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Elevated blood mercury and neuro-otological observations in children of the Ecuadorian gold mines.

The prevalence of mercury (Hg) intoxication was investigated in 114 Andean Saraguro and non-Saraguro (Mestizo) children living in remote gold-mining settlements in Nambija and Portovelo, Ecuador. Venous blood samples showed a mean total blood mercury (B-Hg) level of 18.2 microg/L (SD 15.5; range 2-89.) for 77 Saraguro and non-Saraguro children in the Nambija settlement, which was significantly higher than that of children in the Portovelo and reference groups. Comparison of groups showed mean B-Hg levels of 26.4 microg/L (range 4-89 microg/L) for 32 indigenous/Saraguro children; 12.3 microg/L (range 2-33 microg/L) for 45 non-Saraguro children; 4.9 microg/L (range 1-10 microg/L) for 37 children in Portovelo; and 2.4 microg/L (range 1-6 microg/L) for a reference group of 15 children. Fisher's post hoc analysis revealed significant differences among groups, except between the Portovelo and the reference groups. Neuro-otological symptoms and abnormalities were observed in Saraguro, non-Saraguro, and Portovelo children. Samples of soil collected at sites near the local school were found to contain Hg levels ranging from 0. 1 to 38 ppm, cadmium (Cd) levels from 0.07 to 0.82 ppm and arsenic (As) levels from < 1 to 3.9 ppm. in conclusion, the children of Nambija, particularly the Saraguro "Amer-Indians," exhibited elevated B-Hg levels from exposure to Hg used in the gold-mining process, and are at risk for neurological impairment. The children of Portovelo who reported neuro-otological symptoms but had low B-Hg levels (<10 microg/L) may be affected by exposure to sodium cyanide, which is used extensively in the local gold-mining operations.

Adolescent↗

Effect of rimantadine treatment on clinical manifestations and otologic complications in adults experimentally infected with influenza A (H1N1) virus.

Susceptible adults (n = 105) were enrolled into a randomized double-blind study of rimantadine treatment of experimental influenza A infection. Subjects were cloistered for 8 days and challenged with a rimantadine-sensitive strain of influenza A H1N1 virus at the end of the first day. Forty-eight hours after challenge and for 8 days, 54 subjects received placebo and 51 received rimantadine (100 mg orally, twice a day). Symptoms, signs, and pathophysiologies were monitored. Nine subjects were not infected. Seventeen subjects (38%) in the rimantadine and 26 (53%) in the placebo group became ill. A beneficial effect of rimantadine was documented for virus shedding, symptom load, and sinus pain. Rimantadine had no effect on nasal patency, mucociliary clearance, nasal signs, or on symptoms and signs of otologic complications. These results do not support a preventive effect of rimantadine on the development of otologic manifestations of influenza A infection in adults.

Adolescent↗

Applications of distortion-product emissions to an otological practice.

OBJECTIVES: The multiple clinical applications of distortion-product emissions (DPEs) to an otological practice are reviewed. Through an examination of studies involving thousands of infants, as well as adult ears, the relationships between measurements of DPEs and PTTs (PTTs) are examined. The cochlear physiology underlying generation of DPEs and the interpretation of these measurements are described in some detail. We study the contribution of phase measurements of DPEs to calculation of delay in cochlear traveling waves. The clinical applications of these measurements are described. The objective of this work is to demonstrate the clinical utility of DPEs through an examination of the correlation of the features of DPEs with PTTs in patients varying in age from newborn to the elderly. STUDY DESIGN METHODS: Experimental design and methodology involved careful prospective selection of large numbers of subjects in each age group, novel techniques for recording DPEs, and multivariate statistical analysis of the data. Three separate experiments encompass issues surrounding the age groups of adults, neonates, and elderly individuals. RESULTS: The results show that current techniques allow a 90% correct prediction of auditory PTTs for frequencies varying from 1,000 to 6,000 Hz, for patients of all ages. CONCLUSION: Techniques for measurement of DPEs currently represent an integral diagnostic component for an otological practice. The potential future application of measures of DPEs to diagnosis of tinnitus is described. With an extension of the technique for measuring DPEs to the phenomenon of reflectance, there may be promise of application to novel approaches to the treatment of tinnitus and the design of hearing aids.

