EDUDEAF: Enlarged Vestibular Aqueduct Syndrome

Key Words: Deaf Education Information, Deafness Related Issues, Additional Disorders

Document 1 of 6

Document: 1 2 3 4 5 6

Date: Fri, 1 Nov 1996 09:01:32 -0800
Reply-To: A Practical Discussion List Regarding Deaf Education
Sender: A Practical Discussion List Regarding Deaf Education
Subject: Enlarged Vestibular Aqueduct Syndrome
To: Multiple recipients of list EDUDEAF

I wanted to see if anyone else here knew of this syndrome that my 9 yr old son seems to have. He was diagnosed earlier this year with EVA Syndrome (enlarged vestibular aqueduct) and has had a progressive hearing loss. He just started wearing hearing aides in August, and 2 weeks ago showed a big drop in his hearing in his "good" ear. Last week, at our ARD mtg, it was suggested by the HOH/deaf program of our county that we visit another elem. school where he would be with others who are deaf/HOH, an oral school. It has been especially frustrating for him for the past 2-3 weeks (very loud in the cafeteria, resulting in him screaming a lot.) This other elem. school is also where the deaf/HOH staff is, although of course a deaf/HOH special ed teacher comes now to his present school. Also, he would still be able to get the services he needs for LD. My husband and I visit the school on Mon. and need to decide. After we see the school we can talk to our son about it. Any input would be appreciated.

Marcie

Document 2 of 6

Document: 1 2 3 4 5 6

Date: Fri, 1 Nov 1996 20:50:14 -0500
Reply-To: A Practical Discussion List Regarding Deaf Education
Sender: A Practical Discussion List Regarding Deaf Education
Subject: Re: Enlarged Vestibular Aqueduct Syndrome
To: Multiple recipients of list EDUDEAF

I can't believe I'm seeing this! For the past couple of weeks I've been searching ERIC and various other databases looking for information about this syndrome, with no joy, and tonight I see it in your subject.

Unfortunately I don't know much about it, but I still have a couple of possible sources to check out. I'm very interested because our project has had two referrals in the past 6 months of children with Enlarged Vestibular Aqueduct Syndrome - both are girls around kindergarten age, in quite different parts of our state, both with progressive losses, etc, much as you describe for your son. Unfortunately tonight I can't tell you anything you don't already know, but as soon as I can I will contact the excellent audiological clinic at Dartmouth-Hitchcock Medical Center and pick their brains. Whatever I get I will of course share. My guess is that there's not a lot known - it sounds like it's fairly newly identified and identifiable because of technology (ie, CAT scan). I would further guess that in fact it's responsible for some still unknown percentage of those hitherto "unknown" etiologies of hearing loss.

Anyway, whatever I find I'll forward to you.
Robin
henne@moose.ncia.net

Document 3 of 6

Document: 1 2 3 4 5 6

Date: Mon, 4 Nov 1996 11:13:06 -0500
Reply-To: A Practical Discussion List Regarding Deaf Education
Sender: A Practical Discussion List Regarding Deaf Education
Subject: Re: Enlarged Vestibular Aqueduct Syndrome
To: Multiple recipients of list EDUDEAF

Hey guys,
Do you have any info at all on this...there are about 5-6 parents of the 30 I regularly correspond with that have children with unexplainable conductive-type loss. The ENTs don't know what to do...I want a CATSCAN however Alex has trouble with sedatives so it's a major nightmare to do. ENT seems to be very complacent and audiologists have no clue as to cause. At first Alex had negative pressure but now he's at -55 and his hearing has gotten even worse than when he had -130 negative pressure. He does have allergies, but allergy meds don't seem to make a difference. The only thing that I've noticed in any way different is that the molars under his only "good" ear are finally almost finished coming through. Very upsetting as Alex is now deaf in both ears instead of deaf in one and hoh in the other. Yet, he still dips down to 25 db in one frequency that makes it hard to fit an aid on him. Just wondering if this might be something that's going on with the 5-6 other ones of us. Any info on symptoms, causes, how to detect it?

HELP!
Linda S

Document 4 of 6

Document: 1 2 3 4 5 6

Date: Tue, 5 Nov 1996 12:24:21 -0600
Reply-To: A Practical Discussion List Regarding Deaf Education
Sender: A Practical Discussion List Regarding Deaf Education
Subject: EVA journal references (long)
To: Multiple recipients of list EDUDEAF

The following references were taken from Medline, and should be available to every medical professional (not necessarily immediately, but they should be able to get the articles thru inter-library loan).

