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M Hultcrantz

Publications and source records attributed to M Hultcrantz.

At least 37 records · Page 2Linked to original sources

Otological problems in children with Turner's syndrome.

Ear and hearing disorders are common problems among girls and women with Turner's syndrome. During infancy and childhood the girls often suffer from repeated attacks of acute otitis media and later in life the women frequently complain of a rapid onset of social hearing problems due to sensorineural hearing impairment. A study of 56 girls aged 4-15 years with Turner's syndrome was performed to investigate the prevalence of eardrum pathology and hearing impairment in young children and teenagers with Turner's syndrome. A possible relation to karyotype was also investigated. A high prevalence (61%) of recurrent acute otitis media was found in the study group and 32% had been treated with ventilation tubes. Fifty-seven percent showed eardrum pathology, such as effusion, myringosclerosis, atrophic scars, retraction pockets and perforations. Auricular anomalies were noted in 23% of the cases, most commonly in the 45, X group. The audiometric analysis showed conductive hearing loss (air-bone gap > 10 dB HL) in 43% and the typical sensorineural dip in the middle frequencies was found in 58% of the girls, of whom the youngest was 6 years old. Four percent were using hearing aids. The data of this study further confirm that the dip is progressive over time and may be detectable as early as at the age of 6, giving a chance to predict a future hearing loss. The findings emphasize the importance of regular otological examinations and audiological evaluations of all girls with Turner's syndrome early in life.

Acute Disease↗

Distribution of Na,K-ATPase is normal in the inner ear of a mouse with a null mutation of the glucocorticoid receptor.

This study was performed in order to test the hypothesis that the glucocorticoid hormone stimulates the formation of Na,K-ATPase in the inner ear of the mouse. An immunohistochemical study with respect to the presence and distribution of glucocorticoid receptors (GR) and Na,K-ATPase in the vestibular and cochlear regions of the inner ear was performed on a C57BL mouse with a null mutation of the glucocorticoid receptor (GR mutant mouse). The wild type C57BL mouse and the CBA mouse served as normal controls. As expected, the homozygous GR mutant mouse showed no specific staining for GR in the inner ear. The heterozygous GR mutant mouse showed faint staining of GR in the spiral limbus, the spiral ganglion, the organ of Corti and the utricle. This staining was markedly less than in the wild type C57BL mouse. Antibody labelling of Na,K-ATPase in the inner ear showed no significant difference between the homozygous and the heterozygous GR mutant mouse as compared to the control wild type C57BL mouse or the CBA mouse. Although earlier studies have shown a positive correlation between levels of glucocorticoid hormone in serum and the concentration of Na,K-ATPase in the inner ear, the hypothesis that glucocorticoid hormones alone stimulate the formation of Na,K-ATPase in the inner ear could not be confirmed by this study. Thus other regulating substances must be considered.

Adrenal Cortex Hormones↗

Aspects of diagnosis of acute otitis media.

BACKGROUND: Acute otitis media is a common disease, particularly among children. The importance of a correct diagnosis is crucial, especially as unjustified prescription of antibiotics has become a major problem in clinical praxis. OBJECTIVES: Our aim was to evaluate the predictive value of different otological findings in diagnostics and treatment of acute otitis media among GPs and ear specialists and to investigate if the diagnosis could be improved by the use of an ear microscope instead of an otoscope. Furthermore, we aimed to test the value of following an algorithm, METHODS: Thirty-one patients with otalgia at the Emergency Department at the Karolinska Hospital in Stockholm were examined by both a GP and an ear specialist. The GP used an otoscope, whereas the ear specialist first used an otoscope and then an ear microscope. The doctors registered their findings and their proposal for treatment in questionnaires. The ability among participating ear specialists to give a correct diagnosis was confirmed by the use of 12 video-taped selected cases of aural diseases. An algorithm for diagnostics, based on medical facts, was formulated and tested. RESULTS: In general, the concordance between ear specialists and GPs was satisfactory with regard to establishing the diagnosis acute otitis media. The diagnostics were not improved by use of an ear microscope. The algorithm identified most patients with acute otitis media. Conclusion. The results indicate that the following of a simple algorithm may simplify the CONCLUSION: and lead to a correct diagnosis of acute otitis media.

Acute Disease↗

Turner's syndrome and hearing disorders in women aged 16-34.