Adolescent↗

Otological and audiological outcomes five years after tympanostomy in early childhood.

OBJECTIVE: Ventilation tubes in the treatment of otitis media in young children remain controversial. Despite abundant research, few prospective long-term follow-up studies have included even a minority of patients under 1 year old. We investigated long-term otological and audiological outcomes in children with recurrent acute otitis media and otitis media with effusion, who were treated early with ventilation tubes. STUDY DESIGN: Prospective follow-up. METHODS: Three hundred five children under 17 months of age received a primary tympanostomy in the Central Hospital of Central Finland (Jyväskylä, Finland), and those 281 (92.1%) who were monitored prospectively for 5 years made up the study group. At the final examination, pneumatic otoscope and otomicroscope were used and pure-tone audiometric thresholds of air and bone conduction were measured to define the hearing levels (mean of 0.5, 1.0, and 2.0 KHz thresholds). RESULTS: Of ears, 67.3% were healed, 7.1% had a retraction of tympanic membrane in pars flaccida and 9.6% in pars tensa, 7.5% had an ongoing otitis media with effusion, 3.9% had a ventilation tube in place, and 4.6% had a tympanic membrane perforation with mean hearing levels of 7.6, 9.0, 16.0, 18.5, 10.5, and 17.7 dB, respectively. CONCLUSIONS: Hearing in general was well preserved, and no ear presented with adhesive otitis media or cholesteatoma. Adverse otological and audiological outcomes of these young children did not exceed those presented by others for older counterparts. Tympanic membrane perforations, ongoing otitis media with effusion, and pars tensa retractions were causes of mild conductive hearing loss. Because one third of ears continued to have middle ear disease or sequelae, we emphasize the proper follow-up and restoration of middle ear ventilation with repeat ventilation tubes if not otherwise achieved.

Acute Disease↗

Otologic referral in industrial hearing conservation programs.

Baseline and periodic audiometry was performed on noise-exposed workers in 21 industrial plants. Workers showing moderate hearing loss, asymmetry, or significant threshold shifts were referred for otologic evaluation. The overall referral rate was 2.76%. Of 107 referrals, 53% had otologic diagnoses other than noise-induced hearing loss or required specific intervention, or both. The results support the use of criteria at least as stringent as those used in this study.

False Negative Reactions↗

Otologic manifestations of Chiari I malformation.

OBJECTIVE: To assess the prevalence of otologic symptoms in patients undergoing surgical decompression for symptomatic Chiari I malformation. STUDY DESIGN: Cross-sectional, prospective, nonrandomized. SETTING: Urban tertiary referral center. PATIENTS: Patients with Chiari I malformation before surgical intervention. INTERVENTIONS: None. MAIN OUTCOME MEASURE: Results of completed questionnaire. RESULTS: Sixteen consecutive patients with Chiari I malformation completed the self-administered questionnaire. Eighty-one percent of patients reported episodic aural fullness, 81% reported tinnitus, 69% reported vertigo, and 56% reported fluctuating hearing loss. Headaches were reported as frequently as aural fullness and tinnitus. CONCLUSIONS: Most patients with Chiari I malformation have symptoms that mimic primary otologic pathologic changes. The existence of common pathophysiologic mechanisms is proposed.

Adult↗

Sir Charles Alfred Ballance: contributions to otology and neurotology.

