Abstract
We document the first successful transmastoid surgical treatment of facial nerve palsy for a patient with craniometaphyseal dysplasia (CMD), a rare genetic disease. A 9-month-old girl with bilateral facial nerve palsies and conductive hearing loss. Genetic testing made a diagnosis of CMD, and imaging showed narrowing of the facial nerve canals and ossicular fixation. Right transmastoid facial nerve decompression and ossicular chain reconstruction. Facial nerve function (House-Brackmann grade). Facial nerve function initially worsened, then improved within 12 months from House-Brackmann grade IV-V to grade III. Surgical cranial nerve decompression of and ossicular chain reconstruction may be effective treatments for patients with CMD.
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Schedule a callMore From: Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology
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