Otoskleroz (Otoloji/Nörootoloji)
Özet
Otoskleroz, sadece temporal kemikte görülen, otik kapsülün kemik rezorbsiyonu ve yeni kemik oluşumu ile seyreden anormal kemik yeniden yapılanması hastalığıdır. Etyolojisi net olmamakla birlikte genetik ve çevresel faktörlerin (özellikle kızamık virüsü) etkili olduğu düşünülmektedir. Klinik olarak kadınlarda daha sık, genellikle 3. dekatta bilateral ve ilerleyici işitme kaybı ile tinnitus şeklinde ortaya çıkar. Hastaların gürültülü ortamlarda daha iyi duyması Willis Parakuzisi olarak adlandırılır. Odyogramda düşük frekanslı iletim tipi işitme kaybı ve 2000 Hz'de Carhart Çentiği tipiktir. İleri evrelerde kokleanın etkilenmesiyle mikst tip işitme kaybı gelişebilir. Bilgisayarlı tomografide oval pencere anteriorunda hipodens odaklar veya halo işareti saptanabilir. Kesin tanı cerrahi sırasında stapes fiksasyonunun gösterilmesiyle konur. Tedavide hastalığı tamamen iyileştiren bir seçenek yoktur; ilerlemeyi yavaşlatmak için sodyum florid veya bifosfonatlar gibi medikal tedaviler uygulanabilir ya da işitme cihazı tercih edilebilir. En etkili tedavi yöntemi, fikse stapes tabanına delik açılarak piston protez yerleştirilen stapedotomi operasyonudur. Cerrahi sonrasında hastaların büyük kısmında hava-kemik aralığı kapanır. Ameliyatın en ciddi komplikasyonu sensörinöral işitme kaybıdır; ayrıca yüzen taban, korda timpani hasarı ve postoperatif vertigo gibi riskler bulunur. Çok ileri olgularda koklear implantasyon uygulanabilir.
Otosclerosis is a primary bone remodeling disease of the otic capsule confined to the temporal bone, characterized by bone resorption and new bone formation. Although its definitive etiology remains unknown, genetic predisposition and environmental factors, particularly the measles virus, are implicated. Clinically, it predominantly affects females, presenting in the third decade with bilateral, progressive hearing loss and tinnitus. Patients frequently experience improved speech perception in noisy environments, a phenomenon known as Paracusis of Willis. Audiological evaluation typically reveals a low-frequency conductive hearing loss and a characteristic drop at 2000 Hz in bone conduction thresholds, termed Carhart's Notch. Advanced stages involving the cochlear endosteum result in mixed hearing loss. High-resolution computed tomography may demonstrate hypodense areas anterior to the oval window or the halo sign in cochlear involvement. Definitive diagnosis is established by confirming stapes fixation during exploratory tympanotomy. Management options include hearing aids or medical therapies like sodium fluoride and bisphosphonates to slow progression, but the gold standard remains surgical intervention. Stapedotomy, involving a micro-fenestration on the stapes footplate for a piston prosthesis, provides high success rates in closing the air-bone gap. Sensorineural hearing loss constitutes the most severe surgical complication, alongside risks like floating footplate, chorda tympani injury, and postoperative vertigo.
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