Bilateral Vocal Fold Paralysis

Author: Joel A Ernster, MD; Chief Editor: Arlen D Meyers, MD, MBA

Background
Bilateral vocal fold (vocal cord) immobility (BVFI) is a broad term that refers to all forms of reduced or
absent movement of the vocal folds. Bilateral vocal fold (cord) paralysis (BVFP) refers to the
neurologic causes of bilateral vocal fold immobility (BVFI) and specifically refers to the reduced or
absent function of the vagus nerve or its distal branch, the recurrent laryngeal nerve (RLN). Vocal fold
immobility may also result from mechanical derangement of the laryngeal structures, such as the
cricoarytenoid (CA) joint.

Direct laryngoscopic view of the larynx in a

patient who with bilateral vocal fold immobility (BVFI) is shown. Palpation of the arytenoids revealed cricoarytenoid (CA)
joint ankylosis. Close inspection of the interarytenoid space demonstrated interarytenoid scar. This condition is posterior
glottic stenosis (PGS).

Although a small number of conditions account for most cases of vocal cord immobility, this article
presents a comprehensive differential diagnosis, followed by the clinical presentations, diagnostic
workup, and treatment options. The goal of the article is to provide the clinician with a basic
understanding of the rare entity of bilateral vocal fold immobility (BVFI).

History of the Procedure

The history of the procedures used to treat vocal cord immobility begins in 1855 with Garcia's work on
mirror laryngoscopy. In the 1860s, Turk and Knight first described vocal cord paralysis. In 1922,
Chevalier Jackson performed the first surgical procedure for bilateral vocal fold immobility (BVFP)
when he endoscopically resected a vocal cord. He provided an airway at the expense of voice and
airway protection. This dilemma continues to plague present surgeons. Since 1922, pioneers in
laryngology have described arytenoidectomy, described vocal cord lateralization, and introduced the
use of laser.

Etiology
According to Benninger's findings in a series of 117 cases BVFI can be attributed to the following
causes: surgical trauma (44%), malignancies (17%), endotracheal intubation (15%), neurologic
disease (12%), and idiopathic causes (12%). [1]
In adults, conditions that mimic vocal fold immobility include paradoxical vocal fold motion and
functional disorder.
Causes of vocal fold fixation differ in adults and in children. In adults, these include mechanical
causes, inflammatory processes (affecting the CA or larynx), malignancy, surgery, neurologic causes,
radiation injury, metabolic causes, and toxins. Mechanical derangement of the posterior glottis may
also be referred to as posterior glottic stenosis (PGS). Bogdasarian and Olson classified PGS into the
following 4 grades:[2]

Grade I - Interarytenoid scarring with normal posterior commissure

Grade 2 - Interarytenoid and posterior commissure scarring
Grade 3 - Posterior commissure scarring involving one cricoarytenoid joint
Grade 4 - Posterior glottic scarring involving both cricoarytenoid joints

Mechanical causes
See the list below:

o
o

o
o
o
o
o

Acute complications of intubation

Arytenoid dislocation
Injury to the recurrent laryngeal nerve (RLN) because of anterior displacement of
thyroid cartilage relative to the cricoid cartilage
Hyperextension of the neck that stretches the vagus nerve
Laryngeal mask airway
Excessive cuff pressure that compresses the RLN as it enters the larynx
Chronic complications of intubation
Posterior glottic stenosis (PGS) due to prolonged or traumatic intubation
Excessive cuff pressure compressing the RLN as it enters the larynx
Sofferman nasogastric tube syndrome
Stent placement in proximal esophagus [3]

Inflammatory processes that affect the CA

See the list below:

Rheumatoid arthritis
Gout
Tietze syndrome
Ankylosing spondylitis
Reiter syndrome
Crohn disease
Collagen vascular disease
Mumps
Systemic lupus erythematosus

Inflammatory processes that affect the larynx

See the list below:

Wegener granulomatosis
Amyloidosis
Sarcoidosis
Cicatricial pemphigoid
Tuberculosis
Syphilis
Gastroesophageal reflux disease
Relapsing polychondritis

Malignancy
See the list below:

Laryngeal neoplasm
Chondromas and chondrosarcomas
Squamous cell carcinoma

Surgery
See the list below:

Bilateral injury may be caused by the following:

Thyroid surgery
Parathyroid surgery
Esophageal surgery
Tracheal surgery
Brainstem surgery

Contralateral injury after an earlier unrecognized ipsilateral injury may be caused by the
following:
o
Completion thyroid surgery
o
Contralateral carotid endarterectomy
o
Anterior approach to cervical disk, which is becoming an increasingly common
phenomenon.[4]

