Neurogenic

Disorders of
the Larynx
DILLATIA ALMA MEIDINA
Normal speech requires the complex integration of peripheral and central motor control mechanisms.

The voice component of speech (phonation) requires:

1) neural control of the intrinsic and extrinsic laryngeal muscles to shape the glottis, and

2) a steady stream of air flow from the respiratory system to support regular, symmetric, and synchronous vibration of the vocal folds and
accurate shaping of the resonating cavities in the vocal tract.

Thus, the central nervous system must coordinate the respiratory, phonatory and speech mechanisms to produce normal phonatory control during speech
EFFECTS OF SPEECH ON LARYNGEAL VIEW DURING ENDOSCOPY

The larynx does not function in isolation from other structures in the vocal tract. Muscles shaping the pharynx and oral cavity, particularly
those controlling the tongue, lip, and jaw, alter laryngeal tension as they change vocal tract.

Valving above the vocal folds by the ventricular folds, velum, tongue, and lips amplifies certain harmonics of the fundamental frequency
of vocal-fold vibration, based on the posture and shape of the vocal tract.

harmonics within certain resonant frequencies are amplified resulting in increased sound energy in frequency regions called formants The
formant frequencies are responsible for the vowel sounds perceived. In high vowel sounds such as /i/ or “ee”, the posture of the tongue is high and forward exerting
tension from the tip to the base of the tongue.
MULTIDIMENSIONAL ASPECTS OF
VOICE FUNCTION AND DISORDERS
Pharyngeal and/or laryngeal constrictions produce turbulence in air flow which may be increased in neurogenic disorders. In these
disorders, reduced vocal fold adduction can produce valving insufficiency and inefficient voice production leading to a reduction in sound
amplitude, vocal quality and duration of sound production.

Both peripheral and central neurogenic disorders may alter the voice by changing the configuration of the larynx and vocal tract.

Perceptual voice parameters, such as pitch, loudness, voice quality, and their variability, may help to characterize neurogenic and
functional voice disorders. Flexible examination is required when the voice disorder occurs more during connected speech.

Ultrasound, videofluoroscopy, thincut computed tomography (CT), and magnetic resonance imaging (MRI) are tools that have value in the
diagnostic protocol. Use of electromyography (EMG) accompanied by magnetic stimulation of the laryngeal nerve may be valuable in
identifying peripheral neurogenic dysfunction, although laryngeal muscle responses to cortical magnetic stimulation should be validation.
NEUROGENIC DISORDERS
Dysphonia can appear as the first sign of neurogenic disease whereas the other speech symptoms (dysarthria) may only become evident as
the disease progresses. Therefore, neurogenic voice symptoms may evolve from a focal laryngeal disorder to a progressive
neurodegenerative disease.

For example, what may initially appear to be a unilateral idiopathic vocal fold paralysis might later progress to become a vocal-fold
paralysis as part of a peripheral neuropathy.

Functionally, neurogenic disorders resulting in dysphonia may be divided into the following categories:

1. Consistent neurogenic voice disorders are characterized by constant vocal quality, loudness or pitch deviations during speech and
sustained vowels. Examples include flaccid dysarthria, due to lower motor neuron disease such as amyotrophic lateral sclerosis (ALS) or
progressive supranuclear palsy, a peripheral neuropathy or laryngea
2. Spastic dysarthria including dysphonia, is associated with upper motor neuron disease and may involve the corticobulbar tracts. May be
seen in Multiple Sclerosis.

3. Non-rhythmically fluctuating neurogenic voice disorders are characterized by unpredictable, irregular variations in quality, loudness,
and pitch during speech.

4. Rhythmically fluctuating neurogenic voice disorders, including essential voice tremor, a relatively common chronic voice disorder, and
palatopharyngolaryngeal myoclonus, a rare disorder. These dysphonias are marked by regular or rhythmic fluctuations in voice, pitch, and
loudness.
5. Neurogenic voice disorders associated with loss of volitional control of voice production, including apraxia of phonation, respiration or
speech and akinetic mutism, usually follow a cerebrovascular accident or cortical injury. In addition paroxysmal neurogenic voice
disorders exhibit bursts of dysphonic voice, as in Gilles de la Tourette syndrome.

