Acute streptococcal meningitis presenting as bilateral conductive hearing loss.

Bacterial meningitis is a relatively uncommon condition encountered in the emergency department and the constellation of symptoms varies. Hearing loss has been well documented in the literature as a complication of the disease process, but not as the presenting complaint. We describe a case of a 59-year-old female who presented to the emergency department with sudden onset bilateral hearing loss who was found to have S. pneumonia meningitis bacterial meningitis. Even with advances in therapy, bacterial meningitis still carries a significant mortality rate. Early diagnosis and treatment is critical to achieving good outcomes.

Utility of basic fibroblast growth factor in the repair of blast-induced total or near-total tympanic membrane perforations: A pilot study.

OBJECTIVE: A pilot study was performed to investigate the utility of basic fibroblast growth factor (bFGF) in the repair of blast-induced total or near-total tympanic membrane perforations (TMPs). STUDY DESIGN: Prospective clinical study. SETTING: Tertiary university hospital. SUBJECTS AND METHODS: Patients who fulfilled the inclusion criteria were treated with 0.10-0.15 mL of bFGF solution applied directly to total or near-total TMPs once daily until the perforations closed or for a maximum of 6 months. The treatment response was monitored via serial otoendoscopy, and audiometric outcomes were evaluated. RESULTS: Complete TMP closure was achieved in 16 of 17 patients with a blast-induced total or near-total TMP. The mean closure time was 28.4 ± 10.9 days. The improvement in hearing from pre- to post-treatment was statistically significant. There were no complications or adverse outcomes. CONCLUSIONS: The direct application of bFGF to blast-induced total or near-total TMPs is a promising, minimally invasive alternative to conventional tympanoplasty, with a comparable success rate. As reported in the literature, the closure rate was higher than achieved with spontaneous healing. There was no effect of the inverted edge on healing outcome. The use of bFGF in this setting has immediate therapeutic applications for military personnel with blast-induced TMPs who are stationed in isolated, remote environments.

CHInese Medicine NeuroAiD Efficacy on Stroke Recovery - Extension Study (CHIMES-E): A Multicenter Study of Long-Term Efficacy.

BACKGROUND: The CHInese Medicine NeuroAiD Efficacy on Stroke recovery (CHIMES) study was an international randomized double-blind placebo-controlled trial of MLC601 (NeuroAiD) in subjects with cerebral infarction of intermediate severity within 72 h. CHIMES-E (Extension) aimed at evaluating the effects of the initial 3-month treatment with MLC601 on long-term outcome for up to 2 years. METHODS: All subjects randomized in CHIMES were eligible for CHIMES-E. Inclusion criteria for CHIMES were age ≥18, baseline National Institute of Health Stroke Scale of 6-14, and pre-stroke modified Rankin Scale (mRS) ≤1. Initial CHIMES treatment allocation blinding was maintained, although no further study treatment was provided in CHIMES-E. Subjects received standard care and rehabilitation as prescribed by the treating physician. mRS, Barthel Index (BI), and occurrence of medical events were ascertained at months 6, 12, 18, and 24. The primary outcome was mRS at 24 months. Secondary outcomes were mRS and BI at other time points. RESULTS: CHIMES-E included 880 subjects (mean age 61.8 ± 11.3; 36% women). Adjusted OR for mRS ordinal analysis was 1.08 (95% CI 0.85-1.37, p = 0.543) and mRS dichotomy ≤1 was 1.29 (95% CI 0.96-1.74, p = 0.093) at 24 months. However, the treatment effect was significantly in favor of MLC601 for mRS dichotomy ≤1 at 6 months (OR 1.49, 95% CI 1.11-2.01, p = 0.008), 12 months (OR 1.41, 95% CI 1.05-1.90, p = 0.023), and 18 months (OR 1.36, 95% CI 1.01-1.83, p = 0.045), and for BI dichotomy ≥95 at 6 months (OR 1.55, 95% CI 1.14-2.10, p = 0.005) but not at other time points. Subgroup analyses showed no treatment heterogeneity. Rates of death and occurrence of vascular and other medical events were similar between groups. CONCLUSIONS: While the benefits of a 3-month treatment with MLC601 did not reach statistical significance for the primary endpoint at 2 years, the odds of functional independence defined as mRS ≤1 was significantly increased at 6 months and persisted up to 18 months after a stroke.

Atresia and sudden sensorineural hearing loss.

This case study summarizes findings in an adult male, aged 57, who presented to the Adult Audiology Clinic with aural atresia in the right ear resulting in a conductive hearing loss and a sudden sensorineural hearing loss in the left ear. Treatment options included reconstruction surgery in the right ear, bone anchored hearing aid in the right ear to overcome the conductive hearing loss, bone anchored hearing aid in the left ear for single sided deafness, and intratympanic steroid injections in the left ear to salvage hearing. This case study highlights that when a patient is educated on all available options the patient is then able to make a decision comfortable to him and to help improve his hearing.

