Enlarged vestibular aqueduct syndrome: A case of bilateral, sudden sensorineural hearing loss in a child

2006 ◽  
Vol 1 (2) ◽  
pp. 142-144 ◽  
Author(s):  
Stephen Maturo ◽  
Drew Horlbeck
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Enlarged Vestibular Aqueduct ◽  
Vestibular Aqueduct

Enlarged Vestibular Aqueduct in Pediatric SNHL

2008 ◽  
Vol 139 (2_suppl) ◽  
pp. P104-P104
Author(s):  
Karuna Dewan ◽  
Judith C. Lieu
Keyword(s):  
Hearing Loss ◽  
Cochlear Implant ◽  
Sensorineural Hearing Loss ◽  
Temporal Bone ◽  
Pediatric Population ◽  
Sensorineural Hearing ◽  
Enlarged Vestibular Aqueduct ◽  
Vestibular Aqueduct ◽  
Current Estimation ◽  
Temporal Bone Ct

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.

Hyperbaric oxygen therapy for sudden sensorineural hearing loss in large vestibular aqueduct syndrome

2013 ◽  
Vol 128 (S1) ◽  
pp. S50-S54 ◽  
Author(s):  
H Shilton ◽  
M Hodgson ◽  
G Burgess
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Hyperbaric Oxygen ◽  
Hyperbaric Oxygen Therapy ◽  
Oxygen Therapy ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Vestibular Aqueduct ◽  
Large Vestibular Aqueduct Syndrome ◽  
Large Vestibular Aqueduct

AbstractIntroduction:We report the first use in Australia of hyperbaric oxygen therapy for sudden hearing loss following head trauma in a child with large vestibular aqueduct syndrome.Case report:A 12-year-old boy with large vestibular aqueduct syndrome presented with significant hearing loss following head trauma. He was treated with steroids and hyperbaric oxygen therapy, with good improvement of hearing thresholds on audiography. This case represents the first reported use of hyperbaric oxygen therapy for this indication in Australia, following a few previous reports of patients in Japan. We review the literature on management of acute sensorineural hearing loss in large vestibular aqueduct syndrome. The reported case demonstrates a potentially beneficial therapy for a rare condition that usually results in an inevitable decline in hearing.Conclusion:Hyperbaric oxygen therapy can be tolerated well by children, and may represent a potential treatment for sudden sensorineural hearing loss in patients with large vestibular aqueduct syndrome.

The Enlarged Vestibular Aqueduct Syndrome (EVA Syndrome)

1993 ◽  
Vol 72 (11) ◽  
pp. 746-751 ◽  
Author(s):  
Walter M. Belenky ◽  
David N. Madgy ◽  
Jeffrey S. Leider ◽  
Christie J. Becker ◽  
Andrew J. Hotaling
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Round Window ◽  
Sensorineural Hearing ◽  
Fistula Formation ◽  
Enlarged Vestibular Aqueduct ◽  
Children's Hospital ◽  
Vestibular Aqueduct ◽  
Extensive Evaluation ◽  
Children’S Hospital

The presentation to the Department of Pediatric Otolaryngology at the Children's Hospital of Michigan of a series of patients with sensorineural hearing loss and enlargement of the vestibular aqueduct prompted exploratory tympanotomy in three patients (two unilateral and one bilateral), for a total of four ears. These explorations were prompted by progression and/or fluctuation of hearing levels. The discovery of abnormal round windows in all four ears with a post-traumatic fistula present in one ear suggested the presence of a new association. A previously undescribed association of an enlarged vestibular aqueduct, sensorineural hearing loss and round window abnormality with potential fistula formation was identified. A review of the anatomy and physiology, literature review, and a prospective analysis with discussion of eight patients with enlarged vestibular aqueduct syndrome evaluated and treated at Children's Hospital of Michigan, is presented. We conclude that all children with sensorineural hearing loss should undergo extensive evaluation to determine etiology, including radiographic studies of the temporal bone. Further, the presence of an enlarged vestibular aqueduct should prompt the otolaryngologist to consider the presence of a round window abnormality and the potential for predisposition to perilymph fistula.

scholarly journals Sudden Sensorineural Hearing Loss in the Only Hearing Ear: Large Vestibular Aqueduct Syndrome

2016 ◽  
Vol 2016 ◽  
pp. 1-3
Author(s):  
Kemal Koray Bal ◽  
Onur Ismi ◽  
Helen Bucioglu ◽  
Yusuf Vayısoğlu ◽  
Kemal Gorur
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Social Life ◽  
English Literature ◽  
Delayed Diagnosis ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Vestibular Aqueduct ◽  
Large Vestibular Aqueduct Syndrome ◽  
Large Vestibular Aqueduct

Sudden hearing loss in the only hearing ear cases are rarely published in the English literature; most of the cases are idiopathic. It is an otologic emergency needing urgent treatment. Delayed diagnosis can interfere with patient’s social life with interrupting the verbal communication. In this case report we presented a 33-year-old female patient having sudden sensorineural hearing loss in the only hearing ear diagnosed as bilateral large vestibular aqueduct syndrome.

scholarly journals Enlarged vestibular aqueduct in pediatric sensorineural hearing loss

2009 ◽  
Vol 140 (4) ◽  
pp. 552-558 ◽  
Author(s):  
Karuna Dewan ◽  
Franz J. Wippold ◽  
Judith E.C. Lieu
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Enlarged Vestibular Aqueduct ◽  
Vestibular Aqueduct
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