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Patients with neurological abnormalities on history and examination were further assessed by a neurologist to determine the type of disorder present. The frequency of auditory neuropathy spectrum disorder was 1. Sixty percent were found to have neurological involvement.
This included cerebral palsy in children, peripheral neuropathy PN , spinocerebellar ataxia, hereditary motor-sensory neuropathy, spastic paresis, and ponto-bulbar palsy. Neurological lesions did not present simultaneously with hearing loss in most patients. Sixty-six percent of patients with auditory neuropathy spectrum disorder were born of consanguineous marriages.
There is a high prevalence of neurological lesions in auditory neuropathy spectrum disorder which has to be kept in mind while evaluating such patients. Follow-up and counselling regarding the appearance of neuropathies is therefore important in such patients. A hereditary etiology is indicated in a majority of cases of auditory neuropathy spectrum disorder.
Figures - uploaded by Anjali Lepcha. Author content All content in this area was uploaded by Anjali Lepcha. Content may be subject to copyright. Patient profile with regards to neurological evaluation in the 21 patients. Visual evoked potential tracings showing bilateral anterior visual pathway dysfunction. Content uploaded by Anjali Lepcha.
Author content All content in this area was uploaded by Anjali Lepcha on Jun 12, Citations 0. References This research hasn't been cited in any other publications. Episodic and semantic memory in mild cognitive impairment. Little is known about episodic and semantic memory in the early predementia stage of Alzheimer's disease AD , which is referred to as mild cognitive impairment MCI.
A total of 75 subjects participated: As predicted, AD patients showed significant deficits in person naming, item recognition and recall of spatial location placing. Surprisingly, subjects with MCI were also impaired on all components. Analysis of the relationship between semantic naming and episodic recognition and placing components of the FPT revealed a significant association between the two episodic tasks, but not between episodic and semantic performance.
Patients with MCI show deficits of episodic and semantic memory. The extent of impairment suggests dysfunction beyond the medial temporal lobe. To establish that items in the adapted version are equivalent to that in the original. Most ACE items were retained. Full-text available.
Oct Bhagya Bhaskar. Consanguineous marriages are a common practice in the Middle East, Asian and African populations.
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Many studies have stated an association between first cousin marriages and the incidence of autosomal recessive diseases and congenital malformations. High frequency of consanguinity is reported by researchers among South India. The objectives of this study were to determine the prevalence and type of consanguineous marriages among the different communities of Mangalore.
This study was conducted on married women in Mangalore to investigate the prevalence and type of consanguineous marriages. All the women were interviewed personally using a structured questionnaire. Family pedigree was constructed to study the type of consanguineous marriages. A low percentage of consanguinity 6. Frequency of consanguinity between religions was highly significant.
Of these the most frequent were first cousin marriages Within Hindu religion the highest rate of consanguinity was among the Billavas There was no significant difference in the frequency distribution of the types of consanguinity between the religions and also between the different groups among Hindus. The frequency of consanguinity was found to be low in Mangalore.
The findings contradict with the earlier reports of high prevalence of consanguineous marriages in South India. Increased female education, increased socio-economic status and decrease in parental decisions in marriages may be the reasons. On renaming auditory neuropathy as auditory dys-synchrony. Jan Neurological dysfunction and axonal degeneration in Charcot-Marie-Tooth disease type 1A.
Jul Brain. Karen Krajewski. In order to understand further the molecular pathogenesis of CMT1A, as well as to determine which features correlate with neurological dysfunction and might thus be amenable to treatment, we evaluated the clinical and electrophysiological phenotype in 42 patients with CMT1A.
In these patients, muscle weakness, CMAP amplitudes and motor unit number estimates correlated with clinical disability, while motor NCV did not. In addition, loss of joint position sense and reduction in SNAP amplitudes also correlated with clinical disability, while sensory NCV did not. Taken together, these data strongly support the hypothesis that neurological dysfunction and clinical disability in CMT1A are caused by loss or damage to large calibre motor and sensory axons.
Auditory neuropathy spectrum disorder with Brown-Vialetto-Van Laere syndrome: Challenges in hearing rehabilitation. May J Laryngol Otol. Brown-Vialetto-Van Laere syndrome is a rare neurological disorder characterised by pontobulbar palsy and sensorineural hearing loss. Hearing rehabilitation continues to be a challenge because the exact lesion site is unknown.
We examined the clinical and audiological profiles of a case series comprising four siblings with Brown-Vialetto-Van Laere syndrome who had decreased hearing and poor speech discrimination. Audiological investigations revealed normal otoacoustic emissions with absent auditory brainstem responses and middle-ear reflexes in sensorineural hearing loss, suggestive of auditory neuropathy spectrum disorder.
The sensorineural hearing loss in Brown-Vialetto-Van Laere syndrome patients is a retrocochlear pathology resembling auditory neuropathy spectrum disorder, with the lesion being most probably of post-synaptic origin. The licentious pornstar connie a definitely knows how to handle a big cock and when getting roccos stuff at disposal she swallows it hardly and ends up with the handjob.
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