Spasmodic Dysphonia and other related disorders.

What are the various types of spasmodic dysphonia? How will you manage a case of adductor dysphonia? (10) June 2013.

Spasmodic Dysphonia

• Focal task-specific laryngeal dystonia with spasmodic contractions of internal laryngeal musculat ure .
• Adult onset disorder with a female predominance.
• Unknown pathophysiology.
  • Site of pathology involves the basal ganglia .
• 25% of patients with SD report family history of dystonia or other movement disorders.
• Associated with:
  • Essential tremor (30%)
  • Blepharospasm (15%)
  • Writer’s cramp (15%)
• SD affects the speaking voice only.
  • Spares other vocal tasks such as singing or yelling.
  • Spares non-speech laryngeal functions such as breathing, swallowing, or coughing .
• Symptoms exacerbated by:
  • Anxiety.
  • Stress.
  • Public speaking .
• Symptoms suppressed by:
  • Alcohol
  • Sedatives
  • Sensory trick (Geste antagonist) such as:
    • Voicing while chewing.
    • Voicing while biting the tongue.
    • Holding a finger in the comer of the mouth.
  • Insertion of a flexible scope .

• Sometimes it is difficult to distinguish organic spasmodic dysphonia from psychogenic voice disorders or muscle tension dysphonia .

Types of Spasmodic Dysphonia:

ADducted SD

ABducted SD

Mixed SD (Rare)

ADductor Spasmodic Dysphonia (ADdSD):

• Most common type of SD (8 5% ) .
  • Causes vocal fold hyper-ADduct ion :
    • Phonation against a closed glottis.
    • Sudden and strong contraction of ThyroArytenoid muscle.
  • Voice Profile:
    • Harsh strained voice.
    • Strangled quality.
    • Voice breaks  in connected speech.
    • Prominent with words begin with vowels.
      • Counting from 80 to 90.
      • “We eat eggs every day”

• Diagnosis:
  • Flexible Nasolaryngoscopy during connected speech :
    • Hyper-ADduction of vocal folds with hyperfunction of Supraglott is.
  • Stroboscopy.
  • High Speed Camera :
    • Best tool to diagnose SD.

• Treatment:
  • Voice therapy .
  • Botulinum toxin (Botox) injections:
    • Treatment of choice for SD.
    • 90% improvement.
    • Injection of ThyroArytenoid (TA) and Lateral CricoArytenoid (LCA) muscles.
    • Done transcutaneously under LEMG guidance.
    • 0.05-10 U of Botox (average of 1 U) is injected to bilateral TA muscles.
    • Effect lasts for 3 months.
    • Side Eff ect s:
      • Excessive glottal weakness and breathiness.
      • Liquid dysphagia if toxin diffused to the adjacent constrictors .
  • Surgical therapy :
    • Destruction of RLN branches.
    • Resection dystonic musrulature .
    • Type II Thyroplasty
    • Reinnervation of dystonic musculature with a branch of Ansacervicalis.

How to give EMG Guided Botox Injection for Spasmodic Dysphonia

ABductor Spasmodic Dysphonia (AbdSD):

• Less common than AddSD (1 5 % ) .
• Causes vocal fold hyper-ABduction:
  • Phonation against a opened glottis.
  • Sudden and strong contraction of Posterior CricoArytenoid muscle.
• Voice Profile:
  • Abnormal whispered or sustained breathiness with breathy voice breaks during phonation.
  • Especially during voice onset
  • Prominent in vowels following a voiceless consonant :
    • Counting from 60 to 70
    • “The puppy bit the tape”

• Diagnosis:
  • Flexible Nasolaryngoscopy during connected speech:
    • Inappropriate vocal fold ABduction during connected speech.
  • Stroboscopy.
  • High Speed Camera:
    • Best tool to diagnose SD.

