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MANAGEMENT of
POST-FACIAL
PARALYSIS
SYNKINESIS
This page intentionally left blank
     
MANAGEMENT of
POST-FACIAL PARALYSIS
SYNKINESIS
Edited by
BABAK AZIZZADEH MD, FACS
Associate Clinical Professor, David Geffen School of Medicine at UCLA
Diplomat, American Board of Facial Plastic & Reconstructive Surgery
Fellow, American Academy of Facial Plastic & Reconstructive Surgery
Fellow, American College of Surgeons

CHARLES NDUKA MB BS, MA(OXON), MD, FRCS, FRCS(PLAS)


Consultant Plastic Surgeon, Facial Palsy Unit
Department of Plastic & Reconstructive Surgery
Queen Victoria Hospital
East Grinstead, UK

London New York Oxford Philadelphia St Louis Sydney Toronto 2022


© 2022 Elsevier Ltd. All rights reserved.

No part of this publication may be reproduced or transmitted in any form or by any means, electronic or
mechanical, including photocopying, recording, or any information storage and retrieval system, without
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This book and the individual contributions contained in it are protected under copyright by the Publisher
(other than as may be noted herein).

Notices

Practitioners and researchers must always rely on their own experience and knowledge in evaluating and
using any information, methods, compounds or experiments described herein. Because of rapid advances
in the medical sciences, in particular, independent verification of diagnoses and drug dosages should be
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the material herein.

Library of Congress Control Number: 2021939103

ISBN 978-0-323-67331-0

e_ISBN 978-0-323-75510-8

Content Strategist: Jessica McCool/Belinda Kuhn


Content Development Specialist: Trinity Hutton/Veronika Watkins
Project Manager: Julie Taylor
Designer: Bridget Hoette

Printed in the United States


Last digit is the print number: 9 8 7 6 5 4 3 2 1
Contents

Video Table of Contents, vi 7 Assessment and Grading of Synkinesis and


Preface, vii Facial Palsy, 51
Helen Hartley, Wendy Blumenow, Rebecca Williams,
Acknowledgements, viii Adel Fattah
Dedication, ix
Contributors, x 8 Objective Measurement of Outcomes in Facial
Palsy, 59
1 Facial Nerve and Muscle Anatomy, 1 Gerd Fabian Volk, Jovanna Thielker, Oliver Mothes, Orlando
G. Nina Lu, Patrick J. Byrne Guntinas-Lichius

2 Etiology, Epidemiology, and Pathophysiology of 9 Facial Neuromuscular Retraining for


Post-Facial Paralysis Synkinesis, 13 Synkinesis, 75
Kofi D.O. Boahene H. Jacqueline Diels

3 Psychosocial Impact of Facial Palsy, 19 10 Eyelid Rehabilitation in Post-Facial Paralysis With


Ietske J. Siemann, Carien H.G. Beurskens Synkinesis, 91
Amy Patel Jain, Yao Wang, Julia Kerolus, Babak Azizzadeh,
4 Facial Nerve Consultation, 25 Guy Massry
Stephanie Warrington, Sara MacDowell, Laura Hetzler
11 Surgical Management of Post-Facial Paralysis
5 Smile Analysis for Facial Nerve Disorders, 33 Synkinesis, 101
Scott R. Chaiet Mike Zein, Babak Azizzadeh

6 The Role of Botulinum Toxin in Facial Palsy Appendix: Instructions for Sonography of the ­Mimic
Management, 39 Musculature, 109
Ruben Yap Kannan, Charles Nduka Index, 135

v
Video Table of Contents

CHAPTER 1 channel 1 shows M. orbicularis oris; channel 2 shows


Video 1.1 Levator labii superioris vector of pull M. zygomaticus. Sound comes from channel 2
Video 8.5 Montage of video and electromyography
CHAPTER 3 from a participant without synkinesis. Two needle-
Video 3.1 Alternatives for facial expressions EMG - electrodes are inserted in two different mus-
cles: channel 1 shows M. orbicularis oris; channel 2
CHAPTER 4 shows M. zygomaticus. Sound comes from channel 1
Video 4.1 Flaccid paralysis Video 8.6 Montage of video and electromyogra-
Video 4.2 Synkinesis phy from a participant without synkinesis. Two
needle-EMG - electrodes are inserted in two differ-
CHAPTER 7 ent muscles: channel 1 shows M. orbicularis oris;
Video 7.1 Example of iatrogenic synkinesis channel 2 shows M. zygomaticus. Sound comes
Video 7.2 Post-operative rehabilitation therapy for from channel 1
gracilis patients Video 8.7 Sonography of the corner of the mouth
Video 7.3 Additional assessments for bilateral facial while patient with synkinesis is performing eye
palsy patients blinks

CHAPTER 8 CHAPTER 9
Video 8.1 Example of a standard photo session Video 9.1 Inhibition of oculi synkinesis during fNMR
Video 8.2 Example of pathologic spontaneous activity Video 9.2 Inhibition of synkinesis using sEMG
recorded by a needle-electromyography of the mus- feedback
culus orbicularis oris Video 9.3 Movement acquisition during fNMR using
Video 8.3 Montage of video and electromyography proprioception
from a patient with synkinesis. Two needle-EMG - Video 9.4 Dynamic demonstration of synkinetic inhi-
electrodes are inserted in two different muscles: bition during evaluation
channel 1 shows M. orbicularis oris; channel 2 shows Video 9.5 Identifying botulinum toxin injection site(s)
M. zygomaticus. Sound comes from channel 1 for buccinator
Video 8.4 Montage of video and electromyography
from a patient with synkinesis. Two needle-EMG - CHAPTER 10
electrodes are inserted in two different muscles: Video 10.1 Eyelid and periorbital reconstructive surgery

vi
Preface

This book grew from the seed of an idea planted in 2017 Diels, Daniel Labbé, Mark Lucarelli, Guy Massry, Bill
at the 13th International Facial Nerve Symposium, orga- Slattery, and Matt White. It was clear that there is im-
nized and hosted by Babak Azizzadeh and colleagues. mense knowledge about the management of synkinesis
It gathered over 500 professionals from over 40 coun- in the heads of the international faculty that needed to
tries in Los Angeles to share knowledge and research be captured in one place. The result is this book, which
about facial paralysis. There was an excellent panel has drawn upon leading experts from across the world
session on synkinesis with contributions from Jackie to distill their knowledge within these pages.

vii
Acknowledgements

Acknowledgements from Charles Nduka understanding of the facial nerve anatomy, and the
I would like to thank the thousands of patients affect- complexity of treating cranial nerve disorders. I was
ed by synkinesis who have allowed me to be part of privileged to train in facial plastic and reconstructive
their care team. They have provided me with impor- surgery at the Massachusetts Eye & Ear Infirmary
tant insights and inspiration. I am especially grate- which completely changed the trajectory of my career.
ful to the Facial Palsy UK community of patients, During my fellowship at Harvard Medical School, I was
their families, and supporters who have taught me fortunate enough to be trained by Dr. Mack Cheney
so much about the impact that facial paralysis has on who had himself dedicated his life to microsurgery,
their lives. facial plastic surgery, and facial paralysis and, along
I am grateful to my multidisciplinary colleagues at with Drs. Mark Varvares and Daniel Deschler, gave me
the Queen Victoria Hospital in East Grinstead, includ- the necessary medical and surgical tools to further de-
ing facial therapists Trina Neville, Tamsin Gwynn, and velop my skills. After returning to Los Angeles, I was
Karen Young, psychological therapist Beth Jordan, and privileged to be surrounded by a passionate team of
my plastic surgery colleague Ruben Kannan. I am also subspecialists who share the same desire, allowing us to
grateful for the ongoing support of the oculoplastic look at facial nerve disorders from a multidisciplinary
team led by Raman Malhotra and André Litwin, and approach. Drs. Babak L ­ arian, Guy Massry, Bill Slattery,
the facial nerve team at Guy’s Hospital in London. Randy Sherman, Greg ­Lekovic, and Jackie Diels have
Without the encouragement, patience, and support been inspirational and I have learned so much from
of Veronika Watkins, Jessica McCool, and the Elsevier each of them almost every single day. I have also been
team, this book would not have been possible. fortunate to have met so many pioneers of the field
such as Dr. Douglas Harrison who have mentored and
Acknowledgements from Babak Azizzadeh inspired me. I also want to thank my contemporaries
My personal journey as a physician has been so re- Drs. Tessa Hadlock and Patrick Byrne who continue
warding, with my family, patients, mentors, and col- to push the envelope in this emerging field of study.
leagues having an integral role in my passion for treat- I would be remiss not to share how lucky I have been to
ing patients with facial nerve disorders. During the have Dr. Babak Larian as the most compassionate and
very early part of my residency training, one of my talented partner who has been with me every step of
wife’s closest friends developed facial paralysis due to the way for over two decades. He and I have operated
a benign brain tumor and that started my deep curios- together, dreamed together, devised new approaches,
ity into this most challenging of all medical and surgi- and managed the most challenging medical and sur-
cal disorders. During my early days as a resident in gical cases. Finally, this book would not have become
Head & Neck Surgery at UCLA, Drs. Rinaldo Canalis, reality without Jessica McCool, Veronika Watkins, and
Tom Calcatterra, Keith Blackwell, and Gerald Berke the rest of the Elsevier team. They have been the ulti-
were integral in my training in microsurgery, in-depth mate professionals in their own field.

viii
Dedication from Charles Nduka
To my wife Jules for her continued understanding and patience.
Dedication from Babak Azizzadeh
I dedicate this book to all my patients who have entrusted me to help them in their own journeys.

ix
Contributors

Babak Azizzadeh MD Adel Fattah PhD, FRCS(Plast)


Associate Clinical Professor, David Geffen School of Consultant Plastic Surgeon
Medicine at UCLA Regional Paediatric Burns and Plastic Surgery Service
Diplomat, American Board of Facial Plastic & Alder Hey Children’s NHS Foundation Trust
Reconstructive Surgery Liverpool, UK
Fellow, American Academy of Facial Plastic &
Reconstructive Surgery Orlando Guntinas-Lichius MD
Fellow, American College of Surgeons Professor and Chairman
ENT Department
Carien Beurskens PhD Jena University Hospital
Doctor Jena, Germany;
Physiotherapy Facial Nerve Center
Radboud University Medical Center Jena University Hospital
Nijmegen, Netherlands Jena, Germany;
Medical Faculty, Friedrich-Schiller-University
Wendy Blumenow Bsc (Hons), RCSLT, HCPC Jena, Germany
Principal Speech & Language Therapist
Alder Hey Children’s NHS Foundation Trust Helen Hartley MSc, BSc
Liverpool, UK Highly Specialist Paediatric Physiotherapist
Physiotherapy Department
Kofi Boahene MD Alder Hey Children’s NHS Foundation Trust
Professor Liverpool, UK
Department of Otolaryngology, Head and Neck
Surgery Laura Hetzler MD, FACS
Johns Hopkins University School of Medicine Associate Professor and Program Director
Baltimore Department of Otolaryngology – Head and Neck Surgery
MD, USA Louisiana State University
New Orleans
Patrick Byrne MD LA, USA
Chairman
The Cleveland Clinic Head and Neck Institute Ruben Yap Kannan MB, PhD, FRCS(Plast), DOHNS,
Cleveland DLM
OH, USA Consultant
Plastic & Reconstructive Surgery, (Facial Palsy)
Scott Chaiet MD, MBA Queen Victoria Hospital
Assistant Professor East Grinstead
Division of Otolaryngology, Department of Surgery West Sussex, UK
University of Wisconsin School of Medicine & Public
Health Julia Kerolus MD
Madison Assistant Professor
WI, USA Otolaryngology – Head and Neck Surgery
University of Illinois at Chicago
H. Jacqueline Diels OT Chicago
Facial Retraining Specialist IL, USA
Rehabilitation
Facial Retraining LLC G. Nina Lu MD
Madison Assistant Professor
WI, USA Otolaryngology
University of Washington
Seattle
WA, USA

x
CONTRIBUTORS xi

Sara MacDowell PT, DPT Gerd Fabian Volk Priv. Doz. Dr. med. habil.
Doctor of Physical Therapy Senior Consultant
Our Lady of the Lake Hearing and Balance Center ENT Department
Baton Rouge Jena University Hospital
LA, USA Jena, Germany;
Head
Guy Massry MD Facial Nerve Center
Ophthalmic Plastic & Reconstructive Surgery Jena University Hospital
Beverly Hills, California, CA, USA; Jena, Germany;
Clinical Professor of Ophthalmology Medical Faculty, Friedrich-Schiller-University
University of Southern California, Keck School of Jena, Germany
Medicine,
CA, USA; Yao Wang MD
Diplomat, American Board of Ophthalmology; Fellow Physician
Fellow, American Society of Ophthalmic Plastic and Ophthalmology and Surgery
Reconstructive Surgery Cedars-Sinai Medical Center
Los Angeles
Oliver Mothes MSc CA, USA
Computer Vision Group
Friedrich Schiller University Stephanie Warrington MD
Jena Department of Otolaryngology – Head Neck Surgery
Thuringia, Germany LSU Health Sciences Center
New Orleans
Charles Nduka MB BS, MA(OXON), MD, FRCS, LA, USA
FRCS(Plas)
Consultant Plastic Surgeon, Facial Palsy Unit Rebecca Williams BSc (Hons)
Department of Plastic & Reconstructive Surgery Specialist Paediatric Physiotherapist
Queen Victoria Hospital Alder Hey Children’s NHS Foundation Trust
East Grinstead, UK Liverpool, UK

Amy Patel Jain MD Mike Zein MB, MSc


Oculoplastics Bascom Palmer Eye Institute
Cedars-Sinai University of Miami
Los Angeles Miami
CA, USA FL, USA

Ietske Siemann MSc


Medical Psychology
Radboud University Medical Center
Nijmegen, Netherlands

Jovanna Thielker MD
ENT Department
Jena University Hospital
Jena, Germany;
Facial Nerve Center
Jena University Hospital
Jena, Germany;
Medical Faculty, Friedrich-Schiller-University
Jena, Germany
This page intentionally left blank
     
Facial Nerve and Muscle Anatomy 1
G. Nina Lu, Patrick J. Byrne

ganglion cell bodies and travel within the chorda tym-


INTRODUCTION
pani and GSPN. Somatic sensory fibers provide touch
The facial nerve, also known as the seventh cranial sensation to the external auditory canal and conchal
nerve, combines motor, general sensory, special sen- skin of the auricle as well as proprioceptive informa-
sory, and autonomic (visceral) components. The intri- tion from the facial muscles (Fig. 1.1).
cate course of the facial nerve as it runs intracranially,
intratemporally, and extratemporally is essential for INTRACRANIAL
any surgeon operating in the head and neck to under- Facial muscle movement starts with a conscious or un-
stand. The facial nerve innervates the muscles derived conscious impulse in the motor cortex of the precentral
from the second branchial arch and carries sensory and gyrus of the cerebral hemispheres. The majority of ce-
parasympathetic fibers of the nervus intermedius. The rebral fibers then project to the contralateral facial mo-
facial muscles, also known as mimetic muscles, func- tor nucleus via the corticobulbar tract through the thal-
tion to protect the eye, maintain the nasal airway, pro- amus along the posterior limb of the internal capsule.
vide oral continence, and articulate speech. Equally However, a subset of cerebral fibers controlling move-
as important, these mimetic muscles provide humans ment of the frontalis and upper orbicularis oculi proj-
the means for emotional expression and interpersonal ect to both the ipsilateral and contralateral facial motor
communication. This chapter details an anatomic de- nuclei. A central lesion will thus cause frontalis-sparing
scription of the facial nerve and musculature with a fo- facial paralysis due to the bilateral contributions to the
cus on anatomy and physiology as they relate to facial upper face. The precentral gyrus fibers synapse in the
nerve disorder and treatment. facial motor nucleus, residing in the pontine tegmen-
tum. Postsynaptic fibers then travel dorsally within the
brainstem, loop around the floor of the fourth ventricle
FACIAL NERVE
at the facial colliculus, and exit the ventrolateral aspect
The facial nerve provides a diverse range of functions of the brainstem at the caudal border of the pons in the
via efferent and afferent innervation to structures of cerebellopontine angle (Fig. 1.2).
the second branchial arch. The most well-associated Collectively known as the nervus intermedius, pre-
function of the facial nerve is its innervation of striated ganglionic fibers of the superior salivatory nucleus
muscles of facial expression. These functions will be and sensory fibers from the nucleus of tractus solitari-
the focus of this chapter. However, additional effer- us exit the brainstem directly lateral to the facial nerve
ent motor fibers provide innervation to the stapedius motor root. Together, the facial nerve motor root and
muscle, the stylohyoid muscle, and the posterior belly nervus intermedius enter the internal auditory canal
of the digastric. Collectively, these motor fibers repre- (IAC) at the porus acusticus. For the purposes of this
sent the special visceral efferents comprising the majority chapter, the facial motor root and nervus intermedius
of the facial nerve fibers. The remaining efferent fibers will be collectively referred to as the facial nerve.
of the facial nerve are general efferent fibers and form au-
tonomic contributions. These autonomic nerves travel INTRATEMPORAL
via the greater superficial petrosal nerve (GSPN) to the Meatal
lacrimal gland and seromucinous glands of the nasal The facial nerve travels through the temporal bone via
cavity and via the chorda tympani to the subman- a bony channel, the fallopian canal. The first segment of
dibular and sublingual glands. Traveling with this au- the fallopian canal is the meatal segment or IAC. The
tonomic system, visceral afferent fibers supply visceral facial nerve travels in the superior anterior aspect of
sensation to the mucosa of the nose, pharynx, and pal- the IAC. The transverse (falciform) crest separates the
ate. Additional afferent inputs include special sensory facial nerve from the cochlear nerve inferiorly and
fibers and somatic sensory fibers. Special sensory fibers Bill’s bar, a lateral ridge of bone, and separates the fa-
for taste to the anterior two-thirds of the tongue, ton- cial nerve from the superior vestibular nerve posteri-
sillar fossa, and palate originate from the geniculate orly. Within the IAC, the nerve lacks a fibrous sheath
1
2 CHAPTER 1 Facial Nerve and Muscle Anatomy

Geniculate ganglion Solitary nucleus


Gustatory portion of the solitary nucleus Spinal nucleus of
Superior salivatory nucleus trigeminal nerve
Facial colliculus
Nervus Greater petrosal nerve
intermedius Pterygopalatine ganglion

Abducens nucleus
Lacrimal gland
Nasal mucous
glands Facial nucleus
Superior salivatory
nucleus

Taste fibers

NI

Main facial
Corticopontine and nerve
corticospinal fibers
VI

Fig. 1.2 Transverse section of the brainstem showing the intrapontine


Submandibular and course of the facial nerve from the facial motor nucleus, superior
sublingual glands salivatory nucleus, and solitary nucleus. NI, Nervus intermedius. (From
Mtui E, Gruener G, Dockery P. Fitzgerald’s Clinical Neuroanatomy and
Submandibular ganglion
Chorda tympani Neuroscience. 7th ed. Oxford: Elsevier; 2016:218-221.)

