Evaluation of Hearing and Auditory Pathways in Fabry Disease Patients

Authors : Fethi YÖNET, İsmail BALOĞLU, Çiğdem KUCUR YÖNET, Mehmet DÜNDAR, Hakan OZER, Yasin ÖZTÜRK, Kültigin TÜRKMEN

Pages : 84-91

Doi:10.46310/tjim.1209122

View : 9 | Download : 7

Publication Date : 2023-04-29

Article Type : Research Paper

Abstract :Background Hearing and the auditory pathway are affected in Fabry diseases insert ignore into journalissuearticles values(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 insert ignore into journalissuearticles values(ABR);, otoacoustic distortion emission insert ignore into journalissuearticles values(DPOAE);, pure tone audiometry insert ignore into journalissuearticles values(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 insert ignore into journalissuearticles values(F/M: 8/8, age: 33.5±15.4 years); and 16 healthy controls insert ignore into journalissuearticles values(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 insert ignore into journalissuearticles values(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 insert ignore into journalissuearticles values(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 insert ignore into journalissuearticles values(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 insert ignore into journalissuearticles values(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.
Keywords : Fabry disease, hearing, auditory pathways