Evaluation of hearing function in patients with Sağlıker Syndrome

Yıl 2009, Cilt: 36 Sayı: 3, 195 - 199, 01.09.2009

Salih Bakır İsmail Yıldız Yıldız Yahya Sağlıker

Öz

Aim: The aim of this study was to evaluate th hearing functions of patients with Sagliker syndrome. Sagliker syndrome is a new disease entity that firstly has been described at year 2000. The frequency of this syndrome has been reported as approximately 0.5% of patient with chronic renal failure and mostly seen in poor and underdeveloped countries. The definite causes of this entity has not been not been clearly understood yet, however studies on risk factors that can lead to this syndrome are underway. Sagliker Syndrome seems to be related to chronic kidney disease and secondary hyperparathyroidism. Every kind of bone abnormalities including, skull deformities, short stature, maxillary and mandibulary changes, teeth abnormalities, soft and harmless tumoral tissues in the mouth and fingertip changes can develop. Psychological problems and depression may accompany these findings Materials and Methods: In present study, 12 patients with Sagliker Syndrome were retrospectively evaluated. Audiological tests were performed on these patients, including otoscopic examinations, Rinne and Weber tests, pure tone odiometry and timpanometry. Results: Five patients (41.7%) were found to have sensorineural type hearing loss. According to our results with a limited number of patients, hearing loss potential seems to be high with Sagliker Syndrome. Conclusion: Haemodialysis can be suggested as a risk factor on hearing loss of Sagliker syndrome, since all of our patients were on hemodialysis. Keywords: Sagliker Syndrome, hearing loss, Haemodialysis

Anahtar Kelimeler

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Sağlıker sendromlu hastalarda isitme fonksiyonunun değerlendirilmesi

Öz

Amaç: Bu çalısmanın amacı Sağlıker sendromlu hastalardaki isitme fonksiyonlarını değerlendirmektir. Sağlıker sendromu literatüre 2000 yılında girmis olan ve görülme sıklığı KBY hastalarında % 0.5 olarak bildirilen, geri kalmıs ülkelerde sık görülen yeni bir sendromdur. Sendroma neden olan faktörler henüz kesin olarak bilinmemekte ve bu konuda halen çalısmalar devam etmektedir. Bu sendromda kronik böbrek hastalığı ve bunun yetersiz tedavisine bağlı sekonder hiperparatiroidizm yanında, kafada sekil bozuklukları, kısa boy, maksiller ve mandibuler kemikte değisiklikler, dis anomalileri, ağız bosluğu içerisinde yumusak doku tümörleri, parmak uçlarında değisiklik gibi her türlü kemik anomalisi gelisebilir. Bu bulgulara psikolojik sorunlar, depresyon ve isitme kaybı eslik edebilir. Gereç ve Yöntem: Bu çalısmada, sağlıker sendromu nedeniyle takip edilen ve hemodiyaliz tedavisi almakta olan 12 hastada otoskopik muayene, Weber ve Rinne testleri, saf ses odyometrisi ve timpanometri test sonuçları sunuldu. Bulgular: Olguların 5 tanesinde (% 41.7) sensörinöral tipte isitme kaybı saptandı. Bu sonuç Sağlıker sendromunda isitme kaybı olasılığının hayli yüksek olduğunu düsündürmektedir Sonuç: Hastalarımızın tümünün hemodiyaliz tedavisi almakta olusu nedeniyle hemodiyaliz isitme kaybına yol açabilecek bir etken olarak düsünülebilir.

Anahtar Kelimeler

Sağlıker sendromu, işitme kaybı, hemodializ

Kaynakça

• Sagliker Y, Balal M, Sagliker Ozkaynak P, et al. Sagliker syndrome: uglifying human face appearance in late and severe secondary hyperparathyroidism in chronic renal failure. Semin Nephrology 2004;24:449-455.
• Sagliker Y, Acharya V, et al. International study on Sag- liker syndrome and uglifying human face apperance in severe and late secondary hyperparathyroidism in chronic kidney dis- ease. J Renal Nutr 2008;18:114-117.
• Uzel A, Uzel I, Sagliker Y, et al. Cephalometric evaluation of patients with Sagliker syndrome: uglifying human face ap- perance in severe and late secondary hyperparathyroidism in chronic renal failure patients. J Renal Nutr 2006;16:229-232.
• Giray S, Sagliker Y, et al. Neurologic manifestations in Sag- liker syndrome: uglifying human face apperance in severe and late secondary hyperparathyroidism in chronic renal failure patients. J Renal Nutr 2006;16:233-236.
• Gatland D, Tucker B, Chalstrey S, Keene M, Baker L. Hearing loss in chronic renal failure-hearing threshold changes following haemodialysis. J R Soc Med 1991:84:587-589.
• Ömür M, Tezel İ, Hizalan İ. Kronik böbrek yetmezliğine bağlı işitme kayıpları ve bazıserum içerikleri ile ilişkisi.Türk ORL Derneği XVİ. Ulusal Kongre Kitabı. Trabzon 1981;141
• Bast A, Goris RJA. Oxidative stress. Biochemistry and hu- man disease. Pharm Weekbl (Sci) 1989;11:199-206.
• Misra HP, Fridovich İ. Superoxide dismutase: Photochemi- cal ;181:308-312. Paynter Dİ. Changes in activity of the manganese superox- ide dismutase enzyme in tissues of the rarat with changes in dietary manganese. J Nutr 1986;110:437-447.
• Johnson DW, Mathog RH. Hearing function and chronic renal failure. Ann Otol 1976; 85:43-49.
• Ward J. Free radicals, antioxidants and preventive geriat- rics. Austr Family Physician 1994;23:1297-1301.
• Yassin A, Badry A, Fatt-Hi A. The relationship between electrolyte balance and cochlea disturbances in cases of renal failure. J Laryngol Otol 1970;84:429-436.
• Özçağlar HÜ, Dinç O, Fişenk F, Kılıçarslan S. Auditory evoked potentials of the chronic renal failure patients before and after hemodialysis. Proceedings of the XV World Con- gress of ORL, Head Neck Surgery 1993;391-394.
• Gedikli O, Delibaş, N, Doğru H, et al. Kronik böbrek yet- mezliğinde işitme kaybı ve antioksidanlar. KBB İhtisas Derg ;3:594-599. Özen M, Sandıkçı O, Kadıoğlu A, Aguşoğlu N. Audiome- try in chronic renal failure before and after intermittent hemo- dialysis. EDTA 1974;11:203-209.
• Paglia DE, Valentine WN. Studies on the quantitative cha- racterization of erythrocyte glutathione peroxidase. J Lab Clin Med 1979;70:158-168.
• Levander OA, DeLoach DP, Morris VC, et al. Platelet glu- tathione peroxidase activity as a index of selenium status in rats. J Nutr 1983;113:55-63.
• Laitakari H. Vestibular disorders in medically managed chronic renal insufficiency. Acta Otolaryngol (Suppl) ;349:7-10. Sagliker Y, Acharya V, et al. Is survival enough for quality of life in Sagliker syndrome - Uglifying human face apperance in chronic kidney disease, J Nephrol 2008; 21:114-118.
• Altaş E, Çetinkaya R, Kızıltunç A, Tonbul HZ, Üçüncü H, Çapoğlu İ. Kronik hemodiyaliz hastalarında işitme kaybı ve antioksidanlar. Türk Nefrol Diyaliz Transplant Derg ;2:97-101.