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Facial Nerve Paralysis

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Title: Facial Nerve Paralysis


1
Facial Nerve Paralysis
  • Vanessa S. Rothholtz, M.D., M.Sc.
  • UCI Department of Otolaryngology - Head and Neck
    Surgery
  • May 24, 2007

2
Chief Complaint
  • My Starbucks caramel macchiatto dribbled down my
    chin this morning, and it ruined my white coat.
    Now my face isnt working. Do I need a face lift?

3
History
  • Unilateral left-sided otalgia (TMJ)
  • Fever, chills
  • Headache
  • Generalized fatigue
  • Conjunctivitis two weeks ago (resolved with
    antibiotics)
  • My eczema acted up again last week, but it
    looked a little different.
  • Travel Sonoma County for a friends wedding a
    last month

4
Physical
  • Eyes Left eye with injected conjunctiva, pupils
    equal and reactive
  • Ears EAC patent, TM c/m/i
  • Nares Patent, clear
  • OC/OP Dentition intact, tongue midline / mobile,
    No tonsillar hypertrophy
  • Face
  • Normal tone and symmetry at rest
  • Obvious facial asymmetry with effort
  • No perceptible forehead movement
  • Incomplete eye closure
  • Asymmetrical motion of mouth with maximal effort

5
What grade of paralysis is this based on the
House-Brackmann facial nerve grading scale?
  • IV

6
House-Brackmann Facial Nerve Grading Scale
  • I Normal
  • II Normal tone and symmetry at rest
  • Slight weakness on close inspection
  • Good to moderate movement of forehead
  • Complete eye closure with minimum effort
  • Slight asymmetry of mouth with movement
  • III Normal tone and symmetry at rest
  • Obvious but not disfiguring facial asymmetry
  • Synkinesis may be noticeable but not severe
  • /- hemifacial spasm or contracture
  • Slight to moderate movement of forehead
  • Complete eye closure with effort
  • Slight weakness of mouth with maximum effort
  • IV Normal tone and symmetry at rest
  • Asymmetry is disfiguring or results in obvious
    facial weakness
  • No perceptible forehead movement
  • Incomplete eye closure
  • Asymmetrical motion of mouth with maximum effort
  • V Asymmetrical facial appearance at rest
  • Slight, barely noticeable movement
  • No forehead movement
  • Incomplete eye closure
  • Asymmetrical motion of mouth with maximum effort

7
Differential Diagnosis
  • V Anomalous sigmoid sinus, benign intracranial
    hypertension, intratemporal aneurysm of internal
    carotid artery, embolization for epistaxis
    (external carotid artery branches)
  • I Malignant otitis externa, otitis media,
    cholesteatoma, mastoiditis, meningitis,
    parotitis, chicken pox, Ramsay Hunt syndrome,
    encephalitis, poliomyelitis (type I), mumps,
    mononucleosis, leprosy, HIV/AIDS, influenza,
    Coxsackie virus, malaria, syphilis, scleroma, TB,
    botulism, mucormycosis, Lyme disease
  • T Cortical injuries, basilar skull fractures,
    brainstem injuries, penetrating injury to middle
    ear, facial injuries, altitude paralysis
    (barotrauma), SCUBA diving (barotrauma)
  • A Temporal arteritis, periarteritis nodosa,
    Multiple sclerosis, myasthenia gravis,
    sarcoidosis, Wegener granulomatosis, eosinophilic
    granloma
  • M Paget disease, osteopetrosis, diabetes
    mellitus, hyperthyroidism, pregnancy, alcoholic
    neuropathy, bulbopontine paralysis,
    oculopharyngeal muscular dystrophy
  • I Bell palsy, Melkersson-Rosenthal syndrome
    (recurrent facial palsy, furrowed tongue),
    hereditary hypertrophic neuropathy,
    (Charcot-Marietooth disease, Dejerine-Scottis
    disease), Landry-Guillain-Barre syndrome,
    Sarcoidosis, Kawasaki disease, surgery,
    embolization
  • N Acoustic neuroma, glomus jugulare tumor,
    leukemia, meningioma, hemangioblastoma,
    hemangioma, pontine glioma, sarcoma, hydradenoma,
    gacial nerve neuroma, teratoma, fibrous
    dysplasia, von Recklinghausens disease,
    carcinomatous encephalitis, cholesterol
    granuloma, carcinoma (invasive or metastatic)
  • C Molding, forceps delivery, myotoic dystrophy,
    Moebius syndrome
  • D Vaccine for rabies, Antitetanus serum,
    mandibular block anesthesia

