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Biomedical subjects

L M Luxon

Publications and source records attributed to L M Luxon.

At least 37 records · Page 2Linked to original sources
PubMed1998

Vestibular hypersensitivity to clicks is characteristic of the Tullio phenomenon.

OBJECTIVES: The frequency of pathologically reduced click thresholds for vestibular activation was explored in patients with the Tullio phenomenon (sound induced vestibular activation). METHODS: Seven patients (eight affected ears) with symptoms of oscillopsia and unsteadiness in response to loud external sounds or to the patient's own voice were examined. In all but one patient, vestibular hypersensitivity to sound was confirmed by the fact that eye movements could be produced by pure tones of 110 dB intensity or less. Conventional diagnostic imaging was normal in all cases and three of the patients had normal middle ears at surgical exploration. Thresholds for click evoked vestibulocollic reflexes were compared with those of a group of normal subjects. Galvanic stimulation was used as a complementary method of examining the excitability of vestibular reflexes. RESULTS: All the patients showed a reduced threshold for click activation of vestibulocollic reflexes arising from the affected ear. Short latency EMG responses to clicks were also present in posterior neck and leg muscles, suggesting that these muscles receive vestibular projections. Galvanic stimulation produced a normal pattern of body sway in four of the five patients tested. CONCLUSIONS: A pathologically reduced threshold to click activation (< or = 70 dB NHL (average normal hearing level)) seems to be a consistent feature of the Tullio phenomenon and a useful diagnostic criterion. This in turn is most likely to be due to an increased effectiveness of the transmission of sound energy to saccular receptors. Activation of these receptors probably contributed to the vestibular symptoms experienced by the patients.

Acoustic Stimulation↗
PubMed1997

Large endolymphatic sac. A congenital deformity of the inner ear shown by magnetic resonance imaging.

Fluctuant and progressive hearing impairment in a patient with a wide vestibular aqueduct has been called the 'large vestibular aqueduct syndrome'. Recently reports of magnetic resonance imaging (MRI) studies describe enlargement of the endolymphatic sac and duct in patients shown to have large vestibular aqueducts by computed tomography (CT). A patient with progressive deafness was shown to have borderline or slightly enlarged vestibular aqueducts by re-formatted sagittal CT. However, MRI in axial and sagittal planes gave a more satisfactory demonstration of both aqueduct and endolymphatic sac enlargement.

Adult↗
PubMed1997

Migraine-associated vertigo.

A retrospective analysis was performed on a consecutive series of 363 patients presenting with vertigo; 32% had migraine. Of the 224 patients with no pathology other than migraine or vestibular dysfunction, migraineurs had a significantly higher prevalence of normal, central, and combined central and peripheral vestibular dysfunction compared to non-migraineurs. The combination of central and peripheral vestibular signs was a feature of migraine with aura. The results support the hypothesis that migraine-associated vertigo is a diagnostic entity.

Adult↗
PubMed1997

Pendred syndrome--100 years of underascertainment?

Pendred syndrome is an autosomal recessive condition classically characterized by deafness and goitre. Since both cochlear and thyroid pathology are required to secure the diagnosis, it is unclear whether the condition might present without the classical features. The perchlorate discharge test, the gold-standard investigation for Pendred syndrome, is non-specific, and in the absence of alternative means of confirming the diagnosis, its sensitivity is unknown. We used the recent mapping of the gene to chromosome 7q to identify pedigrees with a likely diagnosis of Pendred syndrome, and assessed the prevalence of clinical parameters of disease in affected patients. Thirty-six familial cases showed co-segregation between disease and the Pendred syndrome locus on chromosome 7q. Clinical and investigative findings were compared in index cases (n = 18) vs. affected siblings (n = 18). The overall prevalence of goitre was 73%, higher in index cases (94%) than in siblings (56%), many of whom had not previously been considered to have the condition. One perchlorate discharge test was false-negative (2.9%). Radiological malformations of the cochlea were identified in 86% of cases. Securing a diagnosis of Pendred syndrome may be difficult, especially in the single case. The perchlorate discharge test, although valuable, is difficult to undertake in the younger patient, and radiology may assist in diagnosing such patients.

Adolescent↗
PubMed1997

Pendred syndrome: evidence for genetic homogeneity and further refinement of linkage.

