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• A novel Wheelchair Repair Registry has been systematically developed. • The registry contains repairs from more than 5000 devices and continues to be populated. • Registry data can support research on the reliability of wheeled mobility devices. To describe the development of a wheelchair repair registry from large datasets to attain an understanding of wheelchair failures and service repairs. Guidelines for registry development were applied and anchored around Labor-Tracker, a web-based information management system for wheelchair suppliers to manage and track wheelchair repairs. The registry was designed using online analytical processing, allowing for rapid data queries from multiple dimensions that enable complex data analysis and discovery. The Wheelchair Repair Registry (WRR) was developed through an industry and academic collaboration whereby repair data were collected in the field, entered into the Labor-Tracker system, deidentified, and then transferred to the registry and made available for analyses. Wheelchair supplier service technicians reported data from repair services provided to individuals who use power wheelchairs, manual wheelchairs, and scooters. Not applicable. Wheelchair failure and repair data, including variables related to scheduling, equipment information (eg, manufacturer, model, serial number, purchase date), labor, parts, and reasons for repairs. The WRR was developed to analyze wheelchair repairs and failures from the Labor-Tracker system. Currently, the registry has more than 60,000 repairs conducted on more than 5000 wheelchair devices from 25 manufacturers. The devices include 60% power wheelchairs, 35% manual wheelchairs, and 5% scooters. The WWR creates opportunities to apply large-data analytical methodologies that will serve to inform quality standards, practice, equipment selection, preventative maintenance routines, product design, and policy. [ABSTRACT FROM AUTHOR]

Abstract: The hearing status of an experimental animal is typically assessed in the laboratory setting by the combined use of auditory brainstem response (ABR) and distortion product otoacoustic emissions (DPOAEs), carried out in succession, with the former assay preceding the latter. This study reports a cautionary finding that the use of this accepted regimen yields a reduced DPOAE response. When the DPOAEs were performed after ABR testing, transient reduction of the DPOAE amplitudes was observed at all frequencies in both the inbred, C57/B6 and FVB/N, and the outbred, SW mouse strains. DPOAEs were reduced post-ABR in multiple mouse strains which suggests that this finding is not strain-specific but a general consequence of the preceding ABR analysis. The reduction in DPOAE was temporary: when re-tested at one hour, DPOAE amplitudes recovered to pre-ABR levels. In contrast to the ABR’s impact on DPOAE response, ABR thresholds were not altered or reduced when preceded immediately by DPOAE measurements. The molecular alterations underlying the ABR-induced transient reduction of DPOAE remain to be determined. To investigate the potential role of reactive oxygen species in post-ABR DPOAE reduction, transgenic mice over-expressing SOD1, the cytoplasmic enzyme critical for removal of superoxide radicals were subjected to the same auditory testing regimen. Similar to their wild type littermates, the SOD1 transgenic mice also demonstrated post-ABR DPOAE reduction, and thus do not support a role for superoxide radicals in transient reduction of DPOAE. While toxic noise exposure is known to negatively impact OAE, transient decrease in DPOAE levels following standard ABR assay has not been previously described. A practical outcome from this study is a recommendation for reversal of the traditional order for carrying out auditory tests, with the OAE measurements preceding ABR assessment, thus ensuring that the DPOAE response is unaffected. [Copyright &y& Elsevier]

Abstract: A kindred with inherited macrothrombocytopenia (MTCP) and sensorineural hearing loss (SNHL) from Ghent, Belgium was identified. Currently, joint expression of MTCP and hearing loss are linked to mutations within MYH9 only. Thus, we tested the hypothesis that a mutation within MYH9 is responsible for the autosomal dominant inheritance of MTCP and hearing loss in the Ghent family. A mutation screen of MYH9 coding region including its intron–exon junctions, as well as common hearing loss genes GJB2, GJB3, and GJB6, was performed. However, no pathogenic sequence alteration was identified. Patients'' leukocytes were determined to be normal for NMMHC-A distribution via immunofluorescence analysis and free of Döhle body-like inclusions, identified as aggregates of mutant NMHC-IIA in MYH9 disorders. Also, western blot analysis with anti-NMHC-IIA antibody identified a single 220 kDa immunoreactive band with normal expression level of NMHC-IIA within the platelets and leukocytes of the affected family members. The immunoblot analysis eliminates the possibility of a large deletion within MYH9 that can escape detection by direct sequencing. Collectively, these results suggest that molecular genetic etiology of the Ghent family disorder may be due to as yet unidentified gene whose mutation(s) yields a phenocopy of the MYH9-related disease. [Copyright &y& Elsevier]

