Sudden hearing loss

from Wikipedia, the free encyclopedia
Classification according to ICD-10
H91.2 Sudden hearing loss, idiopathic
ICD-10 online (WHO version 2019)

A sudden loss of hearing or idiopathic sudden hearing loss , and ear infarction , is an unknown cause ( idiopathic ) sudden, usually unilateral sensorineural hearing loss . The hearing loss in sudden hearing loss can range from mild to complete deafness , it can affect all frequencies or be limited to only a few frequency ranges . According to this definition, hearing impairments with an identifiable cause are not considered to be sudden hearing loss. The course of sudden hearing loss is very different; a relatively high rate of spontaneous healing is known . According to the current state of knowledge, it is not possible to make a reliable forecast. There is no known effective drug therapy. Other treatment measures are under discussion.

Symptoms

A sudden, mostly one-sided hearing loss is characteristic and defining. Triggering or causative factors cannot be determined. The diagnosis of sudden hearing loss is therefore a diagnosis of exclusion . The sudden hearing loss is never accompanied by earache . A one-sided feeling of pressure and ringing in the ear ( tinnitus , in 80% of cases, mostly high frequency) in the affected ear can be harbingers. Half of the patients report "fluffy" or numb skin sensations (due to the lack of acoustic feedback when the auricle is touched; there is no real hypesthesia ). 30% complain of dizziness , 15% of double-tone hearing ( diplakusis : a tone is heard higher or lower in the diseased ear) and sensitivity to noise ( hyperacusis ).

Tra Ban Huy calls for diagnosis

  1. a hearing loss of the sensorineural type ( sensorineural hearing loss ),
  2. which develops within 24 hours,
  3. with significant hearing loss in the audio audiogram ,
  4. without an identifiable cause,
  5. in severe cases with dizziness.

Diagnosis

In addition to the questioning, various tests are carried out on the functionality of the ear. With the Otoscopy be ear canal and eardrum examined. The Weber test lateralizes into the healthy ear, the Rinne test is positive on both sides. The functionality of the middle ear is checked with tympanometry . With the tone audiometry extent and frequency range of hearing loss are determined. Otoacoustic emissions (OAE) may allow evidence of a disease of the auditory nerve . Imaging procedures such as magnetic resonance imaging and computed tomography can show tumors such as acoustic neuroma , cerebral circulatory disorders and infections . The brain stem audiometry (BERA, BAER) look for arousal disabilities from the inner ear to the brain stem . Blood tests may reveal causes of circulatory disorders such as hyperviscosity , anemia , hyperlipidemia, thrombophilia , and inflammation . The blood pressure should be measured. Electrocardiography and echocardiography can detect heart disease.

Differential diagnosis

Sudden hearing impairment can be a symptom of other medical conditions. Some examples:

Causes and Risk Factors

The mechanism of sudden hearing loss has not yet been clarified. It is assumed that there is an interplay of various factors that lead to a change in the blood flow in the inner ear . This is where the hair cells , the sensory cells responsible for hearing, are located . The hair cells are supplied with substrates by diffusion from the environment, including oxygen and nutrients . Insufficient blood flow in the cochlea's blood vessels leads to damage to the hair cells. However, studies on the inner ears of acute hearing loss patients who made their organ available to science after their death did not reveal any evidence of such circulatory disorders.

In addition, stress , autoimmune diseases and cracks in the round window membrane are discussed as causes.

Contradicting research results have been published on the connection between viral infections and sudden hearing loss.

Risk factors

Systematic prospective studies of risk factors have not yet been published. According to some experts, people with overweight , high blood pressure , diabetes mellitus and lipid metabolism disorders as well as smokers are particularly at risk . Also stress could be a risk factor.

Epidemiology (frequency and occurrence)

According to studies by Klemm and Saarschmidt (1986), Michel, and Leins, up to 16,000 people per year (up to 20 per 100,000) in Germany are affected by a sudden hearing loss. In the USA in 1984 the incidence was 5-20 per 100,000; 1996 in Flanders and the Netherlands 8-14 per 100,000.

