Risk Of Sensorineural Hearing Loss Research Articles

Treatment for hypotension in the first 24 postnatal hours and the risk of hearing loss among extremely low birth weight infants.

Objective:To evaluate whether treated hypotension in the first 24 postnatal hours is associated with hearing loss in extremely low birth weight (ELBW) infants.Study Design:In a cohort of 735 ELBW infants, we identified 25 with sensorineural hearing loss at 12–24 months adjusted age. For each case we selected 3 controls with normal hearing. Logistic regression models were used to adjust for confounding variables.Results:60% of cases and 25% of controls were treated for hypotension. After adjusting for confounding variables (gestational age, antenatal glucocorticoids, 5 minute Apgar < 6, insertion of an umbilical catheter, treatment with high frequency ventilation, and major cranial ultrasound abnormality), treated hypotension was associated with an increased risk of sensorineural hearing loss (adjusted odds ratio: 3.6; 95% confidence interval: 1.3–9.7).Conclusions:Treated hypotension in ELBW infants in the first 24 hours of life is associated with an increased risk of sensorineural hearing loss.

Hearing outcomes in children of diabetic pregnancies

ObjectiveChildren of diabetic pregnancies (CDPs) face numerous risk factors for hearing loss (HL). The objective of this study was to investigate the hearing outcomes of CDPs on a population scale. MethodsUsing the Audiological and Genetic Database, the prevalence, severity, and progression of HL in CDPs was compared against children of non-diabetic pregnancies (CNDPs) who served as controls. ResultsAmong 311 CDPs, 71.1% demonstrated evidence of HL compared to 45.5% in CNDPs (p < 0.001). The mean age at which CDPs received audiograms was 3.6 years compared to 5.4 years for CNDPs (p < 0.001). Compared to CNDPs, CDPs were similarly affected by common otologic conditions such as acute otitis media (25.7%), chronic otitis media (38.3%), and Eustachian tube dysfunction (41.8%) (all p > 0.05). CDPs were more likely to have bilateral HL (81%) and sensorineural hearing loss (SNHL) (8%) relative to CNDPs (p < 0.001 and p = 0.004, respectively). Rates of conductive HL and mixed HL were not significantly different between groups (p = 0.952 and p = 0.058, respectively). CDPs were at significant risk for the development of HL (aOR 1.66 [1.28–2.17], SNHL (aOR 1.63 [1.01–2.52], and high-frequency HL (aOR 1.32 [1.03–1.68]). Of the comorbidities evaluated, CDPs with hyperbilirubinemia (aOR 1.85 [1.18–2.84]), perinatal asphyxia (aOR 1.90 [1.06–3.16]), or congenital heart disease (aOR 1.21 [1.07–1.37]) demonstrated higher risk of SNHL. ConclusionChildren of diabetic pregnancies face increased risks of developing HL, particularly bilateral and sensorineural hearing loss. Given these findings, we recommend close audiologic follow-up for these children, especially those with complicated birth histories or additional medical problems.

A Hearing Screening Pilot Study With Students With Significant Cognitive Disabilities.

Purpose Many children with severe intellectual and developmental disabilities are at a higher risk for hearing loss than their peers who are typically developing. Unfortunately, they do not consistently participate in routine school-based hearing screenings. The current study investigated the feasibility of increasing their participation using an otoacoustic emissions protocol and documented results relative to student educational profiles. Method A total of 43 students with significant cognitive disabilities enrolled at a public school exclusively serving this population participated in the study. All but 9, who were excluded because of known hearing loss, were screened by a licensed audiologist assisted by audiology doctoral students. The protocol included otoscopy, tympanometry, distortion product otoacoustic emissions (DPOAEs), and teacher surveys. Results DPOAE screening was attempted on 33 students without previously diagnosed hearing loss and successfully completed for 26 (78.8%). Two students (4.6%) with absent otoacoustic emissions and normal tympanograms were referred for further assessment due to concerns about possible sensorineural hearing loss in one or both ears, and 10 (23.3%) had abnormal tympanograms in one or both ears. Conclusions Considering the high risk of sensorineural hearing loss for children with significant disabilities, it is important for them to be included in school hearing screenings. The results of this study demonstrate the feasibility of using DPOAEs for school-based hearing screenings with this population with an interprofessional team of licensed audiologists, educators, and speech-language pathologists. The results further suggest that students with significant disabilities and hearing loss may be unidentified and underserved. Given the complex needs of this population, an interprofessional practice model for hearing screenings and intervention services is recommended.

