Hearing impairment related to age in Usher syndrome types 1B and 2A

Mariette Wagenaar, Annelies Van Aarem, Patrick Huygen, Sandra Pieke-Dahl, William Kimberling, Cor Cremers

Research output: Contribution to journalArticle

31 Citations (Scopus)

Abstract

Objective: To evaluate hearing impairment in 2 common genetic subtypes of Usher syndrome, USH1B and USH2A. Design: Cross-sectional analysis of hearing threshold related to age in patients with genotypes determined by linkage and mutation analysis. Setting: Otolaryngology department, university referral center. Patients: Nineteen patients with USH1B and 27 with USH2A were examined. All participants were living in the Netherlands and Belgium. Main Outcome Measure: Pure tone audiometry of the best ear at last visit. Results: The patients with USH1B had residual hearing without age dependence, with minimum thresholds of 80, 95, and 120 dB at 0.25, 0.5, and 1 to 2 kHz, respectively. Mean thresholds of patients with USH2A were about 45 to 55 dB better than these minimum values. Distinctive audiographic features of patients with USH2A were maximum hearing thresholds of 70, 80, and 100 dB at 0.25, 0.5, and 1 kHz, respectively, only at younger than 40 years. Progression of hearing impairment in USH2A was 0.7 dB/y on average for 0.25 to 4 kHz and could not be explained by presbyacusis alone. Conclusions: The USH1B and USH2A can be easily distinguished by hearing impairment at younger than 40 years at the low frequencies. Hearing impairment m our patients with USH2A could be characterized as progressive.

Original languageEnglish (US)
Pages (from-to)441-445
Number of pages5
JournalArchives of Otolaryngology - Head and Neck Surgery
Volume125
Issue number4
DOIs
StatePublished - Apr 1999

Fingerprint

Hearing Loss
Hearing
Usher Syndromes
Pure-Tone Audiometry
Belgium
Otolaryngology
Type 1B Usher syndrome
Type 2A Usher syndrome
Netherlands
Ear
Referral and Consultation
Cross-Sectional Studies
Genotype
Outcome Assessment (Health Care)
Mutation

ASJC Scopus subject areas

  • Surgery
  • Otorhinolaryngology

Cite this

Wagenaar, M., Van Aarem, A., Huygen, P., Pieke-Dahl, S., Kimberling, W., & Cremers, C. (1999). Hearing impairment related to age in Usher syndrome types 1B and 2A. Archives of Otolaryngology - Head and Neck Surgery, 125(4), 441-445. https://doi.org/10.1001/archotol.125.4.441

Hearing impairment related to age in Usher syndrome types 1B and 2A. / Wagenaar, Mariette; Van Aarem, Annelies; Huygen, Patrick; Pieke-Dahl, Sandra; Kimberling, William; Cremers, Cor.

In: Archives of Otolaryngology - Head and Neck Surgery, Vol. 125, No. 4, 04.1999, p. 441-445.

Research output: Contribution to journalArticle

Wagenaar, M, Van Aarem, A, Huygen, P, Pieke-Dahl, S, Kimberling, W & Cremers, C 1999, 'Hearing impairment related to age in Usher syndrome types 1B and 2A', Archives of Otolaryngology - Head and Neck Surgery, vol. 125, no. 4, pp. 441-445. https://doi.org/10.1001/archotol.125.4.441
Wagenaar, Mariette ; Van Aarem, Annelies ; Huygen, Patrick ; Pieke-Dahl, Sandra ; Kimberling, William ; Cremers, Cor. / Hearing impairment related to age in Usher syndrome types 1B and 2A. In: Archives of Otolaryngology - Head and Neck Surgery. 1999 ; Vol. 125, No. 4. pp. 441-445.
@article{1bc9c6f1adee44cf96da75a90e06c0c9,
title = "Hearing impairment related to age in Usher syndrome types 1B and 2A",
abstract = "Objective: To evaluate hearing impairment in 2 common genetic subtypes of Usher syndrome, USH1B and USH2A. Design: Cross-sectional analysis of hearing threshold related to age in patients with genotypes determined by linkage and mutation analysis. Setting: Otolaryngology department, university referral center. Patients: Nineteen patients with USH1B and 27 with USH2A were examined. All participants were living in the Netherlands and Belgium. Main Outcome Measure: Pure tone audiometry of the best ear at last visit. Results: The patients with USH1B had residual hearing without age dependence, with minimum thresholds of 80, 95, and 120 dB at 0.25, 0.5, and 1 to 2 kHz, respectively. Mean thresholds of patients with USH2A were about 45 to 55 dB better than these minimum values. Distinctive audiographic features of patients with USH2A were maximum hearing thresholds of 70, 80, and 100 dB at 0.25, 0.5, and 1 kHz, respectively, only at younger than 40 years. Progression of hearing impairment in USH2A was 0.7 dB/y on average for 0.25 to 4 kHz and could not be explained by presbyacusis alone. Conclusions: The USH1B and USH2A can be easily distinguished by hearing impairment at younger than 40 years at the low frequencies. Hearing impairment m our patients with USH2A could be characterized as progressive.",
author = "Mariette Wagenaar and {Van Aarem}, Annelies and Patrick Huygen and Sandra Pieke-Dahl and William Kimberling and Cor Cremers",
year = "1999",
month = "4",
doi = "10.1001/archotol.125.4.441",
language = "English (US)",
volume = "125",
pages = "441--445",
journal = "JAMA Otolaryngology - Head and Neck Surgery",
issn = "2168-6181",
publisher = "American Medical Association",
number = "4",

