[Otitis externa: etiology, diagnostic and therapy]. / Otitis externa: Atiologie, Diagnostik und Therapie.

Otitis externa (OE) occurs during the lifetime in approximately 10% of the population, especially in warm and damp climates or from swimming (swimmer's ear). Females are most often affected around the age of 50 years and males around 70 years of age. Both auditory canals are affected in approximately 10% of cases. Causes of infection are mostly bacteria, and more rarely fungi or viruses. OE can be accompanied not only by relatively slight pain and light swelling of the skin of the auditory canal, but also by severe pain, complete obstruction of the external meatus and retroauricular swelling. An uncomplicated infection can normally be treated by cleaning of the meatus by an ENT specialist and local application of a broad-spectrum antibiotic or an antiseptic supplemented with corticoids as well as antimycotics. Complicated infections, such as necrotizing OE, are rare and normally only occur in elderly patients with diabetes mellitus or in an immunosuppressed condition.

Effect of age and sex on maturation of sensory systems and balance control.

Maintenance of postural balance requires an active sensorimotor control system. Current data are limited and sometimes conflicting regarding the influence of the proprioceptive, visual, and vestibular afferent systems on posture control in children. This study investigated the development of sensory organization according to each sensory component in relation to age and sex. A total of 140 children (70 males, 70 females; mean age 10y [SD 4y]; age range 3y 5mo-16y 2mo) and 20 adults (10 males, 10 females; mean age 30y 6mo [SD 8y 4mo]; age range 17y 2mo-49y 1mo) were examined using the Sensory Organization Test. Participants were tested in three visual conditions (eyes open, blindfolded, and sway-referenced visual enclosure) while standing on either a fixed or a sway-referenced force platform. Mean equilibrium scores for the six balance conditions showed rapid increases and maturation ceiling levels for age-related development of the sensorimotor control system. Proprioceptive function seemed to mature at 3 to 4 years of age. Visual and vestibular afferent systems reached adult level at 15 to 16 years of age, revealing differences between young males and females. Characterizing balance impairments can contribute to the diagnostic evaluation of neuromotor disorders.

[Standing stability in children- and young adults. Influence of proprioceptive, visual and vestibular systems in age- and sex dependent changes]. / Standstabilität im Kindes- und Jugendalter. Einfluss des propriozeptiven, visuellen und vestibulären Systems auf alters- und geschlechtsabhängige Veränderungen.

BACKGROUND: The maintenance of balance while standing is ensured by means of a sensory motor control system. As the development of sensory organization on posture control in children is not clear at this time, the aim of this study was to analyse the significance of each sensory component in relation to age and sex. SUBJECTS AND METHOD: A total of 128 children between the ages of 3 and 16 years and 23 adults were examined using the Sensory Organization Test (EquiTest). RESULTS: Age-related changes during the test procedure showed a developmental tendency of the sensory motor process. The influence of the proprioceptive function on stance stability seemed to be completely developed between 3-4 years. The visual and vestibular afferences reached the adult level between 15-16 years with differences between girls and boys. CONCLUSION: Age-related evaluation of the postural mechanisms can be used for differentiation between physiological and pathological results in balance control as well as for monitoring therapy progress in the context of, for example, hyperactivity or learning disability.

Localization of efferent neurotransmitters in the inner ear of the homozygous Bronx waltzer mutant mouse.

