Impaired Binaural Hearing in Children Produced by a Threshold Level of Middle Ear Disease

Otitis media with effusion (OME), a form of middle ear disease, is the most common reason for young children both to visit their family doctor and to have surgery. Almost all children have at least a single episode of OME before their first birthday and annual incidence rates exceed 50% in each of the first five years. For most children, OME occurs infrequently, but about 10–15% of children have OME during more than half of their first six years. Middle ear effusions attenuate and delay sound, causing conductive sound distortion during the crucial years for language acquisition. The many studies of OME effects on language and other indices of development have produced mixed results. However, a consensus is emerging of mild language impairment in the preschool years, with subsequent performance, emotional, and behavioral difficulties. In addition to the peripheral hearing loss produced directly by the disease, binaural and other central auditory deficits can outlive the OME. It has been unclear which children are at risk of central impairment following OME, since the children studied have generally been recruited from otolaryngology clinics. Consequently, a detailed prospective history of the middle ear status of participants has not been available. By studying six-year-old children with a lifetime known history of OME, we show in this study that only those children with a cumulative OME experience of more than about half the time during the first five years consistently have residual impaired binaural hearing.     

Early otitis media with effusion, hearing loss, and auditory processes at school age

OBJECTIVES: To examine the effect of conductive hearing loss (HL) secondary to otitis media with effusion (OME) in the first 3 years of life on physiologic, peripheral, and higher-order behavioral auditory measures examined at school age. METHODS: Peripheral hearing sensitivity for conventional and extended high-frequency audiometric ranges, physiologic (distortion product otoacoustic emissions, contralateral and ipsilateral acoustic middle ear muscle reflexes), auditory brain stem response (ABR), and higher-order auditory processing measures (masking level difference; Virtual Auditory Localization, Speech Intelligibility Gain; adaptive Pediatric Speech Intelligibility task) were examined at the end of the second grade of elementary school in two cohorts (North Carolina, N = 73, and New York, N = 59). All participants (mean age, 8 years) were followed prospectively in infancy and early childhood (7 to 39 months) for middle ear status and hearing loss (using pneumatic otoscopy/tympanometry and repeated conditioned behavioral audiometric response procedures). Multivariate analyses were conducted to address whether early OME and early conductive HL were related to physiologic, peripheral, and higher-order auditory processes. RESULTS: Early hearing loss and OME were significantly associated with peripheral hearing at school age; extended high-frequency thresholds accounted for the result. Similarly, hearing loss in early life and OME were significantly associated with the acoustic middle ear muscle reflex: The contralateral stimulation condition accounted for the association. Significant associations with both early OME and early HL were also found for the auditory brain stem response measure and were explained by the correlations between early hearing loss and the ABR Wave V latency but not other ABR indices. There were no reliable associations between either early OME or early HL on any other auditory processes evaluated at the end of second grade. CONCLUSIONS: Extended high-frequency hearing and brain stem auditory pathway measures in childhood were significantly associated with children's experiences with OME and hearing loss from 7 to 39 months of age. However, no significant associations were found for psychoacoustic measures of binaural processing or a behavioral adaptive speech-in-noise test at school age.     

Effects of otitis media with effusion on auditory temporal resolution

OBJECTIVE: There is mounting evidence to suggest that otitis media with effusion (OME) is associated with auditory processing deficits that persist beyond the resolution of the peripheral hearing loss. This study investigated the residual effects of OME on auditory temporal resolution. EXPERIMENT 1: METHODS: Experiment 1 measured detection thresholds for a brief tone presented either before (backward masking) or during (simultaneous masking) a masking noise, in 6- and 8-year-old children. Six-year-olds were selected from a prospectively studied group with a lifetime known history of OME. Eight-year-old children, with a retrospectively determined history of OME, were also recruited. All children were free of OME at the time of testing. RESULTS: Regardless of OME history, 6-year-old children had similar tone thresholds on all masking tasks. In contrast, 8-year-olds with a history of recurrent OME had 18 and 4dB higher mean thresholds for the backward and simultaneous masking conditions, respectively, compared with age-matched controls. Possible explanations for these results included (i) recruitment bias, rather than OME, contributed to differences in auditory processing abilities amongst 8-year-old children, or (ii) OME impaired performance at both ages, but this was not seen in 6-year-olds due to 'ceiling' effects. EXPERIMENT 2: METHODS: To distinguish between these possibilities, Experiment 2 measured temporal resolution, using backward masking and amplitude modulation detection, in the prospectively studied group of children when they were 8 years old. RESULTS: Regardless of OME history, these 8-year-olds had similar auditory temporal processing abilities. Results from Experiment 2 suggested that recruitment bias was the most likely explanation for the difference in auditory processing abilities between 8-year-old children with and without a history of OME found in Experiment 1. Consistent with previous data, associations were found between backward masking, age and cognitive ability. CONCLUSION: There is no evidence to suggest that OME effects temporal resolution after the recovery of normal pure-tone thresholds.     