Candy Krepel

Unique Identifier

92230647

Authors

Mafee MF. Charletta D. Kumar A. Belmont H.

Institution

Department of Radiology, University of Illinois, Chicago.

Title

Large vestibular aqueduct and congenital sensorineural hearing loss. [Review]

Source


Ajnr: American Journal of Neuroradiology. 13(2):805-19, 1992 Mar-Apr.

Unique Identifier

95101222

Authors

Zalzal GH. Tomaski SM. Vezina LG. Bjornsti P. Grundfast KM.

Institution

Department of Pediatric Otolaryngology--Head and Neck Surgery, Children's National Medical Center, George Washington University, Washington, DC.

Title

Enlarged vestibular aqueduct and sensorineural hearing loss in childhood.

Source

Archives of Otolaryngology -- Head & Neck Surgery. 121(1):23-8, 1995 Jan.

Abstract

OBJECTIVE:
To determine if all children with enlarged vestibular aqueducts (EVAs) have development of uniform progressive sensorineural hearing loss (SNHL). To determine whether the size of the EVA correlates with severity, frequencies involved, and stability of SNHL. To determine if the audiologic pattern of SNHL correlates with likelihood of progression of SNHL. DESIGN: Retrospective study.

SETTING:
Children's National Medical Center, Washington, DC, a tertiary care center with a large otologic practice.

PATIENTS:
Fifteen children (26 ears) with EVA on computed tomographic scan.

METHODS:
History, physical examination, computed tomographic scans, and serial audiograms were reviewed. Factors analyzed included age at diagnosis, audiometric configuration (high tone, midtone, low tone, flat), degree of hearing loss at presentation, length of follow-up, and presence of associated inner ear anomalies.

RESULTS:
Nine ears had progressive SNHL, 16 ears had stable SNHL, and 1 ear had profound SNHL. The predominant audiologic configuration was flat. The audiogram configuration does not correlate with progression of SNHL. The size of the vestibular aqueduct does not correlate with the level, type, or progression of SNHL.

CONCLUSION:
Our study failed to uncover factors that might be predictive of progression of hearing loss. We conclude that until a better understanding of the natural history and pathophysiologic condition of EVAs is achieved, there is no surgical or other intervention that can be demonstrated as being efficacious.

Unique Identifier

93000951

Authors

Hirsch BE. Weissman JL. Curtin HD. Kamerer DB.

Institution

Department of Otolaryngology, University of Pittsburgh, School of Medicine, PA.

Title

Magnetic resonance imaging of the large vestibular aqueduct.

Source

Archives of Otolaryngology -- Head & Neck Surgery. 118(10):1124-7, 1992 Oct.

Abstract

The large vestibular aqueduct syndrome describes an abnormally large endolymphatic duct and sac with associated sensorineural hearing loss. This entity was originally reported in 1978 and has since been identified as a finding in children with progressive hearing loss. The original description of the large vestibular aqueduct employed hypocycloidal polytomography of temporal bone. Subsequent reports studied patients identified with this syndrome using computed tomographic scans. We report magnetic resonance imaging of two patients diagnosed with the large vestibular aqueduct syndrome. The magnetic resonance imaging and computed tomographic scans are compared and the significant findings on magnetic resonance imaging are reviewed. This should assist the otolaryngologist and radiologist with establishing the appropriate diagnosis.

Unique Identifier

94085244

Authors

Belenky WM. Madgy DN. Leider JS. Becker CJ. Hotaling AJ.

Institution

Department of Pediatric Otolaryngology, Children's Hospital of Michigan, Detroit 48201.

Title

The enlarged vestibular aqueduct syndrome (EVA syndrome).

Source

Ear, Nose, & Throat Journal. 72(11):746-51, 1993 Nov.

Abstract

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.

Unique Identifier

95131058

Authors

Shirazi A. Fenton JE. Fagan PA.

Institution

Department of Otology and Neuro-Otology, St Vincents Hospital, Darlinghurst, Sydney, Australia.

Title

Large vestibular aqueduct syndrome and stapes fixation.

