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 MEASURE:: 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 toinvestigate the role of antioxidants, including CoQ, in ISSNHL

Correlation between GJB2 mutations and audiological deficits: personal experience

Mutations in GJB2 gene are the most common cause of genetic deafness. More than 100 mutations have been described. The aim of this work is to describe the personal experience in genetic hearing loss, investigating the audiological and genetical characteristics of Cx26 deafness and correlating genotype and phenotype. We performed audiological and genetical evaluation in 154 patients affected by non-syndromic deafness of different degree. All patients showed a bilateral symmetrical sensorineural hearing loss. From the genetical analysis 127 probands resulted as negatives while 27 as positives (51.8% homozygous for 35 delG, 14.8% compound heterozygosis and 33.3% single mutation); 7.5% of patients had a mild deafness, 37% moderate, 33.3% severe and 22.2% profound. The c.35 delG mutation was detected in 66.6% of patients. Three mutations were found in compound heterozygosis with 35 delG, six different single mutations already described, and a new mutation S138G were also found. Correlation between genotype and phenotype confirmed the high variability of hearing loss

Somatosensory tinnitus: correlation between cranio-cervico-mandibular disorder history and somatic modulation

In a subpopulation of patients, tinnitus can be modulated by movements of the jaw or head and neck due to complex somatosensory-auditory interactions. In some of these subjects, tinnitus could be related to an underlying temporomandibular (TMJ) or craniocervical (NECK) dysfunction that, if correctly identified, could streamline treatment and increase chances of tinnitus improvement. However, it is still unclear whether somatic modulation of tinnitus could be used as a screening tool for identifying such patients. In this study, we included 310 tinnitus patients with normal hearing, no psychiatric comorbidities, and a positive history of TMJ and/or NECK dysfunction and/or a positive modulation of tinnitus to evaluate the characteristics of somatic modulation, investigate the relationship between positive history and positive modulation, and identify factors most strongly associated with somatic modulation. Tinnitus modulation was present in 79.67% of the patients. We found a significant association within the same subjects between a positive history and a positive tinnitus modulation for the same region, mainly for TMJ in unilateral tinnitus patients and for TMJ + NECK in bilateral tinnitus patients. A strong correlation between history and modulation in the same somatic region within the same subgroups of subjects was also identified. Most TMJ maneuvers resulted in an increased loudness, while NECK maneuvers showed an increase in tinnitus loudness in about 59% of cases. High-pitched tinnitus and male gender were associated with a higher prevalence of modulation; no differences were found for tinnitus onset, Tinnitus Handicap Inventory score, and age. In this paper, we report a strong association between history and modulation for the same regions within the same patients; such an association should always be investigated to improve chances of a correct diagnosis of somatosensory tinnitus

Universal Newborn Hearing Screening using A-TEOAE and A-ABR: the experience of a large public hospital

BACKGROUND: Universal Newborn Hearing Screening (UNHS) aims to identify hearing loss in the early postnatal period; prompt detection of bilateral or unilateral hearing loss is mandatory for timely intervention. METHODS: This retrospective study reports the results of the first two years of a UNHS program on 4,719 newborns in a large public Italian hospital. Screening was divided into two levels: Automatic Transient Otoacoustic Emissions were used for first level; Automated Auditory Brainstem Response for second level. Second level included children with a REFER response at first level and babies with a family history for hearing loss or other risk factors. Hearing loss diagnosis was made using clinical Auditory Brainstem Response. RESULTS: During first level, 254 (5.4%) newborns were REFER. At retest, 130 (51.1%) babies were PASS and 48 (18.8%) were REFER. 76 babies dropped out (29.9%). 146 babies (3.1%) were referred to the second level: 48 for a REFER response at first level and 98 for a PASS response but potential hearing loss due to risk factors. 24 babies dropped out (16.4%). Out of 122 newborns tested in the second level, 105 (86.1%) had a PASS response and 17 (13.9%) were REFER. Our screening protocol identified 7 (0.14%) babies with profound hearing loss; 5 had unilateral and 2 had bilateral hearing loss. 2 babies dropped out at diagnostic level (11.8%). CONCLUSIONS: A correct and early diagnosis of hearing loss is mandatory to prevent permanent consequences; the spread of hearing screening programs is the optimal solution to reach this goal

"One-shot" CO2 versus Er:YAG laser stapedotomy: is the outcome the same?

To assess and compare the functional results obtained by means of multiple-shot Erbium: yttrium-aluminum-garnet (Er:YAG) laser to those obtained using "one-shot" CO(2) laser stapedotomy in patients affected by otosclerosis. A retrospective case review was performed. Of the total number of 123 patients (114 ears) who underwent primary small-fenestra stapedotomy from January 2006 to September 2008, seven patients who received multiple-shot laser CO(2) stapedotomy were excluded from the study. The remaining 116 patients (104 ears) were sorted, and "one-shot" CO(2) laser stapedotomy (group A) was performed in 35/104 and Er:YAG laser stapedotomy (group B) in 69/104. After surgery, air conduction-pure tone average (AC-PTA) and air-bone gap (ABG) improved significantly in both groups, whereas sensorineural hearing loss (SNHL) and bone conduction (BC)-PTA did not change in both the groups. In group A, the postoperative ABG was significantly better (12.63 vs. 14.86 dB). Moreover, after "one-shot" stapedotomy, the AC-PTA significantly improved in all tested frequencies. On the contrary, in group B the AC-PTA improved significantly only in two frequencies (0.5 and 1 kHz). Consistent with previous reports, our findings confirm that laser stapedotomy is a safe and effective surgery, regardless of the technique. Based on our functional results, the "one-shot" CO(2) laser technique seems to be associated with a significantly better postoperative ABG if compared to Er:YAG laser stapedotomy