Neonatal screening for hearing impairment.
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Biomedical subjects
Publications and source records attributed to J J Grote.
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In this study, the efficacy of bactericidal/permeability-increasing protein (BPI) was assessed in a rat model of chronic otitis media with effusion. BPI injection prevented disturbance of the mucociliary clearance system of the middle ear. Hence, it is postulated that BPI can be a new therapy for chronic otitis media with effusion.
The middle ears of 48 rats were used to examine the effects of endotoxin injection, eustachian tube obstruction or a combination of eustachian tube obstruction and endotoxin injection. Animals were killed after 1, 2, 4, or 12 weeks and the middle ears processed for light and scanning electron microscopy. Compared to the normal middle ear mucosa, the epithelial layer was more pseudostratified, cuboidal or cylindrical after endotoxin injection or obstruction of the eustachian tube. In the early phase, numerous ciliated cells occurred in areas originally almost devoid of these cells. At 3 months, degeneration of ciliated cells was observed. The combination of eustachian tube obstruction and endotoxin injection also induced a more pseudostratified, cuboidal or cylindrical epithelium with an increased number of goblet cells. However, an early decrease occurred in the number of ciliated cells in the tympanic orifice of the eustachian tube. Furthermore, inflammatory cells, mainly PMNs, macrophages and lymphocytes, invaded the subepithelial layer after eustachian tube obstruction and endotoxin injection. These structural changes resulted in an impairment of the mucociliary transport system for clearance of the middle ear cavity. For this reason we believe that both endotoxin and eustachian tube obstruction or dysfunction play an important role in inducing persistent mucosal changes in the middle ear cavity, thereby prolonging otitis media with effusion.
OBJECTIVES/HYPOTHESIS: This study was performed to elucidate the role of endotoxin and tumor necrosis factor-alpha (TNF-alpha) in the middle ear effusions (MEEs) of children with otitis media with effusion (OME) in relation to the chronicity of the disease and the presence of upper respiratory tract infection (URTI). STUDY DESIGN: In a retrospective study 140 MEEs were collected from 101 children between 2 and 12 years of age, and evaluated for the cytokine TNF-alpha and the lipopolysaccharide endotoxin. The amounts were quantified and correlated with the type of MEE, OME duration, and the presence of URTI. METHODS: Endotoxin levels were measured using a limulus amebocyte lysate assay and TNF-alpha levels were measured with an enzyme-linked immunosorbent assay (ELISA). Means of the different variables were compared using the one-way ANOVA least significance difference test with P<.05. RESULTS: In MEEs classified as mucopurulent (22.8%) both endotoxin and TNF-alpha levels (11.9+/-3 ng/mg total protein and 61.1+/-21 pg/mg total protein, respectively) were significantly higher compared with serous- (23.6%) or mucoid- (53.6%) type effusions. Fifty-five percent of the children who were classified as having chronic OME also had significantly higher amounts of endotoxin and TNF-alpha. The majority of the children (61%) had no URTI, although children with URTI (36%) did also have significantly higher levels of endotoxin and TNF-alpha in their middle ears. CONCLUSIONS: These results indicate that there is a strong correlation between the endotoxin and the TNF-alpha concentration in the middle ear and the type of MEE, the presence of URTI, and the chronicity of the disease.
Hearing impairment occurs in 10% of the Dutch population and may lead to major communication problems and even social isolation. A good method to detect hearing loss in a general practice is the screening audiometer. The treating physician may further use the whispered speech test and tuning fork tests which together give a good impression of the severity and the nature of the hearing loss if any. The whispered speech test is best performed in the standardized form according to the guideline 'Slechthorendheid' [hardness of hearing] of the Dutch College of General Practitioners (NHG), in which certain combinations of letters are recommended. The tuning fork tests according to Rinne and Weber indicate a difference in perception and conduction deafness, and are decisive for any hearing asymmetry. Use of the whispered speech test and of the tuning fork tests is recommended for adults and children from the age of 7.