Effect of sensorineural hearing loss on neurocognitive and adaptive functioning in survivors of pediatric embryonal brain tumor.

Survivors of pediatric embryonal brain tumors (BT) are at high risk for sensorineural hearing loss (SNHL) associated with neurocognitive decline. However, previous studies have not assessed the relationship between SNHL and adaptive functioning. We examined neurocognitive and adaptive functioning in patients with and without SNHL. Participants included 36 patients treated for an embryonal BT with craniospinal irradiation (CSI) and cisplatin chemotherapy who were assessed 6.7years post-treatment on average. The impact of SNHL on neurocognitive performance and parent-rated adaptive functioning was assessed in univariate and multivariate analyses. There were 17 cases with SNHL (mean age at evaluation = 14.4) and 19 cases with NH (mean age at evaluation = 13.8). After accounting for age at diagnosis and additional covariates in multivariable analyses, SNHL was associated with worse overall intellectual functioning (p = 0.027) and perceptual reasoning (p = 0.016) performance. There was no effect of SNHL on adaptive functioning in multivariable models. Age at diagnosis and sex were associated with performance on neurocognitive measures. SNHL in pediatric embryonal BT is associated with increased risk for neurocognitive deficits in conjunction with other demographic and treatment-related factors.

Does Mucosal Chronic Otitis Media Leads to Sensorineural Hearing Loss.

Sensori-neural hearing loss (SNHL) results from inner ear damage or injury to the neural pathways that relay signals from the inner ear to the brain. A serious sequelae of COM is damage to the inner ear. This study aimed at finding the occurrence of SNHL in unilateral mucosal COM. One Hundred patients who had unilateral mucosal COM were enrolled in the study and underwent pure tone audiometry using Audio 4002 system in acoustically treated room. Bone conduction (BC) threshold less than 20dB was taken as normal. Significant SNHL of 23% was seen in mucosal COM. Unilateral mucosal COM leads to significant impact on BC thresholds. The risk of SNHL increases with larger perforation size and increasing duration and active stage of disease.

HUBUNGAN HIPERTENSI DENGAN GANGGUAN PENDENGARAN SENSORINEURAL PADA PASIEN RAWAT JALAN DI POLIKLINIK TELINGA HIDUNG TENGGOROKAN DI RSUD PROVINSI NTB TAHUN 2014-2017

Hearing loss may be conductive hearing loss and sensorineural hearing loss. All cases of hearing loss, 90% are sensorineural hearing loss. The most risk of sensorineural hearing loss are the intensity of noise, age, and history of hypertension disease. Studies show that hypertension causes sensorineural hearing loss. this study is an analytical observational with cross sectional study plan. The sample was covering 96 respondents, consisting of 48 sensorineural hearing loss patients and 48 non-sensorineural hearing loss patients. The study was done using patient medical records at the Ear-Nose-Throat Policlinic of NTB province hospital from 2014 to 2017. Data analysis was performed by contingency coefficient corellation test. The analysis result was significant value p = 0,000 &lt; p (0,05) which showed their significance or correlation of the conducted study. There is a significant correlation between hypertension and sensorineural hearing loss.

Sensorineural Hearing Loss in Newborns Hospitalized in Neonatal Intensive Care Unit: An Observational Study.

