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1/68. Persistent bilateral hearing loss after shunt placement for hydrocephalus. Case report.

    Transient hearing decrease following loss of cerebrospinal fluid (CSF) has been reported in patients undergoing lumbar puncture, spinal anesthesia, myelography, and/or different neurosurgical interventions. The authors present the first well-documented case of a patient with persistent bilateral low-frequency sensorineural hearing loss after shunt placement for hydrocephalus and discuss the possible pathophysiological mechanisms including the role of the cochlear aqueduct. These findings challenge the opinion that hearing decreases after loss of CSF are always transient. The authors provide a suggestion for treatment.
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2/68. Large endolymphatic duct and sac syndrome--a case report.

    A 17-year-old girl with a history of hearing loss, presented with recurrent episodes of vomiting. CT scan revealed bilateral enlarged vestibular aqueducts and MR scanning confirmed the diagnosis of large endolymphatic duct and sac syndrome. This article looks into the anatomy and physiology of the endolymphatic duct and sac as well as possible explanations for the hearing loss associated with this syndrome.
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3/68. Unilateral sensorineural hearing loss and its aetiology in childhood: the contribution of computerised tomography in aetiological diagnosis and management.

    OBJECTIVES: The objective of this study was to identify factors correlated with the CT outcome and to examine the contribution of the CT scan in the aetiological diagnosis and management of unilateral sensorineural hearing loss in childhood. methods: The records of 35 consecutively investigated patients by the audiology Department of Great Ormond Street Hospital between January 1996 and June 1998 were reviewed. The CT results, population sample characteristics, initiation of further investigations after the CT results and management decisions based on the CT results were tabulated and analysed. RESULTS: In a series of 35 consecutively investigated children with unilateral sensorineural hearing loss, 11 CT scans were identified as abnormal. The CT findings were: labyrinthitis ossificans (3), unilaterally dilated vestibular aqueduct (2), bilaterally dilated vestibular aqueduct (2), unilateral deformity of the cochlea ('Mondini') (1), unilateral severe labyrinthine dysplasia (1), unilateral markedly narrow internal acoustic meatus (1), bilaterally dilated lateral semicircular canals (1). The presence of progressive hearing loss was a significant predictor of abnormal CT outcome, while the severity of hearing loss was not. The CT scans offered valuable information regarding the aetiological diagnosis in all cases and, in addition, prompted the appropriate vestibular rehabilitation in three cases, further investigations in four (with dilated vestibular aqueduct) and hearing preservation counselling in two (bilateral DVA) (seven out of 35 = 20%). CONCLUSION: All children with unilateral sensorineural hearing loss should have a CT scan of the petrous pyramids/IAMs performed at some stage, as not only aetiology but also prognosis and management of these cases may be significantly influenced by the CT outcome.
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4/68. Large vestibular aqueduct syndrome treated by hyperbaric oxygen.

    We report the case of a 14-year-old girl with a large vestibular aqueduct (LVA) in whom hyperbaric oxygen (HBO) therapy was effective for the treatment of sensorineural hearing loss. The patient was referred to Nagoya University Hospital for the treatment of hearing loss on 14 September, 1998, because her right hearing level had declined abruptly on 22 August, 1998, and had not changed for 3 weeks since then in spite of steroid and prostaglandin therapy. Her audiogram revealed bilateral profound deafness of more than 110 dB. She had had profound hearing loss on the left side since she was 9 years old. HBO therapy was performed on 22 occasions from 17 September until 19 October, 1998. During the HBO therapy, her right hearing ability returned almost to the level determined prior to the abrupt loss, 60 dB. We therefore recommend HBO therapy for the treatment of sensorineural hearing loss associated with large vestibular aqueduct syndrome if the hearing ability does not recover following conventional treatment.
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keywords = aqueduct
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5/68. Isolated large vestibular aqueduct syndrome in a family.

    This paper presents the second case in the literature of large vestibular aqueduct syndrome without associated cochlear anomalies in 2 members of the same family. The syndrome is frequently associated with sensorineural hearing loss presenting in childhood. The onset is commonly sudden, following an event causing increased intracranial pressure. On the basis of an emerging pattern of inheritance, we recommend screening siblings of an affected child. We also discuss the importance of characterizing the extent of disease of the inner ear.
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keywords = aqueduct
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6/68. hearing loss associated with large internal auditory meatus: a report of five paediatric cases.

