Severity Of Sensorineural Hearing Loss Research Articles

Gram‐negative Bacteria are Associated With Sensorineural Hearing Loss in Chronic Otitis Media

ObjectChronic otitis media (COM) is an inflammatory disease that commonly presents with otorrhea and hearing loss. Bacteria‐induced inflammation can cause inner ear damage, leading to sensorineural hearing loss (SNHL). This study aimed to compare the prevalence and severity of SNHL in patients with gram‐negative versus gram‐positive cultures and examine associations between the concentrations of circulating monocytes and neutrophils with bacteria species and SNHL.MethodsThis was a retrospective study. Cholesteatoma or chronic suppurative otitis media patients with otorrhea were enrolled. Middle ear secretions were collected using sterile swabs under an otoscope, and sent for bacterial detection within 30 min. Pure tone audiometry and circulating leukocyte counts were recorded and analyzed in patients infected with different pathogens. Logistic regression analysis was used to identify the risk factors associated with SNHL.ResultsA total of 137 patients were enrolled, including 45 patients infected with gram‐negative bacteria, 41 with gram‐positive bacteria, 20 with polymicrobial infection, and 31 with no bacterial growth. Logistic regression analysis showed that bacterial culture positive infections (OR = 7.265, 95% CI 2.219–23.786, p = 0.001) were an independent risk factor for SNHL. Patients with gram‐negative bacteria had higher risks of SNHL (p < 0.0001) and more severe hearing loss (p = 0.005) than those with gram‐positive bacteria. COM patients infected with gram‐negative bacteria showed an increase in circulating monocytes, which correlated with the occurrence of SNHL (p = 0.0343).ConclusionGram‐negative bacteria are associated with elevated circulating monocyte counts and have a higher risk of severe SNHL.Level of Evidence4 Laryngoscope, 2024

Relationship of Serum Prestin Levels to the Severity of Sensorineural Hearing Loss.

Prestin is an outer hair cell (OHC) protein responsible for increasing cochlear sensitivity and has been proposed as a biomarker.We aimed to evaluate whether the serum prestin level is related to the severity of chronic sensorineural hearing loss (SNHL). Ninetysubjects were recruited from the patient base at Samarra public hospitals and clinics in Iraq from January to October of 2022. They were divided into three groups equally: agroup of healthy people without hearing loss (G0), a group with moderate SNHL (G1), and a group with severe SNHL (G2). The subjects ranged from 20 to 80 years of age and included 51 males and 39 females. Blood samples were collected, then serum was separated, and enzyme-linked immunosorbent assays were performed to quantify the levels of prestin. Hearing thresholds were sequentially statistically higher across the three groups. While prestin levels were significantly higher in G1 and G2 than that in G0, there were no differences between the G1 and G2 levels. Serum prestin levels were positively correlated with hearing thresholds in G1, but not G2. Our results suggest that in the clinical setting, prestin is sensitive to chronic mild to moderate SNHL (i.e., up to 40-60 dB), not more severe loss.This range is consistent with the added sensitivity provided by OHCs in the cochlea and provides support for prestin as a biomarker of OHC-mediated SNHL.

Hearing loss related outcomes post-lateral temporal bone resection and other oncologic treatment.

