Beidseitige Ertaubung durch Labyrinthitis bei M.Crohn

Zusammenfassung: Vestibulocochleäre Störungen bei chronischen Darmentzündungen sind seltene, aber immer wieder beschriebene Komplikationen oder Manifestationen der Grunderkrankung. Ein 26-jähriger Mann erlitt unter immunsuppressiver Therapie aufgrund eines 6Monate zuvor diagnostizierten M.Crohn einen bilateralen Ausfall der Gleichgewichtsorgane sowie eine bilaterale sensorineurale Hörminderung. Es wurde eine ausführliche Diagnostik (Audiogramm, Sprachaudiogramm, kalorische Vestibularisprüfung, Messung vestibulär evozierter myogener Potenziale, Magnetresonanztomographie) durchgeführt. Therapiert wurde mit Azathioprim, Glukokortikoiden systemisch und intratympanal sowie mit monoklonalen Antikörpern. Darunter zeigte sich ein stark fluktuierender, aber progredienter Verlauf der Beschwerden und Befunde bei nur geringfügiger bis keiner Besserung. Erst die beidseitige Versorgung mit einem Cochleaimplantaten (CI) stellte eine befriedigende Lösung dar. Das Fallbeispiel zeigt diagnostische und therapeutische Möglichkeiten und Grenzen einer immunbedingten Erkrankung. Sie verlangt eine enge interdisziplinäre Zusammenarbeit. Bei beidseitiger Ertaubung ist die frühzeitige CI-Versorgung eine geeignete Form der Rehabilitatio

Evaluation of universal newborn hearing screening in Switzerland 2012 and follow-up data for Zurich

BACKGROUND: The European Consensus Statement of Neonatal Hearing recommended universal newborn hearing screening (UNHS) in 1998. UNHS was introduced in Switzerland in 1999 under the auspices of a "Swiss Working Group Hearing Screening in Newborns". The aim of this study was to evaluate the number of newborns being screened and consequently followed-up in Switzerland for the year 2012. METHODS: Postal questionnaires were sent to all registered maternity clinics and birth-centres in Switzerland. To evaluate follow-up of newborns failing the screening process, a retrospective consecutive cohort analysis of newborns failing screening at the University Hospital Zurich between 2005 and 2010 was performed. RESULTS: A total of 102/110 (92.7%) maternity clinics and 1/14 (7.1%) birth-centres routinely performed UNHS. When weighted according to the number of births in the varying locations, 97.9% of all newborn received hearing screening. At the University Hospital of Zurich, 253/12,080 (2.1%) newborns failed the screening test and in 15/253 (6%) a relevant bilateral hearing impairment was found. This makes an overall incidence of congenitally relevant hearing loss of 0.12%. Unfortunately, 33/253 (13%) of newborns with failed screening were lost to follow-up. CONCLUSION: UNHS is well-established in Switzerland and the vast majority of newborns are screened. However, follow-up of failed screens is disappointing. Further measures need to be taken to improve follow up

Age Dependent Cost-Effectiveness of Cochlear Implantation in Adults: Is There an Age Related Cut-off?

Objective: To analyze the impact of age at implantation on the cost-effectiveness of cochlear implantation (CI). Study Design: Cost-utility analysis in an adapted Markov model. Setting: Adults with profound postlingual hearing loss in a “high income” country. Intervention: Unilateral and sequential CI were compared with hearing aids (HA). Main Outcome Measure: Incremental cost-effectiveness ratio (ICER), calculated as costs per quality adjusted life year (QALY) gained (in CHF/QALY), for individual age and sex combinations in relation to two different willingness to pay thresholds. 1 CHF (Swiss franc) is equivalent to 1.01 USD. Results: When a threshold of 50,000 CHF per QALY is applied, unilateral CI in comparison to HA is cost-effective up to an age of 91 for women and 89 for men. Sequential CI in comparison to HA is cost-effective up to an age of 87 for women and 85 for men. If a more contemporary threshold of 100,000 CHF per QALY is applied, sequential CI in comparison to unilateral CI is cost-effective up to an age of 80 for women and 78 for men. Conclusions: Performing both sequential and unilateral CI is cost-effective up to very advanced ages when compared with hearing aids

Extraction of ozone and chlorophyll-A distribution from AVIRIS data

The potential of airborne imaging spectrometry for assessing and monitoring natural resources is studied. Therefore, an AVIRIS scene of the NASA's MacEurope 1991 campaign - acquired in Central Switzerland - is used. The test site consists of an urban area, the Lake Zug with its surrounding fields, the Rigi mountain in the center of the test site, and the Lake of Four Cantons. The region is covered by the AVIRIS flight #910705, run 6 and 7 of the NASA ER-2 aircraft resulting in an average nominal pixel size of about 18 m. Simultaneous to the ER-2 overflight spectroradiometric measurements have been taken in various locations. Preselected reference targets were measured in the field with a GER Mark V spectroradiometer, and radiance measurements were taken to the lake using a Li-Cor LI 1800UW specroradiometer below and above the water surface. A comprehensive meteorological data set was obtained by joining the POLLUMET experiment which carried out measurements to investigate the summer smog in Switzerland on the same day. The quality assessment for the actual data set can be found in detail in Meyer et al. A parametric approach calculating the location of the airplane was used to simulate the observation geometry. This parametric preprocessing procedure, which takes care of effects of flight line and attitude variations as well as the pixel-by-pixel topographic corrections is described in Meyer

