[Disordered hearing (2). Hearing disorder as a handicap].
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The results of international investigations on connatally acquired hearing loss are compared with the data of the German Registry on Childhood Hearing Loss (4058 cases). The connatal hearing disorders have shown a notable change in the last years regarding to aetiology and prevalence. In contrast to countries of the third world in developed nations the prevalence of permanent childhood hearing loss has been reduced down to 1 in 1.000 births. The results let assume a prevalence of approximately 1:1.200 births in Germany. For instance the number of rubella embryopathia decreased effectively. In contrast CMV infections and alcohol fetopathia are playing an increasing role. In the patients of the German Registry on Childhood Hearing Loss the percentage of certainly progressive hearing loss is 10.3 within the 4058 children with permanent hearing impairment. Diagnostic procedures first of all for the early diagnosis of CMV but also of toxoplasmosis are considerable because these infections may result in treatable hearing loss. Also consequent hearing tests are demanded in children with alcohol fetopathia.
Disordered hearing in childhood has a major negative effect on the entire development, in particular the development of speech. Thus, early detection and proper assessment of a hearing impairment is of considerable importance. Only adequate diagnostic information makes it possible to initiate the necessary corrective measures without delay. The diagnostic possibilities for detecting and differentiating hearing impairments are discussed. The general need to perform a hearing test at the age of six and seven months, and the performance of hearing tests designed for the newborn who are at risk, are emphasized.
After a brief summary of the problems of rehabilitation of hearing-impaired subjects, the optimal conditions for rehabilitation with hearing aids are presented: a) The right timing is crucial. b) Optimal provision of hearing aids must be carried out in close co-operation between the patient, the hearing aid technician and the otolaryngologist. c) Easy handling of the hearing aid and the use of attachments must be guaranteed. d) The hearing-impaired person must be fully informed as to the extent and type of hearing loss. He/she must accept the affliction and know about the possibilities of rehabilitation. The patient's motivation is a pre-requisite for all further steps. e) The patients must learn tactical measures to make optimal use of their hearing ability in relation to their environment. Hearing tactics consist of hearing training and a change in the attitude of the hearing-impaired patients themselves and their attitude towards their surroundings.
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Sensorineural hearing loss is a common disorder that results from damage to the inner ear in over 95% of all cases; therefore, retrocochlear hearing disorders are rare and cannot be differentiated from sensory losses by clinical symptoms alone. Associated vestibular and other neurotological symptoms must lead to appropriate diagnostic procedures. Besides audiological and neurotological tests, e.g. auditory-evoked potentials, otoacoustic emissions or electronystagmography, modern imaging methods are the most valuable tools to detect tumorous, vascular and inflammatory processes. Acoustic neuromas, which are the most frequent causes, require otoneurosurgery with the attempt to preserve hearing and facial nerve function. Multiple sclerosis and vascular lesions of the vertebral arteries or their branches require an interdisciplinary approach. Due to recent advances in diagnostics, imaging and management, retrocochlear disorders have gained much interest from otologists in the last few years. They provide new insight into the physiology and pathophysiology of hearing.
Psychogenic hearing problems exist even in childhood. They must be differentiated from the stimulated or aggravated hearing losses. A characteristic is the discrepancy between the understanding during conversation and the bad results of the hearing tests. The psychical case history of the patient shows his conflict-situation and personal problems. If the psychogenic hearing problems do not improve on their own or by means of oto-rhinolaryngical placebo-therapy, psycho-therapy will be needed. Taking the example of 2 children, where the suspected primary diagnosis was sudden deafness, the problems of diagnosis was shown.
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