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Audiology/Otolaryngology/Total Hearing loss in both ears

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Question
I am diagnosed with Multiple System Atrophy with Orthostatic Hypotension.  (1Yr into diagnosis) Last year August both ears were ringing 24/7.  End of August I lost all hearing.  Went to ENT doctor for testing.  Had comprehensive audiometer threshold evaluation.  Including evoked acoustic emissions testing, and tympanometry impedance testing.  All testing showed no hearing at all.  In January had ABR test.  Shows 120dB hearing loss in best ear.  My question is: Is this hearing loss related to the MSA disease that I have?  Neurologist Specialist guess that my brainstem might have shrank.  (Atrophy)  Any possible answer would be so appreciated.  I can take hearing tests all day, and nothing.  MRI tests, CT test, show everything to be normal. (ENG tests show balance and double vision results which helped in diagnosis of MSA).

Answer
Hi David,
It would be very difficult to directly connect your hearing loss with your MSA, and, in general, hearing loss is not listed as one of the "characteristic" symptoms.  However, in olivopontocerebellar MSA, those parts of the brain and brainstem which affect hearing and balance degenerate. And, since your loss is neural rather than structural, the damage would most likely not show up on imaging tests such as CT and MRI. Therefore, it would stand to reason that your neurologist is correct in his assumption.
I wish you well.
Hope this helped.
Harriet B. Jacobster, AuD
Board Certified in Audiology  

Audiology/Otolaryngology

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Harriet B Jacobster, Au.D.

Expertise

I am a Board Certified Doctor of Audiology with 25 years of experience. I can answer questions regarding hearing loss, both general and specific, dizziness and vertigo, auditory processing disorders, hearing aids, aural rehabilitation as well as specialized audiologic testing, i.e., Auditory Brainstem Response, VideoNystagmography, Otoacoutic Emissions. I have worked with both pediatric and adult populations. Because of my extensive musical background, I am able to respond to questions regarding the special needs of musicians.

Experience

I have been an audiologist for over 25 years with a specialty in electrophysiology of the auditory and vestibular systems and pediatric evaluations. I currently work in my private consulting practice providing services to nursing homes and schools. My Doctoral research was on specialized techniques in Auditory Brainstem Responses.

Organizations
American Academy of Audiology, - American Speech Language Hearing Association, - Hearing Loss Association of America

Education/Credentials
Au.D. (Doctor of Audiology), Nova Southeastern University, 2000. Board Certified Doctor of Audiology (American Board of Audiology) Certificate in Audiology from the American Speech-Language-Hearing Association

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