Coeliac disease and hearing loss: preliminary data on a new possible association.

OBJECTIVE: Coeliac disease (CD), an autoimmune gluten-dependent enteropathy, can be associated with several extra-intestinal manifestations, including neurological disorders. At present, no data are available on the presence of hearing loss disorder in coeliac patients. The aim of the present study was to investigate the prevalence of hearing loss in coeliac patients compared with that in healthy controls. MATERIAL AND METHODS: Twenty-four adult coeliac patients and 24 healthy subjects matched for gender, age, smoking and drinking habits were enrolled in the study. Among the coeliac patients, 6 were newly diagnosed and 18 patients were on a gluten-free diet for at least one year. RESULTS: A hearing loss was found in 10 (47.1%) coeliac patients and 2 (9.1%) healthy controls. All CD patients with hearing loss presented a sensorineural hearing loss. The prevalence of hearing loss was significantly higher in coeliac patients than in healthy controls (p = 0.01) but it was not significantly different between untreated (33.3%) and treated (44.4%) coeliac patients (p: NS). CONCLUSIONS: Despite the low number of subjects evaluated, the present study showed a higher prevalence of hearing loss in coeliac patients than in healthy controls, suggesting an association between CD and hearing loss. Immunological processes such as ear-specific and non-specific autoantibodies and vasculitis could be the basis of this association. Further longitudinal investigations on a larger sample size will be necessary to confirm the present data.

Coenzyme Q 10 and cardiovascular risk factors in idiopathic sudden sensorineural hearing loss patients.

OBJECTIVES: We investigated the association of idiopathic sudden sensorineural hearing loss (ISSNHL) with coenzyme Q (CoQ) and cardiovascular risk factors. STUDY DESIGN: A prospective study. SETTING: Hospital center. PATIENTS: Thirty Italian patients with ISSNHL and 60 healthy Italian subjects. INTERVENTION: Diagnostic. MAIN OUTCOME MEASURES: Evaluation of serum CoQ levels and cardiovascular risk factors (total cholesterol, low-density lipoprotein [LDL], homocysteine [HCY]). The results were compared with variance analysis and Student's t test. Univariate and multivariate analysis were used to evaluate the association between ISSNHL and CoQ, total cholesterol, LDL, and HCY levels. RESULTS: In our series, we found a significant association between ISSNHL and high total cholesterol (p < 0.05), high LDL (p = 0.021), and low CoQ (p < 0.05) levels. We did not find a significant association between ISSNHL and HCY levels. In the univariate analysis, low levels of CoQ, high levels of total cholesterol, and LDL were found to be significantly associated with ISSNHL. In the multivariate analysis, only high levels of total cholesterol and low levels of CoQ remained significantly associated with a high risk of sudden sensorineural hearing loss. CONCLUSION: The studies regarding the role of cardiovascular risk factors in ISSNHL are not conclusive. This is the first report regarding the association of ISSNHL and low serum levels of the antioxidant CoQ. Further studies are needed to investigate the role of antioxidants, including CoQ, in ISSNHL.

Magnetic resonance imaging findings in sudden sensorineural hearing loss.

OBJECTIVE: To investigate the role of magnetic resonance imaging (MRI) in the diagnosis of sudden sensorineural hearing loss (SSNHL). METHODS: Fifty-four consecutive patients affected by SSNHL were investigated using brain MRI. MRI was performed with an eight-channel phased-array head coil to study the entire audiovestibular pathway and the whole brain. The protocol study consisted of a high-resolution study of the temporal bone, internal auditory canal (IAC), cerebellopontine angle (CPA), and brainstem combining 2 mm thin-slice axial T(2)-weighted two-dimensional fast spin echo (FSE) and fluid-attenuated inversion recovery (FLAIR) sequences, pre- and postcontrast (gadolinium-diethylenetriamine pentaacetic acid) administration fat-suppressed axial T(1)-weighted two-dimensional FSE sequences, and a T(2)*-weighted three-dimensional Fourier transformation-constructive interference in steady state sequence (FT-CISS) , with 0.4 mm ultrathin partitions. The rest of the brain was studied with a 4 mm axial T(2)-weighted FLAIR sequence. RESULTS: Thirty-one of 54 (57%) cases of SSNHL presented with MRI abnormalities. In 6 of 54 cases, the detected abnormality was directly correlated to the clinical picture (2 labyrinthine hemorrhage, 1 cochlear inflammation, 1 acoustic neuroma, 1 arachnoid cyst of the CPA, and 1 case of white matter lesions in the pons, compatible with demyelinating plaques along the central audiovestibular nervous pathway, as the first expression of multiple sclerosis). CONCLUSIONS: An extensive MRI study of the audiovestibular nervous pathway and of the whole brain, pre- and postparamagnetic contrast administration, is recommended to rule out the wide spectrum of abnormalities that can cause SSNHL.

