Conductive Hearing Loss In Children Research Articles

PO-0915 Therapeutic Options For Conductive Hearing Loss In Children With Cleft Palate

Background and aim Cleft lip and palate is a common congenital malformation correlated with otological disorders like Eustachian tube dysfunction, otitis media with effusion and conductive hearing loss correlated with speech disorders. Methods In our study we evaluate the therapeutic options for the conductive hearing loss in these children, such as: middle ear ventilation tube insertion, hearing aids and adenoidectomy. We conducted a retrospective chart review on 19 patients who underwent last follow-up during 2013, aged between 1 and 16, affected by cleft palate. We identified 3 cases of Treacher-Collins syndrome, 2 cases of CHARGE syndrome, 2 case sof Pierre Robin, 1 case of Goldhenar and 1 case of Di George syndrome. In 9 cases the cleft palate was isolated. In 14 cases we conducted phoniatric and logopaedic evaluations and 10 of these showed a speech disorder. Results We found a conductive hearing loss in 12 of the 19 children.2 of these patients used hearing aids with an improvementof speech performance; 1 patient underwent adenoidectomy for the appearance of sleep apnea but he modified his quality of voice with hypernasality and nasal emission after surgery; finally 9 children had spontaneous resolution of otitis media with effusion. Conclusions In conclusion we found that the more effective way to resolve the problem of conductive hearing loss and the resulting speech disorder is to use the hearing aids until the resolution of the hearing loss, which normally occurs around 7 years old.

Congenital defects of the middle ear - uncommon cause of pediatric hearing loss

IntroductionIn children, hypoacusis, or conductive hearing loss, is usually acquired; otitis media with effusion is the most common etiology. However, in some cases this condition is congenital, ranging from deformities of the external and middle ear to isolated ossicular chain malformations. The non-ossicular anomalies of the middle ear, for instance, persistent stapedial artery and anomaly of the facial nerve, are uncommon but may accompany the ossicular defects. ObjectiveThis study aimed to describe the clinical presentation, diagnostic tests, and therapeutic options of congenital malformations of the middle ear. MethodsThis was a retrospective study of cases followed in otolaryngologic consultations since 2007 with the diagnosis of congenital malformation of the middle ear according to the Teunissen and Cremers classification. A review of the literature regarding the congenital malformation of the middle ear and its treatment is presented. ConclusionMiddle ear malformations are rarely responsible for conductive hearing loss in children. As a result, there is often a late diagnosis and treatment of these anomalies, which can lead to delays in the development of language and learning.

Gene Mutations in Primary Ciliary Dyskinesia Related to Otitis Media

Otitis media with effusion (OME) is the most common cause of conductive hearing loss in children and is strongly associated with primary ciliary dyskinesia (PCD). Approximately half of the children with PCD require otolaryngology care, posing a major problem in this population. Early diagnosis of PCD is critical in these patients to minimise the collateral damage related to OME. The current gold standard for PCD diagnosis requires determining ciliary structure defects by transmission electron microscopy (TEM) or clearly documenting ciliary dysfunction via digital high-speed video microscopy (DHSV). Although both techniques are useful for PCD diagnosis, they have limitations and need to be supported by new methodologies, including genetic analysis of genes related to PCD. In this article, we review classical and recently associated mutations related to ciliary alterations leading to PCD, which can be useful for early diagnosis of the disease and subsequent early management of OME.

Panel 4: Recent Advances in Otitis Media in Molecular Biology, Biochemistry, Genetics, and Animal Models

