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Biomedical subjects

H-P Zenner

Publications and source records attributed to H-P Zenner.

12 recordsLinked to original sources

Olivocochlear reflex effect on human distortion product otoacoustic emissions is largest at frequencies with distinct fine structure dips.

Activity of the medial olivocochlear efferents can be inferred by measuring the change of the level of distortion product otoacoustic emissions (DPOAE) during ipsilateral or contralateral acoustic stimulation, the so-called medial olivocochlear reflex (MOCR). A limitation of this measurement strategy, however, is the distinct variability of MOCR values depending on DPOAE primary tone levels and frequency, which makes selection of the stimulus parameters difficult. The objective of this study was to evaluate the dependence of MOCR values on DPOAE fine structure in humans. MOCR during contralateral acoustic stimulation was measured at frequencies with distinct non-monotonicity ("dip") in the DPOAE fine structure, and in frequencies with flat fine structure. One hundred and twenty one different primary tone level combinations were used (L(1)=50-60dB SPL, L(2)=35-45dB SPL, 1dB steps). The measurement was repeated on another day. The major findings were: (1) Largest MOCR effects can be found in frequencies which exhibit a distinct dip in DPOAE fine structure. (2) Primary tone levels have a critical influence on the magnitude of the MOCR effect. MOCR changes of up to 23dB following a L(1) change of only 1dB were observed. Averages of the maximum MOCR change per 1dB step were in the 3-5dB-range. Both findings can be interpreted in the light of the DPOAE two-generator model [Heitmann, J., Waldmann, B., Schnitzler, H.U., Plinkert, P.K., Zenner, H.P. 1998. Suppression of distortion product otoacoustic emissions (DPOAE) near 2f1-f2 removes DP-gram fine structure - evidence for a secondary generator. Journal of the Acoustical Society of America 103, 1527-1531]. According to the present results we propose, that assessing MOCR specifically at frequencies with a distinct dip in the DPOAE fine structure, in combination with fine variation of the stimulus tone levels, allows for a more targeted search for maximum MOCR effects. Future studies must show if this approach can contribute to the further clarification of the physiological roles of the olivocochlear efferents.

Acoustic Stimulation↗

[Variable length titanium prostheses for type III tympanoplasty. Intraoperative length adjustment and fixation of the cartilage overlay].

INTRODUCTION: For type III tympanoplasty by partial ossicular replacement prosthesis (PORP) or total ossicular replacement prosthesis (TORP), the length of the prosthesis must match the individual intraoperative anatomical and physiological characteristics. MATERIALS AND METHODS: Databanks were used to determine the necessary sizer length of the sizer disc. The measurement template for the size of the cartilage to overlay the prosthesis headplate was derived from the headplates of the Tübinger titanium prostheses (TTP) and the Dresdener titanium prostheses. Finally all functions were integrated into a synthetic plate. RESULTS: The result was a simple and reasonably priced disposable multifunctional instrument (Tübinger sizer disc TSD) which allowed an exact measurement for every prosthesis in TORP and PORP. For the TTP-Variac, the TSD enabled the simple intraoperative production of prostheses with the length desired by the surgeon. For PORP the TSD enabled an adaptation of the diameter of the prosthesis foot for TTP, TTP-Vario and TTP-Variac and provided a template for the size determination of the cartilage overlay of the titanium prosthesis head. The sizers and the resulting prostheses were used for initial tympanoplastic operations. Audiometric investigations carried out 6 weeks postoperatively gave results corresponding to those previously obtained in a study with TTP and TTP-Vario using the old instrumentation. CONCLUSIONS: The new instrumentation leads to an improvement of the intraoperative practicability and a simplification. The audiological results remain the same.

Disposable Equipment↗

Olivocochlear activity and temporary threshold shift-susceptibility in humans.

STUDY OBJECTIVES: Animal studies (guinea pig, cat, chinchilla) have shown that activity of the medial olivocochlear efferents can exert noise-protective effects on the cochlea. It is not yet known whether such effects are also existent in humans. Olivocochlear activity can be estimated indirectly by contralateral suppression (CS) of otoacoustic emissions (OAE). MATERIAL AND METHODS: We measured Input/Output functions of distortion products of OAE (DPOAE), with and without contralateral acoustic stimulation by white noise, in 94 normal hearing young male subjects. Seven stimuli with L2 between 20 and 60 dB SPL and L1 = 39 dB + 0.4 L2 ("scissor paradigm") were used at f2 = 2, 3, 4, 5, and 6 kHz. The measurement was repeated 2 weeks later. In 83 subjects of the same group, pure tone audiometry was registered before and 6 minutes after shooting exercises to evaluate individual susceptibility to develop a temporary threshold shift (TTS). RESULTS: Test-retest repeatability of CS was generally good. CS averaged 0.98 dB SPL (SD 1.19 dB, median 0.56 dB). As expected, CS was greatest at low stimulus levels (median 1.06 dB at L2 = 20 dB, as compared with 0.33 dB at L2 = 60 dB). The smallest average CS was found at 4 kHz, and the greatest CS appeared at 2 kHz. A TTS occurred in 7 of 83 (8.5%) subjects. Statistical analysis did not reveal any correlation between the amount of CS and individual TTS susceptibility. CONCLUSIONS AND OUTLOOK: 1) Measurement of CS of DPOAE using an extensive measurement paradigm revealed good test-retest repeatability, confirming the reliability of this audiologic tool. 2) CS of DPOAE does not predict individual susceptibility to mild TTS induced by impulse noise in humans. Possible explanations for the missing association are discussed. Future perspectives include longitudinal studies to further elucidate the association between medial olivocochlear bundle-activity and permanent threshold shift in humans. The goal is to develop a diagnostic tool for the prediction of individual noise vulnerability in humans, thereby preventing noise-induced hearing loss.