Men and women are almost equally affected by idiopathic sudden hearing loss. All ages can be affected by idiopathic sudden hearing loss. Children and adolescents are very rarely affected, 75% of all patients are older than 40 years at diagnosis. According to other sources, 60% of all idiopathic hearing loss occurs between the ages of 30 and 60.

therapy

There are several treatment methods, which all have in common that they are more or less controversial from a technical point of view. The Cochrane Collaboration has made assessments of various acute hearing loss treatments.

A scientifically well-proven therapeutic approach is currently not available, as the cause or causes of the sudden hearing loss are still unclear. It is becoming apparent, however, that therapy is currently concentrating on the use of glucocorticoids , in particular based on experience from the USA . The guidelines of the German Society for Ear, Nose and Throat Medicine, Head and Neck Surgery are a consensus paper from the point of view of German-speaking ENT doctors . There, therapy with high-dose glucocorticoids (e.g. prednisolone 250 mg) is proposed :

“The commission recommends systemic, high-dose glucocorticoid therapy as the primary treatment for acute idiopathic sensorineural hearing loss after weighing the side effects. In order to avoid systemic side effects, this can alternatively also take place primarily as intratympanic treatment after consultation with the patient. If the initial systemic treatment is unsatisfactory, it is recommended that the patient be offered intratympanic glucocorticoid therapy. "

A “pulsed” high-dose glucocorticoid therapy is also conceivable and, according to data from a 2007 study, equally effective.

The spontaneous improvement rate of the disease has been given to different levels in different studies and apparently varies depending on the severity of the hearing loss and also the pitch. The reported spontaneous healing rates are very different: In 1984 Weinaug reported a spontaneous healing rate of 68% in 63 patients. Heiden u. a. reported in a literature analysis in 2000 of spontaneous healing rates between 28 and 68%, 50% pooled . Schuknecht gives 40 to 60%.

The discussion about a suitable therapy is also based on the problem of the currently rather “thin” study situation. Effective cures should be significantly better than placebo . For ethical reasons, however, the comparison is usually not made with a placebo but with another treatment method. In two placebo-controlled studies that met modern scientific standards, blood circulation-enhancing drugs did not achieve better results than saline infusions. Systemically applied corticosteroids were not more effective than placebo in 21 randomized controlled studies published between January 1996 and February 2006, not even in combination with antivirals . A meta-analysis by the Cochrane Collaboration from October 2009 came to the conclusion that the effect of vasodilators on hearing loss has also not been proven. Due to the reduced informative value of the existing studies, further research is recommended.

Rheologics

Assuming that a sudden hearing loss is caused by a circulatory disorder in the inner ear , treatment in German-speaking countries is mostly rheological , e.g. B. with infusions from solutions of hydroxyethyl starch (HES), pentoxifylline or low molecular weight dextrans over ten days. In addition to low molecular weight dextrans, hapten dextran is administered to reduce the likelihood of potentially severe shock reactions. Substances such as piracetam or prostaglandins or prostacyclines such as alprostadil and iloprost are used much less frequently . Naftidrofuryl or Ginkgo biloba in ampoule form were withdrawn from the German market in the mid-1990s because of serious side effects.

In Germany, around 500 million euros are spent on infusion therapy every year. Rheological infusion therapy for acute hearing loss is unusual in the Anglo-American and Scandinavian countries.

Headaches, stomach pressure, the urge to urinate or sleep disorders are - depending on the remedy used - common side effects of the infusions. Less common serious side effects, including anaphylactic shock from pentoxifylline or low molecular weight dextrans, may occur. If hydroxyethyl starch is given, the substance can accumulate in the skin, especially after long-term use (when a total amount of approx. 300 g HES is exceeded) and lead to very annoying itching , which is difficult to treat and can last for a long time or even be resistant to therapy . The current guidelines therefore emphasize that the treatment cannot be recommended unconditionally.

