King-Kopetzky syndrome is the term used to describe an obscure auditory dysfunction or auditory processing disorder. Auditory means “pertaining to the auditory system.” This disorder is still relatively understudied, but affects about ten percent of all patients who seek medical treatment for hearing problems. Adults, older children and adolescents are particularly affected.
What is King-Kopetzky syndrome?
King-Kopetzky syndrome (KKS) is part of the clinical picture of auditory processing and perception disorders. The ability to hear with understanding is divided by medical experts into auditory perception and auditory processing of information. If central processes of hearing are disturbed, changes in time, intensity or frequency of acoustic information may not be analyzed and processed properly. In this case, sounds are only inaccurately localized or interfering sounds are not suppressed. King-Kopetzky syndrome specifically refers to a dysfunction with poor speech comprehension in the presence of background noise. The syndrome was named after Samuel J. Kopetzky and P. F. King, who first studied the disorder in more detail in the early 1950s.
Causes
The causes of King-Kopetzky syndrome have not been conclusively determined. Organic and genetic, as well as psychological, influencing factors are suspected. Since a familial accumulation of the syndrome is observed, heredity cannot be excluded. As an example of a psychological trigger of the syndrome is auditory stress. The sensitive inner ear is exposed to a wide variety of acoustic demands on a daily basis and reacts very sensitively as an early indicator of various diseases. For example, latent aircraft noise at night or the traffic noise of a nearby highway can mean acoustic stress, which, if persistent, can manifest itself into a processing and perception disorder or other disease of the ear. Strikingly, a large proportion of those affected experience concurrent psychological abnormalities such as increased levels of social anxiety or obsessive-compulsive disorder.
Symptoms, complaints, and signs
Affected individuals with King-Kopetzky syndrome describe impairments in hearing and understanding speech, especially when noise interference is added in parallel. Different sub-services of auditory perception may be affected individually or in combination. For example, the ability to spatially classify and localize the sound source of the speaker can be impaired. Deficits within auditory selection lead to patients not being able to filter out speech information from all the simultaneously occurring everyday noises (conversations of other people, operating noises of machines and fans, etc.). In school, for example, children with auditory selection disorder have difficulty filtering sounds from words in ambient noise and understanding what the teacher is saying. Similarly, the perceptual and processing problems may occur with similar-sounding tones.
Diagnosis and course of the disease
If a patient with hearing difficulties seeks medical treatment, the examination is usually performed by an otolaryngologist or physician with a focus on pediatric audiology. First, acute organic causes such as a middle ear or ear canal inflammation are examined by otoscopy. The diagnosis also includes a detailed discussion of the symptoms and previous illnesses. If a peripheral hearing disorder, i.e. direct damage to the ear, has been ruled out, various hearing tests are performed. In the clinical picture described, affected persons show minor abnormalities in many parts of the auditory system, mostly in the inner ear, in the sound audiogram. Furthermore, a questionnaire is completed to record the “Social Hearing Handicap Index” (SHHI), which results in conspicuous values in this clinical picture. Many of those affected also have a pronounced hypersensitivity to noise, which causes protective behavior that increases in the course of the disease. If the disease remains untreated, difficulties arise for those affected, especially if psychological abnormalities, such as those mentioned above, occur at the same time. Patients who suffer from social anxiety and have difficulties with speech comprehension quickly fall into social isolation.Furthermore, auditory processing and perception disorders are often accompanied by language development delays, reading and spelling difficulties, and attention deficits. Affected persons cannot perceive sounds of speech correctly and thus cannot reproduce them correctly. A diagnosis as early as possible is therefore essential, especially in children and adolescents, in order to be able to promote further development accordingly.
Complications
King-Kopetzky syndrome predominantly causes complaints and disorders of hearing. These can significantly restrict the daily life of the affected person and further reduce the quality of life. Similarly, there are problems with understanding and hearing speech, so that ordinary communication is usually not possible for the affected person.
King-Kopetzky syndrome can thus also increase certain risks in life, since dangers may not be recognized in time. It is not uncommon for developmental and concentration disorders to occur, especially in children. Those affected also suffer from inflammation of the ear or ear canal. In the worst case, this can lead to complete hearing loss. Furthermore, the children may also suffer from disorders of language development and attention deficit disorders. Treatment of King-Kopetzky syndrome is causal and symptomatic. In many cases, the underlying disease can no longer be limited and the patient is dependent on various therapies to avoid complications in everyday life. Usually, a hearing aid can compensate relatively well for the symptoms.
When should one go to the doctor?