The expression pattern of aquaporin-2 (AQP2), a vasopressin regulated member of the aquaporin gene family, in the cochlea and its potential role in Meniere’s disease was investigated. RT-PCR screen of multiple rat tissues identified AQP2 transcripts in the cochlea, testis and kidney and an absence of tissue-specific splice variants. The level of AQP2 transcript in the cochlea was 10-fold lower relative to its expression in the testis and kidney. Western blot analysis demonstrated a single, 29 kDa band in the membrane fractions from cochlea, testis and the kidney. In the rat and mouse cochlea, AQP2 was expressed in the structures bordering the endolymph, including Reissner’s membrane, the organ of Corti, inner and outer sulcus cells and the spiral limbus. A mutation screen of AQP2 in 12 individuals with Meniere’s disease did not identify any sequence alterations or mutations within the four coding exons of AQP2 and their intron–exon junctions. The physiological role of AQP2 in water transport and its expression pattern in the cochlea suggests an important role for AQP2 in fluid homeostasis of the inner ear; however, its role in the pathogenesis in Meniere’s disease remains to be established. [Copyright &y& Elsevier]

Abstract: NMHC-IIa, a nonmuscle myosin heavy chain isoform encoded by MYH9, is expressed in sensory hair cells and its dysfunction is associated with syndromic and nonsyndromic hearing loss. In this study, we investigate the ultrastructural distribution of NMHC-IIa within murine hair cells to elucidate its potential role in hair cell function. Using previously characterized anti-mouse NMHC-IIa antibody detected with immunogold labelling, NMHC-IIa was observed in the stereocilia, in the cytosol along the plasma membrane, and within mitochondria. Within stereocilia, presence of NMHC-IIa is observed throughout its length along the actin core, from the center to the periphery and at its base in the cuticular plate, suggesting a potential role in structural support. Within the sensory hair cells, NMHC-IIa was distributed throughout the cytoplasm and along the plasma membrane. A novel finding of this study is the localization of NMHC-IIa within the mitochondria, with the majority of the label along its inner membrane folds. The presence of NMHC-IIa within heterogeneous areas of the hair cell suggests that it may play different functional roles in these distinct regions. Thus, mutant NMHC-IIa may cause hearing loss by affecting hair cell dysfunction through structural and or functional disruption of its stereocilia, plasma membrane, and/or mitochondria. [Copyright &y& Elsevier]

Reactive oxygen species (ROS) have been implicated in hearing loss associated with aging and noise exposure. Superoxide dismutases (SODs) form a first line of defense against damage mediated by the superoxide anion, the most common ROS. Absence of Cu/Zn SOD (SOD1) has been shown to potentiate hearing loss related to noise exposure and age. Conversely, overexpression of SOD1 may be hypothesized to afford a protection from age- and noise-related hearing loss. This hypothesis may be tested using a transgenic mouse model carrying the human SOD1 gene. Contrary to expectations, here, we report that no protection against age-related hearing loss was observed in mice up to 7 months of age or from noise-induced hearing loss when 8 week old mice were exposed to broadband noise (4–45 kHz, 110 dB for 1 h). Mitochondrial DNA deletion, an index of aging, was elevated in the acoustic nerve of transgenic mice compared to nontransgenic littermates. The results indicate the complexity of oxidative metabolism in the cochlea is greater than previously hypothesized. [Copyright &y& Elsevier]