STUDY DESIGN: A review of primary and secondary historical and scientific literature concerning the life and writings of Sir Charles Alfred Ballance (1856-1936). RESULTS: Sir Charles Alfred Ballance was a pioneer in otology and neurotology, responsible for many "firsts" in the field, including the complete removal of a cerebellopontine angle tumor with significant patient survival and grafting the mastoid cavity with epithelium to speed healing. He was a strict abdicator of the complete mastoid operation with ligature of the jugular vein and drainage of the lateral sinus, and he advanced many other neurotologic procedures during his lifetime. He successfully sectioned the VIIIth cranial nerve, relieving a patient from intractable vertigo, developed a meticulous system for treating the complications of suppurative infections of the mastoid, diligently studied facial nerve reanastomosis, and was the first to perform a spinal accessory to facial nerve anastomosis for the relief of facial palsy. Ballance published over 75 original articles and was the primary author on two landmark books concerning temporal bone and neurologic surgery. He was the leader of many surgical societies and maintained a life of research and science until his death. CONCLUSION: Sir Charles Ballance was a pioneer in neurotologic and skull base surgery and greatly advanced the field during its inception. This work focuses on the otologic and neurotologic endeavors of Sir Charles Alfred Ballance, bringing his accomplishments into modern relief.

Ear Diseases↗

Otologic manifestations of malignant osteopetrosis.

OBJECTIVE: To determine the incidence of hearing loss and describe the neurotologic manifestations over time in a large series of patients with malignant osteopetrosis. STUDY DESIGN: Longitudinal study. SETTING: Tertiary care referral center. PATIENTS: Thirty-two patients, including 19 infants (< 1 yr of age at initial visit) and 13 children (aged 1-7.6 yr at initial visit), with autosomal recessive osteopetrosis were followed-up during a 10-year period from 1991 to 2001. The average length of follow-up was 2.5 years (range, 0-9.1 yr). INTERVENTIONS: Patients underwent annual otologic evaluations including clinical examination, audiologic evaluation (auditory brainstem response, pure-tone thresholds, speech discrimination scores, and tympanograms), and high-resolution computed tomography of the temporal bone. MAIN OUTCOME MEASURES: Incidence of hearing loss, otitis media, and facial paralysis. Serial changes in temporal bone anatomy by computed tomographic scan. RESULTS: Twenty-six percent of infants' ears demonstrated hearing loss during the first year of life, and 78% of children's ears demonstrated hearing loss during the study period. Of the children's ears with hearing loss, 100% had a conductive component and 26% had an additional sensorineural component (mixed hearing loss); VIIIth nerve conduction was normal in 100% of infants and 78% of children. Five patients (16%) had unilateral facial nerve palsy. Common temporal bone findings included thickening and sclerosis of the calvarium; poor pneumatization of the mastoid bone; and narrowing of the external auditory canal, eustachian tube, and internal auditory canal. CONCLUSION: Otologic manifestations are common in malignant osteopetrosis secondary to the formation of dense, brittle bone. Frequent findings include external auditory canal stenosis, otitis media, conductive and sensorineural hearing loss, and facial nerve paralysis.

Child↗

Stapes ankylosis in a family with a novel NOG mutation: otologic features of the facioaudiosymphalangism syndrome.

OBJECTIVE: To report the phenotype-genotype correlation in a Belgian family that was ascertained to have a novel missense mutation in the NOG gene mapping to chromosome 17q22. STUDY DESIGN: To describe the phenotype, a retrospective case study was performed based on the otologic, audiologic, ophthalmologic, and radiologic data of the mutation carriers of the NOG gene. SETTING: Tertiary referral center. PATIENTS: All members of a Belgian kindred who carried the novel missense mutation in the NOG gene (NOG, Trp205Cys [W205C]; 1426G>C). INTERVENTIONS: Diagnostic otologic and ophthalmologic examination, audiometric analysis, and radiologic imaging. MAIN OUTCOME MEASURES: Phenotype-genotype correlations. RESULTS: All five mutation carriers had a typical facies. Bilateral proximal symphalangism and hyperopia were present in 80%. Five of 10 ears also had progressive early-onset conductive hearing loss caused by stapes ankylosis. CONCLUSIONS: So far, 14 independent NOG mutations have been identified. The autosomal dominant disorder described in the present family was caused by a novel NOG missense mutation (NOG, Trp205Cys [W205C]; 1426G>C). The phenotype correlated well with the facioaudiosymphalangism syndrome. The mutation carriers demonstrated progressive multiple joint fusions, hyperopia, early-onset conductive deafness, and a typical facies.

