1/114. 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.- - - - - - - - - - ranking = 1keywords = labyrinth, ear (Clic here for more details about this article) |
2/114. 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.- - - - - - - - - - ranking = 0.20484616109956keywords = inner ear, inner, ear (Clic here for more details about this article) |
3/114. Superior canal dehiscence syndrome.OBJECTIVE: To present the symptoms, signs, and findings on diagnostic tests of patients with the superior canal dehiscence syndrome and to describe the surgical procedures used to treat the dehiscence in five patients. DESIGN AND SETTING: Prospective study of a series of patients identified as having this syndrome at a tertiary care referral center. patients AND RESULTS: Seventeen patients with vertigo, oscillopsia, or both evoked by intense sounds or stimuli that caused changes in middle ear and/or intracranial pressure were identified over a 4-year period. The evoked eye movements had vertical and torsional components, with the direction corresponding to the effect of the stimuli in causing excitation (Valsalva against pinched nostrils, tragal compression, sounds) or inhibition (Valsalva against a closed glottis or jugular venous compression) of the affected superior semicircular canal. Thirteen (76%) of these patients also experienced chronic dysequilibrium that was often the most debilitating symptom. Dehiscence of bone overlying the superior semicircular canal on the affected side was confirmed with computed tomographic scans in each case. Surgical procedures through the middle fossa approach to plug or resurface the superior canal were performed in five patients (canal plugging in three cases and resurfacing of the dehiscence without plugging in two). The debilitating symptoms resolved or improved after the procedures. Signs of vestibular hypofunction, without loss of hearing, were noted in one patient after plugging of the superior canal and in one other patient after resurfacing of the canal. CONCLUSIONS: The superior canal dehiscence syndrome is identified based on characteristic symptoms, signs, and computed tomographic findings. The clinical presentation and findings can be understood in terms of the effect of the dehiscence on the physiology of the labyrinth. The syndrome is a treatable cause of vestibular disturbance.- - - - - - - - - - ranking = 0.4965474785031keywords = labyrinth, ear (Clic here for more details about this article) |
4/114. Vestibular hypersensitivity to sound (Tullio phenomenon): structural and functional assessment.OBJECTIVES: To establish the role of high-resolution CT imaging and tests of vestibulocollic reflexes in diagnosing and understanding the pathogenesis of the Tullio phenomenon. BACKGROUND: The Tullio phenomenon is a syndrome in which acoustic stimulation produces symptoms and signs of vestibular activation. It has previously been associated with an abnormally low threshold for click-evoked vestibulocollic responses and also with dehiscence of the roof of the anterior (superior) semicircular canal on high-resolution CT scans of the temporal bones. methods: High-resolution CT scans of the temporal bones and vestibulocollic responses in sternocleidomastoid to both clicks and transmastoid galvanic stimulation (3 mA/2 msec) were studied in four patients with the Tullio phenomenon (one bilateral). RESULTS: Click-evoked thresholds were low for all affected ears (four at 65 dB nHL, one at 55 dB nHL) and normal (>70 dB nHL) for the three unaffected ears. In contrast, galvanic-evoked vestibulocollic responses were symmetric and of normal size in all patients. The bony roof of the anterior (superior) semicircular canal was thin, possibly absent, on CT of all affected ears and also in two out of three unaffected ears. CONCLUSIONS: The normal galvanic vestibulocollic responses indicate that sound sensitivity in patients with the Tullio phenomenon is likely to occur distal to the vestibular nerve, probably at the level of the receptors. Both click hypersensitivity and dehiscence of the anterior (superior) semicircular canal are associated with the Tullio phenomenon but as the CT scan abnormality can occur in clinically unaffected ears, click testing is important for specific diagnosis. Abnormal sound sensitivity, as demonstrated by click responses, confirms that the radiologic abnormality is function significant.- - - - - - - - - - ranking = 0.0086313037422449keywords = ear (Clic here for more details about this article) |
5/114. Multiple cranial neuropathy in Cogan's syndrome.A 20 year old woman presented with recurrent alternative keratitis for four months. One month before admission, she developed progressive hearing loss, visual impairment, facial diparesis and bilateral trigeminal neuropathy. Cogan's syndrome was diagnosed. Prompt treatment with corticosteroid resulted in dramatic improvement of the ocular, otological and neurological dysfunctions.- - - - - - - - - - ranking = 0.0034525214968979keywords = ear (Clic here for more details about this article) |
6/114. Audiovestibular phenotype associated with a COL11A1 mutation in Marshall syndrome.BACKGROUND: Marshall syndrome is a dominant disorder characterized by craniofacial and skeletal abnormalities, sensorineural hearing loss, myopia, and cataracts, and is associated with splicing mutations in COL11A1. OBJECTIVE: To determine the auditory and vestibular phenotypes associated with a COL11A1 splicing. DESIGN: Clinical otolaryngologic, audiologic, vestibular, and radiologic evaluations of the auditory and vestibular systems. SUBJECTS: Three affected individuals from a family cosegregating Marshall syndrome and a COL11A1 splice site mutation. RESULTS: The study subjects have progressive sensorineural hearing loss that is predominantly cochlear in origin and asymptomatic dysfunction of the central and peripheral vestibular systems. Computed tomography detected no malformations of temporal bone structures. CONCLUSIONS: The observed auditory and vestibular abnormalities are not caused by defective morphogenesis of the osseous labyrinth, but by more direct effects of the COL11A1 mutation on the membranous labyrinth and the central nervous system. The onset and degree of hearing loss associated with COL11A1 mutations are useful clinical features to differentiate Marshall syndrome from the phenotypically similar Stickler syndrome.- - - - - - - - - - ranking = 0.98964243550931keywords = labyrinth, ear (Clic here for more details about this article) |