Naturally occurring mutant mice provide an excellent model for the study of genetic malformations of the inner ear. Mice homozygous for the Bronx waltzer (bv/bv) mutation are severely hearing impaired or deaf and exhibit a 'waltzing' gait. Functional aspects of cochlear and vestibular efferents in the bv/bv mutant mouse are not well known. The present study was designed to evaluate several candidates of efferent neurotransmitters or neuromodulators including choline acetyltransferase (ChAT), gamma-aminobutyric acid (GABA), and calcitonin gene-related peptide (CGRP) in the inner ear of the bv/bv mutant mouse. Ultrastructural investigations at both light and electron microscopic level were performed. Ultrastructural morphologic evaluations of the cochlea and the vestibular end-organs were also undertaken. It is demonstrated that ChAT, GABA and CGRP immunoreactivities are present in the cochlea and in vestibular end-organs of bv/bv mutant mice. In the organ of Corti, immunoreactivity of ChAT, GABA and CGRP is confined to the inner spiral fibers, tunnel-crossing fibers, and the vesiculated nerve endings synapsing with outer hair cells. Interestingly, immunoreactivity was detectable even where inner hair cells appeared missing. Results also revealed malformations of the outer hair cells with synaptic contacts to efferent nerve endings consistently intact. In the neurosensory epithelia of the vestibular end-organs, the presence of ChAT, GABA, and CGRP immunoreactivity was localized at the vestibular efferents, with the exception of the macula of saccule. In one 8-month-old macula of utricle where the depletion of hair cells appeared highest, ChAT immunostaining was still discernible. Ultrastructural investigation demonstrated that vesiculated efferent nerve endings make synaptic contact with the outer hair cells in the organ of Corti and with type II hair cells in the vestibular end-organs. The present study provides further support that the efferent system in the bv/bv mutant inner ear is morphologically as well as functionally mature. These findings also demonstrate that if and when the onset of efferent degeneration in the bv/bv mutant inner ear occurs, it transpires subsequent to pathological conditions in the hair cells. The present findings give further indication that the efferent systems of the bv/bv mutant inner ear are independent of the afferent systems in many aspects including development, maturation as well as degeneration.

Connexin 26 in human fetal development of the inner ear.

Specialized for intercellular communication, gap junctions have been theorized to provide a means (the epithelial and connective tissue gap junction systems) by which fluid and ions might be transported for maintenance of high levels of endolymphatic K+ [Kikuchi et al., 1994. Acta Otolaryngol. 114, 520-528] in the inner ear. A primary constituent of these gap junctions is connexin 26 (Cx26), a protein encoded by the gene GJB2 and found in both epithelial and connective tissue cells. It has been shown that a mutation in Cx26 accounts for 50% of patients with autosomal recessive nonsyndromic hearing loss. In the present study, we document the emergence and distribution features of Cx26 through various stages (weeks 11-31) of gestation in human, fetal cochleae. Comparative patterns of Cx26 distribution are also presented in the mature rat. The cochleae were fixed in 4% paraformaldehyde within 2 h post mortem. Immunohistochemical studies were performed using a rabbit polyclonal antibody raised against synthetic peptide and corresponding with amino acids 108-122. Specimens were mounted into paraffin sections. Results show that Cx26-like immunoreactivity is evident at a prenatal age of 11 weeks and maintains a high intensity of reactivity through 31 weeks of gestation. The appearance of this reactivity seemed to modulate in parallel with the onset of development and histological maturation as well as provide functional maintenance. In the human fetal cochlea, Cx26-like immunoreactivity distribution resembled adult patterns by fetal week 20. At the completion of morphological development by week 31, reactivity appeared to achieve an adult profile of distribution. Descriptions and discussion of Cx26 distribution patterns are presented in detail.

Selective aspects of human pathology in high-tone hearing loss of the aging inner ear.

Accompanied with aging, the thresholds for high frequency sounds may elevate and result in a progressive hearing loss described as presbycusis. Based on correlations between audiometric measures of aged patients and histologic findings garnered from postmortem examinations, four types of presbycusis have been characterized: sensory-neural, neural, strial, and conductive [Schuknecht, H.F., Gacek, M.R., 1993. Ann. Otol. Rhinol. Laryngol. 102, 1--16]. Otopathologic changes to the inner ear as a direct function of age, however, remain controversial. The focus of this investigation involves the pathological impact on remaining sensory structures in patients having sensory--neural degeneration. The current study presents seven human temporal bones extracted from patients aged 53--67 years with high-tone hearing loss and with no known history of extraordinary environmental events involving head or noise trauma, acoustic overstimulation, or ototoxicity. In previously published findings of these specimens, all but one temporal bone failed to demonstrate a meaningful correlation between audiometric measurements and loss of functional hair cell populations with secondary retrograde degeneration of nerve fibers. Using the block surface method, electron microscopic micrographs demonstrate ultrastructural changes in the cuticular plate, stereocilia, pillar cells, stria vascularis, and the spiral ligament. In all pathological specimens, the greatest incidence of degeneration was seen at the cuticular plate. Conclusively, our findings present three implications in the aging human cochlea: firstly, audiometric measures that represent a high-tone hearing loss may take various forms with respect to ultrastructural patterns of degeneration and surviving structures; secondly, the incidence of lipofuscin and lysosome granules does not correlate with the degree of hearing loss and; thirdly, as shown only in guinea pigs [Anniko, M., 1988. Scanning Microsc. 2, 1035--1041], high-tone hearing loss can be associated with deformation of the cuticular plate.

