Congenital Nonprofound Bilateral Sensorineural Hearing Loss in Children: Comprehensive Characterization of Auditory Function and Hearing Aid Benefit
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Congenital Nonprofound Bilateral Sensorineural Hearing Loss in Children : Comprehensive Characterization of Auditory Function and Hearing Aid Benefit. / Berninger, Erik; Drott, Maria; Romanitan, Mircea; Tranebjærg, Lisbeth; Hellström, Sten.
I: Audiology Research, Bind 12, Nr. 5, 2022, s. 539-563.Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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TY - JOUR
T1 - Congenital Nonprofound Bilateral Sensorineural Hearing Loss in Children
T2 - Comprehensive Characterization of Auditory Function and Hearing Aid Benefit
AU - Berninger, Erik
AU - Drott, Maria
AU - Romanitan, Mircea
AU - Tranebjærg, Lisbeth
AU - Hellström, Sten
N1 - Funding Information: This project was supported by the regional agreement on medical training and clinical research (ALF) between Stockholm County Council and Karolinska Institutet, the Tysta Skolan Foundation, Stingerfonden, Foundation of the Swedish Order of Freemasons (Konung Gustaf VI Adolfs frimurarefond, 2022), Karolinska University Hospital, and Karolinska Institutet.
PY - 2022
Y1 - 2022
N2 - A prospective cross-sectional design was used to characterize congenital bilateral sensorineural hearing loss (SNHL). The underlying material of >30,000 consecutively screened newborns comprised 11 subjects with nonprofound, alleged nonsyndromic, SNHL. Comprehensive audiological testing was performed at ≈11 years of age. Results showed symmetrical sigmoid-like median pure-tone thresholds (PTTs) reaching 50–60 dB HL. The congenital SNHL revealed recruitment, increased upward spread of masking, distortion product otoacoustic emission (DPOAE) dependent on PTT (≤60 dB HL), reduced auditory brainstem response (ABR) amplitude, and normal magnetic resonance imaging. Unaided recognition of speech in spatially separate competing speech (SCS) deteriorated with increasing uncomfortable loudness level (UCL), plausibly linked to reduced afferent signals. Most subjects demonstrated hearing aid (HA) benefit in a demanding laboratory listening situation. Questionnaires revealed HA benefit in real-world listening situations. This functional characterization should be important for the outline of clinical guidelines. The distinct relationship between DPOAE and PTT, up to the theoretical limit of cochlear amplification, and the low ABR amplitude remain to be elucidated. The significant relation between UCL and SCS has implications for HA-fitting. The fitting of HAs based on causes, mechanisms, and functional characterization of the SNHL may be an individualized intervention approach and deserves future research.
AB - A prospective cross-sectional design was used to characterize congenital bilateral sensorineural hearing loss (SNHL). The underlying material of >30,000 consecutively screened newborns comprised 11 subjects with nonprofound, alleged nonsyndromic, SNHL. Comprehensive audiological testing was performed at ≈11 years of age. Results showed symmetrical sigmoid-like median pure-tone thresholds (PTTs) reaching 50–60 dB HL. The congenital SNHL revealed recruitment, increased upward spread of masking, distortion product otoacoustic emission (DPOAE) dependent on PTT (≤60 dB HL), reduced auditory brainstem response (ABR) amplitude, and normal magnetic resonance imaging. Unaided recognition of speech in spatially separate competing speech (SCS) deteriorated with increasing uncomfortable loudness level (UCL), plausibly linked to reduced afferent signals. Most subjects demonstrated hearing aid (HA) benefit in a demanding laboratory listening situation. Questionnaires revealed HA benefit in real-world listening situations. This functional characterization should be important for the outline of clinical guidelines. The distinct relationship between DPOAE and PTT, up to the theoretical limit of cochlear amplification, and the low ABR amplitude remain to be elucidated. The significant relation between UCL and SCS has implications for HA-fitting. The fitting of HAs based on causes, mechanisms, and functional characterization of the SNHL may be an individualized intervention approach and deserves future research.
KW - children
KW - congenital
KW - electrophysiology
KW - hearing
KW - hearing aids
KW - imaging
KW - otoacoustic emissions
KW - sensorineural hearing loss
KW - speech recognition
U2 - 10.3390/audiolres12050054
DO - 10.3390/audiolres12050054
M3 - Journal article
C2 - 36285911
AN - SCOPUS:85140391997
VL - 12
SP - 539
EP - 563
JO - Audiology Research
JF - Audiology Research
SN - 2039-4330
IS - 5
ER -
ID: 327675376