Recovery from sensorineural deafness in Wegener's granulomatosis

Wegener's granulomatosis may present with deafness or other aural symptoms. This report describes two patients with histological evidence of Wegener's granulomatosis who developed reversible sensorineural hearing loss during the course of their illness. The first patient showed complete recovery of a sensorineural hearing loss averaging 50 dB after ten months treatment with cyclophosphamide and high-dose prednisolone. The second patient, who was on maintenance haemodialysis, achieved a 40 dB improvement in sensorineural hearing loss within two weeks of adding cyclophosphamide to pre-existing corticosteroid therapy. These findings suggest that the prognosis of sensorineural hearing loss in Wegener's granulomatosis can be improved with suppression of the vasculitic process by early treatment with combined cytotoxic-immunosuppressive therapy.     

Ear involvement in Wegener's granulomatosis

In 16 of 19 patients with biopsy-proved Wegener's granulomatosis the early manifestations were limited to the ear and nose. The audiological data of 13 patients revealed middle ear involvement in 16 of 26 ears. Twenty-one of 26 ears presented with a low to moderate sensorineural hearing loss. One ear remained deaf after a sudden hearing loss in the early stage of the disease. Serologically, 4 of 6 tested patients with sensorineural hearing loss demonstrated antibodies against sarcolemma. One patient showed antinuclear antibodies. It is remarkable that these antibodies can often be detected in classic inner ear disorders. The course of inner ear function, serum findings and the success of immunosuppressive therapy in Wegener's granulomatosis are comparable with immunologically mediated vasculitis in the inner ear.     

Hearing results in retrolabyrinthine vestibular neurectomy.

Patients having retrolabyrinthine vestibular neurectomy (RLVN) may have complications that compromise hearing. While most reviews have emphasized sensorineural loss, less attention has been given to conductive hearing loss, which may complicate RLVN. Hearing results of 25 consecutive cases of RLVN performed for Meniere's disease with incapacitating vertigo were tabulated according to 1985 American Academy of Otolaryngology (AAO) guidelines. Nine patients (36%) had improved hearing postoperatively, 5 (20%) had no change in hearing, and 11 (44%) had worse hearing postoperatively. The most commonly observed audiometric change was low-frequency conductive hearing loss, presumably secondary to partial ossicular fixation by bone dust or fat fibrosis in the attic and antrum. Five patients (20%) had low-frequency conductive hearing losses that increased by 10 dB or greater over preoperative levels. An additional 7 patients had lesser losses at low frequencies. One patient had a flat conductive hearing loss. Six (24%) of the patients had a decrease in bone levels of greater than 10 dB. Overall hearing results in this study are comparable to those of other series in the literature. Causes and prevention of conductive hearing loss in RLVN are discussed, and a format for presentation of hearing data that will highlight conductive hearing loss after surgery for Meniere's disease is presented.     

Wegener's granulomatosis with otological disorders as primary symptoms

Otological symptoms as initial manifestations of Wegener's granulomatosis have been observed in 3 patients. In one of them, symptoms consisted of a bilateral sensorineural hearing loss, improved by corticoid therapy. No other organ system was involved and laboratory tests remained within normal limits for 2 years after the onset of otological signs. In the other 2 patients, Wegener's granulomatosis manifested mainly as serous otitis media. Otologic involvement underscores the role of the otolaryngologist in the early diagnosis and treatment of this disease.     

