (FEES).

BACKGROUND: A fiberoptic endoscopic evaluation of swallowing (FEES) is well established internationally as gold standard of the instrument-based diagnostics if it is performed according to the Langmore-protocol. For the quality assurance of FEES, videotaping is recommended. However, often no videotaping is carried out due to a limited availability of portable recording systems, for instance, in the bedside diagnostics. This study aimed at the comparison of FEES quality assurance with and without videotaping by rating of the main finding in swallowing disorders, the penetration and aspiration, as defined in the penetration-aspiration scale by Rosenbek. MATERIAL AND METHODS: An examination of agreement of penetration and aspiration ratings with the defined reference standard was conducted by means of 80 videotaped recordings. The ratings were carried out independently by 4 raters in 2 settings: A) "real time" and B) "frame-by-frame". RESULTS: As far as the interrater- and intrarater-reliability is concerned, the association between the ratings in the rating setting B were higher than those in the rating setting A. Furthermore, examination of the validity showed higher correlation in setting B than in setting A. The difference between correlations was significant in favor of setting B (videotaped FEES). CONCLUSION: The results of the present study, namely better rating reliability and validity in case of the penetration and aspiration, emphasize the importance of the videotaping of the FEES diagnostics, at least for the evaluation of penetration or aspiration.

[Secretion scale by Murray et al. for FEES®: comparison of reliability and validity of the German long and short version]. / Sekretbeurteilungsskala nach Murray et al. für FEES(®) : Reliabilitäts- und Validitätsvergleich der deutschen Lang- und Kurzversion.

BACKGROUND: Accumulation of secretions in the hypopharynx, aditus laryngis and trachea constitute a cardinal trait of oropharyngeal dysphagia. For the evaluation of the degree of severity a 4-point secretion scale by Murray et al. is used internationally in a long and a short version. However, a validated German translation of the long version of this scale does not yet exist. Also, it has not yet been scientifically proven that both versions of the scale are equally valid. OBJECTIVES: This study aimed at the validation of the German translation of the long version of the secretion scale by Murray et al. and at a comparison of reliability and validity of the short and long versions. MATERIAL AND METHODS: A total of 40 videos of fiberoptic endoscopic evaluation of swallowing (FEES(®)), 10 for each severity level, were rated by 4 otorhinolaryngologists (ENT specialists) independently and with different randomizations for examination of the reliability and validity. Two rating sessions for each of the scale versions were conducted. Intrarater and interrater reliability as well as the agreement of the ratings with a reference standard were analyzed. RESULTS: Both the intrarater reliability (Spearman correlations: ρs > 0.840***) and the interrater reliability (Krippendorff's alpha: α > 0.850) yielded very good results and the concurrent validity was highly significant (ρs > 0.981***). DISCUSSION: The German translation of the secretion scale by Murray et al. can be considered reliable and valid, with comparable test accuracy of the short and long versions. Hence, the scale can be recommend for the graduation of pharyngolaryngotracheal secretions and should be integrated into the standardized evaluation of FEES(®) diagnostics for clinical and scientific purposes.

[Penetration-Aspiration Scale according to Rosenbek. Validation of the German version for endoscopic dysphagia diagnostics]. / Penetrations-Aspirations-Skala nach Rosenbek. Validierung der deutschen Version für die endoskopische Dysphagiediagnostik.

BACKGROUND: The Penetration-Aspiration Scale was developed by Rosenbek et al. to enable standardized documentation of this cardinal symptom of a swallowing disorder. OBJECTIVES: The objective was to create and validate a German version of the Penetration-Aspiration Scale according to the guidelines governing the translation of foreign language measurement tools. MATERIALS AND METHODS: Both reliability and validity were examined based on the ratings of 80 randomized endoscopically evaluated swallows, 10 for each severity level. Ratings were carried out by four independent raters: two with more than 3 years' experience with dysphagia and a further two with less than 3 years' experience. The swallows were rerated after 4 weeks. RESULTS: Both intrarater (Kendall's Tau: τs > 0.643; median 0.773; ps < 0.001) and interrater reliability were highly significant (two-way mixed single ICC coefficient of 0.799 for the first rating session and 0.728 for the second session; ps < 0.001). Results from the raters with less than 3 years' experience were significantly different from the reference standard in three out of four cases, whereas this was not the case for the more experienced raters. However, for each film, the median of the individual ratings from all four raters correlated almost perfectly with the reference standard (first rating session: τ = 0.894; second rating session: τ = 0.843; ps < 0.001). CONCLUSION: The German version of the Penetration-Aspiration Scale according to Rosenbek presented here was demonstrated to be both reliable and valid. Despite its dependency on the raters' experience, it can therefore be used as a graduation instrument for swallowing disorders in German-speaking countries and make an important contribution to evidence-based medicine in dysphagiology for both clinical and scientific use.