Main facial nerve


Fig. 1.1 The Nervus Intermedius Branches and Functional fibers leave the facial nerve as the GSPN. The GSPN
Targets. Blue and pink arrows indicate the direction of nerve carries preganglionic parasympathetic innervation to
impulse. (From Mtui E, Gruener G, Dockery P. Fitzgerald’s
the lacrimal gland and nasal mucosa. These fibers join
Clinical Neuroanatomy and Neuroscience. 7th ed. Oxford: Elsevier;
2016:218-221.) the deep petrosal nerve from the carotid plexus to be-
come the Vidian (or pterygoid) nerve. The thin bone
above the geniculate and anterior attachment of the
or endoneurium and is surrounded by a thin layer of GSPN exerting intraneural traction place the nerve at
arachnoid.1 increased risk of traumatic injury at this location. After
the geniculate fossa, the facial nerve makes an acute
Labyrinthine posterior and slightly inferior turn, nearly 180 degrees,
Exiting the IAC, the facial nerve turns gently anteri- to enter the tympanic segment or horizontal segment.
orly within the otic capsule bone between the cochlea
and superior semicircular canal into the labyrin- Tympanic (Horizontal)
thine segment. This portion is on average 3 to 6 mm The tympanic segment is located along the medial
in length—the shortest and narrowest section of the wall of the anterior attic, travels superomedial to the
fallopian canal. In this segment, the facial nerve is at cochleariform process, and forms the superior wall of
its thinnest and particularly vulnerable to injury. The the oval window niche posteriorly. On average 8 to 11
vascular supply is a watershed between the terminal mm in length, dehiscence of the horizontal fallopian
arterioles of the vertebrobasilar system (via the laby- canal occurs in up to 20% of patients, with 80% above
rinthine branch of the anterior inferior cerebellar ar- the oval window and 12% anterior to the cochleari-
tery [AICA]) and the external carotid artery (via the form process.2 At the pyramidal eminence, the facial
petrosal branch of the middle meningeal artery). There nerve makes a second genu anteroinferior to the lateral
is also a lack of epineurium vascular plexus along the semicircular canal and enters the mastoid or vertical
labyrinthine segment. segment.
At the distal end of the labyrinthine segment, the
nerve travels superior to the cochlea and opens into Mastoid (Vertical)
the geniculate fossa. The geniculate fossa is separated Greater anatomic variability is seen in the vertical
from the middle fossa floor by a thin layer of bone, segment prior to exiting the stylomastoid foramen
which is dehiscent in about 25% of cases. Here the first than any other segment of the fallopian canal. In this
Facial Nerve and Muscle Anatomy CHAPTER 1 3

Stapedius
Facial nerve [VII]
Geniculate ganglion

Stapedius, tendon

Stapes

Tensor tympani, tendon

Incus

Lesser petrosal nerve

Malleus

Nerve to stapedius

Greater petrosal nerve


Pyramidal process

Tensor tympani
Facial nerve [VII]

Canal for tensor tympani

Chorda tympani

Auditory tube,
bony part

Internal carotid artery

Mastoid process
Auditory tube, Petrosphenoidal Tympanic
cartilaginous part fissure membrane

Chorda tympani Stylomastoid


foramen

Stylohyoid branch Posterior auricular nerve

Digastric branch

Stylohyoid

Digastric, posterior belly

Fig. 1.3 Intratemporal Course and Branches of the Facial Nerve. (From Paulsen F, Waschke J. Sobotta Atlas of
Human Anatomy, Vol. 3. 15th ed. Munich, Germany: Urban & Fischer; 2012:133-160; Gleeson M. External and middle
ear. In: Standring S, et al., eds. Gray’s Anatomy. 41st ed. Oxford, UK: Elsevier; 2016:624-640.e1.)

segment, the facial nerve becomes more superficial stylomastoid foramen, which is lined by the aponeuro-
as it travels within the mastoid bone. Between 9 and sis of the posterior belly of the digastric muscle. This
18 mm in length, the mastoid segment contains the aponeurosis directly supplies blood to the facial nerve
chorda tympani and stapedial branches (Fig. 1.3). The as it exits the fallopian canal.
chorda tympani has a variable take off from the mas-
toid segment but typically exits 6 mm above the stylo- EXTRATEMPORAL
mastoid foramen. Coursing superiorly nearly parallel The facial nerve exits the stylomastoid foramen cov-
to the facial nerve, the chorda angles anteriorly as it en- ered in tough connective tissue. At the stylomastoid
ters the mesotympanum. It exits the temporal bone via foramen, the posterior auricular branch exits the facial
the petrotympanic fissure and joins the lingual nerve nerve and supplies general sensory innervation to the
to convey taste information to the anterior two-thirds posterior ear canal and concha, as well as motor inner-
of the tongue and supplies preganglionic parasympa- vation to the auricular muscles and occipitalis muscle.
thetic innervation to the submandibular, lingual, and The digastric branch supplying motor innervation
minor salivary glands. This final segment ends at the to the posterior belly of the digastric muscle and the
4 CHAPTER 1 Facial Nerve and Muscle Anatomy

1 1
2 2
1
2 3

3 3 II
IV
3 4

4
5
VI 5

4 1
1
1
2
5
2
2

3
I
V 3
III 3
4

4
5
I Type I, 13% 1 temporal branch 5
4
II Type II, 20% 2 zygomatic branch
5
III Type III, 28% 3 buccal branch
IV Type IV, 24% 4 mandibular branch
V Type V, 9% 5 cervical branch
VI Type VI, 6%

Fig. 1.4 Branching Patterns of the Extratemporal Facial Nerve. (From Holmes S. Face and scalp. In: Standring S,
et al., eds. Gray’s Anatomy. 41st ed. Oxford, UK: Elsevier; 2015:475-506.)

stylohyoid branch to the stylohyoid muscle also exit the upper orbicularis oculi run along the undersurface
the facial nerve prior to its entrance into the parotid for 3 to 4 mm before entering the muscle to innervate it.
gland. Within the parotid, the facial nerve branch- One common estimate of the frontotemporal branch
es into a larger zygomaticotemporal division and a is Pitanguy’s line, defined by a line draw from 0.5 cm
smaller cervicomandibular division at the pes anseri- inferior to the tragus to 1.5 cm superior and lateral to
nus and terminates in five classic branches: temporal, the eyebrow. Numerous cadaveric studies have at-
zygomatic, buccal, mandibular, and cervical. In reality, tempted to further define the course and branching
the branching pattern of the facial nerve is highly vari- patterns. Ishikawa demonstrated that three to four
able with multiple communications and the majority branches of the FTN were consistently found between
of variations existing among the zygomaticobuccal di- 3.8 and 6.0 cm posterior to the lateral canthus along
visions.3–5 Through the parotid gland, the nerve runs the superior zygomatic arch in both straight-line and
at the level of the retromandibular vein and separates curved trajectories.8
the superficial and deep lobe of the parotid gland. As
it exits the parotid, the nerve has on average 8 to 15 Zygomaticobuccal Division
branches making up the five divisions6 (Fig. 1.4). The The zygomaticobuccal division consists of five to eight
nerve runs deep to the superficial musculoaponeurotic branches with significant overlap of muscle innerva-
system (SMAS) and innervates the majority of facial tions. These nerves innervate the midfacial muscles
muscles from their deep surface. The mentalis, bucci- spanning the lip elevators to the lower orbicularis
nator, and levator anguli oris are innervated on their oculi. Connections between the lower branches and
superficial surface as these are the deepest layer of fa- marginal mandibular division also exist. Dorafshar
cial muscles. et al. describe “Zucker’s point” as a reliable surface
landmark for identifying the zygomaticobuccal nerve
Temporal Division as it exits the parotid.9 The authors found that the mid-
The temporal division, also known as the frontal divi- point of a line drawn from the root of the helix to the
sion or frontotemporal branch, consists of three to four oral commissure predicted the nerve location within
branches traveling obliquely in between the tempo- an average of 2.3 mm.
roparietal fascia and the superficial layer of the deep
temporal fascia.7 Branches entering the frontalis mus- Marginal Mandibular Division
cle at the level of the supraorbital ridge are located up The marginal mandibular division consists of one to
to 3 cm above the lateral canthus. Branches entering three branches beginning up to 2 cm below the ramus
Facial Nerve and Muscle Anatomy CHAPTER 1 5

of the mandible and arcing upward to cross the man- previously, the transition zone of vascular supply be-
dible halfway between the angle and mental protuber- tween the AICA and the external carotid centers on the
ance. Branches lie on the deep surface of the platysma labyrinthine segment and creates a watershed or weak
and cross superficial to the facial vessels 3.5 cm from zone of arterial supply.
the parotid edge. There are separate branches to the The extrinsic vascular network consists of one or
depressor angularis, depressor labii inferioris (DLI), two main arterial trunks and accompanying venae co-
and mentalis, and variable superior ramus supplying mitantes running between the periosteum of the fal-
upper platysma and lower orbicularis oris. lopian canal and the epineural sheath of the nerve. An
intrinsic vascular network also exists within the epi-
Cervical Division neurial sheath consisting of small arterioles, capillar-
The cervical division consists of one branch leaving ies, and venules.12
the parotid and running on the deep surface of the pla-
tysma. The point of entry into the muscle is 2 to 3 mm
FACIAL MUSCLES
caudal to the platysma muscle branch of the facial ves-
sel. It enters the muscle at the junction of its cranial and The striated muscles of facial expression derive from
middle thirds. the second branchial arch mesoderm and reside within
the SMAS layer. The SMAS layer continues superiorly
with the galea aponeurotica and inferiorly with the
MICROSCOPIC ANATOMY platysma muscle. A total of 17 paired and 1 unpaired
The microanatomy of the facial nerve reveals a het- sphincter muscle constitute the facial musculature.
erogenous organization that explains in part the dif- These originate from the periosteum of the facial bones
ficulty of facial nerve repair. As described by Captier and insert into the skin, allowing for a limitless num-
et al., the facial nerve lacks fascicular organization as ber of facial expressions (Fig. 1.5).
well as epineural or perineural covering from its exit The facial muscles possess a three-dimensional rela-
at the brainstem to the geniculate ganglion.10 Prior to tionship to one another and exist in four layers based
the geniculate ganglion, the nerve is surrounded only on muscle origin as demonstrated by Freilinger et al.13
by an arachnoid sheath. A thin epineural sheath arises The orbicularis oculi, depressor anguli oris (DAO), and
within the tympanic segment and thickens as the nerve superficial aspect of the zygomaticus minor are most
travels towards the stylomastoid foramen. The peri- superficially located as the first layer. The platysma,
geniculate facial nerve demonstrates one or two fascic- risorius, zygomaticus major, deeper portion of the zy-
ular bundles that increase in number and decrease in gomaticus minor, and levator labii superioris alaeque
size as the nerve travels distally through the fallopian nasi compose the second layer. The levator labii supe-
canal. The number of fascicles and their spatial organi- rioris and orbicularis oris represent the third layer. The
zation changes every 2 mm within the fallopian canal. deepest layer is composed of the levator anguli oris,
At the stylomastoid foramen the facial nerve has on the mentalis, and the buccinator—these three muscles
average 11 fascicles and up to 16. As individual fibers are also the only muscles innervated from their super-
emerge, they are surrounded by perineurium and en- ficial surface, as explained by this relationship.
doneurium. In the horizontal segment, the upper mo- Distinct from most skeletal muscles, facial muscles
tor division has been reported to be more superficial are flat, strap-like muscle sheets with interdigitations
(or lateral), whereas the lower motor division has been to the skin, short or absent tendons, and an absent fas-
reported to lie deeper (or medial).11 The nerve bundles cial covering. A comprehensive list and description of
have a spatial organization as they traverse the mastoid facial muscles are detailed in Table 1.1. The most clini-
segment: branches to the lower division lie within the cally relevant facial muscles are described in further
anterior portion of the nerve and branches to the upper detail in the following sections.
division course posteriorly within the epineurium.11
From the brainstem to the mastoid segment, an aver- FRONTALIS
age of 7800 myelinated nerve fibers are maintained. A The frontalis muscle is a broad, thin, bilateral muscle
single neuron can innervate up to 25 muscle fibers. originating from the galea aponeurotica near the coro-
nal suture and inserting onto the superciliary ridge of
VASCULAR ANATOMY the frontal bone and interdigitating with fibers of the
The intracranial (brainstem and IAC) facial nerve re- orbicularis oculi, procerus, and corrugator supercilia.
ceives blood supply from branches of the AICA aris- Densely adherent to the overlying skin, the frontalis
ing from the vertebrobasilar system. The labyrinthine glides over the underlying periosteum to provide brow
artery, a branch of the AICA, provides vascular input elevation. The resting tone of the frontalis also prevents
within the IAC segment. However, the remaining in- brow descent and ptosis. Left- and right-sided frontalis
tratemporal segments receive blood supply through bellies fuse in the midline caudally, often as a fibrous
the middle meningeal artery, a branch of the maxil- junction. The frontalis and occipitalis bellies can be de-
lary artery from the external carotid. As described scribed as distinct muscle bellies or as parts of a single
6 CHAPTER 1 Facial Nerve and Muscle Anatomy

Epicranial aponeurosis

Occipitofrontalis, frontal belly

Depressor supercilii
Procerus

Corrugator supercilii
Orbicularis oculi, palpebral part Levator labii superioris
alaeque nasi
Levator labii superioris Nasalis
alaeque nasi
Orbicularis oculi, orbital part
Levator labii superioris
Levator labii superioris
Zygomaticus minor
Zygomaticus minor
Zygomaticus major
Zygomaticus major
Parotid gland

Orbicularis oris, marginal part


Levator anguli oris
Buccal fat pad
Depressor septi nasi
Risorius Buccinator

Depressor anguli oris


Masseter, superficial part
Depressor labii inferioris Orbicularis oris, labial part

Depressor anguli oris


Mentalis
Depressor labii inferioris

Platysma

Fig. 1.5 Facial Musculature (Frontal View). (From Zuker RM, Gur E, Hussain G, Manktelow RT. Facial paralysis. In:
Neligan PC, ed. Plastic Surgery: Volume 3: Craniofacial, Head and Neck Surgery and Pediatric Plastic Surgery. 4th ed.
Philadelphia: Elsevier; 2018:329-357.e2.)

occipitofrontalis muscle connected by an intermediate movement. The preseptal portion covers the orbital
tendon within the galea aponeurotica. septum and is under more voluntary control. It is less
closely adherent to the skin other than at the medial
ORBICULARIS OCULI and lateral canthi. Both the preseptal and pretarsal
The orbicularis oculi muscle is a paired sphincter components function together during blink. The or-
important in eyelid closure. The pretarsal, preseptal, bital component forms a ring over the bony orbital
and orbital subdivisions of the orbicularis oculi are margin and is recruited during forceful eye closure
defined by the anatomic level of the muscle. The pre- as well as brow depression. This component origi-
tarsal portion is closely adherent to the pretarsal skin, nates medially from the superomedial orbital margin,
covers the tarsal plate and provides reflexive blink the maxillary process of the frontal bone, the medial
Facial Nerve and Muscle Anatomy CHAPTER 1 7

Table 1.1   Facial Muscles.


