The facial nerve palsy and cortisone evaluation (FACE) study in children: protocol for a randomized, placebo-controlled, multicenter trial, in a Borrelia burgdorferi endemic area.

BACKGROUND: Children with acute peripheral facial nerve palsy cannot yet be recommended corticosteroid treatment based on evidence. Adults with idiopathic facial nerve palsy are treated with corticosteroids, according to guidelines resulting from a meta-analysis comprising two major randomized placebo-controlled trials. Corresponding trials in children are lacking. Furthermore, acute facial nerve palsy in childhood is frequently associated with Lyme neuroborreliosis, caused by the spirochete Borrelia burgdorferi. The efficacy and safety of corticosteroid treatment of acute facial nerve palsy associated with Lyme neuroborreliosis, has not yet been determined in prospective trials in children, nor in adults. METHOD: This randomized double-blind, placebo-controlled study will include a total of 500 Swedish children aged 1-17 years, presenting with acute facial nerve palsy of either idiopathic etiology or associated with Lyme neuroborreliosis. Inclusion is ongoing at 12 pediatric departments, all situated in Borrelia burgdorferi endemic areas. Participants are randomized into active treatment with prednisolone 1 mg/kg/day (maximum 50 mg/day) or placebo for oral intake once daily during 10 days without taper. Cases associated with Lyme neuroborreliosis are treated with antibiotics in addition to the study treatment. The House-Brackmann grading scale and the Sunnybrook facial grading system are used for physician-assessed evaluation of facial impairment at baseline, and at the 1- and 12-month follow-ups. Primary outcome is complete recovery, measured by House-Brackmann grading scale, at the 12-month follow-up. Child/parent-assessed questionnaires are used for evaluation of disease-specific quality of life and facial disability and its correlation to physician-assessed facial impairment will be evaluated. Furthermore, the study will evaluate factors of importance for predicting recovery, as well as the safety profile for short-term prednisolone treatment in children with acute facial nerve palsy. DISCUSSION: This article presents the rationale, design and content of a protocol for a study that will determine the efficacy of corticosteroid treatment in children with acute facial nerve palsy of idiopathic etiology, or associated with Lyme neuroborreliosis. Future results will attribute to evidence-based treatment guidelines applicable also in Borrelia burgdorferi endemic areas. TRIAL REGISTRATION: The study protocol was approved by the Swedish Medical Product Agency (EudraCT nr 2017-004187-35) and published at ClinicalTrials.gov ( NCT03781700 , initial release 12/14/2018).

Peripheral facial nerve palsy in children in a Borrelia high-endemic area, a retrospective follow-up study.

AIM: To identify the incidence, aetiology and prognosis of acute peripheral facial nerve palsy (FNP) in children in the Borrelia high-endemic region of Stockholm. METHODS: The present study identified children from 0 to 18 years of age who visited a paediatric emergency department for acute peripheral FNP during a 1-year period from 2014 to 2015. Data were collected retrospectively. The Sunnybrook and House-Brackmann facial grading systems were used to measure clinical outcome. RESULTS: A total of 77 children were identified with FNP, an estimated incidence of 30 per 100 000 children/year. Forty-five children (58%) were diagnosed with neuroborreliosis, 28 (36%) with idiopathic FNP and four (6%) with other rarer causes. Neuroborreliosis was common from June to November and mainly seen in children below 10 years of age. Six patients (8%) had remaining symptoms at least 3 months after onset; three had idiopathic facial palsy (IFP) and were all older than 10 years, one had neuroborreliosis and two had other causes. CONCLUSION: Neuroborreliosis and IFP were the major causes of FNP during the study period. Neuroborreliosis-associated facial palsy had a seasonal variation and dominated in younger ages.

Ten years of experience with the Ponto bone-anchored hearing system-A systematic literature review.

