title: Evaluation of cochlear functions in children with Familial Mediterranean Fever.
abstract: OBJECTIVE
  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.
authors.affiliation: Department of Pediatrics, Dr. Sami Ulus Maternity and Children's Health and Diseases Training Hospital, Ankara, Turkey. Electronic address: begum_lordoglu@hotmail.com.
authors.forename: Begüm
authors.initials: B
authors.lastname: Lordoglu
authors.affiliation: Department of ENT, Kecioren Training and Research Hospital, Ankara, Turkey. Electronic address: dremrah2000@yahoo.com.
authors.forename: Baran
authors.initials: B
authors.lastname: Acar
authors.affiliation: Department of Pediatric Nephrology, Dr. Sami Ulus Maternity and Children's Health and Diseases Training Hospital, Ankara, Turkey. Electronic address: dremrah2000@gmail.com.
authors.forename: Fatma
authors.initials: F
authors.lastname: Yazilitas
authors.affiliation: Department of Pediatric Nephrology, Dr. Sami Ulus Maternity and Children's Health and Diseases Training Hospital, Ankara, Turkey. Electronic address: drsare_ozlu@yahoo.com.
authors.forename: Sare Gulfem
authors.initials: SG
authors.lastname: Ozlu
authors.affiliation: Department of Pediatrics, Dr. Sami Ulus Maternity and Children's Health and Diseases Training Hospital, Ankara, Turkey; Yildirim Beyazit University School of Medicine, Ankara, Turkey. Electronic address: drsaliha007@gmail.com.
authors.forename: Saliha
authors.initials: S
authors.lastname: Senel
mesh_terms: D000293:Adolescent; D001301:Audiometry, Pure-Tone; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D003078:Colchicine / Q000627:therapeutic use; D010505:Familial Mediterranean Fever / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology*; D005260:Female; D034381:Hearing Loss / Q000209:etiology / Q000503:physiopathology*; D006801:Humans; D008297:Male; D017084:Otoacoustic Emissions, Spontaneous; D059629:Signal-To-Noise Ratio; D050257:Tubulin Modulators / Q000627:therapeutic use
chemical_list: D050257:Tubulin Modulators; D003078:Colchicine
keywords: Children; Cochlear function; Colchicine; FMF; Familial Mediterranean Fever; Severity score