Acoustic neuroma presenting with sudden and fluctuating hearing loss--a case report

A case of acoustic neuroma presenting with sudden and fluctuating hearing loss is reported. The patient was a 38-year-old Japanese woman who noticed a sudden onset of hearing loss and tinnitus in the right ear. With a provisional diagnosis of sudden deafness, she was subjected to conservative therapy, including steroid hormone. The hearing started to recover and reached a normal level 2 months after the treatment. Meanwhile, the results of an ABR and plain skull X-rays aroused a suspicion of acoustic neuroma. She was operated on via a middle cranial fossa approach and an acoustic neuroma occupying the internal auditory meatus was removed.     

Acute inner ear deafness]

Sudden inner ear hearing loss initially might suggest a psychogenic disorder of hearing, particularly when it is bilateral and simultaneous. The differential diagnosis includes disseminated encephalitis, syphilitic labyrinthitis and Cogan's syndrome. The history and cause of acute bilateral deafness in meningitis are easy to recognise. Furthermore, unilateral acute inner ear deafness should not be regarded as idiopathic without further consideration. A acoustic neuroma is a possible cause even of a low-tone hearing loss. More controversial is rupture of the round window membrane as a cause of sudden deafness. The deafness after epidemic parotitis obviously leads to a total unilateral hearing loss in every case. Even labyrinthine apoplexy with loss of hearing and vestibular function can be caused by a tumour of the cerebellopontine angle. Idiopathic sudden deafness should be defined as an acute sensory hearing loss whose anatomical basis in an acute vascular endolymphatic hydrops of unknown cause. The sudden deafness affects only one ear; tinnitus and brief vertigo can be accompanying symptoms. A sudden hearing disorder due to other causes should be distinguished from idiopathic lesions.     

突发性聋患者耳鸣程度及耳鸣疗效的相关因素分析

目的 研究影响突发性聋伴耳鸣患者耳鸣严重程度及远期疗效的相关因素.方法 回顾性分析2017年3月～2019年5月荆州市中心医院收治的106例单侧突发性聋伴耳鸣患者随访半年临床资料.观察耳鸣严重程度及耳鸣疗效与性别、年龄、侧别、初始听力损失程度、听力曲线类型、初诊时间、耳聋疗效的关系.结果 耳鸣严重程度与不同性别、年龄、...     

Intratympanic drug therapy with steroids for tinnitus control: a preliminary report

Intratympanic drug therapy (ITDT) is a surgical technique of instilling medication into the middle ear to perfuse the inner ear in treating hearing loss, tinnitus, vertigo, and ear blockage, alone or in combination, in patients with a predominantly inner-ear site of lesion. This preliminary report of ITDT focuses on attempts at tinnitus control (TC). Between November 1997 and February 1999, 10 patients with severe tinnitus were treated with steroid medication and were last seen in February 2000. TC was established in 7 of these 10 patients (70%). The clinical diagnosis of a predominantly cochlear-type tinnitus was established in each patient by a correlation of the clinical history with a medical-audiological tinnitus patient protocol that included cochleovestibular testing. An additional single patient with sudden hearing loss experienced no hearing improvement on ITDT steroid therapy. Vertigo as an associated complaint was reported by 6 of 10 patients with subjective idiopathic tinnitus. Significant control of the associated vertigo complaint was reported by 5 of 10 patients. Duration of tinnitus relief in 7 of 10 patients was hours in 1 of the 7; days in another of the 7; and 1 year or more in 5 of the 7. One of the seven patients reported TC 3 months after the procedure. Complications included tympanic membrane perforation that persisted for more than 6 months in two patients and an increase in the complaint of ear blockage and tinnitus intensity in one patient. In our preliminary study, ITDT with steroid has resulted in both short- and long-term tinnitus relief in 7 of 10 patients (70%) identified to have a predominantly cochlear-type tinnitus.     

Acute low tone sensorineural hearing loss caused by inner ear barotrauma

Three cases of inner ear barotrauma with subjective symptoms and hearing impairment which were similar to the low tone sudden deafness were reported. Case 1 was a 34-year-old man who developed a hearing loss in the next morning of taking an airplane and recovered four days after. Case 2 was a 42-year-old man who developed a hearing loss 2 days after flying in an airplane and hearing loss have recurred 4 times in his right ear for 3 months. Eight months after recovery of previous recurrent attack, a hearing loss occurred in his left ear without flying and recurred twice for 3 weeks. Case 3 was a 25-year-old woman who developed a hearing loss in the right ear after 24 meter depth scuba diving and recurred 4 times for 40 days. Those three patients complained of no vertigo at any attacks and were treated conservatively. From previous reports and the onset and the course of hearing disturbance, acute low tone sensorineural hearing loss in case 1 and case 2 was thought to be caused by circulatory disturbance of the inner ear and in case 3 thought to be caused by inner ear window rupture. But, endolymphatic hydrops was also needed to be take into account in those three cases as a common possible cause. Inner ear barotrauma and so called labyrinthine window rupture were considered to be one of the diseases needed to differentiate from low tone sudden deafness without reference to mono-attack type or recurrent type.     

