Clinical observations and risk factors for tinnitus in a Sicilian cohort.

The aims of this study were to determine the distribution of risk factors associated with tinnitus analysing their role in the development of tinnitus and the effects of their interaction; to evidence the importance of a suitable and adequate clinical and audiologic assessment to avoid those modifiable risk factors responsible for cochlear dysfunction and tinnitus onset. 46 subjects with tinnitus and 74 controls were studied according to: age, sex, Body Mass Index (BMI), neck circumference, tobacco smoking, feeling fatigue or headache, self reporting snoring, hypertension, diabetes, coronary heart disease, and/or hyperlipidemia, and laboratory finding as lipid profile and levels of reactive oxygen metabolites (d-ROM). Audiological assessment was performed by multi-frequency audiometry (PTA(0.5-16 kHz)) and transient-evoked otoacoustic emissions (TEOAE diagnostic). Univariate analysis was performed to examine the association between determinants and occurrence of tinnitus; Mantel-Haenszel test (G.or) was used to investigate the joint effect of determinants on tinnitus. Tinnitus was more frequent among males with age >50 years; BMI >30 kg/m(2), neck circumference >40 cm, headache, hypertension, hypercholesterolemia resulted significant risk factors for tinnitus (P < 0.0001). Tinnitus group had more comorbidity (P < 0.0001) and worse audiometric thresholds (60.87 Vs 21.62% hearing loss; P < 0.0001) with respect to control group. The interaction between hypertension-BMI ≥ 30 kg/m(2) (G.or = 8.45) and smoking-hypercholesterolemia (G.or = 5.08) increases the risk of tinnitus (P < 0.0001). Our results underline that several factors either individually or jointly contribute to tinnitus onset; a comprehensive knowledge about tinnitus risk factors and associated clinical conditions could contribute to minimizing this disorder.

A genotype-phenotype correlation in Sicilian patients with GJB2 biallelic mutations.

The aim of this work was to study the genotype distribution of Sicilian patients with biallelic GJB2 mutations; to correlate genotype classes and/or specific mutations of GJB2 gene (35delG-non-35delG) with audiologic profiles. A total of 10 different mutations and 11 different genotypes were evidenced in 73 SNHL subjects; 35delG (90.36 % of cases) and IVS1+1 (13.69 %) were the most common mutations found in the cohort with a significant difference in the distribution between North and South Sicily. Audiological evaluation revealed a severe (16/73) to profound (47/73) hearing loss (HL) in 86.13 % of cases without significant difference between the degree of HL and the province of origin of the subjects (P = 0.727). The homozygous truncating (T/T) genotype was the most widespread (89.04 % of cases), with a severe-to-profound hearing impairment in 90.36 % of T/T class with respect to truncating/non-truncating (T/NT) and non-truncating/non-truncating (NT/NT) genotypes (P = 0.012). From the comparison of homozygous 35delG and 35delG/non-35delG genotypes, a more profound HL in the homozygous 35delG than in compound heterozygous 35delG/non-35delG (p < 0.0001) resulted. This study confirms that 35delG is the most common mutation in the Mediterranean area with a heterogeneous distribution of the genotypes between North and South Sicily; probands homozygotes for 35delG or presenting a T/T genotype are more apt to have a severe-to-profound HL.

Distribution and phenotype of GJB2 mutations in 102 Sicilian patients with congenital non syndromic sensorineural hearing loss.

OBJECTIVE: To evaluate the frequency of GJB2 mutations and their correlation with phenotype in Sicilian non-syndromic sensorineural hearing loss (NSHL) patients. DESIGN: Sequencing of the coding region, basal promoter, exon 1, and donor splice site of the GJB2 gene; screening for the presence of the two common GJB6 deletions. STUDY SAMPLE: A cohort of 102 Sicilian NSHL patients. RESULTS: Fifteen different mutations in GJB2 and seventeen different genotypes were detected. No GJB6 mutations were found. The hearing impairment was profound in the 64.72% of probands (mean PTA0.25-4 kHz of 88.82 ± 26.52 dB HL). A total of 81.37% of patients harboured at least one c.35delG allele; c.167delT and c.-23 + 1G> A were identified in 10.78% and the 9.8% of patients respectively; c.35delG homozygotes presented more severe hearing impairment (75.59% of profound hearing loss) and a higher mean PTA0.25-4 kHz (96.79 ± 21.11 dB HL) with respect to c.35delG/non-c.35delG and c.35delG/Wt patients (P < 0.05). CONCLUSIONS: This work underlines the role of c.35delG, c.167delT and c.-23 + 1G> A as the most frequent causes of NSHL in Sicily. The c.35delG frequency found is similar to those reported in other populations of the Mediterranean area. The analysis of genetic and audiologic data confirmed a variability in the phenotype associated to a single genotype.

