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.     

Acoustic neuroma presenting as sudden hearing loss

Sudden hearing loss occurs as the initial symptom in 1 to 9 per cent of all acoustic neuromas. A case of an acoustic neuroma presenting with sudden hearing loss is described. In this case, the hearing thresholds returned to normal. To the authors' knowledge, this is the first case in which an acoustic neuroma presenting with sudden hearing loss exhibited a return to normal thresholds. This suggests that such tumors may have an initial reversible effect on hearing thresholds.     

Differenzialdiagnosen einer beidseitigen akuten Hörminderung bei einem Patienten mit metastasierendem Bronchialkarzinom

The first symptom of an acoustic neuroma in about 50% of the patients is hearing loss, which occurs suddenly in about 5-10% of cases. Acute progressive hearing loss is associated with a broad spectrum of differential diagnoses. Cerebellar and hepatic metastases from a bronchial carcinoma were previously diagnosed in the case presented here, and the most probable causes of the progressive hearing loss, e.g. idiopathic sudden deafness, infection and tumor-associated factors, were considered and diagnostically analyzed. The discussion ultimately focused on the clinical and radiological signs of bilateral acoustic neuroma. The patient's history and clinical findings yielded no indication of neurofibromatosis (type 1/2). Nevertheless, the constellation of findings suggests that the bilateral hearing loss was caused by a bilateral acoustic neuroma.     

Sudden hearing loss induced by acoustic neuroma: Significance of small tumors

In our series of 111 patients operated on for acoustic neuroma from 1972 to 1990, 21 (18.9%) had sudden hearing loss. The 21 tumors involved were comprised of 9 small, 5 medium, and 7 large tumors. Emphasis is placed on the fact that even a small tumor has the potential to produce sudden hearing loss (SHL) and that the possibility of seeing patients with SHL is increasing thanks to advances in imaging diagnosis. Recognition of SHL as an initial symptom of acoustic tumor is considered essential to detect small acoustic neuroma.     

Present state of early neurotological diagnosis of acoustic neuroma

The incidence of cases of acoustic neuroma that are manifested as sudden deafness or progressive sensorineural hearing loss was investigated. The findings of the stapedius reflex test, the auditory brain stem response test and the caloric test were analyzed, and the procedure for the diagnosis of acoustic neuroma was discussed.     

Otosclerosis masking coexistent acoustic neuroma

The coexistence of an acoustic neuroma with otosclerosis can prove to be a great diagnostic dilemma. In such cases, the diagnosis of acoustic neuroma is usually delayed while more common causes of sensorineural hearing loss associated with otosclerosis or its surgery are considered. Any asymmetric progression of a sensorineural loss or shift in discrimination after stapes surgery, whether sudden or gradual, should provoke suspicion of a second pathologic process. These include perilymph fistula, labyrinthine otosclerosis or ischemia, and acoustic neuroma. The use of auditory brainstem response and acoustic reflex testing and various imaging techniques is essential for accurate diagnosis. This paper includes two cases that demonstrate this dual pathology, bringing the total to 15 such cases reported in the literature. The purposes of this paper are to alert the clinician to the diagnostic problem of acoustic neuroma coexisting with otosclerosis and to propose a means of evaluating these patients.     

以突发性听力减退为首发症状的听神经瘤临床特点分析

目的 分析突发性听力减退为首发症状的听神经瘤的临床特点,以避免对该病的误诊、误治。方法 对1983－1997年收治听神经瘤92例中20例（21耳）首发症状为突发性听力减退的病例占21.7%)进行回顾性分析。结果 听力学检测：纯音听阈（500Hz、1000Hz、2000Hz听力平均听阈）＞71dBHL者12耳,占57.1%;听性脑干反应（auditory brainstem r esponse,ABR)检测均有异常;声反射检测9例（10耳）均消失。影像学检查CT阳性率为88.89%,阴行者行CT气脑造影或磁共振成像（magnetic resonance imaging,MRI)检查均能确诊。结论 对突发性听力减退可疑听神经瘤的患者应常规检查ABR,出现异常者应进行颞骨CT,必要时行MRI检查。     

以突发性听力减退为首发症状的听神经瘤临床特点分析

目的分析突发性听力减退为首发症状的听神经瘤的临床特点,以避免对该病的误诊、误治。方法对1983～1997年收治听神经瘤92例中20例（21耳）首发症状为突发性听力减退的病例（占21.7%）进行回顾性分析。结果听力学检测纯音听阈（500Hz、1000Hz、2000Hz听力平均听阈）＞71dBHL者12耳,占57.1%;听性脑干反应（auditorybrainstemresponse,ABR）检测均有异常;声反射检测9例（10耳）均消失。影像学检查CT阳性率为88.89%,阴性者行CT气脑造影或磁共振成像（magneticresonanceimaging,MRI）检查均能确诊。结论对突发性听力减退可疑听神经瘤的患者应常规检查ABR,出现异常者应进行颞骨CT,必要时行MRI检查。     

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.     