听神经瘤误诊分析

对经耳鼻咽喉科首诊，最终由神经外科手术全切或部分切除的经病理证实的７２例听神经瘤进行早期误诊分析，误诊时间平均５．５年，误诊病种为神经性耳聋、突发性耳聋、神经性耳鸣、颈椎病、鼻咽癌等。文中重点讨论了误诊原因。     

Rundfensterruptur – Nachweis mittels Fluoreszenzendoskopie

Rupture of the round window membrane as a special cause of inner ear deafness is widely accepted after changing pressure levels, e.g. in diving. However, even without a barotrauma before, the spontaneous rupture of the round window membrane is suspected occasionally in patients with sudden hearing loss and/or vertigo and tinnitus. To carry through the tympanotomy is decided by ENT surgeons often in cases of progressive hearing loss despite infusion therapy. Perilymph fistulas have been detected relatively seldom, compared to the number of reported operations by several authors. However, covering the round niche with connective tissue leads to the improvement of symptoms sometimes even in cases without microscopical evidence of fistula. Within the last 3 years 14 patients suffering sudden hearing loss of one ear underwent tympanotomy in our department. Of these patients 8 reached restitution of the hearing ability. Especially 2 patients with sudden deafness caused by spontaneous rupture of the round window membrane are reported in the following article. Perilymph fistulas were detected in these cases by IV-application of fluorescein and fluorescence endoscopy of the middle ear. Both patients obtained a normal hearing curve within 1 week after surgical intervention and obliteration of the round niche.     

儿童突发性聋患者的临床特征及预后分析

目的 分析儿童突发性聋(简称突聋)患者的临床特征及预后,为该病的临床诊治提供参考.方法 回顾性分析2008年1月～2016年10月确诊并治疗的23例(25耳)儿童突聋患者的临床资料,对所有患者的年龄、性别、耳别、就诊时间、病毒感染史、就诊时言语频率气导纯音听阈及听阈曲线类型、是否伴有耳鸣、眩晕、耳闷及治疗效果进行统计分析,并与同期住院治疗的202例(219耳)成人突聋患者进行对比.结果 本组儿童突聋患者占同期所有突聋患者的10.2％(23/225);儿童突聋患者就诊时平均听阈(87.7±16.1 dB HL)、全聋型比例(72.0％)、眩晕伴发率(52.2％)及病毒感染率(17.4％)均高于成人(分别为72.5±24.7 dB HL、44.7％、29.2％、3.5％),差异均有统计学意义(均为P＜0.05).治疗后儿童突聋患者与成人患者的总有效率分别为52.0％、46.6％,痊愈率分别为4.0％、14.2％,差异均无统计学意义(P＞0.05).结论 本组儿童突聋患者就诊时听力损失较重且常伴有耳鸣及眩晕,其听阈曲线以全聋型为主;疗效与成人突聋患者相当.     

梅毒性感音神经性聋的临床分析

目的:探讨梅毒性感音神经性聋的临床表现、诊断和治疗.方法:回顾性分析3例梅毒性感音神经性聋患者的临床症状、体征、血清学及治疗.结果:3例梅毒性感音神经性聋患者中,2例RPR和TPPA均阳性;1例RPR阴性,TPPA阳性;2例在外院已确诊为梅毒并行驱梅治疗;2例为突发性聋起病,伴有耳鸣,1例为耳鸣起病伴高频听力下降.均已排除了其他原因所致的:千聋耳鸣,治疗后听力无改善.结论:梅毒性感音神经性聋可以突发性聋或耳鸣发病,易漏诊.对病因不明的听方下降,伴有耳鸣、眼球震颤的患者,应行必要的实验室检查以明确诊断.     