Auditory processing abilities in children with chronic otitis media with effusion

Conclusion The study results indicate that children with a history of otitis media with effusion (OME) suffer from auditory processing disorder to some degree. The findings support the hypothesis that fluctuating hearing loss may affect central auditory processing during critical periods.

Objectives Evidence suggests that prolonged OME in children can result in an auditory processing disorder, presumably because hearing has been disrupted during an important developmental period. A lack of auditory stimulation leads to the abnormal development of the hearing pathways in the brain. The aim of the present study was to determine the effects of OME on binaural auditory function and auditory temporal processing.

Method In the present study, the dichotic digit test (DDT) was used for binaural hearing, and the gap in noise (GIN) test was used to evaluate temporal hearing processing.

Results The average values of GIN differed significantly between children with a history of OME and normal controls (p?p?=?0.002).     

Failed newborn hearing screens as presentation for otitis media with effusion in the newborn population

OBJECTIVES: Evaluate the prevalence of middle ear disease in infants failing a newborn hearing screening program. Review the outcomes of those infants diagnosed with or without middle ear disease after failed hearing screen. DESIGN: Retrospective chart review of 76 patients referred to a tertiary care institution for evaluation of a failed newborn hearing screening test. SETTING: Arkansas Children's Hospital, Little Rock, Arkansas. RESULTS: Seventy-six patients were referred for failed OAEs and complete otolaryngology evaluation. Mean age at the time of referral was 3 months (0.25 years) old. OME was identified in 64.5% of the patients. ABR confirmed a suspected hearing loss in 15 patients (78.9%) without middle ear disease. Effusion resolved without surgical intervention in 65.3% of infants, while 17 (34.7%) of the infants required tubes. SNHL was subsequently identified in 11% of infants after resolution of the effusion. CONCLUSIONS: OME is a common cause of failed infant hearing screens, and should be looked for prior to definitive diagnostic hearing testing. OME resolves in the majority of infants, but tube insertion is necessary to allow for diagnostic testing in nearly one third of infants. The majority of infants without OME had SNHL confirmed. SNHL was also identified in 11% of infants with OME after resolution of the effusion.     

上半规管裂综合征的诊断

目的探讨上半规管裂综合征的临床诊断学特点，提高对该病的认识。方法回顾性分析近期诊断的6例上半规管裂综合征患者，总结其临床症状、体征、听力学和影像学特点。结果6例患者中4例患耳表现为低频听力下降，声反射均能引出；1例合并有中耳炎表现为极重度感音神经性听力下降；另1例听力正常。2例次患者有明显的自声增强，不能耐受自己说话的声音。5例次患者有不同程度的眩晕，眩晕持续时间数小时不等，程度不一。2例次患者主诉不能耐受外界噪声。4例次患者利用强声能诱导眩晕。所有患者在强声刺激或增加中耳压力、捏鼻鼓气时能观察到慢相向健侧的垂直旋转性眼球运动，其中1例患者强声刺激能诱发头部运动。所有患者均行颞骨CT检查，并行上半规管重建显示上半规管表面不同程度的骨质缺损。2例患者全身麻醉下行上半规管裂填塞修补术，术后症状明显改善。结论上半规管裂隙综合征的诊断主要依据颞骨高分辨率CT提示上半规管表面覆盖的骨质缺损，同时患者具有相关的前庭耳蜗症状。     

