Evaluation of hearing in pediatric familial Mediterranean fever patients during attack period and attack-free period.

OBJECTIVES: Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease worldwide. It usually has a childhood onset and is characterized with recurrent attacks with irregular intervals. Few studies have been performed to investigate hearing in FMF patients ran with various tests and showed different results. In this study, first time in the literature, we aimed to evaluate and compare auditory functions in pediatric FMF patients during the attack periods and attack-free periods to see the possible effects of autoinflammation that was caused by FMF attacks. METHODS: 40 pediatric FMF patients (80 ears) enrolled in the study as study group and 21 matching (42 ears) healthy children as a control group. Blood samples were taken from patients who presented clinical attack symptoms. Control group and FMF patients that were in attack period underwent audiometric evaluation including pure tone audiometry (0.25, 0.5, 1, 2, 4, 8 kHz) and Distortion product otoaoustic emission (DPOAE) (1, 1.4, 2, 2.8, 4 kHz) test. The tests were repeated in attackfree period. Correlations between hearing results and C-reactive protein, erythrocyte sedimentation rate levels, duration of disease, age at disease onset, colchicine usage time, cumulative dosage of colchicine and the number of attacks in the last six months were studied. RESULTS: Hearing thresholds of FMF patients, compared to control group, were found to be increased at most frequencies. Lower signal/noise ratios (SNR) were detected at most frequencies in the DPAOE test. In the attack period compared to attack-free period, hearing thresholds in the audiometry were found to be increased at some frequencies (p < 0.05). However, this was not supported by the DPOAE test. In correlation analysis, increased colchicine usage time and colchicine cumulative dosage were associated with decreased thresholds in audiogram and increased SNR values in DPOAE. Detailed statistical analyses of all parameters were included in the study. CONCLUSION: Results demonstrated cochlear involvement in FMF patients. Acute changes in hearing thresholds in the attack period may be suggesting the effect of acute inflammation on cochlea. The difference between the audiometry and otoacoustic emission test results of FMF patients and healthy controls suggests the cumulative effect of recurrent inflammation attacks on cochlea. Positive affect of treatment of the disease with long term colchicine in hearing were also demonstrated.

Parapharyngeal giant ganglioneuroma with multifocal bone involvement in a pediatric female patient.

Ganglioneuroma is a rare benign tumor that originates from neural crest. Tumor tends to be slow growing, asymptomatic but can cause symptoms because of pressure to neighboring structures. In the head and neck region they are relatively rarely seen. We hereby present a rare case of multiple ganglioneuromas that were located in parapharyngeal space, iliac bone and other bones in a 13-year-old girl. Patient underwent surgery for the excision of a large mass, extending from parapharyngeal space to neck, with transparotid and transcervical combined approach. After operation, MIBG (iodine-123-meta-iodobenzylguanidine) scintigraphy was performed and involvement of parietooccipital bone, lumbal vertebra, right iliac wing medial cortex and left humerus were detected. No adjuvant therapy was given to the patient. There is no evidence of recurrence in the head and neck region in the following 12 months. In conclusion, complete surgical excision of the tumor, if possible, is the treatment of choice with high success rate. Close clinical and radiological follow-up for these tumors after surgery should be made.

A Rare Location of Angiofibroma in the Inferior Turbinate in Young Woman

Introduction Juvenile nasopharyngeal angiofibroma is a rare benign neoplasm in the nasopharynx. The tumor tends to be locally aggressive and is typically seen in adolescent boys. Extranasopharyngeal angiofibromas have been reported sporadically in the literature. They most commonly originate from the maxillary sinus. Objectives A 26-year-old woman was referred to our clinic with intermittent epistaxis from the right nasal passage for the previous 2 months. Maxillofacial magnetic resonance imaging showed a lobular, contoured mass originating from the right inferior turbinate and hanging in the right nasal cavity, with dense contrast enhancement denoting hypervascularity. Resumed Report Vascular feeding of the mass was seen from the right internal maxillary artery with angiography, and this branch was embolized. On the following day, the patient underwent transnasal endoscopic excision of the mass. An approximately 3-cm-diameter mass was excised by partial turbinectomy, and the posterior edge of the remaining turbinate was cauterized. Conclusion Extranasopharyngeal angiofibromas are rarely seen, and the inferior turbinate is an extremely rare location for them. This young woman is the first case reported in the English literature of angiofibroma originating from the inferior turbinate. We should consider these neoplasms can be found in female, nonadolescent patients with extranasopharyngeal localization, and we should not perform biopsy because of its massive bleeding. .

A rare location of angiofibroma in the inferior turbinate in young woman.

Introduction Juvenile nasopharyngeal angiofibroma is a rare benign neoplasm in the nasopharynx. The tumor tends to be locally aggressive and is typically seen in adolescent boys. Extranasopharyngeal angiofibromas have been reported sporadically in the literature. They most commonly originate from the maxillary sinus. Objectives A 26-year-old woman was referred to our clinic with intermittent epistaxis from the right nasal passage for the previous 2 months. Maxillofacial magnetic resonance imaging showed a lobular, contoured mass originating from the right inferior turbinate and hanging in the right nasal cavity, with dense contrast enhancement denoting hypervascularity. Resumed Report Vascular feeding of the mass was seen from the right internal maxillary artery with angiography, and this branch was embolized. On the following day, the patient underwent transnasal endoscopic excision of the mass. An approximately 3-cm-diameter mass was excised by partial turbinectomy, and the posterior edge of the remaining turbinate was cauterized. Conclusion Extranasopharyngeal angiofibromas are rarely seen, and the inferior turbinate is an extremely rare location for them. This young woman is the first case reported in the English literature of angiofibroma originating from the inferior turbinate. We should consider these neoplasms can be found in female, nonadolescent patients with extranasopharyngeal localization, and we should not perform biopsy because of its massive bleeding.