Joachim Schmutzhard

Health-related quality of life outcome of adult patients after otoplasty.

Prominent ears are relatively frequent. Decreased self-esteem, increased anxiety, behavioral problems and social avoidance may result from disfigurement. In modern medicine it is becoming increasingly important to measure the benefit of surgical intervention by its impact on the patients Quality of Life (QOL). Our study was performed in a retrospective manner at our institution including 40 adult patients with prominent ears. The Glasgow Benefit Inventory (GBI), a reproducible, valid and responsive questionnaire, was the basis of the used inquiry for detecting the changes in HRQOL after otoplasty. We showed an improvement in GBI subscores after intervention. There was no difference in GBI subscores between men and women. The follow-up time as well as critical life event and chronic concomitant disease have no influence on the GBI results. Otoplasty has a positive impact on the HRQOL of adult patients with prominent ears. The importance of this benefit is not only of individual nature. In times of healthcare economization, an amelioration of QOL followed by increased productivity at work of the individual is an important argument to justify health care expenditures. We believe that otoplasty is an appropriate therapy for selected adult patients burdened by prominent ears.

Histology and synchrotron radiation-based microtomography of the inner ear in a molecularly confirmed case of CHARGE syndrome

CHARGE (Coloboma of the iris or retina, heart defects, atresia of the choanae, retardation of growth and/or development, genital anomalies, ear anomalies) syndrome (OMIM #214800) affects about 1 in 10,000 children and is most often caused by chromodomain helicase DNA‐binding protein‐7 (CHD7) mutations. Inner ear defects and vestibular abnormalities are particularly common. Specifically, semicircular canal (SCC) hypoplasia/aplasia and the presence of a Mondini malformation can be considered pathognomonic in the context of congenital malformations of the CHARGE syndrome. We obtained a temporal bone (TB) of a patient with CHARGE syndrome who died from bacteremia at 3 months of age. The clinical diagnosis was confirmed in the patient by direct DNA sequencing and the detection of a de novo, truncating CHD7 mutation, c.6169dup (p.R2057fs). We assessed changes of the TB and the degree of neural preservation, which may influence the potential benefit of cochlear implantation. The TB was analyzed using synchrotron radiation‐based micro computed tomography, and by light microscopy. The vestibular partition consisted of a rudimentary vestibule with agenesis of the SCCs. The cochlea was hypoplastic with poor or deficient interscaling and shortened (Mondini dysplasia). The organ of Corti had near normal structure and innervation. Modiolus and Rosenthals canal were hypoplastic with perikarya displaced along the axon bundles into the internal acoustic meatus, which may be explained by the arrest or limited migration and translocation of the cell nuclei into the cochlear tube during development.

Pilot study: Volatile organic compounds as a diagnostic marker for head and neck tumors

In the last decade, the analysis of volatile organic compounds (VOC) has undergone a rapid development. In this pilot study, patients with HNSCC were tested with a proton transfer reaction‐mass spectrometry in order to establish a minimal invasive screening method.

Resection of accessory parotid gland tumors through a peroral approach with facial nerve monitoring.

Accessory parotid glands are an anatomic variation. Pathologic alterations, which occur in these tissues, are related to those found in the parotid gland. At this time, first-line therapy consists of total resection. In consideration of the delicate anatomy in this region, a careful approach through a lateral parotidectomy or a facelift incision is recommended. In this report, we give an account of a minimally invasive surgical alternative through a peroral approach with facial nerve monitoring. The histologically secured pleomorphic adenoma was completely removed. During surgery, a branch of the facial nerve was detected and secured with active nerve monitoring. We are confident that the peroral resection, supported by active and passive facial nerve monitoring, is a discussable alternative for well-selected tumors of accessory parotid glands.

Murine malaria is associated with significant hearing impairment

BackgroundPlasmodium falciparum malaria has been suspected to cause hearing loss. Developmental, cognitive and language disorders have been observed in children, surviving cerebral malaria. This prospective study aims to evaluate whether malaria influences hearing in mice.MethodsTwenty mice were included in a standardized murine cerebral malaria model. Auditory evoked brainstem responses were assessed before infection and at the peak of the illness.ResultsA significant hearing impairment could be demonstrated in mice with malaria, especially the cerebral form. The control group did not show any alterations. No therapy was used.ConclusionThis suggests that malaria itself leads to a hearing impairment in mice.

Morphology studies of the human fetal cochlea in turner syndrome.

Objectives: Turner syndrome (TS) is the most frequent sex chromosome abnormality, and sensorineural hearing loss is common. We aimed to determine whether there are consistent morphologic cochlear abnormalities during gestational development that could be associated with TS. Design: The histology of nine fetal temporal bones of TS autopsied after spontaneous abortion was studied. Results: Gross morphologic examination of the TS cochleae failed to reveal a pattern of structural abnormalities that would explain the development of sensorineural hearing loss. Mondini-like cochlear dysplasia was observed in one 13-wk-old TS fetus. Conclusion: We could not demonstrate a consistent pattern of cochlear malformations.

