Audiologic and vestibular findings in Wolfram syndrome.
Ear Hear
; 34(6): 809-12, 2013.
Article
em En
| MEDLINE
| ID: mdl-23698626
ABSTRACT
OBJECTIVES:
Assessment of auditory and vestibular function in Wolfram Syndrome (WS) patients, using a standardized protocol.DESIGN:
Prospective cohort study of 11 patients using otoscopic inspection, tympanometry, otoacoustic emissions, pure tones, speech in noise (SIN), the Speech Intelligibility Index, and rotational chair testing.RESULTS:
Mean SNHL diagnosis was 7.3 years with 55% prevalence. Four patients with a Speech Intelligibility Index less than 0.75 (better ear) routinely used bilateral amplification devices. Two patients with normal-hearing sensitivity exhibited abnormal SIN scores. The only patient with significant vestibular dysfunction also had a distinctive low-frequency component to her SNHL.CONCLUSIONS:
Hearing loss may occur earlier than previously suspected, and comprehensive testing including SIN testing may reveal deficits not apparent with pure-tone testing. Particular configurations of hearing loss may indicate a need for comprehensive vestibular assessment. Because SNHL can be the first symptom of WS, audiologists and otolaryngologists should be vigilant about referring patients with hearing loss for ophthalmologic examination.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Audiometria
/
Síndrome de Wolfram
/
Vestíbulo do Labirinto
/
Perda Auditiva Neurossensorial
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
/
Guideline
/
Incidence_studies
/
Observational_studies
/
Risk_factors_studies
Limite:
Adolescent
/
Adult
/
Child
/
Humans
Idioma:
En
Revista:
Ear Hear
Ano de publicação:
2013
Tipo de documento:
Article
País de afiliação:
Estados Unidos