CRIM-negative infantile Pompe disease: 42-month treatment outcome

Pompe disease is a rare lysosomal glycogen storage disorder characterized by deficiency of acid α-glucosidase enzyme (GAA) and caused by mutations in the GAA gene. Infantile-type Pompe disease is a multiorgan disorder presenting with cardiomyopathy, hypotonia, and muscular weakness, which is usually fatal. Enzyme replacement therapy (ERT) with recombinant human GAA (rhGAA) has recently been shown to be effective and subsequently yielded promising results in cross-reactive immunologic material (CRIM)-positive patients. CRIM-negative patients showed a limited response to ERT and died or were ventilator dependant. Over a period of 44 months, we monitored cognitive and motor development, behavior, auditory function, and brain imaging of a CRIM-negative infantile Pompe disease patient on rhGAA and monoclonal anti-immunoglobulin E (anti-IgE) antibody (omalizumab) treatment due to severe allergic reaction. Cardiorespiratory and skeletal muscle response was significant, with almost normal motor development. Cognitive development-in particular, speech and language-deviated increasingly from normal age-appropriate development and was markedly delayed at 44 months, unexplained by moderate sensorineural hearing impairment. Brain magnetic resonance imaging (MRI) at 18, 30, and 44 months of age revealed symmetrical signal alteration of the deep white matter. Titer values of IgG antibodies to rhGAA always remained <1:800. The potential role of omalizumab in immune modulation remains to be elucidated; however, this is the first report presenting a ventilator-free survival of a CRIM-negative patient beyond the age of 36 months. The central nervous system (CNS) findings are hypothesized to be part of a yet not fully described CNS phenotype in treated patients with longer survival

A European Perspective on Auditory Processing Disorder-Current Knowledge and Future Research Focus

Current notions of “hearing impairment,” as reflected in clinical audiological practice, do not acknowledge the needs of individuals who have normal hearing pure tone sensitivity but who experience auditory processing difficulties in everyday life that are indexed by reduced performance in other more sophisticated audiometric tests such as speech audiometry in noise or complex non-speech sound perception. This disorder, defined as “Auditory Processing Disorder” (APD) or “Central Auditory Processing Disorder” is classified in the current tenth version of the International Classification of diseases as H93.25 and in the forthcoming beta eleventh version. APDs may have detrimental effects on the affected individual, with low esteem, anxiety, and depression, and symptoms may remain into adulthood. These disorders may interfere with learning per se and with communication, social, emotional, and academic-work aspects of life. The objective of the present paper is to define a baseline European APD consensus formulated by experienced clinicians and researchers in this specific field of human auditory science. A secondary aim is to identify issues that future research needs to address in order to further clarify the nature of APD and thus assist in optimum diagnosis and evidence-based management. This European consensus presents the main symptoms, conditions, and specific medical history elements that should lead to auditory processing evaluation. Consensus on definition of the disorder, optimum diagnostic pathway, and appropriate management are highlighted alongside a perspective on future research focus

Inventory of current EU paediatric vision and hearing screening programmes

Background: We examined the diversity in paediatric vision and hearing screening programmes in Europe. Methods: Themes relevant for comparison of screening programmes were derived from literature and used to compile three questionnaires on vision, hearing and public-health screening. Tests used, professions involved, age and frequency of testing seem to influence sensitivity, specificity and costs most. Questionnaires were sent to ophthalmologists, orthoptists, otolaryngologists and audiologists involved in paediatric screening in all EU fullmember, candidate and associate states. Answers were cross-checked. Results: Thirty-nine countries participated; 35 have a vision screening programme, 33 a nation-wide neonatal hearing screening programme. Visual acuity (VA) is measured in 35 countries, in 71% more than once. First measurement of VA varies from three to seven years of age, but is usually before the age of five. At age three and four picture charts, including Lea Hyvarinen are used most, in children over four Tumbling-E and Snellen. As first hearing screening test otoacoustic emission (OAE) is used most in healthy neonates, and auditory brainstem response (ABR) in premature newborns. The majority of hearing testing programmes are staged; children are referred after one to four abnormal tests. Vision screening is performed mostly by paediatricians, ophthalmologists or nurses. Funding is mostly by health insurance or state. Coverage was reported as >95% in half of countries, but reporting was often not first-hand. Conclusion: Largest differences were found in VA charts used (12), professions involved in vision screening (10), number of hearing screening tests before referral (1-4) and funding sources (8)