Fiore C, Cagini C, Menduno P, Toniassoni I, Desantis A, Pennacchi A, Ricci G, Molini E
Department of Ophthalmology, University of Perugia, Italy.
Doc Ophthalmol. 1994;87(2):167-76. doi: 10.1007/BF01204794.
The hearing function was studied in 26 patients affected by retinitis pigmentosa (RP) and in their relatives. Sixteen patients showed bilateral normal hearing when examined with traditional audiometric methods. In these normoacusic patients evoked otoacoustic emissions (EOE) have been studied. The EOE offer a unique opportunity to measure objectively the function of outer hair cells: they record the amplitude of the energy produced by the outer hair cells of the coclea following an acoustic stimulation. The data have been statistically compared, using the Student's t-test, with those obtained in a homogeneous control-group of normal subjects. In normoacusic subjects with RP the average values of EOE intensity are statistically lower than those of normal subjects in 64 of the 127 frequency bands examined. Moreover, the distribution of the EOE in patients with retinitis pigmentosa proved to be more discontinous than that observed in the normal subjects. The EOE recorded in 14 normoacusic relatives show in some cases small anomalies but the data, on account of the limited sample group, cannot be statistically evaluated. Therefore a subclinical alteration of the Organ of Corti is found in 100% of the patients affected by RP, although they appear to be normoacusic to usual audiometric tests.
对26例患有色素性视网膜炎（RP）的患者及其亲属的听力功能进行了研究。16例患者在采用传统听力测试方法检查时显示双侧听力正常。对这些听力正常的患者进行了耳声发射（EOE）研究。耳声发射为客观测量外毛细胞的功能提供了独特的机会：它们记录了在声刺激后耳蜗外毛细胞产生的能量幅度。使用学生t检验对这些数据与在一组同质正常受试者中获得的数据进行了统计学比较。在患有RP的听力正常受试者中，在所检查的127个频带中的64个频带中，EOE强度的平均值在统计学上低于正常受试者。此外，色素性视网膜炎患者的EOE分布比正常受试者中观察到的更不连续。在14名听力正常的亲属中记录的EOE在某些情况下显示出小的异常，但由于样本组有限，这些数据无法进行统计学评估。因此，在100%的患有RP的患者中发现了柯蒂氏器的亚临床改变，尽管他们在常规听力测试中似乎听力正常。
