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    Bel lBel l s Palsy:s Palsy:

    To Treat o r Not t o TreatTo Treat o r Not t o Treat

    K. Kevin Ho, M.D.Shawn D. Newlands, M.D., Ph.D., M.B.A.University of Texas Medical Branch at GalvestonGrand Rounds Presentation February 14, 2007

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    Hist or ic a l Perspec t ivesHist or ic a l Perspec t ives

    Sir Charles Bell (1774-1842)

    Studied facial anatomy

    extensively during Battle ofWaterloo

    Concluded that facial nerve

    controlled facial expression

    Respiratory nerve of the Face

    Sir Charles Bell (1774-1842)

    Studied facial anatomy

    extensively during Battle ofWaterloo

    Concluded that facial nerve

    controlled facial expression

    Respiratory nerve of the Face

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    AnatomyAna tomy

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    Bel l s PalsyBe l l s Palsy

    Idiopathic facial paralysis Diagnosis of Exclusion

    Most common diagnosis(> 60%) for acute facial palsy

    30 per 100,000 Peripheral neuropathy

    Generally unilateral

    Rapid onset < 48 hours

    Idiopathic facial paralysis Diagnosis of Exclusion

    Most common diagnosis(> 60%) for acute facial palsy

    30 per 100,000 Peripheral neuropathy

    Generally unilateral

    Rapid onset < 48 hours

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    Age Dis t r ibut ionAge Dis t r ibu t ion

    Peitersen E. Am. J. Otology. 1982

    2002

    Peitersen E. Acta Otolaryngol 2002;549:430.

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    Com plet e Rem ission & AgeCom ple t e Rem ission & Age

    Peitersen E. Acta Otolaryngol 2002;549:430.

    9084

    75

    64

    36

    0-14 15-29 30-44 45-59 > 60Age

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    Ret urn of Musc u lar func t ionRet urn of Musc u lar func t ion

    Peitersen E. Acta Otolaryngol 2002;549:430.

    85 %

    Months

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    Tim e of beg inn ing rem iss ion &Sequelae

    Tim e of beginn ing rem iss ion &Sequelae

    Peitersen E. Am. J. Otology. 1982

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    Com plet e Rec overyCom plet e Rec overy

    Peitersen E. Acta Otolaryngol 2002;549:430.

    71

    6

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    Inc om plet e vs. Com plet eInc om plet e vs . Com ple t e

    Peitersen E. Acta Otolaryngol 2002;549:430.

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    Symptomato logySymptomato logy

    Reduced Stapedial reflex 71%

    Complete palsy @ presentation 69%

    Tear flow 67%

    Post-auricular pain 52%

    Dysgeusia 34%

    Hyperacusis 14%

    Reduced Stapedial reflex 71%

    Complete palsy @ presentation 69%

    Tear flow 67%

    Post-auricular pain 52%

    Dysgeusia 34%

    Hyperacusis 14%

    Peitersen E. Acta Otolaryngol 2002;549:430.

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    Predic t ing Musc ular SequelaePredic t ing Musc ular Sequelae

    Peitersen E. Acta Otolaryngol 2002;549:430.

    91

    83

    91

    63

    27

    5

    Taste Stapedial Lacrimation

    Normal

    Abnormal

    % Muscular

    Sequelae

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    Favorable prognosis for

    fu l l rec overy

    Favorab le prognosis for

    fu l l rec overy

    Incomplete palsy

    Early recovery

    Young patients

    Normal taste, stapedial reflex, lacrimation

    Lack of post-auricular pain

    Incomplete palsy

    Early recovery

    Young patients

    Normal taste, stapedial reflex, lacrimation

    Lack of post-auricular pain

    Peitersen E. Acta Otolaryngol 2002;549:430.