Apoptosis of the fibrocytes type 1 in the spiral ligament and blood labyrinth barrier disturbance cause hearing impairment in murine cerebral malaria

BackgroundExperimental murine malaria has been shown to result in significant hearing impairment. Microscopic evaluation of the temporal bones of these animals has revealed regular morphology of the cochlea duct. Furthermore, the known vascular pathologic changes being associated with malaria could not be found. Immunohistochemistry for ICAM1 showed a strong marking in the stria vascularis, indicating a disturbance of the endocochlear potential. The aim of this study was to evaluate the role of apoptosis and the disturbance of the blood labyrinth barrier in the murine malaria associated hearing impairment.MethodsThe temporal bones of seven mice with cerebral malaria-four with hearing impairment, three without hearing impairment-were evaluated with immunohistochemistry for cleaved caspase 3 to detect apoptosis and connexin 26, a gap junction protein being a cornerstone in the endocochlear potassium recirculation. Furthermore five animals with cerebral malaria were treated with Evans blue prior to sacrification to detect disturbances of the blood labyrinth barrier.ResultsCleaved caspase 3 could clearly be detected by immunohistochemistry in the fibrocytes of the spiral ligament, more intensively in animals with hearing impairment, less intensively in those without. Apoptosis signal was equally distributed in the spiral ligament as was the connexin 26 gap junction protein. The Evans blue testing revealed a strong signal in the malaria animals and no signal in the healthy control animals.ConclusionMalfunction of the fibrocytes type 1 in the spiral ligament and disruption of the blood labyrinth barrier, resulting in a breakdown of the endocochlear potential, are major causes for hearing impairment in murine cerebral malaria.

Does perinatal asphyxia induce apoptosis in the inner ear

Pre- and perinatal asphyxia is known to be an important risk factor in the development of neonatal hearing impairment. This study aims to evaluate the role of apoptosis, which is known to play an essential role in the development of the inner ear structures, in the development of neonatal hearing loss caused by pre- and perinatal asphyxia. Eight temporal bones of six different newborns were included. We performed a morphologic analysis by both light microscopy, and transmission electron microscopy, as well as immunohistochemical staining to detect the cleaved form of caspase 3 as apoptosis marker and Bcl 2 as anti-apoptotic marker. Early and late phases of apoptosis were evidenced by condensation of chromatin (electron-dense, black structure along nuclear membrane) and fragmentation of the nucleus, respectively. Changes in nuclear morphology during apoptosis correlate with cleavage by caspase 3 located downstream of Bcl 2 action. The immunohistochemistry for cleaved caspase 3 showed a particular predilection for the inner and outer hair cells, spiral ganglion cells and the marginal cells of the stria vascularis. The brain of all examined cases did not show signs of apoptosis. In summary, this investigation suggests that apoptosis takes place before brain tissue apoptosis and is probably an earlier event than thought. Apoptosis of the cochlea is known to play an essential role in the development of the inner ear. Additionally, this study shows that apoptosis may play an important role in the development of hearing impairment, caused by pre- and perinatal asphyxia.

Murine cerebral malaria: histopathology and ICAM 1 immunohistochemistry of the inner ear.

Objective  To evaluate the pathophysiologic changes in the inner ear during the course of severe cerebral malaria in an established animal model, C57 BL/6J mice.

Severe malaria in children leads to a significant impairment of transitory otoacoustic emissions - a prospective multicenter cohort study

BackgroundSevere malaria may influence inner ear function, although this possibility has not been examined prospectively. In a retrospective analysis, hearing impairment was found in 9 of 23 patients with cerebral malaria. An objective method to quickly evaluate the function of the inner ear are the otoacoustic emissions. Negative transient otoacoustic emissions are associated with a threshold shift of 20 dB and above.MethodsThis prospective multicenter study analyses otoacoustic emissions in patients with severe malaria up to the age of 10 years. In three study sites (Ghana, Gabon, Kenya) 144 patients with severe malaria and 108 control children were included. All malaria patients were treated with parental artesunate.ResultsIn the control group, 92.6 % (n = 108, 95 % confidence interval 86.19–6.2 %) passed otoacoustic emission screening. In malaria patients, 58.5 % (n = 94, malaria vs controls p < 0.001, 95 % confidence interval 48.4–67.9 %) passed otoacoustic emission screening at the baseline measurement. The value increased to 65.2 % (n = 66, p < 0.001, 95 % confidence interval 53.1–75.5 %) at follow up 14–28 days after diagnosis of malaria.The study population was divided into severe non-cerebral malaria and severe malaria with neurological symptoms (cerebral malaria). Whereas otoacoustic emissions in severe malaria improved to a passing percentage of 72.9 % (n = 48, 95 % confidence interval 59–83.4 %) at follow-up, the patients with cerebral malaria showed a drop in the passing percentage to 33 % (n = 18) 3–7 days after diagnosis. This shows a significant impairment in the cerebral malaria group (p = 0.012 at days 3–7, 95 % confidence interval 16.3–56.3 %; p = 0.031 at day 14–28, 95 % confidence interval 24.5–66.3 %).ConclusionThe presented data show that 40 % of children have involvement of the inner ear early in severe malaria. In children, audiological screening after severe malaria infection is not currently recommended, but is worth investigating in larger studies.