Yoshisato Tanaka

Efficacy of tympanostomy tube insertion for otitis media with effusion in children with Down syndrome

OBJECTIVE Although the insertion of tympanostomy tubes is regarded as an effective treatment for otitis media with effusion in the general population, it remains to be determined whether tympanostomy tube insertion is also effective for otitis media with effusion in children with Down syndrome. The present study was carried out to determine the efficacy of tympanostomy tube insertion in children with Down syndrome. PATIENTS AND METHODS We studied 28 children (18 males and ten females) with Down syndrome and 28 age-matched control children who underwent tympanostomy tube insertion and were followed up for more than 2 years, up to 7 years of age or older. The children were followed up every month for 6 months after the operation and every 2 months thereafter. The tympanostomy tubes were not removed unless granulation tissue appeared around the ventilation tubes. RESULTS The cure rate for otitis media with effusion was lower in the children with Down syndrome than in the age-matched control children. Sequelae of otitis media with effusion (atelectatic eardrum, permanent perforation of the eardrum and middle ear cholesteatoma) were significantly often encountered in the former group. The children with Down syndrome had more frequent episodes of otorrhea from the tympanostomy tubes than the control children and antibiotic-resistant-bacteria were frequently isolated. Moreover, improvement in hearing acuity after the placement of tympanostomy tubes was not always achieved in children with Down syndrome. CONCLUSION The efficacy of the tympanostomy tube insertion for children with Down syndrome was much lower than in control children. We propose that in children with Down syndrome conservative management should be the treatment of first choice and that the insertion of tympanostomy tubes should be indicated only when hearing loss due to middle ear effusion is in a severe degree and when pathological changes of the eardrum, such as adhesion and deep retraction pocket formation, are going to occur.

Neuropathology of Auditory Agnosia Following Bilateral Temporal Lobe Lesions: A Case Study

Our patient was first diagnosed with auditory agnosia following his second cerebral vascular accident (CVA) in 1975 when he was 37 years old. Comprehensive follow-up examinations of auditory function were periodically conducted until his sudden death 15 years later. His brain was studied postmortem for neuropathology. Initial pure-tone audiometry revealed moderate sensorineural hearing loss in the right ear and mild sensorineural hearing loss in the left ear. However, repeated pure-tone audiometry revealed that thresholds became progressively poorer over time, bilaterally. Speech audiometry of both ears consistently revealed that the patient was unable to discriminate any monosyllabic words (i.e. speech intelligibility scores were 0%, bilaterally). In general, speech and hearing tests demonstrated that he could not comprehend spoken words, but could comprehend written commands and gestures. Postmortem neuropathological study of the left hemisphere revealed total defect and neuronal loss of the superior temporal gyrus, including Heschls gyrus, and total gliosis of the medial geniculate body. In the right hemisphere, examination revealed subcortical necrosis, gliosis in the centre of the superior temporal gyrus and partial gliosis of the medial geniculate body. The pathological examination supports clinical results in which the patients imperception of speech sounds, music and environmental sounds could be caused by progressive degeneration of bilateral medial geniculate bodies.Our patient was first diagnosed with auditory agnosia following his second cerebral vascular accident (CVA) in 1975 when he was 37 years old. Comprehensive follow-up examinations of auditory function were periodically conducted until his sudden death 15 years later. His brain was studied postmortem for neuropathology. Initial pure-tone audiometry revealed moderate sensorineural hearing loss in the right ear and mild sensorineural hearing loss in the left ear. However, repeated pure-tone audiometry revealed that thresholds became progressively poorer over time, bilaterally. Speech audiometry of both ears consistently revealed that the patient was unable to discriminate any monosyllabic words (i.e. speech intelligibility scores were 0%, bilaterally). In general, speech and hearing tests demonstrated that he could not comprehend spoken words, but could comprehend written commands and gestures. Postmortem neuropathological study of the left hemisphere revealed total defect and neuronal loss of the superior temporal gyrus, including Heschls gyrus, and total gliosis of the medial geniculate body. In the right hemisphere, examination revealed subcortical necrosis, gliosis in the centre of the superior temporal gyrus and partial gliosis of the medial geniculate body. The pathological examination supports clinical results in which the patients imperception of speech sounds, music and environmental sounds could be caused by progressive degeneration of bilateral medial geniculate bodies.

