Acute profound sensorineural hearing loss as the initial manifestation of acute leukemia--report of a case

This paper reports an unusual case in which acute lymphocytic leukemia presented acute profound sensorineural hearing loss as the initial manifestation of the disease. The patient is a 55-year-old woman who complained of left hearing loss of sudden onset. Pure tone audiometry revealed profound sensorineural hearing loss of the left ear at mid and low frequencies. The patient was tentatively diagnosed as idiopathic sudden deafness and admitted for the treatment, but her laboratory data indicated that she was at an advanced stage of leukemia. The patient’s hearing loss did not improve subjectively until she deceased 1 year after the admission. The mechanism producing acute hearing loss in leukemic patients is reviewed and discussed, and the importance of differentiating possible underlying diseases before we diagnose idiopathic sudden deafness is stressed.     

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.     

Preclinical detection of initial vestibulocochlear abnormalities in a patient with multiple sclerosis

Summary A 49-year-old woman presented with a third-degree continuous spontaneous nystagmus to the left which was followed by a sudden, almost complete deafness of her left ear. These symptoms were established as the sole initial presenting manifestations of multiple sclerosis (MS). Magnetic resonance imaging demonstrated a lesion within the left eight nerve root-entry zone and multiple small central lesions beneath the lateral ventricles. Pure-tone audiometry returned to normalcy after 2 weeks and speech discrimination was 100% after 10 weeks. Auditory brainstem responses (ABRs) showed abnormal parameters during a 10-month follow-up period after the acute hearing loss. Furthermore, desynchronization in ABR testing could be demonstrated 2 days before onset of hearing loss, which was interpreted as a prodromal ABR sign of the incipient MS attack.     

Audiological evaluation of twenty patients receiving pentoxifylline and prednisone after sudden deafness: prospective study

Idiopathic sudden deafness is defined as sudden sensorineural hearing loss of undetermined etiology. As a consequence, various treatments have been developed for this disorder. Our study evaluated the effectiveness of pentoxifylline and prednisone in such treatment. We analyzed this treatment's results in our patients through conventional audiograms, and speech audiometry was performed in the acute stage and during the treatment. We diagnosed idiopathic sudden hearing loss in 20 patients (8 female and 12 male). The left ear was involved in 9 patients and the right in 11. All patients had been examined by us within 15 days from the onset of hearing loss. We compared the hearing threshold results in the different periods in this prospective study.     

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.     

Die Ruptur der runden Fenstermembran — eine Ursache des akuten Hörsturzes

Summary The rupture of the round window has been well described as an occasional lesion with sudden deafness and vertigo after diving, flying and physical stress mostly according to patients history. Apparently it also seems being the cause for sudden progressive hearing loss. Therefore in our clinic the transtympanic endoscopic evaluation of the round window is routinely carried out in patients with acute complete sudden deafness or severely progressive hearing loss even without any outpointing history of a possible trauma.The methods and the results are described.     

Sudden hearing loss in chronic myelogenous leukaemia implicating the hyperviscosity syndrome

Sudden sensorineural hearing loss that presents as the initial sign of haematological disease is very rare. Chronic myelogenous leukaemia has been implicated as a causative factor of sudden sensorineural hearing loss. A 49-year-old male presented with unilateral sudden sensorineural hearing loss. The patient was found to have chronic myelogenous leukaemia during a work-up for his hearing loss. We present a case of a chronic myelogenous leukaemia patient whose first manifestation was sudden sensorineural hearing loss. We presume that cochlear vessel occlusion as a result of elevated blood viscosity was responsible for this patient's hearing loss. Early onset of sudden deafness in a chronic myelogenous leukaemia patient may be due to the hyperviscosity syndrome and it may be possible to reverse hearing loss through early leukapheresis.     

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。     

突发性聋临床疗效与相关因素探讨

目的探讨影响突发性聋疗效的相关因素,为突发性聋治疗提供经验。方法回顾性分析137例突发性聋的临床资料。结果突发性聋的预后与性别、是否伴眩晕、是否伴耳鸣、是否伴心脑血管疾病无关,与发病年龄、初诊时间、听力曲线类型、是否伴有精神心理因素有显著相关性。结论年轻患者,初诊时间越早,听力曲线为上升型,无精神心理因素者预后好,反之较差。     

Labyrinthine window rupture as a cause of acute sensorineural hearing loss

Labyrinthine window rupture (LWR) is one cause of acute sensorineural hearing loss and need for early exploration is clear for good improved hearing. Acute sensorineural hearing loss of 60 dB or more treated from May 2006 to May 2010 were retrospectively analyzed. There were 21 ears of severe deafness, 18 ears of profound deafness, and 10 ears of total deafness. All patients were examined with temporal bone CT. Space-occupying lesions around the labyrinthine windows were suggestive images of LWR. Thirty-five ears were operated for LWR while 14 ears of SHL received conservative treatments. Fifty-seven percent of LWR improved 30 dB or more after sealing of both labyrinthine windows. Of the 15 markedly recovered ears, 14 ears were operated within 2 weeks from the onset. Of the five cured ears, four ears were operated within a week from the onset. As for the hearing prognosis of SHL, 88% of severe and profound deafness improved 30 dB or more but total deafness did not improve more than 30 dB. Exclusion of LWR from SHL and early surgical intervention in LWR will bring about good hearing prognosis to both LWR and SHL.     

