Cochlear ossification in patients with profound hearing loss following bacterial meningitis

The suitability of an air-exposed culture model consisting of a collagen matrix was investigated for constructing an advancing front (AF) of human middle ear epithelium (MEE) and meatal epidermis (ME). Three different culture settings were used: ( i ) MEE; ( ii ) ME; and ( iii ) AF (MEE+ME). Small tissue biopsies were placed on a fibroblast-populated collagen matrix and grown at the air-liquid interface. After 3 weeks of culture, the MEE and ME outgrowth was differentiated. Light, scanning electron and transmission electron microscopy showed no visible differences compared to native MEE and ME. Cytokeratin 8 and cytokeratin 10 expressions were comparable to the expression seen in the native MEE and ME tissues. Proliferation, which was demonstrated by the expression of Ki-67, was present in the basal layers of cultured MEE and ME. A double layer of cells in which the ME covered the MEE formed the AF. In the AF, the MEE and ME showed the same morphological and immunohistochemical characteristics as in their native tissues. The results of the study show that this in vitro system is a well-defined model system offering the possibility to study the effects of external stimuli on the different epithelia of the AF involved in the pathogenesis of cholesteatoma.     

Role of tumor necrosis factor-alpha in sensorineural hearing loss after bacterial meningitis.

HYPOTHESIS: Blockade of tumor necrosis factor-alpha with tumor necrosis factor-alpha antibody will reduce the extent of cochlear injury and hearing loss associated with Streptococcus pneumoniae meningitis. BACKGROUND: Inflammatory mediators play a significant role in the morbidity associated with bacterial meningitis, including hearing loss and labyrinthitis ossificans. Previous studies have shown the attenuation of hearing loss by the nonspecific blockade of such pathways. METHODS: Fifty Mongolian gerbils were divided into four groups. Auditory brainstem response testing was conducted to measure hearing thresholds. Streptococcus pneumoniae meningitis was induced in Groups 1 and 2. Group 2 was then given a single intraperitoneal injection of tumor necrosis factor-alpha antibody, whereas Group 1 received phosphate-buffered saline. Uninfected animals in Groups 3 and 4 were implanted with osmotic pumps that delivered a continuous 8-day intrathecal flow of either tumor necrosis factor-alpha (Group 4) or phosphate-buffered saline (Group 3). After 6 weeks, auditory brainstem response testing was repeated. The cochleas were harvested and analyzed histomorphometrically. RESULTS: Group 2 animals with Streptococcus pneumoniae meningitis that also received tumor necrosis factor-alpha antibody developed significantly less hearing loss than Group 1 animals with meningitis alone. The decrease in the average threshold at 4, 8, 16, and 32 kHz was 31, 30, 25, and 28 dB sound pressure level, respectively (p < 0.0092 for each). Furthermore, histomorphometric analysis showed significantly less damage to the organ of Corti, spiral ganglion, spiral ligament, and stria vascularis in Group 2. Conversely, tumor necrosis factor-alpha induced meningitis animals (Group 3) showed increased hearing loss compared with phosphate-buffered saline controls (Group 4), with p < 0.0001 at all frequencies. CONCLUSION: Tumor necrosis factor-alpha plays an important role in cochlear injury after bacterial meningitis. Blockade of tumor necrosis factor-alpha reduces postmeningitic hearing loss and cochlear injury. Induction of meningitis with intrathecal tumor necrosis factor-alpha also resulted in hearing loss and cochlear injury similar to bacterial meningitis.     

婴幼儿化脓性脑膜炎听力损失及转归特点分析

目的探讨婴幼儿化脓性脑膜炎导致听力下降情况及听力下降的转归情况。方法回顾性分析自2012年7月至2013年3月我院收治的化脓性脑膜炎患儿66例,全部患儿行脑干测听检查。同时回顾性分析了患儿的影像学检查,如颞骨CT,头部核磁,增强核磁等。听力异常者三个月到半年复查脑干测听检查。结果入组的化脓性脑膜炎患儿66例,30例患儿出现听力下降(45.5%),其中21例患儿进行了听力学随访,共10例(47.6%)患儿听力下降未恢复或未完全恢复。此21例患儿中轻度听力下降3例,随访听力全部恢复正常;中度及中重度听力下降9例,7人恢复正常,2人听力未恢复;重度及极重度听力下降9人,1人听力恢复,2人听力部分恢复,余6人听力未恢复。结论化脓性脑膜炎对婴幼儿听力影响明显;合并听力下降的程度与听力损失恢复程度明显相关,轻度听力下降听力多可恢复,听力下降程度较重,则可能导致永久性听力损失。     

Angiostrongylus eosinophilic meningitis associated with sensorineural hearing loss

A 59-year-old woman who presented with chronic headache, neck stiffness and left-sided hearing loss is reported. The diagnosis of angiostrongylus eosinophilic menigitis was made. The patient improved after treatment with prednisolone, including hearing. Angiostrongylus eosinophilic meningitis associated with sensorineural hearing loss has not previously been reported.     