Central effects of residual hearing: implications for choice of ear for cochlear implantation

OBJECTIVES/HYPOTHESIS: The study tested the hypothesis that among patients with similar levels of residual hearing in the nonimplanted ear, speech perception outcome is the same whether or not the implanted ear has profound or severe levels of hearing loss. STUDY DESIGN: Retrospective. METHODS: Levels of hearing loss in postlingually deafened adults who had cochlear implantation at Johns Hopkins University (Baltimore, MD) between 1991 and 2002 were classified according to pure-tone averages as bilateral severe (n = 20), severe-profound (severe in one ear and profound in the other) (n = 23), and bilateral profound (n = 43). There was no significant difference in the age at onset and duration of deafness among the three patient groups. Individuals in the bilateral severe and severe-profound groups had comparable levels of severe hearing loss in their nonimplanted ears, whereas those in severe-profound and bilateral profound groups had comparable levels of profound hearing loss in their implanted ears. Speech perception performance was evaluated using words from the Consonant Nucleus Consonant word list, Hearing in Noise Test sentences in quiet, and Central Institute for the Deaf sentences through recorded presentation at 70 dB sound pressure level (SPL). RESULTS: Despite the profound hearing loss of the implanted ear in the asymmetrical group, there was no significant difference in mean speech perception scores compared with the bilateral severe group within the first year after implant surgery. By comparison, the bilateral profound group had lower speech perception results compared with patients with residual hearing in one or both ears. CONCLUSION: The study results suggest that implantation of the profoundly deafened ear does not diminish the functional advantage conferred by residual hearing in a patient with asymmetrical hearing loss. Therefore, the central auditory pathway may be the site at which persistent auditory function has its most beneficial effects.     

Spontaneous musical auditory perceptions in patients who develop abrupt bilateral sensorineural hearing loss. An uninhibition syndrome?

CONCLUSIONS: Spontaneous musical auditory perceptions commonly occur in patients who develop abrupt bilateral sensorineural hearing loss. The findings in both subjects who were image tested while having these perceptions are suggestive of a biological substrate for this process and of a central locus for auditory memory seemingly located in and around area 39 of Brodmann. When an individual has abrupt bilateral sensorineural hearing loss, stored musical memory can be released and this person can have musical perceptions without an external source. It is likely that an abrupt bilateral loss of inner ear function might uninhibit neuronal groups storing auditory memory. OBJECTIVES: The objectives of this study were as follows. (1) To determine if spontaneous musical auditory perceptions occur in patients who develop abrupt bilateral severe sensorineural hearing loss (not necessarily simultaneously). (2) To determine if there is a biological substrate to the process of recalling previous auditory perceptions. (3) To compare these findings with our normal databases of unstimulated and pure tone-stimulated volunteers. (4) To establish a hypothesis for the mechanisms of these occurrences. PATIENTS AND METHODS: Thirty two patients who had had abrupt bilateral severe sensorineural hearing loss (the interval between the losses of both ears could have been years) were contacted. They were asked if they had ever had the sensation of having musical auditory perceptions without external auditory stimuli. Two of these patients were image tested with single photon computerized emission tomography (neuroSPECT) while they were having these perceptions. RESULTS: All of the 32 subjects had musical auditory perceptions following their hearing loss in the second ear or when hearing loss in both ears occurred simultaneously. The two patients who were image tested with neuroSPECT had similar findings. There was a statistically significant increase in perfusion in area 39 of Brodmann, more intense on the right side, with increased perfusion also in both frontal lobes at the middle gyrus, with bilateral hypoperfusion in area 38 of Brodmann. These findings are similar to those observed in normal volunteers stimulated with pure tones.     

