Perilymphatic fistula in pediatric patients with a preexisting sensorineural loss

A specific group of 28 children (38 ears) with a preexisting sensorineural hearing loss (SNHL) was studied to determine if a perilymphatic fistula (PLF) was the cause of a progression in their hearing loss. A PLF was identified surgically in six of these ears, but there was no evidence of any defect found in 32 ears. The symptoms shared by the cases in which a fistula was found included a traumatic event (including exertion), CT scan evidence of inner ear defects, hearing threshold decreases of 25 to 30 dB, and vertigo or postural instability.     

Perilymph fistulas in children: experience of the Otologic Medical Group

We reviewed records of 86 consecutive fistula explorations over 12 years. Four cases were children under age 18. There were 35 fistulas, all in adults. Since this series, we have identified five more children with fistula explorations for a total of nine patients. Two had bilateral explorations. Presenting symptoms were hearing loss and dizziness. None of the children had a definite fistula. Of the nine patients, one patient had a significant improvement in hearing postoperatively, five had no change, and two had worse hearing. There was no follow-up in two patients or in the second ear of a bilateral case. We feel that a fistula should be considered in any case of progressive or fluctuating sensorineural hearing loss, especially in cases with a congenital inner ear deformity. In such cases, an exploration may be reasonable to rule out a fistula. Otherwise, we are hesitant to explore patients for fistulas regardless of whether they are children or adults. Sudden, progressive, and fluctuating sensorineural hearing loss, dizziness, and meningitis have been attributed to perilymph fistulas in both adults and children. The literature reports fistulas in all types of conditions. The incidence and degree of success of treatment have varied widely. When Goodhill first reported round window rupture as a cause of sudden sensorineural hearing loss (SNHL), the Otologic Medical Group (OMG) began routine exploration of all cases of sudden SNHL for the presence of fistulas. After 50 consecutive cases were explored and no fistulas were found, we became selective in our exploration candidates.(ABSTRACT TRUNCATED AT 250 WORDS)     

Fluctuating and/or progressive sensorineural hearing loss in children

Sensorineural hearing loss (SNHL) which is sudden in onset, fluctuating, and/or progressive complicates medical management, hearing aid selection, and individualized educational planning for a hearing-impaired child. In spite of multidisciplinary evaluation and intervention, a gradual decrease in auditory acuity may continue unabated in a significant number of cases. Intercurrent middle ear disease and inconsistent audiologic technique can account for threshold variation in some cases. The study population consisted of 229 children (132 boys; 97 girls) aged 1 to 19.9 years at first audiogram which revealed at least a mild degree of sensorineural hearing loss in one or both ears (35 unilateral), and who demonstrated threshold variation of 10 dB or more in at least one ear at one or more of the standard audiometric test frequencies (250, 500, 1000, 2000, 4000, and 8000 Hz) and were without concurrent middle ear disease (mean length of follow-up, 4.9 years; mean number of audiograms, 10.3). Of 365 ears demonstrating threshold variation of 10 dB or more, 22 (6%) had purely progressive losses without intercurrent upward fluctuation, 208 (57%) had fluctuating thresholds with gradually progressive losses, and 135 (37%) had intermittent threshold fluctuation without permanent deterioration. The probability of contralateral threshold fluctuation if one ear fluctuated was 0.91, while the probability of contralateral progressive SNHL if one ear progressed was 0.67. Demographic data, presumptive etiology, degree of initial SNHL, audiometric configuration, and symmetry of threshold variation were considered as potential predictors of the likelihood of threshold fluctuation and/or progression.     