Otitis media (OM) is the most common childhood bacterial infection and also the leading cause of conductive hearing loss in children. Currently, there is an urgent need for developing novel therapeutic agents for treating OM based on full understanding of molecular pathogenesis in the areas of molecular biology, biochemistry, genetics, and animal model studies in OM. To provide a state-of-the-art review concerning recent advances in OM in the areas of molecular biology, biochemistry, genetics, and animal model studies and to discuss the future directions of OM studies in these areas. A structured search of the current literature (since June 2007). The authors searched PubMed for published literature in the areas of molecular biology, biochemistry, genetics, and animal model studies in OM. Over the past 4 years, significant progress has been made in the areas of molecular biology, biochemistry, genetics, and animal model studies in OM. These studies brought new insights into our understanding of the molecular and biochemical mechanisms underlying the molecular pathogenesis of OM and helped identify novel therapeutic targets for OM. Our understanding of the molecular pathogenesis of OM has been significantly advanced, particularly in the areas of inflammation, innate immunity, mucus overproduction, mucosal hyperplasia, middle ear and inner ear interaction, genetics, genome sequencing, and animal model studies. Although these studies are still in their experimental stages, they help identify new potential therapeutic targets. Future preclinical and clinical studies will help to translate these exciting experimental research findings into clinical applications.

Conductive hearing loss in children with autism

Infantile autism is a serious comprehensive developmental disorder. The diagnosis of hearing loss or its exclusion, which often suggests suspected autism, is very important for early ENT, psychotherapy, and psychiatric treatment. One hundred children diagnosed with autism aged from 3 to 18 years, with a median age of 5 years, were evaluated. The control group of healthy children consisted of 100 children, aged from 3 to 18 years, with a median age of 6 years. Anamnesis and physical examination, including pediatric assessment and otoscopic examination, were carried out on children in both groups. Each child underwent bilateral otoacoustic emission examination in the 0.7, 1, 2, and 4 kHz bands and impedance audiometry examination. The data obtained were subjected to a basic statistical assessment. Chi2 Pearson's test was used to compare results of tests in both groups. The absence of otoacoustic emission for the 1 and 2 kHz bands was significantly more frequent in the group of autistic children than in the control group. Furthermore, types B and C2 tympanometric curves were significantly more common in the group of autistic children than in the group of healthy children.

Wideband aural acoustic absorbance predicts conductive hearing loss in children

Objective: This study tested the hypothesis that wideband aural absorbance predicts conductive hearing loss (CHL) in children medically classified as having otitis media with effusion. Design: Absorbance was measured in the ear canal over frequencies from 0.25 to 8 kHz at ambient pressure or as a swept tympanogram. CHL was defined using criterion air-bone gaps of 20, 25, and 30 dB at octaves from 0.25 to 4 kHz. A likelihood-ratio predictor of CHL was constructed across frequency for ambient absorbance, and across frequency and pressure for absorbance tympanometry. Performance was evaluated at individual frequencies and for any frequency at which a CHL was present. Study sample: Absorbance and conventional 0.226-kHz tympanograms were measured in children of age three to eight years with CHL and with normal hearing. Results: Absorbance was smaller at frequencies above 0.7 kHz in the CHL group than the control group. Based on the area under the receiver operating characteristic curve, wideband absorbance in ambient and tympanometric tests were significantly better predictors of CHL than tympanometric width, the best 0.226-kHz predictor. Accuracies of ambient and tympanometric wideband absorbance did not differ. Conclusions: Absorbance accurately predicted CHL in children and was more accurate than conventional 0.226-kHz tympanometry.

Phosphodiesterase 4B Mediates Extracellular Signal-regulated Kinase-dependent Up-regulation of Mucin MUC5AC Protein by Streptococcus pneumoniae by Inhibiting cAMP-protein Kinase A-dependent MKP-1 Phosphatase Pathway

