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Epidemiology of hearing disorders. Some factors affecting hearing. The Copenhagen Male Study.

This epidemiological investigation reports on the results from a 10-year follow-up examination concerning hearing and factors known to cause hearing problems. In addition, an evaluation concerning the possible association between hearing impairment and factors affecting the state of health is reported. The original sample comprised 5,050 subjects, and at the present examination 3,387 (67%) males at a median age of 63 years (range 53-75) participated. An increasing prevalence of 30-40% of hearing problems was demonstrated with increasing age. A prevalence of 17% of tinnitus of more than 5 min duration was found; 3% indicated that their tinnitus was so annoying that it interfered with sleep, reading and/or concentration. The prevalence of tinnitus increased up to the age of 70, and seemed to remain constant thereafter. A significant correlation between experienced hearing problems and environmental factors causing damage to the hearing organ was found. Furthermore, a correlation between hearing problems and C3-S complement was found, but no correlations could be established between hearing problems and other blood groups. No correlation was found between cardiovascular disease and hearing problems except for minor circulatory problems, nor between hearing problems and hormonal diseases. It is concluded that the present study supports previous epidemiological investigations concerning the prevalence of hearing problems and factors affecting hearing. The association between hearing problems and C3-S complement, which to the authors' knowledge has not previously been reported, should be further evaluated. No association was found between hearing problems and other blood groups.

Age Factors↗

[The status of psychogenic hearing disorder today].

The general symptoms of psychogenic hearing disorders are pointed out and exemplified by 16 own cases seen by the author in 1987 to 1988. Psychogenic hearing disorders imitate various constellations of findings and histories of genuine organic hearing disorders. The individual's experience and the contemporary consciousness of hearing disorders seem to enter into the individual manifestation. The 16 cases can clearly be divided into 3 groups of equal size: 1. The unilateral acute hearing loss, imitating either an acute traumatic damage or an idiopathic sudden hearing loss. This was seen almost exclusively in children. 2. The bilateral chronic hearing loss. This, too, was seen mainly in children and youths. 3. The acute bilateral recurring or progressing hearing disorder. This was seen predominantly in adults, mimicking dramatic courses ending up in bilateral profound deafness. In part of the cases the psychological background of the neurotic reaction could be explored quite easily. Knowledge of the various manifestations of psychogenic hearing disorders is important, because otherwise, as the reported cases demonstrate, misleading interpretation of findings entails costly diagnostic procedures (computerized tomography, arteriography) and expensive therapeutic measures (hospitalization, fitting of hearing aids).

Adolescent↗

Aspects in diagnostics of central neural hearing disorders.

The central neural hearing disorders - in contrast to central cortical lesions - can be concretely defined audiometrically, and they can be localized realiably within the 2nd-4th neurons. For this purpose a definite test program is needed; especially indicative are the behavior of the acoustico-facial stapedius reflex and the results in dichotic discrimination and ERA. SISI and difference limen mostly point to a neural dysfunction, but - in distinct patterns of impairment - they may correspond to the picture of a peripheral hearing disorder as well. A fatigue of hearing apparently only appears if the myelin is damaged, at - perhaps additional - axonal lesions it shows up less clearly or not at all. The deficiency pattern of the stapedius reflex reflects up less clearly or not at all. The deficiency pattern of the stapedius reflex reflects the course of the reflex arc within the brain stem; accordingly, more central lesions of the auditory pathway do not affect the reflex. ERA mostly makes perceivable a lesion of synchronization, which only becomes noticeable at fast repetition rates with ultra short stimuli (1/4 ms), i.e., solely in the responses of the brain stem. The cortical responses, on the contrary, are not impaired more than the subjective threshold renders to be expected, they even show the same values of the pure tone audiogram. The dichotic discrimination is nearly always disturbed unilaterally, at processes of the brain stem homolaterally, at cortical foci predominantly leftside, perhaps because the left hemisphere dominates in speech discrimination. The otologist should know how to make use of the possibility of neuro-otologic diagnostics.

Adult↗

Vestibular findings in sensorineural hearing disorders. Results of caloric, oculomotor and hearing tests in 205 patients with unilateral hearing dysfunction.