Pediatric fluctuating sensorineural hearing loss: problems in medical management

OBJECTIVES: To discuss the diagnosis and management of children with fluctuating sensorineural hearing loss, especially focusing on those problems dealing with autoimmune inner ear disease. STUDY DESIGN: A retrospective chart review of a large pediatric otolaryngology practice. A series of 40 children with progressive hearing losses was identified. Of that group, 22 children, aged 1.5 to 12.2 years at first audiogram, were considered to have fluctuating sensorineural hearing loss (FSNHL). Criteria for inclusion in the FSNHL group were threshold variations of 15 dB or more in at least one ear at two or more of the standard audiometric frequencies on at least 2 testing days. METHODS: Charts were reviewed for age, sex, otologic history, laboratory evaluations, medical or surgical treatments, significant medical history, and family medical history. RESULTS: Twenty-two children met the criteria for fluctuating sensorineural hearing loss. Of those with fluctuating hearing loss, 15 were idiopathic, 3 had positive lymphocyte transformation tests (LTT) suggestive of autoimmune inner ear disease (AIED), and 4 had fistula on middle ear exploration. Average fluctuation for all groups was 29.1 dB. Average duration of fluctuations was 4.95 years. CONCLUSIONS: The majority of pediatric FSNHL cases (15 of 22) were idiopathic in nature. Of those FSNHL children with positive LTTs, only one was treated with steroid therapy. In the other patients with positive LTTs, parents or other physicians were often reluctant to treat, or the patient was lost to follow-up. Mean fluctuations varied substantially across all standard audiometric frequencies for all groups.     

Sensorineural and Conductive Hearing Loss Associated With Lateral Semicircular Canal Malformation

Objective Lateral semicircular canal (LSCC) malformation is one of the most common radiological inner ear malformations. Traditionally, inner ear malformations are thought to be associated with sensorineural hearing loss (SNHL). Recent experience with patients with LSCC malformation suggested that LSCC malformation may be associated with both SNHL and conductive hearing loss (CHL). The auditory phenotype associated with LSCC malformation is not well delineated. The objective of this study is to define the nature of the hearing loss associated with LSCC malformation. Study Design Retrospective review Methods Retrospective review of clinical records, audiological evaluation, and imaging studies. Results Two patients with unilateral and 13 patients with bilateral LSCC malformation were identified. LSCC malformation was associated with CHL in 14% (4 ears), SNHL in 71% (20 ears), normal hearing in 11% (3 ears) and CHL due to atresia in one ear. Hearing loss varied from mild to profound but did not correlate with the severity of LSCC malformation. In bilateral malformation, the hearing loss was asymmetric in half of the cases. Malformation of the posterior limb of the LSCC was always associated with a large vestibular aqueduct. An absent or rudimentary LSCC was invariably associated with a cochlear abnormality. Conclusions LSCC malformation, like other inner ear malformations such as large vestibular aqueduct and X‐linked mixed deafness with perilymph gusher, can be associated with CHL, SNHL, or normal hearing. Children with unexplained conductive hearing loss often undergo exploratory surgery to improve hearing. Given that inner ear malformations may be associated with a pure CHL, it is critical that children undergo computed tomography scan of the temporal bone prior to undergoing exploratory surgery.     

遗传性无综合征耳聋一家系畸变产物耳声发射测试

目的 探索遗传性进行性无综合征耳聋患者的听功能改变特点。方法 一个常染色体显性遗传进行性无综合征耳聋家系５２名成员及听力正常组１５名,进行了纯音测试及畸变产行耳中发射（ｄｉｓｔｏｒｔｉｏｎ ｐｒｏｄｕｃｔ ｏｔｏａｃｏｕｓｔｉｃ ｅｍｉｓｓｉｏｎｓ,ＤＰＯＡＥ）测试。结果 无综合征耳有系中３４名成员纯音测试为感音神经性夺聋,其中纯音听阀均值（ｐｕｒｅ ｔｏｎｅ ａｖｅｒａｇｅ,ＰＴ）≥４０ｄＢ的５     