MUSCLE ACTION ORIGIN/INSERTION DESCRIBED DIMENSION
MUSCLES OF THE SCALP
Frontalis Raises forehead and eyebrow O: Galea aponeurotica near the Width: 60.9 +/- 8.7 mm19
Occipitalis Draws scalp backwards coronal suture Height: 31.7 +/- 7.5 mm19
I: Superciliary ridge of the frontal
bone (frontalis) and occipital
bone (occipitalis)
Auricularis Anterior Draws auricle forward and O: Galea aponeurotica
upward I: Auricular cartilage
Auricularis Superior Draws auricle upwards
Auricularis Posterior Draws auricle backwards
Temporoparietalis Tightens scalp, raises auricle O: Galea aponeurotica
and temple I: Auricularis muscles
MUSCLES OF THE EYELIDS
Orbicularis Oculi Sphincter muscle of eyelid, O: Frontal bone, maxillary bone, Length: 65 +/- 5.6 mm15
eye closure medial palpebral ligament Width: 60 +/- 9.6 mm15
I: Lateral horizontal raphae
Corrugator Supercilii Draws eyebrow inferior and O: Medial orbital rim and frontal Length: 38–53 mm15
medial (vertical forehead bone
wrinkles) I: Frontalis, orbicularis oculi and
skin20
Depressor Supercilii Depressor of eyebrow O: Frontal process of maxilla
above medial canthal tendon
I: Dermis superior to the medial
canthal tendon
MUSCLES OF THE NOSE
Procerus Draws medial eyebrow O: Caudal nasal bone
downwards (transverse I: Skin of glabella and medial
wrinkles of nose) brow
Nasalis Transverse: depresses O: Maxilla over lateral incisor
cartilaginous part of nose and I: Procerus/Glabellar skin and
draws ala toward the septum lower lateral cartilage
Alar: enlarges nasal aperture
Depressor Septi Nasi Draws ala downwards, O: Incisive fossa of maxilla
constricts aperture I: Nasal septum
MUSCLES OF THE MOUTH
Levator Labii Superioris Raises the medial aspect of O: Inferior orbital rim and maxilla Length: 47 +/- 7.5 mm15
upper lip I: Orbicularis oris of upper lip Males: 33.67 +/- 4.13 mm13
Females: 35.5 +/- 6.69 mm13
Levator Labii Superioris Raises the medial aspect of O: Medial angle of orbital rim 61.6 +/- 7.6 mm13
Alaeque Nasi upper lip and dilates the nostril I: Levator labii superioris Width: 7.2 +/- 1.7 mm15
Levator Anguli Oris Elevates angle of the mouth O: Maxillary canine fossa Length: 42 +/- 2.5 mm15
I: Orbicularis oris of upper lip Male length: 37.83 +/- 4.38
and modiolus mm13
Female length: 38.33 +/- 8.02
mm13
Zygomaticus Major Elevates angle of the mouth O: Zygomatic Arch Length: 65.6 +/- 3.8 mm15
upward and backward I: Modiolus Male length: 70.67 +/- 6.32
mm13
Female length: 69.50 +/- 6.48
mm13
Zygomaticus Minor Elevates upper lip backward, O: Zygomatic Arch Length: 51.8 +/- 7.4 mm15
upward, and outward I: Modiolus
Risorius Retracts angle of the mouth O: Parotid fascia
laterally I: Modiolus
Continued
8 CHAPTER 1 Facial Nerve and Muscle Anatomy

Table 1.1   Facial Muscles—cont’d


MUSCLE ACTION ORIGIN/INSERTION DESCRIBED DIMENSION
Depressor Labii Inferioris Draws lower lip downward and O: Mandible inferior to mental Length: 29 +/-4.9 mm15
laterally foramen
I: Modiolus, orbicularis oris, skin
of lower lip
Depressor Anguli Oris Depresses angle of the mouth O: Mandible inferior to mental Length: 48 +/- 5.1 mm15
foramen
I: Modiolus, orbicularis oris
Mentalis Raises and protrudes lower lip, O: Anterior mandible
wrinkles skin of the chin I: Chin skin
Orbicularis oris Sphincter function of lips, closure Neither bony nor tendinous
of lips origin, integration with perioral
muscles
Buccinator Compresses/flattens the cheek O: Maxilla, pterygomandibular Length: 56 +/- 7.4 mm15
raphe, mandible
I: Modiolus
Platysma Tightening of the neck, O: Supraclavicular skin
downward pull on lower lip I: Continuous with SMAS
  I, Insertion; O, Origin; SMAS, superficial musculoaponeurotic system.

Levator labii superioris alaeque nasi


Levator anguli oris
Levator labii superioris

Zygomaticus minor

Zygomaticus major
Buccinator
Modiolus

Risorius

Platysma

Depressor anguli oris

Depressor labii inferioris Orbicularis oris


Mentalis
Fig. 1.6 Periorbital Muscles Including Lip Elevators and Depressors. (From Drake R, Vogl W, Mitchel A. Gray’s
Basic Anatomy. 2nd ed. Philadelphia: Elsevier; 2018:413-596.)

canthal tendon, the frontal process of the maxilla, and The zygomaticus major is the most superficial of the
the inferomedial margin of the orbit. Within the most three and originates from the zygomatic bone in front
superficial layer of facial muscles, the orbital sub- of the zygomaticotemporal suture. It runs to the angle
division laterally overlies the temporalis fascia, the of the mouth where superficial fibers form the modio-
origins of the zygomaticus major, and levator labii lus together with the DAO, the risorius, the orbicularis
muscles. oris, the buccinator, and the levator anguli oris. The
modiolus represents the interdigitation of all the peri-
LIP ELEVATORS oral muscles. The deeper insertion of the zygomaticus
The perioral muscles contributing to lip elevation and major fuses with the levator anguli oris and the me-
smile are of particular interest to patients and surgeons dial fibers line on the buccinator muscle. The caudal
in facial rehabilitation. The main lip elevators are the fibers continue into the DAO. The zygomaticus muscle
zygomaticus major, the levator labii superioris, and le- elevates the commissure superiorly and laterally at an
vator anguli oris (Fig. 1.6). approximately 45-degree angle.
Facial Nerve and Muscle Anatomy CHAPTER 1 9

The levator labii superioris arises from the lower


margin of the orbit above the infraorbital foramen,
more medially located compared to the zygomati-
cus major. Its medial and upper insertion helps
form the nasolabial sulcus with partial insertion
into this crease. Its lateral fibers descend superfi-
cial to the orbicularis oris and deeper fibers inter-
digitate with the modiolus. Medial levator labii su-
perioris fibers reach the upper lip philtral columns
and insert into the medial vermilion border as far
as the peak of Cupid’s bow. The levator labii su-
perioris elevates the lip vertically and laterally to
expose the upper teeth and deepen the nasolabial
fold (Video 1.1).
The levator anguli oris originates from the maxillae Fig. 1.7 Lower Lip Depressors. Nerve depicted here is the mental
nerve, a branch of the third division of the trigeminal nerve. (From
below the infraorbital foramen, fills the canine fossa, Zuker RM, Gur E, Hussain G, Manktelow RT. Facial paralysis. In:
and runs vertically. It inserts into the modiolus and Neligan PC, ed. Plastic Surgery: Volume 3: Craniofacial, Head and Neck
is in the deepest layer of facial muscles. The levator Surgery and Pediatric Plastic Surgery. 4th ed. Philadelphia: Elsevier;
anguli oris serves to elevate the commissure vertically 2018:329-357.e2.)
and medially.
The origins and insertions for the zygomaticus ma- When acting in conjunction with the platysma
jor, levator labii superioris, and levator anguli oris muscle, the DLI and DAO depress the entire lower lip
determine the vectors of pull. The relative strength of producing a full denture smile. The mentalis muscle
each muscle interdigitating in the modiolus and orbi- similarly originates from around the mental foramen
cularis oris determines the smile configuration unique but passes medially or inferomedially. The mentalis
to each patient. muscle is the deepest layer of the lower lip muscles
and acts to raise the chin with an indirect action of
ORBICULARIS ORIS lower lip elevation.
The orbicularis oris is the only unpaired muscle of fa-
cial expression and serves as a sphincter of the mouth MICROARCHITECTURE
essential for oral competence in both speech and the Most facial muscles exhibit muscle fiber density equal
oral phase of swallow. Superficially, portions of the to other skeletal muscles such as the biceps and tibi-
muscle fibers insert into the philtra columns as well alis anterior.16 Fiber density varies between 1000 and
as along the vermilion border to form the white roll. 1400 fibers/mm2 for the majority of facial muscles,
Superficial components of the muscle can contract in- with the orbicularis oculi and oris having the highest
dependently to provide nuanced expression of the lips density (over 2000 fibers/mm2). The total number of
and the deeper component functions as the main con- muscle fibers varies between 5400 fibers (zygomaticus
strictor. Upper and lower lip depressors interdigitate minor) and 9630 fibers (DAO). Muscle fiber size varies
with the orbicularis oris and work in concert for lower between facial muscles. The frontalis, orbicularis oris,
facial expression. and orbicularis oculi contain smaller muscle fibers (less
than 400 μm2). The zygomaticus major, DAO, buccina-
LIP DEPRESSORS tor, zygomaticus minor, DLI, and levator labii superi-
The lower lip musculature is as functionally complex oris contain intermediate muscle fibers (between 400
as the upper lip. An intricate three-dimensional assem- and 500 μm2). The platysma, levator anguli oris, and
bly of muscular sheets control the shape of the mouth risorius contain large muscles (greater than 500 μm2).
and position of the lips. The DLI, DAO, and platysma Facial muscles also demonstrate a great variability in
comprise the lower lip depressors (Fig. 1.7). fast- versus slow-twitch fiber composition, likely related
The DLI originates along the alveolar bone of the to the multitude of functions they exhibit. Predominantly
mandible inferior and lateral to the mental foramen. It tonic or slow-twitch muscles contain a high percentage
travels laterally to insert into the orbicularis oris mus- of type 1 fibers and predominantly phasic or fast-twitch
cle along its deep surface and attaches to the vermilion muscles contain a high percentage of type 2 fibers. Facial
and middle third lip skin through fibrous septa.14,15 muscle compositions range from 14% to 67% type 1 mus-
The DLI acts to depress and lateralize the lower lip, cle fibers.17 The most phasically composed facial muscle
evert the vermilion, and produce lower dental show. is the orbicularis oculi and the most tonically composed
The DAO arises from the mandible lateral and su- facial muscle is the buccinator (Table 1.2).
perficial to the DLI origin. It inserts directly into the Facial muscles have a unique microarchitecture
skin at the labiomandibular crease medially and into compared to other skeletal muscles in the body and a
the modiolus laterally. Its main action is to draw the complex pattern of innervation. Most human skeletal
corner of the mouth down and medial during frowning. muscles consist of muscle fibers up to 18 cm in length
10 CHAPTER 1 Facial Nerve and Muscle Anatomy

Tablev 1.2   Fiber Composition of Facial Muscles. CONCLUSION


MUSCLE TYPE 1 (%)/TYPE 2 (%) Facial nerve and muscle anatomy elegantly demon-
Orbicularis oculi 15/85 strates the complexities of the head and neck region.
Zygomaticus major 28/72 Comprehensive understanding of the anatomic course
Orbicularis oris 29/71 and contributions of the facial nerve is critically im-
portant for physicians treating facial nerve disorders
Levator labii superioris 31/69
both surgically and medically. Further research and
Depressor anguli oris 37/63 understanding of nerve and muscle microarchitecture
Corrugator superioris 41/59 is critical in understanding the nuances of facial move-
Depressor labii inferioris 46/54 ment and emotional expression.
Occipitofrontalis 57/43
Buccinator 67/33 REFERENCES
Adapted from Freilinger G, Happak W, Burggasser G, Gruber H. Histochemical 1. Miehlke A, Fisch U, Eneroth C-M. Surgery of the Facial Nerve.
mapping and fiber size analysis of mimic muscles. Plast Reconstr Surg. 2nd ed. Philadelphia: Saunders; 1973.
1990;86(3):422-428. 2. Moody MW, Lambert PR. Incidence of dehiscence of the
facial nerve in 416 cases of cholesteatoma. Otol Neurotol.
2007;28(3):400–404.
with a single motor end plate (MEP) in the middle of 3. Tzafetta K, Terzis JK. Essays on the facial nerve: Part I.
the fiber.15 These MEPs form a narrow band that tra- Microanatomy. Plast Reconstr Surg. 2010;125(3):879–889.
4. Katz AD, Catalano P. The clinical significance of the various
verses across the central zone of the skeletal muscles,
anastomotic branches of the facial nerve. Report of 100 pa-
known as a motor band. Facial muscles differ signifi- tients. Arch Otolaryngol Head Neck Surg. 1987;113(9):959–962.
cantly from skeletal muscles in innervation pattern, 5. Davis RA, Anson BJ, Budinger JM, Kurth LR. Surgical anato-
location of motor bands, and arrangement of MEPs. my of the facial nerve and parotid gland based upon a study
These differences may be partially attributed to the of 350 cervicofacial halves. Surgery, Gynecology & Obstetrics.
1956;102(4):385–412.
elaborate branching pattern of the facial nerve. Within
6. Zuker RM, Gur E, Hussain G, Manktelow RT. Facial paraly-
the parotid gland, the facial nerve divides into multiple sis. In: Neligan PC, ed. Plastic Surgery: Volume 3: Craniofacial,
branches with an elaborate network of anastomosis and Head and Neck Surgery and Pediatric Plastic Surgery. 4th ed.
interlaced branches before entering the facial muscles. Philadelphia: Elsevier; 2018:329–357.e2.
Each facial muscle is innervated by multiple terminal 7. Pitanguy I, Ramos AS. The frontal branch of the facial nerve:
the importance of its variations in face lifting. Plast Reconstr
branches of the facial nerve without an accompany-
Surg. 1966;38(4):352–356.
ing vascular pedicle. Happak et al. demonstrate that 8. Ishikawa Y. An anatomical study on the distribution of the
upon entrance into the muscle, round or oval-shaped temporal branch of the facial nerve. J Cranio-Maxillo-Fac
clusters of MEPs are found in the immediate vicin- Surg. 1990;18(7):287–292.
ity of the nerve entrance, described as motor zones. 9. Dorafshar AH, Borsuk DE, Bojovic B, et al. Surface anatomy
of the middle division of the facial nerve: Zuker’s point.
Unlike the central motor band architecture of skeletal
Plast Reconstr Surg. 2013;131(2):253–257.
muscles, motor zones are spread over facial muscles in 10. Captier G, Canovas F, Bonnel F, Seignarbieux F. Organization
a de-centralized fashion with considerable variability and microscopic anatomy of the adult human facial nerve:
determined by pattern of facial nerve branching. The anatomical and histological basis for surgery. Plast Reconstr
orbicularis oris, orbicularis oculi, and buccinator mus- Surg. 2005;115(6):1457–1465.
11. May M. Anatomy of cross section of facial nerve in the tem-
cles demonstrate a great number of small MEP clusters
poral bone: clinical application. In: Fisch U, ed. Facial Nerve
spread evenly over the muscle. The zygomaticus ma- Surgery. Amstelveen, The Netherlands: Kugley Medical
jor demonstrates a dominant motor zone with two to Publications; 1977:40–46.
three additional smaller motor zones. The levator labii 12. Bagger-Sjoback DGM, Thomander L. The intratemporal vas-
superioris contains two to four large motor zones in ec- cular supply of the facial nerve: a light and electron micro-
scopic study. In: Graham MD, House WF, eds. Disorders of
centric positions.
the Facial Nerve: Anatomy, Diagnosis, and Management. New
In experimental studies, facial muscle fibers dem- York: Raven Press; 1982:17–31.
onstrate a “multiply innervated muscle fiber pattern” 13. Freilinger G, Gruber H, Happak W, Pechmann U. Surgical
with multiple MEPs within a distance ranging from anatomy of the mimic muscle system and the facial nerve:
25 to 500 μm on a single muscle fiber. This pattern of importance for reconstructive and aesthetic surgery. Plast
Reconstr Surg. 1987;80(5):686–690.
innervation is also found in laryngeal muscles, per-
14. Hur MS. Anatomical relationship of the inferior bundle of
haps pointing towards a system capable of fine adjust- the incisivus labii inferioris with the depressor labii inferio-
ments.18 It is not yet known if multiple end plates on ris and the platysma. J Craniofac Surg. 2017;28(7):1861–1864.
a single facial muscle fiber are innervated mono- or 15. Happak W, Liu J, Burggasser G, Flowers A, Gruber H,
polyneuronally. The plexus of the facial nerve and high Freilinger G. Human facial muscles: dimensions, motor end-
plate distribution, and presence of muscle fibers with multi-
branching pattern suggest a polyneuronal innervation
ple motor endplates. Anat Rec. 1997;249(2):276–284.
could be present. Further studies are needed to under- 16. Ito J, Moriyama H, Shimada K. Morphological evaluation of
stand the mechanisms of emotional expression and the human facial muscles. Okajimas Folia Anatomica Japonica.
neural control. 2006;83(1):7–14.
Facial Nerve and Muscle Anatomy CHAPTER 1 11