Risk factors for hearing loss from meningitis in children: the Children's Hospital experience.

OBJECTIVES: To identify statistically significant risk factors for hearing loss in children with meningitis, determine the overall incidence of hearing loss in a large group of children with confirmed meningitis, and quantify the percentage of children with progressive or fluctuating hearing loss after meningitis. DESIGN: Retrospective analysis. PATIENTS AND OTHER PARTICIPANTS: Four hundred thirty-two children admitted to the Children's Hospital, Birmingham, Ala, from January 1, 1985, to December 31, 1995, with the diagnosis of meningitis. RESULTS: Of 432 children with meningitis, 59 (13.7%) had the development of hearing loss. Of these 59 children, 46 (78.0%) had stable sensorineural hearing loss and 13 (22.0%) had either progressive or fluctuating hearing loss. Of the variables examined using multiple logistic regression backward-elimination modeling, only 5 appeared to be significantly associated with the development of hearing loss: computed tomographic scan evidence of increased intracranial pressure (estimated odds ratio [OR] = 2.3), male sex (OR= 1.9), the common logarithm of glucose levels in the cerebrospinal fluid (OR = 0.58), Streptococcus pneumoniae as the causative organism (OR= 2.1), and the presence of nuchal rigidity (OR = 1.9). In the children with progressive hearing loss, the time for progression varied from 3 months to 4 years before hearing stabilized. CONCLUSIONS: In this study of children diagnosed as having meningitis, hearing loss developed in 59 (13.7%). Forty-six (78.0%) of these children with hearing loss had stable auditory thresholds over time, and 13 (22.0%) exhibited deterioration or fluctuation of acuity over time. Evidence of increased intracranial pressure by computed tomographic scan, male sex, low glucose levels in the patients' cerebrospinal fluid, S pneumoniae as the causative organism, and the presence of nuchal rigidity appear to be significant predictors for future hearing loss.     

Magnetic resonance imaging in patients with meningitis induced hearing loss

The aim of this multicentre study was to compare T1 with T2 weighted MRI scans of the labyrinth after meningitis and to investigate whether waiting with scanning improved the reliability of diagnosing an ongoing process such as cochlear osteogenesis. Forty-five patients were included who suffered from meningitis induced hearing loss (radiological imaging <1 year after meningitis). Twenty-one gadolinium enhanced T1 and 45 T2 weighted MRI scans were scored by two radiologists regarding the condition of the labyrinth. These radiological observations were compared with the condition of the cochlea as described during cochlear implantation. A higher percentage of agreement with surgery was found for T2 (both radiologists 73%) than for T1 weighted MRI scans (radiologist 1: 62%, radiologist 2: 67%), but this difference is not significant. There was no significant difference between early (0–3 months) and late (>3 months) scanning, showing that radiological imaging soon after meningitis allows early diagnosis without suffering from a lower agreement with surgical findings.     

Experience using antihypoxic drugs in the treatment of acute neurosensory hearing loss in children with meningitis

This paper describes beneficial affects of the therapy of neurosensory hypoacusia that complicated such neuroinfectious diseases as meningococcemia, meningococcic meningitis and meningitides of other etiology using traditional drugs (ATP, aloe, B and C vitamins) in combination with the antihypoxic agent gutimine and its analog. Positive effects were recorded in 17 (81%) out of 21 children. It is indicated that the agent shows higher efficacy when used within the first weeks after the onset of the disease.     

Clinical features of functional hearing loss with inattention problem in Japanese children

ObjectiveFunctional hearing loss is a condition in which hearing is lost without actual pathology. In children, inattention during pure tone audiometry may be due in part to functional hearing loss. This study examined the issue of inattention as a psychological trait by analyzing the clinical features of functional hearing loss children in Japan.MethodsUsing the ADHD-Rating Scale IV, 97 functional hearing loss children were screened for inattention (27 boys, 70 girls; mean age 9 years 5 months, range 5–17 years). Those with high levels of inattention (Inattention group) were compared with others (Attention group) for clinical features statistically. Furthermore observed psychological clinical features were described.Results36.1% were categorized in the Inattention group, which had more boys, lower in age, and had more physical, developmental, and environmental problems than the Attention group. Two groups had very different psychological feature.ConclusionsIn children's functional hearing loss, there exists a group with psychological trait of inattention. Three younger children 5–6 years old with attention problems showed no psychological problems, their FHL was considered to be caused by generalized maturation and development. Nearly all of the rest children showed psychological problems, supporting the notion that FHL in children is psychogenic in nature. Because clinical features in Inattention group children were different from the Attention group significantly, it was concluded that distinguishing the Inattention group as a subtype of functional hearing loss in children would be effective for both diagnosis and treatment. Larger scale studies with many angles needed for the inattention problem in FHL children.     

