{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T15:58:25Z","timestamp":1777478305149,"version":"3.51.4"},"reference-count":48,"publisher":"American Academy of Audiology","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["j am acad audiol"],"published-print":{"date-parts":[[2025,5,1]]},"abstract":"<jats:p><jats:bold>Background:<\/jats:bold> Large vestibular aqueduct syndrome (LVAS) is the most common inner ear malformation in children with hearing loss; however, imaging is required to determine the definitive diagnosis.  <jats:bold>Purpose:<\/jats:bold> This study aimed to preliminarily determine the\n sensitivity and specificity of air-conducted and bone-conducted cervical vestibular evoked myogenic potentials (VEMPs) and ocular VEMPs and wideband acoustic immittance (WAI) as screening tools for LVAS and identify candidates for imaging.  <jats:bold>Research Design:<\/jats:bold> This was a prospective\n observational study.  <jats:bold>Study Sample:<\/jats:bold> The sample included 10 participants (17 ears) with LVAS and 15 control participants.  <jats:bold>Intervention:<\/jats:bold> All participants completed cervical and ocular VEMP testing in response to air-conducted and bone-conducted stimuli\n and WAI testing.  <jats:bold>Results:<\/jats:bold> Ears with LVAS had significantly higher air-conducted cervical and ocular VEMP amplitudes but reduced bone-conducted VEMP amplitudes. Because of this pattern reversal, air-conducted amplitudes were subtracted from bone-conducted amplitudes. This\n novel outcome was the best individual factor for differentiating ears of the control group from ears with LVAS and is a sensitive clinical indicator of LVAS, with 82 percent sensitivity and 80 percent specificity for cervical VEMP and 88 percent sensitivity and 80 percent specificity for ocular\n VEMP. Ears with LVAS exhibited significant differences in WAI absorbance of control ears at select low, mid, and high frequencies, and they also exhibited characteristic low-frequency peaks in individual responses that could be detected using an automated algorithm with moderate sensitivity\n (64 percent) and specificity (72 percent).  <jats:bold>Conclusions:<\/jats:bold> This is the first report of the use of VEMP and WAI responses of individuals as screening tools for LVAS. VEMP and WAI could be used to determine which individuals are good candidates for radiographic imaging and decrease\n the time required to determine a diagnosis. The test results also demonstrated patterns that differed from those of other third window disorders. Therefore, important physiologic differences between LVAS and other third window disorders require further investigation.<\/jats:p>","DOI":"10.3766\/jaaa.240059","type":"journal-article","created":{"date-parts":[[2025,5,27]],"date-time":"2025-05-27T02:05:47Z","timestamp":1748311547000},"page":"160-171","source":"Crossref","is-referenced-by-count":1,"title":["Vestibular Evoked Myogenic Potentials and Wideband Acoustic Immittance as Screening Tools for Large Vestibular Aqueduct Syndrome"],"prefix":"10.3766","volume":"36","author":[{"given":"Kristen L.","family":"Janky","sequence":"first","affiliation":[{"name":"*Boys Town National Research Hospital, Omaha, NE"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jessie N.","family":"Patterson","sequence":"additional","affiliation":[{"name":"*Boys Town National Research Hospital, Omaha, NE"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elizabeth A.","family":"Kelly","sequence":"additional","affiliation":[{"name":"*Boys Town National Research Hospital, Omaha, NE"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gabrielle R.","family":"Merchant","sequence":"additional","affiliation":[{"name":"*Boys Town National Research Hospital, Omaha, NE"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"2087","reference":[{"key":"c1","volume-title":"Guidelines for Audiologic Screening","year":"1997"},{"issue":"(6)","key":"c2","first-page":"363","article-title":"Large vestibular aqueduct syndrome: audiological, radiological, clinical, and genetic features","volume":"26","year":"2005","journal-title":"Am J Otolaryngol"},{"issue":"(2)","key":"c3","first-page":"77","article-title":"The effect of muscle contraction level on the cervical vestibular evoked myogenic potential (cVEMP): usefulness of amplitude normalization","volume":"24","year":"2013","journal-title":"J Am Acad Audiol"},{"key":"c4","article-title":"Recommended procedure tympanometry","year":"2013"},{"issue":"(1)","key":"c5","first-page":"47","article-title":"Classifying the large vestibular aqueduct: morphometry to audiometry","volume":"44","year":"2023","journal-title":"Otol Neurotol"},{"key":"c6","doi-asserted-by":"crossref","first-page":"638574","DOI":"10.3389\/fneur.2021.638574","article-title":"Current trends, controversies, and future directions in the evaluation and management of superior canal dehiscence syndrome","volume":"12","year":"2021","journal-title":"Front Neurol"},{"issue":"(9)","key":"c7","first-page":"1971","article-title":"Enlarged vestibular aqueduct: review of controversial