17. Freilinger G, Happak W, Burggasser G, Gruber H. 19. Jeon A, Kim SD, Han SH. Morphological study of the occipi-
Histochemical mapping and fiber size analysis of mimic tal belly of the occipitofrontalis muscle and its innervation.
muscles. Plast Reconstr Surg. 1990;86(3):422–428. Surg Radiol Anat. 2015;37(9):1087–1092.
18. Rossi G. From the pattern of human vocal muscle fibre in- 20. Hwang K, Lee JH, Lim HJ. Anatomy of the corrugator mus-
nervation to functional remarks. Acta Oto-laryngologica cle. J Craniofac Surg. 2017;28(2):524–527.
Supplementum. 1990;473:1–10.
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Etiology, Epidemiology, and Pathophysiology
of Post-Facial Paralysis Synkinesis
2
Kofi D.O. Boahene

The neural activity responsible for voluntary facial ex- causes of facial paralysis, such as Bell’s palsy, Ramsey
pression relies on an intricate set of interconnections Hunt syndrome, trauma, and postsurgical injury, have
distributed across multiple cortical facial motor areas, all been associated with synkinesis to varying degrees.
the amygdala, and other subcortical areas.1 The tar- In one study, synkinesis was clinically observed in 55%
get end-organs for this intricate neural system are the of patients.3 Using electrophysiologic criteria, synki-
facial muscles directly innervated by last order post- netic movement can be detected in nearly all patients
synaptic axons in the facial nerve. The muscles of the with proximal facial nerve injury.4 In one of the larg-
upper and lower face are controlled by anatomically est studies evaluating the natural course of idiopathic
distinct motor areas reliant on distinct patterns of neu- peripheral facial nerve palsy, synkinesis was recorded
ral activity. Disruption of this intricate neural network, in about 16% of patients.5 In a retrospective study of
when followed by spontaneous recovery or surgical 102 patients with untreated Ramsay Hunt syndrome,
repair, is commonly associated with abnormal facial all patients who initially developed complete paralysis
movements such as synkinesis. Synkinesis is the unde- also developed synkinesis, whereas synkinesis was re-
sired synchronization of an involuntary muscle group corded in 10% to 15% of those with incomplete paraly-
triggered by a desired voluntary contraction of another sis.6 Pregnancy, advanced age, and diabetes have been
muscle group. Synkinesis can occur in any functional studied as potential risk factors for Bell’s palsy but
muscle group in the body but is commonly seen after have not shown to be linked with severity of synkine-
facial nerve injury. A typical example of facial synki- sis. However, there is a clear relationship between the
nesis is seen when a voluntarily blink is accompanied severity of facial palsy and the degree of synkinesis.
by co-contraction of lower facial muscles around the This link is further supported by electrophysiologic
lips (ocular-oral synkinesis) and when narrowing of studies showing that patients with a prognostic elec-
the palpebral fissure occurs when smiling (oral-ocular troneurography (ENoG) value of less than 10% have
synkinesis). In its mildest form, facial synkinesis is tol- higher risks of aberrant regeneration and moderate-to-
erable and often ignored by patients but can be very severe synkinesis than those with an ENoG value of
debilitating when severe. 10% to 20%.7
The pathological mechanism of synkinesis is not
completely elucidated and as a result, current treat-
THE FACIAL NEUROMUSCULAR NETWORK
ment options have mixed efficacy. Presently, synkinesis
treatment is targeted towards peripheral facial nerves The facial nerve is a mixed motor and sensory nerve
and muscles in the form of chemodenervation, selec- composed of approximately 10,000 neurons. About
tive myectomy and selective neurectomy.2 To mini- 7000 of these neurons are motor and innervate the
mize the sequelae of synkinesis post facial nerve injury, muscles of facial expression. The motor fibers originate
and to offer more effective rehabilitative and therapeu- from the motor face area of the frontal lobe and project
tic treatment, it is necessary to better understand the via the corticobulbar tract and the internal capsule to
response of peripheral nerves to injury, the process of the facial nucleus. These afferent motor fibers synapse
nerve regeneration, electrophysiologic changes in pe- with nuclei within the facial nucleus arranged into four
ripheral facial nerves post injury, and any associated major subgroups: dorsomedial, ventromedial, inter-
cortical adaptations. mediate, and lateral. Each nuclei subgroup projects to
a specific group of facial muscles and explains, in part,
the intricate voluntary control of facial muscle move-
ETIOLOGY AND EPIDEMIOLOGY ment. The degree of nuclei representation and projec-
Faulty regeneration is the norm and not the exception tion to muscle groups are related to the functional and
following facial nerve injury of any cause. Facial syn- behavioral importance of each muscle. For example,
kinesis has been reported in a wide range of ages from the subnuclei representing the orbicularis oris and oc-
infants to elderly patients and does not appear to be uli are prominently represented compared to those of
limited to a specific demographic. The most common less important muscles such as the depressor septi nasi
13
14 CHAPTER 2 Etiology, Epidemiology, and Pathophysiology of Post-Facial Paralysis Synkinesis

or auricularis muscles. Much of what is known about regeneration. This transformation to a repair-sustain-
this facial nucleus sub-innervation in humans is ex- ing Schwann cell phenotype is mediated by a complex
trapolated from tracer injection studies in primates.8,9 cellular process that is time dependent. Early after
The corticobulbar projections were defined by inject- nerve injury, the Schwann cells distal to the damaged
ing anterograde tracers into the face representation of area lose contact with axons as they degenerate. The
each motor cortex, and the musculotopic organization loss of contact is by itself a trigger for Schwann cell
of the facial nucleus was defined by injecting fluores- transformation. Invading macrophages secrete bio-
cent retrograde tracers into individual muscles of the active factors which, together with the loss of axonal
upper and lower face. Following these injections, the contact, influence the change in surrounding Schwann
facial nucleus was noted to received input from all face cells toward a repair type cell.10
representations. Injections in all cortical face represen- Within their original basal lamina tubes, these
tations labeled terminals in all nuclear subdivisions transformed repair type Schwann cells aid in clearing
(dorsal, intermediate, medial, and lateral). However, degenerated myelin by autophagy. The released cyto-
significant differences occurred in the proportion of kines call in macrophages for further myelin clearance.
labeled boutons found within each functionally char- The Schwann cells elongate forming regeneration
acterized subdivision. tracts, Bunger bands, which guide regenerating ax-
Once the facial nerve exits the brain stem, the dis- ons towards their target. Axons that successfully grow
crete compartmentalization of motor neurons destined across the regeneration zone induce transformation of
for specific muscle groups is lost.10 Consequently, the fi- the repair Schwann cells back to the myelin phenotype
bers to all facial muscles are distributed throughout the and become ensheathed in a new myelin membrane.
nerve at all levels before peripheral branching occurs in This remarkable, adaptive injury response of neurons
the parotid. Within the parotid gland, the facial nerve and Schwann cells is clinically inadequate in severe
divides into a superior (temporofacial) and an inferior and long-lasting injuries. There are several reasons
(cervicofacial) division, interconnected but containing for this inadequacy.16,17 First, the early regeneration-
fibers destined to specific muscle groups. The delicate enabling environment that enables nerve regeneration
musculotopic arrangement of the facial nucleus and fa- is unstable as the number Schwann cell dwindles. The
cial nerve is disrupted following facial nerve injury and number of cells that can be isolated from nerves after
explains in part abnormal motor recovery.10 6 months of chronic denervation is only 10% to 15% of
that obtained at 4 weeks. Second, with prolonged de-
nervation, the remaining Schwann cells become less ef-
WHEN A NERVE IS INJURED fective with surviving cells gradually down-regulating
The axons that make up a motor nerve are myelinated their repair phenotype, followed by the death of cells
and arranged in a fascicular architecture that is both that have by that time lost most of their regeneration-
protective and suited for rapid nerve conduction. The supportive properties.16 Clinically suboptimal rein-
myelin sheath is made and maintained by Schwann nervation of the facial muscles manifest as hypotonus,
cell membranes wrapped compactly around the axon. hypertonia, myokymia, or synkinesis.
Myelin is in turn coated by a basal lamina, outside of
which lies the endoneurium, which contains fibro-
PROPOSED MECHANISMS FOR SYNKINESIS
blasts, blood vessels, and a few macrophages, which is
ultimately surrounded by a multilayered cellular tube, Three main theories—aberrant regeneration of facial
the perineurium. This assembly of axons, Schwann nerve fibers, ephaptic transmission of impulses, and
cells, and connective tissue makes up a nerve.11 Injury somatotopic reorganization of the facial nucleus—
to the facial nerve may disrupt the nerve architecture have been forwarded as potential mechanisms for
to varying degrees depending on the extent of insult. synkinesis. More recently, there has been increasing
Seddon and Sunderland classified the fundamental understanding of the potential role played by electro-
types of nerve injury based on the extent of disruption physiologic changes in the peripheral nerve and corti-
of the endoneurium and perineum as well as the integ- cal adaptations in postinjury synkinesis.
rity of the axon.2 The imperfect response of peripheral
nerves to injury underlies the clinical manifestation of ABERRANT REGENERATION
synkinesis. Injuries that disrupt the endoneurium can result in
In response to injury, both neurons and Schwann interrupted and misaligned regeneration tracts. In
cells switch to a cell state suited for nerve repair and addition, mechanical barriers at the injury segment
regeneration.11 In damaged neurons, the signal switch compounded by the slow growth of proximal axonal
to repair mode is heralded by the activation of an ex- stumps limits the number of axons that successfully
tensive gene program that facilitates axonal regen- cross, resulting in misrouting and reinnervation errors.
eration, a response classically referred to as the cell Misrouted axons meant for the orbicularis oris muscle
body response.11–15 Similarly, Schwann cells change end up in the orbicularis oculi muscle and result in
into a phenotype primed for repair to facilitate nerve ocular-oral synkinesis. Recent studies have suggested
Etiology, Epidemiology, and Pathophysiology of Post-Facial Paralysis Synkinesis CHAPTER 2 15

that the opportunity for misdirection of axons does not postinjury period within a time line that is neither ex-
only occur at the injury site, but that aberrant axonal plained by aberrant nerve regeneration or ephaptic im-
growth occurs throughout the length of the nerve.18 pulse transmission given that axons grow at a rate of
The most commonly observed clinical patterns of only 1 mm/day. Nuclear hyperexcitability is believed
synkinesis are predictable rather than random, with to occur when inhibitory signals to the facial nucleus
mouth and eyelid coupling the most common occur- are lost in response to peripheral facial nerve injury.
rence. The mechanism of misrouted axons is the most Stripping of synaptic input into the facial nucleus has
commonly stated reason for synkinesis and underlies been observed within days of facial nerve injury.22 The
the treatment method of selective neurectomy, che- time line for this synaptic stripping matches the obser-
modenervation, and myectomy. vation of synkinesis as early as 4 to 8 weeks following
the onset of idiopathic facial palsy.22,23
PERIPHERAL EPHAPTIC TRANSMISSION The first evidence for disconnection of synapses fol-
The ability of Schwann cells to restore myelin sheath in lowing peripheral nerve injury in humans was report-
extensively degenerated nerve is imperfect and incom- ed in an autopsy study of a 77-year-old man who died
plete. Consequently, regenerated axons with defective from an unrelated cause 3 months after developing left
myelin membranes can result in “short circuiting” facial paresis from otitis media.24 Microscopic evalu-
with cross-excitation of adjacent axons meant for dif- ation and immunohistochemistry revealed a striking
ferent target muscles. Ephaptic coupling of transmis- reduction in the number of afferent synaptic contacts
sion between adjacent axons is distinct from impulse and marked chromatolytic changes in the left facial
spread across synapsis. Rather it is the spreading of nucleus. Immunocytochemistry for synaptophysin re-
impulses along and across adjacent axons such that vealed a marked loss of afferent synaptic contacts from
action potential propagating along one axon fires up somatic and stem dendritic surface membranes of all
an adjacent axon. The term ephaptic was coined from chromatolytic motor neurons. Wrapping of a number
the Greek word “ephapse” signifying the act of touch- of neurons by newly formed glial fibrillary acidic pro-
ing (as opposed to “synapse” or linking) by Arvanitaki tein-positive astrocytic cell processes could be detected
in a study of nerve conduction in giant axons of Sepia in the regenerating facial motor nucleus. These chang-
officinalis (common cuttlefish).19 Since then, the term es were absent from the contralateral, normal-appear-
ephaptic interaction has been used to refer to com- ing facial nucleus. The marked occurrence of synaptic
munication between neuronal cells via electrical con- stripping, preferentially of inhibitory nerve, can ex-
duction through the surrounding extracellular space, plain enhanced nuclear hyperexcitability and poorly
as opposed to communication mediated by chemical controlled bursts of undesired muscle contraction.
synapses or gap junctions. Ephaptic transmission is
usually inhibited by myelination but can occur when AXONAL HYPEREXCITABILITY
remyelination is incomplete. The coexcitation of differ- Fundamental to nerve conduction is the maintenance
ent muscle groups will, as a result of ephaptic impulse of a resting membrane potential and rapid impulse
transmission, result in synkinesis. The concept of ep- propagation due to the exchange of sodium (Na+) and
haptic coupling of adjacent axons was investigated by potassium (K+) across ion channels in response to depo-
Katz and Schmitt in 1940, who showed nonsynaptic larizing and hyperpolarizing stimuli. The arrangement
electrical interaction between adjacent nerve fibers.20 of myelin around nerves, with myelin free segments at
In their work, two large parallel nonmyelinated axons the node of Ranvier and the electrophysiologic char-
were isolated from the crab limb nerve. They demon- acteristics of ion channel distribution at the nodal and
strated that: (1) the passage of an impulse in one fiber internodal segments, facilitates the speed of impulse
causes subthreshold excitability changes in the adjacent conduction. Traditionally, nerve conduction studies
fiber and (2) when impulses are set up simultaneously that measure impulse conduction velocity, latency, am-
along both fibers, a mutual interaction occurs that can plitude, and F-waves have been used clinically to as-
lead to speeding up or slowing down of the impulses sess the gross status of peripheral nerves. More recently,
and also possibly to synchronization between the two axonal excitability study has emerged as a noninvasive
impulses, depending on the initial phase relationship. technique that offers complementary information to
In 1984, ephaptic interactions were suggested to play a that provided by conventional nerve conduction stud-
role in hemifacial spasm pathophysiology by causing ies, to gain more insight into axonal function with de-
“cross-talk” between facial nerve fibers.21 tailed information about the axonal membrane potential
and ion channel properties.25 The key axonal excit-
NUCLEAR HYPEREXCITABILITY ability parameters commonly measured after delivery
Enhanced excitability of the facial nucleus has also of impulse to a test nerve include threshold, strength-
been suggested as a possible mechanism for synkine- duration time constant, rheobase, t­hreshold electroto-
sis following facial nerve injury. This is supported by nus, recovery cycle, and current/­ threshold relation-
the predictable and nonrandom pattern of synkinesis ship. Changes in these axonal biophysical parameters
and the clinical observation of synkinesis early in the have been attributed to aberrant ionic conductance,
16 CHAPTER 2 Etiology, Epidemiology, and Pathophysiology of Post-Facial Paralysis Synkinesis

