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17例无血族关系丹麦籍Pcndred氏综合征病人,男6名14至60岁(平均40岁)及女11名21至68岁(平均42岁),13例有家族耳聋史,除2例分别在6至16岁发现耳聋外,耳聋均出现于3岁以前。有3例听力轻度持续下降,其中1例20年中下降20dB。双耳全聋1例和单侧聋1例外,余31耳均有程度不同的残余听力,听力曲线呈下坡型高频音缺损重,但有3例5耳呈水平型。9例无会话交谈能力,其中5例戴助听器也无用。14例进行前庭功能检查,有2
There were 17 unrelated Crohn’s disease patients in Pceth’s syndrome, 6 males aged from 14 to 60 years (average 40 years) and 11 females aged 21 to 68 years (mean 42 years), 13 had family history of deafness, except 2 cases In 6 to 16 years of age found deaf, deafness occurred before the age of 3. Three cases of mild and persistent hearing decline, including a 20-year decline in 20dB. There were 1 case of binaural deafness and 1 case of unilateral deafness. All of the remaining 31 ears had varying degrees of residual hearing, and the hearing curves showed downhill HFD defects. However, 3 cases of 5 ears showed horizontal type. Nine patients had no conversational ability, and five of them were also useless hearing aids. 14 cases of vestibular function test, there are 2