The progress of adrenoleukodystrophy as revealed by auditory brainstem evoked responses and brainstem histology

ZusammenfassungEin sechs Jahre altes Kind erkrankte im 5. Lebensjahr an Adrenoleucodystrophie (progressive metabolische Erkrankung des ZNS mit rapider Degeneration). Die Krankheit begann mit Dysarthrie, Schwerhörigkeit und Gangunsicherheit. Später kam es zur spastischen Paralyse, zu hochgradiger Schwerhörigkeit und Erblindung. Tod durch Atemlähmung.Während des Verlaufs wurden regelmäßig die Hirnstammpotentiale sowie die langsamen Rindenpotentiale abgeleitet. Dabei war mit fortschreitender Erkrankung eine deutliche Verminderung der Potentiale zu beobachten. Histologisch zeigten sich eine Entmyelinisierung des Hörnervs sowie Neuronenverluste im Verlauf der Hörbahn. Die Degeneration im Hirnstamm verlief dabei von rostral nach kaudal.SummarySerial studies of auditory brainstem evoked responses (ABR) and slow vertex responses (SVR) were obtained during the progress of adrenoleukodystrophy in a 6-year-old boy. This child was normal until 5 years of age. His illness began with a gait disturbance, dysarthria, and hearing difficulty. Later, spastic paralysis, serious deafness, and blindness appeared. He died of respiratory failure 2 years after the onset.The ABR was normal at onset but changed to an abnormal pattern. Initially, there was lengthening of the wave V-I interpeak interval. This was followed by the disappearance of the later components as his general condition deteriorated. At the terminal stage, only a prolonged wave I was recordable. The postmortem pathology revealed demyelination of auditory nerves and remarkable neuronal loss in the auditory pathways of the brainstem; in addition, there was a variety of extensive degeneration throughout the cerebrum, in particular the complete degeneration of the white matter with secondarily occurring ganglionic cell changes. These data suggest that degeneration of the brainstem from rostral to caudal levels occurred.

Speech discrimination and lip reading in patients with word deafness or auditory agnosia.

The purpose of this study was to assess the ability of four patients with word deafness or auditory agnosia to discriminate speech by reading lips. The patients were studied using nonsense monosyllables to test for speech discrimination, a lip reading test, the Token Test for auditory comprehension, and the Aphasia test. Our results show that patients with word deafness or auditory agnosia without aphasia can improve speech comprehension by reading lips in combination with listening, as compared with lip reading or listening alone. In conclusion, lip reading was shown to be useful for speech comprehension among these patients.

Auditory brain stem responses and nonsense monosyllable perception test findings for patients with auditory nerve and brain stem lesions.

A nonsense monosyllable audiometric test was administered to 15 patients with eighth nerve or brain stem disorders caused by tumor, hemorrhage, encephalitis, and degenerative disease. Auditory brain stem responses (ABR) were abnormal in all patients. ABR abnormalities were defined by the absence of some or all waves or by a prolongation of the wave V‐I interval. The discrimination scores of the nonsense monosyllables were significantly lower in patients with completely absent ABR, with partial ABR series involving wave I, or with severely prolonged wave V‐I intervals (over 3 SD). However, in patients with wave I only or with only moderately prolonged wave V‐I intervals (less than 3 SD), test scores were within normal range. It is concluded that: 1. perception of nonsense monosyllables could be, though need not be, affected in patients with brain stem lesions; 2. eighth nerve lesions severely disrupt auditory comprehension as well as perception of nonsense monosyllables.

