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.     

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.     

以突发性聋为首发表现的斜坡脊索瘤1例

1病例报告患者,女,55岁,右耳突发听力下降伴耳鸣1d于2010年2月23日入院。患者无眩晕、耳痛及耳溢液。既往有糖尿病史20余年,高血压病史10余年。体检:BP 150/90 mm Hg(1 mm Hg=0.133kPa),双耳廓无畸形,外耳道通畅,鼓膜完整,内陷。纯音测听示:右耳全聋,左耳语言频率轻     

Sudden deafness: long-term follow-up and recurrence

We examined the long-term outcome for patients with idiopathic sudden sensorineural hearing loss (sudden deafness), including the incidence of recurrence of sudden deafness. The subjects were 1,798 individuals who came to Nagoya University hospital within 2 weeks of the onset of unilateral sudden deafness. Only 14 of the patients had a history of being diagnosed with sudden deafness. After their visit to our hospital, one patient had a recurrence in the ipsilateral ear and four patients experienced sudden deafness in the contralateral ear. We performed hearing examinations on 88 patients who revisited our hospital more than 10 years after unilateral sudden deafness. Of these patients, there was one with a recurrence in the ipsilateral ear and one with sudden deafness in the contralateral ear. Thus, in this series the recurrence of sudden deafness was rare.     

Sudden deafness: long‐term follow‐up and recurrence

We examined the long‐term outcome for patients with idiopathic sudden sensorineural hearing loss (sudden deafness), including the incidence of recurrence of sudden deafness. The subjects were 1798 individuals who came to Nagoya University hospital within 2 weeks of the onset of unilateral sudden deafness. Only 14 of the patients had a history of being diagnosed with sudden deafness. After their visit to our hospital, one patient had a recurrence in the ipsilateral ear and four patients experienced sudden deafness in the contralateral ear. We performed hearing examinations on 88 patients who revisited our hospital more than 10 years after unilateral sudden deafness. Of these patients, there was one with a recurrence in the ipsilateral ear and one with sudden deafness in the contralateral ear. Thus, in this series the recurrence of sudden deafness was rare.     

Atresia and sudden sensorineural hearing loss

This case study summarizes findings in an adult male, aged 57, who presented to the Adult Audiology Clinic with aural atresia in the right ear resulting in a conductive hearing loss and a sudden sensorineural hearing loss in the left ear. Treatment options included reconstruction surgery in the right ear, bone anchored hearing aid in the right ear to overcome the conductive hearing loss, bone anchored hearing aid in the left ear for single sided deafness, and intratympanic steroid injections in the left ear to salvage hearing. This case study highlights that when a patient is educated on all available options the patient is then able to make a decision comfortable to him and to help improve his hearing.     

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.     

AICA syndrome with facial palsy following vertigo and acute sensorineural hearing loss

We report a case of infarction of the anterior inferior cerebellar artery (AICA) with peripheral facial palsy following vertigo and acute sensorineural hearing loss. A 39-year-old female presented with vertigo and sudden hearing loss, tinnitus, and aural fullness of the right ear. An audiogram revealed a severe hearing loss at all tested frequencies in the right ear. Spontaneous nystagmus toward the left side was also observed. Otoneurological examinations showed sensorineural hearing loss of the right ear and horizontal and rotatory gaze nystagmus toward the left side, and a caloric reflex test demonstrated canal paresis. Initially, we diagnosed the patient for sudden deafness with vertigo. However, right peripheral facial palsy appeared 2 days later. An eye tracking test (ETT) and optokinetic pattern test (OKP) showed centralis abnormality. The patient's brain was examined by magnetic resonance imaging (MRI) and magnetic resonance angioglaphy (MRA) and showed an infarction localized in the pons and cerebellum. MRI and MRA revealed infarction of the right cerebellar hemisphere indicating occlusion of the AICA. Consequently, the patient was diagnosed with AICA syndrome but demonstrated regression following steroid and edaravone treatment. We suggest that performing MRI and MRA in the early stage of AICA syndrome is important for distinguishing cerebellar infarction resulting from vestibular disease.     

