Evaluation of Hearing and Auditory Pathways in Fabry Disease Patients

Abstract

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