6. Fluctuating vocal fold paralysis can occur in myasthenia gravis mimicking a vocalfold paralysis and can a!ect both voice and
swallowing
Upper Motor Neuron Disorders
Upper motor neuron diseases which affect the voice include Parkinson disease and related syndromes. Parkinsonism is a slowly
progressive disease affecting the function of nigrostriatal dopaminergic neurons in the substantia nigra. Hence, damage to the basal
ganglia can release inhibition of nerve impulses a!ecting the lower motor neurons, resulting in rigidity and reduced rate of movement
(bradykinesia).

A. Hypophonia in Parkinson Disease.

Reduced loudness and breathy vocal quality, referred to as hypophonia, are the hallmark of voice disorders in early Parkinson disease.
The patient may have difficulty with production of glottal stops or voice onset after voiceless consonants such as /s/. A reduced range and
speed of vocal-fold movement, particularly on the more a!ected side, Distinctions between voiceless and voiced sounds become reduced
due to impaired ability to adduct and abduct the vocal folds rapidly. In later stages of the disease, the patient may be unable to produce
phonation
B. Progressive Supranuclear Palsy.

Progressive Supranuclear Palsy (PSP) is a rare degenerative neurological disorder characterized by supranuclear ophthalmoplegia,
complaints of falling backwards, nuchal dystonia in extension, moderate axial dystonia, pseudobulbar palsy, difficulty in swallowing,
dysarthria, bradykinesia, masked facies, nonspecific changes in personality, lability, sleep disturbance, dementia and performance
decrements on various neuropsychological, but without tremor.

Dysphagia is a major problem for these patients. Hypophonia is present with unilaterally reduced vocal fold range and speed of
movement. The associated dysarthria may include palilalia, uncontrolled syllable repetition and oral motor rigidity. No vocal tremor.
C. Multiple Systems Atrophy.

This is a rare degenerative movement disorder with lesions in the cerebellum, brainstem, and basal ganglia. Three clinical variants are
recognized :

1) spinocerebellar degeneration (olivopontocerebellar atrophy, or OPCA);

characterized by progressive cerebellar ataxia, and coordination of the laryngeal muscles is a!ected as in ataxic dysarthria. One sees loss of
muscle coordination (dyssynergia), loss of ability to gauge range of motion (dysmetria), and tremor during voluntary movement (intention
tremor). Dysphonia may take one of several forms: sudden bursts of loudness, irregular increases in pitch and loudness, or coarse voice
tremor.
2) progressive autonomic failure (Shy-Drager syndrome);

voice symptoms typical of Parkinson disease are present, along with progressive autonomic dysfunction. Speech is slowed with a
reduction in vocal intensity, an absence of glottal stops and poor voicing contrasts. Patients may also be predisposed to laryngospasm

3) atypical parkinsonism (striatonigral degeneration).

D. Pseudobulbar Palsy.

Pseudobulbar palsy results from neuronal loss above the level of the nucleus ambiguus in the brainstem and involves the corticobulbar
tracts bilaterally. Dysphonia associated with these types of lesions can be characterized by either weak breathy voice production with loss
of facial expression or strained-strangled, harsh voice.

E. Multiple Sclerosis.

Multiple Sclerosis (MS) is a progressive demyelinating disorder, having sensory and motor impairments, some cognitive problems,
spasticity and tremor. Essential features include staccato speech and harsh voice quality with intermittent hyperadduction of the vocal
folds. Spasticity and tremor may affect all or some regions of the vocal tract, thereby reducing speech intelligibility and making speech
effortful.
F. Myoclonus.

Myoclonus is a sudden, brief, jerking involuntary movement of the involved muscles that occurs in a rhythmic fashion. The distribution
may be focal, multifocal, or generalized, with the presentation spontaneous, active, or stimulus-sensitive.

