Hyperlipidemia in association with childhood sensorineural hearing loss

In adults there has been an association noted between hyperlipidemia and sensorineural hearing loss. Etiologic considerations include hyperviscosity of the serum, vascular occlusion and an increased susceptibility to noise. Until now this correlation, to our knowledge, has not been made in the pediatric population. Several children with bilateral fluctuating sensorineural hearing losses have been identified with hyperlipidemia. The fluctuations in hearing varied with lipid levels. With dietary controls, the cholesterol levels returned to what would be near the norm for the pediatric population and hearing returned to near baseline. Unexplained fluctuating sensorineural hearing losses in children warrant the consideration of hyperlipidemia. Discovering a potentially reversible etiology for hearing loss is significant but more importantly, may lead to the early detection of hyperlipidemia in the young patient.     

The relationship between hearing loss and vestibular dysfunction in patients with sudden sensorineural hearing loss

Objective: To investigate the relationship between hearing loss and vestibular dysfunction in patients with sudden sensorineural hearing loss (SSHL).

Methods: Clinical data including the symptom of vertigo of 149 SSHL patients were investigated retrospectively. Pure tone audiometry, ocular vestibular-evoked myogenic potential (oVEMP) and cervical vestibular-evoked myogenic potential (cVEMP) evoked by air-conducted sound (ACS), and caloric test were employed for cochlear and vestibular function assessment. The relationship between hearing level and vestibular dysfunction was analyzed.

Results: The pure tone averages (PTAs) (mean?±?SD) of SSHL patients with and without vertigo were 88.81?±?21.74 dB HL and 72.49?±?21.88 dB HL (Z?=??4.411, p?=?0.000), respectively. The PTAs of SSHL patients with abnormal and normal caloric test were 84.71?±?22.54 dB HL and 70.41?±?24.07 dB HL (t?=??2.665, p?=?0.009), respectively. Conversely, vertigo and abnormal caloric results also happened more frequently in patients with profound hearing loss. However, no consistent tendency could be found among vestibular evoked myogenic potentials (VEMPs) responses or hearing loss.

Conclusions: SSHL patients with vertigo or abnormal caloric test displayed worse hearing loss; and vice versa, vertigo and abnormal caloric results happened more frequently in SSHL patients with profound hearing loss.     

Correlations between audiogram and objective hearing tests in sensorineural hearing loss

Owing to its subjective nature, behavioral pure-tone audiometry often is an unreliable testing method in uncooperative subjects, and assessing the true hearing threshold becomes difficult. In such cases, objective tests are used for hearing-threshold determination (i.e., auditory brainstem evoked potentials [ABEP] and frequency-specific auditory evoked potentials: slow negative response at 10 msec [SN-10]). The purpose of this study was to evaluate the correlation between pure-tone audiogram shape and the predictive accuracy of SN-10 and ABEP in normal controls and in patients suffering from sensorineural hearing loss (SNHL). One-hundred-and-fifty subjects aged 15 to 70, some with normal hearing and the remainder with SNHL, were tested prospectively in a double-blind design. The battery of tests included pure-tone audiometry (air and bone conduction), speech reception threshold, ABEP, and SN-10. Patients with SNHL were divided into four categories according to audiogram shape (i.e., flat, ascending, descending, and all other shapes). The results showed that ABEP predicts behavioral thresholds at 3 kHz and 4 kHz in cases of high-frequency hearing loss. Also demonstrated was that ABEP threshold estimation at 3 kHz was not affected significantly by audiogram contour. A good correlation was observed between SN-10 and psychoacoustic thresholds at 1 kHz, the only exception being the group of subjects with ascending audiogram, in which SN-10 overestimated the hearing threshold.     

Reversible sensorineural hearing loss

We present an unusual case of temporary sensorineural hearing loss in a 6-year-old child due to carbon monoxide. This was shown on both the audiograms and confirmed with objective testing using otoacoustic emissions. Carbon monoxide poisoning is one of the few recognised causes of reversible sensorineural hearing loss, though it may also lead to a permanent deficit. This is discussed along with other potential causes of reversible sensorineural hearing loss.     

