Non-organic hearing loss: new and confirmed findings

Although non-organic hearing losses are relatively rare, it is important to identify suspicious findings early to be able to administer specific tests, such as objective measurements and specific counseling. In this retrospective study, we searched for findings that were specific ti or typical for non-organic hearing losses. Patient records from a 6 year period (2003–2008) from the University ENT Department of Bern, Switzerland, were reviewed. In this period, 40 subjects were diagnosed with a non-organic hearing loss (22 children, ages 7–16, mean 10.6 years; 18 adults, ages 19–57, mean 39.7 years; 25 females and 15 males). Pure tone audiograms in children and adults showed predominantly sensorineural and frequency-independent hearing losses, mostly in the range of 40–60 dB. In all cases, objective measurements (otoacoustic emissions and/or auditory-evoked potentials) indicated normal or substantially better hearing thresholds than those found in pure tone audiometry. In nine subjects (22.5%; 2 children, 7 adults), hearing aids had been fitted before the first presentation at our center. Six children (27%) had a history of middle ear problems with a transient hearing loss and 11 (50%) knew a person with a hearing loss. Two new and hitherto unreported findings emerged from the analysis: it was observed that a small air–bone gap of 5–20 dB was typical for non-organic hearing losses and that speech audiometry might show considerably poorer results than expected from pure tone audiometry.     

The management of pseudohypacusis in school-age children

Pseudohypacusis is a condition in which there is an apparent hearing loss in the absence of clinical or audiological evidence; indeed, audiological assessment for inconsistencies is the mainstay of diagnosis. It is easier to diagnose in children than in adults, due to a child's inability to produce consistently similar results on repeat tests. We have reviewed 15 children with pseudohypacusis, who had an average loss in the right ear of 57 dB, and of 59 dB in the left ear when tested by pure tone audiometry. Eight children were diagnosed by clinical impression and inconsistent pure tone audiograms but in order to confirm the diagnosis in the other seven children further testing with a speech audiogram was necessary and proved sufficient to establish the diagnosis in all cases. The hearing of all 15 children resolved spontaneously at between 2 and 70 months, when treated by careful monitoring and supportive therapy, and none of the children needed psychiatric referral.     

多频稳态诱发电位评估中度感音神经性聋儿童的应用

目的探讨多频稳态听觉诱发电位评估儿童中度感音神经性聋的可靠性。方法所有患者经纯音测听（PTA）检查筛选出中度感音神经性聋30例5～6岁儿童（共40耳），然后口服10％水合氯醛镇静睡眠后，行多频稳态听觉诱发电位（ASSR）检测，其阈值与纯音听阂阈值进行比较，分析不同频率处听力阈值分布情况及其相关性。结果分别比较语言频率ASSR阂值与纯音听闽阈值，结果显示，0．5kHz处相关性较差，其差值为2-18dB，而在4kHz处相关性最好。结论可以应用多频稳态听觉诱发电位评估中度感音神经性聋儿童的听力阈值，但需要注意0．5kHz处的相关性差异。     

Air and bone conducted speech reception thresholds

The negative psycho-educational effects and the need for early diagnosis and treatment of mild hearing loss in children is documented. Literature is cited which suggests that air and bone conducted speech stimuli may be superior to pure tone stimuli when used to measure the hearing acuity of children. The sparse literature dealing with the use of bone conducted speech stimuli is reviewed. Experimental data obtained from children is presented, which demonstrates that air and bone conducted speech stimuli yield thresholds equivalent to those obtained with pure tones and that thresholds can be obtained faster by using speech stimuli. Pediatric cases are presented which demonstrate that the use of air and bone conducted speech reception thresholds can differentiate conductive and sensori-neural hearing loss and that reliable speech reception thresholds can frequently be obtained from pediatric patients when pure tone thresholds cannot. The use of air and bone conducted speech reception thresholds is advocated as both an alternative and a complement to traditional pure tone audiometry, particularly with pediatric cases.     

