Speech recognition scores related to age and degree of hearing impairment in DFNA2/KCNQ4 and DFNA9/COCH
| Autor: | E.M.R. De Leenheer, Martijn H. Kemperman, C.W.R.J. Cremers, G. Van Camp, F. P. M. Cremers, Arjan J. Bosman, Henri A. M. Marres, Francois X Lemaire, R.J.H. Ensink, Steven J. H. Bom, Patrick L. M. Huygen, Wim I. M. Verhagen, Henricus P. M. Kunst |
|---|---|
| Rok vydání: | 2001 |
| Předmět: |
Adult
medicine.medical_specialty Speech perception Potassium Channels Cross-sectional study Hearing loss Elucidation of hereditary disorders and their molecular diagnosis Speech recognition Audiology Severity of Illness Index Severity of illness medicine otorhinolaryngologic diseases Gehoor en communicatie Humans Hearing Loss High-Frequency Aged Absolute threshold of hearing KCNQ Potassium Channels business.industry Age Factors General Medicine Middle Aged Cross-Sectional Studies Otorhinolaryngology Hearing level El Niño Potassium Channels Voltage-Gated Hearing and Communication Disorders Speech Perception Surgery medicine.symptom Opheldering van erfelijke ziekten en hun moleculaire diagnostiek business KCNQ4 |
| Zdroj: | Archives of otolaryngology, head and neck surgery Archives of Otolaryngology--Head & Neck Surgery, 127, 1045-8 Archives of Otolaryngology--Head & Neck Surgery, 127, 9, pp. 1045-8 |
| ISSN: | 0886-4470 |
| Popis: | Item does not contain fulltext 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|