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    Pathophysio logyPathophysio logy

    Exact etiology unknown

    Viral infection Herpes Simplex

    Vascular ischemia

    Autoimmune disorder

    Hereditary

    Exact etiology unknown

    Viral infection

    Herpes Simplex

    Vascular ischemia

    Autoimmune disorder

    Hereditary

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    Role of HSV-1Role o f HSV-1

    Murakami: Ann Intern Med, Volume 124(1).January 1, 1996.27-30

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    Diabet es Mel l i t usDiabet es Mel l i t us

    Bells patients with DM

    14 % (Korczyn AD 71)

    21 % (Alford BR 71)

    38 % (Yasuda K 75) 66% demonstrate glucose intolerance

    Functional recovery poorer in diabetics

    Bells patients with DM

    14 % (Korczyn AD 71)

    21 % (Alford BR 71)

    38 % (Yasuda K 75) 66% demonstrate glucose intolerance

    Functional recovery poorer in diabetics

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    PregnancyPregnancy

    Incidence of Bells palsy 3-4 x higher

    (Hilsinger, Cohen et al.)

    Third trimester with highest risk

    Higher risk of complete palsy

    Lower chance of complete recovery(Gillman et al.)

    Preeclampsia 6 x prevalence in pregnantwomen with facial palsy

    Incidence of Bells palsy 3-4 x higher

    (Hilsinger, Cohen et al.)

    Third trimester with highest risk

    Higher risk of complete palsy

    Lower chance of complete recovery(Gillman et al.)

    Preeclampsia 6 x prevalence in pregnantwomen with facial palsy

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    Dif ferent ia l DiagnosisAc ut e fac ial pa lsy

    Di f ferent ia l Diagnosi sAc ut e fac ial palsy

    Infection Herpes Zoster Oticus(Ramsey Hunt Syndrome)

    Lyme disease Acute Otitis media +/- mastoiditis

    Congenital Treacher Collins syndrome

    Mobius syndrome Trauma

    Temporal Bone fracture Barotrauma

    Metabolic- Diabetes- Hypothyroidism

    Vascular Benign intracranial hypertension

    Neoplasm Facial neuroma Acoustic neuroma

    Toxic

    Thalidoide Iatrogenic

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    Earl y Grad ing Syst emEar ly Grading Syst em

    Peitersen E. Am. J. Otology. 1982

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    House-Brac k m an Grad ing Syst emHouse-Brac k m an Grad ing Syst em

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    MRIMRI

    Pre-GADPost-GAD

    Kinoshita T et al. Clin. Radiology 2001; 56: 926-32

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    Cont rast Enhanc em ent :Bel l s Palsy vs . Cont ro lCont ras t Enhanc em ent :Bel l s Palsy vs. Cont ro l

    Kinoshita T et al. Clin. Radiology 2001; 56: 926-32

    Bells Palsy

    Control

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    Topognost ic TestTopognost ic Test

    Lacrimal

    Schirmers Test

    Stapedial reflex

    Taste

    Salivary flow

    Lacrimal

    Schirmers Test

    Stapedial reflex

    Taste

    Salivary flow

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    Elec t r i c a l Test

    Nerve Excitation test (NET)

    Maximal Stimulation test (MST)

    Electroneurography (ENoG)

    Electromyography (EMG)

    Nerve Excitation test (NET)

    Maximal Stimulation test (MST)

    Electroneurography (ENoG)

    Electromyography (EMG)

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    Sunder land c lass i f i c a t ion o f per iphera l nerve in jurySunder land c lass i f i c a t ion o f per iphera l nerve in jury

    Neurapraxia

    Axonotmesis

    Neurotmesis

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    Elec t roneurography (ENoG)Elec t roneurography (ENoG)

    Transcutaneous stimulation (Evoked EMG)

    Compound muscle action potential (CMAP)

    Most useful in acute phase within

    3 days 3 weeks of palsy

    But no info on class of injury

    (axonotmesis vs. neurotmesis)

    Transcutaneous stimulation (Evoked EMG)

    Compound muscle action potential (CMAP)

    Most useful in acute phase within

    3 days 3 weeks of palsy

    But no info on class of injury

    (axonotmesis vs. neurotmesis)

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    Tim e c ourse o f Degenerat ionT im e c ourse o f Degenerat ion

    Gantz: Laryngoscope, Volume 109(8).August 1999.1177-1188Fisch U. Am J. Otology. 1984

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    Fisch U. Am J. Otology. 1984

    Fisc h 1984Fisc h 1984

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    Elec t rom yography (EMG)Elec t rom yography (EMG)

