Can you hear me now? A genetic model of otitis media with effusion

Lazaridis, Evelyn; Saunders, James C.

doi:10.1172/jci33716

Cited by 3 publications

(2 citation statements)

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“…Risk factors for OM include infection, altered immune status, exposure to tobacco smoke, and anatomical defects such as cleft palate [ 14 ] . In addition, although the pathogenesis of OM is multifactorial, a role for genetic predisposition is increasingly recognized [ 15 , 16 ]. In 22q11DS, studies have shown that a majority of patients have a history of chronic or recurrent OM [ 17 , 18 ].…”

Section: Introductionmentioning

confidence: 99%

Hearing Loss in a Mouse Model of 22q11.2 Deletion Syndrome

et al. 2013

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22q11.2 Deletion Syndrome (22q11DS) arises from an interstitial chromosomal microdeletion encompassing at least 30 genes. This disorder is one of the most significant known cytogenetic risk factors for schizophrenia, and can also cause heart abnormalities, cognitive deficits, hearing difficulties, and a variety of other medical problems. The Df1/+ hemizygous knockout mouse, a model for human 22q11DS, recapitulates many of the deficits observed in the human syndrome including heart defects, impaired memory, and abnormal auditory sensorimotor gating. Here we show that Df1/+ mice, like human 22q11DS patients, have substantial rates of hearing loss arising from chronic middle ear infection. Auditory brainstem response (ABR) measurements revealed significant elevation of click-response thresholds in 48% of Df1/+ mice, often in only one ear. Anatomical and histological analysis of the middle ear demonstrated no gross structural abnormalities, but frequent signs of otitis media (OM, chronic inflammation of the middle ear), including excessive effusion and thickened mucosa. In mice for which both in vivo ABR thresholds and post mortem middle-ear histology were obtained, the severity of signs of OM correlated directly with the level of hearing impairment. These results suggest that abnormal auditory sensorimotor gating previously reported in mouse models of 22q11DS could arise from abnormalities in auditory processing. Furthermore, the findings indicate that Df1/+ mice are an excellent model for increased risk of OM in human 22q11DS patients. Given the frequently monaural nature of OM in Df1/+ mice, these animals could also be a powerful tool for investigating the interplay between genetic and environmental causes of OM.

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Section: Introductionmentioning

confidence: 99%

Hearing Loss in a Mouse Model of 22q11.2 Deletion Syndrome

et al. 2013

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“…Using murine models, a crucial role for proteins in the Toll receptor pathways was identified. [69][70][71] A mouse model of deafness known to develop chronic suppurative OM, Jeff, carries a mutation in an F-box gene, Fbxo11, predisposing to development of cleft palate. 54,60 Indeed, defects in chemokines such as IL-8, MCP-1 and CCL3, which play integral roles in promoting early neutrophilic inflammation, [63][64][65] can predispose animals to AOM.…”

Section: Eustachian Tube Obstructionmentioning

confidence: 99%