Forty women with Turner's syndrome aged 16-34 years were tested clinically and audiometrically according to their ear problems and hearing. A high incidence of middle-ear infections was demonstrated. A mid-frequency sensorineural hearing loss was frequently diagnosed and could be correlated to the karyotype. The dip showed a progression with age. Middle-ear problems were more common among women with a dip. An early high-frequency hearing loss could be noted in the present group among the older women. In some cases this had already led to social hearing problems and use of hearing aids. When comparing these women with a group of elderly Turner women the dip was not as deep, the maximum peak was seen in the 2 kHz region and social hearing problems and hearing aids were not as frequent. If no dip was found no major hearing problems could be detected or expected in future life. The data emphasize the importance of early audiological evaluation and information about predisposition to hearing impairment in Turner's syndrome.

Adolescent↗

Presence of glycosaminoglycans in the endolymphatic sac.

Earlier morphological investigations have revealed that the endolymph, which is present in the endolymphatic sac (ES) seems to differ from that found elsewhere in the labyrinth, in that it contains a stainable substance. Histochemical investigations indicate that this substance is rich in glycosaminoglycans (GAGs). It has been speculated that the stainable substance might play a role in regulation of fluid and ions and also, possibly, the pressure within the endolymphatic sac compartment. Endolymphatic sac specimens obtained from adult guinea pigs, rats, mice and in vitro cultured fetal inner ears were incubated with monoclonal antibodies against epitopes, and after enzymatic digestion revealed five different GAGs: hyaluronan, chondroitin-4-sulphate+dermatan sulphate, chondroitin-6-sulphate and keratan sulphate. In order to verify the specificity of these antibodies, otocysts from fetal mice were incubated in the same way. These cartilaginous specimens are known to contain GAGs in abundance and served as positive controls. The results indicate that the hyaluronan visualized by the monoclonal antibody is present to a large extent in the lumen of the ES and in the epithelial cells. Keratan sulphate and chondroitin-4-sulphate+dermatan sulphate are present within the epithelial lining, in the subepithelial tissue, and to some minor extent in the lumen, while chondroitin-6-sulphate does not show any specific staining.

Animals↗

Pathology of the cochlea following a spontaneous mutation in DBA/2 mice.

The DBA/2 strain of mice usually presents with noise-induced epileptic seizures and hearing disorders. After a spontaneous mutation a strain with early hearing loss and circling behaviour was produced. This strain presents with clinical symptoms found in diseases connected to inner ear disorders. These animals do not suffer from periodical disorders, however, but have functional disturbances continuously and can therefore serve as an animal model for diseases originating from both parts of the inner ear. The genetic inheritance appears to be autosomal recessive. Offspring showed circling behaviour and severe pathology in the vestibular part of the inner ear. In the present study pathology of the cochlear part of the inner ear was visualized using conventional microscopical techniques. The content of actin and fodrin was labelled immunohistochemically, and hearing was assessed with auditory brainstem recordings. After 1 month the animals showed deterioration of the cochlear part of the inner ear. At 6 months no organ of Corti remained and the animals were deaf. Transmission and scanning electron microscopy revealed severe apical hair cell changes. The content of alpha-actinin and fodrin in the DBA/2 mouse was already fainter than that in age-matched CBA control mice at the age of 1 month. Labelling of antibodies against fodrin increased in the supporting cells of the older animals, probably owing to the replacement of hair cells.

Actins↗

Development of tympanosclerosis: can predicting factors be identified?

HYPOTHESIS: The etiological hypothesis is that there might be factors triggering an immunological chain reaction that eventually leads to tympanosclerosis formation. BACKGROUND: Tympanosclerosis is a condition leading to a calcification process in the middle ear and, occasionally, also to the lining of the inner ear. This sometimes leads to hearing loss due to fixation of the middle ear ossicles. In severe cases. deafness may occur as a result of the inner ear impairment. Surgery is the treatment offered, often with poor long-term results, and, alternatively, prescription of hearing aids. Some patients develop tympanosclerosis after mild inflammatory otitis media processes whereas some heal without tympanosclerosis after more aggressive infections. This difference may be due to individual variations in the inflammatory response. The biological mechanism of calcification in tympanosclerosis is probably similar to that occurring in other calcifying tissues due to diseases. METHODS: The present investigation was performed to develop methods for immunohistochemical analyses of this delicate tissue consisting of both hard bone and the very thin tympanic membrane. Sprague-Dawley rats were inoculated with a suspension of Streptococcus pneumoniae, type 3, into the middle ear and sacrificed after 1 week up to 6 months. A new technique was elaborated where the whole specimen was prefixed briefly and then en bloc incubated with the primary antibodies and after that decalcified in edetic acid (EDTA). Primary antibodies against macrophages were used for the immunohistochemical staining. RESULTS: Acute otitis media was successfully induced in the rats and myringosclerosis was seen in 30% of the animals, often localized close to the bony frame where macrophages could also be detected. CONCLUSIONS: Acute otitis media and myringosclerosis were introduced in the animals. Conventional immunological techniques were tested on this delicate tissue. A new method for immunohistochemical staining was elaborated in which specimens were stained en bloc before decalcification and sectioning were performed. Expression of macrophages was demonstrated in the tympanic membrane.