Children hospitalized in Neonatal Intensive Care Units (NICU) present an increased risk for Sensorineural Hearing Loss (SNHL) due to prematurity, hypoxia-ischemia, hyperventilation, low birth weight and the use of ototoxic drugs. The aim of this study was to assess the prevalence of SNHL in newborns hospitalized in a NICU using Transient Evoked Otoacoustic Emissions (TEOAE) and Automated Auditory Brainstem Responses (A-ABR) and analyze the associated risk factors. A sample of 153 newborns hospitalized in NICU underwent TEOAE, A-ABR and clinical ABR to evaluate the presence of hearing deficits. Prevalence of SNHL was calculated and odds ratio for specific risk factors was measured. One-hundred fifteen babies (86.7%) presented normal hearing at TEOAE and A-ABR. Fifteen children had a REFER response at TEOAE and a PASS response at A-ABR. Twenty-five children (16.3%) had a REFER A-ABR and were addressed to clinical ABR. A diagnosis of SNHL was made in 12 (7.8%) newborns. An increased risk of SNHL was observed in preterm children <28 weeks (p=0.0135), in children with neurological disorders (p=0.02), that underwent surgery (p=0.0002), affected from premature retinopathy (p=0.0006), craniofacial malformation (p=0.007) and that had sepsis (p=0.04). Additional risk factors for SNHL in our sample were a maternal disease during pregnancy (p=0.0011), cesarean delivery (p<0.0001) and a twin pregnancy (p<0.0001). SNHL in newborns is correlated with hospitalization in NICU. An accurate hearing screening associated to a rigorous clinical medical collection of data is necessary to promptly identify cases of SNHL in children with a special attention to those hospitalized in NICU and plan proper intervention.

Prevalence of Sensorineural Hearing Loss in Patients with Congenital Hypothyroidism in Qazvin, Iran (2015)

Background: Congenital hypothyroidism increases the risk of sensorineural hearing loss (SNHL). Children with hearing impairment are prone to communication disorders. The present study aimed to determine the prevalence of SNHL in patients with congenital hypothyroidism in Qazvin, Iran. Methods: This cross-sectional study was conducted on children with transient or permanent congenital hypothyroidism in Qazvin province, Iran in 2015. The patients were identified through national neonatal thyroid screening during 2006-2011. Hearing loss was assessed using the auditory brainstem response (ABR). A minimum of three years of follow-up was carried out to determine the permanency of congenital hypothyroidism. Data analysis was performed using t-test and Chi-square. Results: In total, 155 children with congenital hypothyroidism were enrolled in the study, including 67 females (43.2%) and 151 cases of primary congenital hypothyroidism. Abnormal ABR was observed in 10 patients (6.45%) (6.6% in permanent cases and 6.1% in transient cases), seven of whom were female. In addition, eight patients with primary congenital hypothyroidism and two patients with central congenital hypothyroidism had abnormal ABR (P=0.165). Mean primary thyroid-stimulating hormone levels (52.45±39.91 versus 38.23±28.03 IU/l; P=0.355) and T4 (6.07±4.33 versus 6.98±3.40 µg/dl; P=0.307) had no significant differences in the children with SNHL and other patients. At the beginning of the treatment, mean age of the children with SNHL was 28.50±22.13 days, while it was 28.87±30.34 days in the other subjects (P=0.909). Conclusion: According to the results, the prevalence of SNHL was lower in the patients with congenital hypothyroidism compared to the reported rates in other countries. However, the assessment of hearing loss should be prioritized in the infants with congenital hypothyroidism.

Azithromycin and Sensorineural Hearing Loss in Adults: A Retrospective Cohort Study.

