Sunday, 6 July 2014

Clinic Speak: MS-related hyperacusis

Have you ever had pain from hearing loud noises? Another MS related problem. #ClinicSpeak #MSBlog #MSResearch

"This is post is in response to a comment on hyperacusis in MS. A rare symptomatic problem in MS is hyperacusis, which refers to pain in response to hearing loud noises or specific sounds. The case series below is of three MSers who had different stimuli that generated hyperacusis. All these MSers had evidence of lesions affecting the auditory pathways in the brain-stem. In essence all these cases of hyperacusis represent a rare manifestation of a brain-stem relapse. Most symptoms associated with early relapses usually resolve as the lesion regresses. However, some MSers may be left with residual hyperacusis or intermittent hyperacusis when the damage pathway malfunctions due to fatigue or temperature-related changes. In my experience hyperacusis is more common in association with a so called facial nerve palsy; this occurs because a small muscle, called the stapedius muscle,  that tenses the ear drum is paralysed. The stapedius muscle tenses the ear drum as a reflex response to loud noises; by doing this it dampens the amount of sound that is transmitted to the inner ear to protect the sensitive hair cells from too much noise. Hyperacusis in the latter setting is easy to diagnose clinically it occurs in association with paralysis of the facial muscles on the side of the lesion. Facial muscle paralysis means you can't wrinkle your forehead, blow out your cheeks or smile. In this case it is relatively easy to diagnose."

"Hyperacusis is very difficult to treat in MS; you can either sit it out and wait for it to recover or you can  try different symptomatic therapies. There is some data suggesting that serotonin re-uptake inhibitors or SSRIs may help. If hypeacusis is intermittent you could try the usual drugs we use for over active nerves; gabapentin, pregabalin and sodium channel blockers (carbamazepine, oxcarbazepine, phenytoin, lamotrigine, etc.). If as in some of  the cases described below the hyperacusis is due to specific frequency you can get specialised acoustic lens or filters fitted that filter out sounds. These are specialist devices and need to be obtained through an audiologist."


Left facial palsy

"Should all MSers be investigated if they develop hyperacusis? Yes, there are other causes for this symptom and it is sufficiently rare in MS to warrant investigation to exclude other causes. How rare is it? I don't know but do you mind completing this short survey below to see? Thanks."


Weber et al. Central hyperacusis with phonophobia in multiple sclerosis. Mult Scler. 2002 Dec;8(6):505-9.

Background: Hearing disorders are a well-described symptom in MSers. Unilateral or bilateral hyperacusis or deafness in MSers with normal sound audiometry is often attributed to demyelinating lesions in the central auditory pathway. Less known in MS is a central phonophobia, whereby acoustic stimuli provoke unpleasant and painful paresthesia and lead to the corresponding avoidance behaviour.

Case studies: In our comparison collective, MSer 1 described acute shooting pain attacks in his right cheek each time set off by the ringing of the telephone. MSer 2 complained of intensified, unbearable noise sensations when hearing non-language acoustic stimuli. MSer 3 noticed hearing unpleasant echoes and disorders of the directional hearing. All MSers had a clinical brainstem syndrome. ENT inspection, sound audiometry and stapedius reflex were normal. All three MSers had pathologically changed auditory evoked potentials (AEPs) with indications of a brainstem lesion, and in magnetic resonance imaging (MRI) demyelinating lesions in the ipsilateral pons and in the central auditory pathway. The origin we presume in case 1 is an abnormal impulse conduction from the leminiscus lateralis to the central trigeminus pathway and, in the other cases, a disturbance in the central sensory modulation. All MSers developed in the further course a clinically definite MS. Having excluded peripheral causes for a hyperacusis, such as, e.g., an idiopathic facial nerve palsy or myasthenia gravis, one should always consider the possibility of MS in a case of central phonophobia. 

Conclusion: Therapeutic possibilities include the giving of serotonin reuptake inhibitors or acoustic lenses for clearly definable disturbing frequencies.

8 comments:

  1. Is there any bodily function which MS doesn't affect? It really is the cruellest disease and one of the most difficult to live with. Please refocus your efforts on repair. If you actually asked MSers what they wanted it would be regaining lost functione.g. Going from EDSS 4 to EDSS 2. For too long the focus of research has been about what interests researchers, not what MSers actually want.

    ReplyDelete
  2. Prof G. Hyperacusis is discussed in facial nerve palsy in MS. What about hyperacusis caused by a VI nerve palsy in MS? Would the VI nerve palsy have an affect on the stapedius muscle? thanks.

    ReplyDelete
    Replies
    1. Re: "What about hyperacusis caused by a VI nerve palsy in MS? Would the VI nerve palsy have an affect on the stapedius muscle?"

      The VIth or abducens nerve innervates the lateral rectus muscle that pull the eye outwards. The VIth cranial nerve does not innervate the stapedius muscle and hence does not cause hyperacusis. However, the VIth and VIIth (facial nerve) have their nuclei (nerve cell bodies) close together in the brain stem; hence a large MS lesion in the brain stem can cause both a 6th and 7th nerve palsy amongst other things and hence can be associated with hyperacusis.

      Delete
    2. Thanks for your reply. The 6th and 7th nerve plasy's can be quite involved MS symptoms it seems and may be best to treat promptly.

      Delete
    3. Phonophobia is really nasty I had this with a 6th nerve palsy. I'm seeing a clinical psycholgist/psychiatrist as it was so traumatic. Also the anxiety from the phonophobia really messed me up and contributed to my massive relapse.

      Delete
  3. The hyperacusis and hypervigilence I had with the VI nerve palsy was traumatic. I became so senstive to sounds especially at night. Unfortunately I had a housemate at the time who was directly below me and they would stay up until 4am, it was not fair on me as I was unwell. I went to bed at 10pm and I would lay awake anticipating these sounds and became an insomniac. I was suffering from lack of awareness of these at the time during the day so wasn't able to tell anyone. I had nasty anxiety attacks every night when I heard these noises for a month. I became very unwell in the head and had low blood pressure/a long blackout two days before my first MRI scan. I deteroirated very badly. I also woke up from something head shaking in my sleep (anxiety), teeth chattering in sleep (anxiety and/or neurological such as MS), sweating (anxiety) and sleep apnea (anxiety). When I went home after receiving my MRI results by phone it felt like my nervous system was in a very poor state with the extreme severe anxiety. I really could of done with some anti-anxiety drug. It just shows how some MSers may be ok with a VI nerve palsy while others can become very unwell. I was unaware of how unwell I was getting. The prolonged extreme anxiety, stress and lack of sleep triggered my following long severe relapse. It felt like the steroids reacted to the extreme anxiety. I guess there may have been a hidden infection may be. Brain to spine in 30 days. I had an unusual symptom I have not heard before it felt like my spine was straightening over and over about ten times. My neurologist doesn't want me to think about it but I am due to see a neuropsychiatrist soon as I have been diagnosed with PTSD. I hope to never go through that again.

    ReplyDelete
    Replies
    1. I think I also had phonophobia as this is anxiety in anticipating sounds or fear of sounds. I also became in insomniac when anticipating the sounds at night time.

      Delete
    2. Anonymous Sunday December 11th 2016 I also noise problem multiple voices in the room sounds music all of it everyday the only way to describe it it really really hurts my head a lot I was hoping when I look this up I would find some better news Ms is a very horrible disease and most people don't understand what we go through there needs to be more done for MS and for others to understand what we're going through thank you

      Delete

Please note that all comments are moderated and any personal or marketing-related submissions will not be shown.