Animals↗

Na,K-ATPase alpha- and beta-isoforms in the developing cochlea of the mouse.

Immunohistochemistry was used to investigate the presence of Na,K-ATPase alpha- and beta-subunits isoforms (alpha 1, alpha 2, alpha 3, beta 1 and beta 2) in the cochlea of the mouse at different ages between embryological day (E) 19 and postnatal day (P) + 30. alpha 1 was mainly found in the stria vascularis and in the spiral ligament; it increased steadily from p+4. These data correlates well with the morphological and electrophysiological maturation of the cochlea. alpha 3 predominated in the spiral ganglia and the cochlear nerve. This finding is well in accordance with reports that alpha 3 seems to be associated with the nervous system. The beta-subunit was found mainly in those tissues where staining of the alpha-subunit also was seen. Both subunits were localized in tissue regions where fluid regulation is expected to play an important role. For some isoforms, the expression pattern of Na,K-ATPase during development in the mouse is different from that in the rat. The expression of Na,K-ATPase and that of glucocorticoid receptors during development in the inner ear of the mouse show a similar pattern, which may indicate that glucocorticoid receptors could be involved in regulating the expression of Na,K-ATPase.

Animals↗

Inner ear content of glycosaminoglycans as shown by monoclonal antibodies.

Inner ear cells are known to contain combinations of proteins and sugar, which histochemically have been identified to be proteoglycans. To visualize these components in the macula utriculi, crista ampullaris and in the cochlea four different monoclonal antibodies against hyaluronan (HA), keratan sulphate (KS), chondroitin-6-sulphate (C-6-S) and chondroitin-4-sulphate (C-4-S) were used. The results indicate that KS is predominantly present in the lining of the vestibular hair cells, in the otoconial layer and in nerve tissue. C-4-S is present in the sensory hairs as well as in the subepithelial layer but also in the tectorial and basilar membranes of the cochlea. HA is generally present in the surface area of vestibular hair cells, to some extent in the cochlear hair cells and in the stria vascularis. C-6-S is only present to a lesser degree in the inner ear.

Animals↗

Appearance of glucocorticoid receptors in the inner ear of the mouse during development.

CBA mice were sacrificed at different ages of developments at embryonic day 13 (E13), E14, E16, E19, E20 and postnatal day 1 (P1), P2, P3, P4, P6, P8, P10, P14, P16, P18, P20 and P30. The temporal bones were quickly removed and deep frozen in order to prepare cryosections for immunohistochemical staining with polyclonal antibodies against glucocorticoid receptors. The avidin-biotin, ABC-method was used to visualize binding. Both the vestibular and the cochlear regions of the inner ear were analysed. A faint staining of the crista ampullaris, the utricle and the cochlear duct was seen at E19; and staining became clearly visible at P1. A decrease in labelling was found at day 2-5 post partum whereafter an increased staining was again noticed until postnatal day 14 when an adult pattern was observed. The appearance of glucocorticoid receptors in the inner ear during development does not follow a linear curve. Further, the labelling pattern may indicate an impact of glucocorticoid receptors on the embryologic maturation itself as well as a functional role in the adult ear.

Animals↗

[Glimpses from the history of otitis media].

The history of otitis media has much in common with the history of other infectious diseases. Over the years France, England and Germany respectively dominated the field and treatment of otitis media. During the French period (eighteenth and nineteenth century), Louis Petit for the first time performed an operation with opening of the mastoid cells. In the last decades of the nineteenth century, during the English period, many famous ear doctors spread their names over the world, namely J. Toynbee, J. Hinton and W. Wild of which the latter 1853, wrote the first text book in the field called "Practical observations on aural surgery and the nature and treatment of diseases of the ear." During the German period the surgical treatment (mastoidectomy) of otitis media and its sequele was fullfilled by A. von Tröltsch, H. Schwartze and A. Politzer who together started the first journal "Archiv für Ohrenheilkunde". Politzer also 1873, started the first ear clinic in Europe, in Vienna. It was almost a rule, that all ear surgeons in Europe during that time, had to visit the ear clinic in "Allgemaines Krankenhaus" in Vienna to gain a proper education. 1940 B. Chain, V. Fleming and H. Florey invented the penicillin and were rewarded with the Nobel prize, and from then on the treatment of otitis media changed dramatically.

Europe↗

Glucocorticoid receptor expression in the postnatal rat cochlea.