Endolaryngeal surgery with a carbon dioxide laser may injure the posterior glottis.
o
o
o
o
o

Neurologic causes
See the list below:

Arnold-Chiari malformation
Meningomyelocele
Diabetes mellitus
Amyotrophic lateral sclerosis
Myasthenia gravis
Mbius syndrome
Charcot-Marie-Tooth disease
Postpolio syndrome
Shy-Drager syndrome
Creutzfeldt-Jacob disease
Hydrocephalus
Synkinesis of the RLN
Lyme disease
Neoplasms or sarcoidosis involving nodes in the mediastinum that impact the RLNs

Radiation injury
See the list below:

Radiation therapy
Postirradiation fibrosis of the CA, vocal folds, or both
Chondronecrosis

Metabolic causes
See the list below:

Hypokalemia
Hypocalcemia
Diabetes mellitus
Renal insufficiency with Alport syndrome

[5]

Toxins
See the list below:

Vincristine
Taxol
Organophosphates [6]
In children, causes of bilateral vocal fold immobility (BVFI) include central neurologic abnormalities,
idiopathic causes, and iatrogenic causes.

Central neurologic abnormalities

Central neurologic abnormalities account for most cases of childhood bilateral vocal fold paralysis
(BVFP). Arnold-Chiari deformity with meningomyelocele and hydrocephalus is the most common
abnormality. Other CNS insults (eg, infarct, craniotomy, asphyxia) account for some cases, according
to the findings in a study by Rosin et al.[7]

Idiopathic causes
Idiopathic causes are the second most common causes of childhood bilateral vocal fold paralysis
(BVFP). Some researchers postulate that the etiology in some children with bilateral vocal fold
paralysis (BVFI) is an imbalance between the adductors and abductors of the larynx that results in
adducted vocal folds. With time, a balance is restored and symptoms abate as children mature.
Although conjectural, this explanation fits with the clinical course of most children with bilateral vocal
fold paralysis (BVFI) who spontaneously improve with time. Gacek hypothesized that fewer abductor
fibers exist; therefore, injury to the nerve is more likely to cause abductor dysfunction. [8] He also
conjectured that, since abductor fibers are phylogenetically younger than adductor fibers, they may be
more fragile.

Iatrogenic causes
Iatrogenic causes, including mediastinal procedures, cervical procedures, prolonged intubation, and
birthing trauma, account for the remaining cases.

Pathophysiology
Although a comprehensive discussion of each of the causes is beyond the scope of this article, some
principles should be emphasized. With the first episode of bilateral vocal fold paralysis (BVFP),
patients may have dysphonia because the vocal cords are too far apart. Over time, however, the vocal
cords can move to a medial position, and the patient may have a good voice and cough despite
stridor and bilateral vocal fold paralysis (BVFP). As the vocal cords migrate toward the midline, the
voice (and cough) improves, while the airway worsens. Clinicians should not mistake a good voice
and cough as signs of a functioning larynx, especially in a patient with stridor. Aspiration and
dysphagia may or may not be present in patients with vocal cord paralysis.
In terms of the pathophysiology of CA fixation, inflammatory or fibrotic changes can paralyze or
reduce the mobility of the joint. Various disorders can cause these changes.

Presentation
History
The importance of a complete history cannot be overstated. The history should include the following:

Chief symptom, as related to airway, voice, or swallowing

Onset of symptoms (acute, subacute, chronic)
Changes in the voice and airway over time
Related events such as intubation, surgery, or other medical conditions that can affect vocal
cord mobility
Tobacco use
In children, obtaining a history of birth trauma, central nervous system abnormality, intubations, or
surgeries is important.

Physical examination
The physical examination should include listening to the voice and airway as the patient relays his or
her history.

The voice can be breathy or normal.

Airway findings can range from biphasic stridor to normal.
Unless patients describe gross aspiration with swallowing, their swallowing function can be
challenged by having them sip a small amount of water.
The standard head and neck examination should include careful evaluation of the larynx. Evaluate the
following:

Mucosal color and condition

Stenosis or scarring of the posterior glottis
Mobility of the arytenoids
Muscle mass and tone of each vocal cord
Length of each vocal cord
Asymmetry of the vocal cords