Delayed deterioration of hearing following bacterial meningitis

Bacterial meningitis is an important cause of acquired sensorineural deafness in childhood. Deafness following meningitis may be progressive. Previous reports have shown deterioration in hearing up to 12 years after the illness. We present two cases of sensorineural deafness following meningitis. Severe to profound sensorineural hearing losses were detected immediately after meningitis in these patients. The hearing subsequently deteriorated in both cases. Deterioration in hearing thresholds occurred 17 years after the illness in one case. In the other patient the hearing got progressively worse three years after meningitis. She subsequently required a cochlear implant.     

Novel-word learning in children with normal hearing and hearing loss

OBJECTIVE: The goal of this study was to assess performance on a novel-word learning task by normal-hearing and hearing-impaired children for words varying in form (noun versus verb), stimulus level (50 versus 60 dB SPL), and number of repetitions (4 versus 6). It was hypothesized that novel-word learning would be significantly poorer in the subjects with hearing loss, would increase with both level and repetition, and would be better for nouns than verbs. DESIGN: Twenty normal-hearing and 11 hearing-impaired children (6 to 9 yr old) participated in this study. Each child viewed a 4-minute animated slide show containing 8 novel words. The effects of hearing status, word form, repetition, and stimulus level were examined systematically. The influence of audibility, word recognition, chronological age, and lexical development also were evaluated. After hearing the story twice, children were asked to identify each word from a set of four pictures. RESULTS: Overall performance was 60% for the normal-hearing children and 41% for the children with hearing loss. Significant predictors of performance were PPVT raw scores, hearing status, stimulus level, and repetitions. The variables age, audibility, word recognition scores, and word form were not significant predictors. CONCLUSIONS: Results suggest that a child's ability to learn new words can be predicted from vocabulary size, stimulus level, number of exposures, and hearing status. Further, the sensitivity to presentation level observed in this novel-word learning task suggests that this type of paradigm may be an effective tool for studying various forms of hearing aid signal processing algorithms.     

Sudden bilateral hearing loss and meningitis in adults

Sudden profound bilateral sensorineural hearing loss is a serious complication of bacterial meningitis. With high dosage Dexamethasone it is possible to halt, and, in some instances, to reverse the progression of the hearing loss. The efficacy of treatment depends upon early diagnosis. Hearing may worsen upon steroid cessation and low dosage oral steroid may be required for several months. The eventual hearing loss may be considerably worse than anticipated. Two cases illustrating differing aspects of the disease are described.     

Progressive hearing loss following Haemophilus influenzae meningitis

Loss of hearing is a sequelae of meningitis. This hearing loss has been known to fluctuate for as long as one year following the acute infection. This paper presents a case of an 11-year documented downward fluctuating hearing loss following Haemophilus influenzae meningitis. The case indicates that patients should be followed audiologically for years following meningitis. A review of the literature of hearing loss in meningitis is presented with special emphasis on cases in which the hearing loss fluctuated. The cases in the literature were usually not followed for more than one year. In this case of acquired hearing loss secondary to Haemophilus influenzae meningitis, the patient's hearing fluctuated over 11 years, in a downward progression. It is not clear what type of mechanism is involved in the ongoing damage to the cochlea that could account for this gradual fluctuation. It is suggested that as the possibility of long-term fluctuation exists, patients with meningitis should be monitored audiologically for many years.     

Amplification considerations for children with minimal or mild bilateral hearing loss and unilateral hearing loss

Children with minimal or mild bilateral hearing loss and unilateral hearing loss are at higher risk for academic, speech-language, and social-emotional difficulties than their normal hearing peers. The choice to fit infants with moderate or greater degrees of bilateral hearing loss has been standard practice for most clinicians, but for those with minimal or mild bilateral hearing loss or unilateral hearing loss, the fitting of hearing technology must be based on limited data. Evidence does not yet exist to support all the management decisions that an audiologist must make upon identifying an infant with minimal or mild bilateral hearing loss or unilateral hearing loss. It is not yet known which children are at the greatest risk for educational problems nor is it known if the provision of early amplification in this population will help a child avoid later difficulties. Some of these considerations and current hearing technology options for children with minimal or mild bilateral hearing loss or unilateral hearing loss are reviewed in this article.     