Asymmetry of temporal processing in listeners with normal hearing and unilaterally deaf subjects

OBJECTIVES: In general, auditory cortex on the left side of the brain is specialized for processing of acoustic stimuli with complex temporal structure including speech, and the right hemisphere is primary for spectral processing and favors tonal stimuli and music. This asymmetry in processing is further emphasized when hemisphere-favored stimuli are presented to the contralateral ear. The purpose of the first experiment is to further investigate the properties that dictate lateralized processing of auditory stimuli by ear and the relationship between auditory task and stimulus type. Next, it is not clear what compensation may exist for the loss of function of one ear and consequently, reduced access to functions primary performed in the opposite hemisphere, in the case of early unilateral profound hearing loss. The purpose of experiment 2 is to determine if any compensation for loss of function is seen in persons with early unilateral deafness. DESIGN: Experiment 1: Gap detection thresholds were determined in 30 right-handed listeners with normal hearing using wide-band noise markers (temporally complex), 400 and 4000 Hz pure tones presented individually to the left and right ears. Experiment 2: The same procedure was administered to listeners with early-onset, severe-to-profound unilateral deafness (seven left ear deaf and five right ear deaf) in the hearing ear alone. RESULTS: A significant right ear advantage was found for gap detection threshold using noise markers and a smaller left ear advantage was found for tonal stimuli. Listeners with unilateral deafness demonstrated that the hearing ear, left or right, performed in a manner similar to listeners with normal hearing. CONCLUSIONS: Results indicate that (1) gap marker, more than task, was the salient feature in determining laterality of processing in this experiment, (2) the two ears have distinct processing capacity based on stimulus type, and (3) compensation for loss is not apparent in persons with congenital unilateral deafness.     

A sudden bilateral hearing loss caused by inner ear hemorrhage

IntroductionLabyrinthine hemorrhage is a rare cause of sudden deafness and generally concerns only on one side.Case summaryAn 84-year-old man with a past medical history of myelomonocytic chronic leukemia (CMML) suffered from sudden bilateral hearing loss associated with vertigo. The audiogram revealed a left cophosis and a right profound deafness. Videonystagmography showed a left vestibular deficit. The MRI showed a spontaneous strong T1 weighted signal in the left and right labyrinths, corresponding to a bilateral inner ear hemorrhage (IEH). Dizziness resolved rapidly following vestibular physiotherapy, in contrast to hearing which did not improve at all and let the patient isolated in his environment. The patient successfully underwent cochlear implantation so that he could communicate.DiscussionMost IEHs are unilateral and due to anticoagulants treatments and hematological diseases. Only rare cases have described bilateral labyrinth hemorrhage. This is the first case reported of bilateral labyrinth hemorrhage due to CMML.     

Cortical auditory evoked responses from an implanted ear after 50 years of profound unilateral deafness

A male with unilateral deafness in the right ear since 8 years of age developed a sudden hearing loss in the left ear at age 63. A hearing aid was fitted in the left ear with limited benefit. The right ear received a cochlear implant (CI) 20 months later. Cortical auditory evoked potentials (CAEPs) and speech recognition scores (SRS) were measured in free-field three, six and nine months after implantation with the hearing aid alone, CI alone and bimodal condition (hearing aid and CI together). Three months after implantation the cortical responses for the two ears were similar, despite more than 50 years of unilateral auditory deprivation. CAEPs measured over time show evidence of binaural interaction and improvements in SRS.     

The progression of hearing loss in the early stages of sudden deafness

Summary The pathogenesis of sudden deafness is still not known. Therefore, to clarify its pathophysiology, it is important to know whether the progression of hearing loss occurs in the early stage of sudden deafness. The subjects were 11 patients with sudden deafness showing the progression of hearing loss by pure tone audiometry after the onset of the initial attack of hearing loss. The progression of hearing loss was mostly observed within 4–7 days after the onset of the initial attack. Average hearing loss in initial audiometry was severe and the recovery was poor in the majority of cases. An exploratory tympanotomy was performed in five cases and revealed one case of perilymphatic leak from the round window and another case of suspected round-window membrane rupture. As far as the causes of the progression of hearing loss in the early stage of sudden deafness are concerned, the following could be considered (1) aggravation of viral labyrinthitis, (2) aggravation of the vascular lesion of the inner ear, and (3) rupture of the membranous labyrinth of window(s).This paper was presented at the XVIth International Congress of Audiology, May 23–27, 1982, held in Helsinki, Finland     

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.     