Palatal myoclonus is focal a!ecting the movements of the palate either unilaterally or bilaterally at 1.5 to 3 Hz. It is frequently
accompanied by synchronous movements of adjacent structures such as the pharynx, tongue, larynx and occasionally by the extraocular
muscles, face, neck, or diaphragm. When the soft palate is involved, abrupt, rhythmic, anteroposterior, and vertical movements are present.
This rhythmic activity can open and close the eustachian tubes, thereby producing a clicking sound transmitted to the middle ear. Voicing
deficits may be not often perceived during connected speech but will be apparent on vowel prolongation.
Lower Motor Neuron Disorders
Amyotrophic lateral sclerosis is a degenerative disease of the corticobulbar tract and lower motor neuron nuclei.

The speech and voice symptoms may vary depending on the predominance of spastic or flaccid components. Flaccid symptoms present as
hypoadduction of one or both vocal folds and pooling of saliva in the pyriform sinuses on laryngeal examination. A mixed form of
dysphonia may result in voicing characterized by both flaccid and spastic components.

Amyotrophic lateral sclerosis may present with primarily pyramidal tract signs or lower motor neuron signs of progressive muscular
atrophy. Speech, voice and swallowing symptoms may be the first signs in the bulbar type. Facial muscle weakness, palatal weakness, and
lip, tongue, and jaw weakness with tongue fasciculations are predominant and cause poor speech intelligibility. Voicing is weak and
breathy because of flaccidity of the vocal folds
A. Myastenia Gravis

Myasthenia gravis is a disorder of acetylcholine transfer at the neuromuscular junction, characterized by weakness and fatigability of
striated muscle. This disorder causes a flaccid dysphonia, characterized by breathy, weak phonation. Sometimes stridor can develop with
bilateral abductor muscle weakness. The voice intensity range is reduced, and sustained e!ort causes progressive weakness. This disorder
may affect phonation (larynx), resonance (velum), and articulation (lip, tongue, and jaw), and these systems may be affected separately or
serially as the disease progresses. Flaccid dysphonia may be an early symptom of neurogenic disease .

B. Wallenberg Syndrome.

Occlusion of the posterior inferior cerebellar artery may produce infarction of the lateral medulla, resulting in Wallenberg syndrome, also
known as lateral medullary syndrome
This syndrome is marked by dysarthria and dysphagia, ipsilateral impairment of pain and temperature sensation on the face, and
contralateral loss of pain and temperature in the trunk and extremities. Major symptoms include vertigo, nausea, vomiting, intractable
hiccupping, ipsilateral facial pain, and diplopia. Unilateral vocal fold paralysis and flaccid dysphonia occur when the nucleus ambiguus or
corticobulbar tracts leading to the nucleus ambiguus are affected.

C. Postpolio Syndrome

Patients with PPS who complain of swallowing di"culties are at risk for laryngeal dysfunction. This syndrome is characterized by the new
onset of progressive muscle weakness, fatigue, and pain. Postpolio syndrome may occur 30 to 40 years after the initial infection with
polio. Some patients may develop progressive vocal fold involvement leading to bilateral vocal fold paralysis and acute respiratory dist
Disorders of the Peripheral
Nervous System
The recurrent laryngeal nerves (RLNs) and external branches of the superior laryngeal nerves (SLNs) supply motor innervation to the
laryngeal muscles, providing control, The conduction velocity is high, estimated to be 50 m/s.

Although the innervation ratio of fibers per motor unit in the human larynx is unknown, the laryngeal muscles are somewhat unique in that
single muscle fibers have multiple neuromuscular junctions from the same nerve fiber.

