Retrospective Review of Midpoint Vestibular Aqueduct Size in the 45° Oblique (Pöschl) Plane and Correlation with Hearing Loss in Patients with Enlarged Vestibular Aqueduct

Problem Current diagnostic criteria for enlarged vestibular aqueduct (EVA), >1.5mm at the midpoint, was determined in the pre-CT era by Valvassori. Recent research, based on 73 CTs from children with no sensorineural hearing loss (SNHL), suggests new criteria for the diagnosis of EVA—midpoint of >0.9mm or operculum >1.9mm. We evaluated the proposed new radiographic, Cincinnati criteria for the diagnosis of EVA. Methods In a retrospective cohort study, we reviewed temporal bone CT scans of 130 pediatric cochlear implant recipients to measure the vestibular aqueduct midpoint and opercular width and 5 other temporal bone dimensions. Results The Cincinnati criteria identified 44% of patients with EVA versus 16% with the Valvassori criterion (P < 0.01). Of those with EVA, 45% were unilateral and 55% were bilateral using Cincinnati criteria; 64% were unilateral and 36% bilateral using Valvassori criterion (P<0.01). Right and left side measurements of vestibular aqueduct operculum (r=0.67, P<0.01) and midpoint (r=0.58, P<0.01) correlated substantially. The Cincinnati criteria diagnosed 70 ears with EVA classified as normal using the Valvassori criterion (P<0.01). Of these 70 ears, 59 had no other medical explanation for their hearing loss. Conclusion The Cincinnati criteria identified a large percentage of pediatric cochlear implant patients with EVA who otherwise had no known etiology for their deafness. Significant correlations between right and left side measurements suggest that EVA may not be morphologically asymmetric as previously thought. Significance The Cincinnati criteria potentially alters the current estimation of the most common etiologies of bilateral severe-to-profound sensorineural hearing loss in the pediatric population. Support KD is a Doris Duke Clinical Research Fellow, supported by the Doris Duke Foundation.

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Subgroups of enlarged vestibular aqueduct in relation toSLC26A4mutations and hearing loss

The Laryngoscope ◽

10.1002/lary.24368 ◽

2013 ◽

Vol 124 (4) ◽

pp. E134-E140 ◽

Cited By ~ 17

Author(s):

Yasuhide Okamoto ◽

Hideki Mutai ◽

Atsuko Nakano ◽

Yukiko Arimoto ◽

Tomoko Sugiuchi ◽

...

Keyword(s):

Hearing Loss ◽

Enlarged Vestibular Aqueduct ◽

Vestibular Aqueduct

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Vestibular Aqueduct Midpoint Width and Hearing Loss in Patients With an Enlarged Vestibular Aqueduct

JAMA Otolaryngology–Head & Neck Surgery ◽

10.1001/jamaoto.2016.4522 ◽

2017 ◽

Vol 143 (6) ◽

pp. 601 ◽

Cited By ~ 7

Author(s):

Mustafa S. Ascha ◽

Nauman Manzoor ◽

Amit Gupta ◽

Maroun Semaan ◽

Cliff Megerian ◽

...

Keyword(s):

Hearing Loss ◽

Enlarged Vestibular Aqueduct ◽

Vestibular Aqueduct

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Hearing Loss in Enlarged Vestibular Aqueduct

Otology & Neurotology ◽

10.1097/mao.0000000000002843 ◽

2020 ◽

Vol Publish Ahead of Print ◽

Author(s):

Haroon Shakeel Saeed ◽

Jonny Kenth ◽

Graeme Black ◽

Shakeel R. Saeed ◽

Stavros Stivaros ◽

...

Keyword(s):

Hearing Loss ◽

Enlarged Vestibular Aqueduct ◽

Vestibular Aqueduct

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Progressive Fluctuant Hearing Loss, Enlarged Vestibular Aqueduct, and Cochlear Hypoplasia in Branchio-oto-renal Syndrome

Otology & Neurotology ◽

10.1097/00129492-200109000-00014 ◽

2001 ◽

Vol 22 (5) ◽

pp. 637-643 ◽

Cited By ~ 22

Author(s):

M. H. Kemperman ◽

C. Stinckens ◽

S. Kumar ◽

P. L. M. Huygen ◽

F. B. M. Joosten ◽

...

