先天性小耳畸形全耳廓成形及听力重建术

目的:探讨先天性小耳畸形的临床特点及全耳廓成形及听力重建术的方法和效果。方法:统计、整理2005-01-2010-10期间在我科住院治疗的58例(62耳)小耳畸形患者的资料,本组患者均为先天性耳廓畸形伴外耳道闭锁、听骨链畸形、重度传导性聋。所有患者术前行颞骨CT检查并三维重建,听力学检查。手术分2期进行,一期行耳廓一次成形,外耳道重建,中耳重建手术;二期行耳后植皮,颅耳角再造,对术前、术后的资料进行回顾性分析。结果:耳廓成形加外耳道、中耳重建术后再造耳廓外形良好,耳廓和外耳道口位置接近正常,大部分患者术后听力获得改善。结论:对先天性耳廓畸形并外耳道闭锁的患者,术前应精心设计,根据多层螺旋CT三维重建结果确定听力重建入路及中耳畸形程度,全耳廓成形及听力重建术后可以同时改善耳廓外形和提高听力。     

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.     

对称性聋患者单耳助听后双耳言语识别率差别探讨

目的通过检测对称性聋患者单耳助听后双耳言语识别率的差别来进一步研究听觉剥夺效应。方法选取右耳助听4～5年的双耳对称性感音神经性聋患者15名,在标准隔声室中,测试其双耳的纯音气导和骨导听闻,然后再采用汉语普通话单音节词表分别进行左右裸耳言语识别率测试,并将所得数据进行对比研究。结果15名受试者右耳配戴助听器前及配戴4～5年后两耳间平均听阈无显著性差异（P〉0．05）;受试者右耳助听前及助听4～5年后左、右耳平均听阀前后无显著性差异（P〉0．05）;右耳助听4～5年后,受试者左、右耳裸耳言语识别率存在显著性差异（t=2．76,P=0．02〈0．05）。结论对称性感音神经性聋患者单耳助听后裸耳平均听阈无显著改变,但非助听耳言语识别能力显著下降。     

Immunohistochemical detection of humoral autoantibodies in patients with hearing loss in the last hearing ear

BACKGROUND: An autoimmune etiology similar to the sympathetic ophthalmia has been discussed for sensorineural hearing loss on the last hearing ear following deafness in the first ear. In sympathetic cochleolabyrinthitis inner ear proteins are thought to be released after laterobasal fracture, which may induce an autoimmune process in the last hearing ear. Animal models have failed to clearly demonstrate the location of the target in the labyrinth, attacked by immunologic processes. Furthermore, it is unclear whether the humoral or cellular pathway is initiating this process. METHODS AND PATIENTS: Serum was acquired from 15 patients with traumatic or post-inflammatory unilateral deafness and slowly progressive or sudden sensorineural hearing loss on the last hearing ear. Deparaffinized sections of rat temporal bones were incubated with patient serum and subjected to immunohistochemical examination. RESULTS: A specific but heterogeneous binding pattern of the labyrinth was found in 14 of 15 patients. CONCLUSION: Our results indicate different autoantibodies in the patient serum, which may be the cause of the hearing loss. Therefore, in patients with sensorineural hearing loss on the last hearing ear, we recommend a therapeutic trial with corticosteroids.     

Hearing results of the total middle ear reconstruction

Objective:To evaluate the success of hearing results in total middle ear reconstruction with cartilage ossiculoplasty for the patients with old open- mastoidectomy cavities. Method: A retrospective review of 69 patients who underwent total middle ear reconstruction with cartilage ossiculoplasty was performed. Results: In this series, the successful hearing results were obtained in 61 % of the patients after operation, but 93% of the ears were dry. For patients with stapes, the successful rate was 65%; for patients without stapes,it was 58 %. The mean hearing gain was 10.7dB. Conclusion: Total middle ear reconstruction offers an effective treatment algorithia of hearing reconstruction for patients with old mastoidectomy cavities. 1. Stapes is important for hearing improvement; 2. Staged operation is good for hearing restoration; 3. For a large number of patients with a problematic cavitites, hearing restoration is possible by total middle ear reconstruction.     