Assessment of the effect of chemotherapy and radiotherapy on the auditory function of children with cancer

Forty children with malignant disease underwent full audiological assessment with pure tone audiometry, tympanometry and measurement of brain stem auditory evoked potentials in an attempt to detect auditory dysfunction resulting from treatment. Significant auditory dysfunction was observed in 8 children. Three children with evidence of a conductive or sensorineural deafness on pure tone audiometry had normal brain stem evoked potentials. One child with a high tone hearing loss and 4 children with no detectable hearing loss on audiometry showed significant differences in the interaural interpeak latencies of selected brain stem auditory evoked potentials. In 2 children these abnormalities had resolved on subsequent testing, 2 had evidence of persistent retrocochlear dysfunction and the remaining child died prior to further evaluation. Routine measurement of brain stem auditory evoked potentials is probably not necessary for the majority of children undergoing treatment for cancer, although such measurements are of value when differentiation between cochlear and retrocochlear damage is required.     

成人感音神经性聋的听觉稳态反应反应阈与纯音听阈

目的探讨成人感音神经性聋的听觉稳态反应（auditory steady-state responses,ASSR）反应阈与纯音听阈的关系。方法选择中国医科大学附属一院耳鼻咽喉科门诊感音神经性聋的成人患者,分别进行纯音听力测试、ASSR检查,比较ASSR在0.5、1、2、4 kHz频率处的反应阈与纯音听阈的相关性及按听力损失程度比较两者的差值。结果 ASSR反应阈与纯音听阈在各频率处的相关系数分别为0.840、0.905、0.886、0.924;随着感音神经性听力损失的加重二者的差值明显缩小。随着频率的增加,两者的差值明显缩小。结论成人感音神经性聋ASSR反应阈与纯音听阈有显著相关性,随着听力损失的加重,ASSR反应阈愈接近纯音听阈,ASSR作为成人感音神经性聋听力定量诊断的客观方法有很大的临床应用价值。     

Bilateral sensorineural hearing disorders in children: etiology of deafness and evaluation of hearing tests

OBJECTIVE: The purpose of this study was to determine the etiology of bilateral sensorineural hearing disorders in children and to evaluate the performed hearing tests by comparison of the results of the objective and subjective tests. METHODS: The medical history and the hearing tests (behavioral observation audiometry, acoustic evoked potentials and pure tone audiometry) of 106 bilaterally hearing impaired children were analyzed in a retrospective follow-up study. RESULTS: The total group included 52 males and 54 females. The ages at first diagnosis ranged from 4 months to 11 years with a mean age of 42 months and a median of 33 months. The degree of hearing loss for the better hearing ear was mild in one child, moderate in 28 children, severe in 29 children, profound in 32 children and total in 16 children. The delay between the first examination and diagnosis ranged from 0 to 597 days with a mean of 83 days and a median of 28 days. In 47 children (44%) no cause of hearing impairment could be determined. Nineteen children (18%) had a history of familial hearing loss, 40 (38%) suffered from acquired hearing loss (seven children had prenatal causes, 21 perinatal and 12 postnatal). A comparison between behavioral observation audiometry and brainstem evoked response audiometry revealed a statistically good agreement. Twenty-nine children (32%) showed progressive hearing loss, which was defined as a threshold shift of +10 dB or more in the pure tone average in at least one ear. CONCLUSIONS: In a significant number of children with early hearing impairments the etiology still remains uncertain. Further research in the field of genetic disorders will diminish this number. Evaluation of hearing tests showed that behavioral observation audiometry still is an excellent tool in the hands of an experienced examiner. The age at identification of hearing disorders in industrialized countries still is unacceptably high. To obtain ideal care of hearing impaired children, universal neonatal hearing screening programs are mandatory.     