Goldenhar's syndrome: congenital hearing deficit of conductive or sensorineural origin? Temporal bone histopathologic study.

BACKGROUND: Oculoauriculovertebral dysplasia (OAVD) (Goldenhar's syndrome) is a congenital syndrome with ipsilateral deformity of the ear and face, epibulbar lipodermoids, coloboma, and vertebral anomalies. Goldenhar's anomaly has often been associated with a degree of congenital hearing deficits, almost always of a conductive origin, but a sensorineural component is also suspected in some cases, evident through malformations of the inner ear. PATIENTS AND METHODS: Both temporal bones of a 10-day-old deceased patient with oculoauriculovertebral dysplasia were examined by light microscopy. RESULTS: The ear deformities included deformity of the auricle, atresia of the external auditory canal, and malformation of the tympanic cavity and ossicles. Abnormalities of the stria vascularis and the semicircular canals were also demonstrated. Further inner ear deformities were not identified in this case. CONCLUSION: These histopathologic findings appear to confirm the conductive component of the congenital hearing deficit, but a sensorineural component could not be omitted. The ear alterations favor early developmental field defects. The causes of this condition are controversial. Recent results in genetic research pertaining to the MSX class genes permit better understanding of the variety, variability, and different degrees of severity of the anomalies described here.

Immunohistochemical and morphological studies on the human fetal cochlea: a comparative view on methods.

The preservation of morphology and antigenicity can vary uncontrollably with human fetuses since these rely heavily on immediate fixation of the temporal bone following spontaneous abortion. Once good fixation is established, there is the question of the approach taken for morphologic and immunohistochemical studies. To achieve maximal preservation for the purpose of studying normal and pathologic fetal cochleae, commonly used preparation methods for analyzing the cochlea were reviewed and compared for both immunohistochemical and morphologic studies. Cochleae obtained after spontaneous abortion ranged from the 9th gestational week to birth. Four different methods were compared for morphologic study: the block surface method; a microslicing technique; paraffin; and celloidin sectioning. For immunohistochemical study, three methods were compared: pre-embedding; paraffin; and frozen sectioning. For morphologic preservation, the block surface method gave best overall results, showing good representation of the fetal cochlea for surface preparation, light, and electron microscopy. Celloidin sectioning was also found to show good light microscopic results for both the middle and inner ear. To achieve optimal results, preservation quality, fixation procedures, and antibody all contribute to the efficacy of a methods choice.

Juvenile nasopharyngeal angiofibroma: management and therapy.

OBJECTIVE: To conduct a review of contemporary approaches on the diagnostic-preoperative, operative, and postoperative methods in the management of juvenile nasopharyngeal angiofibroma (JNA). STUDY DESIGN: Retrospective study of 14 cases of JNA resection at the Department of Otorhinolaryngology, University of Innsbruck (Innsbruck, Austria) between 1987 and 1998. METHODS: Data was obtained for each patient regarding age, presenting symptoms, duration of symptoms, biopsy findings, tumor location, administration of preoperative angiography and embolization, and surgical approach. The follow-up period ranged from 1 to 13 years. RESULTS: Based on the histological evaluation by the preoperative biopsy and the tumor location, several surgical approaches were applied. A transnasal endoscopic procedure was employed in seven cases. The preoperative embolization and the intranasal approach with the potassium titanyl phosphate laser minimized blood loss. The recurrence rate was at a low of 15%. CONCLUSION: The surgical approach should be determined by tumor location, tumor size, and effectiveness of tumor embolization. For patients with JNA with tumor extension involving the nasopharynx, the nasal cavity, the paranasal sinuses, and the pterygopalatine fossa, the transnasal endoscopic technique offers a minimally invasive resection of the entire tumor mass with minimal morphological disturbance.