Abnormalities, Multiple↗

Evaluation of a computer interview system for use with neuro-otology patients.

The effectiveness, feasibility and credibility of a computer assessment package was evaluated in a group of 60 neuro-otological outpatients. The computer system was compared with a standard questionnaire and used to elicit basic neuro-otological information prior to clinical interview. The majority of patients (81%) preferred computer assessment. There were no differences in attitude or difficulties between diagnostic groups. Older patients had more difficulties and took longer than younger patients. The computer system was also more efficient than the form from an information handling and administrative point of view.

Attitude to Computers↗

The paediatric otological caseload resulting from improved screening in the first year of life.

Improved test technique by health visitors has been shown to lead to higher accuracy in the screen of hearing aimed traditionally at prelingual sensorineural deafness. However, it gives greatly increased referrals of children having otitis media with effusion (OME) around the end of the first year of life. Two samples of children (n = 29 and 61) were tested in a children's hearing assessment clinic with properly documented testing techniques and trained personnel. The samples were each formed on a fixed population base, one before and one after screen improvements. This enabled characterization of two outcome groups within each sample (severe/persistent enough to refer to ENT vs discharged, despite slight hearing impairment). The average audiometric criterion for onward referral to ENT rose only very little, despite the increased assessment caseload resulting from more detections by the screen. This usefully permitted the conclusion that the number of true cases found due to the screen and assessed as lying beyond a specifiable degree of severity (average cut-off approximately 47 dB(A) or 35 dBHTL) had increased not through lower criteria for referral to ENT, but through the improvements to the screen. The increase was from approximately 0.4% to 1.3% of the base population screened. Thus a change materially enhancing the sensitivity and positive predictive value of a screen considerably enlarges the eventual otological caseload of children with middle-ear disease thought to justify concern at around the end of their first year. If done properly, screening is hence in practical terms about OME, not about prelingual sensorineural hearing impairment. This conclusion presses the urgency of evaluation and consensus on the otological management of the young child with OME.

Analysis of Variance↗

The burden of screening for acoustic neuroma: asymmetric otological symptoms in the ENT clinic.

The rising numbers of legal cases relating to delay in the diagnosis of acoustic neuroma, combined with the increasing availability of magnetic resonance, is increasing pressure on otologists to make an early definitive diagnosis of cerebellopontine angle tumours. Unilateral or asymmetrical otologic symptoms not explained by external or middle ear disease are elicited in 16.6% of 500 consecutive attenders to an otolaryngology clinic. An agreed policy of risk stratification of patients with unexplained asymmetric otological symptoms is required if expense is to be limited and litigation minimized.

Adolescent↗

The effect of consultant-led interactive pre-clinic case note review on follow-up rates of an otology outpatient clinic.

The effect of pre-clinic consultant-led interactive case note discussion on the follow-up rate of an otology outpatient clinic was assessed. The clinic was divided into two groups. In one group, all cases were delegated by the consultant at the beginning of clinic without discussion. In the other group, before the start of clinic, the team reviewed all case notes and formed a management plan for each patient. Overall, lower follow-up rates were observed in cases which were discussed prior to the start of clinic (53.2-45.7%; p = 0.02). This was found in all doctor grades, although statistically significant only for middle grade (consultant: 48.3-41.6%, p = 0.10; middle grade: 58.5-45.5%, p = 0.05 and senior house officer: 60.2-57.8%, p = 0.73). Junior doctor's follow-up rates were much higher than the consultant's rates (p < 0.01). In conclusion, pre-clinic consultant-led case note review reduced otology outpatient follow-up rate.

Ambulatory Care↗

Neuro-otological abnormalities in Friedreich's ataxia.

Ten patients with an accepted diagnosis of Friedreich's ataxia have been examined neuro-otologically, and oculomotor, vestibular and auditory function assessed. Brainstem auditory evoked potentials (BAEPs) were also recorded. A high incidence of various eye movement disorders was noted. Some of these were indicative of cerebellar dysfunction. Reduced vestibular function and impaired hearing were common to most of the patients. BAEPs were also abnormal in the majority; reasons underlying these abnormalities are discussed. Neuro-otologically, the patients did not constitute an homogeneous group. The findings cast doubt upon the accuracy and validity of the currently accepted criteria for the diagnosis and classification of the spinocerebellar degenerations.