OBJECTIVE: The results of the first cohort of 60 cavity reconstructions with hydroxyapatite with a minimum follow-up period of 15 years were studied. STUDY DESIGN: The study design was a retrospective study. PATIENTS: A total of 60 patients had a follow-up period of >15 years. In four patients, not all data were available. Therefore, 56 patients were included in the study. They had a combination of cavity problems and hearing loss. INTERVENTION: The ear canal was reconstructed with a canal wall prosthesis of porous hydroxyapatite. The ossicular chain was reconstructed with an incus or incus-stapes prosthesis of dense hydroxyapatite. RESULTS: After 15 years, 42 patients (75%) had an intact reconstructed ear canal. The main problem for failure was the recurrent purulent middle ear infection and not cholesteatoma. The histology of the retrieved canal wall showed a good remodeling in living bone tissue. After 15 years, 34 patients had a normal ear canal and an ossicular chain. Of these patients, 7.05% had an air-bone gap closure within 20 dB. CONCLUSIONS: Long-term results of cavity reconstruction with hydroxyapatite are possible. The main problem is recurrent mucosal disease of the new middle ear-mastoid cleft.
Otitis media with effusion usually resolves spontaneously. If not, long-term hearing impairment has to be prevented. The available literature indicates that antibiotic treatment has at most a short-term effect. Therefore it is not indicated for the treatment of otitis media with effusion.
HYPOTHESIS: We sought to classify the shape and structure of the anterior epitympanic space (AES) and produce measurements of its dimensions together with its relationships with the facial nerve and geniculate ganglion. BACKGROUND: The AES is limited by the middle cranial fossa superiorly, zygoma root anteriorly, cog posteriorly, chorda tympani laterally, facial nerve medially, and tensor tympanic semicanal inferiorly. METHODS: The AES was examined in 30 human temporal bones using two different methods. Twenty bones were cut vertically and a modified radical mastoidectomy was performed in the other 10 bones. RESULTS: The AES showed two types in the vertically cut bones according to its shape and structure. Type I, found in 17 (85%) of the bones, showed two cavities that were separated by a bony landmark and the tensor tympanic fold. The name "supratubal ridge" is suggested for this bony landmark. In type II, which was seen in three (15%) of the bones, there was only one cavity. In the mastoidectomy group, again two types of AES were found: eight (80%) were type I and two (20%) were found to be type II. If we combine these findings with the vertically cut bones, we find that 25 (83.3%) possess an AES type I, whereas five (16.7%) are type II. CONCLUSIONS: These variations in the structure of the AES and its close relationships with a number of vital structures such as the facial nerve, cochlea, and middle fossa dura must be taken into account during the surgical management of middle ear disease.
OBJECTIVE: The relations between response thresholds obtained by 1) transtympanic (TT) and 2) extratympanic (ET) electrocochleography, and by 3) pure tone audiometry, were investigated in a population of patients with cochlear hearing loss of various types and degrees, with the prediction of audiometric from electrocochleographic thresholds as the primary focus. DESIGN: Analyses were made of the relation between TT response thresholds for tone bursts with octave frequencies from 500 to 8000 Hz and audiometric thresholds in 148 ears. Similar analyses of ET thresholds were given for a subset of 30 ears in which TT and ET physiological responses were simultaneously recorded. RESULTS: TT electrocochleographic thresholds are highly correlated with audiometric thresholds. Linear regression analysis shows that audiometric thresholds can be predicted from physiological thresholds with an error in the estimate of 11 dB. ET electrocochleography permits similar predictions but with a larger uncertainty of 16 dB. Both methods demonstrate a clear frequency specificity. As a remarkable secondary observation, it appears that electrocochleographic thresholds increase slightly less with increasing cochlear dysfunction than do pure tone thresholds. The hypothesis is elaborated that this is due to the different stimulus durations on which the two threshold measurements are based in interaction with the difference in temporal integration between normal and pathological ears. CONCLUSION: Electrocochleography is a powerful method for the objective and frequency-specific determination of cochlear response thresholds. TT recording is the preferred method, but ET recording is suitable with a larger uncertainty in the estimate. Electrocochleographic thresholds directly measure the cochlear sensitivity as such, whereas pure tone thresholds measure the combination of loss of cochlear sensitivity and of reduced temporal integration.
