Enlarged vestibular aqueduct syndrome mimicking otosclerosis in adults

Abstract Introduction An enlarged vestibular aqueduct (EVA) results in hearing loss which is often progressive and heterogenous, the long-term natural history of which is not well understood. Patients born before the era of newborn hearing screening can present as adults with previously undiagnosed...

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Bibliographic Details
Published in:American journal of otolaryngology 2013-11, Vol.34 (6), p.619-625
Main Authors: Wieczorek, Stephanie S., BS, Anderson, Martin E., MD, Harris, Dave A., PhD, Mikulec, Anthony A., MD, FACS
Format: Article
Language:English
Subjects:
Adult
Audiometry
Delayed Diagnosis
Diagnosis, Differential
Ears & hearing
Female
Hearing aids
Hearing loss
Hearing Loss, Mixed Conductive-Sensorineural - etiology
Humans
Male
Middle Aged
Occupational safety
Otolaryngology
Otosclerosis - diagnosis
Reflex, Abnormal - physiology
Reflex, Acoustic - physiology
Retrospective Studies
Stapes - physiopathology
Surgery
Tomography, X-Ray Computed
Vestibular Aqueduct - abnormalities
Vestibular Aqueduct - diagnostic imaging
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Summary:Abstract Introduction An enlarged vestibular aqueduct (EVA) results in hearing loss which is often progressive and heterogenous, the long-term natural history of which is not well understood. Patients born before the era of newborn hearing screening can present as adults with previously undiagnosed EVA. Methods A retrospective chart review of patients seen at a tertiary adult academic otologic clinic from 2004 to 2012 were reviewed and cases of EVA were identified. Results Adult EVA was found to present with a mixed progressive hearing loss with absent stapedial reflexes. Conclusion Enlarged vestibular aqueduct abnormality can present in adulthood and mimic other more common pathology such as otosclerosis.
ISSN:0196-0709
1532-818X
DOI:10.1016/j.amjoto.2013.07.015