Severe to profound hearing loss in patients with progressed Alport's syndrome

Conclusion: The concept of hearing loss severity must be redefined, as there is a clear need for more active hearing management in Alport's syndrome patients with severe and profound hearing loss. Objectives: Sensorineural hearing loss (SNHL) caused by Alport's syndrome generally does not exceed 6070 dB, because a cochlear lesion is responsible for this hearing loss. Careful management of renal function improves the prognosis and the longevity of Alport's syndrome patients; it is useful to reassess SNHL caused by Alport's syndrome. Patients and methods: Thirty-two patients with Alport's syndrome were analyzed retrospectively. Pure tone audiograms (PTAs), speech audiograms, and transiently evoked otoacoustic emissions (TEOAEs) were performed. Hearing loss severity was compared to duration of disease and severity of renal dysfunction. We also evaluated the correlation between OAEs and PTAs according to the hypothesis that evoked OAEs would be abnormal even in early stage SNHL in Alport's syndrome. Results: The level of hearing was positively correlated with disease duration. The hearing of the end-stage renal disease (ESRD) group, whose hearing threshold could exceed 70 dB, was worse than that of the non-ESRD group. OAEs were found in patients with normal hearing and mild hearing loss and had no significant early detection value.