    This paper describes the abnormality of a large internal auditory meatus (LIAM). Computed tomography (CT) scans show the otic capsule to be affected by a widened, bulbar internal auditory meatus with loss of or reduction of the bony wall dividing the lateral fundus of the meatus from the cochlea. The vestibule is abnormally dilated. We report five cases of children with LIAM and profound hearing loss. Three of these children are girls and two children were boys. Three had congenital progressive hearing loss, one of these had an accompanying large vestibular aqueduct and dysplasia of the cochlea. Two patients had had meningitis resulting in profound loss.
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keywords = aqueduct
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7/68. Traces of perilymph detected in epipharyngeal fluid: perilymphatic fistula as a cause of sudden hearing loss diagnosed with beta-trace protein (prostaglandin D synthase) immunoelectrophoresis.

    The incidence of perilymphatic fistula as cause of sudden hearing loss is not known. We present a case with sudden unilateral hearing loss associated with a positive beta-trace protein test of an epipharyngeal fluid sample. The patient presented with sudden sensorineural hearing loss on the right side. A stapedotomy had been performed nine months previously due to otosclerosis. Intravenous therapy for the treatment of sudden hearing loss was unsuccessful. At the time of sudden hearing loss, epipharyngeal fluid was collected using a Raucocel sinus pack. Investigation using rocket immunoelectrophoresis showed the presence of beta-trace protein. Upon repeating tympanoscopy there was no obvious labyrinthine fluid egress, but the oval window was sealed with fibrin sponge and fibrin glue. The patient's hearing improved over a period of five months.
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keywords = perilymphatic
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8/68. Clinical and molecular analysis of three Mexican families with Pendred's syndrome.

    BACKGROUND: The autosomal recessive Pendred's syndrome is defined by congenital sensorineural deafness, goiter, and impaired iodide organification. It is caused by mutations in the Pendred's syndrome (PDS) gene that encodes pendrin, a chloride/iodide transporter expressed in the thyroid, the inner ear, and the kidney. OBJECTIVE: To perform a detailed clinical and molecular analysis of patients with Pendred's syndrome from four patients from three unrelated Mexican families. methods: thyroid function tests, perchlorate test, thyroid scintigraphy, audiometry, computer tomography and magnetic resonance imaging were performed in all affected individuals. Haplotype analyses were performed using microsatellite markers flanking the PDS locus, and the PDS gene was submitted to direct sequence analysis. RESULTS: All patients presented with sensorineural deafness, Mondini malformations of the cochlea, an enlarged vestibular aqueduct, goiter, and a positive perchlorate test. Two patients were hypothyroid, two individuals were euthyroid. sequence analysis revealed a complex homozygous deletion/insertion mutation at the end of exon 4 in the index patient of family 1 resulting in a premature stop codon at position 138. In family 2, the affected individuals were compound heterozygous for a splice acceptor mutation (IVS2 -1G>A) and a 1231G>C transversion substituting alanine 411 by proline (A411P). In family 3, the index patient was found to be homozygous for a transversion 412G>T in exon 4 replacing valine 138 by phenylalanine (V138F). CONCLUSIONS: All patients included in this study presented with the classic Pendred syndrome triad and molecular analysis revealed pendrin mutations as the underlying cause. The identification of three novel mutations, one of them of complex structure, expands the spectrum of mutations in the PDS gene and emphasizes that they display marked allelic heterogeneity.
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9/68. Long-term audiological feature in Pendred syndrome caused by PDS mutation.

    Pendred syndrome is an autosomal recessive disorder characterized by profound deafness in childhood and goiter. We report a case of Pendred syndrome in a 27-year-old woman who had a diffuse goiter and progressive sensorineural hearing loss with fluctuation and a missense mutation (His723Arg) in the PDS gene identified in a homozygous state. Audiological findings were observed clinically over a 20-year period. Progressive hearing loss with fluctuation occurred before age 12 years. An enlarged vestibular aqueduct with enlargement of the endolymphatic duct and sac was confirmed with 3-dimensional magnetic resonance imaging hydrography.
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keywords = aqueduct
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10/68. Familial enlarged vestibular aqueduct syndrome.

    Enlarged vestibular aqueduct syndrome is a clinical disease entity associated with anatomic abnormality of the bony canal in the temporal bone containing the endolymphatic duct and sac. The definition of this syndrome is progressive sensorineural hearing loss with an isolated enlarged vestibular aqueduct. Familial inheritance of enlarged vestibular aqueduct syndrome is rare, and the correct mode of inheritance has not been discovered. This report is the study of familial inheritance with enlarged vestibular aqueduct syndrome. Clinical audiological, radiographic, and chromosomal analyses were performed in this case, which reports on two female probands who are offspring of normal parents. According to the study of pedigree, familial inheritance of enlarged vestibular aqueduct syndrome is strongly suspected as an autosomal recessive trait. Further study should be focused on discovering the genetic evaluation of familial inheritance of enlarged vestibular aqueduct syndrome.
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ranking = 10
keywords = aqueduct
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