EDUDEAF: Vestibular Duct Syndrome

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

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Subj: vestibular duct syndrome any info
Date: 97-03-06 00:26:06 EST
From: randy@CYBERSPC.MB.CA (Randy)
Sender: EDUDEAF@LSV.UKY.EDU (A Practical Discussion List Regarding Deaf Education)
Reply-to: EDUDEAF@LSV.UKY.EDU (A Practical Discussion List Regarding Deaf Education)
To: EDUDEAF@LSV.UKY.EDU (Multiple recipients of list EDUDEAF)

Hello,

My name is Karen and I am a consultant (intinerent teacher for DHH) in Manitoba, Canada. A new student on my caseload has an etiology of hearing loss diagnosed as Vestibular Duct Syndrome. Both his audiologist and myself have been trying to access some information on this condition. Has anyone heard of this one before and be able to pass on some info. or direct me to a site that may be helpful? Thanking you in advance.

Karen

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Subj: Re: vestibular duct syndrome any info
Date: 97-03-06 01:46:20 EST
From: careyp@earthlink.net (Phillip & Susan Carey)
Sender: EDUDEAF@LSV.UKY.EDU (A Practical Discussion List Regarding Deaf Education)
Reply-to: careyp@earthlink.net
To: EDUDEAF@LSV.UKY.EDU (Multiple recipients of list EDUDEAF)

I found these related sites:

http://www.bme.jhu.edu/labs/chb/disorders/disorder.html

http://www.teleport.com/~veda/"

http://www.mayo.edu/vest-rehab/

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Subj: Re: vestibular duct syndrome any info
Date: 97-03-06 11:43:48 EST
From: wolfhub@GCOL.COM (Marcie)
Sender: EDUDEAF@LSV.UKY.EDU (A Practical Discussion List Regarding Deaf Education)
Reply-to: EDUDEAF@LSV.UKY.EDU (A Practical Discussion List Regarding Deaf Education)
To: EDUDEAF@LSV.UKY.EDU (Multiple recipients of list EDUDEAF)

When I was asking about enlarged vestibular aqueduct syndrome, C. Krepel gave me this info, so hope this helps.

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.

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