OBJECTIVE: The aim of this study is to explore the applicability of two specific methods in extratympanic (ET) electrocochleography that have rarely been used there, but are more commonly applied in transtympanic (TT) approaches. These two methods are the use of tone burst stimulation in addition to clicks, and the analysis of amplitude input/output curves as a measure of cochlear recruitment due to a pathological spread of excitation. DESIGN: Simultaneous extratympanic and transtympanic recordings were made in 30 patients with various types and degrees of cochlear hearing loss. ET recordings were also made in a group of normal subjects. Stimuli were tone bursts at octave frequencies from 500 to 8000 Hz and clicks, at intensity levels of about 90 dB down to response threshold in 10 dB steps. First, a general comparison is made of ET versus TT response properties, then the initial slopes of I/O curves in both sets of data are evaluated. RESULTS: ET responses are reduced in amplitude with respect to TT responses by a factor of 0.43 on average. Within each subject this factor is independent of stimulus type or level, but it has a substantial intersubject variation. ET and TT latencies are identical. From the normal data a criterion for abnormality of the slope of the I/O curve is derived in a similar way as available for TT data. Using this criterion ET I/O curves appear to be significantly steeper than normal in recruiting ears, albeit with a lesser sensitivity than for the associated TT data. CONCLUSIONS: Extratympanically recorded responses to tone burst stimuli provide basically the same information as transtympanic recordings. However, for a reliable interpretation of responses to low-intensity stimuli a larger amount of signal averaging is required. The analysis of I/O curves is a useful extension of current ET methods as a contribution to assessing a pathological spread of excitation in the cochlea.
The effect of human monoclonal antibody HA-1A (Centoxin) on the effect of endotoxin on cultured rat middle ear epithelium was investigated. The addition of endotoxin to the standard culture medium revealed a concentration-related proliferative effect on cultured rat middle ear epithelium, leading to cobblestone cells, cell tracks, and stratification of epithelium, whereas rat middle ear epithelium cultured in standard medium grew as a monolayer composed of flat polygonal cells. Addition of HA-1A to standard medium supplemented with endotoxin gave rise to a statistically significant suppression of the proliferative effects of endotoxin on these cells. The morphology of rat middle ear epithelium cultured in the presence of HA-1A and endotoxin showed that these cells still had a tendency to form cobblestone-like cells and cell tracks, but to a substantially lower degree. The present results support the hypothesis that HA-1A suppresses the proliferative and morphological effects of endotoxin on rat middle ear epithelium and may play an important role in the pathogenesis of otitis media.
The formation of Cholesteatoma is often accompanied by infection, and is very likely to be influenced by inflammatory mediators. Endotoxin, a strong inflammatory mediator, may play an important role in cholesteatoma pathogenesis. It has been demonstrated in middle ear effusions, and it causes marked reactions in middle ear epithelium and epidermis, both in vivo and in vitro. The effect of endotoxin on a simulation of the advancing front of cholesteatoma was investigated in this study. We developed a co-culture model composed of a simultaneous culture of rat middle ear mucosa and rat meatal epidermis in one culture dish, to which endotoxin was added. During the culture period the addition of endotoxin to the co-culture delayed take-over of the meatal epidermis by the middle ear mucosa, in comparison with control cultures. Morphological changes included the clustering of microvilli in co-cultures exposed to endotoxin. Although these results are preliminary, they suggest that endotoxin disturbs the normal healing process of the middle ear cavity.
We have performed retinal fluorescein angiography and audiometry in 32 familial and 7 sporadic cases of facioscapulohumeral muscular dystrophy. A mild to moderate retinal vasculopathy, consisting of retinal teleangiectasis and microaneurysms, was present in 18 of 37 evaluable angiograms (49%); 5 patients had minimal changes and 14 angiograms (38%) were normal. High frequency hearing loss was found in 25 (64%) out of 39 patients. Retinal changes were absent in 5 of 18 families (6 cases examined), and after correction for age and sex, hearing function was normal in 5 of 19 families (7 cases examined). Age and severity of the myopathy did not have a clear relationship with the retinal vasculopathy or the hearing loss. There were no differences between families in which the myopathy was linked to chromosome 4q35 and families in which linkage could not be proven. Minimal retinal vascular changes and high tone hearing loss can be observed occasionally in the normal population. Therefore, although retinal vasculopathy and hearing loss are part of the clinical picture of FSHD, these signs cannot be accepted as decisive criteria for FSHD in clinically equivocal cases.
