突发性耳聋伴眩晕的临床分析

目的 探讨突发性耳聋(简称突聋)伴良性阵发性位置性眩晕(BPPV)或突聋伴眩晕综合征(非BP-PV)与突聋不伴眩晕的临床特征及疗效.方法 选取2018年1月-2019年12月收治的400例突聋患者,根据是否伴有眩晕症状,分为突聋伴眩晕组(163例)和突聋不伴眩晕组(237例)两组.163例突聋伴眩晕组患者进一步分为突聋...     

Van Der Hoeve-De Kleiyn’S syndrome as a rare cause of hearing loss - two case reports

It is relatively rare, dominant form of syndrome characterized by combination of blue sclerae brittle banes and Hearing loss. One of the conditions may be absent. Two cases, one 22 year old mtde with UltUtgray selrae md the other a 25 year old female with blue sclerae presented to us with hearing loss. Family history of hearing toss and blue sclerae were positive.     

Demyelinating disease as the assumed cause of hearing loss and vertigo

Summary A patient initially presenting typical symptoms of idiopathic sudden deafness later developed disabling episodic vertigo, which led to translabyrinthine eighth nerve transection. Morphological examination of the removed cochlear nerve specimen revealed a demyelinating process in the neuroglial portion of the nerve. The major part of the peripheral, neurolemmal portion of the cochlear nerve was normal. The inferior vestibular nerve was fibrotic. The major part of the superior vestibular nerve was normal. Some of its peripheral bundles showed increased endoneurial fibrosis. It is suggested that a demyelinating process was the cause of the patient's symptoms.     

Otitis media as a cause of significant hearing loss among Nigerians

Mild or worse hearing loss, defined as pure tone average >25 decibel, is seen commonly with preventable etiologies.

Settings

A tertiary care, urban referral hospital.

Methods

Retrospective analysis of prospectively collected data of individuals attending the hearing loss clinic of National Hospital, Abuja, between May 2005 and April 2007. Data matching the diagnosis of acute otitis media (AOM), otitis media with effusion (OME) and chronic suppurative otitis media (CSOM) were extracted from the database and analyzed.

Result

A total of 298 cases with primary presenting complaints of hard of hearing were seen. A total of 77 cases had hearing loss due to otitis media. 44 (57.1%) had OME, 26 (33.8%) had CSOM, while 7 (9.1%) had AOM. The observed distribution of the OME was left OME (18.1%, n = 14), right OME (9.1%, n = 7) and bilateral OME (29.9%, n = 23), while the CSOM is distributed into left CSOM (15.5%, n = 12), bilateral CSOM (11.7%, n = 9) and right CSOM (6.4%, n = 5), and AOM is distributed into right AOM (2.6%, n = 2), left AOM (3.9%, n = 3), and bilateral AOM (2.6%, n = 2). The mean pure tone averages for the three groups are AOM (30.5 dB), OME (41.5 dB) and CSOM (56.9 dB).

Conclusion

Otitis media is an important cause of preventable hearing loss in developing countries, and the predominance of unilateral otitis media in the left ear observed in this study deserves further studies.     

Progressive hearing loss in Fabry’s disease: a case report

Fabry’s disease is a chromosomal X-linked inherited disease, which causes a lack of the lysosomal alpha-galactosidase A enzyme leading to a cellular accumulation of glycosphingolipids. This accumulation leads to various clinical disorders, including inner ear lesions, with sensorineural hearing loss and dizziness. This article proposes to describe a clinical case of a patient suffering from Fabry’s disease with inner ear associated problems and to review the literature focusing on this subject.     

