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PubMed1976

Cogan syndrome associated with mesenteric vascular insufficiency.

Nonsyphilitic interstitial keratitis and deafness was first described as a distinct entity by Cogan in 1945. For years thereafter, it was believed to involve only the ocular and aural systems. It is gradually becoming apparent that the syndrome can also include other organ systems by affecting their blood supply. A patient with diagnosed Cogan syndrome subsequently developed mesenteric vascular insufficiency that was surgically corrected. This report lends more support to the belief that nonsyphilitic interstitial keratitis with deafness is only one manifestation of a more generalized vascular disease.

Adult↗
PubMed2005

Etanercept therapy for immune-mediated cochleovestibular disorders: a multi-center, open-label, pilot study.

OBJECTIVE: Immune-mediated cochleovestibular disorders (IMCVDs) continue to present a diagnostic and therapeutic challenge. Antirheumatic agents, commonly employed for IMCVDs, are associated with variable efficacy and sometimes with serious side effects. The objective of the current study was to preliminarily evaluate the efficacy of etanercept therapy for IMCVD. METHODS: In this open-label prospective pilot study, 23 patients with bilateral IMCVDs or symptoms of bilateral Meniere's disease were treated with etanercept (25 mg twice weekly, by subcutaneous injection) for 24 weeks. All participants showed progressive hearing loss within 3 months prior to the study and responded to prednisone therapy. Hearing improvement was defined as an improvement of sensorineural hearing from baseline, in at least one ear, of 15 dB or more in the pure-tone air conduction thresholds, or an increase of more than 12% in word identification score. When present, vertigo and tinnitus were assessed by frequency and severity of attack and a functional level scale. Limited serial positron emission tomography (PET) of the inner ear region was performed in 5 patients to assess disease activity. RESULTS: There were 12 female (52%) and 11 male patients with a mean age of 48 years. Hearing improved in 7 (30%) patients, was unchanged in 13 (57%), and worsened in 3 (13%). Of 21 patients with tinnitus, this symptom improved in 7 (33%), was unchanged in 10 (48%), and worsened in 3 (13%). Of 16 patients with vertigo, 8 (50%) were improved, 7 (47%) unchanged, and 1 (3%) worse at the end of the study. Etanercept was generally well tolerated. PET was positive on one ear of 2 of 5 patients, remained positive with treatment on 1 of these, and was initially positive in 1 deaf ear, becoming negative at followup. CONCLUSION: These prospective pilot data suggest that etanercept therapy is safe among patients with IMCVDs. However, these data do not suggest substantial efficacy of etanercept among patients with IMCVDs in improving hearing loss. There appeared to be stabilization or improvement of hearing in 87% in this group of patients with pretreatment intractable progressive hearing loss. However, the study endpoint of improvement in 70% of patients was not attained. This short-term effect of possible stabilization requires further study. PET scanning was not useful as a tool to evaluate hearing loss in a limited subset of patients.

Adult↗
PubMed1980

Cerebellar infarction--a presentation of vertigo.

The differential diagnosis of acute vertigo includes cerebellar infarction. In the past a mortality rate of 50% - 80% was reported with cerebellar infarction. This is no doubt related to an inability to accurately diagnose small lesions which carry a better prognosis. The advent of computerized tomography (CT) now permits accurate diagnosis of small cerebellar infarctions. Seven patients admitted with acute onset of vertigo which mimicked a peripheral labyrinthine disorder are presented. In all patients a cerebellar etiology was demonstrated by CT. One patient had been subjected to therapeutic labyrinthectomy; three of seven patients had associated unilateral hearing loss suggesting partial brain stem involvemtation. Computerized tomography should be included in the routine work-up of acute vertigo with any associated neurologic findings to aid in proper diagnosis and effect appropriate treatment.

Acute Disease↗
PubMed1976

[Morbus Menière and acousticus-neurinoma (author's transl)].

It is questionable whether a hydrops endolymphaticus can develop on the basis of an acousticus-neurinoma. Histological evidence is also lacking. The fact that an acousticus-neurinoma can very rarely lead to the classical symptoms of Menière's disease has practical consequences. If a significant decrease in caloric function and an asymmetry in the inner ear ducts exist, a meato-cisternography is indicated. In the meato-cisternogramme typical filling defects are found; but also in 50% of the patients with Menière's disease, certain types of filling defects are seen. This observation suggests that perhaps other pathological processes in the inner ear duct provoke symptoms similar to those of Menière's disease. In our patient material we have indications that isolated intrameatal liquor circulation disturbances and intrameatal angioma are much more often associated with the clinical symptoms of Meniere's disease than an acousticus neurinoma.

