Neurologic Evaluation of the Larynx and Pharynx


Key Points

  • Evaluation of the larynx and pharynx is a multidisciplinary endeavor that may involve otolaryngologists, neurologists, speech-language pathologists, and radiologists.

  • Normal functions of the larynx include regulation of respiratory mechanics, airway protection during deglutition, and phonation.

  • Normal function of the pharynx includes maintenance of patency for respiration and effective constriction for swallowing with prevention of reflux into the nose or mouth.

  • Neurologic evaluation begins with observation of voluntary and involuntary movements and having the patient perform various phonatory tasks.

  • Flexible laryngoscopy and stroboscopy give information on vocal cord movement, compensatory mechanisms, mucosal wave and pliability, pharyngeal tone, and velopharyngeal competence.

  • Video recording of physical examinations facilitates later review and consultation with colleagues.

  • Acoustic analysis gives objective measures of fundamental frequency, perturbation, and spectral analysis.

  • Electromyography can be used to assess the integrity of a neural pathway and to facilitate the diagnosis of neurologic disease. It is useful to prognosticate the potential for recovery of motion in a patient with a fully immobile vocal fold, particularly those in whom the prognosis for recovery of motion is poor.

  • Modified barium swallow (MBS) and functional endoscopic evaluation of swallowing (FEES) can assess the efficacy and safety of the swallow. MBS is superior to FEES for assessing the oral and esophageal phases of swallowing.

  • Functional endoscopic evaluation of swallowing with sensory testing (FEESST) quantifies sensory loss and can give information for evaluating the site of a neurologic deficiency.

The upper aerodigestive tract serves the diverse purposes of breathing, eating, and communicating. These activities require some orthogonal functions. For example, pharyngeal patency should be maintained during respiration, but the pharynx should be forcibly constricted during swallowing. In addition, the anatomic structure of the upper aerodigestive tract is precarious, with ingested food and inspired air traversing the same space. Precise coordination of motor activity and appropriate response to sensory feedback are essential for normal function. Neurologic disorders can impair upper aerodigestive tract function by diverse mechanisms, including motor weakness, incoordination, and impairment of sensation.

The diagnosis of disorders in patients with neurologically impaired function of the upper aerodigestive tract is often elusive, particularly when the problem is isolated to that region. The reason is that the ability of the throat and larynx to function cannot be directly observed. Patients with complaints of hoarseness, dysarthria, dysphagia, aspiration, or airway obstruction are often seen by otolaryngologists, whose training emphasizes morphologic assessment rather than functional evaluation; neurologists, whose forte is functional evaluation, generally confine their evaluations to more accessible structures, such as limbs. If the anatomy is normal, and if no functional deficit is noted on a traditional neurologic examination, a patient who actually has a neurologic impairment is often incorrectly assumed to have a functional or psychiatric disorder. Thus, the diagnosis of a generalized neurologic disorder, such as myasthenia gravis or amyotrophic lateral sclerosis (ALS), may be delayed until the disease process is more widespread and apparent. As a result, patients may receive well-intentioned but ineffective therapy, instead of potentially effective treatment.

In patients whose neurologic diagnosis has been established, assessment of laryngeal and pharyngeal function is an important component and may be lifesaving. Optimal treatment and rehabilitation of patients with swallowing, speech, or phonation disorders require identification of the precise pathophysiology of the problem so that appropriate therapy may be designed.

The diagnosis of neurologic impairment of the upper airway requires an awareness of the possibility of neural dysfunction, familiarity with the signs and symptoms of neural dysfunction, and a systematic approach to the examination of the throat and pharynx. Box 56.1 lists some symptoms that suggest neurologic impairment. The patient's interests are best served by the collaborative efforts of the otolaryngologist, who is highly skilled in visualizing the throat and larynx, and the neurologist, who is knowledgeable in the pathophysiologic processes. This chapter reviews normal physiology and salient features of specific neurologic diseases that affect function, outlines the approach to the history and physical examination of patients in whom such diseases are suspected, and discusses how ancillary tests may be useful.