Prevalence and risk factors for sensorineural hearing loss: Western Sicily overview.

The objective of this work was to evaluate the prevalence of sensorineural hearing loss (SNHL) and distribution of the main risk factors associated to it focusing on their role in the development of deafness and their interaction. We performed a global audiological assessment (through TEOAE, tympanometry and ABR) in 508 infants at risk studying the main risk factors reported by Joint Committee on Infant Hearing (2007). Fifty-one infants (10.03 %) were diagnosed with SNHL (45 bilateral and 6 unilateral) with a mean hearing threshold of 87.39 ± 28.25 dB HL; family history of hearing impairment (HI) and TORCH infections indicated independent significant risk factors (P < 0.00001 and P = 0.024, respectively). High SNHL percentages were evidenced also in NICU babies, due to the various pathologies and risk factors presented by these infants, and among newborns who suffered from hyperbilirubinemia (11.97 and 9.52 %, respectively). The mean degree of hearing loss for children with family history of HI (>100 dB HL) emphasizes the necessity of an early diagnosis to avoid the consequences of auditory deprivation. Craniofacial abnormalities and syndromes associated to HI showed an important relationship (P < 0.00001) with conductive hearing loss. A progressive increase was evidenced in SNHL incidence as the number of risk factors rises (from 5.12 for 2 risk factors to 28.5 % for 5 or more) with a significant difference among the groups (P = 0.049); multiple risk factors showed an additional cofactor for HL (r (2) = 0.93). Considering the high SNHL prevalence (10.03 %) in infants at risk, this study highlights the necessity to implement a neonatal hearing screening program in Western Sicily.

Otitis media with effusion with or without atopy: audiological findings on primary schoolchildren.

OBJECTIVE: The objective of the study was to evaluate the role of atopy in otitis media with effusion (OME) in children attending primary school, focusing on the audiometric and tympanometric measurements among atopic and nonatopic subjects suffering from OME. MATERIALS AND METHODS: Three hundred ten children (5-6 years old) were screened in Western Sicily by skin tests and divided into atopics (G1) and nonatopics (G2). The samples were evaluated for OME by pneumatic otoscopy, tympanogram, and acoustic reflex tests. The parameters considered were as follows: documented persistent middle ear effusion by otoscopic examination for a minimum of 3 months, presence of B or C tympanogram, absence of ipsilateral acoustic reflex, and a conductive hearing loss greater than 25 dB at any one of the frequencies from 250 Hz through 4 kHz. RESULTS: The overall prevalence rate of OME was 12.9% (42.85% for G1 and 6.30% for G2, odds ratio = 11.16); OME was bilateral in 28 children (70%). B tympanogram was evidenced in 48 ears (70.59%), with a significative difference between G1 and G2 (P < .001). The analysis of mean air conduction pure tone (31.97 dB for G1 and 29.8 dB for G2) and of tympanometric measurements such as ear canal volume, tympanometric peak pressure, and static compliance by analysis of variance test showed a significative difference between G1 and G2 (P < .05). CONCLUSIONS: The higher prevalence of OME in atopic children and the statistically significant differences in audiometric and tympanometric measurements among atopic and nonatopic subjects suffering from OME suggest the important role of allergy in the genesis and recurrence of OME.

Sudden sensorineural hearing loss as prodromal symptom of anterior inferior cerebellar artery infarction.

Sudden sensorineural hearing loss is a clinical condition characterized by a sudden onset of unilateral or bilateral hearing loss. In recent years sudden deafness has been frequently described in association with anterior inferior cerebellar artery (AICA) infarction generally presenting along with other brainstem and cerebellar signs such as ataxia, dysmetria and peripheral facial palsy. The authors report a rare clinical case of a 53-year-old man who suddenly developed hearing loss and tinnitus without any brainstem or cerebellar signs. Computed tomography of his brain was normal, and the audiological results localized the lesion causing deafness to the inner ear. Surprisingly, magnetic resonance imaging showed an ischemic infarct in the right AICA territory. This case represents the fifth in the literature to date but it confirms that AICA occlusion can cause sudden deafness even without brainstem or cerebellar signs. Therefore, we recommend submitting the patient for neuroimaging, as an emergency, in order to exclude infarction of the AICA territory. By doing this, it may be possible to limit the extent of the lesion by commencing early therapy.

Assessing audiological, pathophysiological and psychological variables in tinnitus patients with or without hearing loss.

