Sudden sensorineural hearing loss as presenting symptom of multiple sclerosis in a 15-year-old girl

Sudden sensorineural hearing loss as presenting symptom of multiple sclerosis in a 15-year-old girl

International Journal of Pediatric Otorhinolaryngology Extra (2006) 1, 97—99 www.elsevier.com/locate/ijporl CASE REPORT Sudden sensorineural hearin...

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International Journal of Pediatric Otorhinolaryngology Extra (2006) 1, 97—99

www.elsevier.com/locate/ijporl

CASE REPORT

Sudden sensorineural hearing loss as presenting symptom of multiple sclerosis in a 15-year-old girl G. Cadoni *, S. Agostino, S. Scipione, A. Cianfoni Departments of Otorhinolaryngology and Radiology, Catholic University of the S. Heart, Largo A. Gemelli 8, 00168 Rome, Italy Received 7 October 2005; accepted 17 November 2005

KEYWORDS Sudden hearing loss; Multiple sclerosis

Summary Multiple sclerosis (MS), a chronic inflammatory disorder of the central nervous system results in damage to axons and their surrounding myelin sheath. Sensorineural hearing loss (SNHL) is an uncommon symptom of MS. We describe the case of a 15-year-old girl with unilateral sudden hearing loss as presenting symptom of multiple sclerosis. # 2005 Elsevier Ireland Ltd. All rights reserved.

1. Introduction Multiple sclerosis is the most common neurologic demyelinating disease [1]. MS is a chronic inflammatory disorder of the central nervous system and results in damage to axons and their myelin sheath. Genetic and environmental factors, including infections, are implicated in the pathogenesis of MS, although the nature of the antigen stimulus remains unknown. MS can affect the brain, optic nerve and spinal cord, thus causing many neurological symptoms [2]. Sudden sensorineural hearing loss (SNHL) is rarely a presenting symptom in MS. Only 6% of MS patients initially complain of hearing loss, while brainstem or cerebellar signs, such as

* Corresponding author. E-mail addresses: [email protected], [email protected] (G. Cadoni).

vertigo, gait instability and ophthalmoplegia are present in 80% of reported cases. The cause remains uncertain and the treatment of hearing loss is controversial [3—5]. We describe the case of a young female in which a sudden SNHL was the initial symptom of MS.

2. Case report A 15-year-old girl presented with a 6 weeks history of right sudden hearing loss and subjective tinnitus without vertigo, otalgia, or headache. She was already affected by a congenital left severe SNHL of unknown aetiology. There were no history of ototoxicity, recent trauma, familiar hearing loss, viral infection or vaccine preceding disease up to 6 weeks before symptoms developed. Results of complete physical head and neck examination were unremarkable.

1871-4048/$ — see front matter # 2005 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.pedex.2005.11.002

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Fig. 1 Audiometry showed moderate flat sensorineural hearing loss on the right and severe left sensorineural hearing loss.

There was not focal neurologic findings (meningismus, encephalopathy, vomiting, fatigue, headache, fever and optic neuritis). A normal autoimmune profile was obtained and normal were the blood count, serum biochemical screen and urinalysis. Virological and microbiological tests for herpes simplex type 1, cytomegalovirus, influenza and parainfluenza, Epstein—Barr, Coxsackie, Hepatitis B and C viruses infection, Toxoplasma gondii and venereal disease resulted negative. Audiometry showed a mild right and a severe left flat SNHL (Fig. 1). Her eardrum and tympanometric test were normal. Acoustic reflexes (AR) were absent. The analysis of auditory brainstem responses (ABR) showed a significant increase in the right wave V latency. No vestibular dysfunction

G. Cadoni et al. was found. A combined regimen of steroids (methylprednisone 1 mg/kg/day), plasma expander (low molecular weight dextran 500 ml/day) and aspirin (100 mg/day) was administrated until an MR of the brain was performed. MR axial FSE T2-W image (Fig. 2A) through the posterior fossa demonstrated multiple lesions in the white matter of the pons, in the middle cerebellar peduncles and cerebellar hemispheres. One lesion was located in the pons, at the root entry zone of the right acoustic nerve. Sagittal T2-W FLAIR image (Fig. 2B) showed multiple high-intensity ovoid lesions in the white matter of the supratentorial compartment, typically located along the periventricular regions, distributed in a perivenular manner in the centrum semiovale and in the corpus callosum. These lesions were compatible with a demyelinating process that proved to be multiple sclerosis. CSF analysis showed positive oligoclonal bands. After diagnosis of MS, aspirin and plasma expander were discontinued, while tapered steroids were administrated for 1 month. Within 2 months after diagnosis, the right hearing had a total improvement and 12 months after her initial presentation with sudden SNHL, the patient had normal right hearing. She is no longer taking any drugs.

3. Discussion Hearing impairment is a rare initial or prominent symptom of MS. Hearing loss in MS has been characterised as unilateral or bilateral, acute, subacute or insidious, associated with or without tinnitus and vertigo, transient or permanent [3]. The occurrence of sudden SNHL has been estimated at about 1% of patients with MS [6].