自身免疫性感音神经性聋

自身免疫性感音神经性聋(autoimmune sensorineural hearing loss,ASNHL)是侵犯耳蜗[1、2]及蜗后[3]的自身免疫性疾病,这一概念由美国学者McCabe在1979年     

The relationship between thiol-disulfide balance and idiopathic sudden sensorineural hearing loss

IntroductionImpaired cochlear perfusion is a major etiological factor in idiopathic sudden sensorineural hearing loss. Oxidative stress has been shown to be a risk factor for oxidative damage.ObjectivesWe investigated the role of oxidative stress in idiopathic sudden sensorineural hearing loss by comparing serum levels of oxidant and antioxidant molecules including thiol/disulfide homeostasis paraoxonase, stimulated thiol/disulfide homeostasis paraoxonase, arylesterase, ceruloplasmin and myeloperoxidase in patients who did and did not recover after treatment.MethodsThe amount of dynamic disulfide was calculated by determining half of the difference between the total thiols and native thiols. After the determination of native, total thiol, and disulfide amounts, the disulfide/total thiol percent ratio, native thiol/total thiol ratio and disulfide/native thiol percent ratio were calculated and then compared between the two groups. Additionally, clinical relationship between audiological recovery and native thiol, disulfide, disulfide/native thiol percent ratio, and disulfide/total thiol percent ratio levels was investigated. Blood samples were also analyzed for the assessment of thiol/disulfide homeostasis paraoxonase, stimulated thiol/disulfide homeostasis paraoxonase, arylesterase, ceruloplasmin, and myeloperoxidase levels.ResultsA significant difference was found between the two groups with regard to total oxidant status disulfide, disulfide/native thiol percent ratio, disulfide/total thiol percent ratio, and native thiol/total thiol ratio levels (p = 0.001, p = 0.001, p = 0.001, p = 0.003, p = 0.001, p = 0.002, respectively). However, no significant difference was found between the two groups with regard to thiol/disulfide homeostasis paraoxonase, stimulated thiol/disulfide homeostasis paraoxonase, ceruloplasmin, and myeloperoxidase levels (p > 0.05 for all).ConclusionThe results supported the common hypothesis that vascular pathologies are the primary cause of idiopathic sudden sensorineural hearing loss and that other etiological factors ultimately result in vascular pathologies. The oxidant-antioxidant and thiol-disulfide balances were impaired in the idiopathic sudden sensorineural hearing loss group.     

Macrovascular sensorineural hearing loss

Sensorineural hearing loss (SNHL) has many etiologies including vascular sources. Vascular causes of SNHL can occur at the microvascular level. Macrovascular level may be described as arising from vessels proximal to the labyrinthine artery or those vessels that can be visualized without the aid of a microscope. Otologic symptomatology and diagnostic evaluation of the SNHL may reveal a macroscopic vascular source. Representative cases of macrovascular sensorineural hearing loss due to aneurysm, arteriovenous malformation, and vertebrobasilar artery dolichoectasia are presented.     

Treatable sensorineural hearing loss

Sensorineural hearing loss is generally felt to be an untreatable medical condition. However, in some cases, prompt diagnosis and treatment of the underlying condition may reverse the deafness. This article summarizes various treatable forms of sensorineural hearing loss and provides illustrative cases histories of patients who have had sensorineural hearing losses that were improved by medical or surgical intervention. Patients with reversible sensorineural deafness due to inadvertent aminoglycoside over-dosage, congenital cholesteatoma, Meniere's syndrome, blood coagulopathy, and perilymphatic fistula all had improvements in auditory function after medical or surgical intervention. Recent experimental studies on animals may explain the basic mechanisms behind hearing loss and recovery. Aminoglycoside ototoxicity appears to have an initial reversible step, followed by a permanent process. Early endolymphatic hydrops and fistulas may cause mechanical effects in the cochlea which can be corrected. Coagulopathy may cause hypoxia which reverses after anticoagulation. These observations reveal that animal experiments can be useful in explaining human auditory dysfunction of the reversible type.     