    Recording of voluntary muscleaction potentials by needleselectrodes

    Does not differentiateaxonotmesis & neurotmesis

    More useful 2-3 weeks afteronset of complete paralysis

    Perform EMG if ENoG > 95%

    degeneration

    Recording of voluntary muscleaction potentials by needleselectrodes

    Does not differentiateaxonotmesis & neurotmesis

    More useful 2-3 weeks afteronset of complete paralysis

    Perform EMG if ENoG > 95%

    degeneration

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    EMG In t erpret a t ionEMG Int erpret a t ion

    Active voluntary motor units (MU) Intact motor axon

    Myogenic fibrillation potention &Absent voluntary MU

    Complete nerve degeneration

    Fibrillation + MU Partial degeneration

    Polyphasic MU Regenerating nerve

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    Managem ent of Bel l s PalsyManagem ent of Bel l s Palsy

    Observation

    Medical Treatment

    Steroid

    Anti-viral agents

    Surgery Decompression

    Dynamic vs. static reanimation

    Facial Rehabilitation

    Observation

    Medical Treatment

    Steroid

    Anti-viral agents

    Surgery Decompression

    Dynamic vs. static reanimation

    Facial Rehabilitation

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    Coc hrane review on Ef f ic ac y o f s te ro ids

    Coc hrane review on Ef f ic ac y o f s te ro ids

    4 trials of 179 patients Trial 1: Cortisone vs. placebo

    Trial 2: Prednisone + vitamins vs. vitamins

    Trial 3: High dose prednisone vs. saline Trial 4: Methylprednisolone

    Primary endpoint: VII recovery @ 6 mos Conclusions: NO significant benefit for givingsteroids to Bells palsy patients

    Drawbacks: Individual studies underpowered.Steroid regimens differ.

    4 trials of 179 patients Trial 1: Cortisone vs. placebo

    Trial 2: Prednisone + vitamins vs. vitamins

    Trial 3: High dose prednisone vs. saline Trial 4: Methylprednisolone

    Primary endpoint: VII recovery @ 6 mos Conclusions: NO significant benefit for givingsteroids to Bells palsy patients

    Drawbacks: Individual studies underpowered.Steroid regimens differ.

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    Ef f ic ac y of St eroid t reat m entEf f ic ac y o f St ero id t reat m ent

    Prospective RCT 56 patients

    Arm I: Steroids Arm II: Placebo Success = HB I or II

    F/u @ 3 and 6 weeks No significant difference in response in the

    2 groups

    Prospective RCT 56 patients

    Arm I: Steroids Arm II: Placebo Success = HB I or II

    F/u @ 3 and 6 weeks No significant difference in response in the

    2 groups

    Turk-Boru U et al. Kulak Burun Bogaz Ihtis Derg. 2005;14(3-4):62-6.

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    St ero ids in Com plet e para lys isSt ero ids in Com plet e para lys is

    Meta-analysis of 3 prospective trials 230 patients with HB VI

    Treatment within 7 days of onset

    Total prednisone dose > 400 mg

    (405-425 mg)

    Complete Recovery: HB VI I Steroid group has 17% higher rate of CR

    than control (placebo/ no treatment)

    Meta-analysis of 3 prospective trials 230 patients with HB VI

    Treatment within 7 days of onset

    Total prednisone dose > 400 mg

    (405-425 mg)

    Complete Recovery: HB VI I Steroid group has 17% higher rate of CR

    than control (placebo/ no treatment)

    Ramsey MJ et al. Laryngoscope 2000; 110: 335-341

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    St ero id vs . St ero id + Ac yc lov i r St ero id vs . St ero id + Ac yc lov i r

    Double-blind RCT 99 Bells palsy patients

    53 treated with acyclovir- prednisone

    46 with placebo prednisone Prednisone dose 400 mg five times daily x 10 days

    Combined therapy is better in terms of:

    Return of muscle motion

    Prevention of partial nerve degeneration

    Double-blind RCT

    99 Bells palsy patients

    53 treated with acyclovir- prednisone

    46 with placebo prednisone Prednisone dose 400 mg five times daily x 10 days

    Combined therapy is better in terms of:

    Return of muscle motion

    Prevention of partial nerve degeneration

    Adour KK 1996Ann Otol Rhinol Laryngol. 1996 May;105(5):371-8

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    Tim ing of Medic a l T reat m entT im ing o f Medic a l T reat m ent