Glutathione s-transferase gene polymorphisms in Italian patients with sudden sensorineural hearing loss.

HYPOTHESIS: To investigate the association between glutathione S-transferase (GST) gene polymorphisms and sudden sensorineural hearing loss (SSNHL) in a population of consecutive Italian patients. Assuming that reactive oxygen species formation may play a role in inner ear damage, we investigated whether profiles of GSTs antioxidant enzymes M1 and T1 genotypes may be associated with the risk of SSNHL. STUDY DESIGN: A prospective study in patients with SSNHL. PATIENTS AND METHODS: We investigated 80 Italian patients with SSNHL for the frequency of GSTT1 and GSTM1 polymorphisms. Genotype distribution of all factors found in patients were compared with those of 80 healthy control subjects of the same ethnic background using chi and odds-ratio analysis. Statistical significance was accepted at a level of p < 0.05. RESULTS: In our series, the frequencies of GSTM1 and GSTT1 null genotypes did not differ from those of the control subjects. CONCLUSION: The few studies regarding genetic polymorphisms of GSTs in SSNHL are not conclusive. Further studies are needed to investigate the role of antioxidants including GSTs in SSNHL and to provide the lacking information to improve our knowledge in the field before implementing the use of genetic polymorphism in the SSNHL medicine daily practice.

Lack of association between inherited thrombophilic risk factors and idiopathic sudden sensorineural hearing loss in Italian patients.

OBJECTIVES: We investigated the presence of congenital thrombophilic risk factors in a population of consecutive Italian patients affected by idiopathic sudden sensorineural hearing loss (SSNHL). METHODS: We investigated 48 patients with idiopathic SSNHL for the presence of congenital thrombophilic risk factors. The factor V Leiden G1691A, the prothrombin G20210A allele, and methylenetetrahydrofolate reductase (MTHFR) C677T genotypes were investigated. Allele frequencies and genotype distribution of all factors found in patients were compared to those of 48 healthy subjects of the same ethnic background by Chi2 and odds-ratio analysis. Odds ratios and 95% confidence intervals were calculated for allele and genotype frequencies of all thrombophilia variants. Statistical significance was accepted with a p value of less than .05. We also performed the following blood tests: hemacytometric analysis including platelet count, prothrombin time, activated partial thromboplastin time, fibrinogen, erythrocyte sedimentation rate, C-reactive protein, protein S, protein C, antithrombin III, and activated protein C resistance. RESULTS: In our series, we did not find an association between SSNHL and abnormal levels of antithrombin III, protein C, protein S, D-dimer, or fibrinogen; activated protein C resistance; or factor V G1691A, prothrombin G20210A, or MTHFR C677T mutations. CONCLUSIONS: At present, the few studies regarding genetic polymorphisms of congenital thrombophilic factors in SSNHL are not conclusive. According to our data, factor V G1691A, prothrombin G20210A, and MTHFR C677T variants should be not considered risk factors for SSNHL. Further large prospective studies are needed to provide currently lacking information and to improve our knowledge in the field before we recommend the determination of genetic polymorphism in SSNHL as routine practice.

Sudden sensorineural hearing loss: our experience in diagnosis, treatment, and outcome.

OBJECTIVES: To describe our experience concerning sudden sensorineural hearing loss (SSNHL) in a large single-institution series of SSNHL patients and to discuss the results. METHOD: This was a retrospective study, and the charts of 156 consecutive inpatients (65 males, mean age 44 years, range 10-74 years; 91 females, mean age 46 years, range 15-75 years) with the diagnosis of SSNHL from 1987 to 2000 were reviewed. One hundred forty-three of 156 patients received multidrug therapy (plasma expanders, antiaggregants, steroids), whereas only 13 SSNHL patients received hyperbaric oxygen therapy. RESULTS: Old age, vascular and metabolic risk factors, and cigarette smoking do not a have a high prevalence in the SSNHL population. An etiologic factor was detected in 23 of 156 (15%) cases (16 cases of acute infection, 4 cases of neurovascular conflicts, 2 cases of cerebellar angiomas, 1 case of cochleovestibular schwannoma). The outcome was not related to the laterality, age, or hearing loss type. On the contrary, a statistically significant association between poor recovery and male sex, both tinnitus and vertigo, and the initial severity of the hearing loss was observed. CONCLUSIONS: Mostly, SSNHL results in idiopathic disease. At present, diagnostic and therapeutic efforts appear to be inadequate to improve the prognosis of SSNHL. Further studies are needed to obtain better knowledge about the etiopathogenesis of SSNHL so that new therapeutic strategies can be considered in the treatment of this challenging ear disease.