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Cochlear involvement in Familial Mediterranean Fever: a new feature of an old disease

Serap Koybasi1, Halil İbrahim Atasoy, Yusuf Ozgur Bicer

  • 1Abant Izzet Baysal University, Medical Faculty, Department of Otolaryngology, Turkey. serapkoybasi@yahoo.com

International Journal of Pediatric Otorhinolaryngology
|December 20, 2011
PubMed
Abstract

Insights

Children with Familial Mediterranean Fever show early cochlear changes, including higher frequency hearing loss and reduced otoacoustic emissions. These auditory alterations correlate with disease duration and age of onset.

Area of Science:

  • Otorhinolaryngology
  • Pediatric Rheumatology
  • Genetics

Background:

  • Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder.
  • Otopathology is not a commonly recognized feature of FMF.
  • Early detection of subclinical cochlear dysfunction is crucial in chronic childhood diseases.

Purpose of the Study:

  • To evaluate cochlear function in children diagnosed with Familial Mediterranean Fever.
  • To explore the relationship between hearing status and clinical parameters of FMF, such as disease duration, age of onset, genetic mutations, and colchicine treatment.

Main Methods:

  • A case-control study involving 34 children with FMF and 27 age-matched healthy controls.
  • Audiometric evaluation including Pure Tone Average (PTA) and Distortion Product Otoacoustic Emission (DPOAE) testing.
  • Statistical analysis to compare audiological results between groups and correlate with FMF clinical data.

Main Results:

  • While PTA hearing levels were generally normal, FMF patients exhibited elevated hearing thresholds at high frequencies (8-16 kHz).
  • DPOAE testing revealed reduced distortion products and signal-to-noise ratios in FMF children across tested frequencies (1.4-4 kHz).
  • The interaction between disease duration and age of onset significantly predicted hearing levels and DPOAE measures in FMF patients.

Conclusions:

  • This study is the first to report evidence of cochlear involvement in pediatric FMF, characterized by high-frequency hearing threshold elevation and impaired DPOAE responses.
  • These findings suggest subclinical cochlear dysfunction in children with FMF.
  • Further research is recommended to investigate these effects in adults, elucidate the underlying mechanisms, and assess the impact of colchicine therapy on cochlear function.

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