突发性极重度聋与全聋的预后特点及差异

Objective To clarify the different prognostic characteristics between profound sudden sensorineural hearing loss (SSNHL) and total SSNHL. Methods The patients with SSNHL who visited Eye Ear Nose and Throat Hospital from June 2007 to September 2008 were reviewed retrospectively. All the 204 patients, with pure tone average (PTA) threshold more than 90 dB, were enrolled and divided into two groups, including total SSNHL and profound SSNHL groups. The relationship between recovery rate and prognostic factors including the age, complications, time period between onset and therapy was analyzed.Results There were 57 cases of total SSNHL and 147 cases of profound SSNHL in this series. Tinnitus was complained in more than 90% of the patients in both groups, which was higher than that of dizziness and ear fullness. Dizziness was present in 64. 9% (37/57) patient with total SSNHL group and 45.6% (67/147)patients with profound SSNHL, which had significant difference between the two groups (x2 = 5.72,P =0. 017). The PTA threshold improvement in total SSNHL group and profound SSNHL group was (36. 4 ±19.3) dB and (40. 2 ±21.3) dB respectively, which was no significant difference between the two groups (t = 1. 165 ,P =0. 245). The cured patients were all those received therapy within 1 week following the onset of SSNHL, which was of 2. 6% (1/38) patients in the total SSNHL group and 14. 3% (14/98) patients in the profound SSNHL group(P =0. 045). Furthermore, 3.5% (2/57) patients in total SSNHL group as well as 29. 9% (44/147) patients in profound SSNHL group obtained a good result with PTA threshold ≤50 dB after therapy(x2 = 15.92,P = 0. 001 ). In addition, the favorable prognosis was related with the onsettherapy time point( P = 0. 001 ), but not related to the patients' age. Conclusion Profound SSNHL and total SSNHL though both with PTA threshold > 90 dB had significant differences recovery rate and need to be studied separately.     

Carbamazepine-induced sensorineural hearing loss

Carbamazepine is a commonly prescribed anticonvulsant medication that affects various levels of the nervous system. We report a case of temporary sensorineural hearing loss in a patient after overdosing with 36 g of carbamazepine. Six days after the overdose, the patient complained of bilateral hearing loss and tinnitus. Audiograms revealed a 30- to 40-dB sensorineural hearing loss bilaterally. Another audiogram 2 weeks later showed a complete recovery in both ears accompanied by a clinical resolution in audiovestibular symptoms. Carbamazepine is used to treat partial and generalized seizures, trigeminal neuralgia, and bipolar illness. Adverse effects are not common but most frequently include dizziness, drowziness, nausea, and skin rashes; rare complications are agranulocytosis, bradycardia, and heart block. Documented hearing loss as a side effect of carbamazepine has not been reported, to our knowledge.     

突发性极重度聋与全聋的预后特点及差异

Objective To clarify the different prognostic characteristics between profound sudden sensorineural hearing loss (SSNHL) and total SSNHL. Methods The patients with SSNHL who visited Eye Ear Nose and Throat Hospital from June 2007 to September 2008 were reviewed retrospectively. All the 204 patients, with pure tone average (PTA) threshold more than 90 dB, were enrolled and divided into two groups, including total SSNHL and profound SSNHL groups. The relationship between recovery rate and prognostic factors including the age, complications, time period between onset and therapy was analyzed.Results There were 57 cases of total SSNHL and 147 cases of profound SSNHL in this series. Tinnitus was complained in more than 90% of the patients in both groups, which was higher than that of dizziness and ear fullness. Dizziness was present in 64. 9% (37/57) patient with total SSNHL group and 45.6% (67/147)patients with profound SSNHL, which had significant difference between the two groups (x2 = 5.72,P =0. 017). The PTA threshold improvement in total SSNHL group and profound SSNHL group was (36. 4 ±19.3) dB and (40. 2 ±21.3) dB respectively, which was no significant difference between the two groups (t = 1. 165 ,P =0. 245). The cured patients were all those received therapy within 1 week following the onset of SSNHL, which was of 2. 6% (1/38) patients in the total SSNHL group and 14. 3% (14/98) patients in the profound SSNHL group(P =0. 045). Furthermore, 3.5% (2/57) patients in total SSNHL group as well as 29. 9% (44/147) patients in profound SSNHL group obtained a good result with PTA threshold ≤50 dB after therapy(x2 = 15.92,P = 0. 001 ). In addition, the favorable prognosis was related with the onsettherapy time point( P = 0. 001 ), but not related to the patients' age. Conclusion Profound SSNHL and total SSNHL though both with PTA threshold > 90 dB had significant differences recovery rate and need to be studied separately.     