Source

Journal of Laryngology & Otology. 108(11):989-90, 1994 Nov.

Abstract

An abnormally large vestibular aqueduct has a well recognized association with inner ear anomalies and it has been assumed previously to be a variant of a Mondini type of deformity (Shuknecht, 1980; Emmett, 1985). The sole radiological finding in some patients with progressive sensorineural loss has been a large vestibular aqueduct (Valvassori and Clemis, 1978; Valvassori, 1983), which is now accepted as a separate clinical entity, i.e. the large vestibular aqueduct syndrome (LVAS). A case is presented which is believed to be the first reported with unilateral LVAS and stapes fixation and also the first stapes gusher described in association with LVAS.

Unique Identifier

96252332

Authors

Cox LC. MacDonald CB.

Institution

Department of Otolaryngology, Boston University Medical Center, Massachusetts, USA.

Title

Large vestibular aqueduct syndrome: a tutorial and three case studies.

Source

Journal of the American Academy of Audiology. 7(2):71-6, 1996 Apr.

Abstract

The large vestibular aqueduct (LVA) syndrome is a congenital malformation that predisposes the patient ultimately to a loss of hearing and possible continuing vestibular disorder. If the LVA patient is diagnosed, it typically is not until later life, when he/she exhibits profound sensorineural hearing loss. To better understand this disorder and to bring it to the attention of audiologists, we provide a brief tutorial of LVA and present three case studies that illustrate the syndrome.

Unique Identifier

96320333

Authors

Griffith AJ. Arts A. Downs C. Innis JW. Shepard NT. Sheldon S. Gebarski SS.

Institution

Department of Otolaryngology/Head and Neck Surgery, University of Michigan, Ann Arbor., USA.

Title

Familial large vestibular aqueduct syndrome.

Source

Laryngoscope. 106(8):960-5, 1996 Aug.

Abstract

The large vestibular aqueduct syndrome (LVAS) is a distinct clinical entity characterized by stepwise progressive sensorineural hearing loss associated with isolated enlargement of the vestibular aqueduct. A correlative clinical, audiologic, vestibular, cytogenetic, and radiographic analysis of a family with inherited LVAS was performed. The male proband and his affected brother are offspring of unaffected parents, and have no other abnormalities. Pedigree analysis suggests autosomal recessive or X-linked inheritance with variable expressivity of LVAS in this family. This study is the first description of familial inheritance of LVAS. LVAS may account for a significant number of patients with nonsyndromal, genetic sensorineural hearing loss. Future molecular analyses of this study family may identify the causative gene(s) in LVAS.

Unique Identifier

95182777

Authors

Okumura T. Takahashi H. Honjo I. Takagi A. Mitamura K.

Institution

Department of Otolaryngology, Otsu Red Cross Hospital, Nagara, Japan.

Title

Sensorineural hearing loss in patients with large vestibular aqueduct.

Source

Laryngoscope. 105(3 Pt 1):289-93; discussion 293-4, 1995 Mar.

Abstract

In examining 181 patients (327 ears) with sensorineural hearing loss of unknown etiology and 25 people (50 ears) with normal hearing by high-resolution computed tomography (CT), the image of the large vestibular aqueduct (VA) was defined as being a visible large aperture (> or = 4 mm), and small distance between vestibule and traceable part of the VA nearest to the vestibule (> or = 1 mm). The large VA was found in 13 patients (23 ears, 7.0%); it was relatively frequent following hypoplastic cochlea (33 ears, 10.1%) in all the inner ear anomalies detected. In patients with large VA, high-frequency hearing was affected more than low frequency, and history of sudden hearing loss was observed frequently (61% of ears with large VA), which was found to be triggered by characteristic episodes such as minor head trauma, etc. Those clinical features were observed more in those without cochlear anomaly than in those accompanying cochlear anomaly. Pathogenesis of sensorineural hearing loss and characteristic fluctuation of hearing in those patients are discussed.

Unique Identifier

96068620

Authors

Reussner LA. Dutcher PO. House WF.

Institution

Division of Otolaryngology-Head and Neck Surgery, University of Rochester School of Medicine, NY 14642, USA.

Title

Large vestibular aqueduct syndrome with massive endolymphatic sacs. [Review]

Source

Otolaryngology - Head & Neck Surgery. 113(5):606-10, 1995 Nov.