Box 56.1
Laryngeal and Pharyngeal Symptoms That Suggest Neuropathology

Speech Problems

  • Lack of volume

  • Breathiness

  • Instability of pitch or volume

  • Lack of voice inflection

  • Abnormal resonance

  • Dysarthria

Swallowing Problems

  • Oral incompetence

  • Velopharyngeal incompetence

  • Inability to initiate a swallow

  • Aspiration

Breathing Abnormalities

  • Fluctuating inspiratory stridor

  • Weak, breathy cough

  • “Gurgly” breathing noises

Normal Function

Phylogenetically, the most important function of the larynx is protective; the larynx prevents ingested food and drink from entering the lungs. To fulfill this role in humans, the larynx should be open during breathing and tightly closed during swallowing. Phonation is a more advanced evolutionary function. Glottic closure during forced exhalation results in an effective cough to clean the lungs and prevent atelectasis. Laryngeal closure is required to generate positive intrathoracic pressure for defecation, childbirth, and lifting heavy objects and for the stabilization of the thorax. The larynx also seems to play a sophisticated role in controlling airflow and pressure during breathing. The vocal folds open just before respiration and then close gradually during exhalation; thus, they act as a brake on expiratory airflow and thereby influence the rate of breathing. The sensory feedback loops that control these respiratory functions of the larynx are not well understood, but it is clear that feedback from a variety of receptors is involved.

Swallowing is a deceptively simple function. Food prepared in the mouth is ejected into the pharynx and then rapidly propelled downward around the glottis, through the piriform sinus, and into the esophagus. During the swallow, the same pharynx that remains patent during breathing is responsible for constricting tightly in an organized sequence from top to bottom. Regurgitation into the nose or reflux into the mouth is prevented by actions of the soft palate, tonsillar pillars, and base of the tongue. The larynx is pulled upward and forward, away from the flow of the bolus; this action also decompresses the sphincter between the pharynx and esophagus, and flexion of the epiglottis and closure of the glottis prevent the entry of any errant material into the airway. After this complex and coordinated activity, when the bolus reaches the caudal end of the pharynx, the cricopharyngeal muscle should be relaxed to permit its passage into the esophagus; if pharyngeal peristalsis is inadequate, or if the upper esophageal sphincter is insufficiently opened, residual material in the pharynx could penetrate the airway during the next inhalation. The unique anatomic configuration in humans after infancy renders swallowing more difficult. In all other mammals, the feeding and breathing channels are offered a degree of separation by interdigitation of the epiglottis and uvula. In humans, these structures are separated during early development by the descent of the larynx relative to the palate.

Speech is an audible communication that results from phonation, resonance, and articulation. The production of sound, or phonation, requires several conditions: expired airflow and pressure should be sufficient to induce oscillation of the vocal folds, which should be appropriately approximated; if the folds are closed tightly, excessive expiratory force is required and results in a strained, harsh voice or in complete aphonia; if the vocal folds are too far apart, increased expiratory airflow volume is required, such that the voice becomes weaker and breathier or even fades to a whisper. The three-dimensional shape of the vocal fold is also important in imparting favorable aerodynamic features to the glottis. Atrophy of the vocal fold causes concavity in the axial and coronal planes; concavity in the axial plane results in incomplete glottal closure, even during tight approximation of the vocal processes, whereas concavity in the coronal plane results in a convergent airflow tract. Control of length and tension is required to produce normal inflections in pitch and tone. In the absence of such control, the voice may be flat and expressionless, or it may be distorted by uncontrolled pitch breaks. The mucosa of the vocal folds should be supple to permit free vibration. All but the last of these requirements for phonation are susceptible to derangement by neurologic dysfunction.