突发性聋伴发良性阵发性位置性眩晕的临床特点及疗效观察

目的　探讨突发性聋伴发良性阵发性位置性眩晕（benign paroxysmal positional vertigo,BPPV）的临床特点,观察其疗效。方法　观察2012年10月～2014年6月耳 鼻咽喉科收治的36例突发性聋伴发BPPV发病情况,并与同期原发性BPPV患者40例及突发性聋不伴眩晕患者40例进行疗效比较。结果　伴发BPPV的36例突发性聋患者,受累半规管均与突发性聋发病侧一致,其中水平半规管BPPV 6例,占16.7%（6/36）;后半规管BPPV 27例,占75.0%（27/36）;混合管BPPV 3例,占8.3%（3/36）。36例患者出现BPPV的时间均在突发性聋发病后数小时至数天（＜10天）发生。突发性聋伴发BPPV组1次治愈率明显低于原发性BPPV组,但两种治疗方法的总治愈率均为100%。不伴眩晕突发性聋组的痊愈率、显效率及有效率均高于伴发BPPV组。结论　伴发于突发性聋的BPPV以后半规管多见,与原发性BPPV经耳石复位治疗后均可取得较好疗效。而不伴眩晕的突发性聋其疗效优于伴发BPPV的突发性聋。     

Double challenge: cochlear implantation in the only hearing ear with progressive hearing loss following meningitis and vestibular dysfunction after implantation

ObjectiveVestibular dysfunction associated with cochlear implantation is rare. It is usually seen in patients with otosclerosis due to spread of electrical activity throughout the demineralized bone. A 17-year old female with progressive hearing loss 2 years after meningitis and vestibular dysfunction in the implanted ear is presented in this study.FindingsThe patient had mild hearing loss in the right ear and total hearing loss on the left side because of complete ossification of the cochlea following meningitis. She had to have cochlear implantation in the right ear because of progression of hearing loss. She had successful implantation but she experienced vestibular dysfunction following activation of cochlear electrodes. Closure of two electrodes caused disruption of auditory programming. Then the patient was subjected to long term vestibular rehabilitation program.ConclusionTiming for implantation before the completion of cochlear ossification is crucial not to miss the chance for hearing restoration. However, difficulties in hearing rehabilitation due to extensive ossification can be doubled by vestibular problems triggered by stimulation of the vestibular nerve by cochlear electrodes. Attempts to reduce the balance problem will complicate auditory programming. Vestibular rehabilitation for long term helps to carry on hearing progress.     

Suppressed sneezing as a cause of hearing loss and vertigo

Two cases of inner ear injury caused by suppressed sneezing are described. One patient experienced vestibular symptoms in the form of reflexogenic vertigo that was relieved by surgical section of the tensor tympani tendon. The other patient had a sudden severe permanent sensorineural hearing loss. It is proposed that the aerodynamic pressure increase associated with suppressed sneezing is transmitted via the eustachian tube to cause an implosive fistula of either the round or oval window with injury to the membranous labyrinth.     

Clinical characteristics and prognosis of low frequency sensorineural hearing loss without vertigo

Conclusion: Factors prognostic of recovery from low frequency sensorineural hearing loss without vertigo are co-occurrence of tinnitus, PTA determined hearing level on the affected side before treatment initiation, and time from onset to treatment initiation. The rate of hearing recovery was higher in the oral steroid than in the intra-tympanic steroid and combined groups. Objectives: Although many studies have assessed acute sudden hearing loss, few have analyzed low-frequency hearing loss. Clinical characteristics of patients with hearing loss may vary by type of hearing loss. This study, therefore, analyzed the clinical characteristics, recovery rates, and factors associated with hearing recovery in patients with low-frequency sensorineural hearing loss unaccompanied by vertigo. Method: This study included 50 patients with severe low frequency hearing loss unaccompanied by vertigo and with normal tympanic membrane status who visited hospitals from July 2005 to May 2014 due to sudden tinnitus, ear fullness, or hearing loss. Of these patients, 29 were treated with oral steroids, eight with tympanic steroid injections, and 13 with both. Clinical and auditory characteristics before and after treatment, as well as treatment outcomes, were compared in these three groups. Results: Age, sex, affected side, co-occurrence of ear fullness, and accompanying chronic diseases were similar in the three groups. All patients started on treatment within 10 days of hearing loss showed significant recovery, with complete recovery, unaccompanied by tinnitus, observed in 10 patients. Treatment outcomes were not affected by hearing thresholds on the unaffected side. Of the patients treated with both oral and intra-tympanic steroids, 39% showed complete recovery, and 77% showed audiometric improvement. The complete recovery rate was significantly higher in the oral steroid than in the other two groups (p?=?0.029); and the audiometric improvement rate tended to be lower in the intra-tympanic steroid group than in the other groups. Cure rates, defined as complete disappearance of symptoms and normal findings on pure tone audiograms (PTA), in the oral, intra-tympanic, and combined steroid groups were 49%, 25%, and 23%, respectively. There were no significant differences among the three groups in cure, subjective improvement, and audiometric improvement rates. Factors prognostic of non-recovery of hearing included late treatment initiation (p?=?0.044), accompanying tinnitus (p?=?0.049), and higher hearing thresholds on the affected side before treatment initiation (p?=?0.005).     

