CMT 1: Congenital bilateral vocal fold paralysis

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Congenital bilateral vocal fold paralysis and Charcot-Marie-Tooth disease.

AF, Carron JD, Vedanarayanan V.

Department of Otolaryngology and Communicative Sciences, University of

Mississippi Medical Center, , Mississippi 39206, USA.

We present the case of a patient with Charcot-Marie-Tooth disease (CMT) type 1

with congenital bilateral vocal fold paralysis in order to emphasize the

treatment options and long-term outcome. The case is reviewed with regard to

presentation, differential diagnosis, and treatment.

We also reviewed the literature to determine the frequency of congenital and

childhood presentations of bilateral vocal fold paralysis associated with CMT,

most specifically CMT type 1.

We found only 14 children reported to have bilateral vocal fold paralysis

associated with CMT, and only 1 of these cases was associated with CMT type 1.

None of these patients had congenital vocal fold paralysis.

Because of the degenerative nature of the disease, our patient underwent

endoscopic cordotomy to avoid tracheotomy. We conclude that CMT should be

included in the differential diagnosis in evaluating neonates with bilateral

vocal fold paralysis. If CMT is definitively diagnosed, it could alter the

course of treatment.