Central Annals of Otolaryngology and Rhinology

Case Report *Corresponding author

Sébastien Schmerber, Department of Oto-Rhino- Laryngology, Otology, Neurotology, Auditory implants, Multiple Sclerosis Revealed by Cochlear Implant Centre of the French Alps, University Hospital of Grenoble, CHU A. Michallon, BP 217 38043 Grenoble cedex 09 France, Tel : 33 4 76 76 56 62 ; Fax : Intrapontine Axial Lesion of 33 4 76 76 51 20; Email: Submitted: 11 April 2016 Peripheral Nerves Accepted: 01 August 2016 Published: 03 August 2016 Georges Dumas1, M. Filidoro2, Cindy Colombé1, and Sébastien ISSN: 2379-948X Schmerber1* Copyright 1Department of Otolaryngology-Head and Neck Surgery, Grenoble University Hospital, © 2016 Schmerber et al. France 2Department of ENT, CHR Chambery, France OPEN ACCESS

Abstract Keywords • Multiple sclerosis Multiple sclerosis(MS) is most often revealed by motor, sensitive symptoms with • Cranial nerves paresthesia, ocular symptoms, and more seldom by symptoms with rapidly installed • loss and vertigo.We report an observation with initialsymptoms of a peripheral • Facial palsyl pathology mimicking a meningo-neuritis. A 22 years old young woman was addressed • as anemergency for a sudden peripheral symptomatology associating a sudden right hearing loss with ear fullness,vertigo with vomiting and a right peripheral facial palsy. The patient had a spontaneous beating toward the left side suppressed by fixation and sensory neural hearing loss on low frequencies. The Fukuda tests initially deviated toward the right side. The caloric test showed a right hypofunction at 75% and a correlated consistent left preponderance. The head shaking test (HST) and skull vibration induced nystagmus test (SVINT) revealed a left nystagmus. Blood samples results andcervical vestibular evoked myogenic potentials (cVEMP) were normal. Brainstem evoked response (BERA) demonstrated a retro- cochlear disease with a Wave V prolonged latency on the right side. A CSF lumbar puncturerevealedaoligoclonal band on electrophoresis and the MRI a pons intra-axial nodular image on the VII and VIIIth nerve pathway in the immediate vicinity of the right vestibular nucleus (hyper signal T2 and T1 Gadolinium contrast fixation). The evolution surveyed in neurology was totally regressive. A further disease relapseconfirmed dissemination in time and location of the pathology. Conclusion: Inaugural vestibular symptoms are seldom in MS. The ensnaring initial pseudo-peripheral presentation of this case is explained by the plaquelocation in close vicinity on the pons intra-axial cranial nerves pathway route. BERA are essential to reveal a neuronal conduction impairment on auditory nerve related to a demyelination process.

INTRODUCTION CASE PRESENTATION

Multiple sclerosis (MS) described for the first time in 1868 A young woman of 22 years old, student in nursery, having no by Charcot [1] is a rather frequent pathology in young adults (45 peculiar medical history was addressed at the ENT consultation cases for 100,000 inhabitants), twice as frequent in women as by the Emergency Unit for a right unilateral hearing loss with ear in men,and constitutes 3 to 10% of the central nervous system fullnessand a vertigo associated with vomiting (one throw up). diseases [2- 4].This chronic inflammatory pathology of the The examination revealed a horizontal left beating spontaneous central nervous system (CNS) is more often initialized by motor nystagmus observed with the eye in median straight sighting or sensitive symptoms (45% of cases), visual symptoms (optic position and in the left sided sight (gaze)(degree 2 of Alexander neuritis, double vision, inter nuclear ophtalmoplegia are observed classification). This nystagmus was minimized with fixation. in 20% of cases) than by properly isolated cochleo-vestibular There was a delayed right peripheral facialth palsy (House- symptoms [4, 5].We report in this work a seldom presentation of Brackmannstage 2). The anamnesis revealed that 4 days ahead a first attack of MS disease associated with isolated audiological, the patient complained of paresthesia in the V nerve territory. vestibular and then facial symptoms mimicking a peripheral ENT The Fukuda-Unterberger test was initially deviated rightward. pathology. The audiogram showed a sensory neural hearing loss (SNHL) Cite this article: Dumas G, Filidoro M, Colombé C, Schmerber S (2016) Multiple Sclerosis Revealed by Intrapontine Axial Lesion of Peripheral Nerves. Ann Otolaryngol Rhinol 3(9): 1130. Schmerber et al. (2016) Email: Central

