Elevation of bone conduction threshold in children with middle ear effusion

A retrospective study of children having otitis media with effusion revealed fluctuations in bone conduction thresholds as well as in air conduction thresholds. Previous investigations in this area presented both low- and high-tone bone conduction hearing loss which were reversible. We conducted a detailed study including complete otologic, audiologic and tympanometric evaluation of 27 (41 ears) children who had fluctuating bone conduction hearing loss. From these audiologic examinations, 3 types of bone conduction hearing loss could be classified: high-tone, low-tone and flat-type bone conduction hearing loss. We observed the shift of bone conduction thresholds in children after removal of middle-ear fluids by the appropriate medical management.     

Sensorineural hearing loss and Kawasaki disease: a prospective study

PURPOSE: Kawasaki disease (KD) is an acute, self-limited vasculitis of infants and children that is now the most common cause of acquired heart disease in the pediatric age group in the United States and Japan. Reports have documented the association of acute KD with sensorineural hearing loss. To assess the prevalence of hearing loss following acute KD in a geographically and ethnically diverse population, a prospective, multicenter study of hearing loss in patients with KD was conducted. MATERIALS AND METHODS: Patients with acute KD were enrolled in 7 clinical centers and underwent a primary audiologic evaluation within 30 days of the onset of fever. Patients were subsequently reevaluated after resolution of the acute phase of the disease. A questionnaire assessing risk factors for hearing loss was also administered. RESULTS: A total of 62 patients were evaluated during the 29-month study period. At the first audiologic evaluation, 19 patients (30.6%) had sensorineural hearing loss, 6 patients (9.7%) had conductive hearing loss, 17 patients (27.4%) had normal hearing, and 20 patients (32.3%) had inconclusive studies. Overall, 2 of 36 patients (5.5%) had sensorineural hearing loss documented on their second audiologic evaluation. No risk factors for hearing loss were identified by the questionnaire. CONCLUSIONS: Transient sensorineural hearing loss (20 to 35 dB) is a frequent complication of acute KD and may be related to salicylate toxicity in some patients. Persistent sensorineural hearing loss is uncommon. Parents and primary care providers should be made aware of the potential for persistent sensorineural hearing loss following resolution of KD, but routine audiologic screening of this patient population does not appear to be warranted.     

Audiologic and otologic phenotype in children with Duane's Retraction Syndrome: A rare ophthalmologic disorder

ObjectiveThe focus of this study was to evaluate the prevalence, type, and severity of hearing impairment in patients with Duane's Retraction Syndrome and to relate these measures to patient demographics and other otologic and audiologic factors.Study designRetrospective AudGen Database analysisSettingTertiary academic referral centerPatientsPediatric patients in AudGenDB with a diagnosis of Duane's Retraction Syndrome (DRS).InterventionsAppropriate audiologic, otologic, and demographic data were recorded.Main outcome measureSeventy-nine patients (n = 79) met inclusion criteria. The first encounter with available audiometric data or the first encounter with hearing loss were documented. Audiograms were stratified by type and severity of hearing loss, and common associated medical issues were documented.Results57 children had normal hearing; 22 had hearing loss. 9 ears had pure conductive hearing loss, 1 had pure sensorineural, 14 ears had components of both, and 79 had hearing loss that could not be specified. Multivariate regression revealed episodes of chronic otitis and craniofacial anomalies are associated with worse hearing loss.ConclusionThis study presents a detailed characterization of hearing loss in patients with Duane's retraction syndrome. Conductive and sensorineural hearing loss are both prevalent among these children. Careful and early audiologic evaluation of all patients with DRS is important.     

Noncongenital hereditary hearing loss in children. Prospective documentation

Younger siblings of children with sensorineural hearing loss of possible hereditary cause underwent interval audiologic examination. Seven siblings (in unrelated families) were found to have progressive sensorineural hearing loss despite early audiograms documenting normal hearing levels for age. Continued testing of these children allowed for early identification and intervention. We advocate regular otolaryngologic and audiologic follow-up even after normal audiologic assessments are made for younger siblings of children with documented sensorineural hearing loss, unless a definite nongenetic origin of the hearing loss in the older child is known. Recessive sensorineural hearing loss with onset in infancy or childhood may present with no antecedent family history and with normal behavioral audiograms early in life.     