Acute onset Wegener's granulomatosis presenting as otitis media; a case report

We report the case of a 57-year-old woman with Wegener's granulomatosis who presented with otitis media. The patient presented with a 2-month history of bilateral hearing loss and dizziness. Antibiotic treatment was not effective, and the patient was confirmed to have bilateral sensorineural hearing loss. Serum was positive for cytoplasmic anti-neutrophil cytoplasmic antibody [C (PR3)-ANCA] but negative for P(MPO)-ANCA (perinuclear ANCA). While the test results were pending, the patients' general condition worsened ; in particular, signs of active inflammation, cardiorespiratory failure and scleritis developed suddenly. Emergency chest computed tomography revealed evidence of lung involvement and hydrothorax; thus, the patient was diagnosed to have the generalized form of Wegener's granulomatosis. The patient was started on pulse methylprednisolone therapy at 1000mg/day for 3 days, which resulted in marked clinical improvement, and then, the drug therapy was switched to prednisolone 60mg/day and cyclophosphamide 50mg/day and gradually tapered. Early diagnosis of Wegener's granulomatosis is often difficult because of atypical manifestations of the disease; particular attention must be paid to acute onset of the disease, such as in our case. ANCA is a very useful marker for early diagnosis, but about one week is needed to obtain the test results. We believe that early steroid and cyclophosphamide therapy is an effective therapeutic option for patients with signs of severe inflammation and generalized involvement.     

Wegener's granulomatosis: otologic and clinico-immunologic aspects]

Clinical, audiometric and immunological data of 26 patients with histological proven Wegener's granulomatosis are presented. In 21 patients the middle ear was involved with serous otitis media, adhesive process or subacute otitis media. 28 of 36 audiometric examinations showed a mild to moderate sensorineural hearing loss. One ear did not recover from a sudden deafness in the early stage of the disease despite of immunosuppressive therapy. Beside clinical and morphological findings in Wegener's granulomatosis the detection of anticytoplasmic antibodies against neutrophil granulocytes (ANCA) is a reliable method in diagnosing the disease. In the present study, the serum of 19 out of 26 patients with histologically proven Wegener's granulomatosis was examined for ANCA by immunofluorescence test. In high activity stage of Wegener's granulomatosis all sera showed a positive ANCA-test. 39 patients with systemic vasculitis served as controls. Only two sera from patients with leucocytoclastic vasculitis and two with panarteriitis nodosa were ANCA positive. The follow-up (35 serum probes) of 8 patients showed no ANCA after immunosuppressive induced remission. In four histologically detected cases of recurrence ANCAs were positive again. ANCAs are very useful in primary diagnosis of Wegener's granulomatosis as well as in the follow-up. In addition, the analysis reveals, that immunosuppressive therapy with cyclophosphamide and prednisone facilitates complete remission and a long survival rate of patients with Wegener's granulomatosis.     

Prognostic factors in sudden deafness

Sudden sensorineural hearing loss is still a diagnostic and therapeutic dilemma and is very difficult to predict recovery in it. Different factors may influence a prognosis like severity of hearing loss, duration of symptoms before treatment, presence of vertigo, type of audiogram and age of patients. The aim of the study was an evaluation of the hearing improvement in sudden deafness in relation to some of these elements. A retrospective analysis of 119 patients treated for sudden sensorineural hearing loss during 10 years was done. Clinical otolaryngological, neurological and ophthalmological examination, audiologic and ENG tests were carried out. Hearing improvement was obtained in 51 patients (43%). Hearing recovery was recorded in 38 patients (66%) in whom duration of disease before treatment was no longer than 7 days, in 9 patients (25%) with a period 8-14 days and in 4 patients (16%)--with period 15-30 days (66% vs 25% vs 16%, p < 0.001, 25% vs 16% p = 0.39). Hearing improvement was more frequent in patients with initially mild (51.6%) than severe (38.7%) and profound hearing loss (25%) (51.5% vs 25%, p < 0.05). Hearing recovery was observed in 18 patients (33.3%) with vertigo and in 33 patients (50.8%) without vertigo (p = 0.056). In analysis of age (five groups: until 30 years, 31-40 years, 41-50 years, 51-60 years, over 60 years) in comparison with hearing recovery it was not found any significance (45.5% vs 45.8% vs 46.4% vs 37% vs 38.9%, p = 0.94). It was stated that in patients with sudden deafness duration of the disease before treatment and level of hearing loss may significantly influence an outcome, also vertigo may worsen a recovery, contrary age of the patients does not seem as important prognostic factor.     