People who notice limitations in their hearing should see a doctor. If other symptoms are added, for example, problems with processing what is said or neurological deficits, it is necessary to go to an otolaryngologist or ear, nose and throat specialist with the complaints on the same day. The physician can rule out acute causes and make the diagnosis. People who are repeatedly exposed to auditory stress are particularly likely to develop King-Kopetzky syndrome. The same applies to people who suffer from social anxiety or other psychological complaints. Previous diseases of the inner ear can also lead to the development of King-Kopetzky syndrome. Those who belong to these risk groups are best advised to consult their doctor immediately and have their complaints clarified. Affected children must be taken to the pediatrician. If no treatment is given, auditory dysfunction will progress and lead to communication problems and other complications as it progresses. Anyone who notices signs of auditory dysfunction in a relative or friend should seek medical evaluation.
Treatment and therapy
King-Kopetzky syndrome can be treated in several ways, depending on the suspected cause. Targeted functional auditory training to improve phonological awareness has a positive effect on the ability to differentiate sounds, according to numerous scientific studies. Ideally, this also results in an improvement in performance in the case of reading and spelling difficulties. If the underlying causes are psychological and the disease is accompanied by other neurological symptoms, it is also recommended that treatment be coordinated with a psychotherapist or psychiatrist. Holistic behavioral therapy concepts in conjunction with stress reduction and the administration of medication support the recovery process and help to reduce anxious tension. In group situations, in addition to attentive listening, patients are trained, for example, to ask questions when they do not understand or to create mnemonic devices. Often, a special coping strategy is recommended. In the case of hearing difficulties, those affected should consciously use additional, inaudible sources of information and, for example, read lips in parallel. If measurable hearing loss has been identified during the hearing test, therapy may also recommend the use of a hearing aid if necessary.
Outlook and prognosis
Because King-Kopetzky syndrome often occurs in conjunction with anxiety and obsessive-compulsive disorders, affected individuals should first clarify whether such disorders are present in them. If this is the case, targeted psychotherapy can bring great success and at the same time also improve the present perceptual and processing disorders.Auditory stress such as construction noise, loud music but also screaming children, can be triggers of the disease and also worsen it with renewed noise. In everyday life, it is often difficult to escape the ambient noise, so it is important to ensure the necessary quiet, at least in the private environment. The installation of multiple soundproof windows can be a remedy, but in the case of rental properties, this must be coordinated with the landlord. If one does not want to do without going to concerts or similar events, appropriate hearing protection must be purchased in consultation with the doctor. For affected children and adolescents, avoiding auditory stress is often much more difficult, as they naturally act louder during their leisure activities. Talking to teachers, allows children to spend their breaks protected from noise inside the school. Furthermore, teachers can make an effort to ensure that noise levels remain low during lessons. Regular examinations also make it possible to see if a modern hearing aid could lead to significant symptom relief.
Prevention
The probable causes of King-Kopetzky syndrome have not been adequately investigated or understood. Direct preventive measures can therefore not be formulated. This is especially true if the presumption of heritability of the clinical picture is confirmed. Therefore, only the recommendation of a holistically healthy lifestyle and the avoidance of stress can be cited as measures for prevention.
Follow-up
In most cases, the measures of aftercare in King-Kopetzky syndrome are severely limited or, in some cases, not available to the affected person at all. In this disease, the first priority is to see a doctor very early to prevent further complications or symptoms from developing. An early diagnosis usually has a very positive effect on the further course of the disease and can also prevent a further worsening of the symptoms. Most of those affected by King-Kopetzky syndrome are dependent on psychological treatment. The support of friends and family is often very important in order to prevent further deterioration of the psychological condition. In case of hearing difficulties, the affected person should definitely wear a hearing aid to prevent further damage to the auditory canal. In general, help and support from one’s family has a very positive effect on the further course of King-Kopetzky syndrome. Should the affected person wish to have children, genetic testing and counseling can also be performed. This can prevent the recurrence of the disease in the affected person.
What you can do yourself
The causes of King-Kopetzky syndrome have not yet been conclusively determined. However, the syndrome is strikingly associated with obsessive-compulsive disorder and social anxiety. Patients who suffer from King-Kopetzky syndrome and are mentally ill at the same time should definitely have these illnesses treated. There is a good chance that auditory processing and perceptual disorders will also improve significantly at the same time. Furthermore, King-Kopetzky syndrome can not only be triggered by auditory stress, but can also be exacerbated. Those affected would therefore do well to avoid noise as much as possible. Loud music, screaming children and construction noise are considered particularly harmful. These stress factors should be avoided. This is not always easy, especially for children and adolescents who interact with their peers. Provided the syndrome allows attendance at a normal school, teachers should definitely be informed about the disorder and allow the affected children to spend the breaks, which are usually particularly noisy, in a protected area. It is also helpful if the noise level is kept at a low level during lessons, which benefits not only the sick child but ultimately all the pupils. In any case, however, those affected should also check whether their hearing condition can be improved by a modern hearing aid, which is often the case.