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。     

听神经瘤引起突发性听力减退

为了避免听神经瘤的误诊误治，对１９８６￣１９９５年收治听神经瘤１０４例中２３例（２４耳）首发症状表现为突发性听力减退者（占２３％）的病例进行分析。听力学检测：纯音听阈〉７１ｄＢ ＨＬ者１３耳，占５４．２％，听性脑干反应（ＡＢＲ）检测均有异常；耳蜗电图－ＳＰ／ＡＰ检测９耳中７耳〉０．４，占７７．８％，声反射检测１１耳均消失。眼震电图检测１８例，１７例异常（占９４．４％），影像学检查ＣＴ阳性率８８．８     

听神经瘤引起突发性听力减退

为了避免听神经瘤的误诊误治，对１９８６～１９９５年收治听神经瘤１０４例中２３例（２４耳）首发症状表现为突发性听力减退者（占２３％）的病例进行分析。听力学检测：纯音听阈＞７１ｄＢＨＬ者１３耳，占５４．２％；听性脑干反应（ＡＢＲ）检测均有异常；耳蜗电图－ＳＰ／ＡＰ检测９耳中７耳＞０．４，占７７．８％；声反射检测１１耳均消失。眼震电图检测１８例，１７例异常（占９４．４％）。影像学检查ＣＴ阳性率８８．８％，阴性者行ＣＴ气脑造影或磁共振（ＭＲＩ）检查均能确诊。提示对突发性听力减退患者应常规检查ＡＢＲ，若异常应行颞骨ＣＴ，必要时ＭＲＩ影像学检查。     

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.     

Sudden hearing loss as a presenting symptom of acoustic neuroma.

PURPOSE: Retrocochlear pathological findings may be suggested by findings on the brainstem response and acoustic sensorineural reflex (AR) tests. We describe the incidence of acoustic neuroma presenting as sudden hearing loss (SHL) and the effectiveness of the discrimination (DISC) test, the brainstem-evoked response, and AR test in predicting acoustic neuroma in patients with SHL. METHODS: We retrospectively reviewed the charts of all adult patients who presented to our center with sensorineural SHL between 1989 and 1995. Two groups were defined: those with cerebellopontine angle (CPA) tumor and those with negative imaging findings. Results of the brainstem-evoked response, AR, and DISC tests were compared. RESULTS: Forty patients were admitted with sensorineural SHL, of whom 19 (47.5%) had a CPA tumor. The latter group showed a significantly lower mean age and better results for the low frequencies on pure tone audiometry, as well as better brainstem-evoked response test results than the patients with negative imaging findings. There was also a significant difference between the groups for both the affected and unaffected ears on the discrimination test. CONCLUSION: Acoustic tumors may be a more common cause of sudden sensorineural hearing loss than previously suspected. The DISC test is a useful screening tool for acoustic tumor, whereas the brainstem-evoked response test shows poorer results in affected patients with sensorineural hearing loss than in other subgroups with different signs of acoustic neuroma. We recommend that young patients presenting with mild SHL who have normal results on the AR and brainstem-evoked response tests undergo magnetic resonance imaging to rule out CPA tumor.     

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.     

