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Audiologic Findings in Charcot-Marie-Tooth Disease | JAMA ...
https://jamanetwork.com/journals/jamaotolaryngology/fullarticle/610121
• An audiologic study of a 54-year-old patient with Charcot-Marie-Tooth disease whose initial symptom was hearing difficulty is presented. While findings from basic audiologic tests were not consistent with the degree of auditory difficulty reported by the patient, special testing did indicate a notable deficit.
Detailed Audiological Evaluation of a Patient with ...
https://pubmed.ncbi.nlm.nih.gov/28054914/
Approximately 25% of patients also exhibit neural degeneration, which includes progressive mental deterioration, cortical thinning, and sensorineural hearing loss. Purpose: Herein, we describe the audiological and genetic findings in a patient with XP subtype D with neural degeneration and hearing loss. Research design: This is a case report of a patient with XP …
Hearing loss in Charcot-Marie-Tooth (CMT) disease …
https://n.neurology.org/content/84/14_Supplement/P5.071
Audiologic results were consistent with HL that is partly cochlear and partly neural in nature, particularly those with CMT1A. BAER waveforms were robust even in the absence of acoustic reflexes, except for two patients with CMT1B and one patient with CMT2E, who had abnormal BAER recordings that were not consistent with hearing thresholds.
Auditory function in children with Charcot–Marie–Tooth ...
https://academic.oup.com/brain/article/135/5/1412/310285
Auditory perception was also affected, with >60% of subjects with Charcot–Marie–Tooth type 1 and >85% of Charcot–Marie–Tooth type 2 suffering impaired processing of auditory temporal (timing) cues and/or abnormal speech understanding in everyday listening conditions.
This Disease Can Lead to Hearing Loss - Audiology Associates
https://www.audiologyassociates.com/hearing-loss-articles/this-disease-can-lead-to-hearing-loss/
There’s one particular condition, called Charcot-Marie-Tooth (CMT) disease, which can affect the nervous system on a fairly large scale, though the symptoms usually manifest primarily in the extremities. high-frequency hearing loss can also be triggered by CMT according to some evidence. What Is Charcot-Marie-Tooth Disease?
Charcot-Marie-Tooth Disease Fact Sheet | National ...
https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Charcot-Marie-Tooth-Disease-Fact-Sheet
Charcot-Marie-Tooth disease (CMT) is one of a group of disorders that cause damage to the peripheral nerves—the nerves that transmit information and signals from the brain and spinal cord to and from the rest of the body, as well as sensory …
Steroid-dependent sensorineural hearing loss in a patient ...
https://pubmed.ncbi.nlm.nih.gov/25440412/
Charcot-Marie-Tooth disease (CMT) is the most common form of hereditary sensorimotor neuropathy and sometimes involves disorders of the peripheral auditory system. We present a case of steroid-dependent auditory neuropathy associated with CMT, in which the patient experienced 3 episodes of acute exacerbation of hearing loss and successful rescue of …
Charcot-Marie-Tooth Disease | Johns Hopkins Medicine
https://www.hopkinsmedicine.org/health/conditions-and-diseases/charcotmarietooth-disease
Charcot-Marie-Tooth disease is an inherited nerve defect that causes abnormalities in the nerves that supply your feet, legs, hands, and arms. It affects both your motor and sensory nerves. Motor nerves carry signals from your brain to your muscles, telling them to move. Sensory nerves carry sensations, such as heat, cold, and pain back to your ...
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