particularly pertaining to Na+ and K+ exchange, and the known ability of the brain to undergo plasticity, the
has been characterized for a diverse range of conditions role of cortical changes following facial nerve injury
including toxic, metabolic, both acquired and inherited as a potential cause of defective motor control is be-
demyelinating neuropathies, and neurodegenerative ing investigated. The development of sophisticated
disorders such as amyotrophic lateral sclerosis (ALS), noninvasive neuroimaging techniques over the past
as well as providing insight into pathophysiologic decade has made it possible to study cortical changes
changes occurring at the peripheral nerve level in dis- in response to peripheral nerve injury. In a study of pa-
orders of the central nervous system such as stroke, spi- tients with facial synkinesis, functional magnetic reso-
nal cord injury, and multiple sclerosis.26,27 Using axonal nance imaging (fMRI) demonstrated that the distance
excitability techniques, recent studies have explored the between motor cortical subsites activated on ipsilateral
possible contribution of electrophysiologic changes in blinking and smiling tasks was decreased on the affect-
peripheral facial nerves following injury that may pre- ed half of the face compared with the unaffected side.30
dispose to ectopic activity such as spasms and synki- In another study investigating resting state fMRI pa-
nesis. In one study, facial nerve excitability parameters rameters in post-facial paralysis patients, there was an
were compared between patients with synkinesis and abnormal state of hypercompensation in motor control
those without following facial paralysis.28 Synkinesis areas in patients with compared with patients without
patients demonstrated marked alterations in a number facial synkinesis.31,32 Together, these studies support
of excitability parameters compared with normal con- the role of a functionally aberrant motor control sys-
trols. Specifically, there was depolarized resting mem- tem in facial synkinesis. Facial retraining therapy with
brane potential in the synkinesis group predisposing to biofeedback is an integral aspect of the management
axonal hyperexcitability hyperkinesis and myokymia. of facial synkinesis. Recent reports indicate that people
As in ALS, the alteration in resting membrane poten- are able to develop control over the activity of specific
tial of injured facial nerves was attributed to changes in brain areas when provided with real time–fMRI bio-
the distribution of Na+ channels in the injured nerves. feedback.33,34 The use of real time–fMRI biofeedback
Acute Na+ channel dysfunction has been directly im- in the treatment of facial synkinesis is an interesting
plicated in the pathophysiology of virus-induced facial research tool that may hold therapeutic potential.
palsy and has been linked to the hyperexcitability ob-
served in postinjury facial nerve.26,27 In one study, the
SUMMARY
neurotropic virus implicated in Bell’s palsy, herpes sim-
plex type 1 (HSV-1), was found to inhibit the excitabil- Faulty facial nerve regeneration manifesting as syn-
ity of the facial nerve by an elective, precipitous, and kinesis is a challenging sequelae of facial nerve injury
complete internalization of voltage-activated sodium for both clinicians and patients. The varied patho-
channel proteins from the plasma membrane of the physiologic mechanisms of synkinesis, encompassing
neurons.29 This change in the transmembrane sodium misdirected axons, ephaptic coupling, synaptic strip-
channel was RNA-mediated and occurred directly at ping with neuronal hyperexcitability, axonal excit-
the axonal site of virus transfection. This ability of an ability from altered ion channel function, and cortical
axon to modify protein transcription at the site of the vi- adaptation, underscores the difficulty in establishing a
ral exposure or injury has emerged as a key mechanism universally effective preventative and therapeutic in-
in the response of peripheral nerves to injury. Axonal ex- tervention. However, improved understanding of the
citability studies in Bell’s palsy patients shows a pattern electrophysiologic alterations that accompany periph-
that lends further support to the role of Na+ channels in eral nerve injury and new clinical tools available for
the sequelae of facial nerve injury.28 This evolving un- characterizing the excitability parameters of individual
derstanding of the pathophysiology of peripheral facial nerve branches offer a selective and targeted rebalanc-
nerve injury offers opportunities for preventative and ing of the disorganized facial neuromuscular circuit
therapeutic intervention. For example, medications that using cortical retraining therapy, effective pharmaco-
stabilize membrane potential through their effect on therapeutics, selective myectomy, and neurectomy.
Na+ channels may be considered early in the recovery
of idiopathic facial nerve paralysis even before synkine- REFERENCES
sis becomes clinically evident. Additionally, excitability 1. Gothard KM. The amygdala-motor pathways and the con-
parameters may be used to select appropriate nerves trol of facial expressions. Front Neurosci. 2014;8:43.
for reanimation procedures or selective neurectomy. 2. Boahene KDO, May M. The facial nerve after injury. In: Hom
DB, Hebda P, Friedman C, Gosain A, eds. Essential Tissue
CORTICAL ADAPTATION Healing of the Face and Neck. Shelton, CT: PMPH; 2006.
3. Yamamoto E, Nishimura H, Hirono Y. Occurrence of seque-
The fine voluntary control of facial muscles begins in lae in Bell’s palsy. Acta Otolaryngol Suppl. 1988;446:93–96.
the motor cortex. The previously described mecha- 4. Kimura J, Rodnitzky RL, Okawara SH. Electrophysiologic
nisms for synkinesis all pertain to the facial nerve from analysis of aberrant regeneration after facial nerve paralysis.
the nucleus in the pons to the extracranial nerve. Given Neurology. 1975;25(10):989–993.
Etiology, Epidemiology, and Pathophysiology of Post-Facial Paralysis Synkinesis CHAPTER 2 17

5. Peitersen E. Bell’s palsy: the spontaneous course of 2,500 20. Katz B, Schmitt OH. Electric interaction between two adja-
peripheral facial nerve palsies of different etiologies. Acta cent nerve fibres. J Physiol. 1940;97(4):471–488.
Otolaryngol. 2002;(549):4–30. 21. Nielsen VK. Pathophysiology of hemifacial spasm: I.
6. Devriese PP, Moesker WH. The natural history of fa- Ephaptic transmission and ectopic excitation. Neurology.
cial paralysis in herpes zoster. Clin Otolaryngol Allied Sci. 1984;34(4):418–426.
1988;13(4):289–298. 22. Blinzinger K, Kreutzberg G. Displacement of synaptic termi-
7. Nakano H, Haginomori SI, Wada SI, Ayani Y, Kawata nals from regenerating motoneurons by microglial cells. Z
R, Saura R. Electroneurography value as an indicator of Zellforsch Mikrosk Anat. 1968;85:145–157.
high risk for the development of moderate-to-severe syn- 23. Bratzlavsky M, van der Eecken H. Altered synaptic or-
kinesis after Bell’s palsy and Ramsay Hunt syndrome. Acta ganization in facial nucleus following facial nerve regen-
Otolaryngol. 2019;139(9):823–827. eration: an electrophysiological study in man. Ann Neurol.
8. Morecraft RJ, Louie JL, Herrick JL, Stilwell-Morecraft KS. 1977;2(1):71–73.
Cortical innervation of the facial nucleus in the non-human 24. Graeber MB, Bise K, Mehraein P. Synaptic stripping in the
primate: a new interpretation of the effects of stroke and re- human facial nucleus. Acta Neuropathol. 1993;86:179.
lated subtotal brain trauma on the muscles of facial expres- 25. Krishnan AV, Lin CS, Park SB, Kiernan MC. Axonal ion
sion. Brain. 2001;124(Pt 1):176–208. channels from bench to bedside: a translational neuroscience
9. Horta-Júnior JA, Tamega OJ, Cruz-Rizzolo RJ. Cytoarchitecture perspective. Prog Neurobiol. 2009;89(3):288–313.
and musculotopic organization of the facial motor nucleus in 26. Huynh W, Kiernan MC. Peripheral nerve axonal excitability
Cebus apella monkey. J Anat. 2004;204(Pt 3):175–190. studies: expanding the neurophysiologist’s armamentari-
10. Choi D, Raisman G. Somatotopic organization of the facial um. Cerebellum Ataxias. 2015;2:4.
nucleus is disrupted after lesioning and regeneration of the 27. Persson AK, Kim I, Zhao P, Estacion M, Black JA, Waxman
facial nerve: the histological representation of synkinesis. SG. Sodium channels contribute to degeneration of dor-
Neurosurgery. 2002;50(2):355–363. sal root ganglion neurites induced by mitochondrial dys-
11. Jessen KR, Mirsky R. The success and failure of the Schwann function in an in vitro model of axonal injury. J Neurosci.
cell response to nerve injury. Front Cell Neurosci. 2019;13:33. 2013;33:19250–19261.
12. Allodi I, Udina E, Navarro X. Specificity of peripheral nerve 28. Eviston TJ. Mechanisms of Axonal Dysfunction in Facial Nerve
regeneration: interactions at the axon level. Prog Neurobiol. Disorders [Ph.D. Thesis]. University of New South Wales;
2012;98:16–37. 2016.
13. Blesch A, Lu P, Tsukada S, et al. Conditioning lesions before 29. Storey N, Latchman D, Bevan S. Selective internalization of
or after spinal cord injury recruit broad genetic mechanisms sodium channels in rat dorsal root ganglion neurons infected
that sustain axonal regeneration: superiority to cAMP-medi- with herpes simplex virus-1. J Cell Biol. 2002;158:1251–1262.
ated effects. Exp Neurol. 2012;235:162–173. 30. Wang Y, Yang L, Wang W, Ding W, Liu H. Decreased dis-
14. Fu SY, Gordon T. The cellular and molecular basis of periph- tance between representation sites of distinct facial move-
eral nerve regeneration. Mol Neurobiol. 1997;14:67–116. ments in facial synkinesis—a task fMRI study. Neuroscience.
15. Doron-Mandel E, Fainzilber M, Terenzio M. Growth con- 2019;397:12–17.
trol mechanisms in neuronal regeneration. FEBS Lett. 31. Wu JJ, Lu YC, Zheng MX, et al. Motor control deficits in fa-
2015;589:1669–1677. cial synkinesis patients: neuroimaging evidences of cerebral
16. Li H, Wigley C, Hall SM. Chronically denervated rat cortex involvement. Neural Plast. 2019;2019:7235808.
Schwann cells respond to GGF in vitro. Glia. 1998;24:290–303. 32. Wang Y, Wang WW, Hua XY, Liu HQ, Ding W. Patterns of
17. Jonsson S, Wiberg R, McGrath AM, et al. Effect of delayed cortical reorganization in facial synkinesis: a task function-
peripheral nerve repair on nerve regeneration, Schwann cell al magnetic resonance imaging study. Neural Regeneration
function and target muscle recovery. PloS One. 2013;8:e56484. Research. 2018;13(9):1637–1642.
https://doi.org/10.1371/journal.pone.0056484. 33. de Charms RC, Christoff K, Glover GH. Learned regulation
18. Choi D, Raisman G. After facial nerve damage, regenerating of spatially localized brain activation using real-time fMRI.
axons become aberrant throughout the length of the nerve Neuroimage. 2004;21:436–443.
and not only at the site of the lesion: an experimental study. 34. Rota G, Sitaram R, Veit R. Self-regulation of regional corti-
Br J Neurosurg. 2004;18(1):45–48. cal activity using real-time fMRI: the right inferior fron-
19. Arvanitaki A. Effects evoked in an axon by the activity of a tal gyrus and linguistic processing. Hum Brain Mapp.
contiguous one. J Neurophysiol. 1942;5(2):89–108. 2009;30:1605–1614.
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Psychosocial Impact of Facial Palsy 3
Ietske J. Siemann, Carien H.G. Beurskens

According to several studies, facial palsy is signifi-


INTRODUCTION
cantly correlated with not only reduced quality of life
“Societal interest in appearance has a long history,” but also significant rates of depression and psychologi-
and today this seems more important than ever.1 cal distress.8,9 Significant correlations have also been
Social media, the importance of “the perfect picture,” found between perception of facial palsy and level of
and the desire and possibilities to change and enhance psychological distress. The authors also found that fe-
the body create more unrest and discomfort. Older male patients with facial palsy were more severely im-
studies2 show that physical attraction is an important pacted with depression. Etiology of the facial paralysis
predictor in entering relations and dating. Physical at- also appears to play a part in the degree of depressive
traction is also associated with success, happiness, and disorders. For instance, patients who develop facial
better job prospects.3 paralysis secondary to a tumor are simply glad to be
Rojas et al. believed that the face plays a crucial alive and often accept the paralysis more readily.
role in social interaction.4 People with facial palsy feel Coulson et al. also showed that 50% of facial palsy
that their face is no longer “right” due to asymmetry patients are unable to accurately express one or sev-
and negative physical side effects such as incomplete eral emotions (happiness, anger, fear, sadness, disgust,
eye closure, limited mouth movement, and synkine- surprise).11 The partners of these patients also consid-
sis. Additionally, facial palsy is mentally burdening; ered their expressions more difficult to read. There
patients often indicate they prefer not to look in the were also some significant correlations between the
mirror because their face startles them. Moreover, they duration and the timeline and emotional representa-
find it difficult when other people look at them and tion subscales of the Illness Perception Questionnaire-
they cannot express their emotions. Because of the Revised (IPQ-R), as well as the Hospital Anxiety and
latter, emotions can often be misunderstood, result- Depression Scale.12,13 This indicated that the dura-
ing in miscommunication. For example, patients want tion of the facial palsy affected participants’ levels
to show their happiness, but due to asymmetric and of distress.9 In a sample of patients with facial palsy,
paradoxical facial movements they may seem sad or Bradbury et al. also found distress levels of 11.4% for
angry. depression and 35% for fear.14
It is of great importance that clinicians take into ac-
count the impact of facial palsy. Research has been per-
formed on facial palsy patients concerning self-percep-
PSYCHOSOCIAL CONSEQUENCES OF FACIAL PALSY
tion, difficulties with social encounters, and depressive The face is where all social interactions begin. Within a
disorders.5–10 Although facial palsy has been recog- fraction of a second assumptions are made concerning
nized to influence patient-perceived quality of life, the age, gender, status, health, mood, and intentions—all
psychosocial disability may not be related to the sever- by looking at a person’s face for just nanoseconds. In
ity of the condition.5 In 2000, evidence was found of a general, this is an efficient way of processing informa-
large individual variation in adaptation to facial palsy. tion, as we learn to “read” complex social stimuli from
At the same time, a strong relation between the degree a very early age.
and severity of the limitations and levels of stress was Facial palsy disturbs this process and changes the
not found.6 Recently, the literature has shown that even position of both the patient and the conversational
a condition we qualify as minor may have severe psy- partner in the interaction. In the literature, it is men-
chosocial consequences, and that a patient with severe tioned that damage to the face may also cause damage
facial palsy or disfiguring synkinesis may not neces- to “the self.”15 Various psychological mechanisms are
sarily experience any problems.5 Health care providers triggered, and patients and their partners in conversa-
need to be aware of the fact that patients are distressed tion need to look for a new balance.
but that it may not correlate with the severity of the As described earlier, the responses of patients vary
palsy. Patients with low self-esteem, younger patients, widely. Two factors play an important role: circum-
and women suffer more from distress.7 stances that caused the facial palsy and the existing
19
20 CHAPTER 3 Psychosocial Impact of Facial Palsy

Social isolation Patients indicate they are preoccupied with their own
feelings and their facial palsy. They find it difficult to
Worrying
Reduced show an interest in other people and are mainly trying
confidence to “survive”.
Problems with Reactions of
relationships others
Feelings of Uselessness

Reduced “I can understand my boss is reconsidering my contract. I


Depression mean, just look at my face... I am not exactly an ambassa-
quality of life
dor for the company, am I?”
  
Social fear Shame
Patients often feel useless because they feel they can no
Stigmatization
longer function as a full member of society.
Fig. 3.1 Impact of New Appearance.
Despair
personality traits such as self-esteem and intrinsic self-
worth.7 Factors surrounding the facial palsy such as “And then this doctor just says: you’ll have to learn to live
the etiology, age, gender, the manner in which the situ- with it. Does he have any idea what he’s saying? Does he
ation has been explained to them, and social support even know what he’s talking about?”
  
also have significant influence in outcome. The over-
all impact of a new appearance therefore is extremely The despair of patients can not only impact their cur-
diverse and the most important consequences are de- rent situation but also future prospects. They will
pression and anxiety (Fig. 3.1). doubt if they will ever see an improvement or if they
will ever be able to get better.
DEPRESSION
The most common psychosocial response to facial pal- Concentration Problems
sy is depression. At the onset, feelings and complaints
of depression may not be pathological per se. They can “I notice that since I have this condition I cannot concen-
be seen as a normal way of mourning the loss of some- trate anymore. I used to read books, but nowadays I can
thing the patient held dear—a “normal” face. hardly manage.”
  
If the depressive response is out of proportion and
continues for a very long time, it can become patholog- As a result of the events surrounding the facial palsy
ical. Sometimes, it may be the case that the patient was and the consequences for the patient, the patient may
familiar with feelings of depression before the facial be fully preoccupied with the new situation, and con-
palsy occurred but managed to cope with them. Facial centrating on other matters may be difficult. One might
palsy then becomes an amplifier for something that say that the brain is completely preoccupied with the
had been there all along. The most obvious manifesta- condition, and there is no room for other thoughts and
tions of depression are: worrying, loss of interest, feel- feelings.
ing useless, being unable to make decisions, despair,
and concentration problems. Dissatisfaction
Dissatisfaction can be characteristic of a patient with
Worrying feelings of depression. Patients may be dissatisfied
with the treatment, progress, and doctor’s statements.
“I hardly recognize myself. Before, I never worried about They may demand certain interventions that have not
anything, but now I will think about everything that might go been recommended, such as surgical procedures, and
wrong. I am no longer spontaneous; I worry about so many they may decide to go and see other physicians hoping
things now.”
  
that they will give them want they want.