    Hato N. Otol & Neurotol: 24(6) 2003

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    Sam ple Treat m entSam ple Treat m ent

    Corticosteroids Prednisone 60 mg PO daily x 5 days, taper

    Anti-viral

    Valacyclovir 1000 mg PO TID Eye care

    Glasses/ Sunglasses/ avoid contact lens

    Artificial tears, lacrilube Taping

    Gold weight to upper eyelid

    Opthalmologic consultation

    Corticosteroids Prednisone 60 mg PO daily x 5 days, taper

    Anti-viral

    Valacyclovir 1000 mg PO TID Eye care

    Glasses/ Sunglasses/ avoid contact lens

    Artificial tears, lacrilube Taping

    Gold weight to upper eyelid

    Opthalmologic consultation

    Pensak ML. Assessment and Management of the Paralyzed face. Otol. & Neurotol. Update. Nov 2006

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    Surg ic a l Dec om press ionSurg ic a l Dec om press ion

    Middle Fossa

    Transmastoid Translabyrinthine

    Retrolabyrinthine

    Retrosigmoid

    Middle Fossa

    Transmastoid

    Translabyrinthine

    Retrolabyrinthine

    Retrosigmoid

    Hi t f S i l D iH is t ory of Surg ic a l Dec om press ion

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    Hist ory o f Surg ic a l Dec om pressionHis t ory o f Surg ic a l Dec om press ion

    Adour KK. 2002 Jan;259(1):40-7

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    Anat om y of Fac ia l CanalAnat om y of Fac ia l Canal

    Coker NJ. Atlas of Otologic Surgery p.339

    0.68 mm

    Labyrinthine1.02 mm

    Tympanic1.53 mm

    Mastoid1.48 mm

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    Cont roversy over Surg ic a l Dec om press ion

    Cont roversy over Surg ic a l Dec om press ion

    In favor of: Gantz BJ 99

    Sillman JS 92 Huges GB 88

    Goin DW 82

    Fisch U 81

    Brackmann DE 80

    Giancarlo HR 70

    In favor of: Gantz BJ 99

    Sillman JS 92

    Huges GB 88

    Goin DW 82

    Fisch U 81

    Brackmann DE 80

    Giancarlo HR 70

    Against: Adour KK 01

    Aoyagi M 88 May M 84

    Gacek RR 81

    McNeill R 74

    Adour KK 71

    Mechelse K 71

    Against: Adour KK 01

    Aoyagi M 88

    May M 84

    Gacek RR 81

    McNeill R 74

    Adour KK 71

    Mechelse K 71

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    Resu lt s of Midd le Fossa Approac hResu l t s of Midd le Fossa Approac h

    Grade Iowa Michigan Baylor Total

    I 3 5 0 8

    II 7 2 6 15

    III 1 1 0 2

    IV 0 1 0 1

    Gantz: Laryngoscope, Volume 109(8).August 1999.1177-1188

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    Mic h igan St udy:MCF vs . St eroidsMic h igan St udy:MCF vs . St eroids

    010203040506070

    I I I I I I IV

    S t e r o i d sMCF

    Grade

    %

    Glasscock M, Shambaugh G: Facial nerve surgery. In Surgery of the ear, 1990:434-465.

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    Ear ly MCFEar ly MCF

    Gantz: Laryngoscope, Volume 109(8).August 1999.1177-1188

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    Gantz: Laryngoscope, Volume 109(8).August 1999.1177-1188

    Tim ing o f Dec om press ionTim ing of Dec om press ion

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    Gantz: Laryngoscope, Volume 109(8).August 1999.1177-1188

    Algor i thmAlgor i thm

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    Fac t ors t o c ons ider fo r Surgic a lDecompress ion

    Fac t ors t o c onsider fo r Surgic a lDecompress ion

    Age Comorbidities

    ENoG Endpoint

    Progression / velocity of degeneration Days from onset of paralysis

    Return of muscle function

    Age Comorbidities

    ENoG Endpoint

    Progression / velocity of degeneration Days from onset of paralysis

    Return of muscle function

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    Thank you

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    Thank you