1 62 THE CRETINIC DEGENERATION 



anomalies of the brain, and which have nothing to do with the goiter noxus. A 

 very great number, however, belong to endemic cretinism (according to 

 E. Bircher, So per cent, in Switzerland). Also in Austria, and especially in 

 the Stiermark, are found an enormous number of deaf-mutes. Scholz found 

 among the cretins examined by him 29 per cent, deaf-mutes and 32 per cent, 

 hard-of-hearing. The intensity of the disturbance of hearing does not go 

 parallel throughout with that of the remaining cretinic symptoms. There 

 are complete cretins that show only slight disturbances of hearing and speech. 

 In others the mutism may be the chief symptom of the cretinic degeneration 

 (larval form of v. Eiselsberg) . 



The statements as to the functional disturbances or the pathologico- 

 anatomical findings in the deaf cretins and in the endemic deaf-mutes diverge 

 greatly. Hammerschlag, whom we have to thank for the first exact investiga- 

 tions, found on the one hand alterations in the peripheral hearing apparatus, 

 and on the other only disturbance of the perception of sound, as also found 

 Scholz, Froschel, and others. Further, there was found in endemic cretinism 

 incomplete ossification of the stapes, inhibition of development of the epi- 

 thelial cells in the ductus cochlearis (Habermann, Alexander), shortening of 

 the base of the skull and thereby disturbance in the development of the organ 

 of hearing (Danzinger, Bircher), incomplete ossification of the organ of hearing 

 with hyperostotic growths at other places (Moos and Steinbrugge) . Anom- 

 alies of the malleus (Nager), myxedematous thickening of the tympanic 

 mucous membrane, etc., have been regarded as the cause of the hardness- 

 of-hearing. Recently, E. Bircher has strenuously criticised a portion of these 

 findings or their significance. To-day we may safely assume that we may 

 ascribe great importance to the degeneration in the cortical centers or the 

 developmental inhibitions in the cortical centers, and that the different altera- 

 tions are directly elicited by the goiter noxus and are coordinated with an 

 insufficiency of the thyroid, which finally sets in (Pineles). 



The inhibition of the development of speech in endemic cretinism is extra- 

 ordinarily different [in degree]; where hearing is entirely absent, of course, 

 the development of speech is also absent. We do, however, see cases in 

 which in spite of high-grade disturbances of hearing only relatively slight 

 defects of intelligence are present. Of course, in these cases articulation is 

 poor (Hammer schlag) . In other cases, in spite of good hearing, intelligence 

 and the development of speech are minimal. Here also we assume a de- 

 velopmental inhibition of cortical centers (Scholz and Zingerle). Where 

 the endemic shows less intensity, defects of intelligence may be entirely 

 absent (H. Bircher). 



Investigations as to the metabolism in endemic cretins have been pub- 

 lished only by Scholz. Scholz designates the metabolism as very sluggish. 

 The amounts of urine were very slight, as well as the exchange of protein and 

 of salts. The excretion of nitrogen, uric acid, creatinin, and sodium chloride 