伴有大前庭水管的突发性聋(附病例报告及文献复习)

目的:探讨不伴耳蜗畸形的大前庭水管综合征的临床表现和高分辨率CT对大前庭管的诊断。方法:报告1例18岁男性患者从2岁时开始患双耳感音神经性听力损失,2001年1月和11月两耳先后出现突发性聋;颞骨轴位高分辨率CT扫描示双侧前庭水管扩大,不伴耳蜗畸形。此外并进行了文献复习。结果:不伴耳蜗畸形的大前庭水管综合征可出现后天性感音神经性听力损失,并有突发性聋的危险。结论:对儿童时期发生的不明原因的感音神经性听力损失或(和)突发性聋,应行高分辨率CT扫描,以了解是否存在不伴耳蜗畸形的大前庭水管综合征。     

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.     

Bilateral cochlear implants in long-term and short-term deafness

This case is of a 70-year-old man with single-sided deafness (SSD) in the right ear since childhood, who developed a sudden severe hearing loss in the left ear at the age of 63. Eventually, after he received cochlear implants in both ears, he started to present behavioural auditory processing skills associated with binaural hearing, such as improved ability to understand speech in the presence of background noise, and sound localization. Outcomes were measured using cortical auditory evoked potentials, speech perception in noise, sound localization tests, and a self-rating questionnaire. The results suggest that even after more than 50 years of unilateral deafness it was possible to develop binaural interaction and sound localization as a result of electric auditory stimulation.     

Cortical auditory evoked responses from an implanted ear after 50 years of profound unilateral deafness

Abstract

A male with unilateral deafness in the right ear since 8 years of age developed a sudden hearing loss in the left ear at age 63. A hearing aid was fitted in the left ear with limited benefit. The right ear received a cochlear implant (CI) 20 months later. Cortical auditory evoked potentials (CAEPs) and speech recognition scores (SRS) were measured in free-field three, six and nine months after implantation with the hearing aid alone, CI alone and bimodal condition (hearing aid and CI together). Three months after implantation the cortical responses for the two ears were similar, despite more than 50 years of unilateral auditory deprivation. CAEPs measured over time show evidence of binaural interaction and improvements in SRS. Copyright © 2007 John Wiley & Sons, Ltd.     

Simultaneous bilateral sudden deafness. Report of a clinical case

Bilateral sudden deafness is uncommon and characterized by an acute sensorineural hearing loss in both ears of 30 dB or more in 3 consecutive frequencies. We report the case of a 57 yeras old female with this diagnosis who presented a right anacusia and a severe audiometric loss on left ear without vestibular pathology associated. She was admitted and treated by the protocol of medical therapy that we have performed in our E.N.T. Department (4 days EV and then 10 ones oral medication) with improvement in her audition 25-30 dB average. If sudden bilateral sensorineural deafnes is founded, we must consider an immuno-mediated inner ear disease (IMIED).     

Progressive cochleo-vestibular labyrinthitis

OBJECTIVE: We report a rare case of sudden deafness and benign paroxysmal positional vertigo (BPPV) corresponding to a canalithiasis of the ipsilateral left posterior semicircular canal (PSC). CASE REPORT: The initial examination showed intact lateral semicircular canal (LSC) function. Cerebral MRI results were normal. However evoked otoacoustics emissions were absent in the left ear. The patient developed, in the course of one week, symptoms and signs of an acute unilateral vestibulopathy in the same ear. However there were no change in hearing loss after it. CONCLUSION: The association of BPPV acute vestibulopathy and sudden deafness is uncommon but usually the former develops well after the second and third. This case is best explained by a vestibulocochlear labyrinthitis.     

Cochlear implantation in a patient with eosinophilic otitis media

Eosinophilic otitis media is an intractable middle ear disease with gelatinous mucoid fluid containing eosinophils associated with bronchial asthma and nasal allergy that sometimes induces deterioration of sensorineural hearing loss. Here, we report a case of eosinophilic otitis media in a 50-year-old woman who received a Nucleus 22 multi-channel cochlear implant in the right ear at the age of 42 years. She had received treatment for bronchial asthma, chronic sinusitis with nasal allergy and otitis media with effusion since the age of 30 years and had noted bilateral sudden deafness and vertigo at the age of 35 years. Preoperative CT and MRI showed cochlear ossification in the left ear, in which mastoidectomy was performed as treatment of eosinophilic otitis media. Long-term follow-up revealed that cochlear implant is indicated for deafness induced by eosinophilic otitis media, and an early decision for cochlear implant surgery is necessary. Steroid administration was remarkably useful in controlling eosinophilic otitis media in patients with bronchial asthma and chronic sinusitis with nasal allergy.