To examine whether short-term use of azithromycin increases the risk of sensorineural hearing loss (SNHL) in adults with uncomplicated infections. A retrospective cohort study using Medicaid claims data, 1999 to 2010. Adults (18-64 years old) who had continuous enrollment in Medicaid for the 12 months (baseline) before the date of first dispensation (index date) of oral azithromycin or amoxicillin ± clavulanate for uncomplicated infections. We operationalized sensorineural hearing loss (SNHL) by a charge for audiometry and followed by a diagnosis of SNHL within 30 days. We adjusted for the baseline covariates through propensity score matching. Adults were followed for up to 120 days after the index date. The hazard of SNHL in azithromycin-exposed adults was compared with those who had amoxicillin ± clavulanate using a Cox proportional hazard model. We performed several sensitivity analyses by varying the follow-up time, SNHL definition, adjusting for cumulative antibiotic use, and switching exposure status during the follow-up period. A total of 493,774 patients entered the study cohort. The unadjusted incidence rates of SNHL were 38 and 41 cases per 10,000 patient-years following exposure to azithromycin and amoxicillin ± clavulanate, respectively. The adjusted (matched) hazard ratio (HR) of SNHL for azithromycin versus amoxicillin ± clavulanate was 0.91 (95% confidence interval [CI], 0.77-1.07). The sensitivity analyses findings were consistent with the primary analysis. Azithromycin short-term use was not associated with an increased risk of SNHL in comparison to amoxicillin ± clavulanate.

Sudden Sensorineural Hearing Loss during Acute Otitis Media due to Cochlear Otosclerosis

Purpose: The risk of sensorineural hearing loss (SNHL) as an acute otitis media (AOM) complication is a well-recognized condition. Here, we present a sudden sensorineural hearing loss due to acute otitis media in a patient with cochlear otosclerosis (COS). We hypothesize that the demineralization of otosclerosis around cochlea may have constituted a weak spot and facilitated the crossing of noxious substances into the inner ear. Although many possible means of connection between the middle ear and inner ear has been described, an increased risk of SNHL as an otitis complication at COS patients has not been mentioned in the literature. In order to decrease this risk, preventive pneumococcal vaccination should be offered to COS patients and in an event of AOM, a more aggressive treatment approach should be considered including early myringotomy together with antibiotics.

Impact of radiation technique, radiation fraction dose, and total cisplatin dose on hearing : Retrospective analysis of 29medulloblastoma patients.

To analyze the incidence and degree of sensorineural hearing loss (SNHL) resulting from different radiation techniques, fractionation dose, mean cochlear radiation dose (Dmean), and total cisplatin dose. In all, 29children with medulloblastoma (58ears) with subclinical pretreatment hearing thresholds participated. Radiotherapy (RT) and cisplatin had been applied sequentially according to the HIT MED Guidance. Audiological outcomes up to the latest follow-up (median 2.6years) were compared. Bilateral high-frequency SNHL was observed in 26patients (90%). No significant differences were found in mean hearing threshold between left and right ears at any frequency. Asignificantly better audiological outcome (p< 0.05) was found after tomotherapy at the 6 kHz bone-conduction threshold (BCT) and left-sided 8 kHz air-conduction threshold (ACT) than after acombined radiotherapy technique (CT). Fraction dose was not found to have any impact on the incidence, degree, and time-to-onset of SNHL. Patients treated with CT had agreater risk of SNHL at high frequencies than tomotherapy patients even though Dmean was similar. Increase in severity of SNHL was seen when the total cisplatin dose reached above 210 mg/m2, with the highest abnormal level found 8-12months after RT regardless of radiation technique or fraction dose. The cochlear radiation dose should be kept as low as possible in patients who receive simultaneous cisplatin-based chemotherapy. The risk of clinically relevant HL was shown when Dmean exceeds 45 Gy independent of radiation technique or radiation regime. Cisplatin ototoxicity was shown to have adose-dependent effect on bilateral SNHL, which was more pronounced in higher frequencies.