Reconstruction of a totally empty middle ear is difficult. This study reports results of 15 patients implanted with a total artificial middle ear developed on the principles of a total homologous middle ear and consisting of a canal wall prosthesis, a tympanic membrane/malleus prosthesis, and an incus/stapes prosthesis. Satisfactory results were noted in 12 of 15 patients at 1-year follow-up, with air-bone gap closure within 30 dB; within 20 dB in seven of these twelve. However, at 3-year follow-up examination the total artificial middle ear was removed in seven cases due to obliteration and infection of the middle ear. These failures related to the extensive middle ear disease initially noted in these seven patients. These long-term results indicate the necessity of careful patient selection for reconstruction.
In 1983, 1338 liveborn infants with a gestational age of less than 32 weeks and/or a birthweight of less than 1500 g, were enrolled in a national follow-up study in The Netherlands. At the age of 5 years, 966 children were alive. Of these, 927 (96%) were assessed on a home visit 2-6 weeks after their fifth birthday by three specially trained paediatricians. An assessment of ENT morbidity was made and compared with ENT morbidity in full-term children of the same age group. Markedly preterm birth or very low birthweight does not seem to be a risk factor for developing middle ear disease in childhood, however, the rate of ENT problems seems to be higher than in the general population of Dutch pre-school children.
A group of 266 children (515 ears), ranging in age from 5 months to 11 years, was studied. These children were candidates for the insertion of ventilation tubes, or adenoidectomy and/or tonsillectomy with myringotomy. Before surgery, tympanometry was performed. The surgical and tympanometric findings were compared afterwards. Two different tympanometers were used (GSI-27A and TYMP-85TT). This study showed a comparable validity of these two tympanometers. The sensitivity and specificity of tympanometry in the age group of 5 months to 2 years did not show a significant difference from that in the age group of 2-12 years. Otoscopy has limited value for the diagnosis of middle ear effusion in this age group.
Several factors seem to contribute to the series of events in the pathogenesis of otitis media and cholesteatoma. Endotoxin is likely to be one of these factors, since it has been found in human middle ear effusions and since injection of this substance into the middle ear, in animal experiments, gave rise to prominent reactions. Provoking of epithelial cells in vitro with endotoxin led to distinct cell responses that might be associated with cholesteatoma formation. In this study the effect of endotoxin on serially cultured rat middle ear epithelium, rat meatal epidermis, and human keratinocytes was investigated. Endotoxin strongly stimulated the proliferation of middle ear epithelium and human keratinocytes and inhibited that of meatal epidermis. Furthermore, endotoxin affected the morphology of the three types of tissue. Rat middle ear epithelium revealed epithelial cell tracks with interconnecting bridge-like structures protruding above the culture plane, whereas rat meatal epidermis showed increased terminal differentiation expressing large areas of blister-like structures detaching from the culture dish. Cross-linked envelope analysis of human keratinocytes showed an increased terminal differentiation that was morphologically confirmed but was not confirmed by cytokeratin analysis. The results of this study support the hypothesis that endotoxin may play an important role in the pathogenesis of otitis media and cholesteatoma.
In a geographically defined population of very preterm and very low birthweight infants (gestational age < 32 weeks and/or birthweight < 1500 g) hearing was evaluated in 890 children by pure-tone audiometry at the age of 5 years. Hearing loss was conductive/unspecified in 123 (13.8%) and sensorineural in 13 (1.5%) children. The prevalence of sensorineural hearing loss was 15 times as high as in 5-7 year old children in the Dutch population at large. The sensorineural hearing loss prevalence in very low birthweight and extremely low birthweight infants was similar. On account of communication disorders 10 (1.1%) children were classified as disabled and 6 (0.7%) as handicapped, following the definitions of the International Classification of Impairments, Disabilities, and Handicaps of the World Health Organisation. Children with conductive hearing loss had a higher risk of impairments, disabilities and handicaps of language and speech development, than children with normal hearing, the difference being statistically significant. The same holds for children with sensorineural hearing loss; moreover they had a significantly higher risk of impairments, disabilities and handicaps of mental development. Overall comparison of children with and without sensorineural hearing loss proved that the children with sensorineural hearing loss had a significantly less favourable outcome, based on 15 perinatal factors simultaneously. The age at which sensorineural hearing loss in very preterm and/or very low birthweight infants is detected has to be improved.