Perilymphatic fistula in children with preexisting sensorineural hearing loss

Progression of preexisting sensorineural hearing loss (SNHL) in infants and children has been considered an indication for fistula exploration: is this approach warranted? On exploring 36 middle ears in 26 such children, we found a perilymphatic fistula (PLF) in four ears (11%). Although there was radiographic evidence of inner-ear deformity in one half of these children, a definite fistula was found in only four of 18 radiographically abnormal ears explored (22%). Even in the four patients with a history of an “event” that could implicate a fistula such as exertion or barotrauma, a fistula was found in only one. There are a number of possible causes for progression of a preexisting SNHL, and surgical exploration of the middle ear should not be recommended on the basis of progression alone. Instead, exploration for a suspected PLF should be strongly considered when there is also a history of an “event,” and/or radiographic evidence of inner-ear abnormalities. Even under these conditions, one should be aware that fistulae are not likely to be found.     

Audiometric patterns in ototoxicity of intra-arterial Cisplatin chemoradiation in patients with locally advanced head and neck cancer

This study describes audiometric patterns of ototoxicity in a consecutive series of patients uniformly treated with intra-arterial high-dose cisplatin chemoirradiation for advanced cancer of the head and neck. Air conduction thresholds were measured from 0.125 to 16 kHz and bone conduction thresholds were measured from 0.5 to 4 kHz. The overall audiometric pattern was characterized by maximum threshold shifts after the 2nd cisplatin infusion and a maximum total threshold shift at 8 kHz, irrespective of gender, age, pretreatment sensorineural hearing loss (SNHL) or subjective complaints during therapy. A hearing deterioration gradient was observed from (ultra-) high to low frequencies, worse with increasing pre-existent SNHL and with increasing cumulative dose of cisplatin chemoradiation. Cisplatin chemoradiation-induced hearing loss seemed to reach a plateau at higher levels (75-80 dB HL) for frequencies above 8 kHz compared to frequencies up to 8 kHz (45-60 dB HL). Recovery of SNHL was found after therapy in 27 ears characterized by extensive hearing loss at frequencies 1, 2 and 4 kHz.     

High frequency hearing following stapes surgery

Abstract Conclusion: Our results indicate that a pre-existing sensorineural hearing loss (SNHL) is not a potential risk factor for further hearing loss in stapes surgery. Objective: The study evaluated whether pre-existing SNHL in otosclerosis constitutes a risk factor for further hearing loss in stapedotomy. Methods: Preoperative and postoperative audiometric evaluation including air (AC) and bone conduction (BC) hearing levels were assessed together with collection of surgical records from 338 consecutively operated cases for primary otosclerosis using a database. Patients were operated by the same surgeon between 2000 and 2006. In all, 291 patients were operated on 1 side and 47 patients were operated on both sides. Ages ranged from 16 to 76 years. Stapedotomy was performed in all cases except five (stapedectomy). Cases were separated into four different groups based on preoperative AC hearing levels at 4, 6, and 8 kHz: group I, <30 dB HL; group II, 30-50 dB HL; group III, 51-70 dB HL; group IV, >70 dB HL). Results: Hearing deterioration at 4, 6, and 8 kHz (>10 dB) was observed in 6.5% of all cases. Patients with normal preoperative hearing were found to be more prone to further SNHL 4, 6, and 8 kHz (range 13-25 dB) at surgery, while patients in group IV, with preoperative SNHL impairment, remained unaffected.     

Profile and stability of sensorineural hearing loss in persistent pulmonary hypertension of the newborn.

