Evaluation of cochlear function using transient evoked otoacoustic emission in children with Familial Mediterranean Fever

Department of Otorhinolaryngology, Cumhuriyet University School of Medicine, Sivas, Turkey.
International journal of pediatric otorhinolaryngology (Impact Factor: 1.19). 03/2012; 76(3):379-81. DOI: 10.1016/j.ijporl.2011.12.015
Source: PubMed


The aim of this study was to investigate cochlear functions in children with Familial Mediterranean Fever (FMF).
Fifty-six FMF patients (112 ears) and 30 healthy control subjects (60 ears) were included in the study. Transient evoked otoacoustic emission (TEOAE) was investigated. Numerical measurements of TEOAE, except the correlation percentage (%), included response amplitude (dB) and signal/noise (SN) ratio.
There was no statistically significant difference in age and sex in the two groups. Mean TEOAE correlation percentage, signal/noise ratio, TEOAE amplitudes in 1, 1.5, 2, 3 and 4 Hz frequency values were not different between the two groups (p>0.05).
In this study using the TEOAE test, we found that FMF did not cause outer cell hair damage in children. In the literature, there is no study on outer cell hair damage in children or adults with FMF, so this is the first investigational study.

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    ABSTRACT: Familial Mediterranean fever (FMF) is a common and well-understood hereditary periodic fever syndrome. Hereditary periodic fever syndromes include a group of multisystem diseases characterized by recurrent fever attacks with inflammation affecting skin, joints, and some other tissues. These are FMF, tumor necrosis factor receptor, tumor necrosis factor receptor associated periodic syndrome, hyperimmunglobulinemia D syndrome, Muckle-Wells syndrome, and familial cold urticaria. In literature, it is determined that some of these diseases cause hearing loss. In light of the foregoing, we thought that FMF patients may have the same type of subclinical hearing loss and, therefore, the hearing ability of these patients was evaluated with otoacoustic emission and high frequency audiometry tests.
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    ABSTRACT: Familial Mediterranean Fever is the most common congenital, periodic fever condition that affects over 100,000 people worldwide. In the literature, there is limited number of studies about hearing levels in children with Familial Mediterranean Fever. In the present study, we aimed to investigate hearing levels and cochlear functions by using Distortion product Otoacoustic Emission and High Frequency Audiometry (250-20,000Hz) in pediatric patients with Familial Mediterranean Fever. The study included 62 children with Familial Mediterranean Fever and 27 healthy children with similar age and gender. After otoscopic examination, both groups underwent audiological evaluation including High Frequency Audiometry (250-20,000Hz) and Distortion product Otoacoustic Emissions. The results obtained were assessed among groups. In addition, these results were compared regarding colchicine use, age at the onset of disease and duration of the diseases in the Familial Mediterranean Fever group. Of the Familial Mediterranean Fever patients, 93.5% were on colchicine therapy and mean duration of colchicine use was 19.9±13.9 months. The mean age at diagnosis was 6.57±2.86 years (min-max: 2-14) and mean duration of disease was 23±17 months (min-max: 6-84). Pure tone audiometry values, and hearing levels between 9000 and 20,000Hz were similar and within normal range in both groups. The Distortion product Otoacoustic Emissions responses at the frequencies of 1020, 2040, 3000, 4080 and 5040Hz were similar for both groups. To the best of our knowledge, this is the first study evaluating hearing levels at the frequencies of 18kHz and 20kHz in children with Familial Mediterranean Fever in the literature. In children with Familial Mediterranean Fever, Pure tone audiometry values, hearing values obtained at all frequencies from 250 to 20,000Hz, and Distortion product Otoacoustic Emissions levels were within normal range. Furthermore, hearing levels were found to be similar to those in healthy children.
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