Prognostic validity of brainstem electric response audiometry in infants of a neonatal intensive care unit.

This study compared the results of brainstem electric response audiometry (BERA) in infants of a neonatal intensive care unit to those obtained on the same children with pure-tone audiometry at 3 years of age. Six hundred children were initially tested in infancy, and complete follow-up information was obtained on 333. In 297 (89%) the BERA results accurately predicted the hearing status at the age of 3 years. Twenty-nine (9%) of the discrepancies were related to conductive hearing losses: 17 patients with a conductive hearing loss in the first few months of life had normal hearing at 3 years, and 12 patients normal in infancy had a conductive loss at 3 years. Two patients evaluated as a sensorineural hearing loss by BERA had normal hearing. These may have been due to a conductive loss. Six patients assessed as normal by BERA had significant hearing losses at the age of 3 years. Five of these had normal hearing at one frequency between 1,000 and 4,000 Hz. The sixth may have developed a sensorineural hearing loss after birth.     

Evaluation of hearing organ in patients with Turner syndrome

Hearing loss and middle ear diseases are often reported in some of Turner patients. In most of the reports hearing organ was evaluated with the use of subjective methods. The aim of the work was subjective and objective evaluation of hearing organ with an attempt to set the correlation between the results and the genotype of the patients with Turner syndrome (Ts). MATERIAL: 51 Ts patients aged 14.3 years on average. There were 29 girls with monosomy X and 22 having mosaicism. A detailed medical history was taken in each case with attention given to the hearing loss risk factors. METHOD: Physical ENT examination, hearing evaluation: pure tone audiometry, impedance audiometry, distortion products otoacoustic emissions (DPOAEs), brain auditory evoked potentials (BAEP). The control group consisted of 30 healthy patients. RESULTS: Recurrent acute otitis media was reported by 19.6% of Ts patients. Pure tone audiometry was improper in 36.3% ears; conductive hearing loss was present in 11.7% ears, mixed hearing loss in 5.9% ears and the moderate sensorineural hearing loss in 18.6% ears. Impedance audiometry was impaired in 14.7% of the cases. DPOAE disturbances were present in 41.4% of Ts patients, BAEP was improper in 52.0%. The percentage of the disturbances in DOPAEs and in BAEP in patients with mosaicism was 45.4 and 40.9% while in patients with monosomy 68.9 and 62%. CONCLUSIONS: Ts patients present predisposition to hearing disturbances. The disturbances seem to be connected with middle ear infections and with sensorineural hearing losses. Hearing loss in Ts women is not clinically apparent in most of the cases; this fact reflects the need of early evaluation and further monitoring of hearing organ in those patients. Sensorineural hearing loss seems to prevail in patients with 45,X genotype, so perhaps attention should be paid to this subgroup of Ts patients.     

大前庭导水管综合征9例临床分析

目的: 探讨大前庭导水管综合征（LVAS）的诊断、听力学表现及治疗。方法:回顾分析我科2000至2005年9例LVAS的病史、听力学及前庭功能检查、影像学检查及治疗结果。结果:9例均为双耳发病,并呈进行性听力下降,8例纯音测听虽以感音神经性聋为主,但低频段存在一定的气骨导差,保守治疗远期效果不理想。结论:对于儿童不明原因的听力下降,首先应排除先天性疾病,合并其他因素造成的听力下降会增加LVAS诊断的难度。对LVAS进行性听力下降者应尽早选配助听器,并考虑行人工耳蜗植入术。     

Unilateral hearing loss in childhood. An empirical analysis comparing bilateral hearing loss

