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Hoarseness, caused by a change in vocal cord function, is generally benign and brief. Mitochondrial patients can become hoarse as they become tired since normal vocal cord function is energy-consuming. However, like patients with other neurologic conditions (such as multiple sclerosis and Parkinson disease), they may experience vocal cord paralysis.

In mitochondrial disease, the hoarseness may correlate with the degree of patient fatigue or weakness. Therefore as the day progresses (or with physical or emotional stress), the quality of the voice may change, becoming more hoarse. There may also be some degree of respiratory distress (tachypnea, "can't catch my breath") due to respiratory muscle weakness.

Other primary concerns like aspiration or reflux and swallowing dysfunction may also be associated with a secondary change in the character of the voice, perhaps with stridor.

Mitochondrial Differential Diagnosis

1. Vocal cord dysfunction:

a. Fatigue

b. Neurologic disease, causing paralysis

2. Problems with swallowing:

a. Fatigue

b. Motor incoordination

3. Problems with aspiration or gastroesophageal reflux.

Assessment and Recommendations

1. Determine what symptoms are associated with the hoarseness and whether or not there are any trigger factors, specifically problems with swallowing or possible aspiration.

2. Assess for stridor and/or respiratory distress.

3. Assess for reflux and delayed gastric emptying:

a. Does the patient have post-prandial pain? Is the patient unable to eat a large amount at one time?

b. Is the patient a grazer (perhaps suggesting that the patient can't tolerate easting big meals) or a meal-eater?


1. For gastroesophageal reflux:

  1. Gastroenterology referral as appropriate (particularly one knowledgeable in but motility issues):
  2. If reflux is present, a trial of anti-reflux medication should be considered.

2. For hoarseness, no matter what the cause:

  1. Refer to otolaryngologist;
  2. Refer to speech pathologist;
  3. Maintain good hydration;
  4. Use of a vaporizer at night;
  5. Avoidance of irritants such as smoke, alcohol, excessive air conditioning or heat, inhaled chemicals, cleaning agents or dust;
  6. Avoidance of excessive stress on the throat including throat-clearing, coughing, whispering;
  7. Avoidance of medications that may compromise the voice;
  8. Reflux laryngitis precautions (Tufts-NEMC, 2007) -
    1. Allow 3 hours between last big meal and going to bed.
    2. Sit upright for at least 1 hour after a meal.
    3. Eat slowly and consider smaller, more frequent meals.
    4. Keep the head of the bed up at a 30° angle or higher.
    5. Take anti-reflux medications.
    6. Avoid foods and drinks that could contribute to reflux laryngitis.
  9. Conserve the voice (Tufts-NEMC, 2007) -

1. Practice voice rest and pacing.

2. Limit talking in noisy places.

3. Touch-distance talking (talk to people only within touching distance).

4. Talk only when facing the listener. Use good posture and alignment.


Diamantopoulou P, Ward VM, Harries ML. Kearns-Sayre syndrome: Presenting with vocal cord palsy. J Laryngol Otol 2001;115(12):1021-2.

Feit H, Silbergleit A, Schneider LB, et al. Vocal cord and pharyngeal weakness with autosomal dominant distal myopathy: Clinical description and gene localization to 5q31. Am J Hum Genet 1998;63(6):1732-42.

Lin YC, Lee WT, Wang PJ, Shen YZ. Vocal cord paralysis and hypoventilation in a patient with suspected Leigh disease. Pediatr Neurol 1999;20(3):223-5.

Tufts-New England Medical Center (Tufts-NEMC) - Department of Speech-Language Pathology and Audiology. Voice care guidelines. 2007.

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