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.     

Ipsilateral and contralateral vestibular schwannomas in unilateral long-term deaf ears

In the past decade, we have encountered two patients with unilateral long-term deaf ear having vestibular schwannoma. One was on the deaf ear in a 53 years’ woman and the other was on the contralateral hearing ear in a 62 years’ woman. These two patients had total deafness on the right ear since childhood, associated with absent auditory brainstem response (ABR) and caloric responses, whereas normal ABR and caloric responses were shown on the left ear. Although similar physiological findings were disclosed on the deaf ears, MRI scan demonstrated vestibular schwannoma on the different side, that is, one in deaf ear and the other in hearing ear. For the former, absent ABR and caloric results indicate that the tumor originates from the superior vestibular nerve. In contrast with the latter, normal ABR and caloric responses, but absent vestibular-evoked myogenic potentials on the hearing ear imply that the tumor originates from the inferior vestibular nerve. The ipsilateral tumor on the deaf ear underwent intracranial surgical excision, followed by radiosurgery due to tumor recurrence 10 years later, while the contralateral one on the only hearing ear received radiosurgery only. Both patients were rather well, 3 years after last treatment.     

Delayed endolymphatic hydrops. Special emphasis on nystagmus associated with episodes and contribution of chemical labyrinthectomy

ObjectivesThe main objective was to describe spontaneous nystagmus characteristics during an episode of delayed endolymphatic hydrops (DEH), including an initial vertical upbeating nystagmus in one patient. The secondary objective was to highlight the contribution of chemical labyrinthectomy.MethodsEpisodic vertigo after a prolonged period of time of sensorineural hearing loss (profound or total) in one ear characterized ipsilateral DEH and was associated with the development of hearing loss in the opposite ear in contralateral DEH.ResultsTen patients met the criteria for DEH: 7 ipsilateral and 3 contralateral. Three (all ipsilateral DEH) were examined during a vertigo episode. Two patients had a typical horizontal-torsional nystagmus beating contralaterally to the hearing loss. One patient showed atypic initial vertical upbeating nystagmus with a slight torsional component, which secondarily became horizontal-torsional beating contralaterally to the hearing loss. Four patients had disabling vertigo with unilateral total deafness (ipsilateral DEH), successfully treated by 1-3 transtympanic gentamycin (Gentalline^®) injections.ConclusionNystagmus direction during vertigo episodes varies, and may initially present as vertical upbeating nystagmus, which, to our knowledge, has not been previously reported in DEH or Menière's disease. This nystagmus might reflect an inhibition of the superior semicircular canal (on the hearing-impaired side), suggesting incipient hydrops in this canal. Chemical labyrinthectomy is a simple and effective procedure in unilateral DEH, especially as the patient often suffers from total deafness.     

Delayed endolymphatic hydrops as a clinical entity

Delayed endolymphatic hydrops (DEH) is a clinical entity that can be differentiated from Ménière's disease and is typically observed in patients who have been suffering from longstanding unilateral profound inner-ear hearing loss. DEH probably is caused by delayed atrophy or fibrous obliteration of the endolymphatic resorptive system of the membranous labyrinth. The time that elapses between the occurrence of hearing loss and the onset of DEH can range from 1 to 74 years. The most common cause of hearing loss preceding DEH is juvenile-onset unilateral profound deafness (early childhood unilateral profound sensorineural hearing loss of unknown etiology), followed by labyrinthitis from various causes and physical and acoustic traumas to the inner ear. Two types of DEH exist: the ipsilateral type, in which the ear with profound hearing loss suffers progressive endolymphatic hydrops, and the contralateral type, in which the formation of progressive endolymphatic hydrops takes place in the ear opposite to the previously deafened ear. The incidence of the ipsilateral type is higher than that of the contralateral type, and the contralateral type is more common in older patients. When recurrent episodic vertigo cannot be remedied through conservative treatment, labyrinthectomy and vestibular neurectomy on the deaf ear are curative for ipsilateral DEH. However, no such surgical treatment is available for the contralateral type.     