BACKGROUND: Bone-anchored hearing systems (BAHSs) are widely used for hearing rehabilitation and are indicated in cases of conductive and mixed hearing loss and in single-sided deafness. The Ponto system, that is one available option, has been on the market since 2009. OBJECTIVE OF REVIEW: The aim of this study is to systematically review the literature reporting on the Ponto system, with regard to audiological and surgical outcomes and patient's quality-of-life scores. TYPE OF REVIEW: A systematic literature search was performed in the PubMed database 2009-July 2019. SEARCH STRATEGY: Search term: ((osseointegrated hearing aid) OR (bone conduction implant) OR (bone anchored hearing) OR BAHA OR BAHS OR BAHI). Pre-defined inclusion and exclusion criteria were applied. EVALUATION METHOD: English-language articles reporting original clinical data (audiological, surgical or quality-of-life outcomes) on the Ponto system were included. Articles reporting on Ponto and another BAHS system where the results on Ponto constituted less than 50% of the patient population or including only results on testband or softband devices were excluded. RESULTS: Audiological outcomes were discussed in 20 publications. Improvement against the unaided thresholds was demonstrated. The functional improvement was on average 33.9 dB. The effective gain or remaining air-bone gap was on average 6.7 dB. All evaluated data showed aided speech reception thresholds significantly below normal speech level. Twenty-seven publications reported surgical and follow-up data for the Ponto system. Implant survival was 97.7%, adverse skin reactions (Holgers ≥ 2) were 5% across visits and 15% across patients. No complications were life-threatening, causing permanent disability/damage or requiring a hospitalisation. Five studies reported quality of life using the Glasgow benefit inventory, 98% reported an improvement when analysing the score on an individual level. CONCLUSIONS: The outcomes of this systematic review confirm that percutaneous systems provide consistent audiological benefits and improved quality of life for patients. Further, the review demonstrates that the percutaneous systems are safe, with relatively low complication rates. Skin-related complications are the most common complication type and are experienced by approximately one patient out of seven, or in less than one of 20 follow-up visits.

Hearing outcome after myringoplasty in Sweden: A nationwide registry-based cohort study.

OBJECTIVES: To present hearing results after successful primary myringoplasty surgeries registered in the Swedish Quality Registry for Myringoplasty and to evaluate the chance of hearing improvement and the risk of hearing loss. DESIGN: A retrospective nationwide cohort study based on prospectively collected registry data between 2002 and 2012. SETTINGS: Registry data from secondary and tertiary hospitals performing myringoplasty. PARTICIPANTS: Patients with healed tympanic membrane after primary myringoplasty surgery performed from 2002 to 2012 in Sweden. MAIN OUTCOME MEASURES: Postoperative hearing results, hearing gain and air-bone gap (ABG). RESULTS: In 2226 myringoplasties, air conduction audiograms were recorded, and the average preoperative pure tone average (PTA4 ) of the group was 28.5 dB, which improved postoperatively to 19.6 dB with an average of 8.8 dB improvement. Bone conduction was measured for 1476 procedures. Closure of the ABG to 10 dB or less was achieved in 51% of the ears and to less than 20 dB in 89% of the ears. Sixty-one percent of patients with preoperatively deteriorated hearing experienced improved hearing, but 3% of all patients experienced deteriorated hearing. After the surgery, 93% of the patients were satisfied. CONCLUSIONS: Hearing results after successful myringoplasty surgery are often favourable, but although the tympanic membrane is healed, hearing improvement is not guaranteed, and hearing deterioration can also occur.

The bone conduction implant - a review and 1-year follow-up.

Objective: The objective of this study is to evaluate its safety and effectiveness of the bone conduction implant (BCI) having an implanted transducer and to review similar bone conduction devices.Design: This is a consecutive prospective case series study where the patients were evaluated after 1, 3, 6 and 12 months. Outcome measures were focussed on intraoperative and postoperative safety, the effectiveness of the device in terms of audiological performance and patient's experience.Study sample: Sixteen patients with average age of 40.2 (range 18-74) years have been included. Thirteen patients were operated in Gothenburg and three in Stockholm.Results: It was found that the procedure for installing the BCI is safe and the transmission condition was stable over the follow-up time. No serious adverse events or severe adverse device effects occurred. The hearing sensitivity, speech in noise and the self-assessment as compared with the unaided condition improved significantly with the BCI. These patients also performed similar or better than with a conventional bone conduction reference device on a softband.Conclusions: In summary, it was found that the BCI can provide a safe and effective hearing rehabilitation alternative for patients with mild-to-moderate conductive or mixed hearing impairments.

