{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,4]],"date-time":"2025-11-04T11:15:41Z","timestamp":1762254941413,"version":"3.37.3"},"reference-count":26,"publisher":"Georg Thieme Verlag KG","issue":"03","license":[{"start":{"date-parts":[[2024,7,5]],"date-time":"2024-07-05T00:00:00Z","timestamp":1720137600000},"content-version":"vor","delay-in-days":4,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Int Arch Otorhinolaryngol"],"published-print":{"date-parts":[[2024,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>\n          Introduction\u2003Williams syndrome (WS) is a genetic disorder caused by a microdeletion in chromosome 7, affecting \u223c 28 genes. Studies have demonstrated conductive losses seemingly related to the absence of the elastin gene and mild to profound sensorineural losses due to cochlear fragility.<\/jats:p><jats:p>\n          Objective\u2003To characterize and compare the peripheral auditory system and auditory brainstem response (ABR) of adults with WS and neurotypical adults matched by age and gender.<\/jats:p><jats:p>\n          Methods\u2003We conducted a cross-sectional observational study with 30 individuals of both sexes, aged 18 to 37 years \u2013 15 of them with WS (study group) and 15 with neither the syndrome nor hearing complaints (control group), matched for sex and age. The subjects underwent pure-tone and speech audiometry, acoustic immittance, transient-evoked otoacoustic emissions (TEOAEs), and ABR.<\/jats:p><jats:p>\n          Results\u2003Early-onset sensorineural hearing loss was found in 53.3% of the study sample, mostly mild, occurring above 3\u2009kHz. The TEOAEs were absent in 53.3% of assessed subjects; for those in whom they were present, the signal-to-noise responses were significantly lower than in the control group. In the ABR, increased absolute latencies were observed in waves I and III.<\/jats:p><jats:p>\n          Conclusion\u2003Individuals with WS have early and progressive cochlear impairments, mainly affecting the basal region of the cochlea. They may have low brainstem changes which seem to begin in adulthood.<\/jats:p>","DOI":"10.1055\/s-0044-1785457","type":"journal-article","created":{"date-parts":[[2024,7,5]],"date-time":"2024-07-05T23:31:13Z","timestamp":1720222273000},"page":"e502-e508","source":"Crossref","is-referenced-by-count":4,"title":["Peripheral Auditory Pathway and ABR Characterization in Adults with Williams Syndrome"],"prefix":"10.1055","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8646-6335","authenticated-orcid":false,"given":"Jacqueline Aquino do","family":"Nascimento","sequence":"additional","affiliation":[{"name":"Department of Physical, Speech-Language-Hearing, and Occupational Therapies, School of Medicine, Universidade de S\u00e3o Paulo (FMUSP), S\u00e3o Paulo, SP, Brazil."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8985-0447","authenticated-orcid":false,"given":"Liliane Aparecida Fagundes","family":"Silva","sequence":"additional","affiliation":[{"name":"Department of Physical, Speech-Language-Hearing, and Occupational Therapies, School of Medicine, Universidade de S\u00e3o Paulo (FMUSP), S\u00e3o Paulo, SP, Brazil."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7164-8942","authenticated-orcid":false,"given":"Alessandra Gianella","family":"Samelli","sequence":"additional","affiliation":[{"name":"Department of Physical, Speech-Language-Hearing, and Occupational Therapies, School of Medicine, Universidade de S\u00e3o Paulo (FMUSP), S\u00e3o Paulo, SP, Brazil."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9408-7172","authenticated-orcid":false,"given":"Carla Gentile","family":"Matas","sequence":"additional","affiliation":[{"name":"Department of Physical, Speech-Language-Hearing, and Occupational Therapies, School of Medicine, Universidade de S\u00e3o Paulo (FMUSP), 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