Audiologic evaluation of infants and preschoolers: a practical approach

Many children suspected of having hearing loss are referred for audiologic evaluation every day. Cross-checking the results from more than one audiologic tests is considered crucial in pediatric audiology, preferably combining subjective and objective methods. The current recommended approach for hearing assessment of infants and preschoolers is based on physiologic tests, immittance measurements, and behavioral responses. As a consequence, a full examination usually takes more than 90 minutes. Because the number of referrals may be much greater than the actual performance of a modern audiologic department, it would be desirable to shorten the evaluation time without reducing its validity. The largest part of the population referred to our department for hearing evaluation consists of children 1(1/2) to 4 to 5 years old suspected of having hearing loss. The proposed triad history/otoscopy --> speech evaluation --> otoacoustic emissions (OAEs) has been proven very effective in sorting out all those children with normal hearing bilaterally. The suggested algorithm shows several advantages compared to the conventional approach. It is safe, inexpensive, noninvasive, and gives reliable results in a significantly faster way, thus increasing compliance and applicability in very young children. In this way, we can save time, "money," and "diagnostic energy," which could be used for those children who really need them.     

Auditory neuropathy in childhood

Objectives: Auditory neuropathy is a recently described disorder in which patients demonstrate hearing loss for pure tones, impaired word discrimination out of proportion to pure tone loss, absent or abnormal auditory brainstem responses, and normal outer hair cell function as measured by otoacoustic emissions and cochlear microphonics. We have identified eight pediatric patients having hearing deficits that are most likely due to a neuropathy of the eighth nerve. In this study, the results of audiologic testing performed with these eight children are described. Study Design: Retrospective review of audiologic findings in eight children with auditory neuropathy. Methods. Each subject was tested with pure tone and speech audiologic testing, auditory brainstem response, and click-evoked otoacoustic emissions. Results of these tests were tabulated and summarized. Results: Pure tone audiologic testing revealed five children with upsloping sensorineural hearing loss, two with high frequency loss, and one with a mild, flat configuration. Six children demonstrated poor word discrimination scores, and the other two had fair to good word discrimination. All eight subjects had normal distortion product and transient otoacoustic emissions. All eight children demonstrated absent or marked abnormalities of brainstem auditory evoked potentials. These findings suggest that while cochlear outer hair cell function is normal, the lesion is located at the eighth nerve. Conclusions: Recent advances in otoacoustic emissions testing permit differentiation of neural deafness from sensory deafness. This paper describes the clinical presentation and audiologic findings in pediatric auditory neuropathy, as well as the recommended management of these patients. Otolaryngologists should be aware of this disorder and implications for its management, which differs from treatment of sensorineural hearing loss. Key Words: Auditory neuropathy, childhood, hearing loss, auditory brainstem response, evoked otoacoustic emissions.     

Complete evaluation of the child identified as a poor listener.

With increasing awareness among educators of the importance of early identification of hearing impairment, growing numbers of children are being referred for evaluation when a teacher or day care supervisor perceives that a child is having difficulty listening. Some children who manifest difficulty listening in a pre-school play group or the classroom may have conductive or sensorineural hearing loss, while others have normal hearing with an underlying and yet-to-be-detected behavioral or psychoeducational disorder. This report presents suggestions for evaluation of the child referred for difficulty listening. The otologist should consider that a child may have an attention deficit disorder when results of initial audiologic assessment indicate there is no hearing loss or when the degree of hearing loss appears to be small in relation to the degree of inattentiveness that has been observed. The features of Attention-deficit Hyperactivity Disorder (ADHD) and Specific Developmental Disorder (SDD) are described, and illustrative case studies are presented. Clues to diagnosis are provided and a distinction between overlapping disorders is made.     

Sudden hearing loss and cerebellopontine angle tumors

Lesions of the cerebellopontine angle may cause a sudden hearing loss. To illustrate and reaffirm the importance of a thorough neurotologic evaluation we performed a retrospective review of the audiologic and clinical data available on 506 patients with proven lesions of the cerebellopontine angle. Seventy-seven patients (15.2%) were found to have a history commensurate with sudden hearing loss. We reviewed the relevant audiologic data as well as the clinical circumstances of this select patient population.     