BACKGROUND: Unilateral cochlear hearing loss is considered as a risk factor for auditory, verbal-communicative, behavioral and academic development. An early diagnosis is therefore necessary. METHOD/PATIENTS: 182 consecutive patients with an age up to 10 years were diagnosed with permanent hearing loss in the mild to profound range in a defined 5-years-period (1.10.1994-30.9.1999) in the Pedaudiologic Outpatient Clinic of the University G?ttingen. Fifty children (27.5%) had a unilateral hearing loss (30 sensorineural, 20 conductive caused by aural atresia with or without microtia), 132 a bilateral one. This paper presents the data of the consecutive series of the 30 sensorineural unilaterally hearing-impaired children (> 25 dB). RESULTS: A slight male predominance was present (53.3%). The left ear was affected in 43.3%, the right ear in 56.7%. In the majority of the cases the hearing loss was severe and profound. The hearing-impairments were diagnosed by the median age of 69.5 months and all aided by the median age of 70 months. The etiology remained unknown in 60 per cent of the cases. Hearing aid acceptance at the first follow-up (on average after 6 months) was found to be 79 per cent. CONCLUSIONS: The data suggest the relevance and necessity of a pedaudiometric prevention. They demonstrate the urgent necessity of a molecular genetic cause investigation. Recessive sensorineural hearing loss with onset in infancy may exist with no antecedent family history.     

Hearing impairment and osteogenesis imperfecta: Literature review

The goal is to clarify the epidemiology of hearing loss in patients with osteogenesis imperfecta (OI), so as to improve management. A literature review analyzed data from 15 patient series. Hearing loss prevalence in OI varied widely, from 2% to 94.1%. Typically, hearing loss was conductive in young patients and sensorineural in older patients. Prevalence increased with age, but after 50 years the increase was slight, and seldom became total. Hearing loss was usually bilateral, but not necessarily symmetrical. There were no correlations between type of mutation (COL1A1 or COL1A2), prevalence, type or severity of hearing loss, or age of symptom onset; there was intra-familial variability. There was also no correlation between mutated gene, type of mutation and auditory phenotype. Frequency, type and severity of hearing loss were unrelated to other clinical parameters. Hearing loss prevalence depended on type of OI: higher in type I and lower in type IV. Incidence of otitis media was higher in children with OI, related to the associated craniofacial dysmorphia. Hearing screening before 5 years of age with long-term follow-up are recommended.     

Epidemiological studies on hearing impairment with reference to genetic factors in Sichuan, China

Hearing impairment is the most common disorder of sensorineural function and is an economically and socially important cause of human morbidity. A large-scale epidemiological survey of hearing loss was conducted with 126,876 unselected subjects (63,741 male and 63,135 female) from Sichuan, China. The overall prevalence of hearing loss was 3.28% (4,164 of 126,876), and the prevalence increased with age, reaching 12.8% (1,465 of 11,421) at 60 years of age. In 73.03% of all cases (3,041 of 4,164), the hearing loss was sensorineural, and in 20.39% (849 of 4,164), it was conductive; the remaining cases (6%) were mixed hearing loss. Bilateral loss was found in 74.5% of cases (3,103 of 4,164). In 63.79% of cases (2,656 of 4,164), the degree of hearing loss was less than 55 dB hearing level (HL), and in 5.67% of cases (236 of 4,164), it was greater than 90 dB HL. The prevalence of hearing loss in childhood (<15 years of age) was 0.67% (227 of 34,157), of which 57.7% of cases were conductive and 38.8% were sensorineural. The prevalence of genetic hearing loss was 0.28% (349 of 126,876). Persons who lived in the flatlands appeared to have a higher prevalence than those who lived in the hills. Several ethnic groups, including Tibetans, the Yi, and the Lisu, had a higher prevalence of hearing loss. Presbycusis, otitis media, and genetic factors were the most commonly recognized causes of hearing impairment overall, but otitis media and genetic factors were the main causes of hearing loss in children. Causes for the observed differences in prevalence and etiologic factors between China and industrialized countries will be discussed. In China, infections and genetic factors appear to be of major importance as causes of hearing loss.     

