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Original Article
September 2001

Speech Recognition Scores Related to Age and Degree of Hearing Impairment in DFNA2/KCNQ4 and DFNA9/COCH

Author Affiliations

From the Departments of Otorhinolaryngology (Drs Bom, De Leenheer, Kemperman, Marres, Kunst, Ensink, Bosman, Huygen, and C.W.R.J. Cremers) and Human Genetics (Dr F.P.M. Cremers), University Medical Centre St Radboud, and Department of Neurology, Canisius-Wilhelmina Hospital (Dr Verhagen) Nijmegen, the Netherlands; Department of Otorhinolaryngology, Head and Neck Surgery, University Hospital Leuven, Leuven, Belgium (Drs Lemaire and C.W.R.J. Cremers); and Department of Medical Genetics, University of Antwerp, Belgium (Dr Van Camp).

Arch Otolaryngol Head Neck Surg. 2001;127(9):1045-1048. doi:10.1001/archotol.127.9.1045

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.