The clinical outcome and microbiological profile of bone-anchored hearing systems (BAHS) with different abutment topographies: a prospective pilot study.

PURPOSE: In this prospective clinical pilot study, abutments with different topologies (machined versus polished) were compared with respect to the clinical outcome and the microbiological profile. Furthermore, three different sampling methods (retrieval of abutment, collection of peri-abutment exudate using paper-points, and a small peri-abutment soft-tissue biopsy) were evaluated for the identification and quantification of colonising bacteria. METHODS: Twelve patients, seven with machined abutment and five with polished abutment, were included in the analysis. Three different sampling procedures were employed for the identification and quantification of colonising bacteria from baseline up to 12 months, using quantitative culturing. Clinical outcome measures (Holgers score, hygiene, pain, numbness and implant stability) were investigated. RESULTS: The clinical parameters, and total viable bacteria per abutment or in tissue biopsies did not differ significantly between the polished and machined abutments. The total CFU/mm2 abutment and CFU/peri-abutment fluid space of anaerobes, aerobes and staphylococci were significantly higher for the polished abutment. Anaerobic bacteria were detected in the tissue biopsies before BAHS implantation. Anaerobes and Staphylococcus spp. were detected in all three compartments after BAHS installation. For most patients (10/12), the same staphylococcal species were found in at least two of the three compartments at the same time-point. The common skin coloniser Staphylococcus epidermidis was identified in all patients but one (11/12), whereas the pathogen Staphylococcus aureus was isolated in five of the patients. Several associations between clinical and microbiological parameters were found. CONCLUSIONS: There was no difference in the clinical outcome with the use of polished versus machined abutment at 3 and 12 months after implantation. The present pilot trial largely confirmed a suitable study design, sampling and analytical methodology to determine the effects of modified BAHS abutment properties. LEVEL OF EVIDENCE: 2. Controlled prospective comparative study.

Audiometric features in young adults with Turner syndrome.

OBJECTIVE: Hearing loss (HL) is a known problem in adults with Turner syndrome (TS). The aim of this study was to investigate audiometric features in young adults with TS and the extent of hearing aid provision. DESIGN: Patients were recruited from the Turner centre at Karolinska University Hospital. Analysis of audiograms was made in relation to hearing aid use, a Swedish normal hearing cohort and the need for hearing rehabilitation. STUDY SAMPLE: Sixty-four women with TS aged 25-38 years at the time of their audiological testing. RESULTS: Fifty-two percent had impaired hearing in at least one ear. Sensorineural hearing loss (SNHL) was the most common type of HL, most often characterised by a high-frequency loss and/or a mid-frequency dip. Conductive HL was uncommon in young adults with TS, even though 47 percent were otitis prone as children. Eight of 64 women had previously been fitted with hearing aids. CONCLUSIONS: There is undoubtedly a need for hearing rehabilitation in young adults with TS. Questions about hearing must be asked by all doctors treating women with TS to identify those in need for hearing rehabilitation, even if they have an audiogram with a normal pure tone average.

Asymmetry in Cortical Thickness of the Heschl's Gyrus in Unilateral Ear Canal Atresia.