The burden of hearing loss in Kaduna, Nigeria: a 4-year study at the National Ear Care Centre

Hearing loss among people in developing countries has been recognized as a major source of disability. Many of its causes are preventable, and others are curable. We reviewed the records of 5,485 patients who had presented during a 4-year period to the National Ear Care Centre in Kaduna, Nigeria. Of these, we identified 1,435 patients-812 males and 623 females, aged 9 months to 90 years (mean: 29.2 yr)-who had been diagnosed with hearing loss (26.2%). In addition to demographic data, we compiled information on each patient's type and degree of hearing loss, the affected side, and the predisposing factors. Sex and age cross-tabulations revealed that the greatest proportion of hearing loss according to sex occurred between the ages of 11 and 20 years for males and 21 and 30 years for females. The most common type of hearing loss was sensorineural, which was seen in 78.9% of patients; conductive hearing loss was seen in 17.7% and mixed in 3.4%. More than three-quarters of hearing losses were either moderate, moderately severe, or severe. Bilateral losses were far more common than unilateral losses; among the latter, the left side was affected slightly more often than the right. Predisposing factors were not documented in the vast majority of cases (87.6%), but when they were, the most common were chronic suppurative otitis media, meningitis, febrile convulsion, measles, and trauma. We present these findings to highlight the burden of hearing loss in our part of the world.     

鼻咽癌放疗后长期存活者的听力观察

目的了解鼻咽癌放疗后长期存活者的听力情况。方法对126例(252耳)鼻咽癌放疗后存活5年以上的患者进行纯音听阈、声导抗测试,耳、鼻咽部CT及耳内窥镜检查,对结果进行分析。结果 126例中,放疗后有听力损失87例172耳,占69.05%,其中感音神经性聋26例51耳,占29.89%(26/87),传导性聋5例9耳,占5.75%(5/87),混合性聋56例112耳,占64.37%(56/87);CT示中耳乳突炎121耳(70.35%,121/172);耳内窥镜检查所有鼓膜均浑浊内陷,无穿孔。结论鼻咽癌放疗后长期存活者听力损失的发生率高,以混合性听力损失发生率最高,感音神经性次之,传导性最低。     

Sensorineural hearing loss and Kawasaki disease: a prospective study

PURPOSE: Kawasaki disease (KD) is an acute, self-limited vasculitis of infants and children that is now the most common cause of acquired heart disease in the pediatric age group in the United States and Japan. Reports have documented the association of acute KD with sensorineural hearing loss. To assess the prevalence of hearing loss following acute KD in a geographically and ethnically diverse population, a prospective, multicenter study of hearing loss in patients with KD was conducted. MATERIALS AND METHODS: Patients with acute KD were enrolled in 7 clinical centers and underwent a primary audiologic evaluation within 30 days of the onset of fever. Patients were subsequently reevaluated after resolution of the acute phase of the disease. A questionnaire assessing risk factors for hearing loss was also administered. RESULTS: A total of 62 patients were evaluated during the 29-month study period. At the first audiologic evaluation, 19 patients (30.6%) had sensorineural hearing loss, 6 patients (9.7%) had conductive hearing loss, 17 patients (27.4%) had normal hearing, and 20 patients (32.3%) had inconclusive studies. Overall, 2 of 36 patients (5.5%) had sensorineural hearing loss documented on their second audiologic evaluation. No risk factors for hearing loss were identified by the questionnaire. CONCLUSIONS: Transient sensorineural hearing loss (20 to 35 dB) is a frequent complication of acute KD and may be related to salicylate toxicity in some patients. Persistent sensorineural hearing loss is uncommon. Parents and primary care providers should be made aware of the potential for persistent sensorineural hearing loss following resolution of KD, but routine audiologic screening of this patient population does not appear to be warranted.     