Biochemical alteration in children with idiopathic nephrotic syndrome associated with an increased risk of sensorineural hearing loss; additional insights in cochlear renal relationship

ObjectivesChildren with Idiopathic Nephrotic Syndrome (INS) are at risk of hearing loss due to the adverse impact of medications and related immunological and genetic factors on both cochlea and kidney. So this work was planned to evaluate hearing status in children with INS and to clarify the possible associated risk factors by interpreting the clinical and laboratory profiles of those children. MethodsNinety children with INS aged 5–14 years [30 patients with steroid-sensitive nephrotic syndrome (SSNS), 30 patients with steroid dependent/frequently relapsing nephrotic syndrome (SDNS/FRNS), and 30 patients with steroid-resistant nephrotic syndrome (SRNS)], and 90 age and sex matched normal controls were enrolled into this study. Laboratory measurements of serum calcium, creatinine, cholesterol, blood urea and other relevant investigations were done. Pure tone audiometry was done with the sensory-neural hearing loss (SNHL) diagnosed when the level bone conduction was >20 dB and the difference in air to the bone gap was <15 dB. Results40% children with INS had SNHL, mostly of mild degree HL and primarily occurred at the lower frequencies. A highly significant statistical difference between controls and various types of nephrotic syndrome regarding pure tone audiometry measurements at frequencies 250, 500, 1000 Hz, whereas insignificant difference interpreting pure tone audiometry measurements in 2000, 4000 and 8000 Hz. ConclusionsChildren with different phenotypes of nephrotic syndrome are at risk of sensorineural hearing impairment. The hazards associated with this impairment were higher blood pressure, hypercholesterolemia, hypoalbuminemia, and hypocalcemia.

A cross sectional study to assess effects of mastoid drill on hearing levels in chronic suppurative otitis media cases in a tertiary care hospital in northern Maharashtra

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; Chronic suppurative otitis media (CSOM) is most common middle ear disease that is encountered in daily practice. It is accepted that middle ear surgery carries a small risk of sensorineural hearing loss. The present study was done to assess the effects of mastoid drilling on hearing loss in operating ear and contralateral ear in cases of CSOM.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; 80 patients with CSOM (with or without complications) age group 15 - 60 years attended the ENT outpatient department of Government Medical College and Hospital after taking consent were selected for the study using universal sampling technique, between August 2014 to August 2016. &lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; The study was conducted on 80 patients, aged between 15 to 60 years, who underwent ear surgery for CSOM at Government Medical College Hospital, from August 2014 to August 2016. We found no postoperative SNHL in 28 patients, while 52 patients (48 patients with temporary SNHL &amp;amp; 4 patients with permanent SNHL) had suffered from development of mild SNHL after middle ear surgery.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; Mastoid drilling used during ear surgery can cause mild sensorineural hearing loss in immediate postoperative period in operated ear. &lt;/p&gt;

Hearing Loss in Children With Asymptomatic Congenital Cytomegalovirus Infection.

To assess the prevalence, characteristics, and risk of sensorineural hearing loss (SNHL) in children with congenital cytomegalovirus infection identified through hospital-based newborn screening who were asymptomatic at birth compared with uninfected children. We included 92 case-patients and 51 controls assessed by using auditory brainstem response and behavioral audiometry. We used Kaplan-Meier survival analysis to estimate the prevalence of SNHL, defined as ≥25 dB hearing level at any frequency and Cox proportional hazards regression analyses to compare SNHL risk between groups. At age 18 years, SNHL prevalence was 25% (95% confidence interval [CI]: 17%-36%) among case-patients and 8% (95% CI: 3%-22%) in controls (hazard ratio [HR]: 4.0; 95% CI: 1.2-14.5; P = .02). Among children without SNHL by age 5 years, the risk of delayed-onset SNHL was not significantly greater for case-patients than for controls (HR: 1.6; 95% CI: 0.4-6.1; P = .5). Among case-patients, the risk of delayed-onset SNHL was significantly greater among those with unilateral congenital/early-onset hearing loss than those without (HR: 6.9; 95% CI: 2.5-19.1; P < .01). The prevalence of severe to profound bilateral SNHL among case-patients was 2% (95% CI: 1%-9%). Delayed-onset and progression of SNHL among children with asymptomatic congenital cytomegalovirus infection continued to occur throughout adolescence. However, the risk of developing SNHL after age 5 years among case-patients was not different than in uninfected children. Overall, 2% of case-patients developed SNHL that was severe enough for them to be candidates for cochlear implantation.

