Auditory function in children with Charcot-Marie-Tooth disease

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Auditory function in children with Charcot-Marie-Tooth disease.

The peripheral manifestations of the inherited neuropathies are increasingly well characterized, but their effects upon cranial nerve function are not well understood. Hearing loss is recognized in a minority of children with this condition, but has not previously been systemically studied. A clear understanding of the prevalence and degree of auditory difficulties in this population is importa...

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Auditory Function in Patients with Charcot-marie-tooth Syndrome

The Charcot-Marie-Tooth (CMT) disease or hereditary motor and sensory neuropathy (HMSN) is a relatively common neurological syndrome, which has seldom been associated with hearing dysfunction, particularly sudden sensorineural hearing loss (SNHL). Families with autosomal dominant, autosomal recessive and X-linked forms of inheritance have been described. It strikes one or more neuronals systems...

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Charcot-Marie-Tooth disease

Charcot-Marie-Tooth (CMT) disease is the most prevalent peripheral inherited neuropathy (1/2500 to 10 000; 2.8/10 000 in Spain), and the mean age at onset is 16 years (range 2 to 50 years, but presentation in the early infancy and as late as the 80's has been reported). Patients present with motor and sensory polyneuropathic semiology (distal lower limb weakness and atrophy, gait abnormalities ...

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Charcot-Marie-Tooth disease

Charcot-Marie-Tooth (CMT) disease is the most prevalent peripheral inherited neuropathy (1/2500 to 10 000; 2.8/10 000 in Spain), and the mean age at onset is 16 years (range 2 to 50 years, but presentation in the early infancy and as late as the 80's has been reported). Patients present with motor and sensory polyneuropathic semiology (distal lower limb weakness and atrophy, gait abnormalities ...

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Charcot–Marie–Tooth disease: Genetics, epidemiology and complications

Background and aims: Charcot Marie Tooth disease (CMT) is the most prevalent hereditary neuropathy and its frequency is 1 in 2500. CMT is a heterogeneous disease and has different clinical symptoms. The prevalence of CMT and involved genes differ in different countries. CMT patients experience considerable sleep problems and a higher risk of decreased quality of life. In this w...

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ژورنال

عنوان ژورنال: Brain

سال: 2012

ISSN: 0006-8950,1460-2156

DOI: 10.1093/brain/aws085