Otitis media (OM) is the most common childhood bacterial infection and the major cause of conductive hearing loss in children. Mucus overproduction is a hallmark of OM. Streptococcus pneumoniae is the most common gram-positive bacterial pathogen causing OM. Among many mucin genes, MUC5AC has been found to be greatly up-regulated in the middle ear mucosa of human patients with OM. We previously reported that S. pneumoniae up-regulates MUC5AC expression in a MAPK ERK-dependent manner. We also found that MAPK phosphatase-1 (MKP-1) negatively regulates S. pneumoniae-induced ERK-dependent MUC5AC up-regulation. Therapeutic strategies for up-regulating the expression of negative regulators such as MKP-1 may have significant therapeutic potential for treating mucus overproduction in OM. However, the underlying molecular mechanism by which MKP-1 expression is negatively regulated during S. pneumoniae infection is unknown. In this study we show that phosphodiesterase 4B (PDE4B) mediates S. pneumoniae-induced MUC5AC up-regulation by inhibiting the expression of a negative regulator MKP-1, which in turn leads to enhanced MAPK ERK activation and subsequent up-regulation of MUC5AC. PDE4B inhibits MKP-1 expression in a cAMP-PKA-dependent manner. PDE4-specific inhibitor rolipram inhibits S. pneumoniae-induced MUC5AC up-regulation both in vitro and in vivo. Moreover, we show that PDE4B plays a critical role in MUC5AC induction. Finally, topical and post-infection administration of rolipram into the middle ear potently inhibited S. pneumoniae-induced MUC5AC up-regulation. Collectively, these data demonstrate that PDE4B mediates ERK-dependent up-regulation of mucin MUC5AC by S. pneumoniae by inhibiting cAMP-PKA-dependent MKP-1 pathway. This study may lead to novel therapeutic strategy for inhibiting mucus overproduction.

Conductive hearing loss in children with bronchopulmonary dysplasia: A longitudinal follow-up study in children aged between 6 and 24 months

Aims To determine the occurrence of isolated and recurrent episodes of conductive hearing loss (CHL) during the first two years of life in very low birth weight (VLBW) infants with and without bronchopulmonary dysplasia (BPD). Study design, subjects and outcome measures In a longitudinal clinical study, 187 children were evaluated at 6, 9, 12, 15 18 and 24 months of age by visual reinforcement audiometry, tympanometry and auditory brain response system. Results Of the children with BPD, 54.5% presented with episodes of CHL, as opposed to 34.7% of the children without BPD. This difference was found to be statistically significant. The recurrent or persistent episodes were more frequent among children with BPD (25.7%) than among those without BPD (8.3%). The independent variables that contributed to this finding were small for gestational age and a 5 min Apgar score. Conclusions Recurrent CHL episodes are more frequent among VLBW infants with BPD than among VLBW infants without BPD.

Inner Ear Anomalies and Conductive Hearing Loss in Children With Apert Syndrome

To identify the occurrence of inner ear structural anomalies and conductive hearing loss (CHL) in children with Apert syndrome. Retrospective review. Pediatric tertiary referral center. Twenty pediatric patients with Apert syndrome were found; all patients (38/40 ears) had inner ear anomalies. Computerized tomography of the head/temporal bone, pure-tone (including air and bone conduction) audiometry, and tympanometry. Imaging demonstrating inner ear anomalies, including malformations of the cochlea, dilated vestibule, and/or semicircular canal; audiologic findings of air-bone gap(s). Hearing loss was found in 90% of the patients with Apert syndrome, and 80% of them had CHL. Air-bone gaps were found at all frequencies, with larger gaps at low frequencies. Fifty percent (20/40) of the ears had better than 0 dB hearing level bone conduction thresholds at 250 and/or 500 Hz. Normal middle ear pressure and mobility were found in all ears with intact eardrum. Inner ear anomalies were found in all patients, and 90% of them had bilateral involvement. Most frequently observed inner ear anomalies were dilated vestibule, malformed lateral semicircular canal, and cochlear dysplasia. Children with Apert syndrome may present with significant CHL that cannot be explained by minor middle ear pathologies alone. This conductive loss may be, at least partially, attributed to the inner ear anomalies; however, these structural anomalies are usually not recognized in these patients. Failure to close air-bone gap after surgical intervention may raise the suspicion of inner ear anomalies, and computed tomographic scan of the temporal bone can provide definitive proof.