ABR in patients with congenital/early acquired sensorineural hearing loss, abnormal stapedius reflex thresholds and speech retardation

The auditory brain stem response (ABR) was recorded in 14 young subjects with mild to moderately severe congenital/early acquired sensorineural hearing loss and abnormal stapedius reflex thresholds. The speech problems of these patients as evaluated by experienced examiners were considered to be more pronounced than could be explained from the hearing loss as measured by the pure tone audiometry. Psychological testing yielded intelligence scores within normal limits or above average for age in 11. They all gave abnormal ABR, indicating dysfunction of the auditory brain stem pathways or, in a few cases, of the cochlear part of the auditory nerve. Presumably, the electrophysiologically demonstrable pathological changes were caused by perinatal complications or early life infectious disease. ABR may prove valuable in the evaluation of children with speech retardation.     

Sudden hearing loss due to meningeal carcinomatosis from rectal carcinoma

We present a case of meningeal carcinomatosis with bilateral hearing loss secondary to a rectal adenocarcinoma. A 60-year-old woman developed progressive loss of hearing in the left ear 19 months after an abdominoperineal resection for an adenocarcinoma of the rectum. Three months after the onset of left hearing loss, she visited our hospital. Pure tone audiometry revealed profound sensorineural hearing loss in the left ear and mild sensorineural hearing loss in the right ear. Gadolinium-enhanced MRI revealed tumor in the left internal auditory canal and cerebellopontine angle and enhancement in the right internal auditory canal. Six days after the first examination, pure tone audiometry revealed profound loss of hearing in the right ear. DPOAE of the right ear were still detected 6 days after the first examination, but were clearly decreased 9 days after it, and reached noise level 10 days after it. Gadolinium-enhanced MRI revealed rapid growth of the tumor of the right internal auditory canal and cerebellopontine angle. We clearly demonstrate here the rapid course of hearing loss using pure tone audiometry, MRI, and DPOAE.     

Evaluation of the use of a questionnaire to detect hearing loss in Kenyan pre-school children

In developing countries, there is a lack of trained personnel and testing equipment to facilitate the early detection of hearing impairment in children. A questionnaire offers a low cost option and the value of this for detecting hearing impairment in pre-school children was determined in several districts in Kenya. The questionnaire was completed by either teachers, parents/carers or community nurses. The children were subsequently tested using pure tone audiometry and visual examination of the ear by ENT Clinical Officers, who were not given prior access to the results of the questionnaire. A total of 757 (88%) questionnaires were completed. Of the 735 children, who could be tested using pure tone audiometry, four were found to have a unilateral hearing impairment and one was detected by the questionnaire. A total of 13 children had a bilateral hearing impairment >40 dB HL. All were detected using the questionnaire. There were eight males and five females with ages ranging from 4.2 to 6.9 years, mean age 5.7 years and median age 5.8 years. Eight had a sensorineural hearing impairment and two a mixed hearing impairment. Three of the children with a sensorineural hearing loss had a family history of hearing impairment. No question detected all children with a hearing impairment and some questions were more discerning than others. There was 100% sensitivity for the questionnaire when a hearing loss of >40 dB was considered, but specificity was lower at 75%. Negative predictive value was 100%, but the positive predictive value was low, 6.75%. It was concluded that a questionnaire of this nature could be usefully applied at Primary Health Care level for detecting hearing impairment at the pre-school stage. There would be need for services available for diagnosis, treatment and habilitation before a screening programme was introduced.     