Optimum side for fitting a monaural hearing aid. 1. Patients' preference

The criteria generally used to decide in which ear to fit a monaural hearing aid are based on theory or anecdote and have not been verified by experimental observation. In this study to determine optimum side of fitting, 58 new hearing aid users were fitted with a hearing aid in one ear for 10 weeks, and then fitted in the other ear for a further 10 weeks. Subsequently, the subjects were asked their preference for side of hearing aid use and their reasons for this preference. 30 (52%) subjects reported better hearing with their aid in one ear than in the other; 24 (80%) of these preferred the ear with the poorer half-peak level elevation on a speech audiogram. The remaining 28 (48%) reported no difference in their aided hearing ability between ears, though 20 preferred one ear for practical reasons, unrelated to audiometric parameters. A patient's preference for side of fitting can be predicted from audiometric data but with only modest reliability. It is, therefore, recommended that bilateral ear moulds should be routinely provided for bilateral mild to moderate hearing impairment (speech frequency average less than 75 dB HL). If it is decided that only one ear mould should be provided, this should be made for the poorer hearing ear. The poorer hearing ear is best identified from the half-peak level elevation on a speech audiogram.     

A study of the relationship between hearing-aid usage and residual hearing

Fifty-eight children (from 4 to 19 years of age) with bilateral, symmetrical, sensorineural hearing loss, who had used a hearing aid in only one ear for 1 to 19 years were investigated to study the influence of hearing-aid upon the residual hearing between the used and no used ears. The results were as follows, 1) The average hearing level for the used ear at seven frequencies was 49. 5 dB of pre-hearing-aid usage and was 53. 8 dB of post-hearing-aid usage. The discrepancy of audiograms between the aided and unaided ear was not remarkable. 2) The hearings for both the aided and the unaided ears in Audiograms was progressive for a long time. But no significance relation was observed between changes in used-ear hearing of the aided and unaided ears. 3) When the hearing levels of the children were scrutinised on an individual basis, it was found that 9 cases (15.5%) in both ears, 2 cases (3.4%) in only used ear and 1 case (1.7%) in only no used ear out of 58 cases showed deterioration of hearing. 4) These data did not lead us to the conclusion that hearing-aid usage was detrimental on the residual hearing of children with sensorineural hearing loss.     

Long-term results of bone-anchored hearing aid recipients who had previously used air-conduction hearing aids

OBJECTIVE: To study the long-term results (use, care, satisfaction, ear infections, and audiometry) of the application of a bone-anchored hearing aid (BAHA) to patients with conventional indications who had previously used air-conduction hearing aids. DESIGN: Follow-up study (mean duration, 9 years). SETTING: Tertiary referral center. PATIENTS: The study population comprised 27 patients with conductive or mixed hearing loss and who had participated in a previous study (N = 34). Seven could not be included anymore as a result of death, Alzheimer disease, or problems related to the implant. Everyone filled out the questionnaire, and 23 patients underwent audiometric evaluation. MAIN OUTCOME MEASURES: The patients filled out the adapted Nijmegen questionnaire. Aided free-field thresholds were measured as well as scores for speech in noise and in quiet. Results were compared with those obtained in the initial study. RESULTS: All 27 patients were still using their BAHA and appreciated it with regard to speech recognition in quiet, sound comfort, and improvements in ear infections. The audiometric results showed that most patients tested had stable bone-conduction thresholds over the years (after correction for age). Despite the treatment with BAHA, a significant deterioration in the cochlear hearing was observed in the other patients in the ear under study (their best hearing ear). CONCLUSIONS: Positive patient outcome measures emphasized the importance of BAHA application to patients with conventional indications. The audiometric data showed fairly stable cochlear function but not for all patients. This underlines that conservative treatment should be chosen (fitting of bone-conduction devices).     

Ear involvement in Wegener's granulomatosis

In 16 of 19 patients with biopsy-proved Wegener's granulomatosis the early manifestations were limited to the ear and nose. The audiological data of 13 patients revealed middle ear involvement in 16 of 26 ears. Twenty-one of 26 ears presented with a low to moderate sensorineural hearing loss. One ear remained deaf after a sudden hearing loss in the early stage of the disease. Serologically, 4 of 6 tested patients with sensorineural hearing loss demonstrated antibodies against sarcolemma. One patient showed antinuclear antibodies. It is remarkable that these antibodies can often be detected in classic inner ear disorders. The course of inner ear function, serum findings and the success of immunosuppressive therapy in Wegener's granulomatosis are comparable with immunologically mediated vasculitis in the inner ear.     