The purpose of this study was to examine the incidence, profile, and stability of sensorineural hearing loss (SNHL) in infants diagnosed with persistent pulmonary hypertension of the newborn (PPHN). Over a five-year period, 19 of 51 infants (37%) with PPHN were diagnosed with SNHL: 16 with bilateral and 3 with unilateral impairment. This incidence of SNHL is approximately 25 times greater than observed in graduates of our intensive care nursery. The profile of SNHL in the group of 19 children was typically downward sloping. However, there was considerable variation with respect to degree of loss. In addition, progressive SNHL was identified in 5 children whose hearing worsened an average of 55 dB at 2 to 4 kHz. On retrospective analysis, the perinatal variables associated with PPHN were comparable between hearing-impaired and normal-hearing infants. In contrast, the two groups were significantly different when treatment variables, such as the duration of mechanical ventilation or amikacin, were compared. Children with SNHL were treated for longer durations as compared to normal-hearing infants. Children with SNHL were subdivided into two groups, mild and severe, based on degree of loss and treatment variables, and compared again to the normal-hearing group. Two findings emerged from this analysis. First, the duration of hyperventilation was now the only variable significantly different between children with mild SNHL and children with normal hearing. Second, comparison of the mild with the severe SNHL groups showed that the duration of hyperventilation was similar. The pathophysiology and underlying mechanisms resulting in SNHL in PPHN survivors are discussed.     

Longitudinal investigation of hearing disorders in children with congenital cytomegalovirus

This investigation consisted of a longitudinal study of the effects of congenital cytomegalovirus (CMV) infection on hearing sensitivity in 860 children with documented asymptomatic or symptomatic congenital CMV infection. Of the 651 children with asymptomatic CMV infection, 48 (7.4%) developed sensorineural hearing loss (SNHL), compared to 85 (40.7%) of the children with symptomatic CMV infection. Children in both groups experienced latent effects consisting of delayed onset of loss, threshold fluctuations, and/or progressive loss of hearing. It can be concluded that congenital CMV infection is a leading cause of SNHL in children. The late onset and progression of loss necessitates continued monitoring of hearing sensitivity in this population.     

Gellé test in patients of Menière's disease and fluctuating low tone hearing loss

The threshold shift at 500 Hz bone conduction under positive and negative static pressure of 400 mmH2O applied to the external auditory canal was examined in 20 patients of Meniere's disease with fluctuating low tone hearing loss, 21 patients of sensorineural hearing loss without fluctuating and 24 normal controls. In contrast to the 10 to 15 dB threshold shift in the latter two groups (non-fluctuating group, 14.8 +/- 5.1 dB at positive and 11.1 +/- 5.7 dB at negative; normal controls, 13.6 +/- 5.4 dB at positive and 9.3 +/- 5.3 dB at negative), the former showed a significantly smaller shift (3.2 +/- 6.1 dB at positive and 2.3 +/- 4.5 dB at negative) while hearing was depressed. However, when the hearing was improved, the pressure effect became greater even in the former group (11.3 +/- 5.3 dB at positive and 9.1 +/- 4.8 dB at negative). To understand further details of these phenomena, we performed electrophysiological animal experiments. Through a small hole made on the guinea pig's cochlear bony wall, 300 mmH2O pressure was applied to the scala tympani and the threshold of 500 Hz CM was examined in both endolymphatic sac obliterated ears and non-obliterated ears. In 14 non-obliterated ears 21.1 +/- 5.1 dB threshold shift was recorded, whereas it was only 1.6 +/- 2.6 dB in 9 obliterated ears at 4-6th postoperative days. These results seem to reflect that the endolymphatic pressure is great during the early stage of hydrops.(ABSTRACT TRUNCATED AT 250 WORDS)     