热休克蛋白70抗体检测对自身免疫性聋诊断的意义

目的 分析自身免疫性聋的听力学特点，探讨用免疫印迹法(Western blot)检测热休克蛋白70抗体在自身免疫性耳聋诊断中的意义。方法自身免疫性聋14例28耳，其它感音神经性聋8例16耳，正常对照6例12耳，采用免疫印迹法检测各组受试者血清中的热休克蛋白70抗体，并分别作临床听力学检查。结果自身免疫性耳聋者纯音听力图多数为平坦型，TEOAE和／或DPOAE未引出，听性脑干反应多数正常，轻、中度耳聋者可引出镫骨肌声发射，该组8例(57．1％)热休克蛋白70抗体阳性。其它感音神经性聋者纯音听力图多数为高频减退型和平坦型，TEOAE和DPOAE未引出，重度耳聋者ABR未引出，轻、中度耳聋者可引出镫骨肌声发射，1例(12．5%)热休克蛋白70抗体阳性。正常对照组1例(16．7％)热休克蛋白70抗体阳性。结论免疫印迹法检测热休克蛋白70抗体对自身免疫性耳聋诊断有参考价值，对该病的确诊还应结合临床听力学特点。     

Normal auditory brainstem response in patients with acoustic neuroma

Auditory brainstem response testing has been a major breakthrough in audiologic screening for acoustic neuroma because of its high degree of sensitivity. Although it is not uncommon for other cerebellopontine angle masses to present with normal ABR findings, reports of eighth nerve tumors with false-negative auditory brainstem response tests are quite rare. A series of 120 acoustic neuromas resected at the University of Michigan was reviewed and revealed two such patients. These two patients presented with asymmetric sensorineural hearing loss and unilateral tinnitus and were found to have completely normal auditory brainstem response. The diagnosis of acoustic neuroma would have been delayed if a comprehensive evaluation had not been pursued.     

The role of audiologic evaluation in progressive audiologic tinnitus management

Progressive Audiologic Tinnitus Management (PATM) is based on the premise that tinnitus is managed most efficiently using a hierarchy of clinical services that address different levels of need. PATM includes five levels of management: (a) triage; (b) audiologic evaluation; (c) group education; (d) tinnitus evaluation; and (e) individualized management. This article provides an overview of PATM and focuses on the procedures that make up the Level 2 Audiologic Evaluation. The evaluation is conducted to assess the potential need for medical, audiologic (hearing loss, tinnitus, hyperacusis), and/or mental health services. The Tinnitus Handicap Inventory, Hearing Handicap Inventory, and Tinnitus and Hearing Survey are used to differentiate effects of tinnitus and hearing loss. If indicated, patients are interviewed with the Tinnitus-Impact Screening Interview. Patients requiring amplification receive hearing aids. Often, management of hearing loss at Level 2 addresses any problems that were attributed to the tinnitus, which obviates further tinnitus-specific intervention.     

Ear and hearing problems in relation to karyotype in children with Turner syndrome

The aim of the study was to report otologic and audiologic characteristics in a group of children with Turner syndrome (TS) and correlate these findings to karyotype. Additionally, we give recommendations for the otologic care of these children. Sixty children (age 1.7-21.2 years) were included in this retrospective study. Medical history and karyotypes were recorded and otologic and audiologic evaluation was performed. A history of recurrent otitis media was reported in 41/60 (68%) children and 3/60 (5%) had suffered from cholesteatoma. Audiometric data in 56 children revealed that normal hearing was only present in 33/112 (29%) ears. All other ears 79/112 (71%) were classified in five different audiometric categories for hearing loss. Hearing thresholds in general appeared to be about 10-11 dB worse in children with a monosomy 45,X or isochromosome (both have a total deletion of the short (p) arm of the X-chromosome) compared to those having a mosaicism or structural anomaly (partial deletion, or total deletion in only a few cells). Our findings support the hypothesis that hearing can be affected by loss of the p-arm of the X-chromosome. It is for the first time that a relation between hearing problems and karyotype is statistically confirmed in a large group of children with TS.     

Issues associated with the measurement of psychosocial benefits of group audiologic rehabilitation programs

The purpose of this review is to describe the psychosocial effects of hearing loss, review the literature that has attempted to measure the psychosocial benefits of group audiologic rehabilitation programs, and offer suggestions for the design of future studies. The psychosocial aspects of hearing loss are multidimensional and may include the emotional, cognitive, interpersonal, behavioral, and physical responses to hearing loss. As a result of the stigma of hearing loss, individuals may isolate themselves, avoid social interactions, and/or bluff their way through communication breakdowns. Participation in group audiologic rehabilitation programs is a straightforward way to deal with the stigma and the loss of social identity associated with hearing loss, and these groups are an ideal forum for teaching communication strategies. Yet, the literature on the efficacy of group audiologic rehabilitation has produced equivocal results. Several factors may have contributed to the lack of psychosocial benefits reported in the literature, including the theoretical framework upon which the program was designed, the actual class content, the effectiveness of the outcome measures, and the demographic characteristics of the participants in the programs. Future research should determine (1) if the theoretical framework and/or the content covered in group audiologic rehabilitation programs influences the psychosocial outcomes, (2) if instructor training influences psychosocial outcomes, (3) the ideal test battery to assess the psychosocial benefits of group audiologic rehabilitation programs, (4) the participant demographic and personality characteristics that influence psychosocial outcomes, and (5) whether experienced hearing aid users have different hearing-loss related psychosocial needs than new hearing aid users.     

