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dc.contributor.advisorSENNAROĞLU, GONCA
dc.contributor.authorÖZGEDİK, Dilek
dc.date.accessioned2019-07-10T09:01:20Z
dc.date.issued2019-07-08
dc.date.submitted2019-06-20
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dc.identifier.urihttp://hdl.handle.net/11655/7828
dc.description.abstractÖzgedik, D. Audiologic Findings in peesons with Glutaric Asiduri Type 1. Hacettepe University, Graduate School of Health Sciences, Programme of Audiology and Speech Disorders, PhD Thesis, Ankara, 2019.Glutaric Asiduri Type 1 is an organic type of asiduri which shows autosomal recessive properties and is caused by the defects in the enzyme required for mitochondria. The main objective of this study was to observe the audiologic findings of patients with Glutaric asiduri Type 1, which has limited scientific literature and thus very difficult to evaluate and diagnose. In the study, which consists of 17 patients diagnosed with glutaric asiduri Type 1 as the experiment group and 20 healthy individuals as the control group, all the subjects have been evaluated by using audiological test battery. Following the otoscopic examination, pure tone audiometry hearing tests, timpanometric evaluations, otoacustic emission and contra lateral suppression measurements, as well as the measurements of auditory brainstem response evaluations were carried out in all subjects. In the study group, distortion product emission amplitudes and the measured values of contralateral distortion product suppression were significantly lower than those of the control group (p<0,05). In the analysis of the study group, although they have normal hearing ability or a level close to the normal, it has been found that they have morphologically different auditory brainstem responses than the control group, which is considered as a remarkable finding. When the absolute latencies of auditory brainstem responses of the experiment group were examined, the wave absolute latencies of I, III and IV, as well as interpic latencies of I-V were observed to be prolonged significantly compared to the control group (p<0,05).As a result of we emphasise that the study person diagnosed GA-1 whose early detection and using audiologic test equipment beneficial for them. Key Words: Glutaric Asiduri Type 1, Hearing, Audiologic findingstr_TR
dc.description.tableofcontentsONAY SAYFASI iii YAYIMLAMA VE FİKRİ MÜLKİYET HAKLARI BEYANI iv ETİK BEYAN SAYFASI v TEŞEKKÜR vi ÖZET vii ABSTRACT viii İÇİNDEKİLER ix SİMGELER ve KISALTMALAR xii ŞEKİLLER xiv GRAFİKLER xv TABLOLAR xvi 1.GİRİŞ 1 2. GENEL BİLGİLER 3 2.1. Organik Asidemiler 3 2.1.1.Lizin, Hidroksilizin ve Triptofan Katabolizması 4 2.1.2.Glutarik Asidüri Tip 1 6 2.1.3. Glutarik Asidüri Tip 1’de Mitokondrial Fonksiyon Hasar Mekanizması 11 2.1.4. Koklear eksitotoksik süreç ve glutamat mekanizması 12 2.2. İşitme Anatomi ve Fizyolojisi 14 2.2.1. Kemik (Osseöz) Labirent 15 2.2.2. Zar (Membranöz) Labirent 15 2.2.3. Kokleayı besleyen arterler 15 2.2.4. İç Kulak Histolojisi 17 2.2.5. Kokleadaki Olivokoklear İlişkiler 23 2.2.6.Olivokoklear Nörotransmitterler 24 2.2.7.Olivokoklear Fizyoloji 25 2.3. Otoakustik Emisyonlar (OAE) 26 2.3.1. Uyarılmış (Evoked) Otoakustik Emisyonlar 27 2.3.2. Otoakustik Emisyon ve Kontralateral Supresyon (KLS) 28 2.4. Timpanometri ve Akustik