8
Course of the Facial Nerve
  • Intracranial Arises at the pontomedullary
    junction and courses with CNVIII to the internal
    acoustic meatus - 12mm
  • Meatal Anterior to the superior vestibular
    nerve and superior to the cochlear nerve 10mm
  • Intratemporal
  • Labyrinthe segment
  • Passes through narrowest part of fallopian canal
    - 12mm
  • Narrowest part of facial nerve. The most
    susceptible to compression secondary to edema.
  • Tympanic segment
  • From geniculate ganglion to pyramidal turn 11mm
  • Mastoid segment
  • Exits the stylomastoid foramen 13mm
  • Extracranial From stylomastoid foramen to pes
    anserinus

9
  • The longest segment of the facial nerve is
  • A. Vertical of mastoid portion
  • B. Cisternal portion
  • C. Tympanic portion
  • D. Portion in the IAC
  • Captier G. Organization and microscopic anatomy
    of the adult human facial nerve Anatomical and
    histological basis for surgery

10
Blood supply to facial nerve clinical relevance
  • Courses between the epineurium and periosteum
    making the blood supply at risk when mobilizing
    at the first genu
  • Extrinsic
  • Stylomastoid artery (branch of the postauricular
    artery of external carotid artery)
  • Greater petrosal artery (branch of middle
    meningeal artery)
  • Internal auditory artery (branch of the AICA)
  • Labyrinthe segment - lacks anastomosing arterial
    cascades thereby making the area vulnerable to
    ischemia
  • Parhizkar N, Hiltzik DH and Selesnick SH.
    Facial nerve rerouting in skull base surgery.
    Otol Clin N Am. 2005 38(4) 685-710

11
Work Up
  • Basic labs, thyroid function panel, Lyme titers
    ELISA for antibodies
  • Audiogram
  • Stapedial reflex
  • EKG
  • MRI with gadolinium / CT
  • Nerve Excitability Test, Maximal Stimulation
    Test, Electroneuronography (EnoG) - Useful 72
    hours post-injury

12
Topognostic Testing
  • Schirmer test for lacrimation
  • Stapedial reflex test (stapedial branch)
  • Taste testing (chorda tympani nerve)
  • Salivary flow rates and pH (chorda tympani)

13
Topognostic Testing
  • Schirmer Test
  • Greater superficial petrosal nerve
  • Filter paper is placed in the lower conjunctival
    fornix bilaterally
  • 3- 5 minutes
  • Value of 25 or less on the involved side or
    total lacrimation less than 25 mm is considered
    abnormal.

14
Topognostic Testing
  • Stapedial Reflex
  • Stapedius branch of the facial nerve
  • Most objective and reproducible
  • A loud tone is presented to either the
    ipsilateral or contralateral ear ? evokes a
    reflex movement of the stapedius muscle ? changes
    the tension on the TM (which must be intact for a
    valid test) resulting in a change in the
    impedance of the ossicular chain
  • If intact stapedial reflex, complete recovery can
    be expected to begin within six weeks
  • Absence of the stapedial reflex during the first
    two weeks in Bells Palsy is common

15
Topognostic Testing
  • Taste Testing
  • Chorda tympani
  • Extremely subjective
  • Papillae generally disappear within 10 days post
    injury - middle 1/3 of the tongue is most
    indicative, because the anterior 1/3 may receive
    bilateral input.