Pendred syndrome is the association between congenital sensorineural deafness and goitre. The disorder is characterised by the incomplete discharge of radioiodide from a primed thyroid following perchlorate challenge. However, the molecular basis of the association between hearing loss and a defect in organification of iodide remains unclear. Pendred syndrome is inherited as an autosomal recessive trait and has recently been mapped to 7q31 coincident with the non-syndromic deafness locus DFNB4. To define the critical linkage interval for Pendred syndrome we have studied five kindreds, each with members affected by Pendred syndrome. All families support linkage to the chromosome 7 region, defined by the microsatellite markers D7S501-D7S523. Detailed haplotype analysis refines the Pendred syndrome linkage interval to a region flanked by the marker loci D7S501 and D7S525, separated by a genetic distance estimated to be 2.5 cM. As potential candidate genes have as yet not been mapped to this interval, these data will contribute to a positional cloning approach for the identification of the Pendred syndrome gene.

Adolescent↗
PubMed1997

Abnormal audiograms and elevated acoustic reflex thresholds in obligate carriers of autosomal recessive non-syndromic hearing loss.

Pure-tone audiograms and acoustic reflex thresholds were obtained in 24 presumed obligate carriers of autosomal recessive non-syndromic hearing loss and 30 sex and age appropriate control subjects, with a view to evaluating the prevalence of abnormalities on these tests in the two groups, and a possible link between the findings on the two tests, which may help to localize threshold deficits and/or abnormal configurations to different sections of the reflex arc. Six (25%) of the carriers and one control subject had abnormal audiograms, inferred to be of genetic aetiology through careful exclusion of environmental risk factors. Four additional carriers had acoustic reflex threshold abnormalities. None of the carriers had an abnormality on both tests. The audiometric configurations and acoustic reflex patterns of abnormality were diverse, and may be a reflection of the genetic heterogeneity in ARNSHL.

Adult↗
PubMed1997

Latency of contralateral sound-evoked auditory efferent suppression of otoacoustic emissions.

The suppression of transiently evoked otoacoustic emissions by contralateral sound stimulation is thought to occur as a result of the action of the efferent pathway from the superior olivary complex to the cochlea via the medial olivo-cochlear neurons. The purpose of this study was to determine the time taken for this pathway to activate the suppressive mechanism in response to contralateral sound in normal human subjects. The time for onset of suppression was found to be between 7 and 20 ms.

Acoustic Stimulation↗
PubMed1996

Misdiagnosis of hearing loss due to ear canal collapse: a report of two cases.

Collapse of the external auditory meatus during audiometry can lead to spuriously increased hearing thresholds being obtained, particularly at high frequencies, and may simulate conditions such as noise-induced hearing loss, presbyacusis and retrocochlear pathology. Consequently, inappropriate investigations and management may be undertaken. Two patients with elevated thresholds secondary to ear canal collapse are described. The implications of initially failing to identify the true nature of their 'hearing losses' are highlighted and strategies to avoid such pitfalls are discussed.

Adult↗
PubMed1996

Dips on Békésy or audioscan fail to identify carriers of autosomal recessive non-syndromic hearing loss.

Békésy and audioscan sweep audiometry tests were carried out in 24 presumed obligate carriers of autosomal recessive non-syndromic hearing loss and 30 sex and age appropriate controls, with a view to defining the most expedient criteria for dips on either test in respect of possible carrier detection. On Békésy, dips with a minimum depth of 22.5 dB generated the greatest difference between carriers and non-carriers, while on audioscan, the criterion of a minimum dip depth of 15 dB provided the best discriminant. Using these criteria, the prevalence of dips was also evaluated in 8 unaffected siblings and 24 age appropriate control subjects. The findings both in the adult and the paediatric groups do not support the hypothesis that the presence of dips, either on Békésy or audioscan, is linked to the carrier state in ARNSHL.

Acoustic Impedance Tests↗
PubMed1995

Dizziness following head injury: a neuro-otological study.