[Fool's gold standards in language screening. Sensitivity and specificity of the Hessian child language screening test (Kindersprachscreening, KiSS)]. / Katzengoldstandards in der Sprachstandserfassung. Sensitivität und Spezifität des Kindersprachscreenings (KiSS).

BACKGROUND: The German Kindersprachscreening (KiSS) is a universal speech and language screening test for large-scale identification of Hessian kindergarten children requiring special educational language training or clinical speech/language therapy. PARTICIPANTS AND METHODS: To calculate the procedural screening validity, 257 children (aged 4.0 to 4.5 years) were tested using KiSS and four language tests (Reynell Development Language Scales III, Patholinguistische Diagnostik, PLAKSS, AWST-R). The majority or consensus judgements of three speech-language professionals, based on the language test results, served as a reference criterion. The base (fail) rates of the professionals were either self-determined or preset based on known prevalence rates. RESULTS: Screening validity was higher for preset than for self-determined base rates due to higher inter-judge agreement. The confusion matrices of the overall index classification of the KiSS (speech-language abnormalities with educational or clinical needs) with the fixed base rate expert judgement about language impairment, including fluency or voice disorders, yielded a sensitivity of 88% and a specificity of 78%, for just language impairment 84% and 75%, respectively. Specificities for disorders requiring clinical diagnostics in the KiSS (language impairment alone or combined with fluency/voice disorders) related to the test-based consensus expert judgment was about 93%. Sensitivities were unsatisfactory because the differentiation between educational and clinical needs requires improvement. CONCLUSION: Since the judgement concordances between the speech-language professionals was only moderate, the development of a comprehensive German reference test for speech and language disorders with evidence-based algorithmic decision rules rather than subjective clinical judgement is advocated.

[Validity criteria of a short test to assess speech and language competence in 4-year-olds]. / Psychometrische Gütekriterien eines Kurztests zur Erfassung des Sprachstands 4-jähriger Kinder.

BACKGROUND: A psychometrically constructed short test as a prerequisite for screening was developed on the basis of a revision of the Marburger Speech Screening to assess speech/language competence among children in Hessen (Germany). PARTICIPANTS AND METHODS: A total of 257 children (age 4.0 to 4.5 years) performed the test battery for speech/language competence; 214 children repeated the test 1 year later. RESULTS: Test scores correlated highly with scores of two competing language screenings (SSV, HASE) and with a combined score from four diagnostic tests of individual speech/language competences (Reynell III, patholinguistic diagnostics in impaired language development, PLAKSS, AWST-R). Validity was demonstrated by three comparisons: (1) Children with German family language had higher scores than children with another language. (2) The 3-month-older children achieved higher scores than younger children. (3) The difference between the children with German family language and those with another language was higher for the 3-month-older than for the younger children. CONCLUSION: The short test assesses the speech/language competence of 4-year-olds quickly, validly, and comprehensively.

Long-term follow-up of allogeneic bone marrow transplantation for patients with chronic phase chronic myeloid leukemia prepared with a regimen consisting of cyclophosphamide, cytarabine and single-dose total body irradiation conditioning.

We evaluated long-term toxicities and outcomes in 96 patients with chronic phase chronic myeloid leukemia treated with a single bone marrow allograft regimen. Conditioning was cytosine arabinoside, cyclophosphamide (120 mg/kg) and single fraction total body irradiation (500 cGy). Median follow-up was 12.8 years (0.4-19.9 years). Graft failure occurred in one patient, nonfatal veno-occlusive disease in 13 patients (14%). Overall incidences of acute (a) and chronic (c) graft-vs-host disease (GVHD) were 77 and 63%. The 100-day and 1-year transplant-related mortality (TRM) were 1 and 9.2%, respectively, with no change through 5 years. Five- and 10-year event-free survival rates were 56 and 49%, overall survival (OS) rates 72 and 70%, respectively. Forty patients have relapsed: 8 cytogenetic (20%), 10 hematologic (25%) and 22 molecular (55%). Most have been salvaged with donor-leukocyte infusion, second transplants and/or imatinib therapy. Survival was worse for patients transplanted >2 years from diagnosis (10-year OS 56 vs 78%, P=0.01), for patients over 50 years old (10-year OS 44 vs 75%, P=0.05) and for patients without cGVHD (10-year OS 53 vs 86%, P<0.001). This regimen resulted in successful engraftment, low risk of TRM and long-term survival. In an era when imatinib is first line therapy, this regimen offers a potentially low-toxicity, highly successful alternative in the event of poor imatinib response.