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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 22  |  Issue : 2  |  Page : 123-125

Alternating sudden sensorineural hearing loss in demyelinating disorders


1 Department of ENT-HNS, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
2 Department of ENT, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India

Date of Web Publication11-May-2016

Correspondence Address:
Rajeev Gupta
Indira Gandhi Medical College, Room No. 56, Resident Doctor Hostel, Shimla, Himachal Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-7749.182275

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  Abstract 

Multiple sclerosis (MS) is the most common demyelinating disease of the central nervous system. MS is a neuromotor disorder which progresses with remissions and relapsing periods. Symptoms of MS plaques may regress completely or heal by leaving sequelae. Symptomatology of MS may be very variable. These symptoms usually show variations depending on the localization of demyelinated plaques in the central nervous system. In this case, we are presenting a case of sudden sensorineural hearing loss in alternate ears with magnetic resonance images suggestive of demyelinating disorders. These hearing losses are improved completely as disease has remission and relapsing periods.

Keywords: Demyelinating disorder, Multiple sclerosis, Sudden sensory hearing loss


How to cite this article:
Gupta R, Mohindroo N K, Azad R. Alternating sudden sensorineural hearing loss in demyelinating disorders. Indian J Otol 2016;22:123-5

How to cite this URL:
Gupta R, Mohindroo N K, Azad R. Alternating sudden sensorineural hearing loss in demyelinating disorders. Indian J Otol [serial online] 2016 [cited 2019 Nov 22];22:123-5. Available from: http://www.indianjotol.org/text.asp?2016/22/2/123/182275


  Introduction Top


Multiple sclerosis (MS) is the most common demyelinating disease of the central nervous system. MS is a neuromotor disorder which progresses with remissions and relapsing periods. More than 2 million patients in the world are estimated to be diagnosed with MS.[1] MS is classified in four different groups by MS Association of America in 1996 as follows: (1) relapsing remitting, (2) secondary progressive, (3) primary progressive, and (4) progressive relapsing.[2] Symptoms of MS plaques may regress completely or heal by leaving sequelae. Symptomatology of MS may be very variable.

Hypoesthesia, muscle weakness, muscle spasms, ataxia, dysarthria, dysphagia, acute and chronic pain, and visual symptoms are common for the disease.[3] These symptoms usually show variations depending on the localization of demyelinated plaques in the central nervous system.

In the presence of demyelinated plaques affecting white matter of the right perisylvian area and white matter, sudden hearing loss may be encountered. In this article, we discuss MS case which was diagnosed based on symptoms of sudden hearing loss.


  Case Report Top


A 50-year-old male patient presented to ENT Outpatient Department (OPD) in Indira Gandhi Medical College, Shimla, with sudden hearing loss and tinnitus in the right ear since 4 days. There was no history of ear discharge, noise, and barotrauma. For which audiometric evaluation was done, showing pure tone average (PTA) threshold of 60 dbhl [Figure 1] sensorineural hearing loss (SNHL) in the right ear with normal hearing 13 dbhl in the left ear. He was admitted with diagnosis of sudden SNHL left ear and routine laboratory test was sent; there were no abnormal findings in tests. He was posted for magnetic resonance imaging (MRI) showing altered signal intensity foci in right perisylvian, right frontal area and medulla left showing T2-fluid attenuated inversion recovery (FLAIR) hyperintensity suggestive of demyelination features. He was on treatment as per guidelines of SNHL treatment as steroids, vasodilators, and valcivir along with oxygen therapy for 7 days. PTA was performed 3 times during hospitalization period with improvement continuously with the last PTA report showing 38 dbhl SNHL in the right ear. He was discharged with oral treatment and followed up. After 15 days of discharge, he showed complete recovery showing PTA report of 23 dbhl [Figure 2] normal hearing with high-frequency SNHL. He was continually followed up in both ENT and Neurology OPD. He was posted for MRI after 6 months for vertiginous feelings which showed grossly normal MRI report except with few T2 hyperintensities at right cerebral hemisphere without any hearing complaint. No active intervention was needed at that time and followed up regularly. He was again presented with to us after a year from the previous episode with an episode of sudden SNHL, interestingly this time in left ear. Audiometric evaluation was done which showed 43 dbhl [Figure 3] SNHL in left ear with normal right ear 15 dbhl. He was prescribed oral treatment and posted for MRI. This time, MRI showed the presence of new T2-FLAIR hyperintensity in centrum semiovale [Figure 4] B/L and medulla [Figure 5] right side along with old T2-FLAIR hyperintensity in subcortical white matter along a sylvian fissure on the right side and medulla suggestive of progressive demyelinating pathology. Neurology opinion was taken and antineutrophil cytoplasmic antibodies and cerebrospinal fluid examinations were done which showed demyelinating disorder, MS. He was put on treatment to prevent the progression of the disease. The audiometric evaluation was repeated after 7 days which showed improvement with 23 dbhl [Figure 6] in left ear with high-frequency loss. Till now, he was followed up regularly with no fresh complaints. Depending on these findings, his sudden hearing loss alternatively in both ears might be related to MS disease.
Figure 1: Pure tone average showing 60 dbhl in right ear