[Chondrocalcinosis of the temporomandibular joint revealed by a hearing loss: a case report]. / Une chondrocalcinose de l'articulation temporo-mandibulaire révélée par une hypoacousie: à propos d'un cas.

INTRODUCTION: Chondrocalcinosis is a microcrystalline arthropathy that principally affects the knee. It is a rare disorder, usually asymptomatic, that occurs mainly in the elderly people. PURPOSE: To report a case of a temporomandibular joint chondrocalcinosis with ossicular contact revealed by a conductive hearing loss. CASE REPORT: We describe the case of a 57-year-old man with a right conductive sudden hearing loss of 15 dB. The CT scan revealed a lytic lesion in the right attic extended to the middle cerebral fossa in contact with the ossicles with a suspicion of lysis of the head of the malleus. MRI showed a lesion enhancing after gadolinium injection on T1 weighted images. A biopsy revealed a chondrocalcinosis of the temporomandibular joint. Due to the complexity of surgical excision and the benin character of the lesion, a medical treatment and a radiologic follow-up every six months were proposed. CONCLUSION: Chondrocalcinosis of the temporo-mandibular joint is rare especially when it is revealed by a hearing loss. We present here a review of the literature.

A case of chronic otitis media caused by Mycobacterium abscessus.

Although it appears very uncommon in adult COM, Mycobacterium abscessus should be considered as a possible cause of a chronically draining ear. Multi-antibiotic chemotherapy including high-dose clarithromycin can effectively treat adult COM cased by M. abscessus. The first case report of adult chronic otitis media (COM) caused by M. abscessus is described here. A 61-year-old woman presented persistent otorrhea for 2 months, despite treatment with standard antimicrobial drugs. Physical examination revealed a small perforation of the tympanic membrane and edematous middle ear mucosa. Mycobacterial cultures and PCR yielded non-tuberculous mycobacteria (NTM); M. abscessus. Intravenous panipenem/betamipron and amikacin and oral clarithromycin were administered for 36 days. Computed tomography of the temporal bone showed improved aeration in the tympanic cavity, but soft tissue shadow remained unchanged in the mastoid 31 days after starting medication. She therefore underwent tympano-mastoidectomy at 36 days. At surgery, inflammation remained in the middle ear, and edematous pale mucosal tissue was noted around the stapes and ossicular chain. Histopathologic examination showed inflammation and granulation tissue, but no caseating necrosis or acid-fast bacilli. After surgery the symptoms resolved and remained well without evidence of infection recurrence 12 months after the operation.

Tuberculous otitis media: a case presentation and review of the literature.

A 42-year-old farmer being treated for pulmonary tuberculosis was referred to our clinic after developing otorrhoea and hearing loss in his right ear. Examination revealed a large subtotal perforation in the right ear in addition to a purulent discharge with right facial nerve palsy. Audiometry indicated a 35 dB conductive hearing loss at 0.5, 1 and 2 kHz with air-bone gaps of 12, 15 and 10 dB, respectively. A middle ear biopsy was performed under direct visualisation, with a middle ear lavage which was positive for allergic fungal sinusitis (AFS). The final diagnosis was tuberculous otitis media (TOM). Clinical symptoms and signs should be reviewed in every case of chronic otitis media keeping the possibility of tuberculosis in mind. Otorrhoea in a patient with known or suspected active pulmonary tuberculosis should be assumed to be TOM until proven otherwise.

Hearing loss in Fabry disease: data from the Fabry Outcome Survey.

Hearing loss is a common symptom in Fabry disease, but neither its natural course nor its aetiology has been defined precisely. The aim of this study was to provide a detailed epidemiological description of hearing impairment in patients in the Fabry Outcome Survey (FOS), which is the largest available database of Fabry patients. Questionnaires were completed by 566 Fabry patients, of whom 316 reported ear-related symptoms. Pure-tone audiograms from 86 patients, performed before starting enzyme replacement therapy, were analysed and compared with age- and sex-specific normal values (International Organization for Standardization, ISO 7029). When compared to an age-matched population (ISO 7029), 74% of patients had a threshold elevated above the 95th centile in at least one tested frequency. All frequencies were affected to a similar degree. However, only 14 patients (16%) were clinically affected by hearing impairment according to the age-independent World Health Organization (WHO) classification (mean threshold at 0.5, 1 and 2 kHz worse than 25 dB). Hearing loss was sensorineural in 63 patients (73%) of whom 7 patients (8%) had also a conductive component. One patient had a purely conductive hearing loss. Episodes of sudden hearing loss seemed to occur more frequently than in the general population. Men were affected earlier and more severely than women. Hearing in Fabry disease is significantly worse than in an age-matched general population but leads to clinically relevant hearing impairment in only 16% of cases. It resembles accelerated presbycusis with an additional Fabry-specific strial-type hearing loss.

