DOI: 10.1097/mao.0000000000004010 ISSN:
Transmastoid Facial Nerve Decompression for Craniometaphyseal Dysplasia
Andrew S. Lee, Bing M. Teh, George Alexiades- Neurology (clinical)
- Sensory Systems
- Otorhinolaryngology
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.