FAMILIAL HEARING LOSS ASSOCIATED WITH BRANCHIAL FISTULAS
Keyword(s):
A family is presented with cervical and preauricular branchial fistulas and congenital hearing loss. The hearing loss is unusual in having both conductive and sensorineural components. The basis of the conductive defect in the propositus was demonstrated at surgery to be a malformation of the ossicles. Hypoplasia of the mandible was present in two individuals. The syndrome was inherited as an autosomal dominant trait with variable expressivity and apparently incomplete penetrance. The occurrence of branchial fistulas should precipitate a search for defective hearing among relatives, especially in young children where early diagnosis and correction may permit the normal development of speech.
2009 ◽
Vol 118
(9)
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pp. 670-676
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1985 ◽
Vol 22
(3)
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pp. 623-627
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1982 ◽
Vol 9
(3)
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pp. 345-347
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1982 ◽
Vol 92
(9)
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pp. 1028???1037
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