16
Topognostic Testing
  • Salivary flow rates
  • Chorda tympani
  • Cannulation of Wharton's ducts bilaterally
  • 5 minute measurement of output
  • Significant if 25 reduction in flow of the
    involved side as compared to the normal side
  • Salivary pH ? Flow Rate

17
Nerve Excitability Test (NET)
  • Most predictive prognostic factor for recovery of
    facial nerve function
  • Hilger nerve stimulator over stylomastoid foramen
  • Reflects elevated thresholds for neuromuscular
    stimulation due to degeneration / disruption of
    axons (comparison to contralateral side)
  • Difference 2.5 milliamps - poor prognosis
  • Ikeda M et. al. Clinical factors that influence
    the prognosis of facial nerve paralysis and the
    magnitudes of influence. Laryngoscope. 2005
    115855-860.

18
Nerve Excitability Test (NET)
  • Benefits
  • Easy to perform
  • More comfortable for patient
  • Drawbacks
  • Subjectivity (relies on operators visual
    detection of response)
  • May exclude smaller fibers (current thresholds
    are likely to selectively activate larger fibers
    with lower thresholds and not those smaller
    fivers closer to stimulating electrode)

19
Maximal Stimulation Test (MST)
  • Electrical impulse administered to saturate the
    nerve with current and to compare it to
    contralateral side
  • Test is repeated periodically until definitive
    response
  • Response
  • Equivalent to contralateral side
  • Minimally diminished (50)
  • Markedly diminished (
  • Absent
  • Symmetric response within first ten days
    complete recovery in 90
  • No response within first ten days incomplete
    recovery with significant sequelae
  • Superior to NET - test becomes abnormal sooner,
    but drawback is subjectivity

20
Evoked electromyography (EEMG) or
Electroneuronography (EnoG)
  • Records compound muscle action potential (CMAP)
    with surface electrodes placed transcutaneously
    in the nasolabial fold (response) and
    stylomastoid foramen (stimulus)
  • Waveform responses are analyzed to compare
    peak-to-peak amplitudes between normal and
    uninvolved sides where the peak amplitude is
    proportional to the number of intact axons

21
Evoked electromyography (EEMG) or
Electroneuronography (EnoG)
  • Most reliable in first 2-3 weeks post event (as
    neuropraxic fibers recover or regenerate, they
    discharge asynchronously and the response is
    subsequently diminished)
  • Response prognosis
  • Response 90 of normal within 3 weeks of onset
    80-100 probability of recovery
  • Testing every other day
  • Advantages Reliable
  • Disadvantages
  • Uncomfortable
  • Cost
  • Test-retest variability due to position of
    electrodes

22
Electromyography (EMG)
  • Measures post-synaptic membrane di/triphasic
    (polyphasic) potentials with voluntary muscle
    contraction that are present 6-12 weeks prior to
    visible return of function
  • Assesses reinnervation potential of muscles two
    weeks after onset
  • Limited value early in evaluation because
    fibrillation potentials indicating axonal
    degeneration do not appear until 10 14 days
    post onset
  • Detection of motor units in 2 of 3 muscle groups
    87 satisfactory outcome
  • Detection of motor units in 1 muscle group 11
    satisfactory

23
More Methods
  • Antidromic (retrograde) Conduction F-waves
    represent activated motor neurons in facial
    muscles.
  • Transcranial magnetic stimulation Enables
    central activation via a transcranial application
    of induce current via an electromagnetic coil
  • Trigeminofacial Reflex Records action
    potentials reflexively generated in the
    orbicularis oculi muscle in response to an
    electrical stimulus applied to V1

24
Lyme Disease - Borrelia Burgdorferi
  • Ten percent of patients have facial nerve
    paralysis after 1-4 weeks incubation period
  • ELISA to search for IgG and IgM antibodies
  • Facial paralysis resolves in 6 to 12 months
  • Treatment
  • Early antibiotics
  • Reduce symptoms
  • Event long-term sequelae
  • Children - IV penicillin, ceftriaxone or
    cefotaxime
  • Adults - tetracycline
  • Muscular therapy

