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Congenital Facial Paralysis Workup

  • Author: Alan D Bruns, MD, FACS; Chief Editor: Arlen D Meyers, MD, MBA  more...
 
Updated: Mar 04, 2016
 

Laboratory Studies

The workup for congenital facial paralysis does not involve any particular routine battery of lab tests. If the mother has a history of viral infection perinatally, viral titers (eg, herpes simplex virus) and a TORCH screen could be considered, but the probability of one of these infections causing a facial paralysis is low. If a neonate appears syndromic, then chromosomal analysis with technology such as florescent in situ hybridization (FISH) should be considered. In these infants with complete nerve facial palsy, an investigation for chromosome 22q11 deletions is recommended.[18] Molecular testing for CHD7 mutations may help to confirm the diagnosis and differentiate it from the 22q11.2 deletion syndrome.[28] Careful audiologic evaluation with an auditory brainstem response in these patients, and those patients with FSH MD, is advised so that a sensorineural hearing loss can be ruled out.[20]

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Imaging Studies

See the list below:

  • Conventional neuroimaging does not usually contribute to the understanding the pathogenic mechanisms of congenital unilateral facial nerve palsy except in the case of a very rare large pontine lesion,[29] mastoid tumor,[30] or internal auditory canal stenosis.[31] However, congenital bilateral facial nerve palsy is usually accompanied by other congenital disorders that can be identified.[32]
  • A CT scan of the temporal bone in both axial and coronal views may be considered in infants with complete paralyses from trauma that do not resolve and, thus, surgery is being considered. A temporal bone fracture or any bony spicules within the facial canal may be demonstrated. Associated anomalies of the external ear, middle ear, inner ear, mandible, and the vertical portion of the facial nerve would suggest a developmental etiology of the paralysis.
  • An MRI study provides better definition of the nerve and the surrounding soft tissue. Aplasia or hypoplasia of the nerve may be apparent; these findings strongly suggest a developmental anomaly. In addition, a hematoma or surrounding soft tissue swelling may be present when the paralysis is associated with trauma. This may be enhanced with a 3D-CISS MRI.[32]
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Other Tests

Electrophysiology tests of facial nerve function can be useful to determine the extent of nerve disruption and to assist with future surgical planning.

  • Electroneuronography (EnoG) is usually the study of choice.
    • This test involves a quantitative analysis of the extent of degeneration. It is not dependent upon the observer.
    • The summation potential is recorded.
    • If more than 90% degeneration has occurred in traumatic congenital facial paralysis consider surgical decompression. (In newborns, waiting 5 weeks is prudent.)
    • An EnoG within 48 hours of a congenital traumatic injury typically reveals normal facial nerve function, whereas, in congenital developmental paralysis, the initial EnoG reveals facial nerve function to be absent or weak because of longstanding neural degeneration or nerve absence.[9]
  • Nerve excitability test (NET)
    • This test compares current thresholds required to elicit minimal muscle contraction on the normal side with that of the weak side.
    • A difference of 3.5 µA is significant.
  • Maximal stimulation test (MST)
    • This test is similar to NET but uses maximal stimulation. It is valuable for determining the status of neuromuscular units.
    • If nerve conduction is neurapraxic, response is positive; if nerve conduction is degenerated, response is absent.
    • Sectioned nerve can still be stimulated for 24-72 hours after injury; thus, the test cannot be interpreted until 3 days later.
    • The test is graded subjectively (equal, decreased, absent).
  • Electromyography (EMG)
    • This test determines the amount of activity of muscle itself. It records motor unit potentials of voluntary and involuntary muscle contraction, as well as spontaneous muscle fiber activity.
    • Degeneration of lower motor neuron is followed by fibrillation potentials at 14-21 days.
    • Polyphasic potentials can be observed 6-12 weeks before clinical improvement.
  • Topodiagnostic studies: Not performed routinely in the workup for congenital facial paralysis.
    • Schirmer test: This test evaluates function of the greater superficial petrosal nerve (lacrimation). A reduction of more than 30% or less than 25 mm in 5 minutes is significant.
    • Stapedial reflex: If the lesion involves the nerve proximal to the branch to the stapedius muscle, the stapedius muscle does not contract and no change in impedance is evident when testing the acoustic reflex.
    • Salivary flow: Wharton papillae are cannulated, and salivary flow is measured in response to a gustatory stimulus. An abnormal result is a reduction of 25% in salivary flow compared with the noninvolved side.
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Contributor Information and Disclosures
Author

