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Audiologic evaluation in Charcot-Marie-Tooth disease.
H Pérez1, J Vílchez, T Sevilla
1Department of Otolaryngology and Neurology, Hospital La Fe, Valencia, Spain.
Scandinavian Audiology. Supplementum
|January 1, 1988
Summary
Hearing loss affects 28% of patients with hereditary motor and sensory neuropathy (Charcot-Marie Tooth disease). Abnormalities are linked to the VIIIth nerve and brainstem, with no difference between disease types.
Area of Science:
- Neurology
- Audiology
- Genetics
Background:
- Hereditary motor and sensory neuropathy, also known as Charcot-Marie Tooth disease, is a group of inherited disorders.
- These disorders affect peripheral nerves, leading to muscle weakness and sensory loss.
- Hearing impairment is not a commonly recognized symptom of Charcot-Marie Tooth disease.
Purpose of the Study:
- To investigate the prevalence and characteristics of hearing loss in patients with Charcot-Marie Tooth disease.
- To determine if hearing abnormalities are associated with specific types or aspects of the disease.
Main Methods:
- Pure tone audiometry was used to assess hearing thresholds.
- Auditory brainstem response (ABR) testing was performed to evaluate neural pathway function.
- 39 patients diagnosed with Charcot-Marie Tooth disease participated in the study.
Main Results:
- A significant percentage of patients (28%) exhibited hearing deficiency.
- Hearing abnormalities were attributed to potential disorders of the VIIIth cranial nerve and brainstem.
- No significant differences in hearing outcomes were observed between Type I and Type II Charcot-Marie Tooth disease patients.
Conclusions:
- Hearing impairment is a notable comorbidity in a subset of Charcot-Marie Tooth disease patients.
- The VIIIth nerve and brainstem are likely affected areas contributing to hearing loss in this population.
- Disease classification (Type I vs. Type II) does not appear to influence the presence or severity of hearing deficits.