Bilateral simultaneous sudden deafness

Although sudden deafness has many causes, it is often a sensorineural hearing loss with an abrupt onset and unknown origin. The majority of these latter cases are unilateral in their occurrences, while bilateral simultaneous involvement is rarely seen. During the past 13 years, we have treated 997 patients with sudden deafness. All patients were seen at our university hospital within 2 weeks after the onset of their hearing losses and were observed until the hearing level was fixed. Among them, 10 patients were found to have bilateral simultaneous onsets. The average age and the incidence of vestibular symptoms were similar to those with unilateral deafness. The hearing recovery was much better in the ear with the lesser deafness. No improvement was observed on the side with no sound perception. Those patients in whom common cold and fever seemed to trigger the onset of deafness were also noted to have high viral antibody titers, and were more commonly seen than were those cases with unilateral deafness.     

Bilateral simultaneous sudden deafness

Summary Although sudden deafness has many causes, it is often a sensorineural hearing loss with an abrupt onset and unknown origin. The majority of these latter cases are unilateral in their occurrences, while bilateral simultaneous involvement is rarely seen. During the past 13 years, we have treated 997 patients with sudden deafness. All patients were seen at our university hospital within 2 weeks after the onset of their hearing losses and were observed until the hearing level was fixed. Among them, 10 patients were found to have bilateral simultaneous onsets. The average age and the incidence of vestibular symptoms were similar to those with unilateral deafness. The hearing recovery was much better in the ear with the lesser deafness. No improvement was observed on the side with no sound perception. Those patients in whom common cold and fever seemed to trigger the onset of deafness were also noted to have high viral antibody titers, and were more commonly seen than were those cases with unilateral deafness.     

单侧耳蜗性聋患者对侧耳耳声发射的表现

目的 :探讨致聋因素在致单耳发病时对对侧耳潜在的影响。方法 :利用OtodynamicILO96耳声发射分析仪 ,对 35例单侧耳蜗性聋患者 (分为 5组 :发病 2个月以内的突发性聋 9例为S1组 ,发病 1年以上的突发性聋 6例为S2 组 ,梅尼埃病 13例分为M1、M2 组 ,不明机理的耳蜗性聋 7例为UNK组 )对侧耳瞬态诱发性耳声发射 (TEOAE)的反应幅值、反应重复率及频带的重复率 ,畸变产物耳声发射 (DPOAE)曲线特点及 1～ 5kHz反应幅值进行分析比较。结果 :S1组、M2 组TEOAE的反应幅值、反应重复性、频带重复性明显低于正常值 ,DPOAE幅值明显降低 ;S2 组、M1组、UNK组TEOAE和DPOAE正常。结论 :在不同致聋因素中 ,有的对单耳产生损伤作用 ,有的对双耳产生损伤作用。     

El implante coclear como tratamiento del acúfeno

Introduction and objectivesTinnitus is a symptom of high prevalence in patients with cochlear pathology. We studied the evolution of tinnitus in patients undergoing unilateral cochlear implantation for treatment of profound hearing loss.MethodsThis was a longitudinal, retrospective study of patients that underwent unilateral cochlear implantation and who had bilateral tinnitus. Tinnitus was assessed quantitatively and qualitatively before surgery and at 6 and 12 months after surgery.ResultsWe evaluated 20 patients that underwent unilateral cochlear implantation with a Nucleus^® CI24RE Contour Advance? electrode device. During the periods in which the device was in operation, improvement or disappearance of tinnitus was evidenced in the ipsilateral ear in 65% of patients, and in the contralateral ear, in 50%. In periods in which the device was disconnected, improvement or disappearance of tinnitus was found in the ipsilateral ear in 50% of patients, and in the ear contralateral to the implant in 45% of the patients. In 10% of the patients, a new tinnitus appeared in the ipsilateral ear.ConclusionsThe patients with profound hearing loss and bilateral tinnitus treated with unilateral cochlear implantation improved in a high percentage of cases, in the ipsilateral ear and in the contralateral ear.     

Clinical features in patients with delayed endolymphatic hydrops

We report clinical features in patients with delayed endolymphatic hydrops (DEH) with juvenile unilateral deafness. Among 23 patients with DEH, 15 cases were diagnosed as ipsilateral DEH and 8 cases as contralateral DEH. The distribution of onset age showed two peaks at ages of < 30 years and > 40 years. In 80% of the ipsilateral DEH cases, the onset of episodic vertigo was at younger ages. On the other hand, in 75% of the contralateral DEH cases, the onset of fluctuation hearing loss of the contralateral ear was at older ages. Ispilateral DEH and Meniere's disease may show different pathophysiologies. The incidence of dominant negative summating potential in the better-hearing ear was 20% in the ispilateral DEH cases and 60% in the contralateral DEH cases. It is suggested that endolymphatic hydrops is in the better-hearing ear of contralateral DEH.     