Sensorineural hearing loss as the initial manifestation of polyarteritis nodosa

A 74-year-old male was referred for the sudden onset of bilateral sudden deafness. The patient had no history of any disease or trauma to the head. Pure tone audiometry revealed bilateral moderate, to severe, sensorineural hearing loss. Auditory brain stem responses (ABRs) showed normal peak and interpeak latencies. These audiological findings suggested that his hearing loss could be attributed to inner ear lesions. However, we felt an alternative explanation for this sudden deafness was likely to exist because the patient also had a month-long fever of unknown origin (FUO) and weight loss of 5 kg/month. Using the criteria of The American College of Rheumatology, we made the diagnosis of polyarteritis nodosa (PAN). Serum MPO-ANCA was positive (x 661). For treatment, the patient was begun on prednisolone and cyclophosphamide. Nine months later, fever, hypertension, nephritis, pneumonitis, and arthritis had completely resolved, the MPO-ANCA became negative (MPO-ANCA < x 10). Furthermore, his hearing improved.     

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.     

Psychogenic hearing loss with panic anxiety attack after the onset of acute inner ear disorder

A very rare case of a 50-year-old female showing psychogenic hearing loss with a panic anxiety attack that complicated an acute organic sensorineural hearing loss is reported. At the first visit to our clinic, the patient showed left sensorineural hearing loss with an inner ear disorder pattern. Five days after the onset, her left hearing threshold markedly increased without any subjective signs. On the next day, she suddenly experienced a severe panic attack with anxiety. After the attack, she felt mildly anxious and depressed. A combined therapy using primary corticosteroid therapy for the acute inner ear disorder, psychiatric counseling based on cognitive therapy and the administration of a minor tranquilizer was performed. Her left hearing threshold recovered to within normal ranges except in the high-frequency ranges immediately after the treatment. This case was considered very rare because: (1) the panic anxiety attack occurred in the conversion disorder as psychogenic hearing loss and (2) the psychogenic hearing loss complicated the primary sudden deafness. We suggest that otorhinolaryngologists should have psychiatric knowledge and be able to treat psychogenic hearing loss as a primary care.     

Sudden sensorineural hearing loss as prodromal symptom of anterior inferior cerebellar artery infarction

Sudden sensorineural hearing loss is a clinical condition characterized by a sudden onset of unilateral or bilateral hearing loss. In recent years sudden deafness has been frequently described in association with anterior inferior cerebellar artery (AICA) infarction generally presenting along with other brainstem and cerebellar signs such as ataxia, dysmetria and peripheral facial palsy. The authors report a rare clinical case of a 53-year-old man who suddenly developed hearing loss and tinnitus without any brainstem or cerebellar signs. Computed tomography of his brain was normal, and the audiological results localized the lesion causing deafness to the inner ear. Surprisingly, magnetic resonance imaging showed an ischemic infarct in the right AICA territory. This case represents the fifth in the literature to date but it confirms that AICA occlusion can cause sudden deafness even without brainstem or cerebellar signs. Therefore, we recommend submitting the patient for neuroimaging, as an emergency, in order to exclude infarction of the AICA territory. By doing this, it may be possible to limit the extent of the lesion by commencing early therapy.     

头颅磁共振成像检查在以突发性聋为首发症状患者病因诊断价值中的研究

突发性聋是突然发生的听力下降,有急性发作(多在72 h内)、病因复杂的特点,可由一种或多种病因同时引起,也可作为多种疾病的首发症状出现,如急性脑血管事件,有研究表明突发性聋治疗有效时间窗是2～4周,因此需要对其进行早期诊断、治疗.针对突发性聋患者的听力学检查主要是纯音测听、听性脑干反应试验、耳声发射、40 Hz听觉相关电位等,影像学检查主要是磁共振成像检查.本文主要以突发性聋为首发症状患者病因及头颅MRI在其中的应用价值进行综述.     

