Cochlear active mechanisms in young normal-hearing subjects affected by Williams syndrome: time-frequency analysis of otoacoustic emissions.
The aim of this study was to investigate the functionality of cochlear active mechanisms in normal-hearing subjects affected by Williams syndrome (WS). Transient evoked otoacoustic emissions (TEOAEs) were recorded in a group of young WS subjects and a group of typically developing control subjects, all having normal-hearing thresholds and normal middle-ear functionality. We also analysed the narrow-band frequency components of TEOAEs, extracted from the broad-band TEOAE recordings by using a time-frequency analysis algorithm based on the Wavelet transform. We observed that TEOAEs and the frequency components extracted from TEOAEs measured in WS subjects had significantly lower energy compared to the controls. Also, the narrow-band frequency components of TEOAEs measured in WS subjects had slightly increased latency compared to the controls. Overall, results would suggest a subtle (i.e., sub-clinical) dysfunction of the cochlear active mechanisms in WS subjects with otherwise normal hearing. Also, results point out the relevance of using otoacoustic emissions in the audiological evaluation and monitoring of WS subjects to early identify possible subtle auditory dysfunctions, before the onset of mild or moderate hearing loss that could exacerbate language or cognitive impairments associated with WS.
Istituto di Ingegneria Biomedica, Consiglio Nazionale delle Ricerche (ISIB-CNR), Piazza Leonardo da Vinci 32, I-20133 Milano, Italy. alessia.paglialonga@polimi.it
Alessia
A
Paglialonga
Stefania
S
Barozzi
Daniele
D
Brambilla
Daniela
D
Soi
Antonio
A
Cesarani
Chiara
C
Gagliardi
Elisabetta
E
Comiotto
Emanuela
E
Spreafico
Gabriella
G
Tognola
D000158:Acoustic Impedance Tests; D000161:Acoustic Stimulation; D000293:Adolescent; D000704:Analysis of Variance; D001301:Audiometry, Pure-Tone; D001309:Auditory Threshold; D016022:Case-Control Studies; D002648:Child; D003051:Cochlea / Q000503:physiopathology*; D005260:Female; D006309:Hearing; D006801:Humans; D008297:Male; D017084:Otoacoustic Emissions, Spontaneous; D010641:Phenotype; D011930:Reaction Time; D012815:Signal Processing, Computer-Assisted; D013018:Sound Spectrography; D013997:Time Factors; D018980:Williams Syndrome / Q000503:physiopathology*; D055815:Young Adult