语前聋患儿一侧人工耳蜗对侧助听器效果分析

目的:通过对人工耳蜗植入对侧耳不同听力损失的患儿联合使用助听器与人工耳蜗语前聋患儿的听觉、语言及学习能力进行评估和比较,探索对患儿更为有效的助听方法,帮助患儿获得最大限度的言语交流。方法:将30例3～6岁语前聋患儿按照植入人工耳蜗对侧耳听力损失程度及是否佩戴助听器,分为一侧人工耳蜗+对侧重度听力损失助听器组(CI+SHA组)、一侧人工耳蜗+对侧极重度听力损失助听器组(CI+PHA组)、单耳人工耳蜗组(CI组)。评估各组在康复3、6、9、12、15、18个月时听觉、语言及学习能力,并记录结果。结果:随着术后康复时间的延长,聋儿听觉、语言及学习能力逐渐提高(P<0.05),CI+SHA组听觉能力优于CI+PHA组及CI组(均P<0.05),语言能力及学习能力无明显差异(P>0.05)。结论:语前聋患儿单耳人工耳蜗植入后,若对侧耳尚有残余听力,佩戴助听器后听觉能力效果显著,长期佩戴有助于患儿的康复。     

Hyperbaric oxygenation in early treatment of sudden deafness

BACKGROUND: The treatment of sudden deafness with hyperbaric oxygenation (HBO) is a new method, which is a routine application in some German centers and subject to contraindications can be assessed as relatively reliable. PATIENTS AND METHODS: In a retrospective study, data were analyzed from 49 patients who had received primary HBO therapy because of sudden deafness (up to 3 weeks old). In comparison to the standard infusion therapy according to Michel, the primary HBO therapy led to inferior results (no change in hearing: right ear 63.88%, left ear 60.98%; improvement in hearing: right ear 22.49%, left ear 21.71%; decrease in hearing: right ear 13.64%, left ear 17.32%). RESULTS: Considering the spontaneous remission of sudden deafness, neither the results of the infusion therapy nor those of the hyperbaric oxygenation surpass the rate of complete spontaneous remission. On the basis of our research, primary treatment of "fresh" sudden deafness with hyperbaric oxygenation cannot be recommended. CONCLUSIONS: The question remains open whether HBO as a secondary treatment for sudden deafness leads to improvements for the patient after unsuccessful standard therapy.     

New hearing aid users' perception of the "hearing aid effect"

The negative attitude elicited by the presence of a hearing aid has been termed the "hearing aid effect." The purpose of this study was to examine the negative reactions associated with hearing aids among new hearing aid users themselves and their perception of the hearing aid effect in their immediate environment. This was accomplished through a questionnaire completed just prior to hearing aid fitting and again 6 months after initial use of amplification. The responses to the questionnaires were compared between individuals who had chosen either in the ear or behind the ear type hearing aids, and between those using either monaural or binaural instruments. The effect of age on the data was also examined. The results indicated that the hearing aid effect was not expected or perceived very widely among these subjects, as only about 10% of them reported sensing negative attitudes to the use of hearing aids. Positive responses to the benefits of hearing aid use from other people often fell short of expectations.     

Functional results of 394 bilateral stapedotomies evaluated with the Glasgow Benefit Plot

This study aimed to evaluate the results of 394 bilateral stapedotomies according to the American Academy of Otolaryngology–Head and Neck Surgery (AAO–HNS) guidelines and with the Glasgow Benefit Plot (GBP) and also analyze the benefit of a second-ear stapedotomy in achievement of normal and symmetrical hearing using retrospective chart review. The charts of 1,369 stapedotomies performed by senior author (J.H.) from 1991 to 2006 were reviewed. Results of 394 bilateral stapedotomies were included. The hearing results were evaluated according to the 1995 AAO–HNS Committee on Hearing and Equilibrium (CHE) guidelines and the GBP criteria. Success rates after the first and second ear surgeries were separately analysed. ABG closure ≤20 dB was demonstrated in 98% of cases after both first and second procedures. Postoperative AC gain was higher in the first ear surgery compared with the second ear surgery in all groups of preoperative hearing impairment. As a result of first ear surgery, 142 (72%) patients changed into the category of unilateral hearing loss. As a result of second ear surgery, 125 (64%) patients changed into the category of normal and symmetric hearing. In this study the largest group of 394 primary bilateral stapedotomies was simultaneously evaluated according to AAO–HNS guidelines and GBP criteria. The results showed that the first ear surgery was more successful in achievement of normal hearing in the operated ear. Second ear surgery was more beneficial in providing symmetric hearing.     