Inner Ear Anomalies and Conductive Hearing Loss in Children With Apert Syndrome

Inner Ear Anomalies and Conductive Hearing Loss in Children With Apert Syndrome

Effect of Early Otitis Media on Speech Identification

Otitis media (OM) is the most common cause of conductive hearing loss in children in India. Hearing loss secondary to OM has been reported to result in deficits in auditory processing. It was hypothesised that such deficits can be more deleterious if OM occurs in the first year of life as there is maximum development of auditory neural pathways during this period. Therefore, the purpose of this study was to document the effects of reduced auditory experience secondary to OM in the first year of life on speech identification. Another purpose was to study the persistence of the negative effects of auditory deprivation on speech perception. A cross-sectional approach and a standard group comparison design was used in the study. Twenty one children, aged 5.1 years to 6.6 years, who had OM between 6 months and 12 months of age participated in the study along with age and gender matched normal children. Speech identification scores were obtained from these children for natural words, spectrally distorted, and time compressed words. Results showed that the children with early OM had significantly poorer speech identification scores for spectrally and temporally distorted words compared to normal children. There was no significant difference in the speech identification scores for natural speech between the two groups. These results have important implications for the way the hearing needs of children with early onset OM are taken care of in the classrooms of schools in India, and perhaps other developing countries.

Fully implantable hearing device as a new treatment of conductive hearing loss in Franceschetti syndrome

The objective is to investigate a new therapeutic option for treatment of conductive hearing loss in children with Franceschetti syndrome. A Carina fully implantable device with the MET V transducer for conductive applications was implanted in a 14-year-old male suffering from bilateral conductive hearing loss (mean PTA loss: 70 dB on the right ear and 64 dB on the left ear) secondary to Franceschetti (a.k.a Treacher Collins) syndrome with bilateral agenesis of external ear canal and ossicular malformation. Postoperative unaided PTA was unchanged by the surgical procedure. When the implant was activated, mean PTA was 29 dB improved on the implanted ear. The capacity of the Carina MET ossicular stimulator to provide appropriate gain relative to the degree of hearing loss indicates that the device may be a viable treatment for children with agenesis of external auditory canal and severe malformation of the middle ear. However, these initial results need to be furthered by: (1) additional studies including a greater number of patients to confirm these preliminary results and (2) a long-term follow-up must be carried out to detect possible long-term cochlear adverse effects, in particular on the basilar membrane.

Congenital conductive hearing loss: Imaging and treatment

Objectives: Conductive hearing loss in children is most often due to nonossicular problems, such as middle ear fluid or cholesteatoma. Hearing loss, however, can also be caused by congenital abnormalities of the conductive mechanism. This study evaluates the operative findings, results, and the role of imaging in children with congenital conductive hearing loss. Methods: A retrospective case review was performed at an academic medical center. Children with hearing loss due to trauma, chronic infections, or external auditory canal malformations were excluded. Twenty-five cases were included. Preoperative air and bone pure tone and speech thresholds were compared with 1-year postoperative hearing results. All preoperative computed tomography (CT) scans were reviewed, and the incidence of preoperative CT defined abnormalities was reported. Results: The most common causes of conductive hearing loss were malleus fixation (33%) and stapes fixation (33%). Fifty percent of abnormalities were identified on preoperative CT scan. Postoperative hearing results were mixed with only 30% of cases achieving a PTA of less than 30 dB. Fifty-five percent of cases had an improvement in postoperative air bone gap by 10 dB from preoperative levels. Cases of malleus fixation had better results overall, with 60% of these achieving a postoperative PTA of less than 30 dB and 60% improving by greater than 10 dB in postoperative air bone gap. Conclusions: CT scan is a useful diagnostic test in children with congenital conductive hearing loss. While surgical intervention overall has mixed results, isolated malleus fixation has the best prognosis for hearing improvement.

Simulated conductive hearing loss in children.

