114 Kulak Burun Bogaz Ihtis Derg 2016;26(2):114-117 Case Report / Olgu Sunumu B E H B U T C E V A N ŞİR K U LA K BU R U N B O Ğ A Z H A S T A L I K LA R I V E B A Ş B O Y U N C E R R A Hİ Sİ D E R N E Ğ İ . . doi: 10.5606/kbbihtisas.2016.71324 Unilateral sudden hearing loss: a rare symptom of Moyamoya disease Tek taraflı ani işitme kaybı: Moyamoya hastalığının nadir bir semptomu Fatih Gül, MD., 1 Sami Berçin, MD., 2 Togay Müderris, MD., 1 Gökhan Yalçıner, MD., 1 Özkan Ünal, MD., 2 Muzaffer Kırış, MD. 2 ABSTRACT A 38-year-old female patient experienced a sudden onset of unilateral sensorineural hearing loss due to Moyamoya disease. A detailed summary of audiological and neurological findings indicated that the sudden hearing loss might be due to Moyamoya disease resulting in occlusion of posterior and middle cerebral arteries. Intravenous prednisolone and trimetazidine dihydrochloride may improve hearing thresholds and speech understanding. To our knowledge, this is the first article in the literature reporting a case of sudden hearing loss as the first manifestation of Moyamoya disease in a young adult. Keywords: Hearing loss; Moyamoya; sudden; unilateral. ÖZ Otuz sekiz yaşında bir kadın hasta Moyamoya hastalığına bağlı olarak tek taraflı ani başlangıçlı işitme kaybı ile başvurdu. Odyolojik ve nörolojik ayrıntılı muayene ve tetkikler, posterior ve middle serebral arterlerin tıkandığını ve bunun Moyamoya hastalığından kaynaklanabileceğini gösterdi. İntravenöz prednizolon ve trimetazidin dihidroklorür işitme eşiklerini ve konuşmayı anlama skorlarını düzeltmede yardımcı oldu. Bildiğimiz kadarıyla, bu olgu sunumu Moyamoya hastalığının ilk belirtisi olarak ani işitme kaybı geçiren genç bir erişkini bildiren ilk yazıdır. Anahtar Sözcükler: İşitme kaybı; Moyamoya; ani; tek taraflı. Moyamoya disease is a chronic cerebrovascular disorder characterized by progressive stenosis or occlusion of bilateral internal carotid arteries with an extensive network of cerebral collaterals. The disease was first described in 1957 by Takeuchi and Shimizu. [1] In 1969, Suzuki and Takaku [2] named it Moyamoya which means puff of smoke that represents the characteristic reticulate appearance on angiography. The cause of Moyamoya disease is not known although there is evidence that the disease is hereditary. Its average annual incidence is 0.54 per 100,000 persons but it is the most common pediatric cerebrovascular disease in Far East Asia. [3] The reported incidence in the USA is approximately 0.086 per 100,000 patients. [4] The disease has a bimodal age distribution. The first peak occurs in childhood and cerebral ischemic events are more common in children. The second 1 Department of Otolaryngology, Atatürk Training and Research Hospital, Ankara, Turkey 2 Department of Otolaryngology, Medical Faculty of Yıldırım Beyazıt University, Ankara, Turkey Received / Geliş tarihi: April 14, 2014 Accepted / Kabul tarihi: October 27, 2015 Correspondence / İletişim adresi: Fatih Gül, MD. Atatürk Eğitim ve Araştırma Hastanesi Kulak Burun Boğaz Hastalıkları Kliniği, 06800 Bilkent, Ankara Turkey. Tel: +90 312 - 291 25 25 e-mail (e-posta): drfatihgul@gmail.com Available online at www.kbbihtisas.org doi: 10.5606/kbbihtisas.2016.71324 QR (Quick Response) Code