The Conductive Hearing Loss Due to an Experimentally Induced Middle Ear Effusion Alters the Interaural Level and Time Difference Cues to Sound Location

Otitis media with effusion (OME) is a pathologic condition of the middle ear that leads to a mild to moderate conductive hearing loss as a result of fluid in the middle ear. Recurring OME in children during the first few years of life has been shown to be associated with poor detection and recognition of sounds in noisy environments, hypothesized to result due to altered sound localization cues. To explore this hypothesis, we simulated a middle ear effusion by filling the middle ear space of chinchillas with different viscosities and volumes of silicone oil to simulate varying degrees of OME. While the effects of middle ear effusions on the interaural level difference (ILD) cue to location are known, little is known about whether and how middle ear effusions affect interaural time differences (ITDs). Cochlear microphonic amplitudes and phases were measured in response to sounds delivered from several locations in azimuth before and after filling the middle ear with fluid. Significant attenuations (20–40 dB) of sound were observed when the middle ear was filled with at least 1.0 ml of fluid with a viscosity of 3.5 Poise (P) or greater. As expected, ILDs were altered by ~30 dB. Additionally, ITDs were shifted by ~600 μs for low frequency stimuli (<4 kHz) due to a delay in the transmission of sound to the inner ear. The data show that in an experimental model of OME, ILDs and ITDs are shifted in the spatial direction of the ear without the experimental effusion.     

Mechanisms of hearing loss resulting from middle-ear fluid

Fluid in the middle ear, a defining feature of otitis media with effusion (OME), is commonly associated with a 20- to 30-dB conductive hearing loss. The effects and relative importance of various mechanisms leading to conductive hearing loss were investigated in a human temporal bone preparation. Umbo velocity in response to ear-canal sound was measured with a laser vibrometer while saline and silicone fluids of viscosity 5-12,000 cSt were introduced into the middle ear to contact part or all of the tympanic membrane (TM) and fill part or all of the middle ear. At low frequencies, reductions in umbo velocity (deltaVU) of up to 25 dB depended on the percentage of the original middle-ear air space that remained air-filled, which suggests that the primary mechanism in hearing loss at low frequencies is a reduction of the admittance of the middle-ear air space due to displacement of air with fluid. At higher frequencies, deltaVU (of up to 35 dB) depended on the percentage of the TM contacted by fluid, which suggests that the primary mechanism at high frequencies is an increase in tympanic membrane mass by entrained fluid. The viscosity of the fluid had no significant effect on umbo velocity. deltaVU for the fluid-filled middle ear matched hearing losses reported in patients whose middle ear was believed to be completely filled with fluid. The difference between deltaVU for a partly-filled middle ear and hearing losses reported in patients whose middle ear was believed to be incompletely fluid-filled is consistent with the reported effect of middle-ear underpressure (commonly seen in OME) on umbo velocity. Small amounts of air in the middle ear are sufficient to facilitate umbo motion at low frequencies.     

Predictors of outcome in children with otitis media with effusion

Otitis media with effusion (OME) is the commonest cause of hearing impairment in young children. The fluctuating nature of the condition makes identification of those with persistent disease difficult without subjecting each child to a period of ‘watchful waiting’. The aim of this study was to determine if the outcome of this observation period could in any way be predicted. The study involved the retrospective analysis of 517 children, aged 3–15 years (mean 5 years and 4 months) in whom the diagnosis of OME had been established. All children had been subjected to an observation period before a decision on surgery was taken. There was a significant correlation between the degree of hearing loss at presentation and after the period of observation. Sex was not a reliable predictor of outcome, but age less than 4 years and presentation in autumn or winter were associated with a poor audiometric outcome. This study identifies a predictive influence on the resolution of OME for these three factors and points the way for future research aimed at identifying the subgroup of children with OME who would benefit from early surgical intervention.     

Bilateral cochlear implants in long-term and short-term deafness

This case is of a 70-year-old man with single-sided deafness (SSD) in the right ear since childhood, who developed a sudden severe hearing loss in the left ear at the age of 63. Eventually, after he received cochlear implants in both ears, he started to present behavioural auditory processing skills associated with binaural hearing, such as improved ability to understand speech in the presence of background noise, and sound localization. Outcomes were measured using cortical auditory evoked potentials, speech perception in noise, sound localization tests, and a self-rating questionnaire. The results suggest that even after more than 50 years of unilateral deafness it was possible to develop binaural interaction and sound localization as a result of electric auditory stimulation.     

