Hearing loss in Usher syndrome type II is nonprogressive

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

    Research output: Contribution to journalArticlepeer-review

    21 Scopus citations


    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
    Issue number12
    StatePublished - Dec 1 2002


    • Hearing loss
    • Usher syndrome type II

    ASJC Scopus subject areas

    • Otorhinolaryngology


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