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Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
Evaluation of cochlear functions in children with Familial Mediterranean Fever
Begüm Lordoglu1, Baran Acar2, Fatma Yazilitas3
1Department of Pediatrics, Dr. Sami Ulus Maternity and Children's Health and Diseases Training Hospital, Ankara, Turkey.
Insights
Familial Mediterranean Fever (FMF) can impact hearing, particularly at high frequencies. This study found differences in cochlear function in FMF patients compared to healthy individuals, correlating with disease severity and treatment duration.
Area of Science:
- Otorhinolaryngology
- Genetics
- Rheumatology
Background:
- Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder.
- Hearing impairment is a potential extra-intestinal manifestation of FMF.
- Cochlear function in FMF patients requires further investigation.
Purpose of the Study:
- To assess cochlear functions in pediatric FMF patients.
- To correlate auditory findings with FMF disease severity.
- To evaluate the relationship between treatment duration and cochlear function.
Main Methods:
- Audiologic evaluation, including high-frequency pure-tone audiometry and distortion product otoacoustic emissions (DPOAE), was performed on 50 FMF patients (4-18 years) and 39 healthy controls.
- Disease severity was assessed using the Pras et al. scoring system.
- Colchicine treatment duration was recorded for all FMF patients.
Main Results:
- FMF patients exhibited significantly higher pure tone averages at 500, 4000, and 8000 Hz compared to controls.
- DPOAE signal values and signal-to-noise ratio (SNR) were significantly altered in FMF patients at high frequencies (6-8 kHz).
- Hearing deficits correlated with disease severity (higher pure tone average at 2000 Hz in moderate/severe groups) and treatment duration (differences at 250-500 Hz).
Conclusions:
- Familial Mediterranean Fever adversely affects cochlear functions, especially at high frequencies.
- Audiologic abnormalities in FMF patients are linked to disease severity and duration of colchicine treatment.
- High-frequency hearing assessment is recommended for FMF patients.
Objectives:
To evaluate cochlear functions in patients with Familial Mediterranean Fever in relation to the disease severity score and treatment duration.
Methods:
50 patients (4-18 years) who had been followed-up with the diagnosis of FMF and regularly receiving appropriate colchicine treatment and 39 healthy controls were included in the study. All the patients and controls were evaluated by audiologic evaluation, including high-frequency pure-tone audiometry and distortion product otoacoustic emission tests (DPOAE). The disease severity was determined by scoring system developed by Pras et al.
Results:
Fifty patients (52% female, 48% male; mean age12.2 ± 4.1 years) and 39 controls (58.9% female, 41.1% male, mean age 11.1 ± 3.4 years) were enrolled the study. The pure tone average of FMF patients was significantly higher than that of the control group at 500, 4000, and 8000 Hz frequencies. The patients' DPOAE signal values at 6 kHz, 8 kHz frequencies and SNR values at 8 kHz were significantly higher than control group. The patients' audiometry and DPOAE results were compared with the disease severity scores. Pure tone average was significantly higher in severe and moderate patient groups compared to the mild patient group at 2000 Hz frequency. DPOAE signal values showed statistically significant differences between the patient severity scores at 1.4 and 2.8 kHz frequencies. The mean colchicine treatment duration was found to be 5.1 ± 3.7 years. There were significant differences at 250 and 500 Hz frequencies when patients' audiometry results were compared with the treatment periods.
Conclusions:
FMF affects cochlear functions particularly at high frequencies.
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