Determination of hearing levels in patients with Familial Mediterranean Fever

Çevik, Cengiz; Şehitoğlu, İbrahim; Arıca, Vefik; Yengil, Erhan; Akbay, Ercan; Sarac, Tuba; Başarslan, Fatmagül; Akoğlu, Ertap

doi:10.1016/j.ijporl.2013.10.003

Cited by 10 publications

(8 citation statements)

References 16 publications

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“…To our knowledge, three studies of cochlear involvement in children with a diagnosis of FMF exist [4][5][6] . Uysal et al [5] presented similar results between a control group and patients with FMF for mean TEOAE correlation percentage, signal-noise ratio, and TEOAE amplitudes at 1, 1.5, 2, 3, and 4 Hz frequency values.…”

Section: Discussionmentioning

confidence: 99%

“…On the other hand, Koybası et al found increased hearing thresholds, including high frequencies (8000, 10,000, 12,500, and 16,000 Hz) in HFA, and showed decreased distortion products and signal-noise ratios for FMF children, including frequencies (1400 Hz to 4000 Hz) in the otoacoustic emission evaluation [4] . Recently, Cevik et al [6] showed normal DPOAE values ranging from 1020 Hz to 5040 Hz and normal hearing values in an auditory evaluation including HFA between children with FMF and controls. In contrast, in our study using DPOAEs and HFA, in pediatric patients with FMF, hearing levels were clearly lower than in the healthy control group, including frequencies of 1000 Hz to 4000 Hz, Table 1, the difference between the hearing thresholds of the two groups can be seen more prominently at higher frequencies, and despite a statistically significant difference, the hearing thresholds of the two groups were within the normal range at tonal audiometry frequencies.…”

Section: Discussionmentioning

confidence: 99%

“…Is this a possibility? To our knowledge, only three studies exist (having controversial findings) in the literature with regard to children with FMF [4][5][6] . Development of vasculitic disease is seen with increasing frequency in patients with FMF [4,5,7] .…”

Section: Introductionmentioning

confidence: 99%

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Inner Ear Involvement in Children with Familial Mediterranean Fever

Bucak¹,

Ulu²,

Bükülmez³

et al. 2014

Int Adv Otol

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OBJECTIVE:Familial Mediterranean fever (FMF) is the most common and best understood disease of hereditary periodic fever syndromes. Various degrees of sensorineural hearing loss can be seen in the progression of some hereditary periodic fever syndromes. There are very limited and controversial studies in the literature with regard to hearing loss in patients diagnosed with FMF. We aimed to evaluate cochlear function in children with FMF. MATERIALS and METHODS:Forty-nine patients diagnosed with FMF and 49 age-and sex-matched controls were included in the study. All of the children had undergone a routine ear-nose-throat examination. Patients with pathological findings in the otologic examination or a history of recurrent otitis media; using proven ototoxic drugs; or having an abnormal tympanometry finding were excluded from the study. Following audiologic evaluation, tympanometry and distortion product otoacoustic emissions testing were conducted on all subjects. Pure-tone and high-frequency audiometry were carried out on 40 subjects from both groups who could cooperate in the testing. RESULTS:Hearing thresholds of FMF patients were found to be increased at all frequencies (250 to 16,000) (p<0.001). The difference between the hearing thresholds of the two groups could be seen more prominently at higher frequencies, and despite a statistically significant difference, the hearing thresholds of the two groups were within the normal range of tonal audiometry frequencies. In the otoacoustic emission evaluation, the distortion products and signal/noise ratio of children with FMF were lower in the tested frequencies from 1000 to 4000 Hz (p<0.001). CONCLUSION:Our results demonstrated that FMF disease may cause hearing loss in children with FMF. Hearing loss in children with familial Mediterranean fever has been observed, showing increased hearing thresholds at all frequencies in audiometry, together with decreased distortion products and signal: noise ratios, demonstrated by distortion product otoacoustic emissions testing. We think this might be due to cochlear involvement. Regular follow-up of auditory function in FMF children may be helpful for determining early possible hearing loss.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Inner Ear Involvement in Children with Familial Mediterranean Fever

Bucak¹,

Ulu²,

Bükülmez³

et al. 2014

Int Adv Otol

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“…The majority of these studies were conducted in adults [13], often with controversial findings. In some of these studies, FMF patients, when compared to the healthy group, showed elevated hearing thresholds and decreased otoacoustic emission, supporting cochlear involvement [12,14], whereas other studies report no significant difference between these two groups [6,15].…”

Section: Introductionmentioning

confidence: 93%

“…The symptomatic period is termed as "at-tack". The patients have no symptoms during the intervals between attacks, which is an important feature for diagnosis [6]. The gene associated with the disease (MEFV gene Chr 16p13.3) was first identified in 1997 by two groups [7,8] and encodes a 781-aminoacid protein (pyrin/marenostrin) involved in the regulation of inflammation and apoptosis [9].…”

Section: Introductionmentioning

confidence: 99%

Evaluation of hearing in children with familial Mediterranean fever: A study during attack and attack-free periods

Andaloro¹,

Castro²,

Mantia³

2018

polp

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Introduction: Few studies have investigated the hearing in familial Mediterranean fever (FMF), with conflicting results reported. The aim of this study was to compare the cochlear functions of FMF children with healthy controls and to evaluate any differences in the audiological outcomes of FMF patients during attack and attack-free periods. Material and methods: In this study were recruited 50 FMF children and 31 healthy children as controls. FMF patients that were in an attack period and a control group underwent audiometric evaluation including pure tone audiometry and Distortion Product Otoacoustic Emission (DPOAE) test. The evaluation was repeated in FMF patients in an attack-free period. Additionally, auditory brainstem response (ABR) test was performed during attack and attack-free periods. Results: Hearing thresholds of FMF patients, compared to the control group, were found to be increased at all frequencies on the left and at 1000, 4000, and 8000 Hz on the right (p < 0.05). This was supported by the DPOAE test, with lower values at all frequencies in FMF children (p < 0.05). In the attack period, compared to the attack-free period, hearing thresholds were found to be increased only at certain frequencies, but this was not supported by the DPOAE test (p > 0.05). No statistically significant difference between attack and attack-free period for ABR test were found (all p > 0.05). Conclusions: Our results demonstrated cochlear involvement in FMF patients, but no objective acute impairment in hearing in the attack period, which suggests that hearing impairment in FMF is due to chronic autoinflammation process.

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