Dizziness is a frequent and debilitating complications of head injury and accounts for increasing numbers of medico-legal claims. A detailed neuro-otological study was carried out from the records of 100 patients with post-traumatic dizziness to explore the neuro-otological basis of their symptoms: 50 patients presenting for medico-legal purposes (group I) and 50 presenting for management of their vestibular symptoms (group II). The two groups showed a similar sex distribution, a similar range of causes of head injury and similar severity of head injury (72 minor, 24 moderate and 4 severe). Of the 100, 88 showed at least one audio-vestibular abnormality on testing. Vertigo of the benign positional paroxysmal type was the commonest vestibular diagnosis in both groups (61/100), and only 8 patients showed central vestibular abnormalities. Fifty-three patients had audiometric abnormalities attributable to the head injury, the commonest of which was a high-tone sensorineural hearing loss. There was no significant difference in the incidence of any of the abnormalities in the medico-legal group (group I) when compared with the symptom management group (group II). The results provide strong evidence for an organic basis to recurring dizziness after head injury, whether or not a claim for compensation is pending, and emphasize the need for specialist neuro-otological investigation if abnormalities are to be identified and managed correctly.

Adult↗
PubMed1995

Relationship between balance system function and agoraphobic avoidance.

There is a striking similarity between the situations avoided by people with agoraphobia and the environments which provoke disorientation in people with organic balance disorders. This study investigated the possibility that agoraphobia might be linked to balance system dysfunction by comparing the results of balance system tests in 36 people with symptoms of panic and agoraphobia and 20 normal controls. A traditional battery of audiovestibular tests was supplemented with moving platform posturography, which assesses the postural instability induced by disorienting perceptual conditions. Subjects also completed questionnaire measures of somatic symptoms of dizziness and anxiety, agoraphobic cognitions, avoidance behaviour and state anxiety. Over 60% of the Ss with symptoms of panic and agoraphobia were destabilised by the disorienting perceptual conditions, compared with just 10% of the normal controls. Postural instability was strongly related to reported agoraphobic avoidance (r = 0.63, P < 0.01), even after controlling for symptoms, anxiety and agoraphobic cognitions. In our discussion we consider alternative interpretations of these findings, future directions for research, and implications for therapy.

Adult↗
PubMed1995

Audiovestibular complications of non-otological surgery.

Post-operative dizziness or imbalance is often regarded as 'trivial' and self-limiting and vaguely ascribed to the drugs used in anaesthesia or to haemodynamic changes. While hearing loss after anaesthesia has been documented in several studies, very little attention has been paid to a possible vestibular cause for post-operative dizziness. Twelve patients with post-operative vestibular disorders, with or without concomitant hearing loss, and one patient with tinnitus are described. As effective management for vestibular dysfunction exists, the importance of identifying this potential cause of dizziness is emphasized.

Adult↗
PubMed1995

Bilateral loss of vestibular function.

The clinical findings in 53 patients with bilateral vestibular failure (BVF) seen in a neurological hospital are reported. Bilateral acoustic neuromas were excluded. Seven patients (13%) had progressive cerebellar degeneration; these patients had no hearing complaints but showed gait ataxia, abnormal eye movements and cerebellar atrophy on neuro-imaging. Referral in these patients was primarily for eye movement assessment, and BVF was usually unsuspected. Neuropathies were present in 5 patients (9%), usually with normal central (brainstem-cerebellar) ocular motor function and variable patterns of hearing loss. The single largest group was idiopathic BVF (11 patients, 21%), patients presenting with vertiginous episodes, progressive unsteadiness or brief paroxysms of oscillopsia; auditory function, eye movements, neurological examination and imaging were usually normal. Nine patients (17%) suffered ototoxicity, mostly due to gentamicin; hearing was normal or mildly impaired. In 6 patients (11%) BVF was post-meningitic, with concomitant auditory loss. Autoimmune disease was found in 5 patients (9%); other organs were involved by the disease, and hearing was impaired but eye movements were spared. Miscellaneous neurological, otological or neoplastic diseases accounted for the remaining 10 patients. This study suggests that i) in patients with cerebellar degenerations, BVF may be underdiagnosed as the unsteadiness may be attributed only to the cerebellar disorder, ii) some patients with idiopathic BVF present with only minor visual or vestibular symptoms, and iii) detailed immunological screenings should be undertaken more often, in view of the significant proportion of patients with autoimmune and idiopathic BVF.

Caloric Tests↗