Round window atresia in association with congenital stapes fixation

Objective: To document an association of round window atresia with congenital stapes fixation, discuss the diagnostic role of computed tomography, and implicate the clinical significance of these anomalies. Study Design: Retrospective chart review and review of the literature. Results: The authors r...

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Bibliographic Details
Published in:The Laryngoscope 1998-08, Vol.108 (8), p.1115-1118
Main Authors: Pappas Jr, Dennis G., Pappas Sr, Dennis G., Hedlin, Gary
Format: Article
Language:English
Subjects:
Biological and medical sciences
Child
congenital stapes fixation
Ear, auditive nerve, cochleovestibular tract, facial nerve: diseases, semeiology
Hearing Loss, Conductive - etiology
Humans
Male
Medical sciences
Non tumoral diseases
Otorhinolaryngology. Stomatology
Retrospective Studies
Round window atresia
Round Window, Ear - abnormalities
Round Window, Ear - diagnostic imaging
Stapes - abnormalities
Stapes - diagnostic imaging
Stapes Surgery
Tomography, X-Ray Computed
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Summary:Objective: To document an association of round window atresia with congenital stapes fixation, discuss the diagnostic role of computed tomography, and implicate the clinical significance of these anomalies. Study Design: Retrospective chart review and review of the literature. Results: The authors report three pediatric cases of round window atresia. All cases presented with hearing loss of a conductive nature (two cases bilateral, one unilateral). Two patients underwent middle ear exploration, demonstrating stapes fixation and the absence of a round window. Stapedectomy was performed in both cases without postoperative hearing improvement. Subsequent revision stapedectomy was also unsuccessful in one of these cases. The unoperated case represents the fraternal twin of one patient. In all cases, computed tomography scanning demonstrated findings consistent with atresia of the round window niche without cochlear anomaly. Conclusions: An association of round window atresia and congenital stapes fixation is apparent. The mechanism of the conductive hearing loss following stapedectomy remains poorly understood. Computed tomography imaging and surgical confirmation are probably both necessary to establish the diagnosis of round window atresia.
ISSN:0023-852X
1531-4995
DOI:10.1097/00005537-199808000-00002