Nichtorganische (funktionelle) Hörstörungen bei Kindern

Nonorganic (functional) hearing loss in children is characterized by hearing loss without a detectable corresponding pathology in the auditory system. It is not an uncommon disease in childhood. Typically, there is a discrepancy between elevated pure tone thresholds and normal speech discrimination in everyday life. We evaluated 85 original publications, 27 reviews and 4 textbook articles. Mean age at diagnosis was 11.3 years. Girls were affected twice as often as boys. Patient histories showed a high prevalence of emotional and school problems. Pre-existing organic hearing loss can be worsened by nonorganic causes. A brainstem audiometry should confirm the diagnosis. The differential diagnosis includes auditory processing disorder, elevated thresholds in mental retardation and auditory neuropathy. We recommend taking a personal history including biographical factors, a psychological assessment including intelligence testing and referral to a child psychiatrist. Prognosis seems to be dependent on the severity of the patient’s school and/or personal problems. Categorization following the Austen-Lynch model can be a valuable prognostic factor.     

听觉中枢功能状态对老年感音神经性听障患者听力语言康复的影响

目的探究听觉中枢功能状态对老年感音神经性听障患者听力语言康复的影响。方法选取我院2018年1月~2020年8月诊治的老年感音神经性听障患者109例,分别进行纯音测听、助听听阈测试、皮层听觉诱发电位测试及言语识别测试。以55 dB声压级(sound pressure level,SPL)下皮层听觉诱发电位P1是否全部引出波形分为A组(引出波形,n=47)和B组(未引出波形,n=62);比较两组患者纯音测听、助听听阈结果和言语识别率,分析助听听阈与听觉诱发电位P1波潜伏期、幅值的相关性。结果A组患者纯音听阈显著高于B组(P<0.05);A组在低频、中频、高频的助听听阈值均显著低于B组(P<0.05)。配戴助听器后两组患者的言语识别率均增加,且A组显著高于B组(P<0.05)。P1波幅值随着刺激强度增强逐渐降低,助听听阈随着引出P1的刺激强度增强逐渐提高。助听听阈与幅值呈显著负相关(P<0.05),与P1波潜伏期无相关性(P>0.05)。结论皮层听觉诱发电位与助听听阈测试结果具有相关性。     

Auditory manifestations of Keratitis-Ichthyosis-Deafness (KID) syndrome

OBJECTIVE: Evaluation of the auditory manifestations of Keratitis-Ichthyosis-Deafness (KID) syndrome, a rare genodermatosis characterized by follicular hyperkeratosis, vascularizing keratitis, and congenital hearing loss. STUDY DESIGN: Five individuals with sporadic KID syndrome were evaluated in the outpatient audiology clinic at the Warren Grant Magnuson Clinical Center of the National Institutes of Health. METHODS: Audiologic examinations included pure-tone audiometry, speech audiometry, and middle ear immittance testing. Auditory brainstem responses and otoacoustic emissions were analyzed in 2 subjects. RESULTS: Four subjects had prelingual, bilateral, profound sensorineural hearing loss, whereas the fifth subject had significant residual hearing that exhibited no progression on serial audiograms. All 5 subjects had a history of non-erosive keratosis obturans and cutaneous cysts in the external ear canals that prevented continuous use of ear molds. CONCLUSIONS: The sensorineural hearing loss in KID syndrome is generally prelingual and profound. This combination of auditory and cutaneous phenotypes is similar to those previously reported for KID syndrome. KID syndrome presents a difficult challenge for communication rehabilitation because keratitis may impair the perception of sign and spoken language, and the cutaneous manifestations routinely curtail use of external amplification devices.     

Nonsyndromic Hearing Loss Caused by a Mitochondrial T7511C Mutation

Objectives The aims of the present study were to identify a mutation in a Japanese family showing nonsyndromic sensorineural hearing loss and to relate the mutation to characteristics of patients, including audiovestibular findings. Study Design Familial cohort study. Methods Mutation analysis was performed using genomic DNA extracted from blood samples. Subjects underwent audiovestibular examinations, including pure‐tone audiograms, tympanometry, self‐recording audiometry, acoustic reflex threshold, speech discrimination testing, evoked and distortion‐product otoacoustic emissions, auditory brainstem responses, and caloric testing. Results We identified a T7511C mutation in the mitochondrial tRNA^Ser(UCN) gene previously reported in one other family. The degree of heteroplasmy for the T7511C mutation ranged from 84% to 92%, and did not correlate with age at examination or severity of hearing loss. Extensive audiologic evaluation suggested both cochlear and retrocochlear involvement. Conclusion Families with maternally transmitted nonsyndromic hearing loss should be investigated for mutations in the tRNA^Ser(UCN) gene.     

