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


METHODS
Case series with cross-sectional analysis of phoneme recognition scores related to age and hearing level.


METHODS
University hospital.


METHODS
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).


CONCLUSIONS
At similar levels of hearing impairment, DFNA2/KCNQ4-affected patients showed better speech recognition performance than DFNA9/COCH-affected patients.
Bom
S J
SJ
Department of Otorhinolaryngology, University Medical Centre St Radboud, Nijmegen, the Netherlands. S.Bom@kno.azn.nl
De Leenheer
E M
EM
Lemaire
F X
FX
Kemperman
M H
MH
Verhagen
W I
WI
Marres
H A
HA
Kunst
H P
HP
Ensink
R J
RJ
Bosman
A J
AJ
Van Camp
G
G
Cremers
F P
FP
Huygen
P L
PL
Cremers
C W
CW
D000328:Adult; D000367:Age Factors; D000368:Aged; D003430:Cross-Sectional Studies; D006316:Hearing Loss, High-Frequency / Q000235:genetics* / Q000503:physiopathology*; D006801:Humans; D051656:KCNQ Potassium Channels; D008875:Middle Aged; D015221:Potassium Channels / Q000235:genetics*; D024642:Potassium Channels, Voltage-Gated; D012720:Severity of Illness Index; D013067:Speech Perception
D051656:KCNQ Potassium Channels; C117367:KCNQ4 protein, human; D015221:Potassium Channels; D024642:Potassium Channels, Voltage-Gated