Neurological Voice Disorders:
Voice problems caused by abnormal control, coordination, or strength of voice box muscles due to an underlying neurological disease such as: stroke, Parkinson’s disease, multiple sclerosis, myasthenia gravis, or ALS
Difficulty forming words – presenting with imprecise consonants and hard-to-understand speech as seen with stroke patients
Malfunction of the tongue and/or lip muscles resulting in garbled words or parts or words.
What are the typical symptoms of voice dysfunction in patients with ALS?
ALS is a progressive neurological disease that may present initially with speech/voice difficulties as the primary symptoms in up to 10 to 15 percent of patients, including:
- Spastic/strained voice
- Slurred speech
- Hypernasal voice
Eventually, other symptoms, such as weakness and spasms of the limbs, will appear and predominate. Most patients, however, notice weakness or non-coordination in their arms or legs before they begin to have vocal changes.
What is the cause of the disease?
Who is at risk?
- The cause of ALS is unknown and no risk factors have been identified in most patients.
- A small percentage (5 to 10 percent) of cases run in families.
- The average age of onset is between 50 to 60 years of age.
How is ALS diagnosed?
A careful history and physical examination by a neurologist, along with blood tests and X-rays to rule out other (rare) causes, will suffice in making the diagnosis.
Difficulties in Diagnosis: Challenges in Identifying ALS
- Early stages of ALS can easily be missed. Vocal difficulties associated with ALS can mimic spasmodic dysphonia (if vocal spasms predominate) or vocal fold paresis (if vocal fold weakness and flaccid symptoms predominate). Patients who have only voice/speech problems may be sent to an otolaryngologist for care.
- Typically, within weeks to months ALS progresses to involve the arms/legs, at which point the diagnosis becomes more obvious. A consultation with a neurologist should result in a correct diagnosis.
How is ALS treated and what is the prognosis?
- There is no cure for ALS – only supportive care.
- The disease is variable, but half of ALS patients die from respiratory failure within three years of onset of symptoms.
- With some slowly progressive forms of the disease, patients may live for 10 years. In these select cases, an otolaryngologist may perform the following:
- Injections of Botulinum toxin, type A into the vocal folds, to relieve vocal spasms
- Framework surgery that brings the vocal folds together to improve vocal fold vibration during sound production (medialization laryngoplasty), to relieve a breathy voice
Patient education material presented here does not substitute for medical consultation or examination, nor is this material intended to provide advice on the medical treatment appropriate to any specific circumstances.
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