双耳感音神经性聋患儿合并分泌性中耳炎的临床诊治

目的 探讨双耳感音神经性聋并发分泌性中耳炎患儿的症状特点, 为及时诊治此类患者提供临床依据。方法 收集经手术治疗的双耳感音神经性聋并发分泌性中耳炎患儿(A组)17例(34耳)的病历资料, 分析其误诊原因、临床特点及并发症发生率, 并与同期行手术治疗的单纯双耳单纯分泌性中耳炎患儿(B组)17例(34耳)进行鼓室粘连发生率的比较。手术前后应用听性脑干反应(ABR)检查随诊听力变化。结果 A组均以家属发现听力下降为首诊症状, 在当地首诊曾诊断为突发性聋7例, 耳闷塞感、耳鸣、耳痛等症状叙述不清, 均无法采集到确切的分泌性中耳炎发病时间;行双耳鼓膜置管时发现中耳粘连5例(7耳), 手术前后ABR检查Ⅴ波阈值改善0～30 dB nHL, 平均17.3 dB nHL, 手术前后Ⅴ波阈值改善, 差异有统计学意义(P < 0.05)。B组患儿无1例误诊, 首诊诉耳痛或耳鸣、耳闷塞感等耳部不适症状15例, 发病时间明确, 首诊诉听力下降2例, 鼓膜置管时中耳粘连1例(1耳)。A组并发症发生率高于B组, 差异有统计学意义(P < 0.05)。结论 双耳感音神经性聋并发分泌性中耳炎患儿临床病史采集困难, 易误诊, 临床并发症发生率高, 应及时干预;鼓膜置管对听力改善效果明显。     

Patulous internal auditory canal

OBJECTIVE: To investigate a rare anomaly of the internal auditory canal known as a patulous canal and its relationship to hearing impairment. METHODS: High-resolution computed tomographic scans of the temporal bones of patients who presented between August 2001 and August 2002 were reviewed. The patients' medical charts were evaluated for age, sex, and hearing impairment, and the computed tomographic scans were examined for the presence of a patulous canal. RESULTS: The study group included 645 patients who underwent high-resolution computed tomography of the temporal bones for various reasons, including sensorineural hearing loss (50% of patients). A patulous canal without any associated anomaly of the labyrinth was the only finding in 2 patients. Both patients had chronic middle ear disease along with conductive hearing loss. CONCLUSION: Patulous canal is a rare anatomical variant of the internal auditory canal (0.3%), and its association with inflammatory ear disease accompanied by conductive hearing impairment appears to have been incidental in both cases in the present study.     

Update zur Physiologie und Pathophysiologie des Innenohrs

Hearing impairment is the most common form of human sensory deficit. The most frequent form, sensorineural hearing loss (SNHL), which accounts for approximately 70% of cases, encompasses various pathologies in both the inner ear and the auditory nerve. The individual hearing impairment and its outcome following aiding with hearing devices critically depend on the underlying disorder. Here recent progress in our understanding of the cellular mechanisms of SNHL in genetically engineered mouse models is reviewed. First, insights gained from models for specific defects in cochlear sound amplification and ion homeostasis are discussed followed by a focus on disorders of the inner hair cell synapses (auditory synaptopathy) and the auditory nerve (auditory neuropathy). Both nosological entities have also attracted substantial clinical interest in recent years and share an impaired temporal processing of auditory stimuli. This results in poor speech recognition, often out of proportion to the pure tone threshold. Hearing loss can range from mild variants with exclusive deficits of temporal processing to complete deafness. At least initially, signs of normal outer hair cell function such as evoked otoacoustic emissions can be found. In summary, well-characterized animal models allow us to refine our pathophysiological understanding of SNHL and offer invaluable help in defining toolboxes for investigating the mechanism(s) underlying the SNHL of affected individuals. Together, this will contribute to custom-tailored diagnostics and rehabilitation of SNHL patients.     

