UNASSIGNED: Approximately 10  % of the population worldwide suffers from @entity393 ( @entity393 ) and about 60  % of @entity1 with early onset @entity393 have hereditary @entity393 due to genetic mutations. Highly efficient mutagenesis in @entity19 with the chemical mutagen, @entity330 ( @entity331 ), associated with relevant phenotypic tools represents a powerful approach in producing @entity19 models for @entity393 . A benefit of this strategy is to generate alleles to form a series revealing the full spectrum of gene function in vivo. It can also mimic the range of @entity1 mutations and polymorphisms for @entity393 . In the course of a genome @entity331 mutagenesis program, we selected a new @entity19 model for @entity393 based on a dysmorphological screen. We identified by gene mapping the mutation responsible for this phenotype and characterized it at the histological level of the inner ear and evaluated the vestibule by following the recommendations of the standard operating procedures, IMPReSS. We have identified and characterized a new recessive allele of the @entity69613 gene, @entity69613 (vbd/vbd) , due to a homozygous one base pair substitution at the splice donor site of intron 29. This mutation leads to a frame-shift and a premature stop codon. We observed a decrease in the amount of sensory cells in the maculae of @entity69613 (vbd/vbd) @entity19 as well as an apparent drastically decreased density to almost absence of the otoconial membrane. Compared to @entity69613 (tm1Prs) and twister, the two other existing @entity69613 alleles, the detailed analysis of @entity69613 (vbd/vbd) revealed that these @entity19 share some common behavioural characteristics either with @entity69613 (tm1Prs) or twister whereas the fine vestibular phenotype and the @entity393 are different. Our results emphasize the importance of detecting and characterizing a new allele of a gene in order to get comprehensive information about the gene function.

A new @entity69613 XXXX @entity19 model for autosomal-recessive nonsyndromic moderate hearing impairment.

@entity1 :: ('9606', 'Species') :: ['human', 'persons'] @entity330 :: ('MESH:D005038', 'Chemical') :: ['ethylnitrosourea'] @entity19 :: ('10090', 'Species') :: ['mice', 'mouse'] @entity69613 :: ('18419(Tax:10090)', 'Gene') :: ['Otog', 'otogelin'] @entity331 :: ('CHEBI:23995', 'Chemical') :: ['ENU'] @entity393 :: ('MESH:D034381', 'Disease') :: ['HL', 'hearing loss', 'hearing defect', 'hearing impairment']
@entity331 :: (CHEBI:23995,Chemical) :: ['ENU']
