Navegando por Palavras-chave "nefrite hereditária"

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    Dados clínicos e da audição em indivíduos com Síndrome de Alport
    (ABORL-CCF Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial, 2008-12-01) Alves, Fatima Regina Abreu; Ribeiro, Fernando De Andrade Quintanilha [UNIFESP]; FCMSC-SP ORL; Universidade Federal de São Paulo (UNIFESP); Santa Casa de Misericórdia
    Alport Syndrome (AS) is a hereditary disease, characterized by nephropathy, often times with sensorineural hearing loss and ocular defects. AIM: to analyze the clinical and hearing information from individuals with AS, more specifically the correlation between renal disorder and hearing loss (HL). STUDY DESIGN: clinical prospective with cross-sectional cohort. MATERIALS AND METHODS: 37 individuals underwent otorhinolaryngological evaluation and were submitted to audiologic tests. For HL statistical analysis we considered only the results from the pure tone audiometries. RESULTS: of the 28 individuals with clinical alterations, we found 46.4% of DLX and 53.6% of AD. HL happened to 46.1% of the individuals evaluated. 12 patients presented HL in the audiometric test: 11.5% mild and 34.6% moderate. Comparing the normal relatives with those with renal disorder; all that had HL also had renal disorder. In 30.8% the curve shape was mild descending in the high frequencies and in 11.5% it was flat. CONCLUSIONS: The inheritance pattern distribution does not match literature descriptions. HL is a frequent extra-renal finding. There is an association between renal involvement and HL (p= 0.009). The most frequent curve shapes: mild descending in the high frequencies and flat. There was no association between HL and age. There is no correlation between the HL and gender in this group.
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    Percepção auditiva em síndrome de Alport
    (ABORL-CCF Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial, 2006-12-01) Viveiros, Carla Mherlyn [UNIFESP]; Pereira, Liliane Desgualdo [UNIFESP]; Mastroianni Kirsztajn, Gianna [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)
    Alport s Syndrome is characterized by the presence of renal, hearing and visual disorders. Objective: To characterize the TOAE and the MOES activity (suppression effect) in individuals with Alport s Syndrome. MATERIAL AND METHOD: This is a prospective study of a sample included ten individuals with a diagnosis of Alport s Syndrome. MOES recording was made in the presence and absence of contralateral stimulation (CLS) stimulation using the computer software ILO 92 - Otodynamics. RESULTS: TOAE was present in the global response (A) and in frequency ranges of 1000, 1500, 2000 and 3000 Hz in 4 individuals (40%), and absent in 6 individuals (60%) with hearing loss. We observed no responses at 4000 Hz in the right and left ears. Individuals that presented global responses to TOAE also suppressed that response when there was noise. CONCLUSION: The suppression effect also occurs with TOAE, suggesting that the hearing loss is predominantly the result of cochlear dysfunction.