Konjenital stapes/oval pencere anomalisi

Özet

Konjenital oval pencere atrezisi veya stapes displazisi, çocuklukta ya da erişkinlikte iletim tipi işitme kaybının nadir gelişimsel nedenleri arasında yer alır. İnce kesit, kontrastsız temporal kemik BT; oval pencere nişini, stapesin küçük kruralarını ve komşu fasiyal sinir kanalını kemik ayrıntısıyla gösterir. Oval pencere kapanması stapes anomalisiyle, bazen de aşağı yerleşimli veya açıkta seyreden timpanik fasiyal sinir kanalıyla birliktedir. Bu anatominin atlanması doğuştan anomalinin edinsel hastalık sanılmasına ve cerrahi yaklaşımda sinir seyrinin hesaba katılmamasına neden olabilir.

Faz ve pencere

Kontrastsız tanısal

Yüksek çözünürlüklü temporal kemik BT, orta kulağın kemik yapılarını ve fasiyal sinir kanalındaki anomalileri değerlendirmede kullanılır.

BT bulguları

• Oval pencere atrezisi veya displazisinde nişte kemik lameller görülebilir ya da pencere aplazik veya ileri derecede displazik olabilir.
• İzole konjenital kemikçik deformiteleri içinde en sık stapes süperstrüktürü ve inkusun uzun çıkıntısı tutulur.
• Stapes anomalisi — Oval pencere atrezisine stapes malformasyonu veya yokluğu eşlik edebilir.
• Timpanik fasiyal sinir kanalının konum anomalisi — Fasiyal sinir oval pencerenin altında yerleşebilir veya oval pencereyi örtebilir; kanalın kemik örtüsünde açıklık da bulunabilir.
• Oval pencere atrezisine fasiyal sinir seyir anomalisi eşlik edebilir; sinirin oval pencerenin altında yerleşmesi veya pencereyi örtmesi BT'de araştırılmalıdır.
• Eşlik eden kemikçik anomalisi — inkusun lentiküler çıkıntısı ya da uzun kolu hipoplazik, yok veya aşağı yerleşimli olabilir.
• Karşı kulak ve dış kulak yolu — iki taraflı pencere/stapes gelişim kusuru veya dış kulak yolu darlığı-atrezisi gibi ek gelişimsel bulgular bulunabilir.
• Oval pencere atrezisine stapes malformasyonu veya yokluğu eşlik edebilir; stapes kruraları da değerlendirilmelidir.
• Konjenital kemikçik anomalileri Teunissen–Cremers yaklaşımında izole stapes ankilozu; diğer kemikçik anomalileriyle stapes ankilozu; hareketli stapes tabanıyla kemikçik zincir deformitesi; oval veya yuvarlak pencerenin ağır aplazi/displazisi gruplarında ele alınabilir.

Normalde

Oval pencere ve stapes gibi orta kulak kemik yapıları HRCT'de değerlendirilebilir. Timpanik fasiyal sinirin oval pencereye göre seyri değişken olabilir; HRCT'de bu ilişkiyi değerlendirin.

Ayırıcı tanı

Fenestral otoskleroz

fissula ante fenestra çevresinde fokal hipodens odak veya stapes tabanı çevresinde otik kapsül remodelingi görülür; gelişimsel niş kapanması ve eşlik eden stapes displazisi beklenen patern değildir.

Timpanosklerozda orta kulak veya timpanik membran tutulumu görülebilir; oval pencere atrezisi ise doğuştan bir gelişim kusurudur.

Konjenital stapes fiksasyonu

oval pencere açıklığı ve stapes tabanı mevcut olabilir; yalnız kemik BT ile hareket kısıtlılığı doğrudan kanıtlanamaz.

Orta kulak efüzyonu, çocuklarda iletim tipi işitme kaybının en sık nedenidir ve konjenital orta kulak anomalileriyle karışabilir.

Kemikçik zincir travması

Yüksek çözünürlüklü BT travmaya bağlı kemikçik bozulmalarını gösterebilir; konjenital orta kulak anomalileri de kemik yapıların yüksek çözünürlüklü BT ile değerlendirilmesinde incelenir.

Otosklerozda HRCT'de oval pencerenin önünde hipodens odak görülebilir; konjenital oval pencere atrezisi BT'de gösterilebilir ve stapes veya fasiyal sinir anomalileriyle birlikte bulunabilir.

Tuzaklar

• Stapesin kemik yapılarının değerlendirilmesinde yüksek çözünürlüklü BT tercih edilir; kesin tanıyı en güvenilir biçimde eksploratuvar timpanotomi koyar.
• Orta kulak anomalileri seröz otitle karışabilir; orta kulak opasifikasyonu stapesi örtebileceğinden, stapesin seçilememesini yokluk olarak yorumlamayın.
• Oval pencerenin altında yerleşen fasiyal sinir, stapes cerrahisinde kullanılabilecek teknikleri etkileyebilir; ameliyat öncesinde sinirin seyri BT'de değerlendirilmelidir.
• Oval pencere atrezisi erişkinlerde otosklerozu klinik olarak taklit edebilir; yüksek çözünürlüklü BT'de atretik pencerenin aranması ayırıcı tanıya katkı sağlar.

Kendini dene

1. Teunissen–Cremers sınıflamasında oval veya yuvarlak pencerenin ağır aplazisi ya da displazisi hangi sınıftadır?

   • Sınıf 1: izole stapes ankilozu
   • Sınıf 2: ankiloz + kemikçik anomalisi
   • Sınıf 3: hareketli taban, zincir deformitesi
   • Sınıf 4: pencere aplazisi/displazisi
   Cevabı göster

   Sınıf 4: pencere aplazisi/displazisi. Sınıflama, oval veya yuvarlak pencerenin ağır aplazi ya da displazisini 4. gruba yerleştirir.

2. S1'de bildirilen birinci olgunun yüksek çözünürlüklü BT'sinde oval pencere ve stapes için hangi bulgu saptanmıştır?

   • Her ikisinin yokluğu
   • Oval pencerenin açık, üzenginin normal olması
   • Yalnızca oval pencerenin dar olması
   • Yalnızca üzengi başının yer değiştirmesi
   Cevabı göster

   Her ikisinin yokluğu. Kaynakta bildirilen olguda yüksek çözünürlüklü BT, oval pencere ve üzenginin yokluğunu göstermiştir.

İlgili konular

• Ossiküler zincir fiksasyonu/erozyonu

Kaynaklar

Bu sayfadaki 36 cümle ve 2 quiz sorusu aşağıdaki kaynaklardan alıntılarla otomatik olarak karşılaştırıldı; 23 cümle bu karşılaştırmada düzeltildi (2026-10-09).

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Eğitim amaçlıdır; hasta başında tanı ve tedavi kararının yerine geçmez, tıbbi tavsiye değildir. Metinler yapay zekâ desteğiyle yazıldı ve otomatik bir adımda kaynak alıntılarıyla karşılaştırıldı; bu bir tıbbi doğrulama değildir ve içerik uzman radyolog incelemesinden geçmedi. Klinik kararlarda güncel kılavuzları, kurum protokollerini ve radyoloji raporunu esas alın.