Ers of risk of sensorineural hearing loss development

The complex analysis of lipid metabolism in patients with sensorineural hearing loss (SNHL) has shown that the auditory dysfunction is associated with the increase of the lipid profile atherogenicity. However, there were no significant differences in conventional lipid metabolism parameters (total cholesterol, triglycerides, high density lipoprotein cholesterol (HDL-C)) between the patients with SNHL and the control group subjects. The higher values of apolipoprotein (apo) B/apoA-I ratio, atherogenic index of plasma (AIP) and atherogenic index (ATH index) in patients with SNHL indicate the increase of lipid profile atherogenicity. The atherogenic index (ATH index) has high predictive power of SNHL risk.

Enlarged vestibular aqueducts and other inner-ear abnormalities in patients with Down syndrome.

Histopathological anomalies of inner-ear structures in individuals with Down syndrome have been well documented; however, few studies have examined the radiological features. A retrospective study was conducted of temporal bone computed tomography images in 38 individuals (75 ears) with Down syndrome to evaluate the prevalence of inner-ear abnormalities and assess vestibular aqueduct widths. Inner-ear anomalies were identified in 20 of the 38 individuals (52.6 per cent). Seven of the 75 temporal bones (9.3 per cent) were found to have higher than previously reported. A dilated internal auditory canal and vestibule were more common among the present study group, while prior studies have demonstrated internal auditory canal stenosis and decreased vestibule size. Down syndrome patients exhibit a high prevalence of dysplastic inner-ear features that confer substantial risk of sensorineural hearing loss. Computed tomography is a useful screening aid to detect inner-ear abnormalities, particularly enlarged vestibular aqueducts, which cause preventable sensorineural hearing loss in this population.

Risk of sensorineural hearing loss with macrolide antibiotics: A nested case-control study.

To determine the association between a diagnosis of sensorineural hearing loss (SNHL) and the prescription of a macrolide antibiotic. Retrospective nested case-control study. From the LifeLink (IMS, Danbury, CT) health claims database, we randomly selected a cohort of subjects 15 to 60 years old from 2006 to 2014. Cases were identified as patients diagnosed with SNHL, each matched by age and calendar time to 10 controls selected from the same cohort. All macrolide prescriptions (erythromycin, azithromycin, clarithromycin, and telithromycin) were identified, and statistical comparison of usage was compared between cases and controls. Amoxicillin and fluoroquinolone antibiotics were used as positive controls to further investigate confounding by infection. Albuterol was used as a negative control because this is a drug class not expected to be associated with SNHL or with a confounding condition potentially causing SNHL. From a cohort of 6,110,723 subjects, we identified 5,989 cases of SNHL and 59,890 corresponding controls. The rate ratio for one prescription of a macrolide was 1.36 (95% confidence inteval [CI]: 1.24-1.49) and for multiple prescriptions was 1.66 (95% CI: 1.42-1.94). Similar rate ratios were observed with multiple prescriptions of amoxicillin and fluoroquinolones. A significant association between SNHL and macrolide use was likely due to confounding by indication for antibiotic treatment because the risk was also observed with fluoroquinolones and amoxicillin, antibiotics with no known ototoxic potential. Therefore, there does not appear to be an increased risk of SNHL in patients treated with macrolide antibiotics. 3b. Laryngoscope, 127:229-232, 2017.

Hearing Loss in Patients Who Received Cranial Radiation Therapy for Childhood Cancer.

