Oval window agenesis with obvious craniofacial ear deformity is a rarely diagnosed condition. This finding is noticeable nowadays as a result of the development of microsurgery of the ear. The vestibule is the central chamber of the bony labyrinth of the inner ear. It has a lateral opening at the medial wall of the middle ear, which is called the oval window (fenestra ovale), and lies above and slightly behind the promontory, and below the horizontal portion of the facial nerve. It is closed in life by the footplate of the stapes and its annular ligament. It has an area of approximately 3x1.4 mm 2 and a depth of 2.5-3 mm.
2The stapes has a double origin. The main structures derive from the second branchial arch, but the inner (vestibular) aspect of the footplate differentiates from the otic capsule itself. This area is termed the "lamina stapedialis." As development proceeds, the cells immediately adjacent to this lamina normally form the annular ligament, and the lamina becomes incorporated into the footplate of the stapes. Teratogenic agents might affect any stage of the process, producing anomalies including congenital absence of the oval window and others.3 The oval window is indispensable for the transmission of sound waves into the inner ear (cochlea). In the case of oval window occlusion or absence, the stapes is not normally developed and may be rudimentary, hanging above an undifferentiated oval window niche, causing conductive loss of hearing. The adjacent lateral wall of the bony canal of the facial nerve is also developed from the second branchial arch.
4,5The facial nerve may be exposed or displaced downwards. Congenital facial nerve dehiscence of the bony facial canal in the oval window region, a very common finding at tympanotomy, was present at 31% of normal temporal bones examined by Leonard and Alexander in 1968. 6 Differentiation of the otocyst and otic capsule to inner ear is developed independently of the middle ear, which explains the presence of agenesis of footplate as a separate anomaly, and the presence of normal inner ear with deformity of the ossicular chain.5 If the stapes is totally absent, there may be no depression or other indication of the site of the vestibular window, and the facial nerve may be displaced downwards.
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Case ReportA 24-year-old Jordanian female presented to the Otorhinolaryngology Department at King Hussein Medical Centre, Amman, Jordan, in 1995, with a history of bilateral loss of hearing since childhood. There was no positive family history of hearing loss or history of trauma. She was a good lip reader. ENT examination revealed normal tympanic membranes with outstanding small auricles (microtia). Pure tone audiogram showed bilateral, almost symmetrical conductive loss of hearing of 45 dB (Figure 1). Cochlear function and caloric test were normal. Tympanogram showed normal middle ear pressure, and compliance was within the wide normal variation range.After the condition was explained to the patient, she was asked to decide between having a hearing aid fit...