Sensörinöral İşitme Kayıpları
Özet
Sensörinöral işitme kaybı (SNİK), iç kulaktaki sesin algılanması ve vestibülokoklear sinirden santral işitme merkezine kadar olan işitme yollarındaki patolojilere bağlı gelişen bir işitme kaybı tipidir. Bu klinik durum, kazanılmış (edinsel) ve konjenital olmak üzere iki grupta sınıflandırılır. Konjenital SNİK vakalarının yaklaşık %50'si genetik kaynaklı olup, sendromik ve sendromik olmayan (en sık GJB2 gen mutasyonu) şeklinde ayrılırken; genetik olmayan grupta prenatal TORCHS enfeksiyonları, düşük doğum ağırlığı ve hiperbilirubinemi gibi perinatal faktörler etkilidir. Ayrıca, Sennaroğlu sınıflamasına göre incelenen iç kulak kemik anomalileri de konjenital vakaların %20'sini oluşturur. Edinsel SNİK nedenleri arasında en sık rastlanılan yaşa bağlı presbiakuzi (sensöriyel, nöral, metabolik, mekanik), kronik gürültüye bağlı işitme kaybı, aminoglikozid veya sisplatin gibi ajanların tetiklediği ototoksisite, endolenfatik hidrops ile seyreden Meniere hastalığı, idiyopatik ani işitme kaybı, bakteriyel menenjit enfeksiyonları, serebellopontin köşe tümörleri (akustik nörinom), temporal kemik kırıkları ve otoimmün iç kulak hastalıkları bulunur. Tedavi yaklaşımında etiyolojinin netleştirilmesi esas olup, duruma göre doz ayarlı kortikosteroidler, hiperbarik oksijen, işitme cihazları, Sağlık Uygulama Tebliği kriterlerine göre koklear implantasyon veya koklear sinir aplazisinde işitsel beyin sapı implantı (ABI) tercih edilmektedir.
Sensorineural hearing loss (SNHL) is a type of hearing loss caused by pathologies in the sound perception mechanisms of the inner ear and the sensory and neuronal pathways extending from the vestibulocochlear nerve to the central auditory center. This clinical condition is classified into two groups as congenital and acquired. Approximately 50% of congenital SNHL cases are genetic, divided into syndromic and non-syndromic forms (most commonly GJB2 gene mutation), while prenatal TORCHS infections and perinatal factors like low birth weight and hyperbilirubinemia are influential in the non-genetic group. Additionally, inner ear bony anomalies, categorized by the Sennaroglu classification, constitute 20% of congenital cases. Acquired SNHL causes include age-related presbyacusis (sensory, neural, metabolic, mechanical), which is the most common cause, noise-induced hearing loss, ototoxicity triggered by agents like aminoglycosides or cisplatin, Meniere's disease presenting with endolymphatic hydrops, idiopathic sudden hearing loss, bacterial meningitis infections, cerebellopontine angle tumors (acoustic neuroma), temporal bone fractures, and autoimmune inner ear diseases. In the treatment approach, clarifying the etiology is essential; depending on the condition, dose-adjusted corticosteroids, hyperbaric oxygen, hearing aids, cochlear implantation according to healthcare guidelines, or auditory brainstem implants (ABI) in cases of cochlear nerve aplasia are utilized.
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