Hearing results of canal wall reconstruction tympanoplasty for middle ear cholesteatoma in children.

OBJECTIVE: To investigate post-operative hearing results in children with middle ear cholesteatoma, and to analyze the correlation between hearing results and clinical factors and findings before and during the operation. PATIENTS AND METHODS: One hundred and twenty-four ears of 123 children were operated on for middle ear cholesteatoma at the age of 10 years or younger by canal wall reconstruction tympanoplasty and were followed up more than 1 year after the final operation. We evaluated the average air and bone conduction hearing levels at the speech ranges before the first operation (pre-operative hearing) and after the final operation (post-operative hearing). RESULTS: The mean of the average air conduction hearing level of 124 ears was significantly improved from 34.7 to 27.1 dB after the final operation. Among them, 84 ears (67.8%) showed a hearing level of 30 dB or less post-operatively. Post-operative hearing was better in the one-stage group than in the staged group. However, more than one-half of the ears which underwent type IV tympanoplasty in the staged group showed post-operative air conduction hearing level of < or =30 dB. Significant improvement in post-operative hearing was noted in ears with normal middle ear mucosa or middle ear effusion at the final operation. No correlation between hearing improvement and clinical factors such as age, type of cholesteatoma or presence of otitis media with effusion at the first operation was found. CONCLUSIONS: Children with middle ear cholesteatoma at the age of 10 years or younger exhibited good hearing post-operatively. Satisfactory hearing improvement is expected even in ears without the superstructure of the stapes if staged tympanoplasty is conducted. Canal wall reconstruction tympanoplasty for pediatric cholesteatoma was successful in terms of hearing results and the success was unrelated to various clinical factors.     

A longitudinal electrocochleographic study of a case of long-standing bilateral Lermoyez's syndrome

Summary An 18-year follow-up of a case of bilateral Lermoyez's syndrome is presented. The left ear having reached a stabilized hearing loss about 9 years after the onset of the disease, the right ear, apart from some isolated early periods of hearing loss, started to show the full extent of clinical symptoms after about 16 years. Electrocochleographic observations are presented. Studies were performed twice in the right ear during a period of strongly fluctuating hearing thresholds, once in an impaired and once in a relatively good condition. Electrocochleography of the stabilized left ear was performed as well. The data are compared with electrocochleographical observations in the left ear in its early fluctuating stage. Variation of cochlear physiological data during the fluctuating stage of the disease shows remarkable correspondence between the two ears. The stabilized Lermoyez ear is shown to have developed considerable hair cell loss, but may still have preserved its endolymphatic hydrops. These findings in Lermoyez's syndrome fit well into the observations reported in patients with Menière's disease.     

Ear involvement in Wegener's granulomatosis*

In 16 of 19 patients with biopsy-proved Wegener's granulomatosis the early manifestations were limited to the ear and nose. the audiological data of 13 patients revealed middle ear involvement in 16 of 26 ears. Twenty-one of 26 ears presented with a low to moderate sensorineural hearing loss. One ear remained deaf after a sudden hearing loss in the early stage ofthe disease. Serologically, 4 of 6 tested patients with sensorieneural hearing loss demonstrated antibodies against sarcolemma. One patient showed antinuclear antibodies. It is remarkable that these antibodies can often be detected in classic inner ear disorders. The course of inner ear function, serum findings and the success of immunosuppressive therapy in Wegener's granulomatosis are comparable with immunologically mediated vasculitis in the inner ear.     