Patients treated with cranial radiation therapy (RT) are at risk for sensorineural hearing loss (SNHL). Although SNHL is often characterized as a delayed consequence of anticancer therapy, longitudinal reports of SNHL in childhood cancer survivors treated with contemporary RT are limited. We report the incidence, onset, severity, and long-term trajectory of SNHL among children receiving RT. Potential risk factors for SNHL were also identified. Serial audiologic testing was conducted on 235 pediatric patients who were treated with conformal or intensity-modulated RT as part of an institutional phase II trial for localized primary brain tumors, including craniopharyngioma, ependymoma, and juvenile pilocytic astrocytoma. All but one patient had measurable cochlear radiation dose (CRD) greater than 0 Gy. The median follow-up from RT initiation to latest audiogram was 9 years with a median of 11 post-RT audiograms per patient. Audiograms were classified by the Chang Ototoxicity Grading Scale. Progression was defined by an increase in Chang grade from SNHL onset to the most recent evaluation. At last evaluation, SNHL was prevalent in 14% of patients: 2.1% had mild and 11.9% had significant SNHL requiring hearing aids. Median time from RT to SNHL onset was 3.6 years (range, 0.4 to 13.2 years). Among 29 patients with follow-up evaluations after SNHL onset, 65.5% experienced continued decline in hearing sensitivity in either ear and 34.5% had no change. Younger age at RT initiation (hazard ratio [HR], 2.32; 95% CI, 1.21 to 4.46), higher CRD (HR, 1.07; 95% CI, 1.03 to 1.11), and cerebrospinal fluid shunting (HR, 2.02; 95% CI, 1.07 to 3.78) were associated with SNHL. SNHL is a late effect of RT that likely worsens over time. Long-term audiologic follow-up for a minimum of 10 years post-RT is recommended.

Risk Factors for Hearing Loss in Patients with Cystic Fibrosis.

Patients with cystic fibrosis (CF) are at increased risk for sensorineural hearing loss (SNHL) due, at least in part, to the ototoxic side effects of routine CF therapies. However, the prevalence of SNHL and additional factors contributing to the development of SNHL are unknown. To identify risk factors associated with the development of SNHL in a large cohort of CF patients who had been referred for audiometric testing. A retrospective study of audiometric results and medication information in a cohort of patients with CF. Records of 178 CF patients seen at Boston Children's Hospital for audiometric testing from 2007 to 2010 were reviewed. Mean age of patients was 18 yr (standard deviation = 10 yr), and 98 (55%) of the patients were female. Audiometric results, medications, and hospitalizations were recorded. Multivariable logistic regression was used to evaluate the association between SNHL and the number of hospitalizations and chronic antibiotic use in the year prior to the patients' audiometry. In this sample, 37/178 (21%) patients had SNHL. Twenty-nine (78%) of the 37 patients had bilateral SNHL and 8 (22%) had unilateral SNHL. Across all age groups, the majority of patients had a bilateral hearing loss (HL). A multivariable model showed that older age and more frequent hospitalizations were associated with SNHL. The number of courses of chronic antibiotics in the year prior to audiometric testing was not correlated with rate of HL. This study suggests that age and frequency of hospitalizations are key predictors of HL development. Increased awareness and regular screening for SNHL should be included in the routine care of CF patients, particularly those at the highest risk.

Radioprotective Effect of Aminothiol PrC-210 on Irradiated Inner Ear of Guinea Pig

Radiotherapy of individuals suffering with head & neck or brain tumors subserve the risk of sensorineural hearing loss. Here, we evaluated the protective effect of Aminothiol PrC-210 (3-(methyl-amino)-2-((methylamino)methyl)propane-1-thiol) on the irradiated inner ear of guinea pigs. An intra-peritoneal or intra-tympanic dose of PrC-210 was administered prior to receiving a dose of gamma radiation (3000 cGy) to each ear. Auditory Brainstem Responses (ABRs) were recorded one week and two weeks after the radiation and compared with the sham animal group. ABR thresholds of guinea pigs that received an intra-peritoneal dose of PrC-210 were significantly better compared to the non-treated, control animals at one week post-radiation. Morphologic analysis of the inner ear revealed significant inflammation and degeneration of the spiral ganglion in the irradiated animals not treated with PrC-210. In contrast, when treated with PrC-210 the radiation effect and injury to the spiral ganglion was significantly alleviated. PrC-210 had no apparent cytotoxic effect in vivo and did not affect the morphology or count of cochlear hair cells. These findings suggest that aminothiol PrC-210 attenuated radiation-induced cochlea damage for at least one week and protected hearing.