The aim of this work is to study the characteristics of tinnitus both in normal hearing subjects and in patients with hearing loss. The study considered tinnitus sufferers, ranging from 21 to 83 years of age, who were referred to the Audiology Section of Palermo University in the years 2006-2008. The following parameters were considered: age, sex, hearing threshold, tinnitus laterality, tinnitus duration, tinnitus measurements and subjective disturbance caused by tinnitus. The sample was divided into Group1 (G1), 115 subjects with normal hearing, and Group2 (G2), 197 subjects with hearing loss. Especially for G2, there was a predominance of males compared to females (P = 0.011); the highest percentage of tinnitus resulted in the decades 61-70 and >70 with a significant difference for G2 demonstrating that the hearing status and the elderly represent the principal tinnitus-related factors (P < 0.0001). The hearing impairment resulted in most cases of sensorineural hearing loss (SNHL) type and was limited to the high frequencies; the 72.1% of the patients with SNHL had a high-pitched tinnitus, while the 88.4% of the patients with a high-frequency SNHL had a high-pitched tinnitus (P < 0.0001). As to the subjective discomfort, the catastrophic category was the most representative among G1 with a significant difference between the two groups; no correlation was found between the level of tinnitus intensity and the tinnitus annoyance confirming the possibility that tinnitus discomfort is elicited by a certain degree of psychological distress as anxiety, depression, irritability and phobias.

The point prevalence of otitis media with effusion among primary school children in Western Sicily.

The objective of this study is to identify the prevalence of otitis media with effusion (OME) in primary school children and to value the possible predisposing factors focusing on relationship between allergy and OME in Western Sicily. 2,097 children attending primary school were screened from September 2006 to June 2007 in Sciacca. Children underwent pneumatic otoscopy, skin tests, tympanogram and acoustic reflex tests. Audiogram was performed if the child had a type B or a type C tympanogram. The criteria for diagnosis of OME were: documented persistent middle ear effusion by otoscopic examination for a minimum of 3 months, presence of B or C tympanogram, absence of ipsilateral acoustic reflex and a conductive hearing loss greater than 25 dB at any one of the frequencies from 250 Hz to 4 kHz. OME was identified in 143 children, in 61 of whom OME was unilateral and in 82 of whom it was bilateral. The overall prevalence of OME was 6.8%, with a maximum prevalence of 12.9% between 5 and 6 years of age. By increasing age, the prevalence of OME decreased. Also, we found a higher prevalence rate of OME in children with positive skin tests (62.9%) than those with negative skin tests (37.1%). The present study evidences the high social impact of OME, whose prevalence is directly correlated to age and atopy. Moreover, our finding supports the literature data that climatic and environmental factors may also have a role in the occurrence of OME.

Speech perception outcomes after cochlear implantation in prelingually deaf infants: the Western Sicily experience.

OBJECTIVES: To describe audiometric characteristics and speech perception performances of prelingually deaf Sicilian children after cochlear implantation; to identify the influence of cochlear implant (CI) user and family's characteristics on speech recognition and intelligibility outcomes. METHODS: Twenty-eight infants with a congenital or acquired hearing impairment and implanted before the 3rd year of life were studied; all children suffered from bilateral sensorineural hearing loss (SNHL) with evidence of lack of hearing aids benefit and no evidence of intellectual disability. The study of the main characteristics associated with CI user and family's profile was performed with a clinical assessment including pre-implant and post-implant (1, 3, 6, 12 and 18 months) behavioural audiometry (evaluating average threshold for the frequencies 0.5, 1, 2 and 4KHz) and speech recognition tests (IT-MAIS, MUSS, CAP and SIR). RESULTS: Our cohort was characterized by an early diagnosis of SNHL (5.77 and 12.17 months for congenital and acquired HL respectively), a short length of deafness (average=6.78 months) and an implantation before the 3rd year of life (mean=24.25 months; range from 10 to 36). Analysis of audiometric threshold revealed a significantly improved capacity to detect sounds within the conversational speech spectrum after 12 months from implantation (r=0.99; p<0.001). The main speech recognition test evidenced speech perception and speech intelligibility performances (CAP median value of 3; SIR category=3 in 46.42%) equal to those children with same characteristics reported by literature. With the exception of 'daily CI use' (p<0.001), none of the variables associated with CI user and family's profile resulted significant predictor of speech perception improvement. CONCLUSIONS: This work demonstrates that all children of our cohort, with an early diagnosis of SNHL and a CI surgery performed before the 3rd year of life, presented a progressive audiometric and speech improvement through the first 12-18 months after cochlear implantation. The study also highlights that, differently from the others variables studied, a continuous CI use influences significantly speech perception and recognition outcomes.

