Sudden hearing loss and uveitis as a form of presentation of neurosarcoidosis

BACKGROUND: The pathologies and size of adrenal lesions removed through the open posterior and laparoscopic routes share remarkable similarities. This study aims at comparing the relative merits of these two approaches. METHODS: Patients with adrenal tumors operated on by posterior approach (n = 56) and transabdominal laparoscopic approach (n = 12) between January 1981 and May 1997 were retrospectively reviewed. RESULTS: The two groups were comparable in terms of age, and the position, size, and weight of the tumor. The operative time of posterior adrenalectomy was significantly shorter than that of laparoscopic adrenalectomy (median 120 minutes versus 160 minutes), whereas laparoscopic adrenalectomy was associated with less parenteral analgesic requirement (median 0 mg versus 225 mg), a shorter hospital stay (median 3 days versus 5 days), and a shorter duration off work (median 11 days versus 26 days). The estimated blood loss was also significantly reduced in the laparoscopic group (median 50 mL versus 150 mL). CONCLUSIONS: Laparoscopic adrenalectomy is replacing posterior adrenalectomy to become the procedure of choice for the majority of patients undergoing adrenalectomy.     

Sudden sensorineural hearing loss associated with anticardiolipin antibody

We experienced three female patients with sudden sensorineural hearing loss. All the patients showed elevation of anticardiolipin antibody in the serum. Immunoglobulin (Ig) M anticardiolipin antibody was present in case 1 (a 34-year old woman). IgG anticardiolipin antibodies were present in cases 2 (a 50-year-old woman) and 3 (a 9 year old girl). The patient in case 1 showed hearing disturbance of low tone in the left ear and normal vestibular function in both ears. The case 2 patient had an average hearing level of 81.7dB at 0.5. 1, and 2kHz and severe canal paresis in the right ear. In case 3 the patient showed an average hearing level of 53.3dB in the right ear and normal vestibular function in both ears. It was suggested that thrombosis which might have been induced by anticardiolipin antibody could have caused the sudden sensorineural hearing loss in these patients. Steroid therapy cured the hearing disturbance completely in case 1. Steroid and prostaglandin E1 therapy did not improve the hearing disturbance in case 2. Steroid, prostaglandin E1, and ticlopidine hydrochloride therapy improved the hearing disturbance in case 3. It appears that not only steroids but also prostaglandin E1 and ticlopidine hydrochloride therapy may be effective in patients with sensorineural hearing loss associated with anticardiolipin antibody.     

Sudden hearing loss in sickle cell disease: a case report

BACKGROUND: Endoscopic papillary balloon dilatation (EPBD) has been reported as a safe and effective alternative to endoscopic sphincterotomy in the management of common bile duct (CBD) stones; its effect on papillary function has yet to be elucidated. AIM: To investigate sphincter of Oddi (SO) motility before and after EPBD to determine its effect on SO function. PATIENTS AND METHODS: The papillary function of 10 patients with CBD stones was studied using endoscopic manometry before and one week after EPBD. The manometric studies were repeated one month after EPBD in seven patients. RESULTS: One week after EPBD, CBD pressure, SO peak pressure, SO basal pressure, and SO frequency decreased significantly. One month after EPBD, however, all parameters increased although the increases in SO basal pressure and CBD pressure were not significant. There was no significant difference in values of any parameter before and one month after EPBD. No serious complications occurred. CONCLUSION: These data suggest at least partial recovery of papillary function one month after the procedure. EPBD seems to preserve papillary function in treatment of CBD stones; a longer term follow up study with SO manometry should be performed to clarify the effect of EPBD on SO function.     

Sudden hearing loss: frequency of abnormal findings on contrast-enhanced MR studies

BACKGROUND AND PURPOSE: Our purpose was to determine the frequency of abnormal findings on contrast-enhanced high-resolution MR imaging studies in patients with sudden hearing loss. METHODS: Seventy-eight consecutive patients with sudden hearing loss underwent contrast-enhanced MR imaging of the temporal bone, cerebellopontine angle, and brain. Additional tests included audiologic examination, electrocochleography, fistula tests, and serologic tests for viral agents and autoimmune disorders. RESULTS: Probable causes of the sudden hearing loss in these patients included viral or immune-mediated disease, Meniere disease, vascular disorder, syphilis, neoplasm, multiple sclerosis, and perilymphatic fistula. Twenty-four (31%) of the 78 patients were found to have abnormal imaging results early in the course of their work up and treatment. CONCLUSION: The prevalence of abnormal findings on contrast-enhanced MR studies is higher than previously reported in patients with sudden hearing loss.     

