Original Article Inner Ear Involvement in Children with Familial Mediterranean Fever

OBJECTIVE: Familial Mediterranean fever (FMF) is the most common and best understood disease of hereditary periodic fever syndromes. Various degrees of sensorineural hearing loss can be seen in the progression of some hereditary periodic fever syndromes. There are very limited and con-troversial studies in the literature with regard to hearing loss in patients diagnosed with FMF. We aimed to evaluate cochlear function in children with FMF. MATERIALS and METHODS: Forty-nine patients diagnosed with FMF and 49 age- and sex-matched controls were included in the study. All of the children had undergone a routine ear-nose-throat examination. Patients with pathological findings in the otologic examination or a history of recur-rent otitis media; using proven ototoxic drugs; or having an abnormal tympanometry finding were excluded from the study. Following audiologic evaluation, tympanometry and distortion product otoacoustic emissions testing were conducted on all subjects. Pure-tone and high-frequency audiometry were carried out on 40 subjects from both groups who could cooperate in the testing. RESULTS: Hearing thresholds of FMF patients were found to be increased at all frequencies (250 to 16,000) (p<0.001). The difference between the hearing thresholds of the two groups could be seen more prominently at higher frequencies, and despite a statistically significant difference, the hearing thresholds of the two groups were within the normal range of tonal audiometry frequencies. In the otoacoustic emission evaluation, the distortion products and signal/noise ratio of children with FMF were lower in the tested frequencies from 1000 to 4000 Hz (p<0.001). CONCLUSION: Our results demonstrated that FMF disease may cause hearing loss in children with FMF. Hearing loss in children with familial Med