Prelingual sensorineural hearing loss and infants at risk: Western Sicily report.

OBJECTIVE: To evaluate independent etiologic factor associated with sensorineural hearing loss (SNHL) in newborn at risk; to study the role of their interaction especially in NICU infants who present often multiple risk factors for SNHL. METHODS: The main risk factors for SNHL reported by JCIH 2007 were evaluated on 508 infant at risk ranging from 4 to 20 weeks of life, transferred to the Audiology Department of Palermo from the main births centers of Western Sicily. After a global audiological assessment, performed with TEOAE, tympanometry and ABR, the prevalence and the effect of risk factors was statistically studied through univariate and multivariate analysis on the total population (normal and deaf subjects). RESULTS: Fifty-one infants (10.03%) were diagnosed with SNHL (45 bilateral and 6 monolateral) with a mean hearing threshold of 87.39 ± 28.25 dB HL; from logistic regression analysis family history of hearing impairment (HI) and TORCH infections resulted independent significant risk factors (P<0.00001 and P=0.024 respectively). High SNHL percentages were evidenced also in NICU babies, due to the various pathologies and risk factors presented by these infants, and among newborns who suffered from hyperbilirubinemia requiring exchange transfusion (11.97% and 9.52% respectively). Craniofacial abnormalities (CFA) and syndromes associated to HI showed an important relationship (P<0.00001) with conductive hearing loss (CHL). Multiple regression analysis of the variation in SNHL among NICU infants evidenced an increased risk for SNHL of 21.24% and of 19.33% respectively in preterm infants and in case of hyperbilirubinemia if respiratory distress is concomitant with these risk factors. It was also observed an higher risk of SNHL (99.66%) in case of coexistence of prematurity and hyperbilirubinemia. Finally among infants with very low birth weight (VLBW) it was evidenced a statistically difference between the mean weight of SNHL infants respect to NHL newborns (P=0.048). CONCLUSION: The high SNHL prevalence (10.03%) in our cohort underlines how infants at risk are more susceptible to suffer from SNHL; in particular NICU newborns have a 33% greater chance of developing SNHL, because of the presence of multiple risk factors (or=1.33) and their interaction. As the number of coexisting risk factors increases, the prevalence of SNHL also increases (r(2)=0.93).

On the threshold of effective well infant nursery hearing screening in Western Sicily.

OBJECTIVE: To determine the feasibility and effectiveness of well-infant nursery hearing screening programme for the early identification of hearing impairment, based on transient evoked otoacoustic emission (TEOAE) with a high "screen sensitivity" reducing the number of more expensive secondary level exams. METHODS: The newborns were screened by non-specialist health workers in well babies nursery at the twentieth day of life for 6 years consecutive. Based on PASS/FAIL criteria and presence/absence of audiological risk factors the newborns were divided into four groups each one with its personal step programme: G1 - PASS without risk factor, free to go home; G2 - PASS with risk factor, retest at the age of 7 months; G3 - FAIL without risk factor, re-screening after 2 weeks for a maximum of four times before audiology assessment; G4 - FAIL with risk factor, retest after 2 weeks. RESULTS: The coverage rate increased progressively from 89.8% to 92%. The referral rate was 1.51% after second stage with a specificity value of 98.78%. The four-stage screening performed for G3 reduced the numbers of global audiology assessment to 0.91% with a final global specificity of 99.4 ± 0.4%. CONCLUSION: Less than 1% of infants underwent audiological assessment; the false positives resulted 0.62% with hearing loss global incidence of 2.95/1000 and a mean age of confirmation of 3.5 months of age. It is reasonable to think that this screening programme could be implement to overall 42 Western Sicily birth centres within few years.

Audiologic profile of infants at risk: experience of a Western Sicily tertiary care centre.