Document 5 of 6

Document: 1 2 3 4 5 6

Date: Tue, 5 Nov 1996 13:44:00 -0500
Reply-To: A Practical Discussion List Regarding Deaf Education
Sender: A Practical Discussion List Regarding Deaf Education
Subject: Re: Enlarged Vestibular Aqueduct Syndrome (long one)
Comments: To: EDUDEAF@UKCC.uky.edu
To: Multiple recipients of list EDUDEAF

This is a syndrome that I did not previously know. Sorry it took me a couple of days, but I eventually got around to doing a literature search. Here are some recent papers as listed in Medline:

<1>

Unique Identifier

92230647

Authors

Mafee MF. Charletta D. Kumar A. Belmont H.

Title

Large vestibular aqueduct and congenital sensorineural hearing loss. [Review]

Source

Ajnr: American Journal of Neuroradiology. 13(2):805-19, 1992 Mar-Apr.

<2>

Unique Identifier

92015417

Authors

Arcand P. Desrosiers M. Dube J. Abela A.

Title

The large vestibular aqueduct syndrome and sensorineural hearing loss in the pediatric population.

Source

Journal of Otolaryngology. 20(4):247-50, 1991 Aug.

Abstract

Sensorineural hearing loss, associated with a dilated vestibular aqueduct, is often described as progressive. Since 1982, computed tomography of the mastoids has become part of the routine investigation of deaf children at Sainte-Justine Hospital. Using clinical, audiometric and radiological material from 130 patients with sensorineural hearing loss, we identified 18 patients with enlarged vestibular aqueducts. The large vestibular aqueduct population was then compared to the normal vestibular aqueduct patients in regard to the degree and evolution of the sensorineural hearing loss. Progression of hearing loss was noted in 46% of patients in the presence of large vestibular aqueducts as compared to 35% in the absence of this anomaly. Normal radiographic findings did not exclude the possibility of further hearing loss.

<3>

Unique Identifier

89076572

Authors

Levenson MJ. Parisier SC. Jacobs M. Edelstein DR.

Title

The large vestibular aqueduct syndrome in children. A review of 12 cases and the description of a new clinical entity. [Review]

Source

Archives of Otolaryngology -- Head & Neck Surgery. 115(1):54-8, 1989 Jan.

Abstract

The large vestibular aqueduct as an isolated anomaly of the temporal bone has been previously identified radiologically, and its association with sensory neural hearing loss has been recognized. It has not, however, been defined as a distinct clinical entity in children. We studied 12 children, ages 3 to 9 years, with downward-fluctuating progressive high-frequency neurosensory hearing losses whose symptoms were thought to be related to the isolated enlargement of the vestibular aqueduct identified by high-resolution computed tomographic scanning. Previously it had been assumed that a large vestibular aqueduct is a temporal bone dysplasia that is a variant of the Mondini type of deformity and that the associated hearing loss is congenital in nature. Our clinical observations, however, indicate that the hearing loss in children with an isolated enlargement of the vestibular aqueduct is acquired during childhood. The natural history of this progressive deafness is reviewed, and a pathophysiologic hypothesis is presented. [References: 12]

Leon A. Metlay, M.D.,Associate Professor of Pathology and Laboratory Medicine

Document 6 of 6

Document: 1 2 3 4 5 6

Date: Tue, 5 Nov 1996 16:34:27 -0800
Reply-To: A Practical Discussion List Regarding Deaf Education
Sender: A Practical Discussion List Regarding Deaf Education
Subject: Re: EVA journal references (long)
To: Multiple recipients of list EDUDEAF

Thanks Candy (Candace) and Leon Matlay, M.D.

I did read a couple of these articles and got some more summaries from NORD (Natl Organization for Rare Disorders http://www.NORD-RDB.com/~orphan) and NIDCD (Natl Institute on Deafness and Other Communication Disorders (800-241-1044). It was our county's school audiologist that provided me with most of the information that I know about EVA Syndrome. Next monday we have a drs visit with Dr Grundfast who co-authored one of the articles, studies you mentioned so I'm anxious to get his opinion, etc. I really appreciate the research you two did for me. Thanks a lot. It is comforting to know that there are people like you out here (netland) who can help.

Marcie

Uploaded by: Jessica Soltesz/Kent State University/Deaf Education Major