Indications
Adults
Only the patients with severe bilateral vocal fold (cord) immobility (BVFI) require surgical intervention.
Patients with medical conditions (eg, rheumatoid arthritis, Wegener granulomatosis, gout) or
neurologic conditions (eg, amyotrophic lateral sclerosis [ALS], Parkinsonism, stroke) rarely require
surgical intervention because treatment of the underlying condition often improves airway
compromise.
For patients with bilateral vocal fold paralysis (BVFP) due to iatrogenic injury in which the recurrent
laryngeal nerve (RLN) or vagus nerve is injured (neurapraxia) but not severed, permanent surgical
treatment should be postponed for at least 9 months after injury to allow spontaneous recovery.
Laryngeal electromyographic (EMG) monitoring can be helpful in obtaining an index of potential
recovery. Obtaining a baseline EMG 30-40 days after injury and second EMG 1 month later can help
in evaluating the recovery status of the vocal cords (Munin). [9] On the basis of the surgeon's clinical
judgment, tracheostomy for patients with quickly deteriorating airways should be initiated quickly.
For adult patients with bilateral vocal fold (cord) paralysis (BVFP), the literature supports use of an
endoscopic approach, with either posterior cordectomy or limited arytenoidectomy as the initial
procedure of choice. Suture lateralization may play an adjunctive role. All of these are static
permanent procedures; therefore, they should be undertaken only after spontaneous improvement
has failed to occur or if EMG findings suggest permanent injury.
For patients with bilateral vocal fold immobility (BVFI) caused by PGS, serial endoscopic approaches
with scar lysis or microflap trapdoor reconstruction of the interarytenoid (IA) region can be attempted
before the static procedures are used.
Airway obstruction refractory to the above measures is particularly vexing. Treatment options include
laryngofissure with arytenoidectomy, IA reconstruction, posterior cricoidotomy with stent placement, or
posterior cricoidotomy with grafting. The literature is less clear concerning the indications for each of
these approaches than those of other procedures.

Children
Surgical intervention is indicated when respiratory effects are significant. Cordopexy or
arytenoidopexy, along with partial or complete arytenoidectomy, can help solve the airway problem
during the ensuing months or years as one waits for possible recovery of the contralateral cord.
Children with bilateral vocal fold paralysis (BVFP) require tracheostomy only when o the airway fails to
improve with other measures. Findings of a literature review suggest that the airway can be managed
expectantly, without a tracheostomy. Endoscopic management plays a limited role in children and is
useful only for mild fixed stenosis and for revisional procedures in children who have undergone open
procedures.

Relevant Anatomy
A review of vagus nerve and RLN anatomy is necessary to understand potential injuries that can
cause vocal cord paralysis. The vagus nerve originates in the nucleus ambiguus of the medulla
oblongata. At that point, it is composed of cells that receive neural input from the Broca area via
decussating corticobulbar tracts; thus it provides input to both the right and left nuclei. Neural input
from the cerebellum and extrapyramidal centers, as well as from visceral afferents, provides
proprioceptive input that modulates the motor function of the vagus nerve at this site.

The motor fibers or visceral efferents that affect the larynx and pharynx occupy 2 specific sites within
the nucleus ambiguus. One site becomes the superior laryngeal nerve (SLN); the other, the RLN. The
vagus nerve leaves the medulla and enters the jugular foramen, along with the accessory nerve and
jugular vein. Within the jugular foramen, the vagus nerve widens to form the superior ganglion, where
the cell bodies of the sensory component of the nerve reside (somatic sensory). They provide
sensation to the ear canal skin (Arnold nerve). As the vagus nerve exits the jugular foramen, it widens
again to form the nodose ganglion, in which nerve cell bodies containing the sensory or visceral
afferents from the larynx and pharynx reside.
Immediately distal to the nodose ganglion, the SLN exits the vagus nerve and courses along the
carotid artery to the larynx, where it enters the larynx through the thyrohyoid membrane, dividing into
internal and external branches. The internal branch provides sensory function (visceral afferent), and
the external branch provides motor function to the cricothyroid muscle (visceral efferent). The vagus
nerve then descends in the neck immediately lateral to the carotid artery.
The right RLN fibers exit from the vagus nerve as the nerve crosses anteriorly over the subclavian
artery. The RLN loops posteriorly around the subclavian artery to enter the larynx through the KillianJamieson area or superior to the fibers of the cricopharyngeal muscle entering the larynx at the
cricothyroid space.
The left RLN divides much further in the mediastinum, exiting the vagus nerve as it crosses anterior to
the aorta and lateral to the ligamentum arteriosum (ie, remnant of the patent ductus arteriosum
between the aorta and the pulmonary vein). It then extends superiorly to enter the larynx opposite the
right RLN. The RLN branches into the posterior sensory branch and the motor anterior branch to the
posterior cricoarytenoid (PCA), IA, lateral cricoarytenoid (LCA), and thyroarytenoid (TA) muscles. The
IA muscle is the only motor branch that receives bilateral innervation, which allows some movement of
both vocal folds when one RLN is nonfunctional.

