Evaluation of Hearing and Auditory Pathways in Fabry Disease Patients

Background Hearing and the auditory pathway are affected in Fabry diseases (FD). There is limited data on hearing pathways this population. Therefore, we aimed to investigate functions using brainstem responses (ABR), otoacoustic distortion emission (DPOAE), pure tone audiometry (PTA), tympanometry patients with FD compare these results those of healthy individuals.
 Material Methods This study included 16 (F/M: 8/8, age: 33.5±15.4 years) controls 5/11, 33.6±6.3 years). were assessed ABR, DPOAE, PTA, tympanometry.
 Results According conductive loss was detected 4 (25%) FD. When 500-4,000 Hz frequencies assessed, bone threshold both ears significantly higher than control group (p=0.014 p=0.014, respectively). compared DPOAE measurements groups, dB value measured at 2.8 kHz lower patient (p=0.018). ABR measurements, right ear's 3-5 interpeak latency 60 (1.8±0.3 ms vs 2±0.2 ms, p=0.033).
 Conclusions We found that rate statistically group. screening should be systematically performed patients.