Blood circulation-improving substances can also be taken in tablet or capsule form. These include naftidrofuryl , ginkgo biloba extracts, buflomedil , betahistine , cinnarizine and pentoxifylline . Antihypertensive drugs with a vasodilating effect, such as nifedipine or nimodipine, have also been used for acute hearing loss. However, the use of exclusively vasodilating drugs is no longer recommended due to the possible steal effect , which can inadvertently even reduce the blood flow to the inner ear.

In 2017, the IGeL monitor of the MDS ( Medical Service of the Central Association of Health Insurance Funds ) examined the scientific evidence for or against the blood circulation-enhancing infusion therapy for acute hearing loss . Since the studies in question showed that treated patients in the end did not hear better than control patients, but that the agents used (pentoxifylline and dextran) would undoubtedly have side effects, the IGeL monitor rates the infusion therapy, which stimulates the blood flow, as "negative" for acute hearing loss. The IGeL-Monitor is based on two studies in which the blood circulation-promoting agents pentoxifylline and dextran were examined. The study participants in the comparison groups received a saline solution. The result: in none of the two studies did the administration of the active ingredients reduce hearing loss better than the infusion with saline solution. The corresponding medical guidelines do not recommend this type of acute hearing loss therapy either. Blood vessel dilators could not be recommended, nor could solutions containing hydroxyethyl starch (HES) and there were no meaningful studies on pentoxifylline.

Corticosteroids

The use of glucocorticoids is based on their anti-inflammatory and immune-suppressing effects. A decongestant effect could also be important. Predominantly prednisone , prednisolone and methylprednisolone , and rarely dexamethasone, are used. The substances can be swallowed as tablets or injected ; these are systemic forms of application in contrast to local (local) treatment. The guidelines of the German Society for Ear, Nose and Throat Medicine, Head and Neck Surgery currently expressly recommend the use of high-dose glucocorticoids (prednisolone) in systemic form. If the therapy is unsuccessful, an injection should be made into the ear.

In patients with blood pressure that is difficult to control, pregnant women, diabetes with insulin therapy or gastric ulcers, the risk must be weighed against the unproven benefit and the dosage must be changed if necessary. A study published in 2012 found hyperglycaemia in 21% of non-diabetic acute hearing loss patients and in 63% of diabetics after cortisone therapy . Corticosteroids can also cause neuropsychiatric symptoms.

With local administration of corticosteroids directly into the round window niche, a small randomized study could not find any significantly better results than placebo. Another, more recent study found neither advantages nor disadvantages compared to systemic (oral) therapy.

The IGeL monitor of the MDS ( Medical Service of the Central Association of Health Insurance Funds ) rates glucocorticoids in acute hearing loss as “tending to be negative” . Two reviews consistently showed that systemic administration of glucocorticoids did not restore hearing any faster than administration of a dummy drug. The most important source of the IGeL-Monitor is a Cochrane review from 2013. So there is no evidence of a benefit, but evidence of harm. The assessment only relates to systemic administration as tablets or as an infusion, not to the local administration of glucocorticoids (anti-inflammatory agents) directly into the ear. The S1 guideline on sudden hearing loss from 2014 admits that the relevant reviews assess the importance of therapy as unclear. Also, low-dose prednisolone had shown no effectiveness in a high-quality study. The guideline concludes: “It is therefore recommended that higher-dose glucocorticosteroids be used as the initial therapy for acute hearing loss. The glucocorticoid therapy should be carried out for 3 days with 250 mg prednisolone or another synthetic glucocorticosteroid with an equivalent dose. "

Combinations

In Germany there is the so-called Stennert scheme ( Eberhard Stenner , born 1938) and modifications of it. Three drugs are used: as corticosteroid prednisone , prednisolone or methylprednisolone , pentoxifylline , and dextran or hydroxyethyl starch . The administration of the corticosteroids takes place over a period of 14 to 21 days, the daily dose is gradually reduced every 2 to 3 days. Other dosage regimens are shorter, e.g. B. 200 mg prednisolone on day 1, 150 mg on day 2, 100 mg on day 3 and 50 mg on day 4. So far it has not been proven that the combination treatments work better than monotherapy or placebo.