突发性极重度聋与全聋的预后特点及差异

目的 研究突发性极重度聋和全聋的预后特点及差异.方法 回顾性研究复旦大学附属眼耳鼻喉科医院2007年6月至2008年9月收治的初始平均纯音听阈(pure tone average,PTA)>90 dB、随访完整的204例患者,分为全聋和极重度聋两组,对两组的预后进行比较,并对预后与年龄、并发症、发病-就诊时间进行相关性分析.结果 全聋组57例,极重度聋组147例,耳鸣、眩晕、耳闷三大伴随症状中,耳鸣的伴随率最高,两组均达90%以上,眩晕的伴随率全聋组为64.9%(37/57),极重度聋组为45.6%(67/147),两组间差异有统计学意义(x2=5.72,P=0.017).治疗后全聋组PTA下降(36.4±19.3)dB,极重度聋组下降(40.2±21. 3)dB,差异无统计学意义(t=1.165,P=0.245).两组痊愈者均为1周内接受治疗者,全聋组痊愈率为2.6%(1/38),极重度聋组为14.3%(14/98),两组间差异有统计学意义(Fisher确切概率法,P=0.045).全聋组治疗后PTA≤50 dB的患者占3.5%(2/57),极重度聋组达29.9%(44/147),两组间差异有统计学意义(x2=15.92,P=0.001).两组的预后与发病-就诊的时间有关(P值均为0.01),与年龄无关.结论 初始PTA>90 dB的突发性聋可分为极重度聋与全聋,两者预后有差异,需分开进行研究.     

感音神经性耳聋的预防及治疗

人类耳蜗有内外两种毛细胞。内毛细胞把外界传人内耳的声信号转换成电信号。外毛细胞对声信号起到协调作用。听觉神经元把电信号进一步传人听觉通道，最后传人听觉中枢。不幸的是，无论毛细胞或听神经元的损伤都可导致永久性不可逆转的感音神经性耳聋。占人类10％以上的成年人群都患有不同程度的感音神经性耳聋，随着世界人1：3的老龄化，耳聋占人类群体的比例会进一步上升。     

MTHFR AND ApoE genetic variants association with sudden sensorineural hearing loss

Hypothesis

Although the pathogenesis of sudden sensorineural hearing loss (SSNHL) is not clear, however several causes including genetic factors seems to be implicated. We hypothesized that common genetic variants might be involved in SSNHL.

Database for sensorineural hearing loss

We are creating a bank of EBV immortalized lymphoblast cells and extracted DNA taken from the blood of deaf children and their relatives, in order to study the molecular basis of hereditary deafness. We have established a corresponding database for sensorineural hearing loss that records clinical data for each entered specimen. The purpose of this paper is to present the content and design of the computerized relational database. The data model is designed first to identify known etiologies of deafness, either acquired or syndromic, and then to characterize the clinical features of the deaf individual, and both their affected and non-affected family members. The application operates in a graphical environment of visual prompts and message panels. The database is organized by sections which record demographic data, presenting complaints, otologic history, birth and perinatal history, developmental history, symptoms of chronic airway obstruction, family history, neurologic history, congenital infections, hospitalizations and surgical history, medication history, vestibular findings, audiometry, radiology, medical conditions and syndromes and physical examination. The database was developed on a commercially available software product. Our database is presented as a model for use by other clinicians and investigators.