Enlarged vestibular aqueduct: Review of controversial aspects

Gopen, Quinton; Zhou, Guangwei; Whittemore, Kenneth R.; Kenna, Margaret A.

doi:10.1002/lary.22083

Cited by 101 publications

(116 citation statements)

References 73 publications

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“…Gopen et al [11] also mentioned that three long-term studies [12-14] reached similar conclusions. However, Emmett [15] and Zalza et al [16] obtained contradictory results; they reported that most of their patients had stable hearing.…”

Section: Introductionmentioning

confidence: 75%

Timing of Surgical Intervention with Cochlear Implant in Patients with Large Vestibular Aqueduct Syndrome

Liu

Lee

et al. 2013

PLoS ONE

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Objectives(1) To report the speech perception and intelligibility results of Mandarin-speaking patients with large vestibular aqueduct syndrome (LVAS) after cochlear implantation (CI); (2) to compare their performance with a group of CI users without LVAS; (3) to understand the effects of age at implantation and duration of implant use on the CI outcomes. The obtained data may be used to guide decisions about CI candidacy and surgical timing.MethodsForty-two patients with LVAS participating in this study were divided into two groups: the early group received CI before 5 years of age and the late group after 5. Open-set speech perception tests (on Mandarin tones, words and sentences) were administered one year after implantation and at the most recent follow-up visit. Categories of auditory perception (CAP) and Speech Intelligibility Rating (SIR) scale scores were also obtained. ResultsThe patients with LVAS with more than 5 years of implant use (18 cases) achieved a mean score higher than 80% on the most recent speech perception tests and reached the highest level on the CAP/SIR scales. The early group developed speech perception and intelligibility steadily over time, while the late group had a rapid improvement during the first year after implantation. The two groups, regardless of their age at implantation, reached a similar performance level at the most recent follow-up visit.ConclusionHigh levels of speech performance are reached after 5 years of implant use in patients with LVAS. These patients do not necessarily need to wait until their hearing thresholds are higher than 90 dB HL or PB word score lower than 40% to receive CI. They can do it “earlier” when their speech perception and/or speech intelligibility do not reach the performance level suggested in this study.

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Section: Introductionmentioning

confidence: 75%

Timing of Surgical Intervention with Cochlear Implant in Patients with Large Vestibular Aqueduct Syndrome

Liu

Lee

et al. 2013

PLoS ONE

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“…An examination of the literature studies revealed that the hearing loss associated with IP-II and LVA malformations is heterogeneous, and the conductive component is not associated with middle ear pathologies [2,12,13,17,18,[20][21][22][23][24][25][26] . Roesch et al [25] found that patients with IP-II and isolated LVA malformations were diagnosed not only with severe to profound hearing loss but also with moderate asymmetrical hearing loss.…”

Section: Discussionmentioning

confidence: 99%

“…Previous studies investigated the relationship between the size of the vestibular aqueduct and hearing loss, and different results were indicated [22,27,28] . Among these studies, Seo et al [28] found a correlation between the ABG and the size of the vestibular aqueduct.…”

Section: Discussionmentioning

confidence: 99%

Audiological and Radiological Characteristics in Incomplete Partition Malformations

Batuk¹,

Çınar²,

Özgen³

et al. 2017

Int Adv Otol

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OBJECTIVE:To compare the audiological and radiological findings of patients with incomplete partition malformations (IPs) and analyze the relationship between the audiological and radiological findings. MATERIALS and METHODS:The study included 84 patients (168 ears) with IPs as follows: 26 patients with Type I;IP-I (41 ears), 54 patients with Type II;IP-II (108 ears), and 4 patients with Type III;IP-III (8 ears). Remaining 11 ears were diagnosed with other inner ear malformations. Air and bone conduction thresholds were determined with pure tone audiometry, and the air bone gap was recorded in all patients with IPs. Magnetic resonance imaging studies and computerized tomography scans of the temporal bones were analyzed using the PACS system of our university. RESULTS:It was found that all the ears with IP-I were diagnosed with severe to profound hearing loss. The degree of the hearing loss varied from mild to severe/profound in patients with IP-II. Severe to profound mixed hearing loss (MHL) was determined in all ears with IP-III. The air bone gap was larger in the lower frequencies in the IP-II cases diagnosed with MHL. There was not a significant difference between the air bone gap and the size of the vestibular aqueduct in ears with IP-II (p>0.05).CONCLUSION: Each type of IP has different audiological findings. Depending on the type and degree of the hearing loss, it is possible to choose the appropriate audiological intervention. Patients with IP should be evaluated according to the type of malformation.

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“…Many adult patients with EVA may have not been diagnosed in the past due to lack of knowledge about the condition and availability of accurate imaging techniques [3]. Here, we report an interesting case of subsequent inadequate otological and audiological diagnosis leading to a profound hearing loss.…”

Section: Introductionmentioning

confidence: 97%

Misdiagnosis of otosclerosis in a patient with enlarged vestibular aqueduct syndrome: a case report

Távora-Vieira

Miller²

2012

J Med Case Reports

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Introduction In the present case we report on the mismanagement of a patient misdiagnosed with otosclerosis, who was subsequently found to have enlarged vestibular aqueduct syndrome bilaterally. This highlights the need to not only be vigilant in pre-operative assessment of otosclerosis but also in post-operative investigations of stapedectomy failures. Case presentation Our patient, a 56-year-old Caucasian Australian woman, lost the hearing in her right ear following a stapedectomy approximately 25 years ago. It is thought that preoperative imaging was not conducted, while an inadequate (unmasked) audiogram was used to formulate the initial diagnosis of otosclerosis. The hearing in her left ear deteriorated to the point that a cochlear implant was now being considered for her right ear. Imaging performed as part of our pre-cochlear implant battery revealed bilateral enlarged vestibular aqueducts and thus the decision to proceed with a right cochlear implant was made following discussion with our patient and her family in regard to not only general surgical risks but specifically the remote risk that the surgical drilling required during the procedure could risk a deterioration of the hearing in her left ear because of the enlarged vestibular aqueduct on that side. Conclusions This report illustrates a case of misdiagnosis and mismanagement of bilateral enlarged vestibular aqueduct resulting in profound hearing loss. Fortunately our patient has been successfully implanted with a right cochlear implant with remarkable outcomes.

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Enlarged vestibular aqueduct: Review of controversial aspects

Abstract: The enlarged vestibular aqueduct remains a controversial entity with variable presentation, progression, and prognosis.

Cited by 101 publications

References 73 publications

Timing of Surgical Intervention with Cochlear Implant in Patients with Large Vestibular Aqueduct Syndrome

Timing of Surgical Intervention with Cochlear Implant in Patients with Large Vestibular Aqueduct Syndrome

Audiological and Radiological Characteristics in Incomplete Partition Malformations

Misdiagnosis of otosclerosis in a patient with enlarged vestibular aqueduct syndrome: a case report

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