Anesthetics (sodium channel blockers)

In German-speaking countries, in the event of sudden hearing loss - especially in connection with tinnitus  - local anesthetics such as lidocaine or procaine are sometimes administered intravenously in addition to drugs that promote blood circulation . Since these substances can cause seizures, respiratory paralysis and cardiovascular failure, this therapy should be given in hospital. The benefits are controversial. Chronic noises in the ear can be alleviated by intravenous administration of lidocaine, but the effect wears off after a short time. There is no scientific proof of a permanent effect in acute tinnitus or sudden hearing loss. In 2004, the medical service of the health insurance companies saw the use of local anesthetics in diseases of the inner ear “with great concern” due to potentially life-threatening side effects .

Fibrinogen lowering through apheresis

The apheresis is a blood purification method in which a precipitation reaction fibrinogen is reduced from the blood. In addition, there is a reduction in LDL cholesterol and lipoprotein (a).

The use of HELP apheresis ( heparin-induced extracorporeal LDL precipitation ) in acute hearing loss in the early phase of the disease is based on studies up to evidence class 1b: A prospective, randomized, multicenter study published in 2002 suggests that patients with elevated plasma fibrinogen - Levels above 295 mg / dl may benefit compared to standard therapy. There are no long-term reliable data on effectiveness; the 2014 AWMF guideline on the treatment of acute acute hearing loss does not mention this method.

In 2013, the responsible working committee came to the conclusion that there was a lack of evidence for the effectiveness of this treatment and also criticized the quality of the above-mentioned investigation:

“The scientific publications are mainly those of the Suckfüll u. a., from which, however, no valid proof of the benefit could be obtained [...] After detailed discussion of the documents and their evaluation, the committee came to the consensual opinion that the benefit of apheresis for this indication was not proven against the background of the insufficient data situation can apply. Admission to statutory health care is refused. "

- Working Committee "Medical Treatment"

Hyperbaric oxygen therapy

The hyperbaric oxygen therapy (HBO) is based on multiple stays in a medical pressure chamber, where pure oxygen is inhaled under increased ambient pressure. The success of this therapy is controversial; proponents put it at around 50 percent. A randomized study finds that infusion therapy and HBO are equivalent. Even if other, similar studies rate the HBO as having advantages, it follows that the more economical therapy options should be used first and the HBO should be a "reserve therapy" (recommendation of the Association of German Pressure Chamber Centers VDD eV and Arnold 2010) . The guidelines of the American scientific society for ENT were added to the HBO in 2011. The Cochrane Review in October 2012 summarized the study situation as follows:

“For people with acute ISSHL, the application of HBOT significantly improved hearing, but the clinical significance remains unclear […] There is no evidence of a beneficial effect of HBOT on chronic ISSHL or tinnitus and we do not recommend the use of HBOT for this purpose. "

“In people with acute hearing loss, HBO therapy significantly improved hearing, but the clinical significance remains unclear […] There is no evidence of the benefit of HBO therapy for chronic hearing loss and tinnitus, and we do not recommend its use in this indication . "

- Cochrane Review, 2004

The German joint federal committee last analyzed the situation in 2000 and judged it to be insufficient for approval for statutory health insurance. Private health insurances and the allowance usually cover the treatment costs.

The IGeL monitor of the MDS ( Medical Service of the Central Association of Health Insurance Funds ) also sees the study situation critically and rates hyperbaric oxygen therapy for sudden hearing loss as “tending to be negative”. The studies, which are not very conclusive, do not show that hyperbaric oxygen therapy can cure acute hearing loss or even have a beneficial effect on it. Therefore one sees no evidence of a benefit. In contrast, a few patients reported unpleasant side effects. In addition, the mechanism of action is speculative, since the causes of the sudden hearing loss are unclear. The IGeL monitor is based primarily on a Cochrane Review from 2007. The conclusion of this review article: There is no justification for routine use of HBOT, the results are due to the small number of studies, the modest number of patients and the methodological Inadequacies of the studies are not very informative.