Otitis media with effusion (OME) often results in hearing loss for children with the condition. In order to provide appropriate and effective audiologic management, it is important to understand the impact of OME on speech recognition ability when hearing loss is present. This study examined the speech recognition abilities of normal-hearing six- and seven-year-old children (n = 12) and adults (n = 12) using monosyllabic words and nonsense syllables presented at two levels of simulated conductive hearing loss characteristic of OME. Average speech recognition scores decreased as the degree of simulated conductive hearing loss increased. Both age groups scored significantly poorer for nonsense syllables than for monosyllabic words. In general, the children performed more poorly than the adults with the exception of the easiest listening condition for word stimuli. Furthermore, children appeared less able than adults to use their knowledge of familiar words to improve performance. These findings suggest that rehabilitative strategies may best be focused on combining familiarization techniques and amplification options.

Non‐Otitis Conductive Hearing Loss in Children

Non‐Otitis Conductive Hearing Loss in Children

Correction of Conductive Hearing Loss in Children

The focus of this article is the diagnosis and surgical correction of conductive hearing loss in children without aural atresia, cholesteatoma, or otitis media with effusion. An overview of the incidence of congenital conductive hearing loss and the types of middle ear anomalies are provided. Techniques of surgical correction are described with respect to the types of ossicular and middle ear abnormalities encountered for both congenital and acquired conductive hearing loss, including otosclerosis.

Additional conductive hearing loss in children from a school for the deaf in Malaysia

A total of 165 children from a school for the deaf in Malaysia were screened to find out the prevalence of additional conductive hearing loss. Otological examination, tympanometry and pure tone audiometry were performed in all these children. Fifty-one children (30.9%) had additional conductive hearing loss. Middle ear disorders were present in 15 children (9.09%). The deaf children seldom complain about the change in their hearing sensitivity, so there is a need for regular otological examination in deaf children to detect the additional conductive hearing loss.

Hearing loss and temporal bone structure in achondroplasia.

Characteristic temporal bone changes have recently been defined by high resolution CT in nine patients with achondroplasia (Cobb et al., Am J Neuroradiol 9:1195, 1988). These included narrowing of the skull base and "towering" petrous ridges resulting in abnormal orientation of the inner and middle ear structures. In order to determine whether these morphologic changes are the cause of the hearing deficit in achondroplasia, audiometric studies and ENT evaluation were performed in eight of the nine patients. All had a history of frequent otitis media and four had experienced tympanic membrane tube insertion. Three patients had significant sensorineural hearing loss, two had conductive hearing loss and one patient had combined hearing loss. None of the temporal bone morphologic changes were found to be correlated with the degree of either sensorineural or conductive hearing loss. Fusion of the ossicular chain was not present in any of our cases. Appropriate treatment of frequent acute otitis media and early awareness of middle ear effusions and conductive hearing loss in children with achondroplasia may be of great importance in preventing permanent hearing loss.

Auditory brainstem evoked potentials in evaluating the efficacy of surgical ventilation of the middle ear

Conductive hearing loss in children is most commonly associated with Secretory otitis media (S.O.M.). Since auditory brainstem evoked potentials (ABEP) reflect conductive damage in prolonged peak latencies, the present study evaluated the efficacy of surgery for ventilating the middle ear, by comparing changes in peak latencies. These changes were compared in 4 groups of children (4-12 years old): children with S.O.M. in the past, who recovered as a result of conservative treatment; children with S.O.M. in the past, who had been operated on with insertion of ventilation tubes (V.T.); children with S.O.M. in the past, who had been operated on and who still had V.T. at the time of study, and a control group with healthy ears. In all, 183 ears were tested. The results indicate that there is no significant audiometric and electrophysiological difference between the groups who recovered with or without V.T.

Learning disabilities and conductive hearing loss involving otitis media.

This article examines the relationship between mild conductive hearing loss associated with otitis media and auditory, language, and academic performance of children. The nature of conductive hearing loss in children is explained. A review or research studies focuses on the following topics: (1) prevalence of otitis media among learning disabled children, (2) effects of early-onset, persistent otitis media on auditory, language, and academic skills of children, and (3) effects of early onset, resolved otitis media on auditory, language, and academic skills of children. Suggestions are made for identifying children who may be at risk for developing psychoeducational sequelae. In addition, approaches to intervention with at-risk children and directions for future research are presented.