Hostname: page-component-cd9895bd7-8ctnn Total loading time: 0 Render date: 2024-12-24T02:19:31.495Z Has data issue: false hasContentIssue false
  • English
  • Français

Unilateral sudden hearing loss as the first presenting symptom of moyamoya disease

Published online by Cambridge University Press:  15 January 2013

L-S Tseng  and
S-D Luo
L-S Tseng
Affiliation:
Department of Otolaryngology, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan
S-D Luo*
Affiliation:
Department of Otolaryngology, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan
*
Address for correspondence: Dr Sheng-Dean Luo, Department of Otolaryngology, Kaohsiung Chang Gung Memorial Hospital, 123, Dapi Rd, Niaosong Dist, Kaohsiung City 833, Taiwan Fax: +886 7 7313855, E-mail: [email protected]; [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Objective:

We describe a rare case of sudden onset of unilateral sensorineural hearing loss occurring as the first symptom of moyamoya disease, which is characterised by progressive stenosis of the intracranial internal carotid arteries and their proximal anterior cerebral arteries and middle cerebral arteries.

Method:

Case report and review of the world literature regarding moyamoya disease with hearing loss.

Results:

The reported patient had moyamoya disease that initially presented as sudden, unilateral sensorineural hearing loss. Magnetic resonance imaging showed occlusion of the anterior cerebral, middle cerebral and distal internal carotid arteries bilaterally. The possible mechanism of this patient's sudden sensorineural hearing loss may have been vascular occlusion resulting from thrombotic narrowing or blockage by plaque.

Conclusion:

The described patient represents the first reported case of sudden onset, unilateral sensorineural hearing loss occurring as the first symptom of moyamoya disease. The possibility of a vascular lesion such as moyamoya disease should be considered in patients with sudden sensorineural hearing loss, especially children, young adults and Asian patients. Due to this disease's poor outcome, early diagnosis and treatment are important to prevent stroke.

Keywords

Hearing Loss, SensorineuralEar, InnerHearing Loss, SuddenMoyamoya Disease
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 127 , Issue 2 , February 2013 , pp. 196 - 199
Copyright
Copyright © JLO (1984) Limited 2013

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1Takeuchi, K, Shimizu, K. Hypoplasia of the bilateral internal carotid arteries. Brain Nerve 1957;9:37–43Google Scholar
2De Kleyn, A. Sudden complete or partial loss of function of the octavus system in apparently normal persons. Acta Otolaryngol 1944;32:407–29CrossRefGoogle Scholar
3Chau, JK, Lin, JR, Atashband, S, Irvine, RA, Westerberg, BD. Systematic review of the evidence for the etiology of adult sudden sensorineural hearing loss. Laryngoscope 2010;120:1011–21CrossRefGoogle ScholarPubMed
4Scott, RM, Smith, ER. Moyamoya disease and moyamoya syndrome. N Engl J Med 2009;360:1226–37CrossRefGoogle ScholarPubMed
5Uchino, K, Johnston, SC, Becker, KJ, Tirschwell, DL. Moyamoya disease in Washington State and California. Neurology 2005;65:956–8CrossRefGoogle ScholarPubMed
6Setzen, G, Cacace, AT, Eames, F, Riback, P, Lava, N, McFarland, DJ et al. Central deafness in a young child with moyamoya disease: paternal linkage in a Caucasian family: two case reports and a review of the literature. Int J Pediatr Otorhinolaryngol 1999;48:53–76CrossRefGoogle Scholar
7Escamilla, F, Espigares, A, Hervás, R, Fernández, MD, Vela, R, García, T. Fibromuscular dysplasia with moyamoya phenomenon in a patient with Alport's syndrome. A type IV collagen disorder [in Spanish]. Rev Neurol 2000;30:736–40Google Scholar
8Wakabayashi, Y, Nakano, T, Isono, M, Hori, S. Cortical deafness due to bilateral temporal subcortical hemorrhages associated with moyamoya disease: report of a case [in Japanese]. No Shinkei Geka 1999;27:915–19Google ScholarPubMed
9Rasmussen, H. Sudden deafness. Acta Otolaryngol 1949;37:65–70CrossRefGoogle Scholar