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Figure 2: Pure tone average showing improvement (23 dbhl) in right ear

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Figure 3: Pure tone average showing 43 dbhl sensorineural hearing loss in left ear

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Figure 4: Magnetic resonance images showing hyperintensity at centrum semiovale

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Figure 5: Magnetic resonance images showing hyperintensity at medulla

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Figure 6: Pure tone average showing improvement (23 dbhl) in left ear

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  Discussion Top


Sudden hearing loss (SNHL) is a hearing loss of 30 dB or more, over at least three contiguous audiometric frequencies, which develops over a period of a few hours to 3 days. The etiology of the disease is not certain, but autoimmune vascular malformations, thrombotic, and central nervous system diseases are among the main reasons.

MS is a demyelinating disease of the central nervous system that involves the white substance. The disease often has periods of remissions and relapses. Its etiopathogenesis is not totally understood. In genetically inclined patients, viral infection may trigger the autoimmunity that causes the disease.[4] Demyelinated plaques typically affect the periventricular white substance of the central nervous system. Rarely cerebellum, brain stem, and spinal cord can be affected by MS. As a result of autoimmunity, intravascular T-cells attack the myelin sheath and nerve fibers, and then it starts as an inflammatory process. On the other hand, neural regeneration begins to stop the damage at a minimum level. In 4–10% of MS patients, SNHL occurs during periods of relapse or remission.[5],[6] Detection of brain lesions of MS in MRI can provide evidence concerning the dissemination of MS lesions in space and time. Therefore, a diagnosis of MS should be guided by the defined criteria of MRI for this abnormality. Hearing loss in MS disease may be due to plaques which are placed on the brainstem, any area of entrance of cochlear nerve into the brainstem, and auditory cortex.[7]

If sudden hearing loss is related with MS, it usually recovers without sequelae. In a retrospective study of Hellman et al., 253 MS patients with SNHL were scanned, and sudden hearing loss was diagnosed in 11 of them. Nine of these patients recovered without audiological deficits.[8],[9]

In our patient, sudden hearing loss recovered with normal hearing and presented again with sudden SNHL but recovered and no recurrence was observed till follow-up.


  Conclusion Top


Whenever sudden hearing loss is diagnosed in a patient, physicians should request cranial imaging to differentiate for cranial pathologies such as MS. Sudden hearing loss can be the first symptom of MS or may indicate relapse of the disease.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Peterson JW, Trapp BD. Neuropathobiology of multiple sclerosis. Neurol Clin 2005;23:107-29, vi-vii.  Back to cited text no. 1
    
2.
Tiong TS. Prognostic indicators of management of sudden sensorineural hearing loss in an Asian hospital. Singapore Med J 2007;48:45-9.  Back to cited text no. 2
    
3.
Lublin FD, Reingold SC. Defining the clinical course of multiple sclerosis: Results of an international survey. National Multiple Sclerosis Society (USA) Advisory Committee on Clinical Trials of New Agents in Multiple Sclerosis. Neurology 1996;46:907-11.  Back to cited text no. 3
    
4.
Compston A, Coles A. Multiple sclerosis. Lancet 2008;372:1502-17.  Back to cited text no. 4
    
5.
Conlin AE, Parnes LS. Treatment of sudden sensorineural hearing loss: I. A systematic review. Arch Otolaryngol Head Neck Surg 2007;133:573-81.  Back to cited text no. 5
    
6.
Peyvandi A, Naghibzadeh B, Ahmady Roozbahany N. Neuro-otologic manifestations of multiple sclerosis. Arch Iran Med 2010;13:188-92.  Back to cited text no. 6
    
7.
McDonald WI, Compston A, Edan G, Goodkin D, Hartung HP, Lublin FD, et al. Recommended diagnostic criteria for multiple sclerosis: Guidelines from the international panel on the diagnosis of multiple sclerosis. Ann Neurol 2001;50:121-7.  Back to cited text no. 7
    
8.
Protti-Patterson E, Young ML. The use of subjective and objective audiologic test procedures in the diagnosis of multiple sclerosis. Otolaryngol Clin North Am 1985;18:241-55.  Back to cited text no. 8
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9.
Hellmann MA, Steiner I, Mosberg-Galili R. Sudden sensorineural hearing loss in multiple sclerosis: Clinical course and possible pathogenesis. Acta Neurol Scand 2011;124:245-9.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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