Acute Auditory Agnosia Resulted from MELAS Syndrome
10.3969/j.issn.1006-7299.2018.06.004
- VernacularTitle:急性听觉失认症与线粒体脑肌病
- Author:
Zhijian ZHANG
1
;
Mingwan ZHU
;
Baojun XIE
;
Hua LIAO
;
Yang JIANG
;
Tao ZHOU
;
Xilin YANG
;
Qingquan HUA
Author Information
1. 武汉大学人民医院耳鼻咽喉头颈外科 武汉430060
- Keywords:
Acute auditory agnosia;
Acute tinnitus;
MELAS syndrome;
Speech discrimination score;
MRI spectroscopy
- From:
Journal of Audiology and Speech Pathology
2017;25(6):571-574
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the clinical features,etiology,diagnosis and treatment of acute auditory agnosia.Methods We studied the clinical manifestation,diagnosis and treatment of acute auditory agnosia in a patient in our hospital.Results A 28 year oldyoung woman visited our department because she suffered from the tinnitus for 7 days and she could not distinguish the semantics for 1 day.There were no other abnormal symptoms in the central and peripheral nervous system on admission.Audiological testing showed normal,language testing showed that the speech discrimination score was zero.MRI showed extensive damage to temporal lope.MR spectroscopy revealed increased lactate and reduced N-acetyl aspartate.Acute auditory agnosia resulted from mitochondrial myopathy was considered.After symptomatic treatment,the symptoms were significantly improved.Molecular genetics examination showed the A3243G mtDNA mutation,further confirmed the diagnosis of mitochondrial encephalomyopathy with lactic acidosis and stroke like episodes (MELAS) syndrome.Conclusion Acute auditory agnosia and acute tinnitus can be the first symptoms in MELAS,thus,MELAS should be suspected in patients with acute auditory agnosia,acute tinnitus,sudden hearing loss in children and youth.Imaging examination plays an important role in the etiological diagnosis of acute auditory agnosia.