HYPOTHESIS: Unilateral congenital conductive hearing impairment in ear canal atresia leads to atrophy of the gray matter of the contralateral primary auditory cortex or changes in asymmetry pattern if left untreated in childhood. BACKGROUND: Unilateral ear canal atresia with associated severe conductive hearing loss results in deteriorated sound localization and difficulties in understanding of speech in a noisy environment. Cortical atrophy in the Heschl's gyrus has been reported in acquired sensorineural hearing loss but has not been studied in unilateral conductive hearing loss. METHODS: We obtained T1w and T2w FLAIR MRI data from 17 subjects with unilateral congenital ear canal atresia and 17 matched controls. Gray matter volume and thickness were measured in the Heschl's gyrus using Freesurfer. RESULTS: In unilateral congenital ear canal atresia, Heschl's gyrus exhibited cortical thickness asymmetry (right thicker than left, corrected p = 0.0012, mean difference 0.25 mm), while controls had symmetric findings. Gray matter volume and total thickness did not differ from controls with normal hearing. CONCLUSION: We observed cortical thickness asymmetry in congenital unilateral ear canal atresia but no evidence of contralateral cortex atrophy. Further research is needed to understand the implications of this asymmetry on central auditory processing deficits.

Bell's Palsy in Pregnancy and Postpartum: A Retrospective Case-Control Study of 182 Patients.

OBJECTIVE: To assess the incidence of Bell's palsy in pregnant and postpartum women. Additionally, to compare facial outcomes in terms of Sunnybrook score following Bell's palsy with regard to corticosteroid treatment and other confounding factors. STUDY DESIGN: Retrospective case-control study. SETTING: University Hospital, Stockholm, Sweden. METHODS: All women with Bell's palsy in pregnancy or postpartum (6 weeks after birth) with a computerized medical chart in the Stockholm Region 2005 to 2015 were included. The total number of births in the region during this period was retrieved from the Swedish Medical Birth Register. Nonpregnant age-matched women with Bell's palsy served as controls. Characteristics, medication, and Sunnybrook scores were collected. Risk factors for incomplete recovery (Sunnybrook score <96) at 3 months were calculated by logistic regression. RESULTS: In total, 182 pregnant and postpartum women with Bell's palsy were identified. The estimated incidence among pregnant and postpartum women was 60.5/100,000 person-years. The mean Sunnybrook score at 3 months was 74 among pregnant and postpartum women and 83 for controls (p = .002). At 12 months, Sunnybrook score was 81 and 89, respectively (p = .017). Only one-third of the pregnant women received corticosteroid treatment. CONCLUSION: The incidence of Bell's palsy in pregnancy and postpartum was 60.5 per 100,000 women and year in the Stockholm Region. Sunnybrook score was poorer in pregnant women compared with postpartum and nonpregnant women throughout. Corticosteroid treatment had little effect on any patients, however, only one-third of the pregnant women received this treatment.

A pilot study on spatial hearing in children with congenital unilateral aural atresia.

Despite normal hearing in one ear, individuals with congenital unilateral aural atresia may perceive difficulties in everyday listening conditions typically containing multiple sound sources. While previous work shows that intervention with bone conduction devices may aid spatial hearing for some children, testing conditions are often arranged to maximize any benefit and are not very similar to daily life. The benefit from amplification on spatial tasks has been found to vary between individuals, for reasons not entirely clear. This study has sought to expand on the limited knowledge on how children with unilateral aural atresia recognize speech masked by competing speech, and how horizontal sound localization accuracy is affected by the degree of unilateral hearing loss and by amplification using unilateral bone conduction devices when fitted before 3 years of age. In a within-subject, repeated measures design, including 11 children (mean age = 7.9 years), bone conduction hearing device (BCD) amplification did not negatively affect horizontal sound localization accuracy. The effect on speech recognition scores showed greater inter-individual variability. No benefit from amplification on a group level was found. There was no association between age at fitting and the benefit of the BCD. For children with poor unaided sound localization accuracy, there was a greater BCD benefit. Unaided localization accuracy increased as a function of decreasing hearing thresholds in the atretic ear. While it is possible that low sound levels in the atretic ear provided access to interaural localization cues for the children with the lowest hearing thresholds, the association has to be further investigated in a larger sample of children.

