title: Cochlear involvement in Familial Mediterranean Fever: a new feature of an old disease.
abstract: OBJECTIVE
  In this study we first aimed to assess the cochlear functions in children with Familial Mediterranean Fever. The second aim was to investigate the correlation between the hearing levels and some clinical features of Familial Mediterranean Fever including the duration of the disease, age at onset, genetic analysis and colchicine use.
  
  
  METHODS
  Thirty-four children with Familial Mediterranean Fever and 27 age matched children were included in the study. Following otologic examination, all children underwent audiometric evaluation, including Pure Tone Average measurements and Distortion Product Otoaoustic Emission testing. Audiological results of the two groups were compared and correlation between the audiologic status and clinical parameters of the disease like the duration of disease, age at onset, mutations and colchicine treatment were studied.
  
  
  RESULTS
  Pure tone audiometry hearing levels were within normal levels in both groups. Hearing thresholds of Familial Mediterranean Fever patients were found to be increased at frequencies 8000, 10,000, 12,500 and 16,000 (p<0.05). In otoacoustic emission evaluation, distortion products and signal-noise ratio of FMF children were lower in the tested frequencies, from 1400 Hz to 4000 Hz (p<0.05). Interaction of the disease duration and age of disease onset was found to predict hearing levels, distortion products and signal-noise ratios of children with Familial Mediterranean Fever (F value=2.034; p=0.033).
  
  
  CONCLUSIONS
  To our knowledge this is the first study demonstrating cochlear involvement in children with Familial Mediterranean Fever which showed increased hearing thresholds at higher frequencies in audiometry together with decreased distortion products and signal-noise ratios demonstrated by distortion product otoacoustic emission testing. Similar studies must be carried out on adult patients to see if a clinical hearing impairment develops. The possible mechanisms that cause cochlear involvement and the effect of colchicine treatment on cochlear functions must be enlightened.
authors.affiliation: Abant Izzet Baysal University, Medical Faculty, Department of Otolaryngology, Turkey. serapkoybasi@yahoo.com
authors.forename: Serap
authors.initials: S
authors.lastname: Koybasi
authors.forename: Halil İbrahim
authors.initials: Hİ
authors.lastname: Atasoy
authors.forename: Yusuf Ozgur
authors.initials: YO
authors.lastname: Bicer
authors.forename: Esra
authors.initials: E
authors.lastname: Tug
mesh_terms: D000293:Adolescent; D017677:Age Distribution; D001299:Audiometry / Q000379:methods*; D001309:Auditory Threshold / Q000502:physiology; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D015834:Cochlear Diseases / Q000453:epidemiology* / Q000235:genetics / Q000503:physiopathology; D015897:Comorbidity; D010505:Familial Mediterranean Fever / Q000453:epidemiology* / Q000235:genetics / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006319:Hearing Loss, Sensorineural / Q000453:epidemiology / Q000235:genetics / Q000503:physiopathology; D006320:Hearing Tests; D006801:Humans; D015994:Incidence; D008297:Male; D017084:Otoacoustic Emissions, Spontaneous; D018570:Risk Assessment; D012720:Severity of Illness Index; D017678:Sex Distribution; D014421:Turkey / Q000453:epidemiology