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Autor/inn/enVan Eynde, Charlotte; Swillen, Ann; Lambeens, Elien; Verhaert, Nicolas; Desloovere, Christian; Luts, Heleen; Vander Poorten, Vincent; Devriendt, Koenraad; Hens, Greet
TitelPrevalence and Nature of Hearing Loss in 22q11.2 Deletion Syndrome
QuelleIn: Journal of Speech, Language, and Hearing Research, 59 (2016) 3, S.583-589 (7 Seiten)
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Spracheenglisch
Dokumenttypgedruckt; online; Zeitschriftenaufsatz
ISSN1092-4388
DOI10.1044/2015_JSLHR-H-15-0098
SchlagwörterHearing Impairments; Severity (of Disability); Genetic Disorders; Audiology; Acoustics; Age Differences; Incidence; Symptoms (Individual Disorders)
AbstractPurpose: The purpose of this study was to clarify the prevalence, type, severity, and age-dependency of hearing loss in 22q11.2 deletion syndrome. Method: Extensive audiological measurements were conducted in 40 persons with proven 22q11.2 deletion (aged 6-36 years). Besides air and bone conduction thresholds in the frequency range between 0.125 and 8.000 kHz, high-frequency thresholds up to 16.000 kHz were determined and tympanometry, acoustic reflex (AR) measurement, and distortion product otoacoustic emission (DPOAE) testing were performed. Results: Hearing loss was identified in 59% of the tested ears and was mainly conductive in nature. In addition, a high-frequency sensorineural hearing loss with down-sloping curve was found in the majority of patients. Aberrant tympanometric results were recorded in 39% of the ears. In 85% of ears with a Type A or C tympanometric peak, ARs were absent. A DPOAE response in at least 6 frequencies was present in only 23% of the ears with a hearing threshold =30 dB HL. In patients above 14 years of age, there was a significantly lower percentage of measurable DPOAEs. Conclusion: Hearing loss in 22q11.2 deletion syndrome is highly prevalent and both conductive and high-frequency sensorineural in nature. The age-dependent absence of DPOAEs in 22q11.2 deletion syndrome suggests cochlear damage underlying the high-frequency hearing loss. (As Provided).
AnmerkungenAmerican Speech-Language-Hearing Association. 2200 Research Blvd #250, Rockville, MD 20850. Tel: 301-296-5700; Fax: 301-296-8580; e-mail: slhr@asha.org; Web site: http://jslhr.pubs.asha.org
Erfasst vonERIC (Education Resources Information Center), Washington, DC
Update2020/1/01
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