Hearing results of stapedotomy and malleo-vestibulopexy in congenital hearing loss

Aims

To analyze hearing results of surgical treatment of hearing loss associated with the congenital stapes ankylosis with or without malformations of ossicular chain.

Study design

Retrospective chart review.

Methods

The charts of 1369 stapedotomies performed by senior author (JH) from 1991 to 2006 were reviewed. In 40 cases operative findings were consistent with isolated congenital stapes fixation or associated with middle ear malformations. The modified stapedotomy technique was employed in 33 cases and malleo-vestibulopexy was used in 7 cases. Operative findings were standardized according to Cremers’ classification. The outcomes of 40 surgeries were analyzed according to the 1995 AAO-HNS Committee on Hearing and Equilibrium guidelines. High frequency hearing results on 4, 8 and 12 kHz were reported in addition to standard frequencies. Results of stapedotomies and malleo-vestibulopexies were calculated separately. Surgical complications were described.

Results

The mean post-operative air conduction (AC) was 33 dB, bone conduction (BC) 22 dB and speech reception thresholds (SRT) 31 dB. Closure of the air-bone gap (ABG) to within 10 dB was achieved in 24/40 (60%) of cases. Lack of improvement was observed in 3/40 (8%) patients. In 26/32 (81%) of cases with potential for bilaterally serviceable hearing it was achieved. In 24/40 (60%) of cases symmetrical hearing with interaural difference of less than 10 dB was demonstrated.

Conclusion

Significant hearing gain in patients with congenital stapes ankylosis makes surgical treatment a valuable adjunct or an alternative to hearing aids in selected cases.     

耳后注射甲强龙作为补救方案在中高频听力下降型突发性聋治疗中的疗效及可行性分析

目的探析中高频听力下降型突发性聋采取耳后注射甲强龙治疗的临床效果及可行性。方法选取2019年5月~2020年7月本院耳鼻喉科就诊的中高频听力下降型突发性聋患者,共纳入病例54例,以随机法分组,即常规组(n=27)和观察组(n=27),常规组予常规治疗,观察组采取耳后注射甲强龙,比较两组效果。结果观察组有效率相比于常规组显著提高(P<0.05);治疗3周后,观察组2000~8000Hz频率听力提高值与常规组比(P<0.05)。结论中高频听力下降型突发性聋采取耳后注射甲强龙治疗效果理想,值得推广。     

A new model for photochemically induced thrombosis in the inner ear microcirculation and the use of hearing loss as a measure for microcirculatory disorders

Summary A new photochemical method was employed to cause disorders in the inner ear's microcirculation, using the rat as an animal model. Hearing loss was used as a measure for establishing the altered microcirculation. Under pentobarbital anesthesia, the middle ear was opened by a ventral approach. The lateral wall of the cochlea was then illuminated with a filtered xenon lamp (wavelength 540 nm) while rose bengal was infused intravenously. Photoactivated rose bengal produces oxygen radicals and oxygen singlets, which subsequently damage the vascular epithelium to cause the adhesion and aggregation of platelets in the small vessels. Disintegration of the inner ear hair cells at the irradiated site became evident 24 h after the illumination. These findings further suggest that the photochemical occlusion in the inner ear's microcirculation led to ischemic damage of the stria vascularis and the hair cells in the inner ear. When the action potential (AP) of the cochlea was measured with an electrocochleogram a gradual decrease occurred after the illumination. When acetylsalicylic acid was injected intravenously before treatment, the time required to completely suppress the AP was prolonged in a dose-dependent manner. Findings indicate that our method causes a photochemically induced occlusion in the inner ear's microcirculation and is therefore potentially useful for evaluating the various effects of drugs on the ear. Offprint requests to: K. Umemura, Department of Pharmacology, Hamamatsu University School of Medicine, 3600 Handa-cho, Hamamatsu 431-31, Japan     

