[Electrophysiologic tests of the auditory apparatus and vestibular organ in Usher syndrome]

Usher syndrome is a congenital autosomal dominant inherited disease characterised by hearing, balance and vision problems. The aim of the work was an evaluation of hearing and vestibular organ in the chosen group of patients with Usher syndrome diagnosed. Material consisted of 10 persons (5 males and 5 females) aged from 16 to 46 years with Usher syndrome diagnosed. All the patients have been in constant ENT and Ophthalmology Clinics' care. Complex hearing and balance organ, including posturography, as well as complex ophthalmologic examination were performed in each case. Medium or severe perceptive cochlear hearing loss, proper function of vestibular organ and slight elevation of stabilograms parameters were found in 6 cases. In 4 cases there were severe bilateral hearing loss, lack of vestibular function and considerable worsening of stabilograms parameters. In ophthalmologic examination in all the cases there was bilateral symmetrical dystrophy of the retina of various intensification. We concluded that hearing and balance organ examination in Usher syndrome can state accessory diagnostic aspect that might help to distinguish subtypes of the syndrome. In this way audiologic and otoneurologic tests could help to define prognosis of the disease in the individual cases. A necessity of close co-operation of genetics, ophthalmologists and ENT doctors as well as psychologists was stressed.