Ear Neoplasms↗
PubMed1981

Vestibular ototoxicity in children: a retrospective study of 52 cases.

We have carried out a study on 52 patients treated during childhood with potentially ototoxic drugs. The evaluation of vestibular function was carried out long after treatment. In most of the cases there was a parallelism between hearing and vestibular deficit. Manifest impairment of the vestibular function was demonstrated in 4 subjects, without remarkable hearing loss, who had previously been treated with streptomycin during childhood. Furthermore, in the case of 19 subjects, a considerable deambulation delay was noticed (in one case it revealed itself after the fourth year of life) which was attributable to a real injury to the vestibular structures. Finally, almost all examined subjects showed a dysfunction of equilibrium and a noticeable tendency to kinetopathy.

Adolescent↗
PubMed1985

Vestibular disorders following head injury in children.

One hundred and ninety-nine child patients with blunt head injury were examined. Spontaneous and/or positional nystagmus (greater than or equal to 7 degrees/s) was observed immediately after trauma in 46% of cases, 6-12 months (average 10.2 months) later in 20%, and 2-8 years (average 4.7 years) later in 18%. Central ENG disturbances were found immediately after trauma in 43% of cases, 6-12 months later in 24%, and 2-8 years later in 12%. Only 1.5% of the child patients suffered from vertigo more than 6 months after trauma. The results of the study led to the conclusion that head injuries cause about as many similar objective vestibular lesions in children as in adults but fewer subjective symptoms.

Adolescent↗
PubMed1999

Age-dependent hypertension in Mpv17-deficient mice, a transgenic model of glomerulosclerosis and inner ear disease.

The mutant mouse strain Mpv17-/-, carries a retroviral germline integration that inactivates the Mpv17 gene. Mpv17-deficient mice develop progressive glomerulosclerosis and sensineural deafness at early age. Characteristic basement membrane alterations are found in both sites of pathology. Mpv17 is a peroxisomal protein involved in the metabolism of reactive oxygen species, yet its molecular function is unknown. Dysregulation of antioxidant enzymes and basal membrane components has been established in this model and successful therapeutic intervention with antioxidants prove the causal role of reactive oxygen species in the development of the disease phenotype. We here investigated if the Mpv17-/- mice might be hypertensive. Indeed, our study revealed that Mpv17-/- mice developed significant systemic hypertension and tachycardia between 4 weeks and 5 months of age, accompanied by polyuria and elevated natriuresis. Judging from serum and urine parameters, the hypertensive condition develops concomitantly with the renal disease. Biochemical and pharmacological studies that used the endothelin receptor antagonist bosentan and the angiotensin converting enzyme inhibitor cilazapril indicated no involvement of the endothelin and renin-angiotensin systems in this hypertension, suggesting a potential novel mechanism of blood pressure regulation in this new murine hypertension model. Thus, Mpv17-/- mice unravel an intriguing new association between a defect in reactive oxygen metabolism and the age-dependent development of hypertension.

Aging↗
PubMed1979

Vestibulo-toxicity of tobramycin.

The case histories are described of two patients with normal renal function who developed permanent complete bilateral loss of peripheral vestibular function during tobramycin therapy. Despite the low incidence of the cochleotoxic and and vestibulotoxic side-effects of tobramycin, it seems advisable to monitor patients on tobramycin therapy for signs of vestibular dysfunction and hearing loss.

Aged↗
PubMed1985

Usher's syndrome type III: ENG findings in four affected and six unaffected siblings.

ENG recordings showed abnormal findings in subjects affected by Usher's syndrome and in unaffected siblings. Unaffected subjects with hearing impairment may be heterozygote carriers with inner ears more sensitive to various injurious stimuli, e.g. noise, than those of healthy subjects. Vestibular tests may facilitate the detection of heterozygote carriers but the number of patients studied by us is too small to allow definitive conclusions to be reached.

Adult↗
PubMed1989

Otological disease in Turner's syndrome.

