Document Detail


Audiological and vestibular findings in the Kabuki syndrome.
MedLine Citation:
PMID:  19161135     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Since the first description of Kabuki syndrome (KS) in 1981, over 350 cases from a variety of countries have been reported. Even though otolaryngological manifestations are common in KS, only a limited number of the reports provide audiological and vestibular data. The aim of the present study was to investigate the vestibular function and describe the audiological findings in KS. The present study reports no audiological and vestibular features in a group of 10 KS patients (7 males, 3 females), with chronological age ranging from 10 to 25 years (mean age = 14.5): a complete otoneurological and audiological work-up was performed for each patient and included where possible, the measurement of vestibular evoked potentials, caloric tests and static posturography. Hearing loss was found in 65% showing a mix or a conductive impairment; moreover the vestibular function was normal in 95% of the examined ears. In conclusion, audiological and vestibular examination should be considered when evaluating KS subjects.
Authors:
S Barozzi; F Di Berardino; F Atzeri; E Filipponi; M Cerutti; A Selicorni; A Cesarani
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  American journal of medical genetics. Part A     Volume:  149A     ISSN:  1552-4833     ISO Abbreviation:  Am. J. Med. Genet. A     Publication Date:  2009 Feb 
Date Detail:
Created Date:  2009-01-28     Completed Date:  2009-04-06     Revised Date:  2009-04-17    
Medline Journal Info:
Nlm Unique ID:  101235741     Medline TA:  Am J Med Genet A     Country:  United States    
Other Details:
Languages:  eng     Pagination:  171-6     Citation Subset:  IM    
Copyright Information:
(c) 2009 Wiley-Liss, Inc.
Affiliation:
Audiology Unit, IRCCS Fondazione Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Department of Otolaryngology, University of Milan, Milan, Italy. stefania.barozzi@unimi.it
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / diagnosis*
Adolescent
Adult
Caloric Tests
Child
Evoked Potentials, Auditory
Face / abnormalities
Female
Hearing Loss
Humans
Male
Syndrome
Vestibular Diseases / diagnosis
Vestibular Function Tests
Young Adult

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