Schwerhörigkeit

Background

In recent years some studies could show that besides the negative effects of stressful encounters resulting from disorders and handicaps, positive experiences can also be made. The aim of this study was to determine which strategies can help persons with hearing impairments to gain positive experiences.

Materials and methods

A questionnaire in the German language consisting of the Trier Personality Inventory and a German translation of a questionnaire designed to measure the positive experiences in life with acquired hearing impairment was distributed to persons with acquired hearing impairment, who were recruited from hearing aid audiologists and ear nose and throat physicians.

Results

Persons with hearing impairments showed no differences to the general population with respect to their personality profile. Positive experiences are significantly more common by successful communication behavior than by other strategies.

Consequences

Successful communication helps persons with hearing impairments to gain positive experiences.     

Kongenitale Schwerhörigkeit

BACKGROUND: About 50% of congenital non-syndromic hearing impairment is caused by genetic factors. Research on the genetics of deafness has revealed a vast number of relevant genes. Mutations in the GJB2 gene have been shown to be the most common in several populations. METHODS: Mutation analysis of the genes for connexin 26, 30 and 31 (GJB2, GJB6 and GJB3) was performed in 67 patients with profound hearing loss. RESULTS: Of the participants, 9% had two pathogenic mutations in the GJB2 gene. Pedigree information indicates that in these families further offspring have a 25% to a 100% chance of having hearing impairment. CONCLUSIONS: Patients with non-syndromic hearing impairment should be offered molecular diagnostics of the GJB2 gene. Genetic counseling is mandatory for mutation carriers in order to advise them on the individual consequences of the gene test results.     

Schwerhörigkeit und Liquorpunktion

Ohne Zusammenfassung     

Innenohrschwerhörigkeit beim LEOPARD-Syndrom

The rare multiple lentigines (LEOPARD) syndrome represents a complex of skin, cardiac, skeletal, inner ear and other malformations. There is marked variability in expression of the syndrome. We report on a 20 year old man, showing typical lentiginosis, a retardation of growth, tachycardiac conduction abnormality, ophthalmologic manifestations and a sensorineural hearing loss. Pathogenesis, clinical and differential diagnostic aspects are discussed in this case report. The early diagnosis of a senosorineural hearing loss is useful in order to provide appropriate rehabilitation. When lentiginosis is diagnosed, it is important to consider further abnormalities such as cardiomyopathy, which can be associated with a high mortality.     

Rehabilitation der Hochtoninnenohrschwerhörigkeit

Background

In spite of modern technology conventional hearing aids are only helpful in a limited number of patients with sensorineural hearing loss. In particular, patients presenting with moderate to severe high frequency hearing loss but only mild hearing loss in the low frequencies suffer from problems associated with conventional hearing aids such as occlusion of the ear canal and feedback.The aim of the study was the evaluation of rehabilitation of patients with high frequency hearing loss with the active middle ear implant Vibrant Soundbridge®.

Patients and methods

The Vibrant Soundbridge® was surgically implanted into 30 patients, and the floating mass transducer was clipped onto the long process of the incus. Out of the 30 patients 9 presented with a ski slope audiogram of high frequency hearing loss of 25 dB within 1 octave in the frequency range 1000–8000 Hz. Main outcome measures were pure tone audiometry, speech audiometry in quiet and in noise.

Results

Residual hearing was preserved in all cases. Functional hearing gain was in proportion to the individual hearing losses and was remarkably high in the high frequencies up to 8000 Hz. Mean functional gain was 34 dB in the frequencies between 2000–8000 Hz. Speech recognition scores in quiet and in noise were significantly higher with the implant compared to the unaided situation.

Conclusion

The middle ear implant Vibrant Soundbridge® has been shown to be perform extremely well especially in the high frequencies. It offers a new solution for rehabilitation of high frequency hearing loss.     

Erythroblastose und Schwerhörigkeit

Ohne ZusammenfassungMit 9 Textabbildungen     

Klippel-Feilsches Syndrom und Schwerhörigkeit

Zusammenfassung Es wird über einen Fall vonKlippel-Feilschen Syndrom berichtet, bei dem außerdem eine hochgradige Schallempfindungsstörung beider Ohren und eine Recurrensschädigung links vorlag. Die Ätiologie der Schwerhörigkeit und der Recurrensschädigung wird zu erklären versucht.Mit 10 Textabbildungen.     

Hereditäre Schwerhörigkeit

Syndromic hearing loss is responsible for approximately 30?% of cases of inherited hearing loss. The syndromic form can be differentiated from nonsyndromic hearing loss by the presence of associated symptoms in other organ systems. While for many forms of syndromic hearing loss the individual genes responsible have been identified, the etiology of other associated symptoms remains unclear. The role of the ENT physician is to select appropriate clinical and genetic diagnostic tools based on the presentation of the patient and to subsequently initiate and perform the required hearing loss therapy.     

Pathologische Anatomie der tympanogenen Schwerhörigkeit

Ohne Zusammenfassung     

Frequentztransposition zur Hörverbesserung bei Innenohrschwerhörigkeit

Ohne ZusammenfassungMit 6 TextabbildungenIn der Habilitationsschrift des Verfassers sind die Ergebnisse der Sprachverständlichkeitsänderungen bei Frequenzbeschneidung im Detail geschildert worden.     

Einfluss exogener Faktoren auf Altersschwerhörigkeit

Background

Age-related hearing impairment is a complex disorder, the causes for which have been insufficiently studied. Genetic and environmental factors all play a role.

Methods

A total of 406 persons aged between 53 and 67 years old were interviewed about various causes and audiometric data were collected. The audiometric pure tone data were adjusted for sex and age and tested for association with exposure to risk factors.

Results

Significant negative effects of noise exposure, painkillers, overweight, and cardiovascular diseases on hearing loss were found. A positive effect of moderate alcohol consumption could also be shown in the elderly. These results suggest that a healthy lifestyle can positively affect age-related hearing impairment.     

Propionazidämie und Schallempfindungsschwerhörigkeit

CURRENT KNOWLEDGE: Propionic acidemia is caused by a gene defect leading to malfunction of the enzyme propionyl-CoA carboxylase (PCC) and in turn to a pathologic accumulation of propionic acid. Many mutations have been found at the molecular genetic level over the past 20 years, and their implications for the limitation of enzyme activity of PCC in propionic acidemia are discussed. SCIENTIFIC QUESTION AND AIMS OF THE STUDY: As an elevated incidence of deafness has been observed in patients with propionic acidemia, the question arises of whether mutations primarily responsible for this disease could also be the underlying cause for a genetic form of deafness. METHODS AND RESULTS: As well as a standard pure tone audiogram, a pedigree was elaborated and DNA isolated for each family concerned. In one family several subjects displayed mutations of both the PCCA and the PCCB -subunits; these included only one girl whose phenotype was affected, however. CONCLUSIONS: Mutation of the PCCB subunit p.R113X has not previously been mentioned in the literature. According to our present knowledge no connection can be assumed between either of the two mutations and the severe sensorineural hearing loss.     

Wirksamkeit von totalimplantierten Hörendoprothesen bei Innenohrschwerhörigkeit

Am 28./29. Januar 2000 fand an der Universit?t Tübingen ein gut besuchtes Symposium statt über elektronische H?rimplantate bei Innenohrschwerh?rigkeit, die für die klinische Versorgung zugelassen sind. V?llig neu war die Vorstellung der Ergebnisse einer prospektiven Fallkontrollstudie zur Wirksamkeit und Sicherheit eines vollst?ndig implantierbaren H?rger?tes.