Duane's syndrome associated with crocodile tear and ear malformation. A case of stapes anomaly and its reconstruction.
Duane's syndrome is a congenital eye movement disorder characterized by marked limitation or absence of abduction, variable limitation of adduction, and narrowing of the palpebral fissure with retraction of the globe on attempted adduction. We treated a patient with bilateral Duane's syndrome associated with crocodile tear and external, middle, and inner ear anomalies. Exploratory tympanotomy showed an absence of the oval window and a rudimentary superior structure of the stapes that was connected to the abbreviated incudal lenticular process with connective tissue. A small hole was drilled into the vestibulum and the ossicular chain was reconstructed by inserting a Teflon wire piston to minimize the air-bone gap. Pertinent literature is reviewed, and the pathogenesis of Duane's syndrome and the aspect of concomitant anomalies are discussed.
D000015:Abnormalities, Multiple; D000328:Adult; D004370:Duane Retraction Syndrome / Q000150:complications*; D004429:Ear Ossicles / Q000002:abnormalities*; D004431:Ear, External / Q000002:abnormalities; D007758:Ear, Inner / Q000002:abnormalities; D004435:Eating; D005260:Female; D006312:Hearing Loss, Bilateral / Q000209:etiology; D006801:Humans; D009886:Ophthalmoplegia / Q000150:complications*; D012021:Reflex, Abnormal; D013199:Stapes / Q000002:abnormalities*; D013201:Stapes Surgery; D013666:Tears / Q000378:metabolism*