Influence of myelography on the developmental curve of auditory brainstem responses and hearing loss

Auditory threshold and auditory brainstem response studies were conducted in 50 patients before and after myelography. Analysis of amplitudes and latencies of auditory brainstem measurements demonstrated significant disorders of function of the cochlea and auditory pathway. The Jewett I wave showed a prolongation of latency from 1.92 to 1.98 ms using an average of all repetition rates. The Jewett III wave showed prolongation from 4.01 to 4.14 ms and the Jewett V wave prolongation from 6.01 to 6.16 ms. At the same time average amplitudes for Jewett III and V decreased. In most of the patients these disorders of function were found to be subclinical. However, 12 patients had changes ranging from a subjectively slight hearing loss to an audiometrically defined acute hearing loss. The reasons for these disorders could not be clarified. An open cochlear aqueduct through which perilymph can enter the subarachnoid space, leading to secondary endolymphatic hydrops, was suggested as the cause for the losses found. Changes in brainstem audiometry were also explained by changes in osmolality of inner ear fluids, leading to the development of an endolymphatic hydrops.     

Transitory endolymph leakage induced hearing loss and tinnitus: depolarization,biphasic shortening and loss of electromotility of outer hair cells

There are types of deafness and tinnitus in which ruptures or massive changes in the ionic permeability of the membranes lining the endolymphatic space [e.g., of the reticular lamina (RL)] are believed to allow potassium-rich endolymph to deluge the low [K⁺] perilymphatic fluid (e.g., in the small spaces of Nuel). This would result in a K⁺ intoxication of sensory and neural structures. Acute attacks of Ménière's disease have been suggested to be an important example for this event. The present study investigated the effects of transiently elevated [K⁺] due to the addition of artificial endolymph to the basolateral cell surface of outer hair cells (OHC) in replicating endolymph-induced K⁺ intoxication of the perilymph in the small spaces of Nuel. The influence of K⁺ intoxication of the basolateral OHC cell surface on the transduction was then examined. Intoxication resulted in an inhibition of the physiological repolarizing K⁺ efflux from hair cells. This induced unwanted depolarizations of the hair cells, interfering with mechanoelectrical transduction. A pathological longitudinal OHC shortening was also found, with subsequent compression of the organ of Corti possibly influencing the micromechanics of the mechanically active OHC. Both micromechanical and electrophysiological alterations are proposed to contribute to endolymph leakage induced attacks of deafness and possibly also to tinnitus. Moreover, repeated or long-lasting K⁺ intoxications of OHC resulted in a chronic and complete loss of OHC motility. This is suggested to be a pathophysiological basis in some patients with chronic hearing loss resulting from Ménière's syndrome.     

Prevention and management of hearing loss in syndromic craniosynostosis: A case series

ObjectiveTo assess the audiological profile in a cohort of children affected by syndromic craniosynostosis.MethodsEleven children with Apert syndrome (n = 4), Saethre-Chotzen syndrome (n = 3), Muenke syndrome (n = 2), Crouzon syndrome (n = 1) and Pfeiffer syndrome type 1 (n = 1) were submitted to a complete audiologic evaluation including otoscopy, pure-tone audiometry, tympanometry and acoustic reflex testing, ABR, otoacustic emissions, temporal bone High Resolution CT (HRCT) scan. The main outcome measures were prevalence, type and severity of hearing loss, prevalence of chronic otitis media, correlation with the time of first surgical correction.ResultsSeven of 11 patients (64%) presented hearing loss (HL), conductive in 3/7 patients (43%) and mixed in 4/7 (57%). No patients showed a purely sensorineural HL. All hearing impaired patients displayed middle ear disorders: the patients with conductive HL had otitis media with effusion (OME) and 3/4 patients with mixed HL showed tympanic alterations or cholesteatoma. A bilateral vestibular aqueduct enlargement was detected by HRCT scan in one normal hearing patient. The ABRs resulted normal in all cases.ConclusionOur study confirms the high prevalence of otologic diseases in such patients. In contrast with previous studies, middle ear disorders were responsible for the hearing impairment also in patients with mixed HL due to secondary inner ear damage. These findings restate the necessity of a close audiologic follow-up. We did not detect the specific ABR abnormalities previously reported, possibly because of an early correction of the cranial vault malformations.     