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。     

Audiometric findings in patients with acoustic neuroma

OBJECTIVE: Hearing loss remains the most common symptom associated with acoustic neuroma. This study documents the audiometric findings from 721 acoustic neuroma procedures. STUDY DESIGN: This was a retrospective study. The preoperative audiometric data were compiled and were analyzed by patient age, gender, tumor size, time of surgery, and neurofibromatosis Type 2 (NF 2). Postoperative audiometric data were arranged and compiled in the same way. The hearing classification proposed by the AAO-HNS was applied to all preoperative and postoperative cases. SETTING: Tertiary referral center. PATIENTS: Surgically confirmed acoustic neuroma patients who had not previously received surgical or radiosurgical therapy. Patients underwent surgery by the retrosigmoid approach. INTERVENTION: Surgical removal of an acoustic neuroma. MAIN OUTCOME RESULT: Provision of pure tone and speech data from a group of acoustic neuroma patients, including application of the recently introduced and accepted AAO-HNS hearing classification system. RESULTS: Preoperative audiometric data were obtained from 694 of 721 patients (96%), of whom 619 had measurable hearing. Postoperative audiometry was performed on 606 patients; 152 had usable data. The combined preoperative audiometric data revealed a high frequency sensorineural hearing loss. Word recognition was servicable. The postoperative pure tones and word recognition scores were worse than preoperative scores. Age, gender, tumor size, and time of surgery had some impact on the preoperative hearing and the postoperative result; NF 2 did not. CONCLUSIONS: The study confirms that hearing alteration is almost universal in acoustic neuroma patients. Hearing preservation is possible in a significant number of cases; however, the postoperative auditory function tends to be worse.     

Neurofibromatosis 2: two case reports

Our study presents two cases of neurofibromatosis 2 (NF2) that have been diagnosed at the Ear, Nose, and Throat Department of Hradec Králové (Czech Republic). The first case involved a young man with a history of sudden hearing loss accompanied by tinnitus on the left side. The diagnosis of NF2 was made, and an operation for left acoustic neuroma was performed. Looking toward the future, the acoustic neuroma on the right side should be resolved as well. The second case concerned a woman (the mother of our patient 1) examined at the same Ear, Nose, and Throat Department in 1980, after 4 years of gait instability and progressive loss of hearing and tinnitus on the right side. Computed tomography scan detected a bilateral expansion in the pontocerebellar angles, and a large tumor on the right side was removed. The patient is deaf and has facial palsy without progression of symptomatology during long-term follow-up. These two cases document the rare but serious hereditary disease of NF2. Its most frequent first presentation is acoustic neuroma; further, benign tumors of the nervous system and juvenile cortical cataract also are often detected. The variability of number, location, and biological behavior of tumors associated with NF2 require an individual patient treatment approach, long-term follow-up, and insertion of appropriate hearing aids. Important also is a genetic examination to exclude pathological NF2 genes in the first-degree relatives of the affected individuals.     

Hearing loss and growth rate of acoustic neuromas in follow-up observation policy

Objective: It is important to know the growth rate of acoustic neuroma in setting up its observation policy. One aimed to evaluate the significance of growth rate and hearing loss speed from a retrospective study of 31 patients. Methods: Thirty-one patients with acoustic neuroma (mean age, 57.1 years; range, 9–81 years) who had undergone MRI and audiometry were retrospectively examined. Changes in tumor size and hearing level during follow-up periods were analyzed. Results: The mean annual growth rate was 2.4 mm/year (maximum, 17 mm/year), and the average annual hearing loss speed was 2.3 dB/year (−15.0–19.2 dB/year) in the follow-up, and a correlation was recognized between them. Whereas significant difference was noted in annual growth rate and hearing loss speed between the sexes, no correlation was recognized between the annual growth rate or hearing loss speed and the age, tumor size and pure tone average (PTA) at the initial diagnosis. Conclusion: Attention must be paid to the acoustic neuroma cases where tumor growth rate is markedly high and those where hearing level rapidly declines during follow-ups. It is difficult, however, to estimate tumor diameter in relation with the changes in hearing level because the natural course of an acoustic neuroma varies in each individual.     

Conductive hearing loss after removal of acoustic neuroma.

OBJECTIVE: To report 4 patients who have developed a conductive and/or mixed hearing loss due to dehiscence of the inner ear after retrosigmoid approach for removal of acoustic neuroma. PATIENTS: Four patients who presented with conductive and/or mixed hearing loss after retrosigmoid approach for removal of acoustic neuroma. MAIN OUTCOME MEASURE: Evidence of inner ear dehiscence on postoperative computed tomographic scan of the temporal bones. CONCLUSION: The occurrence of conductive hearing loss after the surgical removal of an acoustic neuroma has not previously been documented. Computed tomographic scan of the temporal bones showing inner ear dehiscence may explain this finding. Formal documentation of such cases may allow techniques to be developed to reduce its occurrence or reconstruction of the defect at the time of primary surgery.