Sensorineural hearing loss in experimental purulent otitis media due to Streptococcus pneumoniae

Sensorineural hearing loss (SNHL) has been described clinically following chronic otitis media with effusion, but to the best of our knowledge, no studies have demonstrated SNHL in an animal model of otitis media. Using the chinchilla model of pneumococcal otitis media, significant SNHL was demonstrated after purulent otitis media, especially at higher frequencies. Animals with otitis media received penicillin G procaine treatment for five days after otitis media with effusion (OME) was first documented; resolution of middle ear infection was confirmed by middle ear effusion culture in all animals. Both the inoculated and uninoculated ears were examined by tone burst-elicited compound action potential at threshold. The inoculated ear showed a marked hearing loss of 13 to 36 dB three to four days after OME was first documented; a hearing loss up to 24 dB persisted two to five weeks after inoculation. The change in the compound action potential was highly significant at all frequencies studied. Conductive losses were largely ruled out because there was no middle ear effusion at death and the tympanogram was normal. Purulent labyrinthitis was ruled out by histopathological study. These results indicate that purulent pneumococcal otitis media in the chinchilla model causes significant SNHL and suggest that the pathogenesis of SNHL associated with chronic OME in humans may be studied in this model.     

Temporal bone findings in a case of otopalatodigital syndrome

The principal features of the otopalatodigital syndrome are hearing loss, cleft palate, and skeletal dysplasia of the hands and feet. The right temporal bone was acquired from a boy with this syndrome who died at the age of 2 1/2 years. Behavioral audiometry had indicated a conductive hearing loss, with probable near-normal sensorineural function; brainstem evoked response audiometry indicated a mild sensorineural hearing loss. Histologic studies of the temporal bone revealed dysmorphic features in both the middle ear and the bony labyrinth. The ossicles were deformed, the stapes was fixed, and no round window was present. A defect of the modiolus resulted in a wide communication between the subarachnoid space of the internal auditory canal and the scala vestibuli. These anomalies would clearly have frustrated any attempt to improve the patient's hearing through reconstructive middle ear surgery.     

Auditory brainstem responses and usefulness of hearing aids in hearing impaired children with Cornelia de Lange syndrome

OBJECTIVE: To examine auditory brainstem evoked responses (ABRs) of children with Cornelia de Lange syndrome (CDLS) to evaluate hearing and the utility of hearing aids in hearing impaired cases. SUBJECTS AND METHODS: Thirteen Japanese infants and children with CDLS were studied. Behavioral observation audiometry and ABR were used to evaluate hearing. RESULT: Four different ABR patterns at 85 dB clicks were observed: no response in either ear (6 patients); clear ABRs in both ears (2 patients); no response in one ear but ABRs recorded in the other ear (3 patients); and no peaks after wave III in one ear and ABRs recorded in the other ear (2 patients). However, in 2 patients with no response in either ear at the first measurement, ABRs were recorded in one ear within 2 years. Three out of 13 patients exhibited better responses to sound through the use of hearing aids and auditory training. CONCLUSIONS: The fitting of hearing aids and early consistent training have a significant effect on auditory development in CDLS children in terms of making them aware of sound localization and the different types of environmental sound.     

Contralateral hearing and tympanoplasty

The relationship between the contralateral hearing threshold in the operated ear and the type of tympanoplasty and the hearing threshold in the operated ear was investigated in this report. The subjects of analysis were 863 ears that received tympanoplasty during the past nine years at the Nippon Medical School Main Hospital. Patients who had good hearing in the contralateral ear received type I tympanoplasty more frequently than did those who had poor hearing on the contralateral ear. On the other hand, patients who had poor hearing in the contralateral ear received a modified type III tympanoplasty more frequently. Patients who had good hearing in the contralateral ear tended to have better hearing in the operated ear compared to those who had poor hearing in the contralateral ear. The hearing threshold in patients who had normal contralateral hearing on the average had 20 dB better hearing than did those who had profound hearing loss in the contralateral ear. More than one third of the patients whose hearing thresholds were worse than 30 dB in the operated ear also had a contralateral hearing loss of 30 dB or more. The decision to perform tympanoplasty in cases of the better hearing ear or the only hearing ear must be made very carefully. The contralateral hearing in patients with chronic otitis media, however, is frequently not normal as was evidenced in this investigation. Also, patients who had poor hearing or were deaf in the contralateral ear received more benefit by tympanoplasty than did those who had good hearing in the contralateral ear.(ABSTRACT TRUNCATED AT 250 WORDS)