Hearing loss in Fabry disease: the effect of agalsidase alfa replacement therapy.

The aim of this study was to describe the nature and prevalence of hearing loss in Fabry disease (McKusick 301500), a rare X-linked lysosomal storage disorder, and its response to enzyme replacement therapy with agalsidase alfa. Fifteen hemizygous male Fabry patients (aged 25-49 years) were randomized to receive placebo or enzyme replacement therapy for 6 months; all have received open-label enzyme replacement therapy for an additional 24 months thus far. Pure-tone audiometry, impedance audiometry and otoacoustic emission testing were performed at 0 (baseline), 6, 18 and 30 months. Four patients (27%) had bilateral and 7 (47%) had unilateral high-frequency sensorineural hearing loss (SNHL). Two (13%) had unilateral middle ear effusions with conductive losses persisting beyond 6 months. Only 3 (20%) had normal hearing. High-frequency SNHL deteriorated over the first 6 months in both placebo and active treatment groups by a median 4.3 dB ( p =0.002, Wilcoxon matched pairs). This hearing loss subsequently improved above baseline by 2.1 dB at 18 months ( p =0.02) and by 4.9 dB at 30 months ( p =0.004). In conclusion, significant hearing loss, usually high-frequency SNHL, is a common manifestation of Fabry disease in adults. alpha-Galactosidase A replacement therapy with agalsidase alfa appears to reverse the hearing deterioration in these patients. This improvement is gradual, however, suggesting the need for long-term enzyme replacement therapy.

[Sudden deafness in sickle cell anemia: a case report]. / La surdité brutale chez le drépanocytaire: à propos d'un cas.

The non-expected deafness is quite obvious or easily diagnosed cause. The sickle cell disease is one of the aetiologies of this one. We present one case observed on a 30 years old patient. There is a close connection between the vascular factor of the non-expect deafness and the erythrocytic falciformation of sickle cell anaemia causing the obliteration of the terminal auditory internal artery and generating the ischaemia of the cochlea anoxia. The high sensitivity of the cochlea to anoxia and its great fragility require an early therapy in order to recover auditory capacity. Patients suffering from sickle cell disease should be encouraged to have a regular assessment of audition.

Paediatric hearing loss in rural Pakistan.

OBJECTIVE: The present study was designed to generate population-based data on the prevalence and causes of hearing loss in rural Pakistani children. A community screening programme was utilized to identify and evaluate the hearing impaired. METHODS: The study was performed in conjunction with the Ministry of Population Welfare in Sialkot District, Punjab Province. School-aged children between the ages of 5 and 15 years were screened and examined for hearing impairment according to World Health Organization (WHO) protocols. Case-control analysis of audiometric, physical examination, and risk factors for hearing loss were performed, followed by chi-square analyses. RESULTS: A total of 607 children comprised the study population, with an overall point prevalence of hearing impairment of 7.9%. Fifty percent of all hearing loss was conductive in nature, amenable to either medical or surgical therapy. The risk factors most associated with conductive hearing loss were otorrhea and multiple ear infections greater than 5. In cases of severe hearing loss, 70% were the result of consanguinous marriages. Almost no cases of hearing loss were attributable to measles, mumps, rubella, and the TORCH infections. CONCLUSION: This study has generated some badly needed population-based data on the magnitude of the problem of hearing loss in rural Pakistan. It is hoped that the results of this work will stress the importance of hearing health in Pakistan and to encourage other professionals to pursue similar projects.

Auditory brainstem responses of CBA/J mice with neonatal conductive hearing losses and treatment with GM1 ganglioside.

Exogenous administration of GM1 ganglioside to CBA/J mice with a neonatal conductive hearing loss ameliorates the atrophy of spiral ganglion neurons, ventral cochlear nucleus neurons, and ventral cochlear nucleus volume. The present investigation demonstrates the extent of a conductive loss caused by atresia and tests the hypothesis that GM1 ganglioside treatment will ameliorate the conductive hearing loss. Auditory brainstem responses were recorded from four groups of seven mice each: two groups received daily subcutaneous injections of saline (one group had normal hearing; the other had a conductive hearing loss); the other two groups received daily subcutaneous injections of GM1 ganglioside (one group had normal hearing; the other had a conductive hearing loss). In mice with a conductive loss, decreases in hearing sensitivity were greatest at high frequencies. The decreases were determined by comparing mean ABR thresholds of the conductive loss mice with those of normal hearing mice. The conductive hearing loss induced in the mice in this study was similar to that seen in humans with congenital aural atresias. GM1 ganglioside treatment had no significant effect on ABR wave I thresholds or latencies in either group.