儿童突发性聋的临床特征及预后相关因素分析

目的 分析儿童突发性聋的临床特征、疗效及影响预后的相关因素,为临床治疗及预后评估提供依据。 方法 收集2010年1月至2017年10月就诊的67例突发性聋患儿临床资料,对其临床特征及治疗效果进行回顾性分析,同时根据疗效将患者分为总体有效组(36例)及无效组(31例),采用单因素及多因素分析的方法分析患者的性别、年龄、病程、初诊听阈、是否伴发耳鸣、眩晕、病毒感染史、发病季节和听力曲线类型对预后的影响。 结果 儿童突聋患者中64.18%在春冬季发病,其就诊时听阈为(76.62±25.97)dB HL,耳鸣及眩晕伴发率分别为70.15%和61.19%,病毒感染率为19.40%,听力曲线中10.44%为低频下降型、2.99%为高频下降型、34.33%为平坦型及52.24%为全聋型。经治疗后,患者听阈为(60.41±31.52)dB HL,总体有效率为53.73%,其中痊愈率、显效率及有效率分别为20.90%、16.42%和16.42%。多因素分析结果显示,初诊听阈越高及听力曲线为全聋型,预后越差(P均<0.05);伴有病毒感染的非全聋型患者预后较好(P<0.05)。 结论 儿童突发性聋患者病毒感染率较高且大部分在春冬季发病,就诊时听力损失较重并常伴有耳鸣及眩晕,其听力曲线以平坦型及全聋型为主。就诊时听力损伤程度轻、伴有病毒感染的非全聋型患者预后较好。     

影响突发性聋疗效的相关因素分析

目的探讨影响突发性聋治疗效果的相关性因素。方法对90例突发性聋患者的临床资料应用Logistic多因素逐步回归分析方法作回顾性分析。结果突聋的疗效与年龄、性别、单、双耳发病、是否伴有眩晕和耳鸣无关,而与听力损失的程度、听力曲线的类型、发病到初治的时间有显著的相关性。但听力损失的程度在单因素分析时,对突聋疗效的影响无统计学意义。结论突聋患者听力损失越轻,且听力曲线为上升型,发病后治疗越早者,其疗效越好;反之,疗效越差。     

突发性聋的听力损失和前庭损害对其预后的影响

本文通过对３０例突发性聋患者行纯音听力检查及前庭功能系列检查，比较突发性耳聋听力下降程度和类型与眩晕和前庭功能检查结果的关系、前庭损害类型和程度、以及听力预后与眩晕和前庭损害的关系。得出（１）具有前庭损害的患者９１％为中、重度聋，８２％为平坦型和斜坡型听力损害。半规管麻痹者听力损害全部为中、重度聋，图型为平坦或斜坡型。（２）冷热试验（ＣＰ）值６３％、低频谐波加速试验（ＳＨＡＴ）６６％、重心平衡仪试验（ＶＳＲＺ）３６％呈现异常，以ＳＨＡＴ较为敏感；（３）突聋听力预后与是否伴有眩晕、前庭损害以及损害程度有密切关系。     

Tympanoskopie mit Abschottung der Rundfenstermembran beim idiopathischen Hörsturz

Background

The pathogenesis of idiopathic sudden sensorineural hearing loss (ISSHL) still remains unclear. This retrospective study was performed to evaluate the effectiveness of tympanotomy and sealing of the round window membrane after unilateral idiopathic sudden sensorineural hearing loss.

Methods

A total of 74 patients with idiopathic sudden sensorineural hearing loss were treated with antiphlogistic-rheologic infusion therapy according to Stennert (steroids and pentoxyphylline). In addition, all patients underwent exploratory tympanotomy and sealing of the round window membrane. Pure tone audiometry was performed pre- and postinterventionally.

Results

The average hearing loss (four pure tone average) of all patients was 58.9 dB pre-, and 46 dB postinterventionally, which is an average improvement of 12.9 dB. Patients with hearing loss of more than 60% improved significantly compared to patients with hearing loss less than 60% (33.9% vs. +3.3%). Sealing of the round window membrane was found to be more effective when performed within 8 days after ISSHL. A membrane rupture did not lead to better therapy results. No significant correlation was found between therapy outcome and other clinical symptoms.

Conclusion

Sealing of the window membrane shows equal results to conservative methods. If patients with hearing loss of more than 60% have more benefit in tympanotomy with sealing of the window membrane than patients with less hearing loss—as shown as in this study—has to be proved in randomized examinations. Intraoperatively found ruptures of the round window membrane had no significant effect on the therapy outcome.     

A comparative study of mumps deafness and idiopathic profound sudden deafness

Summary We performed a 10-year comparative study on 95 cases (98 ears) of mumps deafness and 97 cases (97 ears) of profound sudden deafness and found the following results. The age of onset of deafness was less than 9 years in the majority of patients with mumps deafness, while it was frequently 30–50 years in those patients with sudden deafness. Among these latter patients, no cases occurred under 9 years of age. The incidence of tinnitus and vestibular symptoms was more frequent in the patients with sudden deafness, but no significant difference was noted in cases over 10 years of age. When determining prognosis, no cases of hearing improvement were encountered in mumps deafness, while about 70% of sudden deafness showed improvement.