Otologic manifestations of the immotile-cilia syndrome

The immotile-cilia syndrome is caused by a hereditary, inborn, ultrastructural defect of the cilia, rendering them immotile or poorly motile and thereby abolishing mucociliary clearance. Five cases are presented and the otologic manifestations are described in some detail. The syndrome should be suspected in children who have a persistent secretory otitis media with recurring bouts of acute otitis media, and a perpetual cough with repeated episodes of bronchitis. A lobar atelectasis is a frequent finding. Half the cases also have situs inversus. The immotile-cilia syndrome is of special interest to the otologist, as it seems to throw some light on the pathogenesis of secretory otitis media.     

Otologic manifestations of neurofibromatosis

A chart review of 440 patients with neurofibromatosis seen at the Children's Hospital of Philadelphia Neurofibromatosis Clinic between 1980 and 1991 was performed to look for otologic manifestations. Neurofibromatosis 1 was present in 434 patients, and neurofibromatosis 2 was present in 6. A total of 31 otologic abnormalities related to neurofibromatosis were found in 28 patients. Neurofibromas of the external ear, including the pinna and the external auditory canal, were the most common finding. Middle ear neurofibromas were found in two patients. Acoustic neuromas were seen exclusively in patients believed to have neurofibromatosis 2. One cerebellopontine angle neurofibrosarcoma was found in a patient with neurofibromatosis 1.     

Otologic manifestations of the pain dysfunction syndrome of the stomatognathic system]

Phylogenesis, ontogenesis and anatomy explain the close relationship between temporo-mandibular joint and the middle ear and can therefore help understanding otologic symptoms such as: otalgia which often correspond to articular and muscular pain irradiation (coming from sterno-cleido-mastoid, lateral and medial pterygoid, deep layer of the masseter and temporal muscles); acouphens and ear block sensation that could be caused by a spasm extension of the manducatory (i.e. medial pterygoid) to levator and tympani tensors. These three muscles, which originate from the first branchial arch, have a proprioceptive sensitivity and share the same innervation. What is more tensor and levator veli exchange certain muscular fibers. Tensor tympani spasm can be held responsible both for a decrease or abolition of the Klockhoff's reflex, together with a decrease of the stapedian reflex, the latter due to tympani rigidity induced by a spasm of the tensor tympani.     

Otologic manifestations of Wegener's granulomatosis

OBJECTIVE/HYPOTHESIS: To evaluate the clinical features, treatment, and outcomes of otologic manifestations in Wegener's granulomatosis (WG) treated at Hokkaido University Graduate School of Medicine, Sapporo, Japan. STUDY DESIGN: We retrospectively reviewed 15 cases of WG with ear involvement. METHODS: Twenty-five patients with WG were treated at Hokkaido University Graduate School of Medicine between 1992 and 2001. Fifteen of these patients had otologic symptoms. We evaluated the clinical course, method of therapy, and outcomes in all cases. Diagnosis of WG was made when the patients had clinical findings and a positive titer of cytoplasmic pattern antineutrophil cytoplasmic antibodies (c-ANCA), or when there were clear histologic findings. We also present three case reports. RESULTS: In 15 cases, the most frequent finding was chronic otitis media. Sensorineural hearing loss was present in 2 patients. In 7 patients whose otologic manifestations were the primary involvement of WG, all were confirmed positive for c-ANCA and were treated with glucocorticoids and immunosuppressive drugs. Three patients who could be treated within 1 month of symptom onset showed marked improvement. CONCLUSIONS: In localized cases, biopsy specimens are often small, and it is frequently difficult to make a histologic diagnosis. The prognosis for hearing was poor when appropriate treatment was not given in the early stages of the disease. Therefore, WG should be included in the differential diagnosis in cases of atypical inflammatory states of the ear. Early diagnosis and appropriate treatment are important to prevent irreversible changes in the middle ear and inner ear.     

Otologic manifestations of pseudotumor cerebri

Otologic manifestations of pseudotumor cerebri heightened awareness of pseudotumor cerebri and its varied subtle presentations will allow for a more expedient diagnosis by the otolaryngologist. Pseudotumor cerebri is defined as increased intracranial pressure and papilledema without a mass lesion or obstruction of the ventricular system. Presenting symptoms most commonly are headache and visual dysfunction. However, patients may present with associated symptoms of tinnitus, dizziness and hearing loss. These patients may be seen first by the otolaryngologist. Fourteen patients with a diagnosis of pseudotumor cerebri are reviewed. Nine of the 14 patients had neurotologic symptoms during the course of their evaluation. Pseudotumor cerebri is a diagnostic challenge. Keys to diagnosis are a high index of suspicion, careful history and funduscopic examination in patients with unexplained neuro-otologic symptoms.     