Lesions of the tenth cranial nerve at any point along its pathway from the nucleus ambiguus in the brainstem to the musculature can cause
paresis or paralysis of the laryngeal muscles resulting in dysphonia or even aphonia. The extent of vocal-fold weakness and the degree of
dysphonia depend upon the degree of neural injury. High vagal lesions can a!ect all of the intrinsic laryngeal muscles
EVALUATION OF VOICE
FUNCTION
Clinical evaluation of dysphonia includes diagnostic procedures to determine the cause of the voice disorder, the degree of phonatory
deficit, and the prognosis for recovery of function. Some diagnostic parameters measure the degree and nature of vocal impairment to
determine appropriate intervention. Monitoring the patient’s progress throughout treatment is essential to successful outcome.

1. Videostroboscopy

A description of vibratory characteristics of the vocal folds should include basic information about the: symmetry of bilateral movements,,
regularity of vibration, degree of glottal closure, mucosal wave amplitude, mucosal wave excursion, adynamic regions, and the opening-
closing pattern of the vocal folds during vibration

Disadvantages: disorders as voice tremor and spasmodic dysphonia cannot be studied with videolaryngeal stroboscopy
2. Acoustic and Perceptual Measures

Measures of frequency and intensity characteristics of phonation should include fundamental frequency mean and range, measures of
vocal intensity, and vibration periodicity. Fundamental frequency is the acoustic correlate of pitch, measured in Hertz, Intensity, measured
in decibels (dB) The range in each of these parameters reflects the flexibility of laryngeal dynamics available to the subject.

Harmonics-to-noise ratio (HNR) is often used to assess the degree of energy contained in harmonics of the fundamental frequency
compared to turbulent noise between harmonics present during phonation. HNR values are thought to reflect abnormalities in voice
quality due to asymmetries in mass, neural control, tension, vocal-fold closure and biomechanical characteristics of the vocal folds.
3. Aerodynamic Measures

Some measure of airflow or volume velocity is useful in determining how rapidly the air passes through the vocal folds. Mean airflow may
be obtained from averaged measures over several vibratory cycles. When vocal-fold closure is inadequate for sustained phonation, air flow
rates may be abnormally high while sound intensity is reduced.

These measures are made using a pneumotachograph connected to a facemask placed over the face during voice production and, thus, they
provide an indirect measure of laryngeal airflow.

Subglottal pressure is important for vocal-fold vibration and for modulation of vocal intensity. Subglottal pressure may be measured
indirectly by a pressure transducer placed in the oral cavity.
4. Neurophysiological Measures of Voice and Laryngeal Function

Electromyographic evaluation is a useful adjunct in the assessment of neuromuscular disorders and may be used in prognostic judgments
about patients with those disorders. EMG may be useful in di!erentiating a variety of voice disorders: peripheral and central vocal fold
paralysis, functional disorders and arytenoid joint fixation but needs validation as a diagnostic tool. with the use of magnetic stimulation to
differentiate peripheral and central sources of vocal-fold paralysis are that the central stimulation latencies.

During peripheral testing, the contraction of laryngeal muscles is monitored with EMG while the skin surface is stimulated in the area of
the SLN. Transcranial magnetic stimulation over the mastoid can produce an action potential in the RLN as it emits from the skull,
allowing measurement of the muscle’s response latency in the TA muscle.
LARYNGEAL PARALYSIS AND
ASPIRATION
Vocal-fold paralysis alone, rarely results in aspiration unless the patient has a wide glottal gap.

When vocal-fold paralysis occurs in conjunction with other motor or sensory dysfunction, the combination may significantly impair the
complex timing of all levels of airway protection that occur during swallowing via closure of the laryngeal vestibule.

Evaluation of the patient with aspiration begins with a thorough history of neurological disorder or pulmonary status and physical
examination such as neurological examination.

