Modelling costs and outcomes of newborn hearing screening: The economic part of a German health technology assessment project

The prevalence of newborn hearing disorders is 1-3 per 1000. Crucial for later outcome are correct diagnosis and effective treatment in the first year of life. With BERA and TEOAE low-risk techniques for early detection are available. Universal screening is recommended but not realised in most European health care systems. Objective of the study was to examine the scientific evidence of newborn hearing screening, thus to compare cost-effectiveness of different programmes, differentiated by type of strategy (risk screening, universal screening, no screening). Methods: In an interdisciplinary health technology assessment project all relevant studies on newborn hearing screening were identified and data on medical outcome, costs and cost-effectiveness extracted. A Markov model was designed to calculate cost-effectiveness ratios. Results: Economic data were extracted from 20 relevant publications. In the model total costs for screening of 100.000 newborns with a time horizon of ten years were calculated: 2.0 Mio . for universal screening (U), 1.0 Mio. for risk screening (R) and 0.6 Mio. for no screening (N). The costs per child detected: 13,395 (U) respectively 6,715 (R) and 4,125 (N). Conclusions: A remarkable small number of economic publications mainly of low methodo-logical quality was found. In our own model we found reasonable cost-effectiveness ratios also for universal screening. Considering the outcome advantages of higher numbers of cases detected a universal newborn hearing screening is recommended. --

Tomographic assessment of the spine in children with spondylocostal dysotosis syndrome

OBJECTIVE: The aim of this study was to perform a detailed tomographic analysis of the skull base, craniocervical junction, and the entire spine in seven patients with spondylocostal dysostosis syndrome. METHOD: Detailed scanning images have been organized in accordance with the most prominent clinical pathology. The reasons behind plagiocephaly, torticollis, short immobile neck, scoliosis and rigid back have been detected. Radiographic documentation was insufficient modality. RESULTS: Detailed computed tomography scans provided excellent delineation of the osseous abnormality pattern in our patients. CONCLUSION: This article throws light on the most serious osseous manifestations of spondylocostal dysostosissyndrome

Distinctive spinal changes in two patients with unusual forms of autosomal dominant endosteal hyperostosis: a case series

Endosteal hyperostosis was encountered in a 26-year-old-man and his 6-month-old daughter. Both the father and his daughter presented with fractures. Odontoid process hyperplasia, and progressive sclerosis of the posterior spinal elements, was the other significant features. To the best of our knowledge, this is the first clinical report describing distinctive spinal changes in association with fractures and endosteal hyperostosis

Cervico-thoracic kyphosis in a girl with Pierre Robin sequence

Congenital cervico-thoracic kyphosis has been encountered in a girl with Pierre Robin sequence. The constellation of the spine malformation complex such as incomplete development of the vertebral bodies associated with defective ossification of the cervico-thoracic pedicles causing effectively the development of complete spinal cord injury at the kyphotic level of C7/T1 were present. Congenital kyphosis secondary to vertebral body hypoplasia has not been reported in connection with Pierre Robin sequence

Mid-diaphyseal Endosteal Thickening With Subsequent Medullary Narrowing in a Patient With Hallermann-Streiff Syndrome

We report on a 5-year-old girl who presented with the full clinical criteria of Hallermann-Streiff syndrome (HSS). Classically, overtubulation (thin and gracile) bones are the characteristic and constant features in HSS. Interestingly, our present patient manifested unusual mid-diaphyseal endosteal thickening with subsequent medullary narrowing (defective endosteal resorption). To the best of our knowledge no previous reports described such unusual feature in a patient with HSS