Pilot study on the effectiveness of the conventional CROS, the transcranial CROS and the BAHA transcranial CROS in adults with unilateral inner ear deafness

The objective of the present pilot study is to evaluate the effectiveness of three conventional contralateral routing of sound (CROS) hearing aids in adults with unilateral inner ear deafness. The study included tertiary referral center. Ten patients with unilateral inner ear deafness and normal hearing in the contralateral ear were selected to evaluate three different methods of amplification: the CROS hearing aid, the completely in the canal hearing aid and the bone-anchored hearing aid CROS (BAHA). Each of the three hearing aids was tried in a random order for a period of 8 weeks. Audiometric performance, including speech-in-noise, directional hearing and subjective benefit were measured after each trial period, using the APHAB, SSQ and single-sided deafness questionnaire. Sound localization performance was essentially at chance level in all four conditions. Mixed results were seen on the other patient outcome measures that alternated in favor of one of the three CROS devices. After the trial, three patients chose to be fitted with the BAHA CROS and one with the conventional CROS. In conclusion, most of the patients experienced some degree of benefit with each of the three hearing aids. Preference for one of the three hearing aids was independent of the order in which they were tried. It would be worthwhile to formulate selection criteria; still, we recommend that all patients with unilateral inner ear deafness should be offered a trial with at least the BAHA CROS.     

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.     

Autoantibodies against inner ear proteins in patients with delayed endolymphatic hydrops and unilateral juvenile deafness

CONCLUSIONS: Patients with the contralateral type of delayed endolymphatic hydrops (DEH) may undergo an autoimmune attack against the other inner ear. As patients with unilateral juvenile deafness show no progression, despite lengthy observation, the autoantibody against the 68-kDa protein may be unrelated to the pathogenesis of DEH. OBJECTIVE: The contralateral type of DEH is believed to have an autoimmune etiology, and sometimes develops from unilateral juvenile deafness. The purpose of this study was to determine whether autoantibodies are pathogenetically important in DEH. MATERIAL AND METHODS: Sera from 9 patients with DEH, 18 patients with profound unilateral juvenile hearing loss and 15 control volunteer without inner ear diseases were investigated by means of Western blot assay against rat inner ear proteins. RESULTS: Among 8 patients with the contralateral type of DEH, 6 (75%) showed at least 1 reactive band on Western blotting. The protein that reacted most frequently had a molecular weight of 28 kDa, which was consistent with our previous results. Among 18 patients with unilateral juvenile deafness, 5 (28%) showed reactive bands, exclusively at 68 kDa.     

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.     

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.     

Inner ear hearing loss--predisposing factor in acute disorders of inner ear function (sudden deafness and vestibular loss)?

49% of 111 patients with sudden hearing loss had pre-existing inner ear damage to a greater extent than would be expected in a population of the same age. The same age-independent hearing loss of inner ear type was seen in 44% of 72 patients with a sudden unilateral isolated vestibular loss. The accumulated occurrence of diseases of the cardial-vascular system, metabolism, and cervical spine is suggested as the reason for the greater extent of inner ear damage with respect of age and the acute disorders in this area. The high proportion of a pre-existing noise deafness in patients (in 38% of men) with sudden hearing loss suggests an increased vulnerability of the inner ear in pre-existing hearing loss.     

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.     