}

TY - JOUR

T1 - Hearing impairment related to age in Usher syndrome types 1B and 2A

AU - Wagenaar, Mariette

AU - Van Aarem, Annelies

AU - Huygen, Patrick

AU - Pieke-Dahl, Sandra

AU - Kimberling, William

AU - Cremers, Cor

PY - 1999/4

Y1 - 1999/4

N2 - Objective: To evaluate hearing impairment in 2 common genetic subtypes of Usher syndrome, USH1B and USH2A. Design: Cross-sectional analysis of hearing threshold related to age in patients with genotypes determined by linkage and mutation analysis. Setting: Otolaryngology department, university referral center. Patients: Nineteen patients with USH1B and 27 with USH2A were examined. All participants were living in the Netherlands and Belgium. Main Outcome Measure: Pure tone audiometry of the best ear at last visit. Results: The patients with USH1B had residual hearing without age dependence, with minimum thresholds of 80, 95, and 120 dB at 0.25, 0.5, and 1 to 2 kHz, respectively. Mean thresholds of patients with USH2A were about 45 to 55 dB better than these minimum values. Distinctive audiographic features of patients with USH2A were maximum hearing thresholds of 70, 80, and 100 dB at 0.25, 0.5, and 1 kHz, respectively, only at younger than 40 years. Progression of hearing impairment in USH2A was 0.7 dB/y on average for 0.25 to 4 kHz and could not be explained by presbyacusis alone. Conclusions: The USH1B and USH2A can be easily distinguished by hearing impairment at younger than 40 years at the low frequencies. Hearing impairment m our patients with USH2A could be characterized as progressive.

AB - Objective: To evaluate hearing impairment in 2 common genetic subtypes of Usher syndrome, USH1B and USH2A. Design: Cross-sectional analysis of hearing threshold related to age in patients with genotypes determined by linkage and mutation analysis. Setting: Otolaryngology department, university referral center. Patients: Nineteen patients with USH1B and 27 with USH2A were examined. All participants were living in the Netherlands and Belgium. Main Outcome Measure: Pure tone audiometry of the best ear at last visit. Results: The patients with USH1B had residual hearing without age dependence, with minimum thresholds of 80, 95, and 120 dB at 0.25, 0.5, and 1 to 2 kHz, respectively. Mean thresholds of patients with USH2A were about 45 to 55 dB better than these minimum values. Distinctive audiographic features of patients with USH2A were maximum hearing thresholds of 70, 80, and 100 dB at 0.25, 0.5, and 1 kHz, respectively, only at younger than 40 years. Progression of hearing impairment in USH2A was 0.7 dB/y on average for 0.25 to 4 kHz and could not be explained by presbyacusis alone. Conclusions: The USH1B and USH2A can be easily distinguished by hearing impairment at younger than 40 years at the low frequencies. Hearing impairment m our patients with USH2A could be characterized as progressive.

UR - http://www.scopus.com/inward/record.url?scp=0032953188&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0032953188&partnerID=8YFLogxK

U2 - 10.1001/archotol.125.4.441

DO - 10.1001/archotol.125.4.441

M3 - Article

C2 - 10208682

AN - SCOPUS:0032953188

VL - 125

SP - 441

EP - 445

JO - JAMA Otolaryngology - Head and Neck Surgery

JF - JAMA Otolaryngology - Head and Neck Surgery

SN - 2168-6181

IS - 4

ER -