Refleks 29 2.5. İşitsel Beyin Sapı Cevabı (İBC) 30 3. BİREYLER ve YÖNTEM 31 3.1. Bireylerin Seçim Kriterleri 31 3.2. Yöntem 32 3.2.1. Odyolojik Değerlendirme 33 3.2.3 Otoakusik Emisyon Ölçümü 33 3.2.4. Diagnostik İBC Testinin Yapılışı ve Değerlendirilmesi 34 3.3. İstatistiksel Analiz 35 4. BULGULAR 36 4.1.Bireylere Ait Demografik Bulgular 36 4.2. Odyolojik Bulgular 36 4.2.1. Saf Ses Odyometrisi 37 4.3. DPOAE Bulguları 41 4.3.1. Asemptomatik ve Semptomatik grupların DPOAE Bulguları 43 4.4. Kontralateral Supresyon Bulguları 44 4.5. İşitsel Beyinsapı Cevabı (İBC) Bulguları 46 4.5.1. Asemptomatik ve Semptomatik gruplar arasında İBC bulguları 49 4.6.İşitsel Beyinsapı Cevabı İnterpeak Latansları 51 5. TARTIŞMA 53 6. SONUÇ ve ÖNERİLER 63 7. KAYNAKLAR 66 8. EKLER EK-1: Tez Çalışması ile İlgili Etik Kurul İzni EK-2 Araştırma Amaçlı Çalışma İçin Çocuk Rıza Formu EK-3 Araştırma Amaçlı Çalışma İçin Çocuk Rıza Formu EK-4 Yetişkin Veri Toplama Formu EK-5 Çocuk Veri Toplama Formu EK-6 Turnitin Ekran Görüntüsü EK-7 Dijital Makbuz 9. ÖZGEÇMİŞtr_TR
dc.language.isoturtr_TR
dc.publisherSağlık Bilimleri Enstitüsütr_TR
dc.rightsinfo:eu-repo/semantics/openAccesstr_TR
dc.subjectGlutarik Asidüri Tip I, İşitme, Odyolojik Bulgulartr_TR
dc.subject.lcshKonu Başlıkları Listesi::Tıptr_TR
dc.titleGlutarik Asidüri Tip 1'li Bireylerde Odyolojik Bulgulartr_TR
dc.typeinfo:eu-repo/semantics/doctoralThesistr_TR
dc.description.ozetÖzgedik, D. Glutarik Asidüri Tip I’li Bireylerde Odyolojik Bulgular. Hacettepe Üniversitesi Sağlık Bilimleri Enstitüsü Odyoloji ve Konuşma Bozuklukları Programı Doktora Tezi, Ankara, 2019.Glutarik Asidüri Tip I; otosomal resesif özellik gösteren, mitokondri için gerekli olan enzimdeki kusurdan kaynaklı organik bir asidüri tipidir. Çalışmamızda zor değerlendirilen ve tanılanan, çok kısıtlı literatüre sahip Glutarik Asidüri Tip I’li hastalarının odyolojik bulgularını gözlemlemeyi hedefledik. Glutarik Asidüri Tip I tanısı almış 17 hasta ve 20 sağlıklı bireyden oluşan kontrol grubu odyolojik test bataryası ile değerlendirilmiştir. Tüm bireylere otoskopik muayenenin ardından saf ses işitme testleri, timpanometrik değerlendirme, otoakustik emisyon ve kontralateral supresyon ölçümleri ile işitsel beyinsapı cevabı değerlendirmeleri yapılmıştır. Glutarik Asidüri Tip I’li grubun Distortion Product Emisyon amplitüdleri ve Kontralateral Distortion Product Supresyon ölçüm değerleri kontrol grubundan anlamlı derecede düşüktür (p<0,05). Çalışma grubu bireylerinin yapılan değerlendirmelerinde normal/normale yakın işitmeye sahip oldukları halde, kontrol grubundan morfolojik olarak farklı işitsel beyinsapı cevaplarının olması dikkat çekici bulunmuştur. Çalışma grubunun işitsel beyinsapı cevaplarının absolut latansları incelendiğinde I, III ve V. dalga absolut latansları ve I-V interpeak latanslarının kontrol grubuna göre istatistiksel olarak anlamlı ölçüde uzamış olduğu gözlemlenmiştir (p<0,05). Çalışma sonunda GA-1’ li bireylerin erken tanılanması ve odyolojik bataryanın kullanılmasının yararlı olacağı düşüncesindeyiz. Anahtar kelimeler: Glutarik Asidüri Tip I, İşitme, Odyolojik Bulgulartr_TR
dc.contributor.departmentOdyolojitr_TR
dc.embargo.terms6 aytr_TR
dc.embargo.lift2020-01-12T09:01:20Z
dc.identifier.ORCIDhttps://orcid.org/0000-0003-2690-2750tr_TR


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