Major characteristics of this disorder are a wide space between the eyes, a flat nasal space, and eyebrows that connect together. Quite often, there is a variability of the color between the eyes. The hair color usually has a white forelock. At least 20% have some degree of sensorineural hearing loss. Waardenburg syndrome may account for approximately 2% of the congenitally deaf. The incidence may be higher, since only about 50% of the effected individuals have pigmentation abnormalities.

There appears to be two types of hearing loss.

Total deafness, with very little residual hearing at the low frequencies; and

Moderate hearing loss, which is flat for the low and middle frequencies but improves for the higher frequencies.

There is no evidence of progression in the hearing loss over time.

 

Vestibular Systems

Vary from complete loss of vestibular function to normal.

Hereditary

Autosomal dominant transmission with variable expression.

 

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