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.     

畸变产物耳声发射在军事噪声致爆震性聋早期监测中的作用

目的探讨畸变产物耳声发射(DPOAE)在军事噪声致爆震性聋早期监测中的作用。方法将听力正常的炮兵某部男性官兵88例(176耳)随机分成两组,每组44例(88耳),实验组参加火炮实弹射击训练,对照组未参加火炮实弹射击训练。对照组及实验组训练前后进行纯音测听及DPOAE测试,对两组结果进行比较分析。结果实验组训练后80耳中耳传导功能无障碍,其中13耳纯音听阈示感音神经性听力损失,DPOAE检查亦异常;67耳纯音听阈正常,其中15耳DPOAE幅值在4kHz处降低。与训练前、对照组比较,实验组训练后2、4及8kHz纯音听阈升高(P<0.05或P<0.01),以4kHz处升高最为明显;实验组训练后2、3、4、6及8kHzDPOAE幅值降低(P<0.05或P<0.01),其中以4kHz处降低最为明显。结论畸变产物耳声发射在军事噪声致爆震性聋早期监测中有一定应用价值,可对其防护、诊断和治疗提供指导和帮助。     

Bilateral acoustic schwannoma: post-operative hearing in the contralateral ear

A patient with neurofibromatosis two (NF2) presented with bilateral acoustic neuroma. Pre-operative audiometry revealed a dead right ear and severe left-sided sensorineural hearing loss. Following surgical removal of the larger right acoustic neuroma we have documented a sensorineural improvement of 45 dB in the contralateral ear on pure tone audiometry, which as far as the authors are aware has not previously been described.     

Audiological correlates of tumor parameters in acoustic neuromas

The objective of this study was undertaken to evaluate a correlation of pure tone audiometry, speech audiometry with tumor size, the presence of intrameatal extension, contralateral pure tone audiometry, and contralateral speech audiometry results at acoustic neuroma (AN) patients. Seventy-six patients with a unilateral-cerebellopontine angel tumor with/without internal acoustic canal (IAC) were included in this study. Data on pure tone audiometry, speech discrimination scores (SDS), speech reception thresholds (SRT), tumour size, and extension to IAC were analyzed retrospectively. As a result, patients with unilateral ANs had an asymmetric sensorineural hearing loss and had an asymmetric finding on the results of speech audiometry was present. We could not find any correlation between the size of the tumor and the hearing levels at each frequency. In addition, there appeared to be no significant correlation between the extension of tumor to the IAC and the audiological findings.     

听觉神经病

目的　探讨听觉神经病的临床特点、听力学及电生理特征。方法　总结了 17例听觉神经病患者的病史、纯音及语言听力、镫骨肌反射、听性脑干反应 (auditorybrainstemresponse ,ABR)、耳蜗电图、鼓岬电刺激试验、耳声发射及对侧声抑制试验的特点。结果　该病多在青春期后起病 ,女性较多发 ,表现为渐进性双耳对称性听力下降 ,纯音听力以低频损失为主 ,语言接受阈升高 ,分辨率下降 ,镫骨肌反射及ABR引不出或阈值明显升高与纯音听力不成比例 ,耳蜗微音器电位正常 ,听神经动作电位引不出 ,耳声发射波幅高、时程长且不为对侧声刺激所抑制。结论　听觉神经病的诊断应在除外各种已知原因的感音神经性聋后 ,根据其纯音听力与客观测听的不一致及电生理学上的耳蜗活动与中枢系统反应分离的特征作出 ,对该病的认识尚需进一步深入。