• Treatment:
  • Voice therapy.
  • Botulinum toxin (Botox) injections:
    • Treatment of choice for SD.
    • 90% improvement.
    • Injection of Posterior CricoArytenoid (PCA) muscle.
    • Done transcutaneously under LEMG guidance.
    • 2-5 U of Botox is injected to unilateral PCA muscle.
    • Lasts for 3 months.
    • Side Effects:
      • Stridor with airway compromise.
      • Liquid dysphagia if toxin diffused to the adjacent constrictors

Other Questions related to Spasmodic Dysphonia

Essential Tremor:

• Common, benign and inherited movement disorder.
• Female predominance.
• Most common presentation:
  • Shaking of the hands and rhythmic head t it ubat ion .
• 30% of patients will have symptomatic vocal involvement.
  • Tremor in muscles of larynx, pharynx, soft palate and the strap muscles of the neck.
• Clinical picture:
  • Steady shaking voice.
  • Ranging from gentle and continuous to a staccato, almost hiccuping sound.
  • Tremor is rhythmic and steady at 5-7 cycles per second.
  • Occurs in all speech contexts.
  • Symptoms exacerbated by:
    • Anxiety.
    • Stress.
    • Public speaking .
  • Symptoms suppressed by:
    • Alcohol
    • Sedatives
• Essential tremor differs from spasmodic dysphonia by:
  • Its rhythmicity.
  • Present across all speech tasks.
• Treatment:
  • Voice therapy.
  • Medical Management:
    • Beta-blocker (Propranolol)
    • Anti-epileptic (Primidone)
  • Botulinum toxin (Botox) injections:
    • Useful to “dampen” the tremor by weakening the affected musculature, but oscillatory movements will persist at diminished amplitude.
    • Will not eliminate the tremor.
    • Botox is injected into ThyroArytenoid muscle within the vocal fold.
      • TA is responsible for the strength of the staccato, hiccuping effect of the tremor.

Tic Disorders:

• Tics are sudden, recurrent, quick, abnormal movements or vocalizations that abruptly interrupt normal activity.
• There is a compulsion to perform a movement, which is relieved once the movement is completed.
• Mainly affects children with male predominance.
  • Up to 30% of children may have transient tics.
• Many adults have unrecognized simple tics including:
  • Throat clearing
  • Snores
  • Squeals
  • Coughs
  • Belching noises.
• Symptoms exacerbated by:
  • Anxiety.
  • Stress .
  • Public speaking.
• Symptoms suppressed by:
  • Alcohol
  • Sedatives
• Treatment:
  • Voice therapy.
  • Medical Management:
    • Tetra benazine
    • Clonazepam
  • Botulinum toxin (Botox) injections .

Ocuopalatolaryngopharyngeal Myoclonus:

• Uncommon disorder consisting of twitch-like, rhythmic contractions of soft palate, pharynx and larynx .
• Occurs at a rate of 1-2 contractions per second.
• May affect only the palate, or all of the laryngopharynx.
• Causes:
  • Part of a seizure disorder
  • Posttraumatic
  • Viral, toxic, or metabolic encephalopathy.
• Clinical picture:
  • Choppy speech
  • Intermittent hypernasality from palatal dysfunction.
  • Persistent tinnitus (clicking) in the ear.
  • ET dysfunction .
• Treatment:
  • Anti-epileptic medications.
  • Botulinum toxin (Botox) injections.
    • Injections of palate and vocal folds to decrease severity of the contractions
    • Will not improve ET dysfunction and velopharyngeal insufficiency .

Dysphonia caused by other neuroogical disorder:

• Examples:
  • Stroke
  • Multiple Sclerosis
  • Bulbar Palsy
  • Poliomyelitis
  • Guillain-Barre Syndrome
  • Myasthenia Gravis
  • Amyotrophic Lateral Sclerosis
  • Fried reich Ataxia
  • Arnold-Chiari Malformations
  • Parkinson’s disease

Functional Speech disorders (Muscle Tension Dysphonia):

Voice disturbance without structural or neurologic laryngeal pathology. Excessive tension in intrinsic and/or extrinsic laryngeal muscles.

Examples:

• Ventricular Phonation (Plica ventricularis):
  • Voice is produced by ventricular folds (false vocal folds) which have taken over the function of true vocal folds.
  • Diagnosis is made on Endoscopic laryngoscopy:
    • False vocal folds are seen to approximate partially or completely and obscure the view of true cords on phonation.
• Pharyngeal Constriction:
  • Pharyngeal muscles contract excessively while talking, leaving the pharynx very constricted .
• Hyper Abduction.
• Hyper Adduction.