Psychotic depression. An atypical initial presentation of multiple sclerosis

The Roussy-Lévy syndrome (MIM #180800) was described in 1926 as a disorder presenting with pes cavus and tendon areflexia, distal limb weakness, tremor in the upper limbs, gait ataxia and distal sensory loss. We report a family with affected members in four generations, showing these clinical signs of Roussy-Lévy syndrome and a partial duplication at chromosome 17p11.2. This genetic defect is commonly found in patients with the hypertrophic form of the Charcot-Marie-Tooth syndrome. Our finding provides evidence against the Roussy-Lévy syndrome as a distinct entity but suggests a close relation with the Charcot-Marie-Tooth syndrome. What causes the additional features of gait ataxia and essential tremor needs further clarification.     

Sudden hearing loss complicating general surgery: report of a case and review of the literature]

The first outbreak of Salmonella enteritidis phage type (PT) 5a infection to be reported occurred after a party in a residential home for elderly people in May 1995. The party was attended attended by 96 residents, staff and guests. S. enteritidis PT5a was isolated from 14 of the 25 clinical cases identified after the party and S. enteritidis PT4 from another clinical case. Two elderly residents with S. enteritidis PT5a infection died. Infection with S. enteritidis PT5a was associated with consumption of prawn in mayonnaise vol-au-vents, sausage rolls, corned beef sandwiches, and sausages. The investigation of this outbreak illustrated the difficulty that elderly people may have in the completion of questionnaires. It also highlighted areas for intervention; such as reminders about basic hygiene precautions to prevent secondary spread and the importance of coordinated reinforcement in the workplace of formal food hygiene training for cooks. The Food Safety Regulations 1995 came into force soon after this outbreak: their implementation would probably have prevented it.     

Cigarette smoking and hearing loss: the epidemiology of hearing loss study

CONTEXT: Clinical studies have suggested that cigarette smoking may be associated with hearing loss, a common condition affecting older adults. OBJECTIVE: To evaluate the association between smoking and hearing loss. DESIGN: Population-based, cross-sectional study. SETTING: Community of Beaver Dam, Wis. PARTICIPANTS: Adults aged 48 to 92 years. Of 4541 eligible subjects, 3753 (83%) participated in the hearing study. MAIN OUTCOME MEASURES: The examination included otoscopy, screening tympanometry, and pure-tone air-conduction and bone-conduction audiometry. Smoking history was ascertained by self-report. Hearing loss was defined as a pure-tone average (0.5, 1, 2, and 4 kHz) greater than 25-dB hearing level in the worse ear. RESULTS: After adjusting for other factors, current smokers were 1.69 times as likely to have a hearing loss as nonsmokers (95% confidence interval, 1.31-2.17). This relationship remained for those without a history of occupational noise exposure and in analyses excluding those with non-age-related hearing loss. There was weak evidence of a dose-response effect. Nonsmoking participants who lived with a smoker were more likely to have a hearing loss than those who were not exposed to a household member who smoked (odds ratio, 1.94; 95% confidence interval, 1.01-3.74). CONCLUSIONS: These data suggest that environmental exposures may play a role in age-related hearing loss. If longitudinal studies confirm these findings, modification of smoking habits may prevent or delay age-related declines in hearing sensitivity.     

Sudden loss of vision

BACKGROUND: Sudden loss of vision is an ophthalmological emergency. OBJECTIVE: This article explains how history and examination, particularly ophthalmoscopy, will greatly assist the general practitioner to diagnose the cause. DISCUSSION: Vision loss may be transient or prolonged. It is important to consider whether the eye is painful or comfortable, inflamed or not. An accurate diagnosis will direct the emergency management, which frequently determines the prognosis for vision.     

Familial cavernous angiomas masquerading as multiple sclerosis

We report here two cases of cavernous angioma, in the proband and her father, with quite different clinical presentations. The proband presented with a brainstem syndrome, mimicking multiple sclerosis, while the father had a history of mild epilepsy. Both patients were managed conservatively. The cases also demonstrate the utility of magnetic resonance imaging in the diagnosis of cavernous angioma.     

