Audiological features in children with alport syndrome

doi:10.3969/j.issn.1672-2922.2015.03.014

Abstract：Objective To assess audiological features in children with Alport Syndrome and hearing loss prognosis. Methods Audiologic, nephrologic and opthalmologic findings in 43 cases of Alport syndrome be-tween 2007 and 2013 were reviewed. Their progress was followed up by telephone interview. Results For-ty-three individuals (36 males, 7 females, aged from 22 months to 13 years) underwent audiologic tests, which showed normal hearing in 33 and hearing loss in 10. Of these children with AS, 23.2%(all male) had sensori-neural hearing loss (70%at moderate hearing loss level). Audiometric configuration was“U”-shaped in 7, slop-ing in 2 or flat in 1 patient, respectively. Sensorineural hearing loss was asymmetric in 30%of these patients. For AS children with abnormal hearing, there were more severe podocyte injury as compared with those with normal hearing. Telephone follow-up found that one-third of AS children with normal hearing gradually devel-oped hearing loss, and hearing in those with abnormal hearing became progressively worse. One child with se-vere hearing loss developed end stage renal disease (ESRD). Conclusion Hearing loss is a common symptom of AS, which may reflect the severity of podocyte lesions. Audiometric configuration distribution in AS may vary and can be asymmetric. Children with AS all showed continuing progressive hearing loss.