In 205 patients with unilateral hearing loss, 117 of cochlear and 88 of retrocochlear origin, thorough audiovestibular examinations were performed to establish the occurrence and severity of vestibular dysfunction. The results were also analysed with the aim of determining the relationships between the severity of the hearing loss, the etiology and the topical location of the hearing disorder, on the one hand, and vestibular dysfunction, recorded as reduced caloric sensitivity and occurrence of oculomotor disturbances, on the other. No correlation was found between severity of hearing loss and recordable vestibular dysfunction, in either the cochlear or retrocochlear group of patients, or in the etiological subgroups of these main groups. With respect to the relations between the topic locations of the lesions and the results of vestibular tests, distinct characteristics were observed. In the affected ears, totally extinguished caloric reactions were more than six times as frequent in the retrocochlear group as in the cochlear group and appeared as a specific but rather insensitive sign of acoustic neurinoma. Oculomotor disturbances were only exceptionally observed in patients with cochlear lesions, but were noted in nearly half of the retrocochlear group; when only those patients with lesions of the brain stem or cerebellum were considered, all were found to exhibit such disturbances. The relations between audiometric hearing test pattern, caloric sensitivity and oculomotor disturbances seem to form interesting diagnostic paradigms of great value for the topical localization of audiovestibular disorders.

Auditory Pathways↗

Screening for neonatal hearing disorders in the Province of Central Finland.

Screening for congenital hearing disorders in newborn infants has been carried out in the Province of Central Finland since 1967. In 1967-1971, a total of 23 children (12 boys and 11 girls) were found to have congenital hearind disorders. From this it can be calculated that 90 infants are born each year with congenital hearing disorders in Finland. Our sample did not include slight or moderate hearing disorders (under 45 dB). 11 children under the age of 18 months were provided with a hearing aid. In spite of the screening, 12 cases (mean age 2 years) were not diagnosed until they came to the phoniatric clinic because of delayed speech development. These children were presumably also congenitally deaf. Most of the children in the delayed group reacted favourably to the auropalpebral reflex test on the obstetrical ward; their speech development was better and they also profited more from having a hearing aid than those children whose hearing defect had been discovered earlier. Screening for hearing disorders should be made nation-wide in order to avoid the harmful effects of the delay of rehabilitation on the child's development. The possibility of a hearing disorder should be kept in mind in those cases where there is a delay in speech development and also in those cases where the child has some other severe congenital defect which demands the attention of the nursing staff.

Child, Preschool↗

[Diagnosis of hearing disorders in premature infants].

In cases of severe hearing loss in babies an early rehabilitation should prevent a delay in development. More than a half of all hearing disorders in children are acquired; to a large extend these hearing disorders are caused by premature birth and can be detected by suitable screening. Two methods are used for testing: (1) reflex audiometry with a standardized stimulus generator; and (2) bedside recording of brainstem potentials. Having tested the method of bedside recording of brainstem potentials a group of 60 premature infants of varied body weights and no additional diseases was examined as "normal" control group. Only premature birth with all signs of maturation adequate for gestational age is not a greater risk for hearing disorders. In premature infants with birth weights less than 1500 g, having marked hyperbilirubinemia and extensive intracranial bleeding always increased wave V latencies has been observed. In about 10% of these children severe hearing disorders were diagnosed and after leaving the premature department were sent to a paedaudiological dispensaire.

Audiometry, Evoked Response↗

Epidemiology and etiology of acquired hearing disorders in childhood in the Cologne area.

Because of their serious consequences for all aspects of development, severe hearing disorders in children are an important clinical entity. A better understanding of the epidemiology and etiology of acquired hearing disorders may potentially lead to more effective prevention. Marked differences in the epidemiology of acquired hearing disorders are reported in the literature. In order to illuminate the problem of acquired disorders and to define current etiological causes with special regard to ethnological factors in the Cologne area, we started a retrospective study. A total of 314 children in residence at the school for hearing impaired children in Cologne were investigated. We also obtained concise data on the structure of the population in order to determine prevalence of acquired hearing disorders in the German and immigrant portions of the population. We found an overall prevalence of 0.43 per thousand for severe hearing disorders and deafness. German children were most frequently affected by acquired, and non-German children by hereditary hearing disorders. The most common causes of acquired severe hearing disorder were the perinatal complex of premature delivery, anoxia and hyperbilirubinuria or kernicterus. Other causes were congenital rubella infections and meningitis in early childhood. The accumulation of perinatal etiologies seems to be the reason for the remarkable majority of acquired hearing disorders among German children. One reason might be progress in neonatology which exerts two effects. It decreases perinatal mortality but increases perinatal complications like acquired hearing impairment. The incidence of congenital rubella in Germany has decreased during recent years, reaching a low level.

Child↗

Bilateral sensorineural hearing disorders in children: etiology of deafness and evaluation of hearing tests.