Non-invasive sampling procedure revealing the molecular events at different abutments of bone-anchored hearing systems-A prospective clinical pilot study.

Purpose: To investigate the molecular activities in different compartments around the bone-anchored hearing system (BAHS) with either electropolished or machined abutments and to correlate these activities with clinical and microbiological findings. Materials and methods: Twelve patients received machined or electropolished abutments after implant installation of BAHS. Peri-abutment fluid and tissue were collected from baseline to 12 months. Gene expression of cytokines and factors related to tissue healing and inflammation, regeneration and remodelling, as well as bacterial recognition were determined using quantitative-polymerase chain reaction (qPCR). The clinical status was evaluated using the Holgers scoring system, and bacterial colonisation was investigated by culturing. Results: The gene expression of inflammatory cytokines (IL-8, IL-1ß, and IL-10) and bacteria-related Toll-like receptors (2 and 4) was higher in the peri-abutment fluid than at baseline and in the peri-abutment tissue at 3 and 12 months. Conversely, the expression of genes related to tissue regeneration (Coll1a1 and FOXO1) was higher in the tissue samples than in the peri-abutment fluid at 3 and 12 months. Electropolished abutments triggered higher expression of inflammatory cytokines (IL-8 and IL-1ß) (in peri-abutment fluid) and regeneration factor FOXO1 (in peri-abutment tissue) than machined abutments. Several cytokine genes in the peri-abutment fluid correlated positively with the detection of aerobes, anaerobes and Staphylococcus species, as well as with high Holger scores. Conclusion: This study provides unprecedented molecular information on the biological processes of BAHS. Despite being apparently healed, the peri-abutment fluid harbours prolonged inflammatory activity in conjunction with the presence of different bacterial species. An electropolished abutment surface appears to be associated with stronger proinflammatory activity than that with a machined surface. The analysis of the peri-abutment fluid deserves further verification as a non-invasive sampling and diagnostic procedure of BAHS.

Estrogen receptor beta protects against acoustic trauma in mice.

The hormone estradiol affects the auditory system both by itself and by its interaction with neuroprotective factors. In this study, we examined the role of estrogen receptors (ERs) in response to auditory trauma. We found a ligand-dependent protective role for ERbeta in the auditory system by investigating mice deficient in ERalpha (ERKO mice), ERbeta (BERKO mice), and aromatase (ARKO mice). Basal auditory brainstem response (ABR) thresholds were similar in all animals. An acoustic trauma causing a temporary hearing loss raised ABR thresholds in male and female BERKO and ARKO mice compared with WT and ERKO mice. The ERalpha-selective agonist, propyl(1H) pyrazole-1,3,5-triyl-trisphenol (PPT), partially protected ARKO mice from trauma, while the ERbeta-selective agonist, 2,3-bis (4-hydroxyphenyl)-propionitrile (DPN), protected WT and ARKO mice. Immunohistochemistry and western blotting confirmed the expression of ERbeta in cochlea of WT males and females. Levels of brain-derived neurotrophic factor (BDNF), a neuroprotective peptide that can be induced by estrogen, was lower in BERKO and ARKO mice compared with WT. DPN treatment increased BDNF expression in ARKO mice. These data indicate ERbeta-mediated neuroprotection involving BDNF in the auditory system of males and females.

Quality of Life in Bell's Palsy: Correlation with Sunnybrook and House-Brackmann Over Time.