Acoustic Impedance Tests↗

Refinement of the DFNA4 locus to a 1.44 Mb region in 19q13.33.

Many forms of autosomal dominant non-syndromic hearing impairment are known. While the underlying gene defects and causative mutations have been discovered for some forms, the gene responsible for DFNA4 has remained elusive to date. Examination of a German four-generation kindred led to the identification of a 1.44 Mb map segment in contig NT_011109 as being the most likely DFNA4 candidate region in 19q13.33. The recombination breakpoints in this family and the intervals of two previously reported DFNA4 families allowed us to delineate a minimum consensus region between the markers D19S879 and D19S246. In our family, a maximum two-point LOD score of 4.5 was obtained at theta = 0 for the marker D19S867. Within the refined DFNA4 interval the public databases list more than 50 genes, from which several appear to be promising DFNA4 candidates due to similarities with animal models and with other causative genes involved in hearing disability.

Adult↗

[Phenotypic characterization of a DFNA6 family with low-frequency hearing loss].

BACKGROUND: Hereditary hearing impairment is a heterogeneous sensory defect with approximately two-thirds of all cases being nonsyndromic. Only two loci (DFNA1 and DFNA6/14/38) are associated with low frequency sensorineural nonsyndromic hearing impairment. DFNA6 was mapped to chromosome 4p16. Recessive mutations in the WFS1 gene are responsible for Wolfram syndrome; missense mutations inherited as an autosomal dominant result in low frequency sensorineural hearing impairment (LFSNHI). PATIENTS AND METHODS: In this study we analyzed the phenotype of a large Hungarian family with LFSNHI and linkage to DFNA6. The family contains 14 affected persons. RESULTS AND CONCLUSION: In general, these patients show a postlingual, sensorineural, bilateral, symmetric, nonsyndromic low frequency hearing impairment with a slow progression. This impairment is accompanied by normal vision and normal vestibular responses.

Adolescent↗

[Waardenburg syndrome. A heterogenic disorder with variable penetrance].

BACKGROUND: Waardenburg syndrome (WS) is an autosomal dominant disorder characterised by pigmentary anomalies of the skin, hairs, eyes and various defects of other neural crest derived tissues. It accounts for over 2% of congenital hearing impairment. At least four types are recognized on the basis of clinical and genetic criteria. PATIENTS AND METHODS: Based on a screening of congenitally hearing impaired children, 12 families with WS type II were detected. Of special interest was the phenotype of these families, in particular the reduced penetrance of hearing impairment within the families. RESULTS AND CONCLUSION: In all cases a high variability of the disease phenotype was detected and the penetrance of the clinical traits varied accordingly. Therefore, it is not possible to predict the clinical phenotype even in a single family. Based on these studies, we plan to identify the pathogenetic cause of the disease in order to perform a detailed genotype/phenotype analysis.

Genetic Heterogeneity↗

[Phenotype of patients showing hearing impairment based on the 35delG mutation in the connexin 26 gene].

BACKGROUND: Hereditary hearing impairment constitutes a heterogeneous class of disorders showing different patterns of inheritance and involving multiple genes. Mutations in the GJB2 gene, especially the 35delG mutation, have been established as a major cause of inherited and sporadic nonsyndromic hearing impairment in different populations. METHODS: We analyzed 14 northeast Hungarian families and 69 sporadic cases with nonsyndromic hearing impairment for the 35delG mutation. Sixty-five patients showing a homozygous 35delG mutation were examined regarding their audiologic phenotype. RESULTS: In general, these patients (70%) showed a prelingual, sensorineural, bilateral, symmetric hearing impairment without progression. The audiograms demonstrated sloping as well as flat patterns. CONCLUSIONS: The severity of hearing impairment varied in 30% of all analyzed patients, making genetic counseling difficult.

Acoustic Impedance Tests↗

Disabling disturbance of olfaction in a dental technician following exposure to methyl methacrylate.

It is often difficult to diagnose dysosmia due to occupational olfactotoxic substances. The authors present a case of disabling disturbance of olfaction in a dental technician. This is very likely to have been caused by exposure to methyl methacrylate. From 1988-1992, the dental technician had very extensive and sustained contact with a self-polymerizing acrylic resin based on methyl methacrylate. Her perception of smell was still normal in 1988, but it deteriorated up to 1992. The olfactory disorders have persisted and impede social life and occupational rehabilitation.

Dental Technicians↗