Otologic surgery in patients with one hearing ear only

On occasions, the otologist is confronted with the possibility of performing surgery on the ear of a patient with useless or no hearing in the opposite ear. Because any surgical procedure on a patient's ear may result in further loss of hearing or even total deafness in the operated ear, an ill-advised operative procedure in such a patient may result in a catastrophe. Up to 2 percent of patients undergoing surgery for otosclerosis develop further severe sensori-neural hearing losses in the operated ear. Idiopathic sudden deafness in patients following stapedectomy can occur for causes unrelated to surgery. Further loss of hearing can also occur after simple myringoplasty and tympanoplasty. Such a possibility must be considered and to a great degree should influence the otologist's decision as to whether or not surgery is indicated, and if so, what is the surgical procedure of choice? A deaf patient can be compared to a blind patient only superficially, and not at all when it comes to any consideration of surgery upon the remaining organ of hearing and vision. Consider the patient with total blindness in one eye who cannot be helped with glasses. Should blindness of any degree affect the remaining eye, an operative procedure which can restore vision may be the only recourse. On the other hand, should the patient with only one hearing ear lose some hearing acuity in that ear, it may always be corrected or helped by the use of amplification. Therein lies the great difference. Total deafness cannot be helped by a hearing aid, and such a catastrophe cannot be risked when any other choice is available. This must be the guiding principle of all otologic surgeons in such instances.     

A case of Meniere's disease with vertical nystagmus after administration of glycerol

A 62-year-old woman who complained of repetitive vertigo with a left fluctuating hearing loss was admitted to our hospital three times. A glycerol test was done on the third admission. Two hours after the administration of glycerol, the patient complained of rotatory vertigo with a downbeat vertical nystagmus. This nystagmus then changed its direction upward. Her left hearing loss was improved during the glycerol test. On the day after the glycerol test, a caloric test was done. The caloric response of the right ear was remarkably improved. Although she had not felt a hearing loss in the right ear, an overwritten audiogram showed a fluctuation of hearing in the right ear. The bilateral caloric responses fluctuated. From these findings, it appears that the function of the inner ear on both sides fluctuating. We speculate that the administration of glycerol elicited an asymmetry in the function of the inner ear on both sides and ocuured vertigo with vertical nystagmus because of the irritation of both ears.     

陈旧性乳突术后全中耳重建术的听力变化

目的：探讨利用自体软骨进行听骨链成形的全中耳重建术患者听力恢复的可能性。方法：对65例(69耳)慢性化脓性中耳炎乳突手术后出现复发性耳漏再手术者，术中同时利用自体软骨进行Ⅲ或Ⅳ型鼓室成形术。比较手术前后听力的变化。结果：全中耳重建术后45耳(65．2％)成功提高了听力，Ⅲ型者听力成功率为74．2％(23耳)；Ⅳ型者听力成功率为57．9％(22耳)。全部患者平均听力提高10．6dB nHL。结论：全中耳重建术可有效地提高陈旧性乳突手术后患者的听力，存在镫骨者的听力成功率要好于镫骨缺失者；二期手术可更明显提高听力。     

Preliminary evidence of asymmetry in uncomfortable loudness levels after unilateral hearing aid experience: evidence of functional plasticity in the adult auditory system

The aim of the study was to compare uncomfortable loudness levels (ULLs) in a group of adults before and after unilateral hearing aid experience. Twelve participants presented with a symmetrical hearing loss consistent with natural ageing. Pure tones were presented to each ear separately, commencing at 60 dB HL and increased in 5-dB step sizes until ULL was reached. The post-fitting ULLs were typically measured three years after fitting. Hearing thresholds were symmetrical and remained unchanged after fitting. Mean ULL values were symmetrical before fitting. The mean ULL values increased (i.e. greater tolerance) in both ears after fitting; however, the increase was greatest in the fitted ear: 14.5 and 7 dB at 2000-4000 Hz in the fitted and not-fitted ear, respectively. A separate two-factor repeated ANOVA (ear and frequency) was performed on the pre and post-fitting ULL data. There was no statistically significant difference for ear (p > 0.05) when comparing the pre-fitting ULLs. However, there was a statistically significant difference for ear (p < 0.01) when comparing post-fitting ULLs. The underlying mechanism for the asymmetry is unknown but it is consistent with learning induced reorganization within the auditory system.