Fig. 2 (A) Axial T2-W FSE image through the posterior fossa demonstrates multiple lesions in the white matter of the pons, in the middle cerebellar peduncles and cerebellar hemispheres. One lesion is located in the pons, at the root entry zone of the right acoustic nerve; (B) MR sagittal T2-W FLAIR image showed multiple high-intensity ovoid lesions in the white matter of the supratentorial compartment, typically located along the periventricular regions, distributed in a perivenular manner in the centrum semiovale and in the corpus callosum.

Sudden sensorineural hearing loss

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Table 1 Number of cases of sudden SNHL associated with multiple sclerosis in literature Patients with sudden SNHL Fischer et al. [7] Daugherty et al. [4] Franklin et al. [8] Drulovic et al. [9] Rodriguez-Casero et al. [6]

11 7 2 2 1

Few authors have previously reported cases of MS whose first clinical symptom was sudden SNHL. Fischer et al. [7] in a study of 705 adult MS patients found 11 cases who had unilateral acute hearing loss; Daugherty et al. [4] reported seven MS patients with unilateral or bilateral sudden SNHL; Franklin et al. [8] reported two subjects with MS and sudden SNHL; Drulovich et al. [9] reported two patients with acute hearing loss as the first and only manifestation of MS. Rodriguez-Casero et al. [6] described firstly a case of acute SNHL in a child (Table 1). Both AR and auditory brainstem-evoked responses measures have important implications for the diagnosis of MS. Previous studies have reported absent AR in patients with MS at one or more frequencies in 3— 70% of cases [10]. ABR abnormalities have been characterised by absence of waves, increased wave latencies, abnormal morphologic characteristics, reduced wave amplitude and prolonged interwave and interaural latencies. The absolute latency of wave V of the ABR is frequently prolonged and may show reduction of amplitude or may be absent in patients who do not exhibit clinical evidence of an auditory abnormality [11,12]. MS can only be diagnosed if there is radiological evidence of multiple hyperintense lesions on the corresponding T2-weighted sequences of magnetic resonance. MR findings of hyperintense lesions in periventricular regions (91%) and corpus callosum (64%) rather than of diffuse matter changes support the diagnosis of MS versus acute disseminated encephalomyelitis [13].

In conclusion, unilateral sudden sensorineural hearing loss can occasionally be diagnosed in association with multiple sclerosis when a demyelinating process occurs along the VIII nerve. Diagnostic imaging and, in particular, contrast MR of the brain is therefore, mandatory in all cases of SSHL patients, even if a specific cause of SSHL can be identified in only 10—15% of cases.

References [1] Demyelinating diseases, in: E. Braunwald (Ed.), Harrison’s principles of internal medicine, 11th ed., MacGraw Hill Book Company, New York, 1987, pp. 1995—1999. [2] H.P. Hartung, A. Bar-Or, Y. Zoukos, What do we know about the mechanism of action of disease-modifying treatments in MS? J. Neurol. 251 (Suppl.) (2004) 12—29. [3] V.G. Schweitzer, N. Shepard, Sudden hearing loss: an uncommon manifestation of multiple sclerosis, Otolaryngol. Head Neck Surg. 100 (1989) 327—332. [4] W.T. Daugherthy, R.J. Lederman, Hearing loss in multiple sclerosis, Arch. Neurol. 40 (1983) 33—35. [5] J.J. Shea, D.E. Brackmann, Multiple sclerosis manifesting as sudden hearing loss, Otolaryngol. Head Neck Surg. 97 (1987) 335—338. [6] M.V. Rodriguez-Casero, S. Mandelstam, A.J. Kornberg, et al., Acute tinnitus and hearing loss as the initial symptom of multiple sclerosis in a child, Int. J. Pediatr. Otolaryngol. 69 (2005) 123—126. [7] C. Fisher, F. Mauguiere, V. Ibanez, et al., The acute of definite multiple sclerosis: BAEP patterns, Electroencephalogr. Clin. Neurophysiol. 61 (1985) 7—15. [8] D.J. Franklin, N.J. Coker, H.A. Jenkins, Sudden sensorineural hearing loss as a presentation of multiple sclerosis, Arch. Otolaryngol. Head Neck Surg. 115 (1989) 41—45. [9] B. Drulovich, K. Ribaric-Jankes, V.S. Kostic, et al., Sudden hearing loss as the initial monosymptom of multiple sclerosis, Neurology 43 (1993) 2703—2705. [10] J. Jerger, Abnormalities of the acoustic reflex in multiple sclerosis, Am. J. Otolaryngol. 7 (1986) 163—176. [11] J.M.R. Furman, J.D. Durrant, W.L. Hirsch, Eighth nerve signs in a case of multiple sclerosis, Am. J. Otolaryngol. 10 (1989) 376—381. [12] G. Paludetti, F. Ottaviani, V. Gallai, et al., Auditory brainstem responses (ABR) in sclerosis multiple, Scand. Audiol. 14 (1985) 27—34. [13] S.D. Brass, Z. Caramanos, C. Santos, M.E. Dilenge, Y. Lapierre, B. Rosenblatt, Multiple sclerosis vs. acute disseminated encephalomyelitis in childhood, Pediatr. Neurol. 29 (2003) 227—231.