Contraindications
In adults, any definitive procedure to address vocal cord paralysis, whetherunilateral or bilateral, must
not be undertaken while a possibility for recovery exists. Recovery can occur as long as 12 months
after injury. Every attempt must be made to determine if function is likely to return. This determination
should include video direct laryngoscopy, during which the vocal fold can be palpated to assess
mobility and bronchoscopy. In addition, laryngeal EMG can be used to evaluate normal action
potentials (normal nerve), the absence of potentials (nonfunctioning nerve), defibrillating potentials
(worsening nerve), or polyphasic potentials (regenerating nerve). The 12-month wait for return of
function can be shortened by obtaining 2 laryngeal EMGs several months apart and by looking for
evidence of improved function or stabilized function. [9]
As many as 70% of children with bilateral vocal fold (cord) paralysis (BVFP) require a tracheostomy.
However, spontaneous recovery occurs in half of the patients, sometimes in those as old as11 years.
If the condition spontaneously resolves, it typically does so 24-36 months after diagnosis. Therefore,
destructive static procedures should be delayed for approximately 3 years because of this potential for
recovery. Delaying surgery in children with bilateral vocal fold (cord) immobility (BVFI) caused by PGS
is not beneficial; consequently, bilateral vocal fold immobility (BVFI) must be diagnosed correctly in
these children to prevent restriction from surgical repair.

Diagnostic Procedures
Fiberoptic laryngoscopy

This procedure is the mainstay of clinical assessment.

Stroboscopic videolaryngoscopy may provide further information about vocal fold motion
abnormalities when asymmetric mucosal wave patterns are identified.
Malingering or other psychogenic disorders may be identified by asking the patient to sniff or
whistle, since these maneuvers work the abductors without the patient's volition.
Direct laryngoscopy

Examination of the posterior glottis and palpation of the arytenoid cartilages are essential
steps in clarifying the nature of immobile vocal folds.
Cricoarytenoid (CA) joint ankylosis or IA scars that limit arytenoid motion are readily
ascertained with direct laryngoscopy with the patient under general anesthesia and paralysis.
The subglottis, trachea, and main bronchi also may be examined to exclude subglottic
stenosis, subtle infiltrative neoplasms, and other lesions along the entire airway.

Direct laryngoscopic view of the larynx in

a patient who with bilateral vocal fold immobility (BVFI) is shown. Palpation of the arytenoids revealed cricoarytenoid
(CA) joint ankylosis. Close inspection of the interarytenoid space demonstrated interarytenoid scar. This condition is
posterior glottic stenosis (PGS).

Laryngeal EMG

Ideally, laryngeal EMG is used to assess both the TA and the PCA muscles, and it should be
performed with local anesthesia rather than general anesthesia. It has been performed in
anesthetized children.

The TA muscle is approached through the cricothyroid membrane, while the PCA muscle is
approached percutaneously by rotating the larynx.

The glottic compromise caused by bilateral vocal fold immobility (BVFI) may render EMG
hazardous. Therefore, waiting until after tracheostomy to perform the test may be prudent in some
cases.

In evaluating a patient with bilateral vocal fold immobility (BVFI), EMG provides the potentially
useful information in the following:
o
Differentiating between fixation and paralysis
o
Differentiating between neurapraxia and axonal transection
o
Determining the presence of neuromuscular disorders or peripheral neuropathy

In the recording the EMG, correct timing is essential. EMG can be performed as soon as 2
days after injury to aid in differential diagnosis. As a prognostic tool, a baseline EMG should be
obtained at least 30 days after injury and a second one should be obtained 30-60 days after injury.
After 6 months, laryngeal EMG should be used only to differentiate between fixation and paralysis
and not to assess neural regeneration.

Laryneal EMG can predict poor recovery 90% of the time. [10]

Surgical Therapy
Procedures for bilateral vocal fold immobility (BVFI) due to IA scarring with or without CA ankylosis:

o
o
o
o

o
o
o

Tracheostomy
Functional procedures
Microflap trapdoor techniques
Laryngofissure with posterior cricoidotomy with cartilage grafting and stent placement
(or only stent placement)
Local mucosal flap reconstruction
Excision of the scar and mucosal or skin grafting
Static procedures
Posterior cordotomy (unilateral or bilateral)
Arytenoidectomy (endoscopic or external, partial or complete)
Suture lateralization

Procedures for bilateral vocal fold (cord) paralysis (BVFP)

o
o
o

Tracheostomy
Reinnervation techniques (experimental) [12]
Electrical pacing (experimental) [13]
Permanent procedures
Posterior cordotomy (unilateral or bilateral)
Arytenoidectomy (endoscopic or external, partial or complete)
Cordopexy, lateralization of the vocal cord