2 research outputs found
Real world music object recognition
We present solutions to two of the most pressing issues in contemporary optical music recognition (OMR).We improve recognition accuracy on low-quality, real-world (i.e. containing ageing, lighting, or dirt artefacts among others) input data and provide confidence-rated model outputs to enable efficient human post-processing. Specifically, we present (i) a sophisticated input augmentation scheme that can reduce the gap between sanitised benchmarks and realistic tasks through a combination of synthetic data and noisy perturbations of real-world documents; (ii) an adversarial discriminative domain adaptation method that can be employed to improve the performance of OMR systems on low-quality data; (iii) a combination of model ensembles and prediction fusion, which generates trustworthy confidence ratings for each prediction. We evaluate our contributions on a newly created test set consisting of manually annotated pages of varying real-world quality, sourced from International Music Score Library Project (IMSLP) / the Petrucci Music Library. With the presented data augmentation scheme, we achieve a doubling in detection performance from 36.0% to 73.3% on noisy real-world data compared to state-of-the-art training. This result is then combined with robust confidence ratings paving the way forOMR to be deployed in the realworld. Additionally, we showthe merits of unsupervised adversarial domain adaptation for OMR raising the 36.0% baseline to 48.9%. All our code and data are freely available at: https://github.com/raember/s2anet/tree/TISMIR_publication
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Efficacy and Safety of N-Acetyl-L-Leucine in Children and Adults With GM2 Gangliosidoses.
BACKGROUND AND OBJECTIVE
GM2 gangliosidoses (Tay-Sachs and Sandhoff diseases) are rare, autosomal-recessive, neurodegenerative diseases with no available symptomatic or disease modifying treatments. This clinical trial investigated N-acetyl-L-leucine (NALL), an orally administered, modified amino acid in pediatric (≥ 6 years) and adult patients with GM2 gangliosidoses.
METHODS
In this Phase IIb, multi-national, open-label, rater-blinded study (IB1001-202), male and female patients aged ≥6 years with a genetically confirmed diagnosis of GM2 gangliosidoses received orally-administered NALL for a 6-week treatment period (4 g/day in patients ≥13 years, weight-tiered doses for patients 6-12 years), followed by a 6-week post-treatment washout period. For the primary Clinical Impression of Change in Severity analysis, patient performance on a pre-determined primary anchor test (the 8-Meter Walk Test or the 9-Hole Peg Test) at baseline, after 6 weeks on NALL, and again after a 6-week washout period, was videoed and evaluated centrally by blinded raters. Secondary outcomes included assessments of ataxia, clinical global impression, and quality of life.
RESULTS
30 patients between the age of 6 and 55 were enrolled. 29 had an on-treatment assessment and were included in the primary modified intention-to-treat analysis. The study met its CI-CS primary endpoint (mean difference 0.71, SD=2.09, 90% CI 0.00, 1.50, p=0.039), as well as secondary measures of ataxia and global impression. NALL was safe and well-tolerated, with no serious adverse reactions.
CONCLUSIONS
Treatment with NALL was associated with statistically significant and clinically-relevant changes in functioning and quality of life in patients with GM2 gangliosidosis. NALL was safe and well-tolerated, contributing to an overall favourable risk: benefit profile. NALL is a promising, easily administered (oral) therapeutic option for these rare, debilitating diseases with immense unmet medical needs.
CLASSIFICATION OF EVIDENCE
This study provides Class IV evidence that NALL improves outcomes for patients with GM2 gangliosidoses.
TRIAL REGISTRATION INFORMATION
The trial is registered with ClinicalTrials.gov (NCT03759665; registered 30-Nov-2018), EudraCT (2018-004406-25), and DRKS (DRKS00017539). The first patient was enrolled 07-June-2019