Etiological diagnosis of bilateral, sensorineural hearing impairment in a pediatric Greek population

Early diagnosis, evaluation and treatment of childhood deafness are essential for a child's normal growth. Etiological diagnosis of hearing loss makes prevention, family scheduling and more effective therapy feasible goals. Etiological assessment of sensorineural deafness still remains difficult although recently with the progress of genetics it has become more efficient. In this retrospective study, the etiology of bilateral, sensorineural hearing loss with indication for hearing aids has been studied in 153 hearing impaired children. Etiological diagnosis was based on family and patient record, physical, audiological and laboratory examinations. Among the 94 children who completed the diagnostic protocol etiological groups revealed the following distribution: non-hereditary acquired hearing impairment was present in 36 children (38%) and hereditary was present in 44 (47%) children. The etiology remained unknown in 14 (15%) children. Non-syndromic autosomal dominant type accounted for 13 (29% of hereditary hearing loss) children, non-syndromic autosomal recessive type for 21 (48%) children and syndromic deafness for 10 (23%) children. Modern diagnostic methods, such as genetic testing, help diminish the number of cases with hearing impairment of unknown etiology, for the benefit of children who receive early and appropriate medical, audiologic, genetic and educational counseling based on the etiology of their hearing loss.     

Prospective radiological study concerning a series of patients suffering from conductive or mixed hearing loss due to superior semicircular canal dehiscence

The aim of this study is to appreciate the incidence of patients with isolated conductive hearing loss with normal drum due to superior semicircular canal dehiscence (SCD). It is a prospective radiological study. Two hundred and seventy-two patients with a normal drum suffering from isolated unilateral or bilateral conductive or mixed hearing loss were included in a prospective radiological study. A high resolution computerized tomography (HRCT) was performed in all the patients. Those who were found to have a unilateral or bilateral SCD underwent further etiological, clinical, audiologic evaluation. Ten patients with conductive or mixed hearing loss were found to have a unilateral or bilateral SCD. The disease was bilateral in five cases, and most often associated with a dehiscence of the tegmen tympani on both sides, supporting the theory of the congenital nature of the disease. There was no clear correlation between symptoms and the size of the SCD. Because patients were not suffering from incapacitating vestibular symptoms, they were not operated for surgical occlusion of the SCD, and were referred to a hearing aid specialist to improve hearing. Conductive or mixed hearing loss due to SCD is relatively frequent, justifying in our opinion that a systematic HRCT be carried out before surgery of any patient with conductive hearing loss.     

Quantitative and qualitative follow-up outcomes from a preschool audiologic screening program: perspectives over a decade

PURPOSE: This investigation reports on quantitative and qualitative follow-up information obtained from a preschool audiologic screening program covering a 10-year period (1995 to 2004). METHOD: The audiologic screening consisted of a hearing (pure tone) and tympanometry screening. A total of 34,979 children, 3 to 5 years of age, were screened. RESULTS: Eighteen percent (6,337) of the children were referred for further hearing and/or medical ear evaluation. Of 1,421 follow-up responses received, 93% complied with the follow-up recommendations while 7% did not. Of 1,316 children in the follow-up group, outer and/or middle ear disorder in one or both ears was medically confirmed for 37%. Unilateral or bilateral hearing loss was diagnosed in 18% as conductive (12%), sensorineural (1%), mixed (0.4%), or unspecified (5%). Overall, hearing loss and/or otologic disorder was confirmed in 49% of the follow-up group, suggesting a prevalence of 1.8% in a preschool-age population. A small (n = 32) sample of unsolicited comments indicated that physicians most influenced noncompliance with hearing evaluation follow-up. CONCLUSIONS: The quantitative hearing and otologic follow-up outcome data affirm the importance of audiologic screening in the preschool population. Qualitative data suggest that some physicians may not be advocating appropriate screening follow-up services.     