Adolescent↗

Otologic facial palsy: etiology, onset, and symptom duration.

To estimate the occurrence of otogenic facial palsy, we performed a retrospective case record study of all patients hospitalized for otogenic facial palsy in the period 1989 to 1999 at Ullevål University Hospital, which is the only referral hospital for patients with otologic sequelae in Oslo. The facial palsy was a complication of acute otitis media in 10 patients (56%), of acute mastoiditis in 3 patients (17%), of secretory otitis media in 3 patients (17%), and of chronic otitis media in 2 patients (11%). In half of the patients, complete facial palsy was found at the time of diagnosis. Sixteen patients (89%) reported a gradual onset of the facial palsy. The mean duration of otologic symptoms before the onset of facial palsy was 3 days (range, 1 to 9 days), and the median time to remission was 9 weeks (range, 2 to 96 weeks). Total remission was achieved in all patients who received follow-up. Although most patients recover within a few weeks, some patients have long-lasting facial palsy. Multicenter studies are needed to increase the sample size and to identify predictors of facial palsy duration.

Acute Disease↗

A new autologous fibrinogen-based adhesive for otologic surgery.

Many middle ear reconstructive procedures would be facilitated by use of a relatively safe surgical adhesive. A fibrinogen-based adhesive (Tisseel) has been effective in otologic surgery in Europe, but because it is derived from pooled human blood, it carries a risk of transmitting hepatitis, acquired immune deficiency syndrome, and other illnesses. This report details a new procedure for creating an autologous fibrinogen-based adhesive, obviating these risks. The fibrinogen and factor XIII component of the adhesive was isolated by polyethylene glycol precipitation from human plasma within a few hours, and was used either immediately or frozen for use up to 3 weeks later. Fifteen chinchillas had either the single donor adhesive, the commercial European adhesive, or saline placed on the oval and round windows, and no evidence of cochlear, mucosal, or ossicular damage was seen by light microscopy 30 days later. Auditory brain stem response thresholds remained stable, except in three animals that developed otitis media. Based on this investigation, autologous fibrinogen-based adhesive appears promising as a relatively safe, biological bonding material for otologic surgery, and is worthy of further study.

Animals↗

Early diagnosis of otologic Wegener's granulomatosis using the serologic marker C-ANCA.

Wegener's granulomatosis is a systemic vasculitis that may involve any organ system. Otologic manifestations are common, and can be the presenting complaint. In the past, diagnosis often necessitated the development of characteristic pulmonary or renal disease. The identification of a new serologic marker, cytoplasmic pattern antineutrophil cytoplasmic autoantibody (C-ANCA), allows for the early diagnosis of Wegener's granulomatosis and gives the patient the best chance for remission with cytotoxic therapy. We report two patients with Wegener's granulomatosis who presented with refractory otitis media, one of whom subsequently developed facial nerve paralysis, in which an early diagnosis was facilitated by the use of the C-ANCA test. Otologic manifestations of Wegener's granulomatosis and the basis of the C-ANCA test are discussed.

Adult↗

Neuro-otologic abnormalities in myelopathy associated with human T-cell lymphotropic virus type 1.

Auditory testing (pure tone audiometry, auditory brain stem response), and vestibular tests (eye tracking test, optokinetic pattern test, and caloric test) were performed to define neuro-otologic abnormalities in myelopathy associated with human T-cell lymphotropic virus type 1. Of the eight patients tested, seven showed sensorineural hearing loss and one showed mixed hearing loss on pure tone audiometry. The auditory brain stem responses of five patients showed increases of the I-III and I-V interpeak latencies. Two patients showed fast superimposed saccadic movements on the smooth pursuit test, and one other patient showed canal paresis on the caloric test. These findings suggest both the presence of neuro-otologic abnormality and involvement of the brain stem in myelopathy associated with human T-cell lymphotropic virus type 1.

Adult↗