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Transmastoid Facial Nerve Decompression for Craniometaphyseal Dysplasia
Andrew S Lee1, Bing M Teh2, George Alexiades3
1Weill-Cornell School of Medicine, New York, New York.
Objective:
We document the first successful transmastoid surgical treatment of facial nerve palsy for a patient with craniometaphyseal dysplasia (CMD), a rare genetic disease.
Patient:
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.
Intervention:
Right transmastoid facial nerve decompression and ossicular chain reconstruction.
Main Outcome Measure:
Facial nerve function (House-Brackmann grade).
Results:
Facial nerve function initially worsened, then improved within 12 months from House-Brackmann grade IV-V to grade III.
Conclusion:
Surgical cranial nerve decompression of and ossicular chain reconstruction may be effective treatments for patients with CMD.
