Document Detail


Hearing loss in Townes-Brocks syndrome.
MedLine Citation:
PMID:  8084622     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Townes-Brocks syndrome is an autosomal dominant syndrome consisting of anomalies affecting the ear, hand, foot, anus, and kidney. Anomalies affecting the ear include lop ear, preauricular skin tags, ossicular abnormalities, and a mixed hearing loss. The hearing loss in Townes-Brocks syndrome is predominantly sensorineural, affects high-frequency thresholds more than low-frequency thresholds, and has a variable (usually small) conductive component. The sensorineural component of the hearing loss is slowly progressive. It is typically in the mild range (20 to 40 dB hearing level) during early childhood and progresses to the moderate hearing loss range (40 to 60 dB hearing level) by early adulthood. We present a description of the otologic manifestations and an analysis of audiologic findings in six members of a family with Townes-Brocks syndrome.
Authors:
D R Rossmiller; T R Pasic
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery     Volume:  111     ISSN:  0194-5998     ISO Abbreviation:  Otolaryngol Head Neck Surg     Publication Date:  1994 Sep 
Date Detail:
Created Date:  1994-10-10     Completed Date:  1994-10-10     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  8508176     Medline TA:  Otolaryngol Head Neck Surg     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  175-80     Citation Subset:  IM    
Affiliation:
Division of Otolaryngology-Head and Neck Surgery, School of Medicine, University of Wisconsin.
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple
Adult
Anus, Imperforate / genetics
Audiometry, Pure-Tone
Auditory Threshold / physiology
Child
Child, Preschool
Ear, External / abnormalities*
Female
Hearing Loss / genetics*
Hearing Loss, Conductive / genetics
Hearing Loss, High-Frequency / genetics
Hearing Loss, Sensorineural / genetics
Humans
Male
Middle Aged
Syndrome
Thumb / abnormalities
Toes / abnormalities

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


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