OBJECTIVE: To identify the incidence of sensorineural hearing loss (SNHL) on infant at risk and to classify the degree and type of hearing loss describing the main causes associated in Western Sicily. To compare single TEOAE and combined TEOAE/ABR techniques studying the referral rate, the false-positive and false-negative rates through concordance test (κ coefficient), sensitivity (TPR) and specificity (TNR) for each protocol. METHODS: From January 2010 to June 2011, 412 infants at risk, ranging from 4 to 20 weeks of life, transferred to Audiology Department of Palermo from the births centers of Western Sicily, underwent to audiological assessment with TEOAE, tympanometry and ABR. The following risk factors were studied: family history of SNHL, consanguinity, low birth weight, prematurity, cranio-facial abnormality and syndromes associated to SNHL, respiratory distress, intensive care in excess of 5 days (NICU), pregnant maternal diseases, perinatal sepsis or meningitis, hyperbilirubinemia, ototoxic drugs administration. RESULTS: Forty-seven infants (11.41%) were diagnosed with SNHL; median corrected age at final audiological diagnosis was 12 weeks. SNHL resulted moderate in 44.68%, severe in 10.64% and profound in 21 cases with a significant difference in family history and NICU infants (p<0.0001). As the number of coexisting risk factors increases, the percentage value of SNHL in infants (χ(2)=12.31, p=0.01, r(2)=0.98) and the degree of hearing loss (χ(2)=13.40, p=0.0095, r=0.92) also increase. The study of single TEOAE and combined TEOAE/ABR showed a statistical difference (χ(2)=14.89, p<0.001) with a low concordance value (κ=0.87) confirming the importance of combined techniques for NICU group (κ=0.86) where four cases (0.97%) of auditory neuropathy were diagnosed. CONCLUSION: This study demonstrates the necessity to implement a neonatal hearing screening program in Western Sicily because of the high percentage of SNHL in infants at risk. Family history of HL is an independent significant risk factor for SNHL easily diagnosed through single TEOAE technique. Combined TEOAE/ABR is the gold standard for NICU babies which are at risk for auditory neuropathy. Coexisting risk factors are an additional risk factor for HL.

Characteristics of tinnitus with or without hearing loss: clinical observations in Sicilian tinnitus patients.

OBJECTIVE: To analyze the clinical characteristics of tinnitus both in normal hearing subjects and in patients with hearing loss. METHODS: The study considered 312 tinnitus sufferers, 176 males and 136 females, ranging from 21 to 83 years of age, who were referred to the Audiology Section of the Department of Bio-technology of Palermo University. The following parameters were considered: age, sex, hearing threshold, tinnitus laterality, tinnitus duration, tinnitus measurements and subjective disturbance caused by tinnitus. The sample was divided into two groups: Group 1 (G1) subjects with normal hearing; Group 2 (G2) subjects with hearing loss. RESULTS: Among the patients considered, 115 have normal hearing while 197 have a hearing deficit. There is a slight predominance of males respect to females that is more evidenced in G2 (61.42% of males vs. 38.58% of females). The highest percentage of tinnitus results in the decades 41-50 for G1 and >70 for G2 with a statistically significant difference between the two groups (P<0.0001). The hearing impairment results in most cases of sensorineural type (74.62%) and limited to the high frequencies (58.50%), moreover the 72.10% of the patients with SNHL had a high-pitched tinnitus while the 88.37% of the patients high-frequency sensorineural hearing loss had a high-pitched tinnitus (P<0.0001). As for the subjective discomfort, the catastrophic category resulted most representative among subjects with normal hearing with a statistically significant difference between the two groups but no significant correlation was found between the level of tinnitus intensity and the tinnitus annoyance confirming the possibility that tinnitus discomfort is elicited by a certain degree of psychological distress as anxiety, depression, irritability and phobias that do not allow the phenomenon of the 'habituation'. CONCLUSION: This work, according to literature data, suggests that the hearing status and the elderly represent the principal tinnitus related factors; moreover tinnitus characteristics differ in the two groups for tinnitus pitch. There is, in fact, a statistically significant association between high-pitched tinnitus and high-frequency SNHL suggesting that the auditory pathway reorganization induced by hearing loss could be one of the main source of the tinnitus sensation.

Investigation of tinnitus patients in Italy: clinical and audiological characteristics.

Objective. 312 tinnitus sufferers were studied in order to analyze: the clinical characteristics of tinnitus; the presence of tinnitus-age correlation and tinnitus-hearing loss correlation; the impact of tinnitus on subjects' life and where possible the etiological/predisposing factors of tinnitus. Results. There is a slight predominance of males. The highest percentage of tinnitus results in the decades 61-70. Of the tinnitus sufferers, 197 (63.14%) have a hearing deficit (light hearing loss in 37.18% of cases). The hearing impairment results of sensorineural type in 74.62% and limited to the high frequencies in 58.50%. The tinnitus is referred as unilateral in 59.93%, a pure tone in 66.99% and 10 dB above the hearing threshold in 37.7%. It is limited to high frequencies in 72.10% of the patients with sensorineural hearing loss (SNHL) while the 88.37% of the patients with high-frequency SNHL have a high-pitched tinnitus (chi(2) = 66.26;P < .005). Conclusion. Hearing status and age represent the principal tinnitus related factors; there is a statistically significant association between high-pitched tinnitus and high-frequency SNHL. There is no significant correlation between tinnitus severity and tinnitus loudness confirming the possibility that neural connection involved in evoking tinnitus-related negative reactions are governed by conditioned reflexes.