Auditory neuropathy/dyssynchrony as a cause of failed neonatal hearing screening

The prevalence of auditory neuropathy/dyssynchrony (AN/AD) is not exactly known. We retrospectively analysed the prevalence of this condition among 135 infants who failed a neonatal screening. Hearing screening was performed by automated auditory brainstem responses (AABR). Unilateral presence of click-evoked oto-acoustic emissions with absent auditory brainstem responses was found in 4 infants. Magnetic resonance imaging of the posterior fossa showed an aplasia/hypoplasia of the ipsilateral cochlear nerve in these 4 cases. The prevalence of AN/AD was 19% in infants with confirmed hearing loss. Our findings underscore the role of AABR in neonatal hearing screening.     

Solitary parathyroid adenoma: a rare cause of primary hyperparathyroidism in children

Solitary parathyroid adenoma is a rare cause of primary hyperparathyroidism in children. We report the cases of four children, aged 7 to 14 years old, who presented with a variety of symptoms (growth retardation, glomerulonephritis, pathological fracture, mood swings). Diagnosis was based on clinical examination, imaging, and biochemical analysis. Treatment consisted of surgical excision of the adenoma. As diagnosis was delayed in all four patients, we recommend systematically checking serum calcium levels in children with certain nonspecific symptoms. Adenoma resection usually restores normal serum calcium levels and, hence, avoids further complications.     

Hearing loss due to urate deposition in the middle ear: A case report and literature review

Gout is the most common cause of monoarthritis in men occurring classically in the great toe and the knee.Extra-articular gout manifestations are rare.Only a few cases of head and neck urate crystals deposits have been described in the literature.Precipitations in the middle ear cause conductive hearing loss with common otoscopic anomalies and difficult imaging diagnosis.We report a case of a healthy 58-years-old man with a middle ear urate deposit causing a progressive hearing loss as the very first symptom of gout.The nature of the deposit was unsure on computer tomography(CT)due to atypical density.The final diagnosis was revealed after surgical procedure and histologic examination.A review of the literature is also presented.Seven cases of middle ear urate deposit as the first symptom of gout were found and compared.Progressive conductive hearing loss in middle-aged patients with abnormal otoscopy and middle ear atypical density mass on CT scan must lead to a minimal surgical procedure with a histologic examination to exclude urate crystals deposits.     

Risk factors and etiology of childhood hearing loss: a cohort review of 296 subjects

Abstract

Background: The association between the Joint Committee on Infant Hearing (JCIH) risk factors and etiology of hearing loss (HL) is not studied well in children.

Objectives: To clarify the etiologic causes and evaluate the JCIH risk characteristics of children with HL.

Methods: A retrospective study of 296 children with HL born between 2009.01 and 2013.12 in Stokholm. Demographic data, family and medical histories, audiologic results, imaging findings, and genetic results were ascertained and analyzed.

Results: In 221 with bilateral hearing loss (BHL), family history and neonatal risk indicators were the most common risks (59 each), followed by syndrome related risks. In 75 with unilateral hearing loss (UHL), craniofacial anomaly was the most common risk, followed by family history. Etiology was established in 93 with BHL, in which syndromic HL accounted for 37.2%, chromosomal aberrations for 21.3%, and environmental causes for 19.1%. Etiology was established in 35 with UHL, in which ear malformation accounted for the most (74.3%), followed by environmental causes (14.3%).

Conclusions and significance: Childhood HL can be attributed to a variety of causes with an etiology identifiable in 42.5% of BHL and 46.7% of UHL. BHL and UHL have different patterns of JCIH risk exposure and etiology.