Audiologic results in patients with Moebiüs sequence

OBJECTIVE: Moebiüs sequence is a pathology not very well understood regarding to the hearing status. The main goal of this study was to describe the audiologic findings in children and adolescent who carry Moebiüs sequence. METHOD: Participated in this study 17 children and adolescent, with age ranging from 3 to 13 years old. Prior to the testing, the family answered an interview. It was realized external auditory canal inspection, and the hearing testing (auditory instrumentation, pure tone audiometry, speech audiometry, immittanciometric measures, and otoacoustic emissions) on the participants. RESULTS: The auditory instrumentation evaluation (n=6) was present in all participants. The pure tone audiometry presented normal hearing levels in 75.0% of the tested ears, one ear with conductive hearing loss, two ears with sensory neural hearing loss, and one ear with mixed hearing loss. The tympanometric measures showed Type A tympanograms in 63.0% of the ears, Type B in 11.1%, Type C in 18.5%, and Type As in 7.4%. T the acoustic reflexes measure showed contralateral acoustic reflexes present in 50.0% of the ears, and ipsilateral acoustic reflexes present in 34.6% ears. The OAE results showed presence in 73.0%, for the TOAE, and 76.9% for the DPOAE. CONCLUSION: These results support the idea that there is no audiologic pattern for conductive hearing loss. The majority of the participants presented hearing in the normal range. Care should be taken in drawing conclusions regarding to auditory status of the individual with Moebiüs sequence, but what can be said is that not always those individuals present hearing loss.     

Van der Hoeve综合征一家系听力学调查

目的:报导— Van der Hoeve综合征家系并进行听力学检测。方法:对家系进行纯音测听、摄全身骨骼X线片,采集外周静脉血进行基因连锁分析。结果:家系4代24人,听力下降4例7耳,其中传导性聋3例5耳,混合性聋1例2耳。听力下降发生年龄最小5岁,随年龄增加,骨导听力逐渐下降,听力损失加重,出现混合性聋。其中有蓝色巩膜、骨折、传导性聋3人,蓝色巩膜、传导性聋3人,仅有蓝色巩膜1人。骨折幼年发病,多发生在四肢管状骨及锁骨,1例后遗畸形。结论:家系疾病连续多代遗传,符合单基因常染色体显性遗传,且外显率较高,属综合征性遗传性聋。     

Facioscapulohumeral muscular dystrophy and accompanying hearing loss

Facioscapulohumeral (FSH) muscular dystrophy (MD) is an autosomal dominant condition marked by muscle weakness primarily in the face, shoulder girdle, and legs. Intelligence and life span are normal and the spectrum of disability is broad. Flaccid dysarthria results from the facial muscle paralysis. A pair of siblings had FSH MD that was accompanied by the unusual finding of sensorineural hearing loss. Speech and audiologic aspects are presented and etiologic explanations are suggested. Careful audiologic evaluation of all patients with FSH MD is advised so that sensorineural hearing loss can be ruled out.     

Prognostic factors of profound idiopathic sudden sensorineural hearing loss

Profound idiopathic sudden sensorineural hearing loss is thought to have a poor prognosis, but few studies have focused on this condition. We aimed to assess the impact of patient factors, audiologic parameters, and salvage intratympanic steroid injection therapy on the prognosis of profound idiopathic sudden sensorineural hearing loss. The demographic, clinical, and audiologic data, degree of hearing recovery, and efficacy of intratympanic steroid injection therapy in 576 patients with profound idiopathic sudden sensorineural hearing loss (mean age 56.2 ± 14.9 years) who had been admitted at four tertiary referral centers between 2000 and 2011 were retrospectively reviewed. The mean hearing level at the initial presentation was 108.1 ± 9.5 dB. Many patients experienced vertigo (52.1 %) and tinnitus (77.4 %). At the 2-month follow-up, 172 (29.8 %) patients showed some degree of hearing recovery, but only 21 (3.6 %) patients recovered normal hearing. Further, the 116 patients who had received salvage intratympanic steroid injections showed a better audiologic outcome (improvement, 26.1 ± 24.3 vs. 15.7 ± 22.1 dB; P = 0.000) than those who had not (n = 429). In conclusion, a higher degree of hearing loss at the initial presentation indicates a poorer prognosis. Salvage intratympanic steroid injection therapy may improve the hearing of patients with profound idiopathic sudden sensorineural hearing loss after the failure of systemic steroid therapy.