Functional hearing loss in children

This report reviewed 39 school-age children diagnosed as having a functional hearing loss utilizing auditory brainstem response (ABR) audiometry during the past 5 years at the Department of Otolaryngology, Kyushu University Hospital in Japan. Twenty-seven cases were females and 12 were males. Seven cases had a hearing loss unilaterally and 32 bilaterally. Although pure-tone audiometry revealed a variety of audiogram shapes, two-thirds of the cases had a flat or saucer-shaped audiogram with a mild to moderately severe hearing loss. ABR audiometry for the frequencies of 1, 2 and 4 kHz indicated a normal hearing threshold in 65 ears of 35 patients, and mild threshold elevations of at least one frequency in the remaining 6 ears of 4 patients. Three illustrative cases were demonstrated, and a discussion was held regarding the features in audiometric tests, and environmental factors surrounding the children with this condition. We emphasized that the physiological hearing measurement such as ABR audiometry should be performed when any discrepancy was noted between the patient's history and results of pure-tone audiometry, because of not infrequent occurrence of functional hearing loss.     

Unilateral hearing loss in children

Recent reports suggest that early onset, severe unilateral sensorineural hearing loss (USNHL) in children may be associated with significant deficits in auditory and psycholinguistic skills and school performance. This report reviews a consecutive series of 324 children and adolescents (202 males, 122 females) with documented USNHL evaluated at the Boys Town National Research Hospital. The left ear was affected in 168 (52%) and the right ear in 156 (48%). Based on speech frequency threshold averages (i.e., 500, 1000, and 2000 Hz), the losses were classified by severity as follows: borderline, 43 (13%); mild, 51 (16%); moderate, 40 (12%); severe, 19 (6%); profound, 31 (10%), and anacusic, 50 (15%). The remaining 90 children (28%) had high frequency losses (i.e, above 2000 Hz). The mean and median age of diagnosis were 8.78 years and 7.75 years. Temporal bone imaging studies, auditory brainstem responses (ABRs), and vestibular evaluations were performed on selected cases. Etiology was uncertain in 34.8% of cases, while hereditary factors (12.6%), head trauma (10.8%), and perinatal risk factors (10.7%) were the most commonly identified etiologies. Thirty-one percent of the children had scholastic or behavioral problems in school. A concerted effort aimed at early identification and intervention in cases of USNHL is warranted.     

Functional hearing loss in children

The cases of children diagnosed with pseudohypacusis in the Department of Pediatric Otolaryngology were presented. Probable mechanism of its pathogenesis was described. The main stress was put on its correct diagnosis particularly in children with co-existing organic changes. Diagnosis of pseudohypacusis in children is not problematic provided that the occurence of this disease is taken into consideration during diagnostic procedures.     

Sensorineural hearing loss in children

Sensorineural hearing loss in children, either congenital or acquired, has an incidence of 2-4 per million. Molecular diagnosis of early childhood deafness became available for some types of syndromal and non-syndromal forms and will offer different treatment modalities in the future. Severe to profound sensorineural hearing loss can be effectively treated with cochlear implants. There is evidence of cerebral auditory plasticity under electrical stimulation of the auditory nerve with better performance in early implanted children. Other predicting factors are related to the type of schooling, family support and residual hearing. In the long-term, prelingually deafened children will develop considerable speech perception and production. Children with marginal benefit from hearing aid amplification show significant improvements in speech perception following implantation. Implantation is also possible in cases of cochlear malformation. However, special attention has to be given to the facial nerve, a possible CSF leak and electrode misplacement. Apart from hearing improvement cochlear implants have a positive impact on the family situation, schooling and personal well-being.     

儿童分泌性中耳炎相关骨导听力下降的临床分析

目的：分析儿童分泌性中耳炎相关的骨导听力下降的病因、诊断和治疗方法。方法：回顾性分析150例（225耳）分泌性中耳炎儿童中35例（37耳）骨导听力下降的临床资料。结果：35例患儿均给予鼓膜切开置管或（和）鼻内镜下腺样体切除术,术后给予药物治疗。34例患儿骨导听阈恢复正常,1例患儿随访6个月改善不明显。结论：35例（23．3％）患儿的暂时性听阈移位或永久性听阈移位的发病机制与分泌性中耳炎有关。儿童分泌性中耳炎的发病病程中有发展成骨导听力下降的可能,应引起高度重视,及早干预避免病情发展。