aspects","volume":"121","year":"2011","journal-title":"Laryngoscope"},{"issue":"(1)","key":"c8","first-page":"155","article-title":"Air-leak effects on ear-canal acoustic absorbance","volume":"36","year":"2015","journal-title":"Ear Hear"},{"issue":"(3)","key":"c9","first-page":"440","article-title":"Cochlear implantation in children with enlarged vestibular aqueduct: a systematic review of surgical implications and outcomes","volume":"44","year":"2023","journal-title":"Ear Hear"},{"key":"c10","doi-asserted-by":"crossref","first-page":"891","DOI":"10.3389\/fneur.2020.00891","article-title":"Biomechanics of third window syndrome","volume":"11","year":"2020","journal-title":"Front Neurol"},{"issue":"(1)","key":"c11","first-page":"127","article-title":"Air-conducted oVEMPs provide the best separation between intact and superior canal dehiscent labyrinths","volume":"34","year":"2013","journal-title":"Otol Neurotol"},{"issue":"(5)","key":"c12","first-page":"2617","article-title":"Ear-canal impedance and reflection coefficient in human infants and adults","volume":"94","year":"1993","journal-title":"J Acoust Soc Am"},{"issue":"(5)","key":"c13","first-page":"327","article-title":"Click evoked EMG responses in sternocleidomastoid muscles: characteristics in normal subjects","volume":"9","year":"1999","journal-title":"VES"},{"issue":"(1)","key":"c14","first-page":"50","article-title":"Air and bone-conducted vestibular evoked myogenic potentials in children with large vestibular aqueduct syndrome","volume":"141","year":"2021","journal-title":"Acta Otolaryngol"},{"issue":"(1)","key":"c15","first-page":"142","article-title":"An indicator of probable semicircular canal dehiscence: ocular vestibular evoked myogenic potentials to high frequencies","volume":"149","year":"2013","journal-title":"Otolaryngol Head Neck Surg"},{"issue":"(4)","key":"c16","first-page":"508","article-title":"Ocular and cervical vestibular evoked myogenic potentials to 500 Hz fz bone-conducted vibration in superior semicircular canal dehiscence","volume":"33","year":"2012","journal-title":"Ear Hear"},{"issue":"(6)","key":"c17","first-page":"557","article-title":"Vestibular signs and symptoms of volumetric abnormalities of the vestibular aqueduct","volume":"122","year":"2008","journal-title":"J Laryngol Otol"},{"issue":"(1)","key":"c18","first-page":"e2021055340","article-title":"Three years of vestibular infant screening in infants with sensorineural hearing loss","volume":"150","year":"2022","journal-title":"Pediatrics"},{"issue":"(2)","key":"c19","first-page":"385","article-title":"Vestibular infant screening-flanders: What is the most appropriate vestibular screening tool in hearing-impaired children","volume":"44","year":"2023","journal-title":"Ear Hear"},{"issue":"(5)","key":"c20","first-page":"1183","article-title":"Improving the differential diagnosis of otitis media with effusion using wideband acoustic immittance","volume":"42","year":"2021","journal-title":"Ear Hear"},{"key":"c21","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1016\/j.heares.2016.07.018","article-title":"Controlled exploration of the effects of conductive hearing loss on wideband acoustic immittance in human cadaveric preparations","volume":"341","year":"2016","journal-title":"Hear Res"},{"issue":"(1)","key":"c22","first-page":"172","article-title":"Power reflectance as a screening tool for the diagnosis of superior semicircular canal dehiscence","volume":"36","year":"2015","journal-title":"Otol Neurotol"},{"issue":"(5)","key":"c23","first-page":"1111","article-title":"Effect of cochlear implantation on vestibular evoked myogenic potentials and wideband acoustic immittance","volume":"41","year":"2020","journal-title":"Ear Hear"},{"issue":"(7)","key":"c24","first-page":"532","article-title":"Clinical investigation and mechanism of air-bone gaps in large vestibular aqueduct syndrome","volume":"116","year":"2007","journal-title":"Ann Otol Rhinol Laryngol"},{"issue":"(3)","key":"c25","first-page":"282","article-title":"Conductive hearing loss caused by third-window lesions of the inner ear","volume":"29","year":"2008","journal-title":"Otol Neurotol"},{"issue":"(5)","key":"c26","first-page":"3013","article-title":"Incorporating evanescent modes and flow losses into reference impedances in acoustic th\u00e9venin calibration","volume":"142","year":"2017","journal-title":"J Acoust Soc Am"},{"issue":"(7\u201308)","key":"c27","first-page":"165","article-title":"Electrode montage for bilateral cervical vestibular evoked myogenic potential (cVEMP) testing","volume":"35","year":"2024","journal-title":"J Am Acad Audiol"},{"issue":"(6)","key":"c28","first-page":"921","article-title":"Wideband acoustic immittance in superior semicircular canal dehiscence","volume":"49","year":"2022","journal-title":"Auris Nasus Larynx"},{"issue":"(6)","key":"c29","first-page":"817","article-title":"Optimization of cervical and ocular vestibular evoked myogenic potential testing using an impulse hammer in adults, adolescents, and children","volume":"41","year":"2020","journal-title":"Otol Neurotol"},{"issue":"(2)","key":"c30","first-page":"269","article-title":"Effects