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Speech Recognition Scores Related to Age and Degree of Hearing Impairment in DFNA2/KCNQ4 and DFNA9/COCH
Steven J. H. Bom, MD;
Els M. R. De Leenheer, MD;
François X. Lemaire, MD;
Martijn H. Kemperman, MD;
Wim I. M. Verhagen, PhD;
Henri A. M. Marres, PhD;
Henricus P. M. Kunst, PhD;
Robbert J. H. Ensink, PhD;
Arjan J. Bosman, PhD;
Guy Van Camp, PhD;
Frans P. M. Cremers, PhD;
Patrick L. M. Huygen, PhD;
Cor W. R. J. Cremers, PhD
Arch Otolaryngol Head Neck Surg. 2001;127:1045-1048.
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.
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).
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