Vocal Fold Paresis of Charcot-Marie-Tooth Disease

Abstract

No cohesive overview of vocal fold abnormalities associated with Charcot-Marie-Tooth disease (CMT) has been presented in the literature. This study examines a patient in depth and compares the findings with those of published reports to characterize the features of vocal fold paresis in CMT. The affected patient was investigated with nerve conduction testing, laryngeal electromyography, endoscopy, and laryngeal sensory testing. Ten published cases were reviewed for similarities and differences. Vocal fold paresis has been observed in 11 CMT patients ranging in age from 8 to 80 years. Two cases have occurred in the context of CMT type 1, and 9 in CMT type 2. Seven of the 11 cases (64%) were clearly bilateral; only 2 of the 7 cases (29%) required tracheotomy, and both were in children. The electromyographic findings were typical of reinnervation. Sensory findings were present, but did not represent significant disability in the 1 patient so studied. We conclude that CMT does not spare the cranial nerves, as has been previously thought. Furthermore, vocal fold paresis is not restricted to CMT type 2 and should not be considered a hallmark of that category. The available evidence suggests that the neural deficit evolves gradually, may exhibit partial recovery, and often escapes notice for a time. Vocal fold abnormalities are most often bilateral. Because the deficit is generally well tolerated in adults, many cases have probably been overlooked, and no conclusion regarding incidence is possible. Nevertheless, the potential for airway compromise exists, especially in children. Respiratory complaints of CMT patients should be thoroughly investigated.