OBJECTIVES: To compare patient-graded facial and social/well-being function with physician-graded facial function in Bell's palsy over time. STUDY DESIGN: A prospective follow-up study at two tertiary otorhinolaryngological centers. METHODS: A total of 96 patients, 36 women and 60 men, aged 18-77 years, were included. Facial Clinimetric Evaluation (FaCE) scale and Facial Disability Index (FDI) scores were compared with Sunnybrook and House-Brackmann scores. RESULTS: Inclusion was on mean day 7 (96 patients) and follow-up on days 53 (81 patients) and 137 (32 patients). Initially, correlations between FaCE total score, FaCE domains, FDI physical function, FDI social/well-being function and Sunnybrook and House-Brackmann scores were low to fair, except for FaCE facial movement (r = 0.55). Correlations between FaCE total score and Sunnybrook score were very good to excellent at visits 2 (r = 0.83) and 3 (r = 0.81). Women scored FaCE social and FDI social/well-being function lower than men, despite similar Sunnybrook scores. CONCLUSION: In early stages of Bell's palsy, there were low to fair correlations between FaCE/FDI (except for facial movement) and Sunnybrook score. This implies that the design of the quality of life (QoL) instruments is less suited for the acute phase. The high correlations at follow-ups suggest that the questionnaires can be used for evaluation of QoL over time. Our results indicate that women experience more facial palsy-related psychosocial dysfunction. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E612-E618, 2021.

Adults with unilateral congenital ear canal atresia - sound localization ability and recognition of speech in competing speech in unaided condition.

BACKGROUND: Individuals with unilateral hearing loss show poor spatial hearing, but individual variability is high. AIMS/OBJECTIVES: To investigate if the degree of hearing loss in unilateral ear canal atresia affects horizontal sound localization and speech recognition. MATERIALS AND METHODS: Twelve subjects with unilateral ear canal atresia without childhood hearing intervention. Previously published data from eight normal-hearing subjects in normal binaural as well as experimentally induced unilateral hearing loss served as a reference. Horizontal sound localization and recognition of speech in spatially separate competing speech were assessed. RESULTS: Linear regression analysis demonstrated a relationship between sound localization accuracy (SLA) and the air conduction pure tone average of the atretic ear (r = 0.85, p=.007). The large proportion of variability in SLA (72%) explained by the degree of hearing loss of the atretic ear indicates that binaural processing is possible. SLA was worse than for normal hearing individuals (p<.0001), and comparable to moderate simulated unilateral hearing loss (p=.13). Speech discrimination was significantly worse than normal (p<.0001) and not dependent on degree of hearing loss of the atretic ear. CONCLUSIONS AND SIGNIFICANCE: Individuals with congenital unilateral ear canal atresia show impaired horizontal SLA and recognition of speech in competing speech.

Tinnitus and taste disturbances reported after myringoplasty: Data from a national quality registry.

OBJECTIVES/HYPOTHESIS: Postoperative tinnitus and taste disturbances after myringoplasty are more common than previously reported. STUDY DESIGN: This study was a retrospective analysis of prospectively collected data from the Swedish National Quality Registry for Myringoplasty. METHODS: The analysis was performed on extracted data from all counties in Sweden collected from database A from 2002 to 2012 and database B from 2013 to 2016. Tinnitus and taste disturbance complications 1 year after myringoplasty were analyzed in relation to gender, age, procedure, and success rate. In database A, physicians reported tinnitus and taste disturbances. In database B, patients reported the complications. RESULTS: A major difference was found when the complications were reported by physicians compared to when the complications were reported by patients. In database A, tinnitus was reported in 1.2% of the patients and taste disturbances in 0.5%. In database B, the frequencies were 12.3% and 11.2%, respectively. Tinnitus and taste disturbances were more frequent after conventional myringoplasty compared to those after fat grafting and were more frequent after primary compared to those after revision surgery when reported by physicians. Patients, however, reported the same frequency of tinnitus after fat graft myringoplasty compared to that after conventional myringoplasty (12.0% vs. 12.6%) and fewer taste disturbances after revision surgery. In follow-up assessments, complications persisted after surgery over a long time period. CONCLUSION: Tinnitus and taste disturbances are more common after myringoplasty when patients report their symptoms than when physicians report the symptoms. LEVEL OF EVIDENCE: 2b Laryngoscope, 129:209-215, 2019.

Prednisolone and valaciclovir in Bell's palsy: a randomised, double-blind, placebo-controlled, multicentre trial.

