Sh3pxd2b Mice Are a Model for Craniofacial Dysmorphology and Otitis Media

Craniofacial defects that occur through gene mutation during development increase vulnerability to eustachian tube dysfunction. These defects can lead to an increased incidence of otitis media. We examined the effects of a mutation in the Sh3pxd2b gene (Sh3pxd2bnee) on the progression of otitis media and hearing impairment at various developmental stages. We found that all mice that had the Sh3pxd2bnee mutation went on to develop craniofacial dysmorphologies and subsequently otitis media, by as early as 11 days of age. We found noteworthy changes in cilia and goblet cells of the middle ear mucosa in Sh3pxd2bnee mutant mice using scanning electronic microscopy. By measuring craniofacial dimensions, we determined for the first time in an animal model that this mouse has altered eustachian tube morphology consistent with a more horizontal position of the eustachian tube. All mutants were found to have hearing impairment. Expression of TNF-α and TLR2, which correlates with inflammation in otitis media, was up-regulated in the ears of mutant mice when examined by immunohistochemistry and semi-quantitative RT-PCR. The mouse model with a mutation in the Sh3pxd2b gene (Sh3pxd2bnee) mirrors craniofacial dysmorphology and otitis media in humans

Crianças com fissura isolada de palato: desempenho nos testes de processamento auditivo Cleft palate children: performance in auditory processing tests

Muitas crianças com transtorno de processamento auditivo têm uma prevalência alta de otite média, alteração na orelha média de grande ocorrência na população com fissura labiopalatina. OBJETIVO: Verificar o desempenho de crianças com fissura isolada de palato (FP) em testes do processamento auditivo. Estudo prospectivo. MATERIAL E MÉTODO: Vinte crianças (7 a 11 anos) com FP foram submetidas aos testes de localização sonora (LS), memória para sons verbais (MSSV) e não-verbais em seqüência (MSSNV), Fusão Auditiva-Revisado (AFT-R), Teste Pediátrico de Inteligibilidade de Fala/Sentenças Sintéticas (PSI/SSI), Dissílabos alternados (SSW) e Dicótico de dígitos (DD). O desempenho das crianças nos testes foi classificado em ruim e bom. RESULTADOS: Não houve diferença estatística entre os gêneros e orelhas. Os valores médios obtidos foram 2,16, 2,42, 4,37, 60,50ms, de 40,71 a 67,33%, 96,25 a 99,38%, 73,55 a 73,88% e 58,38 a 65,47%, respectivamente, para os testes MSSNV, MSSV, LS, AFT-R, PSI/SSI com mensagem competitiva ipsilateral (PSI/SSIMCI) e contralateral (PSI/SSI/MCC), DD e SSW. CONCLUSÃO: Uma alta porcentagem de crianças demonstrou seus piores desempenhos nos testes AFT-R, DD, SSW e no teste PSI/SSIMCI. Os melhores desempenhos ocorreram nos testes de localização sonora, memória seqüencial para sons não verbais e verbais e para PSI/SSIMCC.<br>Many children with auditory processing disorders have a high prevalence of otitis media, a middle ear alterations greatly prevalent in children with palatine and lip clefts. AIM: to check the performance of children with palate cleft alone (PC) in auditory processing tests. Prospective study. MATERIALS AND METHODS: twenty children (7 to 11 years) with CP were submitted to sound location tests (SL), memory for verbal sounds (MSSV) and non verbal sounds in sequence (MSSNV), Revised auditory fusion (AFT-R), Pediatric test of speech intelligibility/synthetic sentences (PSI/SSI), alternate disyllables (SSW) and digit dichotic (DD). The children performances in the tests were classified in bad and good. RESULTS: there was no statistically significant difference between genders and ears. The average values obtained were 2.16, 2.42, 4.37, 60.50ms; 40.71 to 67.33%; 96.25 to 99.38%; 73.55 to 73.88% and 58.38 to 65.47% respectively for the MSSNV, MSSV, LS, AFT-R, PSI/SSI tests with ipsilateral (PSI/SSIMCI) and contralateral (PSI/SSI/MCC) competitive message, DD and SSW tests. CONCLUSION: a high percentage of children showed worse results in the AFT-R, DD, SSW tests and in the PSI/SSIMCI tests. The best performances happened in the sound location tests, verbal and non-verbal sounds for sequential memory and for PSI/SSIMCC tests