Speech recognition scores related to age and degree of hearing impairment in DFNA2/KCNQ4 and DFNA9/COCH

被引：31

作者：

Bom, SJH

De Leenheer, EMR

Lemaire, FX

Kemperman, MH

Verhagen, WIM

Marres, HAM

Kunst, HPM

Ensink, RJH

Bosman, AJ

Van Camp, G

Cremers, FPM

Huygen, PLM

Cremers, CWRJ

机构：

[1] Radboud Univ Nijmegen Med Ctr, Dept Otorhinolaryngol, NL-6500 HB Nijmegen, Netherlands

[2] Radboud Univ Nijmegen Med Ctr, Dept Human Genet, NL-6500 HB Nijmegen, Netherlands

[3] Canisius Wilhelmina Hosp, Dept Neurol, Nijmegen, Netherlands

[4] Univ Hosp Louvain, Dept Otorhinolaryngol Head & Neck Surg, Louvain, Belgium

[5] Univ Antwerp, Dept Med Genet, Antwerp, Belgium

来源：

ARCHIVES OF OTOLARYNGOLOGY-HEAD & NECK SURGERY | 2001年 / 127卷 / 09期

关键词：

D O I：

10.1001/archotol.127.9.1045

中图分类号：

R76 [耳鼻咽喉科学];

学科分类号：

100213 ;

摘要：

Objective: To analyze the relationship between pure-tone hearing threshold and speech recognition performance in DFNA2/KCNQ4 and DFNA9/COCH, 2 types of high-frequency nonsyndromic hearing impairment. Design: Case series with cross-sectional analysis of phoneme recognition scores related to age and hearing level. Setting: University hospital. Patients: Forty-five members of 4 separate families, all carrying 1 of 3 different mutations in the KCNQ4 gene at the DFNA2 locus (1p34); 42 members of 7 separate families, all carrying the same Pro51Ser mutation in the COCH gene at the DFNA9 locus (14q12-q13). Results: The deterioration of speech recognition dropped to a 90% score at a higher level of hearing impairment (pure-tone-average at 1, 2, and 4 kHz) in DFNA2-affected patients (65 dB) than in DFNA9-affected patients (46 dB). Conclusion: At similar levels of hearing impairment, DFNA2/KCNQ4-affected patients showed better speech recognition performance than DFNA9/COCH-affected patients.

引用

页码：1045 / 1048

页数：4

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