Wegener肉芽肿病的耳部表现

目的探讨Wegener肉芽肿病耳部表现的临床特征，组织病理学和实验室检测特点，做到早诊断和早治疗。方法回顾性分析14例Wegener肉芽肿病耳部表现的临床资料。结果14例中包括慢性化脓性中耳炎10例，其中合并周围性面神经麻痹2例；分泌性中耳炎3例；感音神经性聋1例。14例中3例慢性化脓性中耳炎，1例分泌性中耳炎和1例周围性面神经麻痹是Wegener肉芽肿病的首诊表现，开始表现为耳部单独受累，余9例开始即有多器官受累表现，全部病例均呈现多系统多器官受累。14例组织病理学诊断均符合Wegener肉芽肿病的特点。13例抗中性粒细胞胞质抗体阳性，14例有红细胞沉降率升高，13例C反应蛋白升高。所有病例经糖皮质激素和环磷酰胺治疗后病情均得到控制。11例随访时间3年以上，病情稳定无复发。结论Wegener肉芽肿病的耳部表现常缺乏特异性，早期诊断有赖于临床表现、组织病理学检查和抗中性粒细胞胞质抗体的及时检测。规范使用糖皮质激素和环磷酰胺可以有效地控制病情。     

Otologic manifestations of ectodermal dysplasia

OBJECTIVES: To determine the range and prevalence of otologic disorders in patients with ectodermal dysplasia (ED) and provide a general review of its multiple otolaryngological manifestations. DESIGN: Case series. SETTING: Ectodermal dysplasia family conference. PATIENTS: Sixty-nine individuals with ED were evaluated. The average age was 11 years (range, 1-56 years). Most were male patients (44 [64%]), and most had Christ-Siemens-Touraine syndrome/hypohidrotic ED (42 [61%]), with the remaining having Hay-Wells, Clouston, and ectrodactyly ED clefting syndromes and unclassified types of ED. INTERVENTIONS: Questionnaire including a quality-of-life assessment modeled after the Otitis Media 6 instrument, physical examination, screening audiogram, and tympanometry. RESULTS: Of the 69 patients, 15 (22%) had a known history of hearing loss, and over half reported some level of difficulty processing verbal information (30 patients [43%] reported speech problems and 4 patients [6%] required hearing aids). A history of otitis media was common, with 15 patients (21%) presenting with tympanostomy tubes in place. The mean +/- SD quality-of-life rating was 2.0 +/- 1.1 (range 1-7), with lower scores suggesting less of a problem. On physical examination, 18 patients (26%) had pinna anomalies. One case of advanced cholesteatoma and 2 cases of external auditory canal stenosis were identified. Of the 24 patients who received 4-tone screening audiogram, 2 (8%) had a highest pure-tone average threshold of 50 to 65 dB, whereas 5 patients (21%) had a 30- to 45-dB threshold, with the remaining having a 0- to 25-dB threshold. CONCLUSION: In our study, which is, to our knowledge, the largest reported collection of ED patients evaluated for otologic disease, most patients were found to have 1 or more otologic abnormalities, ranging from auricular anomaly to complications of otitis media to profound hearing loss.     

Otologic manifestations of acoustic neuroma

Conclusion: Many patients with acoustic neuroma (AN) experience hearing loss and tinnitus. Time from first symptoms to diagnosis can be considerable. AN should be suspected, and MRI scans performed, in patients with hearing loss accompanied by asymmetry, tinnitus, low speech discrimination score (SDS), and abnormal auditory brainstem response (ABR). Objectives: To determine the otorhinolaryngological factors associated with AN by analyzing the clinical manifestations and diagnostic test results of patients with AN before MRI scanning. Methods: This study enrolled 114 patients definitively diagnosed with AN after visiting the Ear-Nose-and-Throat and Neurosurgery Departments of Kyung Hee University Medical Center from 2001 to 2013. Factors retrospectively analyzed included patient age, gender, major symptoms, accompanying symptoms, symptom duration, pure-tone audiometry, SDS, asymmetry, tinnitogram, ABR, and MRI scan results. Results: The main symptom of AN was hearing loss, and the most frequent accompanying symptom was tinnitus. More severe deafness correlated significantly with lower SDS (p < 0.05). Asymmetric hearing was observed in 75 of 116 patients (64.6%), and mean SDS was 73.1 ± 34.1%. Of patients with latencies of waves I, III, and V on ABR tests, 56.1%, 92.4%, and 92.4%, had interaural latency differences ≥0.2 ms. However, audiometry results did not correlate with lesion site or tumor size (p > 0.05).     