Otologic and audiologic features of Nager acrofacial dysostosis

OBJECTIVE: To describe the otologic and audiologic characteristics of pediatric patients with Nager acrofacial dysostosis. DESIGN: Retrospective case series. SETTING: Multidisciplinary clinic in a tertiary care children's hospital. SUBJECTS: Patients less than 18 years of age with Nager acrofacial dysostosis. METHODS: Nager syndrome is a mandibulofacial dysostosis associated with preaxial limb abnormalities and multiple craniofacial anomalies. Ten patients with Nager syndrome were reviewed. Relevant literature, 1966 to the present, was reviewed with the assistance of Medline. RESULTS: External and middle ear abnormalities are common in Nager syndrome. All non-atretic ears had significant difficulty with otitis media, requiring an average of two sets of tympanostomy tubes. Cholesteatoma was diagnosed in one patient. Pure conductive hearing loss was identified in eight patients with mixed hearing loss noted in two patients. Conductive hearing loss greater than 30 dB HL was noted in 90% (9/10) of patients, with 40% (4/10) having 55-70 dB HL loss. Although amplification was effective, results of surgical interventions to correct conductive hearing loss were inconsistent. Two patients with mixed hearing loss developed the sensorineural component in later childhood, indicating that progressive or fluctuating sensorineural hearing loss is also possible in this population. CONCLUSIONS: Pediatric patients with Nager acrofacial dysostosis exhibit conductive hearing loss due to middle and external ear pathology. Prolonged ventilation of the middle ear via tympanostomy tubes and amplification with hearing aids are often required. Some patients also demonstrate mixed hearing loss that may be progressive and should be monitored carefully. Early and aggressive management in a multidisciplinary team approach is recommended.     

Characteristics of transient evoked otoacoustic emissions in normal-hearing and hearing-impaired children

OBJECTIVE: Transient evoked otoacoustic emissions (TEOAEs) were measured in children with normal hearing and in children with hearing loss to investigate the characteristics of TEOAEs as they relate to overall amplitude and amplitude spectra of evoking stimuli, and to audiometric status. DESIGN: Three parameters of response measure (signal to noise ratio, amplitude and reproducibility) were assessed to determine accuracy of identification of varying degrees of hearing loss for broadband clicks, frequency-specific click bands and tone bursts. Forty-four children (66 ears) between 4 and 13 yr of age were evaluated for participation in the study. Fifty-nine ears with intact tympanic membranes were included in the final analyses: 14 ears with normal hearing and 45 ears with hearing loss. RESULTS: Children with normal hearing had robust responses that displayed nonlinear growth functions for broadband clicks and for tone bursts. Children with hearing loss had responses that decreased rapidly with decreasing stimulus level, if emissions were present at all. Data were analyzed using clinical decision analysis and receiver operator characteristic curves. Broadband clicks presented at 80 and 86 dB peSPL identified a hearing loss > or = 30 dB HL with a high degree of accuracy. Click responses filtered into octave bands centered at 500 and 1000 Hz did not improve classification of hearing loss, in fact, the 500 Hz band was particularly inaccurate. Results for click responses filtered into half-octave bands centered at 2000 and 4000 Hz were comparable with those for the broadband click, although the 2000 Hz band was superior for identification of hearing loss > or = 20 dB HL for an 80 dB peSPL click, and > or = 30 dB HL for an 86 dB peSPL click. Results for tone bursts centered at 500, 1000, 2000 and 4000 Hz, presented at 80 dB peSPL, were similar to results of the filtered click bands. Accuracy for identifying hearing loss increased with increasing center frequency. The 2000 and 4000 Hz tone bursts provided the best separation between normal-hearing and hearing-impaired ears, with 4000 Hz being slightly better. CONCLUSIONS: Data from this study suggest that TEOAEs in children can separate ears with normal hearing from those with hearing loss using a variety of stimulus and response conditions. Moreover, by using multiple stimulus levels it may be possible to distinguish between mild and moderate hearing losses.     

Autoimmune Sensorineural Hearing Loss in Young Patients: An Exploratory Study

Objectives P0 protein is expressed exclusively in myelinating Schwann cells of the peripheral nervous system. In a previous study from our laboratory, 27% of patients with sensorineural hearing loss (SNHL) had antibodies to P0 protein in their serum. The purpose of the present exploratory study was to examine the relationship between the clinical presentation of SNHL among children and young adults (age range, 5–30 y) and the presence of serum anti‐P0 antibodies. Study Design The data were collected by retrospective questionnaires from Belgian otolaryngologists. Methods Patients were divided for comparison into two groups according to the presence or absence of anti‐P0 antibodies. Results Analyses of clinical data and audiometric results indicated that a progressive hearing loss was more frequently recorded in the patients in the anti‐P0 antibody–positive group (82% [14 of 17]) than in those in the anti‐P0 antibody–negative group (35% [6 of 17]) (P <.005). Conclusions Thus, in the age group in the present study, autoimmune SNHL (as measured in the present study by the presence of anti‐P0 antibodies) is more frequently associated with progressive than with sudden hearing loss. The implications of this finding for preventive screening of hearing loss in children and young adults are discussed.     

