Familial congenital facial palsy.

Congenital unilateral facial palsy has been described as a distinct entity, but there have been few reports of an isolated unilateral familial form, and no reports with supportive neuroimaging. We studied three males from three generations in the same family. Each had a left facial palsy, which was more pronounced with every successive generation. On the House grading scale, the proband, a 9-year-old male, manifested moderately severe facial nerve dysfunction. His 40-year-old father had mild to moderate dysfunction, and his 61-year-old paternal grandfather manifested mild dysfunction. The proband and his father both had abnormal magnetic resonance imaging studies, with the proband's revealing enlargement and enhancement of the tympanic portion of the left facial nerve, and the father's demonstrating enhancement. Temporal bone computed tomography on the 9-year-old confirmed the enlarged left facial nerve. By electromyography/nerve conduction studies there was evidence of a chronic, incomplete, left facial palsy affecting both the father and his son, with substantially lowered compound muscle action potential amplitudes on the left. Congenital unilateral facial palsy spanning three generations with supportive neuroimaging and electromyography/nerve conduction studies has not been previously reported in the literature.