Pseudotransitory ischemic attacks as the initial symptom of multiple system atrophy

A 59-year-old man presented with an 18-month history of episodes of visual disturbance and dysphagia in association with gait unsteadiness and leg weakness lasting between 45 and 120 min. These episodes were interpreted as vertebrobasilar transitory ischemic attacks. However, they had been preceded by impotence for 6 months. Autonomic function tests remained normal until the age of 62 years when the patient developed documented orthostatic hypotension. Parkinsonism was precipitated by neuroleptic treatment at the age of 59 years, at which time pyramidal signs were evident. The patient was unable to tolerate levodopa preparations and subsequently developed the full clinical picture of pathologically proven multiple system atrophy, dying at age 65, 7 years after his first symptom.     

Dystonia as an isolated symptom of multiple sclerosis?

Sustained dystonia has seldom been reported during the course of multiple sclerosis (MS) [5-8, 10], and has been described as the first manifestation of the disease in only three cases [1,3]. We describe a patient with a diagnosis of laboratory-supported, defined MS in which sustained dystonia was the only neurological symptom.     

Pathophysiological mechanisms of hearing loss

An understanding of auditory transduction in the ear can contribute to a better comprehension of the pathophysiological mechanisms which give rise to hearing loss. The incoming sound sets up a mechanical traveling wave which begins at the base and progresses along the basilar membrane, reaching a point of maximal displacement. The region of maximal displacement is a function of stimulus frequency. The mechanical displacement, by directly opening ion channels in the stereocilia of the hair cells, induces changes in the electrical potential of the hair cells. This initial stage is called mechano-electrical transduction, and in the normal ear, is followed by a stage of electro-mechanical transduction based on the ability of the outer hair cells to respond to the electrical changes induced in them with a change in their length. This "electromotility" presumably provides mechanical feedback to the basilar membrane, augmenting its mechanical displacement. This is called the cochlear amplifier, providing the ear with improved sensitivity and frequency discrimination. Most forms of sensori-neural hearing losses (affecting the inner ear) are due to a lesion to some part of this cochlear amplifier (e.g. noise induced hearing loss, ototoxic drugs) and are therefore characterized by auditory threshold elevations and poorer frequency discrimination.     

Establishing the etiology of childhood hearing loss

The cause of hearing loss in children is often difficult to identify. We evaluated a cohort of 114 children (47 boys, 67 girls) referred with newly diagnosed hearing loss (non-otitis media) to identify factors predictive of etiology and type of hearing loss. Clinical (history and physical examination), laboratory, and radiographic data were collected. One hundred children (87.7%) had sensorineural hearing loss, and 14 (12.3%) had conductive or mixed hearing loss. The cause of hearing loss was identified in 54 children (48%). Patients with isolated aural atresia (n = 7) or with a known diagnosis of congenital cytomegalovirus infection (n = 21) were excluded from further data analysis. We conducted statistical analysis to identify factors predictive of the cause and type of hearing loss. Clinical factors that aided in identifying a cause included abnormal physical examination findings (p = 0.001) and craniofacial anomalies (p = 0.006). Computed tomography of the temporal bones was the only diagnostic test predictive of cause (p < 0.001). Factors predictive of the type of hearing loss detected (sensorineural vs. conductive or mixed) were abnormal physical examination findings (p = 0.01) and craniofacial anomalies (p = 0.004). An exhaustive laboratory or radiographic workup did not prove beneficial in identifying the etiology of hearing loss in our series.     

Effect of a hearing aid on progressive hearing loss

A group of 72 children with hearing aids followed in 4 different clinical settings presented progressive increase in sensorineural hearing loss. The mean hearing loss over time, the duration of hearing aid use and the gain were studied to test the hypothesis that continued progression in hearing loss was due to overly powerful amplification. No correlation was found between the progressive increased gain levels were associated with less progression in hearing loss than at lower gain levels. Therefore, the use of hearing aids was not directly and significantly related to the increase in hearing loss in the population studied. However according to the above criteria a few cases (4%) of progressive hearing loss did seem to be associated with hearing aid use.