Electrocochleographic findings in cases of autoimmune disease with sensorineural deafness

Objectives: To present electrocochleographic findings in patients with autoimmune disease (AD) with sensorineural deafness (ADSD), and to discuss the etiologies of sensorineural hearing loss (SNHL) in cases of ADSD. Methods: Study design is a retrospective review of electrocochleographic results of 26 patients with ADSD. To evaluate the electrocochleographic results, average SP/AP ratios were compared between ADSD and normal subjects. In the ADSD group, audiologic pattern, fluctuations in hearing and results of the glycerol test were also reviewed. Electrocochleography (ECoG) was recorded using the extratympanic method, and the SP to AP ratio (SP/AP ratio) was analyzed. Results: The mean of average SP/AP ratios in the ADSD groups (0.46) was significantly higher than that in normal subjects (0.27). Further, 17 of 29 affected ears in patients with ADSD showed fluctuating hearing loss. Eighteen ears showed low tone loss (rising and peak audiologic patterns). Only 5 of 26 ears (19.2%) showed positive results on glycerol test. There was no correlation between glycerol test results and hearing fluctuation or between glycerol test results and the SP/AP ratio on χ²-test. There was no tendency between audiologic pattern and glycerol test results or between audiologic pattern and the SP/AP ratio. Conclusion: These results suggest the etiologies of SNHL in cases of ADSD remain unclear. However, some cases showed clinical findings similar to endolymphatic hydrops. We should bear in mind that clinical Meniere’s syndrome involves ADSD. Further investigation is needed to resolve the etiology of SNHL of ADSD.     

Diagnosis of sensorineural hearing loss with neural networks versus logistic regression modeling of distortion product otoacoustic emissions

We investigated whether modeling with artificial neural networks or logistic regression of distortion product otoacoustic emissions (DPOAE), across diverse frequencies, may achieve an accurate diagnosis of sensorineural hearing loss (SNHL) of cochlear origin. 256 ears (90 with SNHL and 166 with normal hearing) were evaluated with pure-tone audiometry, impedance audiometry, speech audiometry and DPOAE. Ears were split into training (n = 176) and validation (n = 80) sets. Input variables included gender, age, examination time, DPOAE intensity at F(2) frequencies 593, 937, 1906, 3812 and 6031 Hz, and respective values corrected for noise levels. In the validation data set, an average network had an area under the receiver operating characteristic curve (AUC) of 0.86 (accuracy 84%). Logistic regressions including all these variables or those selected by backward elimination had AUC values of 0.91 and 0.92, respectively (accuracy 85% both). Eleven of 12 trained networks had better specificity than the backward elimination logistic regression, and the backward elimination logistic regression had a better sensitivity than 11 of the 12 networks. Both modeling approaches correctly identified all ears with sudden hearing loss, congenital hearing loss, head trauma, nuclear jaundice and ototoxicity, and 2-3 of 5 ears with acoustic trauma, but missed 1-3 of 3 ears with Ménière's disease and 4-6 of 8 ears with abnormal pure-tone thresholds on audiometry which had no accompanying findings. For SNHL exceeding 45 dB HL on a pure-tone threshold, sensitivity was 83% (15/18) by neural networks and 84 or 94% (16/18 or 17/18) by logistic regression. Both neural-network-based analysis and logistic regression modeling of the DPOAE pattern across a range of frequencies offer promising approaches for the objective diagnosis of moderate and severe SNHL.     