[Hearing disorders in children less than 16 months of age after bacterial meningitis with reference to cerebrospinal fluid elastase]. / Hörstörungen bei Kindern unter 16 Monaten nach bakterieller Meningitis unter Berücksichtigung der Liquorelastase.

Hearing impairment as a sequela of acute bacterial meningitis is a well known complication. Dexamethasone therapy in addition to antibiotics is beneficial in the reduction of deafness, implicating that inflammation may be one reason for hearing impairment. The risk of hearing impairment in different types of bacterial meningitis is well studied. In very young children < 1.5 years of life the incidence of hearing loss and the possible correlation of laboratory data with the development of deafness is yet unknown. We therefore examined the brainstem auditory evoked potentials in 25 children between the first month and the 16th month of life who we treated for meningitis during 3 years in our hospital. 11 children were treated with dexamethasone. In 9 children we found abnormal brainstem auditory evoked potentials, which we controlled every 3 months. 7 children had transient conductive hearing impairment with good recovery during the first year after the disease. In 2 cases we found permanent bilateral sensorineural hearing loss. There was a significant relationship between hearing loss and elastase in cerebrospinal fluid. Dexamethasone reduced this relationship. A screening of hearing should be performed as routine control in all patients with acute meningitis. The association of high elastase in cerebrospinal fluid and later hearing impairment indicates a pathophysiological relation between activation of granulocytes and hearing loss.

Mixed hearing loss in otosclerosis: indication for long-term follow-up.

This retrospective study of 146 ears with long-term follow-up after otosclerosis surgery evaluated the stability of hearing results, the incidence of sensorineural hearing loss, and the effect of fluoride treatment. Follow-up was at least 15 years (mean, 25.2 yr; range, 15-44 yr). There were 97 large fenestra stapedectomy operations, 23 lateral canal fenestrations, 7 mobilizations, and 19 revision stapes operations. The level of air-bone gap achieved at surgery remained stable over time; the mean deterioration rate was only 0.2 dB per year. Profound sensorineural hearing loss ( > or = 65 dB bone conduction average) at the most recent follow-up occurred in 13 ears (8.9%). Such hearing loss occurred in all operative groups. Mean bone conduction average immediately postoperatively was significantly higher in these ears than in others in the study. This finding indicates that a mixed hearing loss at surgery is a factor that increases the risk of later profound cochlear loss. Only 3 percent of ears with pure conductive hearing loss, but 28 percent of patients with mixed hearing loss at surgery eventually suffered profound cochlear loss. Sodium fluoride was used to treat 11 ears with progressive cochlear loss. The rate of bone conduction hearing deterioration decreased in all ears after treatment, and none developed profound hearing loss. Follow-up after the first postoperative year is not necessary if pure conductive hearing loss is present at surgery. Annual follow-up with audiograms is recommended if a mixed hearing loss is present. Fluoride treatment is recommended if inner ear hearing loss progresses.

[Allergic reaction in therapy with naftidrofuryl (Dusodril). A case report]. / Allergische Reaktion bei Therapie mit Naftidrofuryl (Dusodril). Ein Fallbericht.

There are many drugs marketed for the purpose of altering vascular blood flow in various regions, especially of the central nervous system and in peripheral arterial insufficiency. More than 50 different methods are described for the treatment of sudden deafness. Considerations of the therapy of sudden deafness are influenced by the fact that the cause of the disease is unknown. The dysfunction of the hair-cells of the organ of CORTI is thought to be caused by a deficit of oxygen due to disorders of micro-circulation in the inner ear. The infusion of vaso-active drugs in the early state of disease can lead to a remarkable improvement of hearing whereas the prospect of improvement without treatment remains uncertain. Nevertheless it may be difficult to distinguish the beneficial effects of vasodilator agents from spontaneous improvement. Naftidrofuryl oxalate (dusodril) has been in use for many years and proved its therapeutic value in many studies. It is regarded as non-toxic and is used extensively in Europe. Side effects are only reported rarely, and include decrease of cerebral blood flow, abdominal distension, diarrhoea, oesophageal ulceration, epileptic seizures, aphasia, disturbances of consciousness, hypotension, hypertensive crisis, vertigo and dizziness, depression of cardiac conduction, thrombophlebitis, and allergy. This case report of allergic reaction in a young female patient demonstrates that the intravenous application of this drug may lead to severe complications.