Nonsurgical management of the patient with aspiration usually consists of either modifying oral intake or providing alternative methods of
alimentation. Short-term feeding may be provided by a nasogastric feeding tube.
Narrow-field laryngectomy remains the oldest, effective surgical treatments of aspiration. Tracheoesophageal puncture can be used to
restore phonation, but the patient rarely want to sacrifice their larynx. Other option has Tracheoesophageal diversion was developed by
Lindeman with the goal of controlling aspiration definitively while preserving the larynx and RLNs and thus the potential for reversal. In
this procedure, the trachea is divided at the level of the third tracheal ring
SPASMODIC DYSPHONIAS
The spasmodic dysphonias (SD) are notable during speech and include: adductor SD (uncontrolled closing of the vocal folds interrupting
voice during vowels), abductor SD (prolonged vocal fold opening after voiceless sounds extending into vowels), or vocal-fold tremor
(modulations in phonatory pitch and loudness most evident during prolonged vowels). These disorders are characterized by involuntary
changes in the ability to maintain voicing during speech either because of intermittent glottal catches (voice breaks) in the adductor type or
breathy breaks due to prolonged vocal fold abductions in the abductor type.

In many patients, speech is affected, singing is less affected and emotional expression (laughter and cry) and shout are unaffected. When
these disorders first affect a patient, the symptoms can be mild and intermittent. Typically, these disorders will worsen over a six to 24
month time interval.
SPASMODIC DYSPHONIAS
Characteristics include:

1. Symptoms are specific to a particular task, gesture or posture of the larynx (vowels and glottal stops in adductor SD, voice onset after
voiceless consonants in abductor SD.)

2. Symptoms are action induced, that is, they appear only with voluntary movement and are not usually apparent at rest.

3. Symptoms become worse with prolonged speaking, practice or performance.

4. Onset is usually gradual.

5. Reflexive and emotional aspects of voice function are una!ected, such as coughing, crying, shouting and laughter.

6. In professional voice users, symptoms may appear with heavy professional schedules or following injury.
• Diagnosis and Assessment

Diagnosis and management of SDs is best accomplished by a voice team including an otorhinolaryngologist, a speech-language pathologist,
a neurologist, and, for some patients, a psychiatrist.

Need to observing the vocal folds during speech and non-speech gestures, flexible laryngoscopy, A neurological examination is necessary, a
trial of voice therapy and a psychosocial interview may be needed to rule out other diagnosis

1. Speech Testing

Voice symptoms should be compared during three tasks to discriminate between adductor spasmodic dysphonia, abductor spasmodic
dysphonia and vocal tremor.
Speech Testing

A. Prolonged vowel phonation usually manifests vocal-fold tremor if it is present. Prolonged vowel production is a!ected only in the more
severe forms of adductor and abductor SD.

B. Production of sentences in which most sounds are voiced and frequent glottal stops at word boundaries, eg “We mow our lawn all year”
and “We eat eels everyday”, are usually most di"- cult and demonstrate frequent breaks or voice arrests in adductor spasmodic dysphonia.

C. Production of sentences with voiceless consonants (s, t, p, k, h), “She speaks pleasingly”, “Keep Tom at the party”, “When he comes
home we’ll feed him”, are usually most difficult in abductor spasmodic dysphonia. Sentences with predominantly voiced sounds are much
easier to produce and smoother for these patients
2. Electromyography

The laryngeal muscle activation abnormalities di!er greatly across patients and can account for the wide variety of symptoms. The
laryngeal muscles can be examined using laryngeal electromyography to determine which muscles contain spasms during speech.

By using an EMG electrode connected to an amplifier with a dual channel storage oscilloscope, one channel for the EMG and another for
the speech waveform.

For the TA and lateral cricoarytenoid (LCA) muscles, items to be examined for adductor spasmodic bursts concurrent with voice breaks
include: prolonged ee, repeated ee-ee-ee-ee, and all voiced sentences. When examining patients with abductor SD, while recording from
the CT or posterior cricoarytenoid, speech should include “see-see-see”, “pea-pea-pea”, “hehe-he”, “Kathy took a potato”, and “Keep Tom
at the party.”
• Treatment

Currently, the following treatments have been used for managing symptoms in adductor spasmodic dysphonia.