25
Bells Palsy
  • 60-70 cases
  • Pathophysiology Impaired axoplasmic flow from
    edema of facial nerve within fallopian canal
  • Rapid onset and evolution
  • May be associated with acute neuropathies of
    cranial nerves V- X
  • Pain or numbness affecting ear, mid-face, tongue
    and taste disturbances
  • Recurrences are more likely (2.5x) in patients
    with family history, immunodeficiency or diabetes

26
Bells Palsy
  • Treatment
  • Oral antivirals - Acyclovir - 10mg/kg (500mg)
    q8hrs x 7 days
  • Corticosteroid taper 1mg / kg / day for 10 days
  • Eye protection - lacrilube
  • Follow progression with serial exams
  • Facial nerve decompression
  • Progression to 90 degeneration on ENOG
  • Performed before irreversible injury to the
    endoneural tubules occurs (two weeks), will allow
    for axonal regeneration to occur

27
Treatment of Bells Palsy with Steroids A
controversial closer look
  • Steroids may have the following effects
  • Reduce risk of denervation
  • Preventing / lessening synkinesis
  • Preventing progression to complete paralysis
  • Hastening recovery
  • Controversy
  • Lack of randomization, controls and definitive
    dosing in most studies
  • Stankiewicz J. Steroids and idiopathic facial
    paralysis. Otlaryngol Head Neck Surg. 1983 91
    672.
  • Wolf S. Wagner J. Davidson S. et.. al. Treatment
    of Bells palsy with prednisone a prospective
    randomized study. Neurology. 1978 28 158.

28
  • Facial Nerve function recovers to HB grade I
    function in what percentage of patients with
    Bells Palsy?
  • A. 50
  • B. 70
  • C. 85
  • D. 95
  • Ikeda M et. al. Clinical factors that influence
    the prognosis of facial nerve paralysis and the
    magnitudes of influence. Laryngoscope. 2005
    115855-860.

29
  • Which of the following factors is a predictor of
    poor facial nerve outcome following Bells Palsy?
  • A. Age over 50 years
  • B. Male Gender
  • C. Loss of lacrimation
  • D. Hypothyroidism
  • Ikeda M et. al. Clinical factors that influence
    the prognosis of facial nerve paralysis and the
    magnitudes of influence. Laryngoscope. 2005
    115855-860.

30
What is this Condition?
Ramsay Hunt Syndrome
31
Herpes Zoster Oticus (Ramsay Hunt syndrome)
  • 10-15 of acute facial palsy cases
  • Lesions may involve the external ear, the skin of
    EAC or soft palate
  • Associated symptoms hearing loss, dysacusis and
    vertigo
  • Additional involvement of CN V, IX and X and
    cervical branches 2, 3 and 4
  • Pathogenesis Neural injury due to edema at
    point between the meatal foramen and the
    geniculate fossa in the labyrinthe segment

32
  • The most common etiology of facial nerve
    paralysis in children is
  • A. Infection
  • B. Congenital
  • C. Trauma
  • D. Iatrogenic
  • Evans AD et. al. Pediatric facial nerve
    paralysis Patients, management and outcomes.
    Int J Ped Otol. 2005 691521-1528.

33
  • Which of the following infections is most likely
    to cause facial paralysis in a pediatric patient?
  • A. Acute otitis media
  • B. Mastoiditis
  • C. Mycobacterium infection
  • D. Disseminated herpes
  • infection
  • Evans AD et. al. Pediatric facial nerve
    paralysis Patients, management and outcomes.
    Int J Ped Otol. 2005 691521-1528.