Resonance is the modification of phonation to produce voice. Pure phonated sound as produced by the vocal folds does not sound like a human voice, rather it is a strident and unpleasant noise. This sound is modified by resonance of the head, neck, and chest so that component frequencies are selectively amplified or dampened. Vocal resonance is largely determined by anatomy, but it is also significantly modulated by motor activity of the pharynx, soft palate, and oral cavity. This principle may be used in speech therapy to achieve stronger voices in patients with impaired function. Certain neurologic disorders such as ALS, stroke, and Guillain-Barré syndrome result in altered vocal resonance. The characteristic sound of the voice in patients with these disorders may be a valuable physical sign to aid in early diagnosis.

Articulation is the shaping of voice into words by actions of the lips, tongue, palate, pharynx, and larynx, and it is highly susceptible to neurologic impairment. Whereas dysarthria in children is caused most often by a hearing deficit, acquired dysarthria in adults usually indicates neurologic impairment. Dysarthria may be a result of impaired motor output—such as weakness, paralysis, or incoordination—but it may also result from cognitive or language defects or from apraxia of speech.

Neurologic Examination of the Mouth, Larynx, and Pharynx

Listening to the patient is the first step of the examination in the patient with a voice complaint. It is often possible to make a working diagnosis within minutes on the basis of the sound of the voice and the patient's description of the problem. The patient should be questioned carefully about vocal fatigue, pain with speaking, increased effort required for speech, glottal tightness, pitch breaks, and tremor.

The following protocol is suggested as a systematic approach to the detection of neurologic dysfunction and has been summarized for quick reference in Table 56.1 . The objectives are to assess the integrity of the lower cranial nerves and to seek signs of central nervous system disorders.

TABLE 56.1
Summary of the Approach to the Patient With Neuropathology of the Larynx and Pharynx
1. E xamination
a. Oral Cavity—Lips/Hard Palate/Tongue

  • Assess motion, strength, and symmetry

    • Athetoid movements suggest tardive dyskinesia

    • Fasciculations of the tongue suggest amyotrophic lateral sclerosis

    • Jaw and tongue thrusting suggest oromandibular dystonia

b. Oropharynx—Soft Palate

  • Assess motion, strength, and symmetry

    • Slow, repetitive jerking suggests palatal myoclonus

2. F lexible L aryngoscopy and S troboscopy
Soft palate “Kitty cat” or /ka/ tests Assesses velopharyngeal closure
Pharynx Pooling of secretions Suggests poor swallowing function
Larynx Tremor at rest Suggests essential tremor
Repeat sniff and /ee/ May unmark paresis
Pitch glide Assesses cricothyroid function
“We eat eggs every Easter” or count from 80 to 90 Adductor spasmodic dysphonia
“Harry's happy hat” or count from 60 to 70 Abductor spasmodic dysphonia
3. A ncillary T esting
a. Voice Handicap Index (VHI)—10
b. Acoustic Analysis

  • Breaks on adductor of abductor specific vowels suggest spasmodic dysphonia

4. P erformance M easures and I nvestigations
a. Aerodynamic Assessment
b. Glottography
c. Electromyography (EMG)

  • If cricothyroid function absent suggests vagus nerve injury

  • If cricothyroid function intact suggests recurrent laryngeal nerve injury

  • Presence of giant waves and fibrillation potentials suggest nerve denervation

  • Fatiguing muscle recruitment suggests myasthenia gravis

  • Regular 4–8 Hz signal suggests essential tremor

d. Cineradiography—Modified Barium Swallow (MBS)
e. Functional Endoscopic Evaluation of Swallowing (FEES)
f. Functional Endoscopic Evaluation of Swallowing with Sensory Testing (FEESST)

  • ≤3 mm Hg indicates normal laryngeal sensation

  • ≥6 and <9 mm Hg indicate mild sensory impairment

  • 9 mm Hg indicates moderate sensory impairment

  • >9 mm Hg indicates severe impairment

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