Keyword(s):

Hearing Loss ◽

Enlarged Vestibular Aqueduct ◽

Vestibular Aqueduct

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Unilateral Enlarged Vestibular Aqueduct Syndrome and Bilateral Endolymphatic Hydrops

Case Reports in Otolaryngology ◽

10.1155/2017/6195317 ◽

2017 ◽

Vol 2017 ◽

pp. 1-6 ◽

Cited By ~ 1

Author(s):

Massimo Ralli ◽

Giuseppe Nola ◽

Luca Sparvoli ◽

Giovanni Ralli

Keyword(s):

Hearing Loss ◽

Intracranial Pressure ◽

Endolymphatic Hydrops ◽

Correct Diagnosis ◽

Ct Scans ◽

Vestibular Disorders ◽

Hearing Improvement ◽

Enlarged Vestibular Aqueduct ◽

Vestibular Aqueduct ◽

Inner Ear Malformation

Enlarged vestibular aqueduct (EVA) syndrome is a common congenital inner ear malformation characterized by a vestibular aqueduct with a diameter larger than 1.5 mm, mixed or sensorineural hearing loss that ranges from mild to profound, and vestibular disorders that may be present with a range from mild imbalance to episodic objective vertigo. In our study, we present the case of a patient with unilateral enlarged vestibular aqueduct and bilateral endolymphatic hydrops (EH). EH was confirmed through anamnestic history and audiological exams; EVA was diagnosed using high-resolution CT scans and MRI images. Therapy included intratympanic infusion of corticosteroids with a significant hearing improvement, more evident in the ear contralateral to EVA. Although most probably unrelated, EVA and EH may present with similar symptoms and therefore the diagnostic workup should always include the proper steps to perform a correct diagnosis. Association between progression of hearing loss and head trauma in patients with a diagnosis of EVA syndrome is still uncertain; however, these individuals should be advised to avoid activities that increase intracranial pressure to prevent further hearing deterioration. Intratympanic treatment with steroids is a safe and well-tolerated procedure that has demonstrated its efficacy in hearing, tinnitus, and vertigo control in EH.

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Hearing loss in enlarged vestibular aqueduct and incomplete partition type II

American Journal of Otolaryngology ◽

10.1016/j.amjoto.2017.06.010 ◽

2017 ◽

Vol 38 (6) ◽

pp. 692-697 ◽

Cited By ~ 6

Author(s):

Emily Ahadizadeh ◽

Mustafa Ascha ◽

Nauman Manzoor ◽

Amit Gupta ◽

Maroun Semaan ◽

...

Keyword(s):

Hearing Loss ◽

Type Ii ◽

Enlarged Vestibular Aqueduct ◽

Vestibular Aqueduct ◽

Incomplete Partition

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Molecular analysis of hearing loss associated with enlarged vestibular aqueduct in the mainland Chinese: a unique SLC26A4 mutation spectrum

Journal of Human Genetics ◽

10.1007/s10038-007-0139-0 ◽

2007 ◽

Vol 52 (6) ◽

pp. 492-497 ◽

Cited By ~ 33

Author(s):

Hao Hu ◽

Lingqian Wu ◽

Yong Feng ◽

Qian Pan ◽

Zhigao Long ◽

...

Keyword(s):

Hearing Loss ◽

Molecular Analysis ◽

Mutation Spectrum ◽

Enlarged Vestibular Aqueduct ◽

Mainland Chinese ◽

Vestibular Aqueduct ◽

Slc26a4 Mutation

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Diagnostic Yield of Computed Tomography Scan for Pediatric Hearing Loss

Otolaryngology ◽

10.1177/0194599814545727 ◽

2014 ◽

Vol 151 (5) ◽

pp. 718-739 ◽

Cited By ~ 16

Author(s):

Jenny X. Chen ◽

Bart Kachniarz ◽

Jennifer J. Shin

Keyword(s):

Computed Tomography ◽

Hearing Loss ◽

Diagnostic Yield ◽

Radiation Risk ◽

Cochlear Nerve ◽

Cochrane Library ◽

Unknown Etiology ◽

Enlarged Vestibular Aqueduct ◽

Vestibular Aqueduct ◽

Pediatric Hearing Loss

Background Computed tomography (CT) has been used in the assessment of pediatric hearing loss, but concern regarding radiation risk and increased utilization of magnetic resonance imaging (MRI) have prompted us toward a more quantitative and sophisticated understanding of CT’s potential diagnostic yield. Objective To perform a systematic review to analyze the diagnostic yield of CT for pediatric hearing loss, including subgroup evaluation according to impairment severity and laterality, as well as the specific findings of enlarged vestibular aqueduct and narrow cochlear nerve canal. Data Sources PubMed, EMBASE, and the Cochrane Library were assessed from the date of their inception to December 2013. In addition, manual searches of bibliographies were performed and topic experts were contacted. Review Methods Data from studies describing the use of CT in the diagnostic evaluation of pediatric patients with hearing loss of unknown etiology were evaluated, according to a priori inclusion/exclusion criteria. Two independent evaluators corroborated the extracted data. Heterogeneity was evaluated according to the I2 statistic. Results In 50 criteria-meeting studies, the overall diagnostic yield of CT ranged from 7% to 74%, with the strongest and aggregate data demonstrating a point estimate of 30%. This estimate corresponded to a number needed to image of 4 (range, 2-15). The most commonly identified findings were enlarged vestibular aqueduct and cochlear anomalies. The largest studies showed a 4% to 7% yield for narrow cochlear nerve canal. Conclusion These data, along with similar analyses of radiation risk and risks/benefits of sedated MRI, may be used to help guide the choice of diagnostic imaging.

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