Cerebro-oculo-facio-skeletal syndrome as a human example for accelerated cochlear nerve degeneration.

BACKGROUND: Cerebro-oculo-facio-skeletal (COFS) syndrome is a rare autosomal-recessive disorder that includes microcephaly, severe mental retardation, and multiple congenital anomalies. Otologic findings are usually limited to descriptions of the auricles. PATIENT AND METHODS: The authors report inner ear histopathologic findings of a deceased 13-year-old patient with COFS. A histologic study of the inner ear in COFS syndrome has not yet been described. This patient was documented as having a profound bilateral sensorineural hearing loss at the age of 2 years. RESULTS: Histologic evaluation revealed accelerated neural and neuronal degeneration at the cochlear and retrocochlear levels. Remaining myelinated nerve fibers, counted in the spiral lamina, had degenerated by up to 97% when compared with normal innervation densities. Afferent nerve fibers innervating inner hair cells were completely absent, whereas medial efferent fibers to outer hair cells were found. Vestibular nerve fibers were less affected. CONCLUSION: The authors report inner ear findings that differ from animal models of primary cochlear neural degeneration and that resemble the pattern of hereditary cochlear nerve degeneration reported in Friedreich's ataxia.

[Effects of standardized optokinetic stimuli on standing stability]. / Einfluss standardisierter optokinetischer Reize auf die Standstabilität.

BACKGROUND: Human standing stability is ensured by means of a sensory-motor control system. Proprioceptivity is most important among sensory afferences. At this time, less is known about the effect of standardized visual stimuli on motor programs towards support of body stability. These investigations were performed with special consideration for different strains on bunions and heels of both feet. METHODS: 42 healthy individuals (averaged age 29.6 years) were investigated with relaxed standing on the static force platform of Portable Multiplate System P. M. S. with optokinetic stimuli in horizontal, vertical and torsional direction (speed 80 degrees/s). Conducted by computer-analysis, the stability index, varying foot-pressure on heels and bunions, Fourier spectral analysis, and weight distribution index were counted. The eye movements were controlled with help of the PENG device. RESULTS: The optokinetic stimulations led to partial statistically significant impairments of stability particularly with torsional stimuli, yet notably improved stability with left stripes pattern. The sensory conflict between visual and other sensory inputs led to a statistically significant shift of sway frequencies to the higher ranges. On stimulation, the distribution of pressure to the feet changed. The preponderance to rest on the left side was equalized. The heels were considerably strained more with visual stimuli in all directions. Individuals felt more agreeable, toward stripes vertically directed while torsionally directed stripes elicited particular strain. CONCLUSION: Sensory conflicts between visual and other sensory inputs for maintaining the upright body position were found to impair the stability. Nevertheless these conflicts were partially compensated by motor programs more straining to the heels.

Head and neck tumors: fractionated frameless stereotactic interstitial brachytherapy-initial experience.

The authors used a frameless stereotactic navigation system, the Vogele-Bale-Hohner head holder, and a targeting device to reproducibly position brachytherapy needles for fractionated interstitial brachytherapy in 12 patients with inoperable cancers of the head and neck. In all cases, deviations of the needle relative to the planned position were within 1-15 mm depending on the location of the tumor.

Immunohistochemical investigation of enkephalins in the human inner ear.

Enkephalins are generally considered as neuropeptides in the central and peripheral nervous system of mammals bound to three large precursor molecules. Several animal studies demonstrated the distribution of met- and leu-enkephalin-like immunoreactivities in neurons and terminals of the lateral olivocochlear system. The immunostainings in the medial system are more controversial. No data about the presence of different enkephalin sequences in the vestibular efferent terminals are known. In the present study, the ultrastructural localization and distribution of immunoreactivities for six different antibodies against met- and leu-enkephalins in the human cochlear and vestibular periphery were investigated. A modified method of pre-embedding immunoelectronmicroscopy was applied. Met- and leu-enkephalin-like immunoreactivities were observed in the efferent terminals of the human outer and inner hair cell region. Using different met- and leu-enkephalin antibodies, the distribution of immunoreactivities remained similar. In the five human vestibular endorgans, enkephalin-like immunostaining was absent.