Hearing loss evaluation of Sjögren's syndrome using distortion product otoacoustic emissions

Sj?gren's syndrome (SS) is a cell-mediated immune disorder primarily affecting the exocrine glands and hearing loss may be the first otological manifestation of this autoimmune disease. In order to assess the degree of sensorineural hearing loss in SS, 22 female patients were examined by means of standard audiometric tests (pure-tone audiometry, acoustic reflexes and impedance testing) and using distortion product otoacoustic emissions (DPOAEs). The results indicated that only 36.3% of the patients had mild sensorineural hearing loss. Hearing level and distortion product threshold estimates were found to be significantly correlated. No relationship was found between the duration of the disease and the DPOAE and hearing threshold variables. The data suggest that SS may not directly cause sensorineural hearing loss.     

Prevalence of hearing loss in children with 22q11.2 deletion syndrome

ObjectiveThe purpose of this study was to determine the prevalence and characterize the types of hearing loss in pediatric patients with 22q11.2 deletion syndrome (22q11DS).MethodsFifty-eight patients were identified via retrospective chart review performed of patients with 22q11DS between 1996 and 2014. Patient demographics, pertinent family history, associated comorbidities, and degree and type of hearing loss were gathered for each patient. A literature review of the National Library of Medicine's database with a focus on hearing loss and 22q11DS was performed.Results22 patients (38%) were found to have hearing impairment: 68% with conductive hearing loss, 14% with sensorineural hearing loss, and 18% with mixed hearing loss. Patients with hearing loss regardless of type had a higher prevalence of developmental delay (55%), cleft palate (23%), articulation disorders (77%), and a greater need for tympanostomy tubes (73%) compared to patients with normal hearing. Temporal bone computed tomography scans of 5 patients revealed a variety of abnormalities in the middle and/or inner ears.ConclusionHearing impairment occurs in up to 38% of 22q11DS patients of both conductive and sensorineural types, with the conductive type being the most common. These patients have a greater need for tympanostomy tubes and a higher prevalence of developmental delay and speech articulation disorders. Early hearing screening and treatment is warranted in this population.     

Middle ear effusion as infrequent cause of sensorineural hearing loss

Middle ear efussion (MEE) is the most frequent condition related to conductive hearing loss in children, being rarely the cause of sensorineural hearing loss (SNHL). We present a 5-years-old child without know previous otologic disease who developed MEE with a very adversely effect on hearing. Hearing tests showed a bilateral moderate-severe neural impariment. The patient were programmed for grommet insertion. Hearing showed a total audiometric recovery three months after surgery. To sum up, MEE can be rarely associated with SNHL.     

Hörvermögen bei Kindern und Jugendlichen mit Down-Syndrom

Background

There are controversial opinions about the incidence of hearing impairment of children and adolescents with Down’s syndrome as an additional cause for obstruction in social and communicative live. Next to congenital diseases of the inner ear, functional limitations can often lead to conductive hearing disorders in patients with Down’s syndrome.

Patients and methods

The hearing ability, otoscopic and audiometric characteristics of 115 patients aged 3.2±2.9 years were analysed retrospectively over 42 months.

Results

A total of 86 children showed morphologic otoscopic peculiarities: transitory evoked otoacoustic emissions (TEOAE) were often non-reproducible, even in patients with normal hearing abilities(14.1% of cases), and were not evaluable in 32.8% of cases. Half of the patients showed hearing loss, 82% of whom suffered from a conductive hearing loss, 6.9% from a combined, and 8.6% from isolated sensorineural hearing loss. Hearing loss was first diagnosed when the children were 4.6±3.4 years old.

Conclusion

The frequent occurrence of hearing loss justifies and requires an early and continuous pedaudiological examination. As a common screening examination, TEOAE is limited due to its cost, however, it fulfils its purpose as a means of distinguishing healthy children from those with possible hearing disorders. In most cases, objective and subjective audiometric methods must be combined in order to precisely define the hearing ability.