Patients indicate they worry a lot more than before ANXIETY


and they think about what other people might think In addition to depression there may also be feel-
of them. They may also worry excessively about their ings of anxiety. It must be stated that anxiety is a
situation at school or work. perfectly normal reaction in a threatening situation.
Anxiety is episodic and can be related to certain
Loss of Interest situations. Anxiety, however, can become restric-
tive if it is considerable and protracted. If patients
“When someone talks to me about silly things like shop- develop a chronic anxiety of rejection and choose
ping or some television program, I may think to myself: yeah exclusion or isolation for fear, the fear may become
right, big deal... I am extremely self-absorbed now.”
   pathological.
Psychosocial Impact of Facial Palsy CHAPTER 3 21

“I must always look immaculate,


Beliefs I don’t look like other people,
I’m deformed, to be successful
I must be beautiful……..”

Physiology Behaviors

Heart rates, palpitations, Mirror gazing, social avoidance,


rapid breathing, faintness, avoid intimacy; safety behaviors:
sweaty, blushing big jackets, makeup, excessive
Feelings grooming, asking for reassurance

Anxious, lonely, despairing, unhappy,


disgusted, ashamed, angry
Fig. 3.2 Cognitive Behavioral Model.

Reactions of Others what they are going to say, and how they can prepare
themselves for all this. This is very exhausting and
“I am always extremely alert: I notice people watching me leads to patients being wary of social contacts in gener-
and wondering what could be wrong with me. I’m just very al. Overall, depression and anxiety can have important
sensitive about that...”
   consequences and can have a major impact on patients
with a facial palsy.
Patients fear facing social situations and may worry
about the possible reactions of other people. They may OTHER PSYCHOSOCIAL CONSEQUENCES
also be afraid to engage in conversation, speak clearly, or At a certain moment in time, patients will be told
spray spittle when talking with someone. This may cause that they are beyond recovery and that they will no
them to be extra alert when encountering a new situa- longer be treated surgically or otherwise and the re-
tion, which in turn means they will be even more wary habilitation process has come to an end. This is the
and sensitive about other people’s looks and remarks. moment patients need to fend for themselves and
think of ways to arrange their own life with the palsy.
Social Fear/Social Isolation
“When I understood that we had run out of options for more
“I always find new situations scary. People will look at me surgery, I arranged for a meeting with my company doctor.
and I am convinced they’re thinking: look at that droopy He literally said: Maybe now it is time for you to work on how
face. I prefer to arrive a bit later and I make sure I sit or stand all of this is affecting you.”
  
at the back.”
  
The consequences of the aforementioned may be
Patients may decide to avoid social situations and re- significant. Patients may avoid social contact or mini-
frain from making contact. The tension that precedes mize their social environment. Studies show that qual-
an encounter may be too much, the fear that the other ity of life is reduced for patients with facial palsy.16
person may say something—or nothing at all—may Fig. 3.2 summarizes the aforementioned consequences
be too big. They may become reserved and withdraw within a cognitive behavioral model.
from everyday life.
COPING MECHANISMS OF FACIAL PALSY
Shame
It is important to understand the various ways that in-
“I have never been very self-assured but that is even worse dividuals deal with the psychosocial impact of facial
now. I am simply very much ashamed of my face.”
   palsy. Each person copes in their own unique manner,
depending on the strategies they employ.
Patients are ashamed of their appearance and extreme- Coping mechanisms refer to each person’s mecha-
ly self-conscious when they walk into a room. People nisms to deal with stress or problems. These are con-
with facial palsy indicate they can no longer be them- scious efforts to regulate cognition, behavior, and
selves: they have lost all spontaneity. Spontaneously emotions as a result of stressful situations. Coping
engaging in an unknown situation is a thing of the past; mechanisms strongly depend on personality and dif-
they need to think about how they may be perceived, fer according to circumstances and age.17
22 CHAPTER 3 Psychosocial Impact of Facial Palsy

• B eginning with mime therapy or neuromuscular


The patient indicated she feels uncomfortable when people
watch her but do not say anything. However, when people do retraining (NMR) may be a good starting point for
say something, she also feels awkward. Therefore, in her treat- dealing with functional problems.19,20 Positive feel-
ment attention has been paid to: what do you like in certain ing is established when patients realize that there
situations, what can you say, and what would you want to say?
   is some improvement with appropriate exercises.
Attention can also be paid to hair, make-up, colorful
Patients try to make an initial assessment when their clothing, jewelry, and the use of nonverbal expres-
face is paralyzed and will immediately think about sions that divert attention away from the face.
the consequences of the condition for their wellbeing. • Behavioral therapy such as cognitive behavioral
They have a need for objective information from a phy- therapy or acceptance and commitment therapy
sician as well as subjective information from friends (ACT) should be considered. Learning to cope with
and family in order to compare themselves to others.18 thoughts and feelings concerning facial palsy may
It is not until later that patients will try to assess inter- improve a patient’s confidence.21 Cognitive behav-
nal and external resources in order to determine what ioral therapy assumes that it is not the events that
they will need to expect from themselves and others. cause a person to have negative feelings and to
They will adapt their behavior to the situation and de- show a certain behavioral pattern, but rather the
termine what might be helpful to help them adjust to biased view with which they see things. If a per-
their facial palsy. son is taught to interpret these negative thoughts
Two general coping reactions are classified: emotion- differently, they will have a more objective take on
oriented and problem-oriented. The emotion-oriented their own feelings and perceptions. This may cause
manner of coping with stress relies on the individual the negative feelings to disappear and will change
to transform or regulate their emotions with respect to behavior. ACT may also be a useful approach as
the problem. The problem-oriented manner of coping patients learn to end their struggles with unpleas-
with stress focuses on searching for potential causes of ant thoughts, emotions, and physical sensations.
the problem in order to transform the problem. Which This leads to them being able to focus their atten-
coping mechanism a patient chooses depends on their tion on the issues that really matter to them in life
past experiences and personality. Obviously, a combi- (values).
nation of the two is also possible. • Interacting with other individuals who have a simi-
This certainly applies to patients with facial palsy. lar condition can be a powerful aid for patients with
Some of the patients concentrate on coping with the facial palsy. Patients often indicate that they feel
situation and on self-examination, whereas others isolated, partly because they do not know anyone
actively investigate possible treatments. Some will with a similar condition. Enabling contact among
do both. Together with the psychologist, the situa- patients who have facial palsy can have a very posi-
tion can be examined to determine the patient needs tive effect as they can now identify with each other.
and appropriate course of action, as well as patient • Patients often feel that other people stare at them. A
response. psychologist can explain that looking another per-
In most cases patients are advised to first attend to son in the eye (i.e., looking at their face) is the start-
the physical function such as facial asymmetry and ing point of any interaction.
synkinesis and then concentrate on the cosmetic as- • Discuss with the patients what they want and make
pects, such as feelings of imperfection. them aware of their preferred strategy: do they or
do they not want to mention the facial palsy when
interacting with others? If patients indicate they
TREATMENT CONSIDERATIONS
would like to say something about their appear-
The manner in which patients cope with facial palsy ance, practice sentences like “I can see you’re look-
differs significantly between individuals and there- ing, I do have a changed facial condition but let’s
fore may result in varying outcomes. First of all, the talk about how you are doing.” They should say
principle of “one size fits all” certainly does not apply. these sentences out loud and practice them.
Health care professionals and the patient will need to • For their own self-confidence, the patient should be
work together to determine the nature of the issue that encouraged to look their best (clothes, make-up, hair).
needs to be addressed. • Utilize other body parts such arms, hands, and
When the psychosocial consequences of facial palsy shoulders more frequently to divert attention from
are discussed, several subjects may be addressed: the face (Video 3.1).
• Psychoeducation: patients have a need for more in- • If the patient wears glasses, they may like to choose
formation about their condition, prospects, possible a dark frame or a colorful frame to distract attention
treatments, and psychological impact. Explaining from the facial palsy.
what it means to have facial palsy may indeed help • Mime therapy or NMR can be utilized to reduce
clarify emotional reactions and offer a context for the stiffness of the face so that patients will be less
the patient. aware of the fact that their face consists of two
Psychosocial Impact of Facial Palsy CHAPTER 3 23

separate halves. If patients do not feel their face, it 8. Nellis JC, Ishii M, Byrne PJ, Boahene KDO, Dey JK, Ishii LE.
is less present and as a consequence the facial palsy Association among facial paralysis, depression, and quality
of life in facial plastic surgery patients. JAMA Facial Plast
will also be less present. Surg. 2017;19(3):190–196.
• 
It may be useful to visualize stressful situations 9. Fu L, Bundy C, Sadiq SA. Psychological distress in people with
(social interaction, unknown people) in advance. disfigurement from facial palsy. Eye. 2011;25(10):1322–1326.
Patients can think of what may happen and think of 10. Hotton M, Huggons E, Hamlet C, et al. The psychosocial im-
how they will respond to possible looks or remarks. pact of facial palsy: A systematic review. Br J Health Psychol.
2020;25(3):695–727.
11. Coulson SE, O’Dwyer NJ, Adams RD, Croxson GR.
CONCLUSION Expression of emotion and quality of life after facial nerve
paralysis. Otol Neurotol. 2004;25(6):1014–1019.
Facial palsy is not only a physical condition, but also 12. Zigmond AS, Snaith RP. The hospital anxiety and depres-
a damage to “the self.” The most common psychoso- sion scale. Acta Psychiatr Scand. 1983;67(6):361–370.
13. Leventhal H, Benyamini Y, Brownlee S, et al. Illness repre-
cial response to facial palsy is depression. In addition sentations: theoretical foundations. In: Petrie KJ, Weinman
to depression there may also be feelings of anxiety. J, eds. Perceptions of Health and Illness. Amsterdam,
Various psychological mechanisms are triggered, and Netherlands: Harwood Academic Publisher; 1997:19–46.
patients need to look for a new balance. A multidis- 14. Bradbury ET, Simons W, Sanders R. Psychological and so-
ciplinary approach for patients with a facial palsy is cial factors in reconstructive surgery for hemi-facial palsy. J
Plastic Reconstr Aesth Surg. 2006;59(3):272–278.
important, whereby a psychologist can make a contri- 15. Dobel C, Miltner WH, Witte OW, Volk GF, Guntinas-Lichius
bution concerning psychosocial problems. O. Emotional impact of facial palsy. Laryngo-Rhino-Otol.
2013;92(1):9–23.
REFERENCES 16. Luijmes RE, Pouwels S, Beurskens CHG, Kleiss IJ, Siemann
I, Ingels KJAO. Quality of life before and after different treat-
1. Rumsey N. The psychology of appearance: why health ment modalities in peripheral facial palsy: a systematic re-
psychologists should “do looks.” Eur Health Psychol. view. Laryngoscope. 2017;127:1044–1051.
2008;10(3):46–50. 17. Lazarus RS, Folkman S. Stress, Appraisal and Coping. New
2. Montepare JM, Dobish H. The contribution of emotion per- York: Springer; 1984.
ceptions and their overgeneralizations to trait impressions. J 18. van den Borne B, Molenaar S. Informing patients about facial
Nonverbal Behavior. 2003;27:237–254. nerve paresis and treatment. In: Beurskens CHG, van Gelder
3. Wolffhechel K, Fagertun J, Jacobsen UP, et al. Interpretation RS, Heymans PG, Manni JJ, Nicolai J-PA, eds. The Facial
of appearance: the effect of facial features on first impres- Palsies, Complementary Approaches. Utrecht, Netherlands:
sions and personality. PloS One. 2014;9(9). https://doi. Lemma Publishers; 2005:404–416.
org/10.1371/journal.pone.0107721. 19. Beurskens CHG, Heymans PG. Mime therapy improves
4. Rojas H, Shah DV, Friedland LA. A communicative ap- facial symmetry in people with long-term facial nerve
proach to social capital. J Communication. 2011;61:689–712. paresis a randomised controlled trial. Austr J Phys Ther.
5. van Veen MM, Tavares-Brito J, van Veen BM, et al. Association 2006;52:177–183.
of regional facial dysfunction with facial palsy-related qual- 20. Diels HJ, Beurskens CHG. Neuromuscular retraining: non-
ity of life. JAMA Facial Plast Surg. 2019;21(1):32–37. https:// surgical therapy for facial palsy. In: Slattery WH, Azizzadeh
doi.org/10.1001/jamafacial.2018.0804. B, eds. The Facial Nerve. New York: Thieme Medical
6. Walker DT, Hallam MJ, Ni Mhurchadha S, McCabe P, Nduka Publishers; 2014:204–212.
C. The psychosocial impact of facial palsy: our experience 21. Hayes SC, Luoma JB, Bond FW, Masuda A, Lillis J.
in one hundred and twenty six patients. Clin Otolaryngol. Acceptance and commitment therapy: model, processes and
2012;37(6):474–477. outcomes. Behav Res Ther. 2006;44(1):1–25.
7. Cross T, Sheard CE, Garrud P, Nikolopoulos TP, O’Donoghue
GM. Impact of facial paralysis on patients with acoustic neu-
roma. Laryngoscope. 2000;110:1539–1542.
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Facial Nerve Consultation 4
Stephanie Warrington, Sara MacDowell, Laura Hetzler

INTRODUCTION past imaging are necessary and may direct further


Facial paralysis and its sequela is a complex medical workup. In the setting of tumor extirpation or onco-
issue that can be both physically and emotionally dis- logic causes of facial paralysis, radiation therapy may
tressing. Patients may present to us at any time along have been performed or may be planned in the future.
the continuum of their recovery or lack of recovery. As each patient has a unique perception of their fa-
In addition to the medical aspects of treatment, psy- cial deficit, it is imperative to ask each patient to ar-
chosocial impacts are common and must also be ad- ticulate what is most bothersome to them, as this may
dressed.1–6 Here we discuss our approach to the evalu- not always correlate to the most notable deformity to
ation and early management of patients with facial the clinician. Patient-driven care can be appropriate,
nerve dysfunction. especially in the partially recovered facial paresis or
synkinetic patient.
HISTORY THE ROLE OF PATIENT-REPORTED OUTCOME
Upon initial evaluation for facial nerve dysfunction, MEASURES
there are a few key components of the history that Facial nerve dysfunction can take a great emotional
are invaluable in diagnosing and ultimately guiding toll on patients, causing them to withdraw from social
management. First, a timeline of the onset of facial pa- interactions. The psychosocial impact of the individu-
ralysis is essential. It is important to understand if the al’s facial paralysis must be directly explored during
paralysis occurred immediately or over the course of a the initial evaluation. Patients often have a negative
few days or months. This needs to be followed up with self-image and low self-esteem. To the observers, these
further questioning about the severity of the weakness, patients can be thought of as displaying a negative
to include partial or complete paralysis. If some time affect, which may lead to social isolation. Quality of
has passed since the onset of facial paralysis, a timeline life studies have shown that facial paralysis is signifi-
of recovery is also needed. Certain etiologies, such as cantly associated with depression.1–6 This profound
Bell’s palsy, are supported by recovery of facial func- emotional component must be part of the assessment
tion within 2 to 3 months. Failure to recover function of patients with facial nerve dysfunction. Some of the
within that period may indicate a different etiology patient-reported outcome measures (PROMs), such as
and further workup with imaging and nerve conduc- the Facial Disability Index (FDI) and Facial Clinimetric
tion studies may need to be initiated.7 Evaluation (FaCE), are very useful in aiding the cli-
The health care provider should also ask specific nician in capturing additional and useful informa-
questions that might indicate more clearly the etiology tion about the patient’s quality of life (Fig. 4.1).2,4,6,9,11
of the dysfunction. Common causes are inflammatory Furthermore, PROMs can be useful in research out-
post viral, neoplasms, trauma, congenital, and infec- come studies to evaluate the efficacy of our treatment
tion including otologic pathology.3,7–10 Questions for methods. It is useful to have resources such as online
diagnostic purposes can include personal history of or in-person support groups and counseling to offer
past facial paralysis, family history, presence of vesicles to facial nerve patients in addition to traditional treat-
or rashes, travel history, or recent surgery or trauma. ment modalities.5
The practitioner should also note other symptoms tem-
porally associated with onset of facial paralysis such as
PHYSICAL EXAMINATION
hearing loss, dizziness, facial pain, facial twitching, or
facial swelling. Once a thorough patient history is elicited, a detailed
The next part of the history should discern if the pa- physical examination assessing the function of the fa-
tient has received any prior treatments for their paraly- cial nerve must be completed. Traditionally, the House-
sis such as steroids or antivirals. Some patients may Brackmann scale has been an objective way to measure
have participated in facial exercises or electrical stimu- facial dysfunction. However, this scale is more of a broad
lation to aid in their recovery.1,6,10 Questions regarding overview of facial nerve function without assessing
25
26 CHAPTER 4 Facial Nerve Consultation

Facial Disability Index (FDI)


Please circle the most appropriate response to the following questions related to
problems associated with the function of your facial muscles. For each question,
consider your function during the past month.
Physical function:
1. How much difficulty did you have keeping food in your mouth, moving food around in
your mouth, or getting food stuck in your cheek while eating?