Study on tinnitus masking--an evaluation of ipsilateral and contralateral masking for tinnitus

In this study, the tinnitus masking curves measured by the ipsilateral and contralateral masking test were evaluated for the unilateral tinnitus cases of following 4 groups; 40 cases without hearing loss, 22 cases with symmetrical sensorineural hearing loss, 12 cases with unilateral deafness, and 70 cases with unilateral sensorineural hearing loss. Then the characteristics of tinnitus masking curves and central masking phenomenon for tinnitus masking were investigated. Consequently, tinnitus was masked by a masking tone given from nontinnitus ear when it reached at some definite loudness level, in spite of the presence of hearing loss or the degree of hearing loss in tinnitus ear. Then it was suggested to be a influence of central masking phenomenon in the contralateral tinnitus masking.     

1158例突发性聋患者听力情况分析

目的分析大样本突聋患者听力损失情况。方法对1158例患者（1203耳）进行病史询问及听力学检查,按听力损失频率及损失频率平均听阈分类,部分患者行颞骨CT及头颅MRI检查。结果1158例患者中14．3％为低、中频听力下降,6．2％为中、高频听力下降,15．1％为高频听力下降,4．4％为低、高频听力下降,60．0％为全频听力下降。1158例中查出1例听神经瘤,2例大前庭水管综合征。结论突聋患者听力损失情况不一,以全频下降为主,应注意排除相关疾病。     

Unilateral sudden hearing loss as the first sign of chronic myeloid leukemia

Chronic myeloid leukemia (CML) is one of the etiologic causes of sudden hearing loss and vertigo. However, deafness in association with vestibular symptoms rarely occurs in CML as the first sign. In this article, a 50-year-old male with CML whose first signs and symptoms were unilateral sudden hearing loss and tinnitus in the right ear, vertigo and nausea was presented. Aetiopathogenetic mechanisms, clinical and radiological aspects and therapeutic options for CML with deafness and vertigo were discussed reviewing the literature.     

突发性聋预后影响因素

目的对影响突发性聋的预后因素进行分析讨论。方法回顾分析249例突发性聋患者临床资料,包括年龄、初诊时间,初诊时听力损失程度,听力曲线类型,是否伴有眩晕和耳鸣,进行畸变产物耳声发射(distortion product otoacoustic emissions,DPOAE)检查结果。结果初诊时间为发病后1～23天,患侧耳初诊时250 Hz～4000 Hz平均听力损失40dB以下31例(12.45%),41 dB～70 dB 80例(32.13%);71 dB～90 dB 74例(29.72%),91 dB以上64例(25.70%)。听力曲线上升型72例,下降型81例,平坦型96例。伴有眩晕96例,伴耳鸣174例。治疗药物包括血管扩张剂、皮质类固醇激素、神经营养剂、抗病毒及能量合剂,疗程2～4周。81例进行畸变耳声发射检查,45例在不同频率被引出,经治疗最终被引出DPOAE的频率听力恢复达痊愈水平。结论高龄患者和年龄小的患者预后不良;初诊时间越早听力恢复越好;听力曲线上升型预后好;伴有眩晕者预后不好;能引出DPOAE者听力恢复好。     

Recovery from repeated sudden hearing loss with corticosteroid use in the presence of an acoustic neuroma.

Sensorineural hearing loss of sudden onset may be the presenting symptom in up to 14% of patients with acoustic neuroma. We present the first reported case of sudden hearing loss in an only hearing ear with recovery to normal levels after steroid therapy on four separate occasions. Evaluation revealed a 1.5-cm acoustic neuroma. After middle cranial fossa decompression, a fifth episode with recovery after steroid use was documented. A review of the recent literature is presented, emphasizing the possible causation of sudden sensorineural hearing loss with recovery to normal in patients with acoustic neuroma. Modalities of therapy for the dilemma of the acoustic neuroma in an only hearing ear are discussed, including surgery, radiotherapy, and chemotherapy. An aggressive approach to the evaluation of the cause of sudden hearing loss is suggested.     

Hearing impairment and tinnitus pitch in patients with unilateral tinnitus: comparison of sudden hearing loss and chronic tinnitus