Middle ear impedance measurements in large vestibular aqueduct syndrome

Objective

To assess the effect of inner ear pressure on middle ear impedance in patients with large vestibular aqueduct syndrome (LVAS).

Methods

Data from admittance tympanometry and multifrequency tympanometry on 8 LVAS patients and control subjects were studied.

Results

Static acoustic compliance (SAC) values for the ears with stable sensorineural hearing loss (SNHL) were within the limits of the mean values of control groups except for two ears. The resonance frequency (RF) values of the ears with stable SNHL were lower than the mean values of control groups except for three ears. SAC values for the two ears with fluctuating SNHL were lower and the RF values were higher than the mean values of control groups.

Conclusion

Decreased SAC values and increased RF values found in the ears with fluctuating SNHL might be an indirect indicator of increased inner ear pressure, while low RF values in the ears with stable SNHL might reflect the decreased inner ear impedance.     

Severe progressive sensorineural hearing loss improved after removal of large jugular foramen schwannoma

We report a very rare case of hearing improvement after removal of the intracranial part of a jugular foramen schwannoma (JFS) presenting with chronic and severe progressive sensorineural hearing loss (SNHL). The patient presented with progressive hearing impairment in his right ear, lasting 2 years. The patient's pure tone audiogram revealed severe SNHL. His speech discrimination score (SDS) was 0%. Auditory-evoked brain responses (ABRs) comprised only I waves following 30-100 dB stimulation, although distortion-product otoacoustic emissions (DPOAEs) had good responses. These test results indicated that his hearing impairment was retrocochlear SNHL. Magnetic resonance imaging revealed within the right jugular foramen a large intracranial-extracranial tumor that compressed the brainstem. The intracranial part of the tumor was resected through retrosigmoidal craniotomy, and the tumor was pathologically diagnosed as a schwannoma. Several months after the operation, the patient's auditory thresholds improved to a level consistent with mild SNHL, ABR V waves emerged following 60-90 dB stimulation, and SDS improved significantly to 95%. This case demonstrates that hearing improvement can be achieved after surgery for JFS presenting with severe and chronic progressive SNHL, and that good DPOAE responses and the presence of ABR I waves may be predictors of postoperative hearing recovery in JFS.     

耳蜗死区对感音神经性听力损失患者言语识别能力的影响

目的 探究耳蜗死区在感音神经性听力损失(sensorineural hearing loss,SNHL)患者中的存在情况及其对言语识别能力的影响.方法 采用纯音听阈测试筛选出41例(81耳)感音神经性听力损失患者,经均衡噪声阈值测试将患者分为有耳蜗死区组(35耳)和无耳蜗死区组(46耳),分别进行言语识别阈(SRT)和言语识别率(SDS)测试,分析81耳耳蜗死区的分布及其对言语识别能力的影响.结果 41例(81耳)感音神经性听力损失患耳中有35耳(43.21％,35/81)存在耳蜗死区,其中轻度SNHL患耳耳蜗死区检出率为0(0/11),中度SNHL患耳耳蜗死区检出率为24.1％(7/29),重度SNHL患耳耳蜗死区检出率为66.7％(24/36),极重度SNHL患耳耳蜗死区检出率为80.0％(4/5),不同听力损失程度耳耳蜗死区检出率差异有统计学意义(P＜0.05);高频耳蜗死区(16耳)明显多于低频耳蜗死区(8耳),但两者的言语识别能力差异无统计学意义(P＞0.05);有耳蜗死区患者的言语识别阈及言语识别率分别为61.63±16.76 dB HL,86.35％±12.03％,无耳蜗死区的患者分别为75.54±9.56 dBHL,64.97％±20.84％,二者间差异有统计学意义(P＜0.05).结论 听力损失越重,耳蜗死区检出率越高;高频耳蜗死区较低频常见,且存在耳蜗死区的感音神经性听力损失患者言语识别能力明显低于无耳蜗死区的患者.