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Clinical Note
May 2004

Massive Endolymphatic Sac and Vestibular Aqueduct in Mondini Dysplasia

Author Affiliations

From the International Hearing Foundation (Drs Sampaio, Cureoglu, and Kusunoki and Ms Schachern), the Department of Otolaryngology, Otitis Media Research Center, University of Minnesota (Dr Cureoglu and Ms Schachern), and the Minnesota Ear Head and Neck Clinic (Dr Paparella), Minneapolis; and Brasilia University Medical School, Brasilia, Brazil (Drs Sampaio and Oliveira). The authors have no relevant financial interest in this article.

Arch Otolaryngol Head Neck Surg. 2004;130(5):678-680. doi:10.1001/archotol.130.5.678

A postmortem study was performed on the bilateral temporal bones of a 90-year-old woman who had a lifelong profound hearing impairment. The histopathologic findings included severe Mondini dysplasia with unusual enlargement of the vestibular aqueducts and endolymphatic sacs. Dehiscent carotid arteries and dehiscent facial nerves were also present. The clinical and surgical aspects of the case are discussed based on the histopathologic findings. The physician should be prepared to diagnose congenital hearing impairment promptly to avoid complications during the clinical and surgical treatment of patients with Mondini dysplasia.

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