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1 However, cost benefit analyses also take into account the observations that m.1555A>G may cause later onset hearing loss in the absence of aminoglycosides and that gradual hearing loss may be an inevitable consequence of the mutation.
Data from follow-up studies of newborn screening cohorts suggest that more than 20% of children progressively lose hearing of 20 dB or more and that from 15%to40%0% of children experience later onset hearing loss (Barreira-Nielsen and Fitzpatrick, Reference Note 1; Fitzpatrick, Reference Note 2; Watkin & Baldwin 2011).
It was diagnosed progressive or late onset hearing loss in children followed until the age of three with less than 28 weeks of gestational age and weighing less than 1250 g [ 35].
In recent years, experimental approaches including laboratory studies of early onset hearing loss in inbred mouse strains, or proteomic analyses of hair cells or hair bundles, have suggested new candidate molecules involved in hearing function.
Osteogenesis imperfecta (OI) is an inherited disorder of connective tissue characterized by bone fragility and fractures; other clinical features include blue sclerae, adult onset hearing loss and joint laxity. 1 The severity ranges from lethal in the perinatal period to occasional fracture.
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It decided to withdraw NHS-funded hearing aids for people with mild to moderate adult-onset hearing loss across a range of towns, until a public outcry made it put the plans on hold until after the election.
Recovery was significantly related to age, onset of hearing loss, and audiogram type; however outcome was not significantly related to gender, vertigo, tinnitus, or laterality (P < 0.05).
The present study was designed to resolve these ambiguities by tracking the hearing abilities of individual C57BL/6J mice from age 16 weeks until the onset of hearing loss in specific listening conditions.
The enlargement of the cochlea lumen begins at embryonic day (E) 14.5, which is ∼19 days before the onset of hearing at postnatal day (P) 12 [13], [14], [14].
Moreover, the data indicate that the overall cochlear gene expression profile of α9−/− mice is distinctly different from that of wild type mice at the onset of hearing.
Major functional defects found by these studies are a reduced endolymphatic potential (EP) and apoptotic hair cell loss after the onset of hearing.
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