Hearing Speech Rehabilitation of Two Patients with IP-Ⅲ Inner Ear Malformation after Cochlear Implantation

CHEN Xu-zhen; LI Jian-hong; DU Fei; ZHOU Yong-qing

doi:10.3969/j.issn.1672-4933.2023.05.006

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Hearing Speech Rehabilitation of Two Patients with IP-Ⅲ Inner Ear Malformation after Cochlear Implantation

Preoperative Evaluation and Postoperative Outcome Assessment of Cochlear Implant | 更新时间：2023-11-03

- Hearing Speech Rehabilitation of Two Patients with IP-Ⅲ Inner Ear Malformation after Cochlear Implantation
- Chinese Scientific Journal of Hearing and Speech Rehabilitation Vol. 21, Issue 5, Pages: 476-479(2023)
- 作者机构：
  
  联勤保障部队第九八〇（白求恩国际和平）医院耳鼻咽喉头颈外科石家庄 050051
- 作者简介：
- 基金信息：
- DOI：10.3969/j.issn.1672-4933.2023.05.006
  CLC：
- Published：15 September 2023，
  
  Received：24 June 2023，
- 稿件说明：
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陈旭真,李建红,杜菲等.2名内耳不完全分隔Ⅲ型患者人工耳蜗植入效果分析[J].中国听力语言康复科学杂志,2023,21(05):476-479.

CHEN Xu-zhen,LI Jian-hong,DU Fei,et al.Hearing Speech Rehabilitation of Two Patients with IP-Ⅲ Inner Ear Malformation after Cochlear Implantation[J].Chinese Scientific Journal of Hearing and Speech Rehabilitation,2023,21(05):476-479.
陈旭真,李建红,杜菲等.2名内耳不完全分隔Ⅲ型患者人工耳蜗植入效果分析[J].中国听力语言康复科学杂志,2023,21(05):476-479. DOI： 10.3969/j.issn.1672-4933.2023.05.006.

CHEN Xu-zhen,LI Jian-hong,DU Fei,et al.Hearing Speech Rehabilitation of Two Patients with IP-Ⅲ Inner Ear Malformation after Cochlear Implantation[J].Chinese Scientific Journal of Hearing and Speech Rehabilitation,2023,21(05):476-479. DOI： 10.3969/j.issn.1672-4933.2023.05.006.

摘要

内耳不完全分隔Ⅲ型（incomplete partition type Ⅲ，IP-Ⅲ）是因

POU3F4

（

DFNX2

）基因突变导致的X连锁遗传性耳聋，表现为男性发病，女性携带。该病人工耳蜗植入手术难度大，易致严重并发症，如脑脊液漏、电极误入内听道等，术后听觉和言语康复疗效不确切。笔者科室分别于2014年和2022年收治2名IP-Ⅲ内耳畸形患儿，均顺利完成右侧人工耳蜗植入手术，无并发症，术后1个月开机，声场助听听阈分别为28.8 dB HL和31.7 dB HL，术后听力言语康复较好。

Abstract

Incomplete partition type Ⅲ(IP-Ⅲ) is an X-linked genetic deafness caused by POU3F4 (DFNX2) gene mutation. Clinical manifestation is male onset

female carrier. The patient with IP-Ⅲ type inner ear malformation is hard to be performed cochlear implantation because some serious complications could occur

such as cerebrospinal fluid otorrhea

electrode enter the internal auditory canal. Furthermore

the rehabilitation efficacies of postoperative hearing and speech are uncertain. Two boys with type IP-Ⅲ inner ear malformation received cochlear implantation on their right ears in 2014 and 2022 in our Department

with no complications occurring. The electronic cochlears were smoothly started up one month after surgeries. The electronic cochlears have been working well until now. At present auditory thresholds of sound field hearing aid were 28.8 dB HL and 31.7 dB HL

respectively. Their hearing and speech rehabilitation efficacies were better than that in the preoperation.

关键词

不完全分隔Ⅲ型内耳畸形耳蜗植入康复疗效

Keywords

Incomplete partition type ⅢInner ear malformationCochlear implantationRehabilitationCurative effect

references

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Stankovic KM, Hennessey AM, Herrmann B, et al. Cochlear implantation in children with congenital X-linked deafness due to novel mutations in POU3F4 gene[J].Annals of Otology, Rhinology&Laryngology,2010,119(12):815-822.

Incesulu A, Adapinar B, Kecik C. Cochlear implantation in cases with incomplete partition type Ⅲ (X-linked anomaly)[J].Eur Arch Otorhinolaryngol, 2008 265(11):1425-1430.

张道行.蜗轴缺失内耳形态学表现与人工耳蜗植入入路的选择[J].临床耳鼻咽喉头颈外科杂志,2014,28(17):1296-1300.

卢宇涵,李佳楠,孙丽,等.内耳不完全分隔Ⅲ型(IP-Ⅲ)畸形病例分析[J].中华耳科学杂志,2018,16(4):451-457.

赵天峰,陈俊,任寸寸,等.复杂内耳畸形患儿人工耳蜗植入术后疗效分析[J].听力学及言语疾病杂志,2021,29(5):504-508.

Alballaa A, Aschendorff A, Arndt S, et al. Incomplete partition type III revisited-long-term results following cochlear implant[J]. HNO, 2020, 68(Suppl 1):25-32.

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