Causes:

• Primary (Normal larynx):
  • Prolonged voice overuse:
    • Teachers
    • Singers and actors
    • People talking on the telephone all day.
  • Learned adaptations after URTI.
  • Increased pharyngolaryngeal tone secondary to LPR.
  • Psychologic.
• Secondary (Abnormal Larynx):
  • Extreme compensation for minor glottic insufficiency and/or underlying mucosal disease.

Clinical picture:

• Breathy or harsh voice with use through the day and recovers with rest.
• Vocal fatigue and st rain .
• Organic changes in vocal cords may occur secondary to such faulty use or overloading.

Management:

• Voice therapy .
• Psychological counseling if needed.

Psychogenic Speech Disorders:

Conversion Dysphonia/ Aphonia:

• Functional disorder mostly seen in emotionally labile females in the age group of 15-30.
• Exists when there is psychological trauma or conflict that is manifested physically.
  • Accident
  • Death
  • Psychologically damaging event
• Clinical picture:
  • Breathy-hoarse dysphonia with a normal laryngeal exam.
  • Aphonia is usually sudden and unaccompanied by other laryngeal symptoms.
  • Patient communicates with whisper.
  • On examination of patient with Conversion aphonia:
    • Vocal folds are seen in ABducted position.
    • Fail to ADduct on phonation.
    • Normal cough voice and with normal vocal folds ADduction during coughing.
• Management:
  • Voice therapy.
  • Psychological counseling.

Puberphonia {Mutationa Fasetto Voice):

• Persistence of childhood high-pitched voice.
• More common in adolescent males, less common in females.
  • Occurs during puberty in boys who are emotionally immature, feel insecure and show excessive fixation to their mother.
  • Psychologically, they shun to assume male responsibilities though their physical and sexual development is normal.
• Pathophysiology:
  • Normally, childhood voice has a higher pitch.
  • When the larynx matures at puberty, vocal folds lengthen, and voice changes to lower pitch.
  • This is a feature exclusive to males.
• Clinical picture:
  • Recurrent “cracked,” shrill, high-pitched voice.
  • Gutzmann’s pressure test:
    • Pressing thyroid prominence in a backward and downward direction relaxes the overstretched folds and low tone voice can be produced.
• Management:
  • Voice therapy.
  • Psychological counseling.

Paradoxica Vocal Fold Motion Disorder (Vocal fold Dysfunction):

• Attacks of stridor in which true vocal folds paradoxically ADduct during inspiration.
• Seen in young females.
• Very common among asthmatics.
• Causes:
  • Organic (Neurogenic):
    • Stroke
    • Multiple Sclerosis
    • Myasthenia Gravis
    • Amyotrophic Lateral Sclerosis
  • Psychogenic (Conversion or Somatization):
    • History of stressful periods (unconsciously and without intentional gain).
• Clinical picture:
  • Easily misdiagnosed.
  • Frequent, episodic attacks of:
    • Cough
    • Inspiratory/expiratory wheeze
    • Dyspnea with/without exertion
    • Stridor
    • Hoarseness
• Diagnosis:
  • Laryngoscopic examination:
    • Paradoxical vocal folds motion (inspiratory anterior vocal cord closure with posterior chinking).
  • Pulmonary Function Test (PFT):
    • PFT with a flow-volume loop is used to confirm vocal fold dysfunction and differentiate it from Asthma.
    • Flow-volume loop include forced inspiratory and expiratory maneuvers.
      • Extra-thoracic upper airway obstruction:
        • Normal expiratory loop.
        • Flat inspiratory loop.
      • Asthma:
        • Both expiratory and inspiratory loop are diminished but with predominant prolongation of expiratory loop.

• Management:
• Acute Management:
  • Heliox:
    • Gaseous mixture of oxygen and helium .
    • Found in ratios of 20/80 and 30/70.
    • Less dense than air.
    • Inhalation reduces turbulence in the airway and eliminates respiratory noise.
    • Reduces anxiety that is the predisposing factor to many attacks.
  • Intermittent or continuous positive airway pressure:
    • Widen Rima glottidis.
  • Benzodiazepines:
    • Reduce anxiety.
  • Intralaryngeal injection of Botox:
    • Recommended in severe cases of PVCM.
• Chronic Management:
  • Voice therapy.
  • Psychological counseling.

Additional Resourses

Understanding Spasmodic Dysphonia

Surgical Procedures for Abductor and Adductor Spasmodic Dysphonia

Voice Therapy for Spasmodic Dysphonia