Alan D Bruns, MD, FACS Chief, Department of Surgery, Evans Army Community Hospital; Clinical Assistant Professor of Surgery, Uniformed Services University of the Health Sciences

Alan D Bruns, MD, FACS is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, Christian Medical and Dental Associations

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Arlen D Meyers, MD, MBA Professor of Otolaryngology, Dentistry, and Engineering, University of Colorado School of Medicine

Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American Head and Neck Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cerescan;RxRevu;SymbiaAllergySolutions<br/>Received income in an amount equal to or greater than $250 from: Symbia<br/>Received from Allergy Solutions, Inc for board membership; Received honoraria from RxRevu for chief medical editor; Received salary from Medvoy for founder and president; Received consulting fee from Corvectra for senior medical advisor; Received ownership interest from Cerescan for consulting; Received consulting fee from Essiahealth for advisor; Received consulting fee from Carespan for advisor; Received consulting fee from Covidien for consulting.

Additional Contributors

Michael J Biavati, MD, FACS, FAAP Clinical Assistant Professor of Otolaryngology, University of Texas Southwestern Medical Center at Dallas, Southwestern Medical School; Private Practice, ENT Care for Kids, Dallas, TX

Michael J Biavati, MD, FACS, FAAP is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Society of Pediatric Otolaryngology, Society for Ear, Nose and Throat Advances in Children, American Academy of Pediatrics, American Cleft Palate-Craniofacial Association, American College of Surgeons, The Triological Society, Texas Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Drugs & Diseases gratefully acknowledge the contributions of previous author Kim Lundstrom, MD, to the development and writing of this article.

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Child with Möbius syndrome, eyes open. Image courtesy of M.J. Biavati.
Child with Möbius syndrome, eyes closed. Image courtesy of M.J. Biavati.
Child with congenital heart disease and left lower lip palsy suggestive of velocardiofacial syndrome. Image courtesy of M.J. Biavati.
Incision marking for a XII-VII crossover with jump graft. Image courtesy of A.D. Bruns.
Surgical exposure of the facial nerve, hypoglossal nerve, and great auricular nerve. Image courtesy of A.D. Bruns.
Surgical photo of a XII-VII crossover with jump graft. The great auricular nerve graft is sutured to the proximal portion of the facial nerve and to a partial sectioned hypoglossal nerve (preserving approximately two thirds of the axons going to the tongue). Image courtesy of A.D. Bruns.
Table. The House-Brackmann Scale
Grade Characteristics
I. NormalNormal facial function in all areas
II. Mild dysfunction 



Gross



Slight weakness noticeable on close inspection



May have slight synkinesis



At rest, normal symmetry and tone



Motion



Forehead - Moderate-to-good function



Eye - Complete closure with minimal effort



Mouth - Slight asymmetry



III. Moderate dysfunction 



Gross



Obvious but not disfiguring difference between sides



Noticeable but not severe synkinesis, contracture, or hemifacial spasm



At rest, normal symmetry and tone



Motion



Forehead - Slight-to-moderate movement



Eye - Complete closure with effort



Mouth - Slightly weak with maximum effort



IV. Moderately severe dysfunction 



Gross



Obvious weakness and/or disfiguring asymmetry



At rest, normal symmetry and tone



Motion



Forehead - None



Eye - Incomplete closure



Mouth - Asymmetric with maximum effort



V. Severe dysfunction 



Gross



Only barely perceptible motion



At rest, asymmetry



Motion



Forehead - None



Eye - Incomplete closure



Mouth - Slight movement



 



VI. Total paralysis



No movement
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