OBJECTIVE: The purpose of this study was to determine the etiology of bilateral sensorineural hearing disorders in children and to evaluate the performed hearing tests by comparison of the results of the objective and subjective tests. METHODS: The medical history and the hearing tests (behavioral observation audiometry, acoustic evoked potentials and pure tone audiometry) of 106 bilaterally hearing impaired children were analyzed in a retrospective follow-up study. RESULTS: The total group included 52 males and 54 females. The ages at first diagnosis ranged from 4 months to 11 years with a mean age of 42 months and a median of 33 months. The degree of hearing loss for the better hearing ear was mild in one child, moderate in 28 children, severe in 29 children, profound in 32 children and total in 16 children. The delay between the first examination and diagnosis ranged from 0 to 597 days with a mean of 83 days and a median of 28 days. In 47 children (44%) no cause of hearing impairment could be determined. Nineteen children (18%) had a history of familial hearing loss, 40 (38%) suffered from acquired hearing loss (seven children had prenatal causes, 21 perinatal and 12 postnatal). A comparison between behavioral observation audiometry and brainstem evoked response audiometry revealed a statistically good agreement. Twenty-nine children (32%) showed progressive hearing loss, which was defined as a threshold shift of +10 dB or more in the pure tone average in at least one ear. CONCLUSIONS: In a significant number of children with early hearing impairments the etiology still remains uncertain. Further research in the field of genetic disorders will diminish this number. Evaluation of hearing tests showed that behavioral observation audiometry still is an excellent tool in the hands of an experienced examiner. The age at identification of hearing disorders in industrialized countries still is unacceptably high. To obtain ideal care of hearing impaired children, universal neonatal hearing screening programs are mandatory.

Age Distribution↗

[Patterns of hearing disorders in normal otoacoustic emissions].

OBJECTIVE: To analyze and outline patterns of hearing disorders in normal otoacoustic emissions. METHOD: Tests of pure tone audiometry, acoustic impedance test, auditory brainstem response (ABR), 40 Hz AERP,spontaneous otoacoustic emissions (SOAE), transient evoked otoacoustic emissions (TEOAE), distortion evoked otoacoustic emissions (DPEOAE)and imaging examinations of CT/MRI scan were performed on all 83 patients with hearing disorders. RESULT: Patterns of hearing disorders in normal otoacoustic emissions including (1) 68 patients with auditory neuropathy; (2) 2 patients with unilateral acoustic neuroma; (3) 3 patients with cortical deafness or central hearing loss; (4) 2 patients with hyperacusis; (5) 2 patients with functional deafness; (6) 6 patients with malingering. CONCLUSIONS: OAE tests play an important role in diagnosis and differential diagnosis of the patients with retrocochlear deafness, central hearing loss, nonorganic hearing loss and some other special hearing disorders.

Adolescent↗

Further observations on the role of the MHC genes and certain hearing disorders.

The pathogenetic mechanism of many hearing disorders have not been fully defined. Studies of certain hearing disorders in man have suggested a role for the major histocompatibility complex (MHC)-encoded genes in disease pathogenesis. In a cohort of unrelated patients with Meniere's Disease, otosclerosis and strial presbycusis as well as other types of sensorineural hearing losses, we have identified an extended MHC haplotype common to the majority of these patients, supporting a hypothesis that a gene(s) within the MHC domain may confer susceptibility to these hearing ailments. In addition, a preliminary study of 27 individuals with various hearing maladies, a striking finding is that 44% of the patients express the following extended MHC haplotype in contrast to only 7% of the general population: DQw2-DR3-C4BSf-C4A0-G11:15-Bf:0.4-C2a-HSP70:7.5-TNF a5-B8-Cw7-A1. The expression of this haplotype by subsets of patients with hearing loss is significant in comparison to regional and international controls.

Blotting, Southern↗

[Significance of ERA for differentiating hearing disorders of different origins].

The reliability on audiometric findings in hearing disorders of different genesis has been considerably improved by the ERA. By no means, the ERA can replace the conventional hearing tests, rather it is an additional technique of examination. Apparently it does not reveal specific findings of psychogenic hearing disorders - unless the prolongation of the cortical response as repeatedly seen by us. In case of aggravation ERA renders us possible to detect the kind of hearing defects and the actual threshold - e.g. with respect to high frequency range by brain stem responses, for 500 and 1,000 Hz according to the cortical responses. The neural hearing impairment caused by tumours always exhibits extremely prolonged latencies even at high intensities, eventually concomitant with a response threshold elevated versus the subjective threshold. These reactions, above all, become apparent in the brain stem rather than in the cortex. In contrast to this the sensoric hearing disorders the ERA elucidates even objectively the recruitment: normal latencies at high intensities. The latency of the objective response, thus, corresponds to the sound stimulus is perceived loud, and it is long for stimuli perceived relatively low - even in spite of great intensity. Comprising the ERA the quote of audiometric failures in excluding an acoustic neuroma is reduced to a minimum.

Adolescent↗