| Auditory manifestations of Keratitis-Ichthyosis-Deafness (KID) syndrome. | |
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MedLine Citation:
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PMID: 11889383 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVE: Evaluation of the auditory manifestations of Keratitis-Ichthyosis-Deafness (KID) syndrome, a rare genodermatosis characterized by follicular hyperkeratosis, vascularizing keratitis, and congenital hearing loss. STUDY DESIGN: Five individuals with sporadic KID syndrome were evaluated in the outpatient audiology clinic at the Warren Grant Magnuson Clinical Center of the National Institutes of Health. METHODS: Audiologic examinations included pure-tone audiometry, speech audiometry, and middle ear immittance testing. Auditory brainstem responses and otoacoustic emissions were analyzed in 2 subjects. RESULTS: Four subjects had prelingual, bilateral, profound sensorineural hearing loss, whereas the fifth subject had significant residual hearing that exhibited no progression on serial audiograms. All 5 subjects had a history of non-erosive keratosis obturans and cutaneous cysts in the external ear canals that prevented continuous use of ear molds. CONCLUSIONS: The sensorineural hearing loss in KID syndrome is generally prelingual and profound. This combination of auditory and cutaneous phenotypes is similar to those previously reported for KID syndrome. KID syndrome presents a difficult challenge for communication rehabilitation because keratitis may impair the perception of sign and spoken language, and the cutaneous manifestations routinely curtail use of external amplification devices. |
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Authors:
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Yvonne M Szymko-Bennett; Laura J Russell; Sherri J Bale; Andrew J Griffith |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: The Laryngoscope Volume: 112 ISSN: 0023-852X ISO Abbreviation: Laryngoscope Publication Date: 2002 Feb |
Date Detail:
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Created Date: 2002-03-12 Completed Date: 2002-03-27 Revised Date: 2007-11-14 |
Medline Journal Info:
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Nlm Unique ID: 8607378 Medline TA: Laryngoscope Country: United States |
Other Details:
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Languages: eng Pagination: 272-80 Citation Subset: IM |
Affiliation:
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National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland, USA. szymkoy@nidcd.nih.gov |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Audiometry / methods Child Cochlear Implants Deafness / complications, diagnosis, genetics, therapy Female Hearing Aids Hearing Loss, Sensorineural / complications, congenital*, diagnosis* Humans Ichthyosis / complications, diagnosis*, genetics Infant Keratitis / complications, diagnosis*, genetics Male Otolaryngology / instrumentation Prognosis Sampling Studies Syndrome |
| Grant Support | |
ID/Acronym/Agency:
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Z01 DC 00055-01/DC/NIDCD NIH HHS; Z01 DC00054-01/DC/NIDCD NIH HHS |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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