2. How much difficulty did you have drinking from a cup?

3. How much difficulty did you have saying specific sounds while speaking?

4. How much difficulty did you have with your eye tearing excessively or becoming dry?

5. How much difficulty did you have with brushing your teeth or rinsing your mouth?

(5) Usually did with no difficulty


(4) Usually did with a little difficulty
(3) Usually did with some difficulty
(2) Usually did with much difficulty
(1) Usually did not do because of health
(0) Usually did not do because of other reasons

Social function:
6. How much of the time have you felt calm and peaceful?

7. How much of the time did you isolate yourself from people around you?

8. How much of the time did you get irritable toward those around you?

9. How often did you wake up early or wake up several times during your nighttime sleep?

10. How often has your facial function kept you from going out to eat, shop, or participate
in family or social activities?

(6) None of the time


(5) A little bit of the time
(4) Some of the time
(3) A good bit of the time
(2) Most of the time
(1) All of the time

Fig. 4.1 Facial Disability Index (FDI) and Facial Clinimetric Evaluation (FaCE).

specific affected facial regions.4,6,8,11,12 A more detailed Another important aspect of assessing facial
characterization of the defect is warranted. It is good dysfunction is facial tone. Abnormal facial tone is
practice to divide the face into five overall sections: fore- evident to the casual observer, even when the pa-
head, periocular, midface, perioral, and cervical. From tient is at rest, and therefore can have a profound
there, documenting the deficits associated with each divi- impact on the patient’s ability to participate in the
sion is ideal (Table 4.1). Once there is a more precise char- community. Facial tone may be either hypertonic or
acterization of deficits and symptoms, this will aid in the hypotonic, both of which may exist in the same in-
decision-making process for rehabilitation and treatment, dividual (Fig. 4.2) (Video 4.1). This distinction is im-
as well as allowing for more precise assessment of treat- portant as there are different treatment approaches
ment results. for both.1,2,5,6,13
A complete head and neck examination is necessary During a patient’s recovery from facial nerve inju-
to evaluate prior incisions and deficits as well as the ry they can experience aberrant neural regeneration,
status of other cranial nerves. Injury to candidate do- which can lead to undesired facial tone and/or synki-
nor nerves for reanimation may dictate the treatment nesis. Synkinesis is uncoordinated facial motion that
options for a patient. Presence of palpable and/or vis- occurs during abnormal facial nerve recovery. The
ible extratemporal causes of facial paralysis must be patient may experience a lack of perceived motion or
ruled out. To the general practitioner, smaller parotid restricted movement that is due to poor coordination
tumors may elude a rudimentary physical examina- of muscle activation rather than paralysis. For ex-
tion. The presence or history of skin cancer can be a ample, during an attempt at normal smile, the zygo-
concerning factor in patients with initially incomplete maticus complex is restricted in its movement from
but progressive facial nerve paralysis. synkinetic smile antagonists such as depressor anguli
Facial Nerve Consultation CHAPTER 4 27

Facial Clinimetric Evaluation (FaCE) scale


You may have answered these or similar questions before. Please answer ALL QUESTIONS as best
you can. The following statements are about how you think your face is moving.
Circle only ONE number One side Both sides I have no difficulty
When I try to move my face, I find that I have difficulty on 1 2 0

If you have problems on BOTH sides, answer the questions in the remainder of the survey with regard
to the more affected side, or with regard to both sides if they are equally affected. In the past week:
Circle only ONE number on each line Not at all Only if I A little Almost Normally
concentrate normally
(1) When I smile, the affected side of
1 2 3 4 5
my mouth goes up
(2) I can raise my eyebrow on the
1 2 3 4 5
affected side
(3) When I pucker my lips, the affected
1 2 3 4 5
side of my mouth moves

The following are statements about how you might feel because of your FACE OR FACIAL PROBLEM:
Please rate how often each of the following statements applied to you during the PAST WEEK.
Circle only ONE number on each line All of the Most of Some of A little of None of
time the time the time the time the time
(4) Parts of my face feel tight, worn
1 2 3 4 5
out, or uncomfortable
(5) My affected eye feels dry, irritated,
1 2 3 4 5
or scratchy
(6) When I try to move my face, I feel
1 2 3 4 5
tension, pain or spasm
(7) I use eye drops or ointment in my
1 2 3 4 5
affected eye
(8) My affected eye is wet or has tears
1 2 3 4 5
in it
(9) I act differently around people
1 2 3 4 5
because of my face or facial problem
(10) People treat me differently
1 2 3 4 5
because of my face or facial problem
(11) I have problems moving food
1 2 3 4 5
around in my mouth
(12) I have problems with drooling or
keeping food or drink in my mouth or 1 2 3 4 5
off my chin and clothes

The following are statements about how you might have felt or been doing in the
PAST WEEK because of your FACE OR FACIAL PROBLEM. Please rate how much
you agree with each statement.
Circle only ONE number on each line Strongly Agree Don’t Disagree Strongly
agree know disagree
(13) My face feels tired or when I try to
move my face, I feel tension, pain, or 1 2 3 4 5
spasm
(14) My appearance has affected my
willingness to participate in social 1 2 3 4 5
activities or to see family or friends
(15) Because of difficulty with the way I
eat, I have avoided eating in 1 2 3 4 5
restaurants or in other people’s homes

Fig. 4.1, cont’d

oris, and platysma as well as other perioral muscles restricting any meaningful motion (Video 4.2). This
including buccinator, and depressor septi nasi. In this must be recognized prior to effectively managing
case, the patient often feels the lack of motion is relat- these patients. Ocular synkinesis is another frequent
ed to weakness but in actuality, it is the result of over- complaint in patients with aberrant facial nerve re-
active tone and contraction of antagonist muscles generation, where attempts at movement such as
28 CHAPTER 4 Facial Nerve Consultation

  The Five Divisions of the Face to Be smile or speech can lead to contraction of the orbi-
TABLE 4.1 Evaluated and the Facial Deficits in Each cularis oculi and thus narrowing the eyelid aperture
That Should Be Documented. (Fig. 4.3).1,3,4,6,10,11,13 This is in direct contrast to lag-
ophthalmos that patients experience in fully dener-
FACIAL DIVISIONS FACIAL DEFICIT
vated facial and eyelid musculature.
Forehead Tone An objective assessment of facial tone, voluntary
Synkinesis
movement, and synkinesis should be performed at
Brow elevation
Brow ptosis
the initial evaluation and can be performed using a
myriad of available grading systems. The authors
Periocular Tone
consistently use the Sunnybrook Facial Grading
Synkinesis
Visual acuity
System during initial evaluation.6,10,12–14 In addition
Corneal integrity to descriptive documentation and objective grading
Tearing systems, it is also recommended that photographic
Bell’s phenomenon and video documentation of patients be captured
Lagophthalmos at each session.13,14 Patients should be recorded in
Lower lid laxity repose, brow elevation, gentle eye closure, firm eye
Lacrimal puncta position closure, closed mouth smile, open mouth smile,
Midface Tone snarl, pucker, wide mouth opening, lower lip de-
Synkinesis pression, and nasal base view. The authors also doc-
Oral commissure excursion ument a “puffing cheeks with air” photograph (Fig.
Nasal obstruction 4.4).14 Videos can be captured of dynamic move-
Perioral Tone ments and speech. This is a useful way to monitor
Synkinesis treatment outcomes and to allow patients to ob-
Oral incompetence serve progress. A subjective questionnaire that can
Articulation errors be used to determine a patient’s perception of syn-
Cervical Tone kinesis is the Synkinesis Assessment Questionnaire
Synkinesis (SAQ) (Fig. 4.5).4,6

A B
Fig. 4.2 (A) An example of the flaccid facial tone on the right. Note dependent oral commissure position and
generalized facial ptosis as well as nasal deviation. (B) An example of hypertonic facial tone on the left. In the
hypertonic face, you can see decreased ocular aperture, deepened nasolabial crease, and platysmal banding in
repose.
Facial Nerve Consultation CHAPTER 4 29

A B
Fig. 4.3 Example of Blepharospasm Synkinesis. Right (A) and left (B) facial paralysis with synkinesis resulting in eye
closure/blepharospasm upon smiling.

RADIOLOGY AND ELECTRODIAGNOSTICS EMG can indicate reinnervation, fibrillations can indi-
Imaging studies may play an important role in the cate muscle fibers are still viable but do not have inner-
evaluation and treatment of facial palsy patients. In vation, and no activity on EMG indicates the muscle
the unrecovered patient with idiopathic facial paraly- fibers no longer have myogenic ability and cannot
sis, imaging studies such as computed tomography be reinnervated. This information can help direct the
(CT) and magnetic resonance imaging (MRI) can be available options for reanimation.7,8
useful to evaluate the presence of tumors along the fa-
cial nerve or to confirm inflammation in patient with
MANAGEMENT
presumed Bell’s palsy.7
Some patients may require surgery to help with As mentioned earlier, allowing the patient to articu-
their facial nerve recovery. For those who have facial late their perception of the deficit is key. The smile is
nerve injury due to previous surgery or trauma, a CT considered a thumbprint of an individual’s personal-
scan can be useful preoperatively to help with surgical ity and loss of this ability can be personally devastat-
planning. ing.5,11 That being said, as clinicians, we must protect
When evaluating the need for or planning for spe- from further harm such as loss of vision. Clear direc-
cific facial reanimation procedures, the type of reani- tion on how to protect the eye should be given and re-
mation procedure an individual can receive depends ferral to an ophthalmologist who can follow the status
on the time since injury, the viability of the facial nerve, of the cornea is ideal.2,3
and the status of the myogenic potential of the facial Expectation management is crucial in our recover-
muscles. Electroneuronography (ENoG) can help de- ing patients. Whether the patients have had an attempt
termine the degree of injury to the nerve. However, at surgical dynamic reanimation or the treatment plan
due to Wallerian degeneration, which takes place 3 to is to allow for natural recovery, the timeline to a new
5 days after injury, an ENoG cannot be performed un- normal may be significant. Sharing that complete and
til after that time. It is recommended to be completed perfect recovery is not likely early on, but expressing
between 3 days to 3 weeks after injury to the facial commitment to optimize their outcome will allow the
nerve. ENoG can only assess early stages of acute fa- patient to both understand the need for patience and
cial paralysis and act as a prognostic indicator in facial the limitations of nerve recovery.
nerve regeneration. Electromyography (EMG) can be The paradigm of care in facial paralysis has shifted
performed acutely and in patients with chronic facial over the past decade to a multispecialty model.11 Patients
paralysis and is typically the study of choice after 3 with facial paralysis require multiple medical special-
weeks post injury. Electric action potentials seen on ties to be involved in their care including neurologists,
30 CHAPTER 4 Facial Nerve Consultation

A B C D

E F G H

I J K
Fig. 4.4 Photographic Documentation of Patients Obtained at Each Evaluation. (A) Repose. (B) Brow elevation.
(C) Gentle eye closure. (D) Firm eye closure. (E) Closed mouth smile. (F) Open mouth smile. (G) Snarl. (H) Pucker. (I)
Wide mouth opening. (J) Lower lip depression. (K) Puffing cheeks with air.

neurotologists, neurosurgeons, oculoplastic surgeons,


CONCLUSION
and facial plastic surgeons/plastic surgeons, as well as Facial nerve dysfunction may have a complex and var-
physical or occupational therapists, nurses, psycholo- ied presentation. Management of these patients requires
gists, and other support staff.6 This allows for a mul- a comprehensive and directed facial nerve evaluation
tidisciplinary plan of care to be determined which can with an individualized work up. The advent of multi-
include: patient education, psychotherapy, soft tissue disciplinary care teams to approach facial nerve dys-
mobilization, neuromuscular retraining, chemodener- function and recovery is likely to improve quality of life
vation, and surgical facial reanimation.2,3,6,10,13 Some for those we treat.5,6,11 Importantly, allowing the patient
treatment options, such as soft tissue mobilization or to articulate their perception of facial deficit is integral to
neuromuscular retraining, will need to be reinforced at their evaluation for both expectation management and
home and therefore an understanding of the purpose overall goals. Beginning with proper assessment, sub-
and goals of treatment is essential.6 Patient education sequent evaluation and a proctored recovery can lead
and psychosocial wellness may be improved by this to improved patient understanding, expectations, and
multidisciplinary approach to treatment. quality of life in those with facial nerve dysfunction.
Facial Nerve Consultation CHAPTER 4 31

Synkinesis Assessment Questionnaire (SAQ)

Date:

Please answer the following questions regarding facial function, on a scale from 1 to 5,
according to the following scale:

1 = seldom or not at all


2 = occasionally, or very mildly
3 = sometimes, or mildly
4 = most of the time or moderately
5 = all the time or severely

Question Score
1 When I smile, my eye closes
2 When I speak, my eye closes
3 When I whistle or pucker my lips, my eye closes
4 When I smile, my neck tightens
5 When I close my eyes, my face gets tight
6 When I close my eyes, the corner of my mouth moves
7 When I close my eyes, my neck tightens
8 When I eat, my eye waters
9 When I move my face, my chin develops a dimpled area

Total synkinesis score: sum of scores 1 to 9 / 45 x 100


Fig. 4.5 The Synkinesis Assessment Questionnaire (SAQ) can be used to objectively measure the patient’s perception
of their synkinesis.