primarily on low frequencies (Figure 1). Fifteen days later the facial palsy disappeared and bedside examination tests revealed at the head shaking test (HST) a horizontal left beating nystagmus. The skull vibration induced nystagmus test (SVINT) showed a left beating horizontal nystagmus (Figure 2). Videonystagmographic(VNG) recordings (Figure 3) demonstrated at the caloric test a right hypofunction at 75%associated with a left nystagmic preponderance at 21°/sec. The cVEMP were normal with symmetric amplitudes on both sides andnormal thresholds at 90 dB HL. The BERA (recorded 3 weeks later)were demonstrative of a right retro-cochlear pathology. Wave V had a prolonged latency (6.7ms measured at 80 dB) and theinteraural I-V difference was 1.2 ms. (Figure 4). The MRI demonstrated a spontaneous hyper signal in T2 weighted sequence and a post gadolinium T1 weightedhypersignal(1cm diameter) on the right and anterior part of the pons (Figure 5). The Biological blood samples were normal including blood cells count, CRP,glycemia, creatinine,ionogram,neurotrope viral serologies (VZV, HSV, HIV, EBV,CMV), Lyme,toxoplasmosis, Syphilis tests, HLA and blood proteins electrophoresis. The lumbar puncture (LP) revealed anoligoclonal band on electrophoresis.Normal cytology (6 white cells/ml). Other biological Tests in the CSF (Lyme, Syphilis, toxoplasmosis) and PCR were negative and there was no cytologic sign of carcinomatous meningitis. Theinitial symptoms were suggestive of a cerebello-pontine angle syndrome mimicking initially a peripheral disease.The Figure 2 patient was initially treated in neurology with 1gr IV steroid treatment per day during 3consecutive days.The evolution Head shaking test and skull vibration induced nystagmus surveyed in neurology was totally resolute regressive 6 weeks test. later with recovery of hearing, facial palsy, disappearance of a. Slow phase velocity recording (SPV) of Head shaking Nystagmus vertigo.The electrophysiological explorations were normalized (HSN) and skull vibration induced Nystagmus Test (SVINT) at 30, 60 and 100Hz applied on right mastoid process (RM).The SPV (slow in the same time. A MRI control 5 months after onset showed a eye movement) is directed toward the lesion side as in a peripheral partial regression of the lesion (Figure 6). The long term evolution disease. was favorable. b. Direct tract of HSN. The nystagmus quick phase beats toward the intact side as in a peripheral disease. c. Direct tract of the vibration induced Nystagmus (VIN) at 100Hz. The VIN horizontal component beats toward the intact side. DISCUSSION

Multiple sclerosis refers to the numerous scars or sclerae, better known as “en plaque sclerosis” [1] that developon the white matter of the brain and spinal cord [6]. It is the most common autoimmune disorder affecting the CNS [7]. A genetic component of the disease has been demonstrated with a higher probability in developing the disease with relatives of an affected person [5]. In identical twins both are affected in about 30% of Figure 1 cases. The genes are linked with the HLA on chromosome 6 and alleles of the major histocompatibility complex defined as DR15 Tonal audiometry. The audiogram shows a right hearing loss and DQ6 [8]. predominant on the lower frequencies usually observed in hydrops. Ann Otolaryngol Rhinol 3(9): 1130 (2016) 2/6 Schmerber et al. (2016) Email: Central

Figure 3

Videonystagmography (VNG). The caloric test shows a right hypofunction of 75%. The caloric test shows a right hypofunction at 75% correlated with a left directional predominance B .The Sinusoidal rotatory test confirms a correlated Left directional predominance and the fixation index confirms the nystagmus suppression as in a peripheral disease. VVOR= Visuo-vestibulo-Ocular Figure 5 Reflex. VOR=Vestibulo-Ocular –Reflex. OF=ocular fixation (or gaze fixation) during rotation (the subject fixes his thumb at 50 cm in front MRI of him). a. There is a lesion in the right Pons with post Gd enhancement showing a demyelinated lesion (indicated by a circle L1). b. There is a hyper signal of the white matter on the right part of the pons visible on coronal and axial view.

Figure 6

MRI 5 months after onset The reduction of the size of the lesion is significant.

The criteria for MS have been described by McDonald 2005 Figure 4 [9], modified in2010 [10] and are based altogether on clinical and radiological criteria. The diagnosis of MS disease is based on Brainstem evoked response audiometry progression from onset: On the right side, the different waves appear desynchronized. Wave V is recognizable still to 70 dB and its latency is increased (6.7ms). 1) One year of disease progression (retrospectively or On the left side the all waves are identified with normal latencies. prospectively determined). The interaural I-V delay is 1.2 ms., suspecting a right retrocochlear pathology. 2) Plus two of the following: a) Positive brain MRI(nine T2 Ann Otolaryngol Rhinol 3(9): 1130 (2016) 3/6 Schmerber et al. (2016) Email: Central

