The Prognosis of Juvenile Bilateral Sensorineural Deafness of Unknown Etiology (JBSNDUE)

The primary goal of this study was to verify the incidence and degree of hearing deterioration of JBSNDUE with a prospective long-term clinical follow-up.Of 173 JBSNDUE cases, 54 were followed up untill their ages reached 25-39 (average 30.7) years. In 23 cases (42.6%) hearing deterioration was confirmed. In 16 cases, discrimination scores for nonsense syllables were as low as 0%.The criteria of the “deterioration” of pure tones were irreversible average hearing level greater than 15dB at three adjoining frequencies between 0.25 and 4kHz.Except 25 of 173 cases, either binaural or monaural amplification was used. However, the incidence of hearing deterioration showed no significant difference between aided and unaided ears.The clinical features of JBSNDUE were summerized.The communication ability of those with deteriorated hearing was discussed.