REFERENCES 8. Lee DH. Clinical efficacy of electroneurography. J Audiol


Otol. 2016;20(1):8–12.
1. Fargher KA, Coulson SE. Effectiveness of electrical stimula- 9. Lee J, Fung K, Lownie SP, Parnes LS. Assessing impair-
tion for rehabilitation of facial nerve paralysis. Phys Ther Rev. ment and disability of facial paralysis in patients with
2017;22(3–4):169–176. vestibular schwannoma. Arch Otolaryngol Head Neck Surg.
2. Henstrom DK, Lindsay RW, Cheney ML, Hadlock TA. 2007;133:56–60.
Surgical treatment of the periocular complex and improve- 10. Pourmomeny AA, Asadi S. Management of synkinesis and
ment of quality of life in patients with facial paralysis. Arch asymmetry in facial nerve palsy: a review article. Iran J
Facial Plast Surg. 2011;13(2):125–128. Otorhinolaryngol. 2014;26(77):251–256.
3. Ishii LE. Facial nerve rehabilitation. Facial Plast Surg Clin N 11. Kahn JB, Gliklich RE, Boyev KP, Stewart MG, Metson RB,
Am. 2016;24:573–575. McKenna MJ. Validation of a patient-graded instrument
4. Mehta RP, Wernick Robinson M, Hadlock TA. Validation for facial nerve paralysis: the FaCE scale. Laryngoscope.
of the synkinesis assessment questionnaire. Laryngoscope. 2001;111:387–398.
2007;117:923–926. 12. Neely JG, Cherian NG, Dickerson DB, Nedzelski JM.
5. Nellis JC, Ishii M, Byrne PJ, Boahene KD, Dey JK, Ishii LE. Sunnybrook Facial Grading System: reliability and criteria
Association among facial paralysis, depression, and quality for grading. Laryngoscope. 2010;120:1038–1045.
of life in facial plastic surgery patients. JAMA Facial Plast 13. Maria CM, Kim J. Individualized management of fa-
Surg. 2017;19(3):190–196. cial synkinesis based on facial function. Acta Otolaryngol.
6. Van Landingham SW, Diels J, Lucarelli MJ. Physical therapy 2017;137(9):1010–1015.
for facial nerve palsy: applications for the physician. Curr 14. Santosa KB, Fattah A, Gavilán J, Hadlock TA, Snyder-
Opin Ophthalmol. 2018;29(5):469–475. Warwick AK. Photographic standards for patients with facial
7. Peitersen E. Bell’s palsy: the spontaneous course of 2,500 palsy and recommendations by members of the Sir Charles
peripheral facial nerve palsies of different etiologies. Acta Bell Society. JAMA Facial Plast Surg. 2017;19(4):275–281.
Otolaryngol Suppl. 2002;549:4–30.
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rest, when the shock came. But the beam was lying against her
back, holding her down, and upon that, around it, rocks were piled.
Rawley set his teeth, carefully removed the rocks between him
and the girl, and crept closer. Hesitating, afraid, he reached out and
touched her fingers, still closed around something which she had
been holding in her hand. Her fingers were cool, pliable,—alive, he
could have sworn. So his heart, that had seemed to stop altogether,
gave a great jump.
Very gently he released the thing she was holding and drew it
toward him. His old, weather-scarred, briar pipe! He looked down at
it dumbly, looked at Nevada and very carefully laid the pipe back,
against her fingers. His eyes were very blue and bright; his face was
very pale. He steadied himself. He would get her out; he must free
her and bring her alive to the safe outside, where—
A fear stabbed him. They were going to shoot in the other dam!
He hadn’t much time, then. Another shock,—Peter had told him to
look out for a blast. It was perhaps a matter of minutes.
He raised himself, looked at the beams. They seemed to be
solidly braced, for the present, though another concussion would be
likely to throw them down. He looked down.
Nevada was sitting on a reed stool, with two cushions upon it to
give her height. He crept closer, raised himself and set a shoulder
against the beam that lay along her bowed shoulders. He steadied it
so while he took firm hold of a cushion and pulled it from beneath
her.
Nevada’s body sagged a bit. Rawley could see daylight now
between her shoulders and the beam. He waited a breath, felt no
settling of the beam, and pulled out the remaining cushion. Still the
beam held fast. Nevada, then, was not being crushed; she had been
pinned down without bearing the weight of the beam.
Rawley went back, crouching under the caved roof. His arms were
round Nevada when he stopped and picked up the pipe, slipping it
into the pocket of her blouse. Then, pulling her gently to him, he
drew her out from under the beam and into the granite-walled
storehouse. As he lifted her in his arms Nevada groaned.
Anita’s arms were uplifted to receive her when Rawley came up
head and shoulders through the gaping hole in the dugout roof. But
he shook his head, stepped out with her in his arms and dug heels in
the soft bank, working his way down to the level.
He still held the girl in his arms, looking for a place where he might
lay her comfortably, when the earth shook beneath his feet. The
terrific boom of the explosion deafened him. The jumble of rock
shook and fell, tighter packed.
The auxiliary dam was in.
CHAPTER THIRTY-THREE
THE EAGLE’S WING
Nevada was lying on the bed in Anita’s shack, trying to convince
Rawley that the doctor knew what he was talking about. The doctor
had declared that Nevada’s injuries were mostly superficial bruises
and the nervous shock of sitting cramped in one position for hours,
expecting every moment to be crushed to death. Nevada had
seemed rather crestfallen when Rawley told her how simple a matter
it had been to free her from the beam.
“The whole thing caught me unawares just when I had stopped a
minute to rest,” she explained defensively. “I think I was half asleep
when it happened, and of course my lamp was smashed too flat
even to think of exploding. It was black dark, and I suppose it was
natural to imagine that I was being crushed when I was merely held
fast. I did not try to move. I was afraid the whole thing would come
down on me. Of course, I should have thought of the cushions,—”
“You’d be a wonder if you had; even more of a wonder than you
are.” Rawley took her hand in both of his and patted it, in a sublime
disregard of the circumstances of his last visit to the basin. “I believe
in omens, Nevada. Fate gave me a splendid one when I found you.”
Rawley smiled at her mysteriously, his eyes twinkling.
“In the general wreck, my old pipe had landed from some cranny
right on the desk beside you. You can’t make me believe that Fate
didn’t mean something by that! The way I interpret it—”
“A freak accident,” interrupted Nevada, her cheeks showing
alarming symptoms of a sudden attack of fever. “That old pipe! You
didn’t take it, and I must have tucked it up somewhere until you
came again. I suppose it rattled down.”
Rawley’s eyes had never been so blue. They were like looking
down upon a sunlit sea. He dipped his fingers into the pocket of
Nevada’s blouse and produced the pipe, turning it tenderly in his
hands.
“God bless the day I learned to smoke!” he murmured, his eyes
still dancing. “It may have rattled down—but I know it’s a good omen.
It means—”
“Yes?” Nevada’s big eyes were upon his face. A faint tremor was
in her lips, as if laughter and tears were fighting for the mastery.
“The omen says that you and I are going to get married within a
week. Well within a week.” He was studying the pipe as a mystic
studies the crystal. “It tells me that the hatchet is forever buried. This
is the pipe of peace, and it passed from me to you. That means that
you and I go through life together. Our trails never separate. It
means—”
“Oh, hush!” Nevada cried sharply and struck at the pipe in his
hand. “Our trails can’t lie together. We can’t marry, ever—ever! You
know that as well as I do. We’re cousins.” She turned her face to the
wall.
Rawley did not speak. He looked up from the pipe, straight into
the eyes of Anita, sitting in a corner like a bronze Buddha disguised
as a squaw.
Anita met his look with stolid obstinacy, never blinking, never a
quiver in her face.
Rawley’s jaw squared a little as he continued to look at her. His
body swayed forward, his eyes boring into her very soul. So had
King, of the Mounted, looked when he demanded that Anita should
choose between himself and Jess Cramer. Rawley did not know why
he stared at her so. He only knew that the truth was there, hidden
behind those unreadable eyes. He knew that the truth would give
him Nevada the moment that truth was spoken. No lips but Anita’s
might speak that truth; other lips were sworn to silence.
The old squaw whimpered under her breath. Her eyes flickered
and could no longer look defiance into those terrible, commanding
blue eyes,—the eyes of King, of the Mounted. Her hand went up to
shield her face from the stare of them. She stirred uneasily in her
chair. She spread her fingers, peering fearfully between them. The
terrible blue eyes looked at her still. Slowly, painfully, scarce knowing
that she did so, Anita pulled herself up from the chair and went
forward as one goes to the bar of justice.
As a flame shoots up suddenly from dying embers, so did a flame
dart out from the ashes of her youth. The stooped, gross old body
straightened. Anita’s head went back. Her eyes glowed with a little of
their old fire. Her voice rang clear, proud with the pride of ancestry
unknown.
“Nevada,” she cried imperiously and spoke rapidly in Indian. “It is
not true that you are his cousin. He is the grandson of a man I loved
in my youth. He is the grandson of Sergeant George King, who was
the father of Peter. I have been ashamed that you should know the
truth. Now I am not ashamed, for I know that stolen love is more
noble than a lie. The father of Peter, him I loved. He was a soldier
and he went away. He promised to return in one month. In three
months he had not come, nor sent me word. I was angry and I let the
man he hated think that I loved him and not my soldier man. Then I
went away, for my heart was sad. I would not follow my soldier man.
I was proud. After a long time—after more than a year had passed I
returned to El Dorado and I brought my child, who was Peter. I
sought for news of my soldier, but there was none. He had not come,
he had not sent me word. So I went to the man I hated. I told him
that Peter was his son, which was a lie. I was very proud. I thought
that some day my soldier would return and would see how I laughed
at him and loved another. But I did not love. And Peter was not the
son of the man my soldier hated. Now the young man comes and
loves, and I am old. Soon I go to my soldier man. It is not right that
others should have sorrow because of my lie.
“So now I speak what is true. I say that this young man is not of
your blood. He is the grandson of the father of Peter, and Peter is his
uncle. You are not his cousin. Now you will be his wife, and you will
hate Anita for the sin of her youth.”
Nevada lay listening, gazing fixedly at her grandmother. She
caught the gnarled old hand of Anita in both her own. She fondled it,
kissed it, laughed softly with tears in her laughter.
“You will not hate Anita?” Tears spilled over the fat lids and trickled
down the cheeks of the old squaw.
Whatever Nevada said, she spoke in Indian, stealing a shy glance
now and then at Rawley. But her voice crooned caresses. Now and
then she kissed the old hand she held in both her own.
Anita tucked in her bangs, drew two fingers across her cheeks to
dry her tears and smiled. She turned heavily toward Rawley.
“My girl say, loves you more—I love your grandfadder. My girl
make you good wife.”
“Hush, Grandmother! He doesn’t want a fighting squaw—”
“Don’t, eh?” Rawley got up and made for her.
At that moment Peter walked in upon them, unconscious of the
fact that he was interrupting a very interesting conversation. Peter’s
face was grave.
“Nevada, do you and mother know anything about Young Jess?
Gladys is all upset over him. She thought he was down in the river
with his father. She heard them talking about getting gold, and then
the dam went, and she hasn’t seen him since. If he’s hiding,” he
added sternly, “he may as well come out and show himself. I think it
can be fixed up. The Governor wants to ask him some questions.”
“How could I know? I was penned in when the cabin fell to
pieces,” Nevada countered. “They certainly said nothing to me,
either one of them. I didn’t see them all afternoon or evening.”
Anita slowly lifted her hand to her face and gropingly tucked in her
bangs. Her eyes were fixed dumbly on Peter’s face.
“Young Jess—by river,” she said reluctantly. “I walk in moonlight,
no can sleep. Comes big shootin’. I fall down. Bimeby I hear Nevada
—she call me come quick. I no see Jess no more. I come.” She
recapitulated slowly. “Jess by river, look on river. Comes shoot. No
see Jess no more. Nevada call loud. Jess no come.”
The eyes of the two men met significantly. Peter turned and went
out, and Rawley followed him.
“Concussion,” Rawley said succinctly. “If he were on the edge of
the bank, it would throw him off, very likely. It’s high, out here, and
pretty steep. He went into the river, in that case.”
“Yes—some folks upriver came near getting it when we shot in the
second dam,” Peter said tonelessly. “I sent a man up on a hill to
wave back any stragglers, but the doctor had to do some patching
on the crowd, nevertheless. Well, I’ll go and look along the river. He
may be hurt, under the bank.”
Rawley did not think so, but he went with Peter and searched the
bank thoroughly. Halfway down, caught behind a bowlder, he found
Young Jess’s hat. He managed to retrieve it and bring it to Peter.
Peter turned it over in his hand, looked at Rawley and nodded.
“It’s his,” he said shortly. “It’s all we’ll ever find.”
He turned away toward the shack, swung back suddenly and
faced the tremendous heap of broken rock visible from midstream to
the farther shore. He lifted both hands high above his head, his face
twisted, his eyes black with sublime fury.
“Damn you!” he cried. “Curse the thought, born in greed, fostered
in rapacity, that put you there! Curse the bitter years that brought you
to pass! For the greed of the gold they would have filched, for the
vulture’s eye that watched and waited all these years, to swoop
down and snatch and grab, with never a thought for the rights of
other men, I curse the thing I helped to make!
“Born in selfishness, you have defiled a mighty river that God
meant should flow through the land and one day be a blessing to
mankind. You have made of the river a monster. It is you that is
driving women and little children from their homes! You, God damn
you! You have been a traitor to the mind that brought you forth. You
have destroyed the two who worked and waited, that you might
pander to their greed. You have tried to destroy the dearest thing I
have on earth, because I saw in you something big and beautiful—
because I was fool enough to think that an idea spawned in devil-
greed could live in noble achievement.
“Look at the slimy thing the vultures have made of the river! The
leprous thing over which the vultures croaked—for a little while—
croaked and went down and died! The Eagle would never stop the
river, leave it a naked, stinking thing under the sky. For the good of
mankind, the Eagle would have tamed the river, without destroying it.
The Eagle would have had it run peacefully within its banks, helping
without hurting. Now the river lies shamed in its bed—that
magnificent stream!—and men flee from it in terror. The two who
thought to feast in the slime—yes, and I, too, could stoop so low as
to root for gold like a hog in the mire!—you have swept them to
destruction, have cheated them at the last of their prey.
“But you have done your worst! I, who helped to make you what
you are, who created you thought by thought, I will tear you down.
For the thing you are, a monument to greed and self, I shall tear you
down stone by stone until the river is once more sweeping
majestically down to the sea. As God is my witness, this thing the
vultures have created shall be forgotten. The Eagle’s wing shall
shadow the Colorado, a river undefiled.”
His voice ceased. He stood, hands clenched beside him, jaw
squared, staring at the dam that had been his dream. A dream
fulfilled,—and hated in the fulfillment. His lips moved, muttering the
prophecy of Johnny Buffalo:
“‘You will succeed, and fail in the succeeding. And from the failure,
—’”
A gloved hand was laid in friendly fashion on Peter’s shoulder. He
turned and looked into the eyes of his Governor.
“It takes a big man, a man of broad vision, to look upon his life’s
work and dare to say what you have said,” the Governor told him
kindly, the look of understanding in his eyes. “Don’t be down-hearted
because your success has proved a failure. The Cramer Dam would
hold, I believe, if we wanted it to hold. But you are right. It is not for
the vulture, but for the Eagle to say what shall be done with the river.
The country needs more men like you, Peter. You shall help to build
another dam—and build it under the Eagle’s wing.”
Peter lifted his right hand and laid it upon the shoulder of his
Governor. His eyes were very blue and very deep. So they stood for
a space and looked into each other’s eyes.
“‘—And from the failure rise to greater things,’” Rawley repeated
under his breath, his eyes shining.
THE END
NOVELS BY B. M. BOWER
THE HERITAGE OF THE SIOUX
A Flying U story in which the Happy Family get mixed up in a
robbery faked for film purposes.
“Altogether a rattling story, that is better in conception and
expression than the conventional thriller on account of its touches of
real humanity in characterization.”—The Philadelphia Public Ledger.

STARR, OF THE DESERT


A story of mystery, love and adventure, which has a Mexican
revolt as its main theme.
“The tale is well written.... A book worth the reading which it is
sure to get from every one who begins it.”—The New York Tribune.

CABIN FEVER
How Bud Moore and his wife, Marie, fared through their attack of
“cabin fever” is the theme of this B. M. Bower story.
“It is breezy and wholesome, with a quiet humor.... Plenty of action
is evident, while the sentimental side of the story is thoroughly
human and altogether delightful.”—The Boston Transcript.

SKYRIDER
A cowboy who becomes an aviator is the hero of this new story of
Western ranch life.
“An engrossing ranch story with a new note of interest woven into
its breezy texture.”—The Philadelphia Public Ledger.

RIM O’ THE WORLD


An engrossing tale of a ranch-feud between “gun-fighters” in
Idaho.
“The author has filled the story with abundant happenings, and the
reader of this class of story will find many a thrill in its pages.”—The
Philadelphia Public Ledger.

THE QUIRT
A story of ranch life in Idaho, with an abundance of action,
adventure and romance.
“Like all the Bower novels, ‘The Quirt’ rings true. Lovers of
Western Stories have long voted Bower a place in the front rank of
those who tell of ranch-life, bad men, range wars and rough
riding.”—The Boston Herald.

COW-COUNTRY
This story of Bud Birnie will appeal to all lovers of tales of the real
West.
“A live, well-told Western romance which bears above all else the
impress of truth in its descriptions of both persons and country.”—
The New York Times.

CASEY RYAN
Lovers of stories of the real West will enjoy this humorous tale.
“This is one of the cleverest and most amusing of all the many
books that have come from B. M. Bower’s pen.... It is a rollicking
story, full of mirth and laughter from beginning to end.”—The New
York Times.

THE TRAIL OF THE WHITE MULE


Another Casey Ryan story in which Casey is funnier than ever.
“The author produces in Casey Ryan a fictional creation, a unique
character that is a worth while addition to our gallery of Western
portraits in fiction.”—The New York Times.

THE VOICE AT JOHNNYWATER


“It is a crackerjack of a story, in B. M. Bower’s best style, the sort
of story that you have to read in one evening, so absorbing is it.”—
The St. Louis Globe-Democrat.
LONESOME LAND
A vigorous tale of ranch life in Montana.
“Montana, described as it really is, is the ‘lonesome land’ of this
delightful Bower story. A prairie fire and the death of the worthless
husband are especially well handled.”—A. L. A. Booklist.

THE RANCH AT THE WOLVERINE


A tale of Idaho ranch life, with a bewitching heroine.
“A ringing tale full of exhilarating cowboy atmosphere, abundantly
and absorbingly illustrating the outstanding features of that alluring
ranch life that is fast vanishing.”—The Chicago Tribune.

THE FLYING U’S LAST STAND


What happened when a company of school teachers and farmers
encamped on the grounds of the Flying U Ranch.
“How the ranchmen saved their grazing grounds is told by the
novelist with breezy humor and an overflow of fanciful incident.”—
The Philadelphia North American.

THE PAROWAN BONANZA


“The reader can always take up a story of B. M. Bower with the
assurance that it will seethe with action, humor, Western color and
romance.... ‘The Parowan Bonanza’ is a smooth-running, well-told
tale that leaves the reader with a comfortable sense of having seen
the desert country at close range, of having known its mysterious,
starlit nights and burning days, and of having participated for a time
in all the surge and rush of a mining town in its making and its
débâcle.”—The New York Times.

THE EAGLE’S WING


A project to dam the Colorado River furnishes the theme of this
characteristically picturesque and exciting Bower story.
Boston—LITTLE, BROWN & COMPANY—Publishers
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