Imaging and clinical evaluation of isolated atresia of the oval window

Timothy N. Booth, Louis G. Vezina, Gerald Karcher, Elizabeth C. Dubovsky

Research output: Contribution to journalArticle

21 Citations (Scopus)

Abstract

BACKGROUND AND PURPOSE: Congenital causes of hearing loss in children commonly are encountered, and imaging aids in diagnosis as well as presurgical evaluation. Atresia of the oval window not associated with atresia of the external auditory canal (EAC) is a rare cause of congenital hearing loss in children. We present the clinical and imaging findings in children with isolated oval-window atresia. METHODS: Atresia of the oval window was defined as the absence of the structure with the presence of a bony plate superimposed between the vestibule and middle ear. The bony plate is within the expected region of the oval window. Using a computerized database, nine patients with isolated oval-window atresia were found. All had been evaluated with high-resolution computed tomography (HRCT) and all had medical records available for review, including audiogram results. Imaging studies were interpreted by the consensus of two pediatric neuroradiologists. RESULTS: Atresia of the oval window was documented in all cases using HRCT criteria. The most common anomalies associated with oval-window atresia were inferomedial malposition of the facial nerve (n = 8), malformed incus (n = 6), and displaced stapes (n = 2). Four patients had symmetric bilateral involvement. Hearing tests were not specific, because conductive, sensorineural, and mixed patterns were found. CONCLUSION: Anomalies of the oval window should be sought in all patients with congenital hearing loss. Associated findings, such as facial nerve aberrancy and ossicular anomalies, are important in both diagnosis and surgical planning.

Original languageEnglish (US)
Pages (from-to)171-174
Number of pages4
JournalAmerican Journal of Neuroradiology
Volume21
Issue number1
StatePublished - 2000

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Hearing Loss
Facial Nerve
Tomography
Labyrinth Vestibule
Incus
Hearing Tests
Stapes
Ear Canal
Middle Ear
Medical Records
Databases
Pediatrics

ASJC Scopus subject areas

  • Clinical Neurology
  • Radiology Nuclear Medicine and imaging
  • Radiological and Ultrasound Technology

Cite this

Imaging and clinical evaluation of isolated atresia of the oval window. / Booth, Timothy N.; Vezina, Louis G.; Karcher, Gerald; Dubovsky, Elizabeth C.

In: American Journal of Neuroradiology, Vol. 21, No. 1, 2000, p. 171-174.

Research output: Contribution to journalArticle

Booth, TN, Vezina, LG, Karcher, G & Dubovsky, EC 2000, 'Imaging and clinical evaluation of isolated atresia of the oval window', American Journal of Neuroradiology, vol. 21, no. 1, pp. 171-174.
Booth, Timothy N. ; Vezina, Louis G. ; Karcher, Gerald ; Dubovsky, Elizabeth C. / Imaging and clinical evaluation of isolated atresia of the oval window. In: American Journal of Neuroradiology. 2000 ; Vol. 21, No. 1. pp. 171-174.
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