1988 was the 50th anniversary of Henry Turner's original publication of the constellation of physical features which were subsequently recognized to be associated with a deprivation of X chromosomal material. The abnormalities of phenotype and karyotype are now eponymously associated with his name. Very many phenotype stigmata have now been recognized to be associated with Turner's syndrome. However, a survey of females with the syndrome revealed that they consider that a major disability associated with the abnormal karyotype results from otological disease and that this is poorly recognized as a significant feature of the syndrome. The incidence of otological disease in patients with this syndrome has been difficult to evaluate because of ascertainment bias and the apparent diversity of otological disorders previously reported. This report reviews the literature and details a study of otological disease and hearing impairment in a sample of Turner's syndrome females enrolled from a defined area.

Adolescent↗
PubMed1998

Unusual lesions of the internal auditory canal.

Acoustic neuromata (AN) account for nearly 90 per cent of internal auditory canal (IAC) and cerebello-pontine angle (CPA) tumours. The second most common tumour is meningioma. Rare lesions include primary cholesteatoma, facial neuroma, lipoma, angioma and various cysts. Two cases of IAC tumour are presented, one of hamartoma in which smooth muscle was prominent and the other of lymphangioma. Of interest are the specific clinical and radiological features associated with these lesions.

Adolescent↗
PubMedDate not supplied

Dural herniations, encephaloceles: an index of neglected chronic otitis media and further complications.

A retrospective review of 110 consecutive cases of chronic otitis media surgery was carried out in a large metropolitan public teaching hospital in the United States that serves the inner-city population. The purpose was to identify dural herniations and encephaloceles (DHE) as well as complications associated with these entities. Twenty-nine such patients (26.4%) were identified, 7 of which (6.4%) were encephaloceles. In patients with DHE, cholesteatoma was found in 83%, a labyrinthine fistula in 48%, preoperative facial paralysis in 14% (4 patients), facial nerve involvement by cholesteatoma in 24%, and suppurative intracranial complications in 21%. DHEs were repaired through the mastoid, via a mini-craniotomy or a formal middle cranial fossa approach. The incidence of DHEs in this patient population is very high, which reflects neglected chronic ear disease. The associated complications require extensive surgery for eradication, and the potential for morbidity and mortality is significant.

Cholesteatoma↗
PubMed2000

Complications associated with labyrinthine fistula in surgery for chronic otitis media.

OBJECTIVE: Labyrinthine fistula (LF) is encountered during surgery for chronic otitis media (COM) with an average frequency of 7.5%. This study was undertaken to investigate the prevalence of coexisting complications with LF in surgery for COM. STUDY DESIGN AND SETTING: A retrospective review of 111 consecutive cases of COM surgery performed by the same surgeon in a large metropolitan teaching public hospital in the United States serving the inner city population. RESULTS: There were 23 (21%) LFs in 111 patients. The majority of cases were revision surgery with an average of 2.6 operations per patient. Other complications were encountered in 18 (78%) of the 23 patients with an LF: 5 (22%) encephaloceles, 9 (39%) dehiscences of the tegmen, 12 (52%) patients with facial nerve involvement by cholesteatoma, and 2 (8.7%) patients with facial nerve paralysis. These patients required extensive surgery for correction of their concurrent complications. CONCLUSION: Compared with those cases in which a fistula was not found, the ratio of the above complications were as follows: encephalocele, 1:9; facial nerve involvement by cholesteatoma, 1:11.3; facial nerve paralysis, 1:3.8. SIGNIFICANCE: Patients with neglected COM have an incidence of an LF twice that reported in the literature. The prevalence of associated complications with LF requiring extensive surgery is significantly higher than that observed in noncomplicated COM cases.

Chronic Disease↗
PubMed2006

Immune-mediated inner ear disease.

The incidence of autoimmune inner ear disease (AIED) is difficult to determine: probably it is a rare disease, accounting for <1% of all cases of hearing impairment or dizziness. Nevertheless, the diagnosis of AIED might be overlooked because of the lack of a specific diagnostic test. The hallmark of this clinically diagnosed condition is the presence of a rapidly progressive, often fluctuating, bilateral sensorineural hearing loss (SNHL) over a period of weeks to months. The progression of hearing loss is too rapid to be diagnostic for presbycusis and too slow to conclude a diagnosis of sudden SNHL. Vestibular symptoms, such as generalized imbalance, ataxia, positional vertigo and episodic vertigo may be present in almost 50% of patients. Occasionally only one ear is affected initially, but bilateral hearing loss occurs in most patients, with symmetric or asymmetric audiometric thresholds. Almost 25-50% of patients also have tinnitus and aural fullness, which can fluctuate. Systemic autoimmune diseases coexist in 15-30% of patients.

Ataxia↗