Evaluation of hearing in familial Mediterranean fever children

Aim of the work: Familial Mediterranean fever (FMF) is the most prevalent monogenic auto-inflammatory illness worldwide. It is characterised by periods of self-limiting fever and polyserositis, including pleuritis as well as peritonitis. This study attempted to assess hearing in paediatric FMF patients on colchicine treatment. Patients and methods Patients and methods: A hundred children with FMF (7-18 years old) (male/female: 43/57) were recruited. Audiological assessments were performed for all patients, including transientevoked otoacoustic emission (TEOAE), tympanometry, as well as pure-tone audiometry (PTA) tests. Severity score, response to treatment and type of MEFV gene mutation were assessed. Results: The mean age of patients was 11 +/- 3 years and disease duration 7.1 +/- 2.7 years. Fourteen patients were normal in all audiological tests, whereas 86 patients had impaired hearing (Sensorineural hearing loss (SNHL) in one or both ears). PTA results were normal in 23 patients and bilaterally impaired in 50 patients and unilaterally impaired in 27 patients. TEOAE results showed that 37 patients had a bilateral pass test, nine patients had a unilateral pass test, 11 patients had a bilateral partial pass test, eight patients had a unilateral partial pass test, and 40 patients had a bilateral fail test, and seven patients had a unilateral fail test. The patients were 22 % homozygous, 52 % heterozygous, and 26 % compound heterozygous. M694I was the most prevalent allele (32 %) in the current study. There were no statistically substantial correlations between audiological parameters and other variables.Conclusions: Children with FMF seem to have an increased risk of hearing impairment due to the affection of cochlear functions.(c) 2022 The Authors. Production and hosting by Elsevier B.V. on behalf of Egyptian Society of Rheumatic Diseases. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/ licenses/by-nc-nd/4.0/).