1. Further delineation of the DFNA5 phenotype: results of speech recognition tests.
- Author
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De Leenheer EM, van Zuijlen DA, Van Laer L, Van Camp G, Huygen PL, Huizing EH, and Cremers CW
- Subjects
- Adolescent, Adult, Aged, Audiometry, Pure-Tone, Child, Cross-Sectional Studies, Hearing Loss, Sensorineural diagnosis, Humans, Middle Aged, Phenotype, Phonetics, Point Mutation genetics, Severity of Illness Index, Speech Reception Threshold Test, Carrier Proteins genetics, Hearing Loss, Sensorineural genetics, Receptors, Estrogen, Speech Perception physiology
- Abstract
Speech recognition scores were analyzed in 34 carriers of a DFNA5 mutation. Cross-sectional linear regression analysis (last visit, maximum recognition score in %Correct on age or PTA1,2,4 kHz) established onset age (score 90%) at 16 years and onset PTA1,2,4 kHz level (score 90%) at 41 dB hearing level. The deterioration rate was 0.7%/y in the plot of maximum score against age, whereas the deterioration gradient was 0.4%/dB in the plot of maximum score against PTA1,2,4 kHz. Given the previously demonstrated rapid progression of hearing impairment, speech recognition was relatively good: at age 70, the score was still >50%.
- Published
- 2002
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