Hearing loss in Usher syndrome type II is nonprogressive

Christoph F.V. Reisser, William J. Kimberling, Christian R. Otterstedde

Research output: Contribution to journalArticle

16 Citations (Scopus)

Abstract

Usher syndrome is an autosomal recessive disorder characterized by sensorineural hearing loss and progressive visual loss secondary to retinitis pigmentosa. In the literature, a possible progression of the moderate to severe hearing loss in Usher syndrome type II (Usher II) is controversial. We studied the development of the hearing loss of 125 patients with a clinical diagnosis of Usher syndrome type II intraindividually and interindividually by repeatedly performing complete audiological and neuro-otologic examinations. Our data show a very characteristic slope of the hearing curve in all Usher II patients and no clinically relevant progression of the hearing loss over up to 17 years. The subjective impression of a deterioration of the communicative abilities of Usher II patients must therefore be attributed to the progressive visual loss. The patients should be reassured that changes in their hearing abilities are unlikely and should be provided with optimally fitted modern hearing aids.

Original languageEnglish (US)
Pages (from-to)1108-1111
Number of pages4
JournalAnnals of Otology, Rhinology and Laryngology
Volume111
Issue number12
DOIs
StatePublished - Dec 1 2002

Fingerprint

Usher Syndromes
Hearing Loss
Aptitude
Hearing
Retinitis Pigmentosa
Hearing Aids
Sensorineural Hearing Loss

Keywords

  • Hearing loss
  • Usher syndrome type II

ASJC Scopus subject areas

  • Otorhinolaryngology

Cite this

Hearing loss in Usher syndrome type II is nonprogressive. / Reisser, Christoph F.V.; Kimberling, William J.; Otterstedde, Christian R.

In: Annals of Otology, Rhinology and Laryngology, Vol. 111, No. 12, 01.12.2002, p. 1108-1111.

Research output: Contribution to journalArticle

Reisser, Christoph F.V. ; Kimberling, William J. ; Otterstedde, Christian R. / Hearing loss in Usher syndrome type II is nonprogressive. In: Annals of Otology, Rhinology and Laryngology. 2002 ; Vol. 111, No. 12. pp. 1108-1111.
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