Hearing loss in Usher syndrome type II is nonprogressive.
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.
Reisser
Christoph F V
CF
Department of Otolaryngology-Head and Neck Surgery, University of Heidelberg, Heidelberg, Germany.
Kimberling
William J
WJ
Otterstedde
Christian R
CR
D000158:Acoustic Impedance Tests; D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D001299:Audiometry; D018450:Disease Progression; D016057:Evoked Potentials, Auditory, Brain Stem; D005808:Genes, Recessive; D030342:Genetic Diseases, Inborn / Q000145:classification / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology*; D005858:Germany; D006319:Hearing Loss, Sensorineural / Q000145:classification / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology*; D006801:Humans; D016014:Linear Models; D008137:Longitudinal Studies; D008875:Middle Aged; D017084:Otoacoustic Emissions, Spontaneous; D012174:Retinitis Pigmentosa / Q000235:genetics*; D012720:Severity of Illness Index; D013577:Syndrome