Document Detail


Audiometric findings in 34 patients with Waardenburg's syndrome.
MedLine Citation:
PMID:  408454     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The dominant hereditary form of deafness as part of Waardenburg's syndrome was found in 12 (35%) of 34 patients never previously described with Waardenburg's syndrome among 5 families in the Netherlands. Extensive audiometric examination could be performed in 11 patients, 5 patients with deafness on both sides and 6 patients with hearing deficiency on one side. The hearing loss could be divided into 4 types: (type I) (sub) total deafness on both sides; (type II) serious lack of hearing on both sides; (type III) (sub) total deafness on one side; (type IV) moderate hearing deficiency on one side, particularly in the low frequencies. An extensive review was made of the more than 1,000 patients with Waardenburg's syndrome described in the literature. This revealed that deafness in both ears (the most serious expression of the syndrome) occurred in about 25% of the patients with Waardenburg's syndrome type I (including dystopia canthorum) and in 50% of the patients with Waardenburg's syndrome type II (without dystopia canthorum). This striking difference is connected with the close relationship between deafness and pigmentary disorders.
Authors:
M J Hageman
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Publication Detail:
Type:  Comparative Study; Journal Article    
Journal Detail:
Title:  The Journal of laryngology and otology     Volume:  91     ISSN:  0022-2151     ISO Abbreviation:  J Laryngol Otol     Publication Date:  1977 Jul 
Date Detail:
Created Date:  1977-10-20     Completed Date:  1977-10-20     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8706896     Medline TA:  J Laryngol Otol     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  575-84     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / complications*
Adolescent
Adult
Aged
Audiometry*
Child
Child, Preschool
Deafness / congenital,  diagnosis*
Eye Abnormalities
Female
Humans
Infant
Male
Middle Aged
Nose Deformities, Acquired
Waardenburg's Syndrome / complications*,  diagnosis,  genetics

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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