Normalization of Poor Auditory Brainstem Response in Infants and Children

In these past 10 years, the usefulness of auditory brainstem response (ABR) has been demonstrated in the clinical evaluation of audiological or neurological disorders, and the absence of an ABR is commonly accepted as consistent with a peripheral hearing loss caused by middle or inner ear diseases. In this article, the authors report nine infants and children who had an absent or difficult to detect ABR at the first examination with subsequent normalization of ABR. Repeated otological examinations were always normal in these patients. The final diagnoses were delayed development, Waardenburg syndrome with congenital heart disease and epilepsy, healthy infant, infantile convulsion, mental retardation with history of low birth weight, in each case, and chromosomal aberration in four patients. The reason for normalization of ABR in our patients is not clear and is yet to be ascertained. However, the authors believe that neurological maturation rather than an improved primary peripheral disorder is responsible for the noted normalization of the ABR, because of the high incidence of neurological abnormalities in these patients.

Application of brain stem response in brain-injured children

The early components of the auditory evoked potential within 10 msec following an auditory stimulus are attributed to the brain stem auditory nuclei and pathways. In pediatric neurology the auditory brain stem response (ABR) can be applied to: 1) differential diagnosis of hearing impairment in young children including objective threshold measurement of hearing, 2) electrophysiological evaluation of maturation of the auditory pathways, 3) diagnosis of the site and/or extent of neurlogical diseases affecting the brain stem and 4) observation of a degenerating process of degenerative diseases in the central nervous system. The paper is especially concerned with the application of ABR to severe neurological diseases in children including central auditory dysfunction, cerebral palsy, infantile spasm, adrenoleucodystrophy, anoxic brain damage and Downs syndrome. Value and limitation of ABR audiometry in the clinical practice were mentioned, and a special emphasis was placed on the fact that all types of auditory tests including behavioral, electrophysiological, and developmental tests are indispensable, because the ABR, like other indicators, also has its own limitation.

Auditory air and bone conduction brainstem responses and damped rotation test for young children with bilateral congenital atresia of the ears.

This paper describes our assessment of auditory and vestibular functions at a very early age using the auditory air and bone conduction brainstem responses and the damped rotation test for infants and children with bilateral congenital atresia of the ear. Twelve infants and children with congenital microtia and occlusion of bilateral auditory canals were evaluated by this assessment to determine their function of hearing and balance and their suitability for fitting and surgical improvement.

Changes in auditory behaviors of multiply handicapped children with deafness after hearing aid fitting

Conclusion. The early diagnosis of deafness and the early fitting of hearing aids in multiply handicapped children are recommended for language development in these children even when their neurological or mental status is poor. Subjects and methods. The subjects consisted of 5 hearing-impaired infants with no other problems and 28 hearing-impaired children with multiple handicaps. Behavioral audiometry and auditory brainstem responses were used for evaluating hearing impairment. The 5 hearing-impaired infants with no other problems underwent hearing aid fitting at approximately 1 year of age and the 28 hearing-impaired children with multiple handicaps underwent hearing aid fitting at various times from 1 to 5 years of age. The effects of their hearing aids were compared on the basis of auditory behavioral changes. The developmental scale of auditory behaviors in infancy that we proposed was introduced to evaluate the development of auditory behaviors. Results. The auditory behaviors of the hearing-impaired children with no other problems showed constant changes with age after hearing aid fitting. However, among the 28 hearing-impaired children with multiple handicaps, 17 showed improvement in auditory behaviors, 5 showed no improvement in auditory behaviors because of the associated severe motor and mental retardation, and epilepsy, and 6 were unable to adapt to wearing hearing aids.

Assessment and Intervention Programmes for Hearing‐impaired Infants in Teikyo University Hospital

The purpose of this paper is to provide an overview of the necessary components in our clinic, and of effective assessment and intervention with young hearing‐impaired infants in the area of language‐speech pathology and audiology. From 1973 to 1994, 4,155 infants were diagnosed as hearing impaired in our clinic. During this period one thousand and nine hundred and ninety‐five cases and their families received our programmes, which are called Home Training Programmes for Hearing‐impaired Infants. The rationale behind these programmes is based on the responsibility of the caregivers for bringing up their children and the efficacy of inter‐relationship between infants and families at home. It is concluded that these habilitation programmes that are conducted by medical and co‐medical professional staffs are a necessary and an effective support for families with hearing impaired infants.