The glucocorticoid receptor (GR) expression in the neonatal rat cochlea was investigated by utilization of a polyclonal antibody against GR, the immunoreactivity of which exhibited a distinct, age-dependent developmental pattern in tissues of the spiral ligament (SL). Immunostaining of GR appeared initially at the 7th postnatal day (PND), increased rapidly between the 14th and 21st PND, and reached adult-like expression levels by the 21st PND. Less pronounced, developmentally regulated expression patterns of GR were observed in cells of the spiral limbus (SLi), spiral ganglion (SG), organ of Corti (OC), and cochlear nerve (CN). For example, high expression levels of GR were observed in the SLi, SG and OC at 3 PND; subsequently, GR immunoreactivity levels decreased from 7 to 14 PND, and then GR immunoreactivity intensified in these regions by 21 PND. No remarkable changes in GR expression were observed in stria vascularis (SV). These data indicate that GR expression in the inner ear is tissue and age-specific, and that GR expression parallels both Na,K-ATPase expression and endocochlear potential development.

Aging↗

Influence of prenatal irradiation on second generation mice.

Pregnant mice were irradiated with 0.5 and 2 Gy on the 13th gestational day. Offspring were raised until sexual maturity at 2 months of age. Mice from prenatally irradiated mothers and unexposed fathers, or from prenatally irradiated fathers and unexposed mothers, or from parents who had both been prenatally irradiated were mated, resulting in the birth of several mice. These second generation mice were tested as adults for hearing thresholds, with the ABR technique, subjectively to balance tests. and investigated morphologically. Hearing levels were found to be normal and the organ of Corti showed only minor changes of its inner and outer hair cells as shown by transmission and scanning electron microscopy. It is concluded that prenatal irradiation does not seem to cause mutations leading to impaired hearing and balance in second generation mice.

Animals↗

[Morris Fishbein].

Explore the source record for details and available documents.

American Medical Association↗

Ear and hearing problems in 44 middle-aged women with Turner's syndrome.

The present study has investigated ear and hearing problems in 44 women with Turner's syndrome (median age 45.5 years). Social hearing problems were common after the age of 40 and 27% were fitted with hearing aids. Audiograms revealed a hearing loss > 20 dB hearing level (HL) in 91% leading to clinically significant hearing problems in 60%. A distinct dip in the 1.5 kHz frequency range, with a mean value of 46 dB was found in 30 women. The occurrence of the dip was correlated to the karyotype. All women with the karyotype 45,X and 45,X/46,X,i(Xq) demonstrated this dip while in the 45,X/46,XX group it was found in 31%. No dips were found among 45,X/46,XY and 45,X/46,XX/47,XXX women. With increasing age a progressive high frequency hearing loss was added to the dip leading to severe hearing problems earlier in the Turner women than age-matched controls. This might be due to a genetic defect leading to premature ageing of their hearing organ. These data emphasize the importance of providing early information to Turner girls of their predisposition to hearing impairment. Patient awareness of importance of audiological evaluations and the benefit of hearing aids should be stressed.

Adult↗

Vestibular morphology in relation to age and circling behavior.

The DBA/2 strain of mice have genetically induced vestibular dysfunction that presents grossly at an early age as circling behavior and abnormal righting reflexes. The vestibular morphology of this strain has not previously been examined. DBA/2 mice of different ages that showed circling behavior were sacrificed and then had their inner ears immediately removed and fixed in glutaraldehyde. The specimens were prepared for light, transmission and scanning electron microscopy. Additional specimens at 10 months of age were fixed with paraformaldehyde for immunohistochemical investigation and labelling of alpha-actinin. Non-circling litter mates served as controls. The morphology and immunohistochemistry of the vestibular end-organs were evaluated as a function of age and circling behavior. The sensory epithelium of the ampulla and utricle in the circling mice showed softening of the cuticle, hair cell cytoplasmic herniation, expelled cellular debris, fused stereocilia and giant hair cells that progressively increased in severity with age. The non-circling litter mates showed similar but less severe pathology of the vestibular sensory epithelium. The immunohistochemical analysis showed no differences at a magnification of 400 x.

Acoustic Maculae↗

[Brühl's collection of anatomical specimens at the ear clinic of the Karolinska hospital].

Gunnar Holmgren, professor in oto-rhino-laryngology at the Sabbatsbergs Hospital in Stockholm, had in 1921 the opportunity to buy a unic temporal bone collection from Germany. As sponsor served the first Swedish, female specialist in oto-rhino-laryngology, Marta P:son Henning. The collection consists of three different sections, namely temporal bones with normal variations as well as under pathological conditions, pathological samples preserved in formalin and about 1000 microscopical slides. This collection is now situated at the ENT clinic, Karolinska Hospital, Stockholm in its total former condition. The article discusses the circumstances under which the collection was collected.

Germany↗