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Auditory Neuropathy/HNPP
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Auditory Neuropathy/HNPP

I was wondering about the connection between auditory neuropathy and HNPP.  I was diagnosed with AN a year ago and recently been having episodes of my legs and arms falling asleep too easily.  My father had friedreich's ataxia and my uncle has it also.  There is some question about whether they were misdiagnosed.  Possibly HNPP? My youngest son may also have AN. Twice in the past year i woke up with my left arm completely numb and it felt very heavy.  It lasted about 2 hours.  I would like to find a neurologist at the Cleveland Clinic.  Do my symptoms seem to warrant an examination?  Thank you.
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I looked through the medical literature for an association as I personally have not seen hearing loss in patients with HNPP, and there were no cases reported - which doesn't necessarily mean there isn't a connection.  However, hearing loss in Friedrich's ataxia is well known.  From the description of your symptoms and family history, I suspect you do have some kind of hereditary neuropathy.  A formal neurology examination as well as EMG testing are definitely warranted for you and possibly your son. If it is Friedrich's ataxia, then this could have tremendous health implications down the line regarding your heart. There are a multitude of other hereditary neuropathies that can cause these symptoms too like Charcot Marie Tooth disease.  Fortunately, there is genetic testing (simple blood test) available for many of these diseases including HNPP and Friedrich's ataxia. Talk to your doctor about getting a referral. The top neuromuscular specialists at the Cleveland Clinic are Bob Shields and Kerry Levin, both of whom are kind and compassionate physicians and are well respected in their field. Best of luck.
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A related discussion, HNPP treatment was started.
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