耳鼻咽喉科臨床
Online ISSN : 1884-4545
Print ISSN : 0032-6313
ISSN-L : 0032-6313
一側性感音難聴が初発症状の橋部に発生した髄芽腫例
佐藤 斎藤崎 俊之和田 匡史窪田 和泉 修司高橋 姿
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2007 年 100 巻 8 号 p. 617-622

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Medulloblastoma is the most common childhood primary central nervous system tumor. In adults, most medulloblastomas occur in patients younger than 40. Common symptoms are cerebellar dysfunction and signs of CSF flow obstruction.
This report describes the case of a 25-year-old female who presented with progressive unilateral sensorineural hearing loss (SNHL) as the first symptom of a medulloblastoma in the right inferior cerebellar peduncle and pons. She was referred to an ENT outpatient clinic, and diagnosed with right low tone-dominant SNHL. Her right SNHL progressed with an additional symptom, left moderate facial palsy, 7 months later, and she consequently visited the clinic again. She was introduced to our clinic for further examination. At that time, magnetic resonance imaging (MRI) revealed a right Brainstem lesion. The ipsilateral stapedial reflex was normal in the right ear but absent in the left. Bekesy audiometry was type III. Electronystagmography showed a spontaneous nystagmus to the left and saccade pursuit. The auditory brainstem response was absent in the right ear.
The presented case showed that unilateral SNHL can be the first symptom of a brainstem tumor lesion. Progressive SNHL cases should be checked by MRI, in order to exclude brainstem lesions just like cerebello-pontine angle lesions.
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