of high sound exposure during air-conducted vestibular evoked myogenic potential testing in children and young adults","volume":"39","year":"2018","journal-title":"Ear Hear"},{"issue":"(1\u20132)","key":"c31","first-page":"161","article-title":"Vestibular-evoked myogenic potentials in three patients with large vestibular aqueduct","volume":"190","year":"2004","journal-title":"Hear Res"},{"issue":"(8)","key":"c32","first-page":"1440","article-title":"Vestibular-evoked myogenic potentials in infancy and early childhood","volume":"115","year":"2005","journal-title":"Laryngoscope"},{"issue":"(8)","key":"c33","first-page":"788","article-title":"The large vestibular aqueduct\u2013case report and review of the literature","volume":"126","year":"2006","journal-title":"Acta Otolaryngol"},{"issue":"(6)","key":"c34","first-page":"768","article-title":"Augmented ocular vestibular evoked myogenic potentials to air-conducted sound in large vestibular aqueduct syndrome","volume":"33","year":"2012","journal-title":"Ear Hear"},{"key":"c35","doi-asserted-by":"crossref","first-page":"580184","DOI":"10.3389\/fneur.2020.580184","article-title":"Bone-conducted oVEMP latency delays assist in the differential diagnosis of large air-conducted oVEMP amplitudes","volume":"11","year":"2020","journal-title":"Front Neurol"},{"issue":"(5)","key":"c36","first-page":"723","article-title":"The large vestibular aqueduct syndrome","volume":"88","year":"1978","journal-title":"Laryngoscope"},{"issue":"(6)","key":"c37","first-page":"1133","article-title":"When is the vestibular aqueduct enlarged? A statistical analysis of the normative distribution of vestibular aqueduct size","volume":"28","year":"2007","journal-title":"AJNR Am J Neuroradiol"},{"issue":"(6)","key":"c38","first-page":"464","article-title":"Vestibular-evoked myogenic potential thresholds normalize on plugging superior canal dehiscence","volume":"70","year":"2008","journal-title":"Neurology"},{"issue":"(10)","key":"c39","first-page":"2344","article-title":"Vestibular pathology in children with enlarged vestibular aqueduct: Vestibular pathology in pediatric EVA","volume":"126","year":"2016","journal-title":"Laryngoscope"},{"issue":"(2)","key":"c40","first-page":"257","article-title":"Vestibular dysfunction in patients with enlarged vestibular aqueduct","volume":"153","year":"2015","journal-title":"Otolaryngol Head Neck Surg"},{"issue":"(1)","key":"c41","first-page":"236","article-title":"Three\u2010dimensional wideband absorbance immittance findings in young adults with large vestibular aqueduct syndrome","volume":"8","year":"2022","journal-title":"Laryngoscope Investig Otolaryngol"},{"key":"c42","doi-asserted-by":"crossref","first-page":"109690","DOI":"10.1016\/j.ijporl.2019.109690","article-title":"Inner ear pressure evaluation using wideband tympanometry in children with large vestibular aqueduct syndrome (LVAS): a pilot study","volume":"128","year":"2020","journal-title":"Int J Pediatr Otorhinolaryngol"},{"issue":"(1)","key":"c43","first-page":"40","article-title":"Vestibular-evoked myogenic potentials in patients with large vestibular aqueduct syndrome","volume":"140","year":"2020","journal-title":"Acta Otolaryngol"},{"issue":"(11)","key":"c44","first-page":"2062","article-title":"Delineating the hearing loss in children with enlarged vestibular aqueduct","volume":"118","year":"2008","journal-title":"Laryngoscope"},{"issue":"(1)","key":"c45","first-page":"220","article-title":"Characteristics of vestibular evoked myogenic potentials in children with enlarged vestibular aqueduct","volume":"121","year":"2011","journal-title":"Laryngoscope"},{"issue":"(8)","key":"c46","first-page":"1368","article-title":"Clinical use of vestibular evoked myogenic potentials in the evaluation of patients with air-bone gaps","volume":"33","year":"2012","journal-title":"Otol Neurotol"},{"issue":"(8)","key":"c47","first-page":"1513","article-title":"Contrasting results of tests of peripheral vestibular function in patients with bilateral large vestibular aqueduct syndrome","volume":"128","year":"2017","journal-title":"Clin Neurophysiol"},{"issue":"(1)","key":"c48","first-page":"121","article-title":"Ocular versus cervical VEMPs in the diagnosis of superior semicircular canal dehiscence syndrome","volume":"34","year":"2013","journal-title":"Otol Neurotol"}],"container-title":["Journal of the American Academy of Audiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ingentaconnect.com\/content\/aaa\/jaaa\/2025\/00000036\/00000003\/art00004","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,10]],"date-time":"2025-09-10T04:42:56Z","timestamp":1757479376000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ingentaconnect.com\/content\/10.3766\/jaaa.240059"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,5,1]]},"references-count":48,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2025,5,1]]}},"alternative-id":["1050-0545(20250501)36:3L.160;1-"],"URL":"https:\/\/doi.org\/10.3766\/jaaa.240059","relation":{},"ISSN":["1050-0545"],"issn-type":[{"value":"1050-0545","type":"print"}],"subject":[],"published":{"date-parts":[[2025,5,1]]}}}