Familial Mediterranean Fever (FMF) is a progressive disease characterized by chronic inflammation, which also has negative effects on cochlear functions and hearing levels. We investigated whether the cochlear functions and hearing levels of FMF patients were different than healthy controls and also evaluated the relationship of hearing levels with the age at diagnosis, duration without treatment, and inflammation and lipid parameters in this study. A total of 60 patients diagnosed with FMF and 48 age, gender and body mass index (BMI)-matched healthy controls were included in the study. The hemogram, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and lipid parameters of the subjects were studied and they all underwent pure tone audiometry and Transient evoked otoacoustic emission tests after an otologic examination. The hearing levels of the FMF group were significantly higher than those of the control group. The TEOAE signal/noise (S/N) ratios were similar in both groups. A positive relationship was present between the audiometric test results and the age, BMI, low-density lipoprotein and triglyceride levels and a negative relationship with the high-density lipoprotein levels. A negative relationship was present between the TEOAE S/N ratios and the age of the patients, duration without treatment, lipid parameters, inflammation markers and the creatinine level. FMF patients are exposed to chronic inflammation and this can influence their hearing levels. The age at diagnosis, duration without treatment, chronic inflammation, unfavorable lipid parameters, and obesity can affect hearing tests negatively.