Otologic manifestations of Hunter syndrome and their relationship with speech development

The excessive storage of mucopolysaccharide in Hunter syndrome leads to various otologic manifestations. We interviewed 19 patients with Hunter syndrome to assess their otologic problems, and conducted audiologic tests and temporal bone CT. Patients with the intermediate or severe form exhibited severe speech delay by more than 2 years (12/14 patients). However, in patients with the mild form (5/5), speech development was not much disturbed (2/5), although otoscopic findings were similar. The hearing threshold determined by the auditory brainstem response differed significantly between the mild and intermediate/severe forms (p < 0.05). Therefore, patients with the mild form may benefit from active otologic intervention such as VT insertion, amplification, and speech therapy.     

Otologic manifestations of experimental rhinovirus infection

Episodes of acute otitis media are commonly associated with viral upper respiratory tract infections. Rhinoviruses account for approximately 40% of these infections, and were previously shown to alter eustachian tube function and middle ear pressures. However, progression to otitis media has not been prospectively documented. In the present study, changes in tympanometric pressures and otoscopic findings resulting from experimental intranasal rhinovirus type-39 inoculation were documented in 60 adult volunteers. Fifty-seven (95%) subjects became infected and 34 (60%) of these had a clinical cold. Prior to viral inoculation, 3 (5%) subjects had middle ear pressures of less than ?100 mm H₂O and two of these subjects developed middle ear effusions following infection. In all, 22 (39%) subjects developed middle ear pressures of less than ?100 mm H₂O. No subject with normal middle ear pressures prior to infection developed evidence of effusion. This study extends the otologic manifestations of rhinovirus infection to include otitis media. Furthermore, these results support the hypothesized relationship between upper respiratory tract infections, eustachian tube dysfunction, and otitis media.     

Otologic manifestations of Langerhans' cell histiocytosis

Eighteen of 62 children diagnosed with Langerhans' cell histiocytosis at the Children's Hospital of Pittsburgh (Pa) between 1970 and 1986 demonstrated ear and temporal bone involvement. In six children, such otologic disease was their sole presenting manifestation. Common signs and symptoms included aural discharge, postauricular swelling, and conductive hearing loss. The otologic findings in these children, if not investigated properly, could easily be attributed to acute or chronic infectious ear disease. Computed tomography with contrast enhancement proved to be particularly valuable as a diagnostic study because of its clear delineation of both osseous and soft-tissue temporal bone involvement. Computed tomographic findings could also be used to enhance local treatment by guiding surgical biopsy and curettage procedures or defining low-dose radiation therapy portals. Eleven of these 18 children with otologic Langerhans' cell histiocytosis additionally required chemotherapy due to the systemic nature of their disease.     

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.     

Otologic manifestations of Langerhans cell histiocytosis

A case of temporal bone eosinophilic granuloma is reported. The patient had no symptoms other than aural discharge, conductive hearing loss and postauricular swelling. Otic features of Langerhans cell histiocytosis, diagnosis preferences and therapeutic choices are reviewed.     

Otologic manifestations of AIDS: the otosyphilis connection

Five cases of otosyphilis presenting in patients with HIV infection are discussed. The group is representative of the known stages of the disease, from asymptomatic carrier to the fully expressed immunodeficiency syndrome, and it is of relevance because otosyphilis appears to have developed at an accelerated rate from the primary infection. Four patients had been treated with penicillin 2 to 5 years previously and had a positive fluorescent treponemal antibody absorption (FTA-ABS) test. The fifth had concurrent neurosyphilis and was VDRL-test (Venereal Disease Research Laboratory) negative 2 years prior to the onset of symptoms. In all five patients, syphilis was in the latent stage. It is proposed that it is during this phase of the disease that HIV may alter its course and hasten the development of otosyphilis. It is also suggested that otosyphilis can present at any stage of HIV infection and should be considered in seropositive patients presenting with otologic complaints.     

原发性小血管炎的耳科学表现

1概述 系统性血管炎是指以血管壁炎症和／或纤维素样坏死为主要病理特征的一组系统性自身免疫性疾病,当病变限于血管直径〈40μm微小动脉、静脉和毛细血管时则为小血管炎。依据病因可分为原发性和继发性小血管炎,继发性小血管炎指有明确病因者,如感染性疾病、