A child with severe inner ear malformations with favorable hearing utilization and balance functions after wearing hearing aids

Yusuke Kimura; Takeshi Masuda; Akifumi Tomizawa; Hideaki Sakata; Kimitaka Kaga

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A child with severe inner ear malformations with favorable hearing utilization and balance functions after wearing hearing aids

机译：戴着助听器的严重内耳畸形儿童，其听力利用率和平衡功能良好

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Infants with congenital deafness caused by severe bilateral inner ear malformations frequently suffer from severe hearing loss and poor balance. Unfortunately, the use of hearing aids is usually ineffective in recovering hearing, necessitating cochlear implants. We report a case of a 6-year-old boy with congenital deafness and bilateral inner ear malformations (right side, incomplete partition type I [IP-I];left side, common cavity deformity). Hearing aids had a remarkable effect in this patient, enabling sufficient and favorable hearing recovery such as to allow the patient to engage in daily conversations. Per-rotatory nystagmus was recorded on an electronystagmogram for both right and left rotations in a damped rotational chair test. It is rare for deaf children with severe bilateral inner ear malformation to demonstrate favorable development in hearing and good equilibrium function. Our findings suggest that auditory—vestibular hair cells in this patient may have been partially preserved despite IP-I in the right ear and common cavity deformity of the left ear. Copyright ? 2017, PLA General Hospital Department of Otolaryngology Head and Neck Surgery. Production and hosting by Elsevier (Singapore) Pte Ltd. This is an open access article under the CC BY-NC-ND license.

机译：由严重的双侧内耳畸形引起的先天性耳聋的婴儿经常患有严重的听力损失和平衡不良。不幸的是，助听器的使用通常不能有效地恢复听力，因此需要植入人工耳蜗。我们报告了一例6岁男孩，患有先天性耳聋和双侧内耳畸形（右侧，不完全I型分区[IP-I];左侧，常见的腔畸形）。助听器在该患者中具有显着效果，可实现充分而有利的听力恢复，从而使患者能够进行日常对话。在阻尼旋转椅测试中，左右旋转的每张眼震记录在眼震图上。患有严重双侧内耳畸形的聋儿很少能表现出良好的听力和良好的平衡功能。我们的研究结果表明，尽管右耳IP-1和左耳常见腔畸形，该患者的听觉-毛发细胞可能已部分保留。版权？ 2017年，解放军总医院耳鼻咽喉头颈外科。由Elsevier（Singapore）Pte Ltd生产和托管。这是CC BY-NC-ND许可下的开放获取文章。

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来源
《中华耳科学杂志（英文版）》 |2017年第001期|41-46|共6页
作者
Yusuke Kimura; Takeshi Masuda; Akifumi Tomizawa; Hideaki Sakata; Kimitaka Kaga;
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作者单位

National Institute of Sensory Organs, National Tokyo Medical Center, Tokyo, Japan;

Department of Otolaryngology—Head and Neck Surgery, Nihon University School of Medicine, Tokyo, Japan;

National Institute of Sensory Organs, National Tokyo Medical Center, Tokyo, Japan;

Department of Otolaryngology—Head and Neck Surgery, Nihon University School of Medicine, Tokyo, Japan;

Department of Speech, Language and Hearing Therapy, Mejiro University, Saitama, Japan;

Department of Speech, Language and Hearing Therapy, Mejiro University, Saitama, Japan;

Kawagoe Otology Institute, Saitama, Japan;

National Institute of Sensory Organs, National Tokyo Medical Center, Tokyo, Japan;

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原文格式 PDF
正文语种 eng
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A child with severe inner ear malformations with favorable hearing utilization and balance functions after wearing hearing aids

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