Braverman I, Jaber L, Levi H, Adelman C, Arons K S, Fischel-Ghodsian N, Shohat M, Elidan J
Department of Otolaryngology-Head and Neck Surgery, Hadassah University Hospital, Jerusalem, Israel.
Arch Otolaryngol Head Neck Surg. 1996 Sep;122(9):1001-4. doi: 10.1001/archotol.1996.01890210073016.
To characterize the audiological and vestibular changes associated with a mitochondrial DNA mutation in an Arab-Israeli family and in other families with mitochondrial predisposition to aminoglycoside-induced hearing loss.
Evaluation of audiological (pure tone thresholds, speech reception thresholds, speech discrimination, tympanometry, acoustic reflex thresholds, tone decay, and auditory brain-stem evoked response recording) and vestibular (complete history, physical examination, and 2-channel electronystagmography) systems. In 5 patients, structural evaluation of the inner ear was done by magnetic resonance imaging.
Fifteen members of an Arab-Israeli family, and 1 Chinese woman with the same mitochondrial DNA mutation who experienced hearing loss after short-term exposure to streptomycin.
Most of the patients had a profound hearing loss due to cochlear involvement. The hearing loss usually was not accompanied by notable peripheral vestibular dysfunction. In the patient with severe hearing loss after exposure to aminoglycoside, the vestibular function was completely normal.
In most of the Arab-Israeli patients with congenital deafness, the vestibular system function was normal, in contrast to the frequency of vestibular abnormality among deaf children, which was described in the literature. This may be related to genetic predisposition to aminoglycoside-induced deafness.
描述一个阿拉伯裔以色列家庭以及其他有氨基糖苷类药物诱发听力损失线粒体易感性的家庭中，与线粒体DNA突变相关的听力学和前庭变化。
对听力学（纯音听阈、言语接受阈、言语辨别、鼓室图、声反射阈、音衰和听觉脑干诱发电位记录）和前庭系统（完整病史、体格检查和双道眼震电图）进行评估。对5例患者进行了内耳磁共振成像结构评估。
一个阿拉伯裔以色列家庭的15名成员，以及1名有相同线粒体DNA突变、短期接触链霉素后出现听力损失的中国女性。
大多数患者因耳蜗受累而有严重听力损失。听力损失通常不伴有明显的外周前庭功能障碍。在接触氨基糖苷类药物后出现严重听力损失的患者中，前庭功能完全正常。
与文献中描述的聋儿前庭异常发生率相比，大多数先天性耳聋的阿拉伯裔以色列患者前庭系统功能正常。这可能与氨基糖苷类药物诱发耳聋的遗传易感性有关。