OBJECTIVES: The objectives were to analyze the results of pitch-matching and loudness-balance testing in patients with unilateral tinnitus and to evaluate the relationship between audiological findings based on the tinnitus-affected and tinnitus-unaffected threshold differences and tinnitus pitch by using linear interpolation methods. In addition, the effects of the duration of the tinnitus on this relationship were investigated. Sudden sensorineural hearing loss with tinnitus was selected for the "acute tinnitus" group, and unilateral tinnitus with unknown disease and a duration of more than 3 months was selected for the "chronic tinnitus" group. STUDY DESIGN: Retrospective study of the clinical records of patients. METHODS: One hundred thirty-two patients with unilateral tinnitus (comprising 68 female [51.5%] and 64 male [48.5%] patients) were investigated as subjects. Their mean age was 50.4 years (SD = 15.8 y). All patients underwent otoneurological testing, including the pure-tone audiogram and pitch-matching and loudness-balance tests. RESULTS: The mean difference in the hearing threshold between the tinnitus-affected ear and the tinnitus-unaffected ear was largest near the tinnitus pitch in both the acute and the chronic tinnitus groups. However, the relationship between hearing impairments and tinnitus pitch was somewhat different in the two groups: It exhibited a single smooth peak in the acute tinnitus group but a bimodal peak in the chronic tinnitus group. CONCLUSION: The results suggest that tinnitus is related to hearing impairment in the same frequency region in patients with sudden sensorineural hearing loss with tinnitus or in patients with chronic tinnitus, whereas some instances of chronic tinnitus are caused by reorganization in cortical cells.     

Secondary Menière's disease

Among 93 patients presenting the typical symptoms of a Ménière's disease associating an unilateral fluctuating hearing loss of sensorineural type, tinnitus and vertiginous attacks lasting minutes to hours, 40 patients (43%) presented in their personal history a particular otologic insult in the ear which later on developed into the full Ménière's symptomatology, or a particular systemic disease with otologic manifestations. The Ménière's triad appeared in these patients six months to twenty nine years after the initial otologic or systemic lesion. Among these initial lesions were 16 cases of sudden partial or complete deafness related to viral or bacterial infection, 3 cases of sudden cochleo-vestibular deficit and 1 case of vestibular neuritis, 5 cases of temporal bone fractures and 4 cases of significant acoustic trauma, 2 cases of otosclerosis, 1 case of chronicotitis media and 1 case of severe hearing loss after otologic surgery, 5 cases of meningo-encephalitis and 2 cases of acquired syphilis. These particular lesion could be, in our opinion, the releasing factor of the inner ear dysfonction leading eventually to a secondary Ménière's syndrome.     

Coexistence of acoustic neuroma and pineal region tumor in patient with sudden deafness

INTRODUCTION: Acoustic neuroma usually presents as an unilateral tumor, seldom - bilateral and rarely in coexistence with other central nervous system neoplasms. The following paper reports such a case of a 21-year-old male patient presented with sudden deafness in left ear accompanied with tinnitus and vertigo. Symptoms started 4 weeks prior hospitalization. Their aggravation has been observed 7 days before admission to the hospital. Audiometry revealed moderate sensorineural hearing loss in left ear (for low and middle frequencies), brainstem auditory evoked potentials were absent on the left side and ENG examination showed left peripheral vestibular impairment. Initially patient received i.v. vasodilatators showing 20-25 dB improvement in low frequencies after 3 days of treatment. MRI study revealed in the left internal acoustic meatus mass (7 x 7 x 14 mm) suggesting acoustic neuroma and an oval mass (7 x 9 x 14 mm) in the pineal gland presenting radiological features of pinealoma. Patient has been qualified for neurosurgical treatment. Acoustic neuroma has been removed by suboccipital approach and pinealoma has been left for further observation as it was found incidentally. Histopathological examination confirmed diagnosis of left VIII nerve schwannoma. The left facial palsy (House-Brackmann III/IV grade) and profound hearing loss appeared after surgery. The postoperative course shows no evidence of acoustic neuroma recurrence.     

2171例耳鸣患者的临床特征分析

目的 探讨耳鸣患者的临床特征,为耳鸣诊治提供参考.方法 收集2014年1月至2015年12月就诊于中山大学孙逸仙纪念医院耳鼻喉科门诊以耳鸣为第一主诉的2 171例(2 736耳)耳鸣患者的临床资料,以耳鸣残疾量表(tinnitus handicap inventory,THI)及视觉模拟评分(visual analogue scale,VAS)评估耳鸣的严重程度,并分析耳鸣的发病年龄、耳鸣侧别、听力状况及可能病因等基本特征.结果 2 171例耳鸣患者平均年龄为44.08±15.37岁,其中41~50岁为分布最集中的年龄段(491例,22.60%),其次为31~40岁(445例,20.50%)1~60岁(438例,20.20%);主观性耳鸣2 120例(97.65%),客观性耳鸣51例(2.35%);单侧耳鸣(1 606例,73.98%)多于双侧耳鸣(539例,24.83%), 颅鸣最少(26例,1.19%);2 736侧耳鸣耳8 000 Hz的平均听阈为69.26±27.70 dB HL,高于1 606侧非耳鸣耳(54.16±30.98 dB HL);THI分级以2、3级为主(55.78%,1 211/2 171),VAS分级以轻、中度耳鸣为主(83.83%,1 820/2 171);音调匹配以高频多见(4~8 kHz,1 199例,55.23%);位于前三位的可能病因为不明原因591例(27.22%)、突发性聋549例(25.29%)及中耳疾病270例(12.44%).结论 本组耳鸣患者中以主观性耳鸣为主,中青年者居多,单侧耳鸣多于双侧耳鸣,耳鸣与听力关系密切;引起耳鸣的病因多而复杂,部分患者不明原因或与耳疾有关,习服治疗是值得推广的治疗方法.     