Presidential Speech

Conclusions. Patients with the contralateral type of delayed endolymphatic hydrops (DEH) may undergo an autoimmune attack against the other inner ear. As patients with unilateral juvenile deafness show no progression, despite lengthy observation, the autoantibody against the 68-kDa protein may be unrelated to the pathogenesis of DEH. Objective. The contralateral type of DEH is believed to have an autoimmune etiology, and sometimes develops from unilateral juvenile deafness. The purpose of this study was to determine whether autoantibodies are pathogenetically important in DEH. Material and methods. Sera from 9 patients with DEH, 18 patients with profound unilateral juvenile hearing loss and 15 control volunteer without inner ear diseases were investigated by means of Western blot assay against rat inner ear proteins. Results. Among 8 patients with the contralateral type of DEH, 6 (75%) showed at least 1 reactive band on Western blotting. The protein that reacted most frequently had a molecular weight of 28?kDa, which was consistent with our previous results. Among 18 patients with unilateral juvenile deafness, 5 (28%) showed reactive bands, exclusively at 68?kDa.     

Results of exploratory tympanotomy following sudden unilateral deafness and its effects on hearing restoration

In cases of acute unilateral deafness, no consensus exists as to whether tympanotomy and sealing of the round window should be performed routinely. To further address this issue, we conducted a retrospective study of pre-, intra-, and postoperative findings in 97 patients who had undergone exploratory tympanotomy (EXT) after the onset of sudden and severe unilateral deafness. Our goal was to ascertain, if we could, whether the benefits of EXT outweigh the risks. We also took into account the effects of perilymph fistula (PLF) on the etiology of sudden hearing loss and postoperative outcomes. We found that routine EXT was indeed beneficial for these patients. It was associated with a very low surgical complication rate, and its effects on hearing as assessed by objective measures were beneficial. The greatest benefits were seen in patients who underwent EXT within 7 days after the onset of their hearing loss. With respect to PLF, we found that the presence or absence of the "typical history" of PLF (i.e., a sudden unilateral hearing loss within 48 hours after a precipitating trauma or physical exertion) had no bearing on whether a PLF was actually present in our group; nor was vertigo a reliable predictor of PLF. We recommend that EXT be performed on all patients with new-onset acute unilateral deafness, barring any contraindications, of course. The absence of a typical history of PLF should not dissuade the surgeon from proceeding with EXT.     

迟发性膜迷路积水的内耳自身免疫因素研究

目的：探讨内耳自身免疫性病理因素在迟发性膜迷路积水(DEH)发病中的作用。方法：对26例(同侧性19例，对侧性7例)DEH患者行临床观察、听觉功能测试、免疫学试验；并施以药物治疗、免疫抑制剂治疗、经迷路-耳蜗神经切断术及迷路后前庭神经切断术治疗，再进行疗效分析。结果：特异性抗体阳性：同侧性4例，对侧性l例；对侧性l例特异性细胞免疫反应阳性；同侧性l例循环免疫复合物阳性。ll例药物治疗可控制眩晕发作，8例经行前庭神经切断术后眩晕消失，1例行对侧原发性聋耳鼓室成形术后眩晕消失，4例免疫抑制剂治疗有效，1例自然缓解，1例经药物、免疫治疗及内淋巴囊引流术后无效。结论：导致先期耳聋的常见病因有突发性聋、脑膜炎、麻疹、乳突手术等；DEH与原发性聋的间隔期为1～34年。DEH症状较典型梅尼埃病更难控制。内耳自身免疫性病理因素在本病发病中可能起着重要作用。     

突发性聋患者内耳三维液体衰减反转恢复成像在预后评估中的价值

目的 探讨突发性聋患者内耳在三维液体衰减反转恢复(fluid attenuated inversion recovery,FLAIR)成像上的表现及其与疗效间关系.方法 23例突发性聋患者行三维FLAIR成像,观察内耳在FLAIR上的表现,并测定入院和治疗后的平均听阈,分析内耳信号与治疗结果之间关系.结果23例患者中8例患侧耳蜗在FLAIR上呈高信号,15例患侧及全部健侧耳蜗无信号.就诊时患侧耳蜗无信号组患者平均((x)±s,下同)听阈(听力级,下同)为(80±24) dB,耳蜗高信号组平均听阈为(92±18)dB,差异无统计学意义(t=1.245,P＞0.05);治疗后两组的平均听阈分别为(60±28)dB和(90±21)dB,有效率分别为60.0％和12.5％,差异均有统计学意义(P值均＜0.05).7例伴有眩晕患者中5例患侧半规管在FLAIR上呈高信号,此5例均治疗无效.结论三维FLAIR可显示突发性聋患者内耳淋巴液改变,呈高信号时疗效及预后差.