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retardation or cerebral palsy Hearing de cit explains many of the other cases, as discussed below, and a few represent what appears to be a lack of maturation of the motor speech areas or an acquired lesion in these parts Only in this small latter group is it appropriate to refer to the language disorder as aphasia ie, a derangement or loss of language due to a cerebral lesion Aphasia, when it occurs as the result of an acquired lesion (vascular, traumatic), is essentially of the motor variety and typically lasts but a few months in the child It may be accompanied by a right-sided hemiplegia An interesting type of acquired aphasia, possibly encephalitic, has been described by Landau and Kleffner in association with seizures and bitemporal focal discharges in the EEG (see page 289) Congenital Deafness Speech delay due to congenital deafness, whether peripheral (loss of pure-tone acuity) or central (pure-tone threshold normal by audiogram), is a most important condition but may at rst be dif cult to discern One suspects that faulty hearing is causal when there is a history of familial deaf mutism, congenital rubella, erythroblastosis fetalis, meningitis, chronic bilateral ear infections, or the administration of ototoxic drugs to the pregnant mother or newborn infant the well-known antecedents of deafness It is estimated that approximately 3 million American children have hearing defects; 01 percent of the school population are deaf and 15 percent are hard of hearing The parents attention may be drawn to a defect in hearing when the infant fails to heed loud noises, to turn the eyes to sound sources outside the immediate visual elds, and to react to music; but in other instances it is the delay in speaking that calls attention to it The deaf child makes the transition from crying to cooing and babbling at the usual age of 3 to 5 months After the sixth month, however, the child becomes much quieter, and the usual repertoire of babbling sounds becomes stereotyped and unchanging, though still uttered with pleasant voice A more conspicuous failure comes somewhat later, when babbling fails to give way to word formation Should deafness develop within the rst few years of life, the child gradually loses such speech as had been acquired but can be retaught by the lipreading method Speech, however, is harsh, poorly modulated, and unpleasant and accompanied by many peculiar squeals and snorting or grunting noises Social and other acquisitions appear at the expected times in the congenitally deaf child, unlike the mentally retarded child The deaf child seems eager to communicate and makes known all his needs by gesture or pantomime often very cleverly The deaf child may attract attention by vivid facial expressions, motions of the lips, nodding, or head shaking The Leiter performance scale, which makes no use of sounds, will show that intelligence is normal Deafness can be demonstrated at an early age by careful observation of the child s responses to sounds and by free- eld audiometry, but the full range of hearing cannot be accurately tested before the age of 3 or 4 years Recording of auditory-evoked brainstem potentials and testing of the labyrinths, which are frequently unresponsive in deaf mutes, may be helpful Early diagnosis is important in order to t the child with a hearing aid, if possible, and to begin appropriate language training In contrast to the child in whom deafness is the only abnormality, the mentally retarded child generally talks little but may display a rich personality Autistic children may also be mute; if they speak, echolalia is prominent and the personal I is avoided Blind children of normal intelligence tend to speak slowly and fail to acquire imitative gestures.

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Congenital Word Deafness This disorder also called developmental receptive dysphasia, verbal auditory agnosia, or central deafness is rare and may be dif cult to distinguish from peripheral deafness Usually the parents have noted that the word-deaf child responds to loud noises and music, but obviously this does not assure perfect hearing, particularly for high tones The worddeaf child does not understand what is said, and delay and distortion of speech are evident Presumably, the receptive auditory elements of the dominant temporal cortex fail to discriminate the complex acoustic patterns of words and to associate them with visual images of people and objects Despite intact pure-tone hearing, the child does not seem to hear word patterns properly and fails to reproduce them in natural speech In other ways the child may be bright, but more often this auditory imperception of words is associated with hyperactivity, inattentiveness, bizarre behavior, or other perceptual defects incident to focal brain damage, particularly of the temporal lobes Word-deaf children may chatter incessantly and often adopt a language of their own design, which the parents come to understand This peculiar type of speech is known as idioglossia It is also observed in children with marked articulatory defects Speech rehabilitation of the bright word-deaf child follows along the same lines as that of the congenitally deaf one Such a child learns to lip-read quickly and is very facile at acting out his or her own ideas Congenital Inarticulation In this developmental defect the child seems unable to coordinate the vocal, articulatory, and respiratory musculature for the purpose of speaking Again, boys are affected more often than girls, and there is often a family history of the disorder, although the data are not quite suf cient to establish the pattern of inheritance The incidence is 1 in every 200 children The motor, sensory, emotional, and social attainments correspond to the norms for age, although in a few cases, a minority in the authors opinion, there has been some indication of cranial nerve abnormality in the rst months of life (ptosis, facial asymmetry, strange neonatal cry, and altered phonation) In children with congenital inarticulation, the prelanguage sounds are probably abnormal, but this aspect of the speech disorder has not been well studied Babbling tends to be de cient, and, in the second year, in attempting to say something, the child makes noises that do not sound at all like language; in this way the child is unlike the late talker already described Again, the understanding of language is entirely normal; the comprehension vocabulary is average for age, and the child can appreciate syntax, as indicated by correct responses to questions by nodding or shaking the head and by the execution of complex spoken commands Usually such patients are shy but otherwise quick in responding, cheerful, and without other behavioral disorders Most are bright, but a combination of congenital inarticulation and mild mental dullness is not uncommon If many of the spontaneous utterances are intelligible, speech correction should be attempted (by a trained therapist) However, if the child makes no sounds that resemble words, the therapeutic effort should be directed toward a modi ed school program, and speech rehabilitation should wait until some words are acquired Studies of the cerebra of such patients are not available, and it is doubtful if they would show any abnormality by the usual techniques of neuropathologic examination Occasionally, suspicion of a lesion is raised by focal changes in the EEG or a slight widening of the temporal horn of the left ventricle All manner of.

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