突发性聋伴耳鸣患者耳鸣特征

目的 分析研究突发性聋伴耳鸣患者的耳鸣特点、耳鸣与听力损失的关系、耳鸣对患者的影响程度, 探讨突聋患者耳鸣的产生机制。 方法 突发性聋伴有耳鸣患者231例常规采集病史, 填写耳鸣致残量表(THI)及视觉模拟得分表(VAS), 同时进行纯音听阈测试、耳声发射、耳鸣检测及掩蔽试验等检查。分析患者的耳鸣特点、耳鸣与听力的关系、耳鸣的THI及VAS得分以及它们特点。 结果 ①听力损失特点：低中频下降型20例(占8.7%), 中高频下降型60例(占26.0%), 平坦型44例(占19.0%), 全聋型79例(占34.2%), 不规则型28例(占12.1%);②耳鸣频率:低频耳鸣(≤500 Hz)49例(占21.2%), 中频耳鸣(51～2 000 Hz)54例(23.4%), 高频耳鸣(>2 000 Hz)122例(52.8%), 未匹配6例(占2.6%)。其中听力损失类型为低中频下降型的患者中, 低频、中频、高频耳鸣的比例分别为75%、15%、10%;中高频下降型患者中, 低频、中频、高频耳鸣的比例分别为13.3%、26.7%、60.0%;全聋型患者中, 低频、中频、高频耳鸣的比例分别为17.1%、19.0%、63.3%。听力下降最明显的频率对数与耳鸣频率对数呈线性关系, r=0.592, P<0.01;③耳鸣响度:0～30 dB HL 45例(占19.5%), 31～60 dB HL 60例(占26.0%), 60～90 dB HL 102例(占44.2%), 大于90 dB HL 18例(占27.8%), 未匹配6例(占2.6%)。耳鸣响度与听力损失程度(250～4 000 Hz平均听阈)有相关性, 相关系数r=0.216, P=0.001<0.05。④耳鸣致残级别:按照Newman等依据THI得分将耳鸣残疾分级, 其中1级27例(占11.7%), 2级耳鸣44例(占19.0%), 3级66例(占28.6%), 4级94例(占40.7%)。⑤THI及VAS得分特点:THI得分与听力损失程度无相关性r=0.087, P=0.287>0.05。VAS得分与听力损失程度无相关性r=0.002, P=0.982>0.05。THI得分与耳鸣频率对数无相关性, 相关系数r=-0.056, P=0.402>0.05。VAS得分与耳鸣频率对数无相关性, 相关系数r=-0.003, P=0.970>0.05。THI得分与耳鸣响度无相关性, 相关系数r=0.039, P=0.563>0.05。VAS得分与耳鸣响度无相关性, 相关系数r=0.136, P=0.110>0.05。结论 ①突发性聋伴耳鸣患者中高频耳鸣最常见;②耳鸣频率与听力损失类型显著相关, 低中频下降型以低频耳鸣多见, 而中高频下降型及全聋型以高频耳鸣多见;③听力损失最大的频率与耳鸣的频率有较高的一致性;④突发性聋伴耳鸣患者急性期的耳鸣致残程度以3～4级为多;⑤THI及VAS得分与听力损失的程度、耳鸣的频率、耳鸣的响度均无相关性。     

Acoustic neuromas presenting as sudden deafness

Caution is required in diagnosing even apparently "obvious" causes of hearing loss. A patient is described who came to medical attention through referral from a lawyer for sudden deafness allegedly caused by exposure to noise in an industrial setting. Thorough evaluation revealed an acoustic neuroma. Review of the literature suggests that presentation of acoustic neuromas as sudden deafness is more common than generally recognized. Clinicians must be alert to this possibility and diligent in their evaluation of any unilateral ear symptoms.