Tympanoscopy

In individual clinics, a tympanoscopy is performed in the event of severe, therapy-resistant or recurring sudden hearing loss . Assuming that a rupture of the round window membrane is responsible for the hearing loss, this tear should be sealed with a tissue flap. Critical voices question the reliability of the diagnostic tests and also stress the unproven effect of the operation on hearing. In the current treatment guidelines, tympanoscopy is only recommended for special individual cases.

Vitamin C Infusion Therapy

There is increasing evidence that an excess of free radicals ( oxidative stress ) and an associated lack of free radical scavengers ( antioxidants ) such as vitamin C play an important role in the development of sudden hearing loss. Because these factors have an impact on blood circulation and inflammation. A Korean working group (Kang et al. 2013) examined the effects of high-dose vitamin C infusion therapy in a prospective, randomized study between 08/2010 and 08/2011 in 72 patients with acute hearing loss (acute idiopathic sensorineural hearing loss). 36 of the 72 patients received a daily vitamin C infusion (200 mg vitamin C per kg body weight) for 10 days in addition to the 14-day glucocorticoid therapy and then 2 g oral vitamin C daily for a further 30 days. The control group of 36 patients received only glucocorticoids . Kidney disease, kidney stones , diabetes, vestibular schwannoma and heart failure were among the exclusion criteria in this study. Before the start of therapy and after about 4 weeks, the course was checked using tone audiometry. At the start of therapy there were no significant group differences in terms of demographic and clinical values. Patients in the vitamin C group benefited from a significantly better hearing sensitivity after 4 weeks. The limit of audibility in tone audiometry decreased in the vitamin C group from an initial 67.6 ± 19.8 dB to 37.1 ± 28.8 dB, whereas the improvement in the control group from 70.3 ± 12.4 to 47.6 ± 25.2 dB was significantly weaker (p = 0.030). In the vitamin C group compared to the control group, significantly more patients showed complete or partial recovery (65.5% versus 42%, p = 0.035). The number of patients with a full recovery was more than twice as high in the vitamin C group (46.8 versus 23.8%). According to the authors, vitamin C therapy reduces the oxidative stress in the inner ear induced by ischemia and inflammation . This study is a first indication that vitamin C, especially in patients with a vitamin C deficiency, could have a beneficial effect on the healing process after sudden hearing loss. Follow-up studies that further investigate these effects are certainly necessary, so that no recommendations regarding therapy can be made at the moment.

Alternative medical therapy options

There are a number of other therapy options. The fact that the causes of a sudden hearing loss are not clear also provides dubious sellers with a space to sell their products or services. Doubts are particularly appropriate when a provider claims that only his therapy is effective, when great successes are reported without reference to the high self-healing rate, when it is claimed that one now knows the causes of sudden hearing loss and when the therapy offered is also known for tinnitus and disease Menière should be suitable.

Fund eligibility for acute hearing loss therapies

Since 2009, within the framework of the new drug directive (AM-RL, Annex III No. 24), acute hearing loss treatment with the active ingredient pentoxifylline can no longer be charged to statutory health insurance companies in Germany. The reason for this is the unproven effect of the drug. Although hydroxyethyl starch and glucocorticoids, in contrast, are not expressly mentioned in the new guideline, a doctor must reckon with claims for recourse from the statutory health insurances due to their likewise unproven effectiveness if he prescribes these drugs at their expense. In addition, there is usually no approval / indication for acute hearing loss therapy ( off-label use ). If a person with statutory health insurance nevertheless wishes to have acute hearing loss therapy with pentoxifylline, HAES or glucocorticoids, this therapy usually has to be billed privately.

Web links

Wiktionary: sudden hearing loss  - explanations of meanings, word origins, synonyms, translations

Individual evidence

  1. H91.2: idiopathic sudden hearing loss ( memento of the original dated June 21, 2015 in the Internet Archive ) Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. @1@ 2Template: Webachiv / IABot / www.dimdi.de
  2. ^ English: sudden idiopathic hearing loss (SIHL) or idiopathic sudden sensorineural hearing loss (ISSHL or ISSNHL) H91.2
  3. Ear infarction
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