TY - JOUR T1 - Usher syndrome type I associated with bronchiectasis and immotile nasal cilia in two brothers. JF - Journal of Medical Genetics JO - J Med Genet SP - 253 LP - 254 DO - 10.1136/jmg.30.3.253 VL - 30 IS - 3 AU - D Bonneau AU - F Raymond AU - C Kremer AU - J M Klossek AU - J Kaplan AU - F Patte Y1 - 1993/03/01 UR - http://jmg.bmj.com/content/30/3/253.abstract N2 - Usher syndrome type I is an autosomal recessive disease characterised by congenital sensorineural deafness, involvement of the vestibular system, and progressive visual loss owing to retinitis pigmentosa. Here we report the association of this disease with bronchiectasis, chronic sinusitis, and reduced nasal mucociliary clearance in two sibs and we suggest Usher syndrome type I could be a primary ciliary disorder. ER -