Clinical data and hearing of individuals with Alport syndrome.

UNLABELLED 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.