34
Acute Otitis Media
  • History and physical exam make the diagnosis
  • Palsy is progressive over 2 to 3 day period
  • Infectious agent Staphylococcus non-aureus,
    Propionobacterium
  • CT temporal bone
  • Treatment
  • Myringotomy
  • Otic antibiotic drops containing topical steroids
  • IV antibiotics and steroids
  • If not improved mastoidectomy

35
Möbius Syndrome
  • Most frequently sporadic
  • Congenital facial weakness with impairment of
    ocular abduction
  • Dysfunction of other cranial nerves III, IV,
    IX, X, XII
  • Skeletal abnormalities (orofacial, limb
    malformations)
  • Pathogenesis Genetic cause vs. Ischemic cause

36
Melkersson-Roenthal syndrome
  • Triad
  • Recurrent orofacial edema
  • Recurrent facial palsy (50-90)
  • Lingua plicata (fissure tongue) 25
  • Lips become chapped, fissured and red-brown in
    appearance
  • Biopies identify granulomatous changes
  • Facial nerve decompression may be indicated if
    facial paralysis is severe and recurrent

37
Neoplastic
  • About 5 of cases of facial nerve paralysis are
    caused by tumors
  • Characteristics of facial nerve palsy
  • Slow developing
  • Additional cranial nerve deficits
  • Recurrent ipsilateral involvement
  • Adenopathy
  • Palpable neck or parotid mass
  • Most common benign tumor - facial nerve
    schwanomma
  • Most common malignant tumors - mucoepidermoid
    carcinoma and adenoid cystic carcinoma of the
    parotid gland.

38
Temporal Bone Fractures
  • Longitudinal fractures
  • 80 incidence but 10-20 with facial nerve injury
  • Transverse fractures
  • 20 incidence, but 50 with facial nerve injury
  • Most common site of fracture
  • Perigeniculate region

39
Temporal Bone Fractures
  • Penetrating injury to extratemporal facial nerve
    branches
  • Injuries medial to a line perpendicular to the
    lateral canthus do not need to be explored
    because they recover spontaneously (draw please)
  • Immediate paralysis after injury lateral to this
    line needs to be explored and repaired with an
    end-to-end anastomosis 48-72 hours after the
    initial injury

40
Sunderland Nerve Injury Classification
  • I Neuropraxia
  • Conduction block from compression and loss of
    axonic flow
  • Complete recovery
  • II Axonotmesis
  • Axon disrupted but endoneurium preserved
  • Wallerian degeneration occurs distal to site of
    injury
  • Complete recovery
  • III Neurotmesis
  • Complete disruption of axon including its
    surrounding myelin and endoneurium
  • Wallerian degeneration
  • Unpredictable outcome High risk for synkinesis
  • IV Complete disruption of perineurium
  • V Complete disruption of epineurium
  • Risk of a neuroma from nerve sprouts outside of
    nerve sheath

41
  • A patient with facial nerve injury following a
    gunshot wound to the temporal bone typically
    presents with which of the following symptoms?
  • A. Midface branch paralysis
  • B. Complete facial paralysis
  • C. Forehead paralysis
  • D. Partial weakness of the facial nerve
  • Bento RF and de Brito RV. Gunshot wounds to
    the facial nerve. Otol Neurotol. 2004 25
    1009-1013.

42
  • Following surgical repair of facial nerve injury
    due to a gunshot wound, the typical facial nerve
    function outcome is House Brackmann grade
  • A. I or II
  • B. III or IV
  • C. V
  • D. VI
  • Bento RF and de Brito RV. Gunshot wounds to
    the facial nerve. Otol Neurotol. 2004 25
    1009-1013.

43
A patient presents to the trauma bay after a
closed head injury. He has a unilateral facial
nerve paralysis and a CT scan confirms a temporal
bone fracture. The family wants your expert
opinion on the prognosis and return of facial
nerve function.
  • Immediate onset as above
  • Delayed onset
  • 94-100 complete recovery
  • Patients with 90 degeneration of neural
    integrity poor recovery

44
What if the facial paralysis doesnt resolve?
  • End-to-End Anastomosis
  • Cable Nerve Graft
  • Hypoglossa-Facial Nerve Anastomosis (Crossover or
    Jump Graft)
  • Muscle transposition (Gracilis)
  • Static Suspension (Gortex, Threads)

45
Complications
  • Keratitis
  • Emotional/Social Issues
  • Synkinesis

46
THANK YOU
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