BACKGROUND: Previous trials of corticosteroid or antiviral treatments for Bell's palsy have been underpowered or have had insufficient follow-up. The aim of this study was to compare the short-term and long-term effects of prednisolone and valaciclovir in the recovery of the affected facial nerve in a large number of patients. METHODS: In this randomised, double-blind, placebo-controlled, multicentre trial, patients aged 18 to 75 years who sought care directly or were referred from emergency departments or general practitioners within 72 h of onset of acute, unilateral, peripheral facial palsy, between May, 2001, and September, 2006, were assessed. Patients were randomly assigned in permuted blocks of eight to receive placebo plus placebo; 60 mg prednisolone per day for 5 days then reduced by 10 mg per day (for a total treatment time of 10 days) plus placebo; 1000 mg valaciclovir three times per day for 7 days plus placebo; or prednisolone (10 days) plus valaciclovir (7 days). Follow-up was for 12 months. The primary outcome event was time to complete recovery of facial function, as assessed with a regional Sunnybrook scale score of 100 points. Analysis was by modified intention to treat. This study is registered with ClinicalTrials.gov, number NCT00510263. FINDINGS: Of 839 patients who were randomly assigned, 829 were included in the modified intention-to-treat analysis: 206 received placebo plus placebo, 210 prednisolone plus placebo, 207 valaciclovir plus placebo, and 206 prednisolone plus valaciclovir. Time to recovery was significantly shorter in the 416 patients who received prednisolone compared with the 413 patients who did not (hazard ratio 1.40, 95% CI 1.18 to 1.64; p<0.0001). There was no difference in time to recovery between the 413 patients treated with valaciclovir and the 416 patients who did not receive valaciclovir (1.01, 0.85 to 1.19; p=0.90). The number of patients with adverse events was similar in all treatment arms. INTERPRETATION: Prednisolone shortened the time to complete recovery in patients with Bell's palsy, whereas valaciclovir did not affect facial recovery.

Stem cells and enhanced healing of chronic tympanic membrane perforation.

CONCLUSIONS: Important information about the basic reparative process of tympanic membrane (TM) healing is shown, which can be incorporated for further clinical understanding. This provides a basis for the exploration of stem cell treatment for TM perforations and holds promise for future improvements. OBJECTIVES: This study aimed to analyse the healing of TM perforation by using stem cells and the stiffness of the membrane was tested in an acute and long-term study. MATERIALS AND METHODS: Sprague-Dawley rats were used in a model of TM perforation. The perforation was performed with a laser system. Stem cells were applied and the healing time and morphological analysis were performed with light and transmission electron microscope. Stiffness was examined by moiré interferometry. RESULTS: The stiffness of the perforated and healed TM was restored after just 2 weeks. In the chronic perforation model, mesenchymal stem cells enhanced the healing.

Absence of autoantibodies connected to autoimmune polyendocrine syndrome type I and II and Addison's disease in girls and women with Turner syndrome.

BACKGROUND: A disturbance in the immune system has been described in Turner syndrome (45,X), with an association to low levels of IgG and IgM and decreased levels of T- and B-lymphocytes. Also different autoimmune diseases have been connected to Turner syndrome (45,X), thyroiditis being the most common. Other autoimmune diseases seen are inflammatory bowel disease, insulin dependent diabetes mellitus, Addison's disease, rheumatoid arthritis, myasthenia gravis, vitiligo, alopecia, pernicious anaemia and hypoparathyroidism, but the association to Turner syndrome is not definite. Besides the typical features of Turner syndrome (short stature, failure to enter puberty spontaneously and infertility due to ovarian insufficiency) ear problems are common. Otitis media and a progressive sensorineural hearing disorder are commonly seen. In the normal population there are known inner ear disorders related to autoimmune diseases. The aim of this study was to investigate patients with Turner syndrome regarding autoantibodies connected to the autoimmune disorders; autoimmune polyendocrine syndrome type I and II and Addison's disease, to screen for overlapping profile of autoantibodies. Blood samples from 110 Turner patients (7-65 years) were investigated using in vitro transcription, translation and immunoprecipitation techniques regarding autoantibodies connected to autoimmune polyendocrine syndrome type I and II and Addison's disease (21-hydroxylase, 17alpha-hydroxylase, side-chain cleavage enzyme, aromatic L-amino acid decarboxylase, tyrosine hydroxylase and tryptophan hydroxylase). RESULTS: The autoantibodies investigated were not overrepresented among the Turner patients. CONCLUSION: The autoimmune disorders associated with Turner syndrome do not seem to be of the same origin as Addison's disease, the type I or II autoimmune polyendocrine syndrome.

