A case of congenital bilateral stapes agenesis

Yıl 2003, Cilt: 11 Sayı: 6, 175 - 178, 24.12.2003

Gürkan Keskin Emre Üstündağ Ahmet Almaç

Öz

Congenital absence of the stapes and the oval window is an anomaly reported in only sporadic cases. We present a 17-year-old male patient with congenital bilater­ al conductive hearing loss. The external auditory canal and the tympanic membrane appeared normal on both sides. An exploratory tympanotomy in the right ear revealed dehiscence of the the horizontal segment of the facial nerve, which was displaced inferiorly, occupying the area of the absent oval window. The stapes was totally absent and a malformed long process of the incus was attached by a fibrous band to the promontory. Manipulation of the facial nerve in association with stapedectomy or vestibulotomy was avoided in order notto injure the nerve. Instead, amplification with hear­ ing aids was recommended to the patient.

Anahtar Kelimeler

Branchial region/anatomy & histology, ear canal/ abnormalities, ear ossicles/abnormalities, ear, middle, facial nerve/abnormalities/embryology, hearing loss, conductive/ diagnosis/etiology, stapes/abnormalities/embryology

Doğuştan iki taraflı stapes agenezisi: Olgu sunumu

Öz

Doğufltan oval pencere ve stapes yokluğu çok na- dir bildirilmifl bir anomalidir. Bu makalede doğum- dan itibaren iki taraflı iletim tipi iflitme kaybı olan 17 yaflındaki bir hasta sunuldu. Hastanın dıfl kulak yo- lu ve timpanik membranın görüntüsü iki tarafta da normaldi. Sağ eksploratif timpanotomi yapıldığında fasyal sinirin horizontal segmentinin açık olduğu ve aflağıya deplase olarak oval pencere alanını tama- men kapladığı gözlendi. Stapesin olmadığı ve mal- forme olan inkus uzun kolunun fibröz bir bant ile promontoryuma bağlandığı görüldü. Fasyal sinire zarar vermemek düflüncesiyle, sinirin düzeltilmesi ve stapedektomi veya vestibülotomi yapılmasından kaçınıldı ve hastaya iflitme cihazı ile amplifikasyon ö n e r i l d i

Anahtar Kelimeler

Brankiyal bölge/anatomi ve histoloji, kulak kanalı/anormallik, kulak osikülü/anormallik, kulak, orta, fasyal sinir/anormallik/embriyoloji, iflitme kaybı, iletimtipi/tanı/etyoloji, stapes/anormallik/embriyoloji

Kaynakça

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