7/114. Relationship between cystic change and rotatory vertigo in patients with acoustic neuroma.Acoustic neuromas are benign neoplasms that most often arise from the vestibular nerve. Many patients with this tumor experience some degree of vestibular symptoms. However, patients rarely complain of rotatory vertigo. Acoustic neuromas are known to exhibit a cystic appearance in some patients. It was hypothesized that cystic change might be a causative factor for rotatory vertigo. A retrospective study of 178 patients with unilateral acoustic neuroma who underwent surgery in the Department of otolaryngology at tokyo Medical and Dental University was carried out. The cystic appearance of the tumors was detected preoperatively by magnetic resonance imaging and confirmed at surgery. The relationship between cystic change of the tumor and presentation with rotatory vertigo was examined. Of the 178 patients studied, only 10 had both cystic change of the tumor and rotatory vertigo, and 120 had neither cystic tumor nor rotatory vertigo. Of the remaining 48 patients, 24 experienced rotatory vertigo with negative findings for cystic tumor and the other 24 had evidence of cystic tumor but no rotatory vertigo. Tumor with cystic change was observed in 34 patients, accounting for 19.1% of all patients studied. Rotatory vertigo was also experienced in 34 patients (19.1%). Of the 34 patients with cystic lesions, 29.4% (10) had rotatory vertigo. The difference in percentage of the two groups did not reach statistical significance. It is suggested that there may be other factors causing rotatory vertigo in patients with acoustic neuromas than cystic change of the tumor.- - - - - - - - - - ranking = 0.0034525214968979keywords = ear (Clic here for more details about this article) |
8/114. dizziness and headache: a common association in children and adolescents.vertigo has long been recognized by the clinician as a frequent accompanying symptom of the adult migraine syndrome. This association has not been so readily identified in the pediatric population, and, as a consequence, children undergo unnecessary evaluations. We reviewed the charts of all children and adolescents referred for vestibular function testing to the Balance Center at the Barrow Neurological Institute between July 1994 and July 2000 (N = 31). Items analyzed included age, gender, symptoms that prompted the referral, test outcomes, family medical history, and final diagnosis. The most common justification for vestibular testing referral was the combination of dizziness and headache. Other less common reasons were "passing out" episodes, poor balance, and blurred vision. Normal test results were obtained from 70% of patients (n = 22). The most common abnormal test outcome was unilateral vestibular dysfunction (n = 5). Bilateral peripheral vestibular dysfunction was present in three patients. One patient had central vestibular dysfunction. The final diagnoses were vestibular migraine (n = 11), benign paroxysmal vertigo of childhood (n = 6), anxiety attacks (n = 3), Meniere's disease (n = 2), idiopathic sudden-onset sensorineural hearing loss (n = 1), vertigo not otherwise specified (n = 1), familial vertigo/ataxia syndrome (n = 1), and malingering (n = 1); in five patients, no definitive diagnosis was established. The stereotypical patient with vestibular migraine was a teenage female with repeated episodes of headache and dizziness, a past history of carsickness, a family history of migraine, and a normal neurologic examination. patients who fit this profile are likely to have migrainous vertigo. Consequently, a trial of prophylactic migraine medication should be considered for both diagnostic and therapeutic purposes. brain imaging and other tests are appropriate for patients whose symptoms deviate from this profile.- - - - - - - - - - ranking = 0.001726260748449keywords = ear (Clic here for more details about this article) |
9/114. The relationship between Usher's syndrome and psychosis with capgras syndrome.Usher's syndrome is a genetic disorder that causes congenital sensorineural hearing loss, visual impairment due to progressive pigmentary retinopathy, and, often, vestibular dysfunction. The aim of this article is to illustrate a case that clearly demonstrates psychotic symptoms in Usher's syndrome Type III and serves to increase clinical awareness of this disorder and its possible link to psychotic symptoms. There is some evidence in the literature of concurrent psychiatric symptoms, particularly psychotic symptoms, associated with Usher's syndrome, and several theories around this association have been proposed. These theories of associations include a genetic link between the genes responsible for schizophrenia and the genes for Usher's syndrome; a neuropathological explanation as radiologic studies have revealed that patients with Usher's syndrome have CNS abnormalities in multiple brain structures; and a sensory deficit model which proposes that the stressors associated with sensory impairment and the brain's adaptation to changes in sensory inputs place an individual at increased risk for psychopathology.- - - - - - - - - - ranking = 0.0034525214968979keywords = ear (Clic here for more details about this article) |
10/114. Cryptococcal meningitis: implications for the otologist.Cryptococcal meningitis can present to the otologist with hearing loss and vestibular dysfunction. A man with cryptococcal meningitis presenting with bilateral profound sensorineural hearing loss and vestibular dysfunction is described. The difficulty in arriving at the diagnosis and consequences of misdiagnosis are discussed. Histological and clinical studies suggest that the cochlear nerve is likely to be damaged in patients who have deafness associated with this disease. Retrocochlear damage may result in cochlear implantation having a poor outcome.- - - - - - - - - - ranking = 0.0086313037422449keywords = ear (Clic here for more details about this article) |
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