티아민 보충으로 치료한 감각신경성 난청 1예
Sensorineural Hearing Loss Corrected by Thiamine Supply : A Case Report
0
조회
0
다운로드
1
인용
0
참고문헌
키워드
초록
Wernicke encephalopathy is a severe neurological disorder caused by thiamine (vitamin B1) deficiency. This disease occurs mostly in alcoholics due to malnutrition or malabsorption of vitamin B1 but also occurs in hyperemesis gravidarum, carcinoma, anorexia nervosa. Auditory neuropathy is characterized by abnormal auditory brainstem and preserved cochlear outer hair cell function. This is a case of Auditory neuropathy caused by thiamine deficiency. Brain MRI scan showed typical Wernicke encephalopathy finding. To evaluate hearing impairment, Pure tone audiogram, Speech audiogram, Otoacoustic emision and Auditory brainstem evoked response were measured and the result were compatible with auditory neuropathy. After thiamine replacement therapy, patient’s hearing imparment and serial speech recognition score showed improvement. From our study, we can assume that thiamine deficiency cause selective destruction of inner ear cell, this will eventually helps us to reveal pathophysiolology of auditory neuropathy
Abstract
Wernicke encephalopathy is a severe neurological disorder caused by thiamine (vitamin B1) deficiency. This disease occurs mostly in alcoholics due to malnutrition or malabsorption of vitamin B1 but also occurs in hyperemesis gravidarum, carcinoma, anorexia nervosa. Auditory neuropathy is characterized by abnormal auditory brainstem and preserved cochlear outer hair cell function. This is a case of Auditory neuropathy caused by thiamine deficiency. Brain MRI scan showed typical Wernicke encephalopathy finding. To evaluate hearing impairment, Pure tone audiogram, Speech audiogram, Otoacoustic emision and Auditory brainstem evoked response were measured and the result were compatible with auditory neuropathy. After thiamine replacement therapy, patient’s hearing imparment and serial speech recognition score showed improvement. From our study, we can assume that thiamine deficiency cause selective destruction of inner ear cell, this will eventually helps us to reveal pathophysiolology of auditory neuropathy
참고문헌 (16)
- So YT, "Deficiency diseases of the nervous system, In Bradley’s Neurology in Clinical Practice", pp.1340~1351 (2012), Elsevier Inc
- Victor M, "The Wernicke-Korsakoff syndrome. A clinical and pathological study of 245 patients, 82 with post-mortem examinations", Contemp Neurol Ser, 7, pp.1~206 (1971)
- Martin PR, "The role of thiamine deficiency in alcoholic brain disease", Alcohol Res Health, 27(2), pp.134~142 (2003)
- Togay-Isikay C, "Wernicke’s encephalopathy due to hyperemesis gravidarum: an under-recognized condition", Aust N Z J Obstet Gynaecol, 41(4), pp.453~456 (2001)
- Sininger YS, "Hearing loss due to auditory neuropathy", Audiol Today, 7, pp.10~13 (1995)
- Liberman MC, "Deletion of SLC19A2, the high affinity thiamine transporter, causes selective inner hair cell loss and an auditory neuropathy phenotype", J Assoc Res Otolaryngol, 7(3), pp.211~217 (2006)
- Victor M., "MR in the diagnosis of Wernicke-Korsakoff syndrome", AJR Am J Roentgenol, 155(6), pp.1315~1316 (1990)
- Simmons JL, "Auditory neuropathy: case study with hyperbilirubinemia", J Am Acad Audiol, 11, pp.337~347 (2000)
- Aydogdu I, "Bilateral transient hearing loss associated with vincristine therapy: case report", J Chemother, 12(6), pp.530~532 (2000)
- Starr A, "Auditory neuropathy", Brain, 119(Pt 3), pp.741~753 (1996)
- Fujikawa S, "Vestibular neuropathy accompanying auditory and peripheral neuropathies", Arch Otolaryngol Head Neck Surg, 126(1), pp.1453~1456 (2000)
- Rance G, "Clinical findings for a group of infants and young children with auditory neuropathy", Ear Hear, 20(3), pp.238~252 (1999)
- Zeng FG, "Temporal and speech processing deficits in auditory neuropathy", Neuroreport, 10(16), pp.3429~3435 (1999)
- Attias J, "Auditory system dysfunction due to infantile thiamine deficiency: long-term auditory sequelae", Audiol Neurootol, 17(5), pp.309~320 (2012)
- 박시영, "청신경종양에서 나타나는 청성뇌간반응 잠복기의 진단적 가치", 임상이비인후과, 21(1), pp.46~49 (2010), 대한이비인후과학회 부산,울산,경남 지부회
- Choung YH, "Clinical Characteristics of Vestibular Schwannoma Presenting as Sudden Hearing Loss", J Clinical Otolaryngol, 16(1), pp.54~59 (2005)