Structural and functional properties of the healed tympanic membrane: a long-term follow-up after laser myringotomy.

HYPOTHESIS: The short-term healing scar that forms after experimental laser myringotomy will revert to a normal lamina propria in the long run. The mechanical stiffness will stay normal. BACKGROUND: Recent studies have shown severe structural changes in the fibrous layer in the early course after experimental laser myringotomy, whereas the scar quickly restored the strength of the tympanic membrane (TM). A reorganization of the fiber layer is expected to occur. METHODS: Potassium titanyl phosphate laser myringotomy was made on one side of the TM in Sprague-Dawley rats. The ear of the other side was untouched and used for control. After half a year of observation, the stiffness and strength of the healed TMs were measured with moiré interferometry and examined with otomicroscopy and light and electron microscopy. RESULTS: The interferometry readings showed a slightly reduced strength in the myringotomized and healed TMs. After half a year, still there were immense structural changes including increased thickness over a wide area of the pars tensa with increased amounts of fibers. An obvious reorganization of the fiber layer was lacking. CONCLUSION: Laser myringotomy causes profound, long-standing, or permanent structural changes in the lamina propria of the pars tensa, whereas the strength of the TM may become slightly reduced.

BAHA in children and adolescents with unilateral or bilateral conductive hearing loss: a study of outcome.

OBJECTIVES: Bilateral BAHAs in adults with bilateral hearing loss (BHL) have proven to be superior to unilateral fitting, in both audiologically measurements and in overall patient satisfaction. There have been no similar studies in children. Furthermore, a recent meta-analysis of children with unilateral hearing loss (UHL) has shown numerous negative consequences. The objectives of the study were to investigate whether fitting of bilateral BAHAs in children with conductive BHL give additional hearing benefits, to investigate the effects of unilateral hearing aids in children with conductive UHL, and to identify different aspects of auditory problems in children with conductive UHL or BHL. STUDY DESIGN: This was a prospective study involving 22 children with either conductive UHL (unaided or with unilateral hearing aid) or conductive BHL (with unilateral or bilateral BAHAs) and 15 controls. METHODS: Baseline audiometry, tone thresholds in a sound field, speech recognition in noise and sound localization were tested without, and with unilateral and bilateral hearing aids. Two questionnaires, MAIS & MUSS and IOI-HA, were completed. RESULTS: Two problem areas were identified in the children with hearing impairment: in reactions to sounds and in intelligibility of speech. An additional BAHA in the children with BHL resulted in a tendency to have improved hearing in terms of better sound localization and speech recognition in noise. Fitting of unilateral hearing aids in the children with UHL gave some supplementary benefit in terms of better speech recognition in noise but no positive effect on ability to localize sound could be detected. Even so, all children fitted with hearing aids - either unilaterally or bilaterally - reported a positive outcome with their devices in the self-assessment questionnaire. CONCLUSIONS: Children with either UHL or BHL displayed several problems within the hearing domain. Fitting of bilateral BAHAs in children with BHL and of a single-sided hearing aid in children with UHL appears to have some supplementary audiological benefits and also renders high patient satisfaction. In order to investigate the possible supplementary effects of hearing aids, a 3-month trial of BAHA on Softband, either unilaterally or bilaterally, may be of value in children with conductive UHL or BHL, respectively.