1. Voice Therapy

Voice therapy can assist only mildly a!ected patients but will not have long lasting effects and is not beneficial in moderate to severely
affected patients

2. Recurrent Laryngeal Nerve Section

The procedure resulted in an initial dramatic reduction or elimination of voice spasms, but symptoms recur in up to 64% of patients.

3. Recurrent Laryngeal Nerve Avulsion.

After mobilization of the ipsilateral thyroid lobe, all branches of the RLN are identified, traced, and avulsed from their muscular insertions
deep to the cricopharyngeus muscle. The total length of RLN removed averages 9 cm. Thus, an overall success rate of 78%
4. Selective Laryngeal Adductor Denervation Reinnervation.

This procedure involves bilateral section of the adductor RLN branches to the TA and LCA muscles while preserving the natural
innervation of the posterior cricoarytenoid muscle. In addition, the distal thyroarytenoid branches are reinnervated using branches of the
ansa cervicalis nerve. The aim of this directed reinnervation is to prevent unwanted reinnervation

5. Botulinum Toxin.

Significant voice improvement occurs in 90% of patients with adductor spasmodic dysphonia for up to three months. This is now
considered the gold standard for management of symptoms in adductor spasmodic dysphonia. The disadvantage of the unilateral injection
approach is that a larger dose of toxin must be administered and immobility of the injected fold often results. An advantage of the bilateral
approach is that much smaller dosages of botulinum toxin can be used, reducing the cost, and e!ective symptom control can be obtained
MUSCULAR-TENSION
DYSPHONIA
dysphonia resulting from increased muscular tension in the larynx and neck associated with:

1) palpably increased muscle tension in the paralaryngeal and suprahyoid muscles during phonation,

2) elevation of the larynx in the neck while speaking,

3) may have occurrence of an open posterior glottic chink between the arytenoid cartilages on phonation, and

4) variable degrees of mucosal changes such as vocal nodules or chronic laryngitis

MUSCULAR-TENSION
DYSPHONIA
Not infrequently patients with vocal fold tremor or SD adductor also present with muscle exercise dysphonia which makes the diagnosis
difficult to enforce. In some patients, muscle tension disappears after Botox injections.

In spasmodic dysphonia, the abnormality is intermittent depending on the movement being examined and whether it contains fast,
disorganized adductor or abductor movements causing intermittent sound pauses, vowels in SD adductor and prolonged voiceless
consonants in SD abductor. In benign essential tremor, these intermittent vocal pauses are regular, usually around 5 Hz and most easily
detected on vowels that are extended for 10 seconds.
PARADOXICAL VOCAL FOLD
MOVEMENT DISORDER
is the adduction of the vocal cords during the inspiratory-expiratory phase that results in impaired inspiratory airflow and produces a
stridor sound. normal vocal cord movement during speech, but experiencing difficulty breathing on inspiration during speech.

There are several forms of this disorder that can be categorized as:

1) idiopathic focal dystonia, or part of Meige's syndrome, when the respiratory obstruction persists while the patient is awake,

2) associated with or masquerading as asthma when the obstruction is episodic,

3) stridor due to exercise

4) psychogenic,

5) associated with laryngopharyngeal reflux.

Many patients with episodic PVFM improve after being educated about triggering events, some lifestyle changes, and speech therapy.
Patients with focal respiratory dystonia report that symptoms do not occur during sleep, but symptoms are often present during the day and
may be exacerbated by exercise and stress.

Patients with Meige's syndrome, an orofacial dyskinesia, may experience pharyngeal spasm, either by pharyngeal constriction or by
obstruction of the epiglottis. the symptoms are absent during sleep, almost always present during the day, but are exacerbated by stress.

Patients with chronic asthma may experience adduction of the laryngeal vocal cords during an asthma attack.

Gastroesophageal reflux is also a common cause of paroxysmal laryngospasm. Patients present with intermittent, sudden, noisy breathing
and short-term episodes of choking. These stridor attacks often occur after eating, after starting to exercise, or after bending over.
Sometimes it happens at night.
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