infectious, inflammatory or tumoral.Meningo neuritis involving the same cranial nerves and responsible for painful shingle rash with facial palsy and possible hearing loss areobserved after Herpes Zoster oticus (Ramsay Hunt syndrome (RHS)). But other viral infections can reveal similar neural symptoms (Herpes simplex, Coxsackie, measles, mumps, rubella, EBV, varicella) [14]. Viral infections are more frequent than after bacterial infections (Lyme disease withborrelia bacteria) or Syphilis.The Ramsay Hunt suyndrome (RHS) is also calledSicard syndrome in European literature. Differential diagnosis depends on clinical symptoms at presentation [15,16]. Devic’sdisease (recently namedneuromyelitis spectrum disorder), neurosarcoidosis (MRI images are different), neurological manifestations of Lupus or Sjögren syndrome, Whipple disease, Favre disease. In MS, the lumbar puncture is positive in 75 to 85 % of cases with an oligoclonal band of IGG on electrophoresis which is significant of an inflammatory marker [9]. Figure (7) depicts why a “pseudo-peripheral” symptomatology of this lesion is observed since in our observation the pons plaque involvedonly the intra-axial course of the nerves (V, VII, VIII) and not their nucleusas observed. A vertigo and hearing impairment as inaugural symptom is unusual as in our observation.Pula and coll. (2013) report 4% of MS related with acute vestibular disorders as inaugural among a population of 170 patients examined for acute vertigo. Their Figure 7 7 cases of MS involved often theintrapontine 8th nerve fascicle [17]. Topography of the lesion The demyelinating lesion is represented by a grey oval shape at 2 Caloric test is modified in 63% of patients with MS [18]. levels of the pons th Unsteadiness and cerebellar symptoms with coordination A: Upper level of the pons: the lesion is located in the intrapontineth difficulties and balance disorders (ataxia) are more often s sensitive root of the V nerve m reported than acute pure rotatory vertigo [5]. V : sensitive root of the fifth nerve;th V : thmotor root of the V nerve; V4: fourth ventricule. B: Lower part of the pons. The lesion is located in the The modifications are more often described as an irritative intrapontine roots of the VII andVIII nerves without involvement type or pseudo-peripheral syndrome in 60% of these cases of the nuclei, explaining the pseudo-peripheral expression of this [18]. SVINT is modified in less than 30% of MS with brainstem central pathology symptoms but is not specific to a peripheral or central disease VI : sixth cranial nerve though its modifications are more often observed in peripheral pathologies [19-21]. lesions or four more T2 lesions with positive visual evoked Rare cases of sudden hearing loss have been related to MS potentials (VEP). b) positive spinal cord MRI (two focal and are signaled in 1% to 10% of cases [22]. Recurrent attacks T2 lesions.c) positive CSF (isoelectric focusing evidence of of bilateral SSNHL can be a warning sign of MS [23].Our case oligoclonal IgG bands or increased IgG index or both). with unilateral apparently peripheral symptoms is particularly The inaugural symptoms may be isolated and mono- ensnaring since at the very beginning the patient reported a low symptomatic in 85% of cases: most often sensitive or motor frequency hearing loss with a feeling of ear fullness as observed symptoms (45%), visual (20%), autonomic (urinary retention, in Hydrops like Menière’s disease. constipation) or ataxia (cerebellar) [5]. In 15% of cases the inaugural attack associates more than one neurological location BERA are essential to demonstrate a neuronal conduction [11]. alteration and are modified in 60% of MS cases associated with th brainstem symptoms [24,25]. BERA were the sole objective Inaugural Brainstem signs are observed in 12 to 22% examination to discriminate between central and peripheral of cases [11] and are most often represented by VI nerve pathology in our observation and were decisive to indicate the paresis. Inaugural isolatedth peripheral ocular motor (III, IV, need of a LP and MRI. VI) or facial mono neuritis may also occur in young patients. Trigeminal neuralgia (V nerve) has been reported in 2-3% The nervous system in MS may respond less actively to of cases with MS [12,13].The involvement of the Facial and stimulation sensory nerves such as the auditory nerve due to cochlea vestibular cranial nerves contributes to a cerebello demyelination on their pathways [25]. Visual or somesthesic pontine angle syndrome suggesting several etiologies such as evoked potentials may also be altered [26]. Ann Otolaryngol Rhinol 3(9): 1130 (2016) 4/6 Schmerber et al. (2016) Email: Central

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Cite this article Dumas G, Filidoro M, Colombé C, Schmerber S (2016) Multiple Sclerosis Revealed by Intrapontine Axial Lesion of Peripheral Nerves. Ann Otolaryngol Rhinol 3(9): 1130.

Ann Otolaryngol Rhinol 3(9): 1130 (2016) 6/6