Characterization of sensorineural hearing loss in children with Alport syndrome

Alport syndrome is a rare genetic disorder of collagen formation affecting the function of kidneys, eyes and ears. Adult patients suffer from progressive sensorineural hearing loss in the mid and high frequencies. However, little is known about the development and characteristics of hearing loss in children with AS. Causal therapy is not available. The multicenter, randomized, placebo-controlled study "EARLY PRO-TECT" investigated the preservation of renal function in 66 children with Alport syndrome (age 8.8±4.2 years) treated with the ACE inhibitor ramipril. Additional data regarding hearing function were obtained from 51 of the 66 patients. Hearing loss was diagnosed in 9 of the 51 children (18 %). The audiograms of 38 children were divided into three age groups. Including 8 follow-up audiograms, a total of 46 audiograms were analyzed. Audiograms of children with hearing loss are characterized by a typically U-shaped configuration with a maximum at 2 kHz. On average, the 4PTA of 5-9-year-olds without significant hearing loss was 9.7 dB (N=15) and that of hearing impaired 43.8 dB (N=2, 12 %). Among 10-14-year-olds, 4PTA were 6.3 dB (healthy, N=17, 23 %) and 44.6 dB (hearing impaired, N=5). For 15-21 year olds, the 4PTA was 7.3 dB (healthy; N=5) and 50.9 dB (hearing impaired, N=2, 29 %). The data collected show that approximately 18 % of children with Alport syndrome develop hearing loss with a maximum of 2 kHz, while the rest remains normal hearing. The proportion of hearing impaired and the extent of hearing loss among those affected increase slightly with age. Poster-PDF A-1524.PDF Publication History Article published online: 10 June 2020 © 2020. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/). © Georg Thieme Verlag KG Stuttgart · New York