e18069 Background: Lateral temporal bone resection (LTBR) is used to treat malignancies originating from the auricle, periauricular skin, external auditory canal, parotid gland, or temporal bone. LTBR is the workhorse of otologic cancer surgery; however, few studies have described audiologic outcomes in a large sample of patients. The current literature focuses on overall survival rates post-surgery; however, hearing loss-related outcomes after surgery and adjuvant therapy are lacking in the literature. Following surgery, patients are expected to have maximal conductive hearing loss in the affected ear which can be augmented by ototoxic effects of adjuvant treatment such as radiation and systemic therapy. This type of hearing loss significantly impacts quality of life, making it difficult to understand speech directed to the surgically closed ear, localize sounds, and understand conversational speech in noisy environments. Less is known about the rate and severity of sensorineural hearing loss (SNHL) following LTBR. Methods: A retrospective chart review was conducted among 252 patients who underwent LTBR at single tertiary care center from 2010 to 2020. We evaluated changes in ipsilateral bone conduction (BC) hearing thresholds after LTBR ± adjuvant therapy, including head and neck radiation, chemotherapy, and immunotherapy. Patients who completed at least 1 preoperative and 1 postoperative audiogram within 2 years of surgery were included. Patients who had preoperative chemotherapy, immunotherapy, or temporal bone radiation were excluded. Of 252 patients reviewed, 94 met the eligibility criteria. Audiometric data included pure tone average (PTA) for BC thresholds. A significant decline in hearing was defined as an increase in PTA ≥ 10 decibels (American Academy of Otolaryngology-Head and Neck Surgery). Three treatment subgroups were analyzed, including LTBR only, LTBR and radiation therapy, and LTBR with radiation and systemic therapy. Results: Among all patients, BC PTA increased on average by 7dB after treatment (p<0.001); 30% of patients had ≥ 10dB shift. Patients undergoing LTBR with adjuvant therapy had a higher rate of hearing loss compared to patients undergoing LTBR alone (22/69, 32% vs. 5/25, 20%, respectively). Conclusions: LTBR can cause significant long-term decline in BC hearing that is worsened with the addition of adjuvant therapy. Therefore, it is critical to systematically obtain preoperative and post-treatment audiologic testing to monitor and rehabilitate hearing loss over time. The findings of this study may help patients understand realistic expectations of long-term treatment outcomes related to hearing loss, as an initial step to help improve quality of life with hearing rehabilitation such as osseointegrated bone conduction devices.

A Study of Relationship Between Serum Lipids and Sensorineural Hearing Loss.

The modern life style poses new challenges on the normal physiological mechanisms of the human body. Drug abuse, tobacco smoking, and alcohol drinking, as well as lack of exercise may also increase the risk of developing certain diseases, especially older age.To study the relationship between degree of sensorineural hearing loss and serum lipid level (total cholesterol, triglyceride, low density lipoproteins, high density lipoproteins).A cross-sectional study was conducted in 150 patients aged between 15 to 60years diagnosed with sensorineural hearing loss at Otorhinolaryngology outpatient department at Sir Sunder Lal hospital, IMS, BHU. All the 150 patients were amongst age group of 15 to 60years who were registered from August 2019 to July 2021.The serum levels of total cholesterol, triglyceride and low-density lipoprotein directly correlate with the severity of sensorineural hearing loss significantly (p < 0.05) whereas the serum levels of high-density lipoprotein had no direct co-relation with the severity of sensorineural hearing loss. Hyperlipidemic state is a major risk factor for SNHL. Regular screening and monitoring of serum lipid might prevent morbid SNHL and improve patients' quality of life in long term.

Retrospective Study of 157 Patients with Otosclerosis to Evaluate Association Between Carhart Notch on the Preoperative Bone-Conduction Audiogram and Postoperative Hearing and Balance Evaluated by the Vestibular Disorders Activities of Daily Living Scale.

BACKGROUND A Carhart notch in the pure tone audiogram can be an indicator of stapes fixation in otosclerosis. This retrospective study of 157 patients with otosclerosis aimed to evaluate the association between the presence of a Carhart notch on the preoperative bone-conduction audiogram and postoperative hearing and balance evaluated by the Vestibular Disorders Activities of Daily Living scale. MATERIAL AND METHODS Patients with suspected otosclerosis based on medical history and audiometric tests were considered. The analysis included 157 consecutive patients who underwent surgery in the years 2016 to 2019, in whom the diagnosis of otosclerosis was confirmed during surgery. Carhart notch was defined as an impairment in the bone conduction threshold of ≥7.5 dB for 2000 Hz frequencies above the mean thresholds at higher and lower adjacent frequencies. The Vestibular Disorders Activities of Daily Living subjective scale was used in the preoperative period and 4 and 12 months after surgery. RESULTS The preoperative presence of Carhart notch and progressive sensorineural hearing loss were statistically significantly correlated with more common onset of tinnitus and then dizziness (P=0.006). Preoperative vertigo was observed in patients who had Carhart notch observed in the preoperative audiometric test. This vertigo more commonly coexisted with profound sensorineural hearing loss and minor or no improvement in average values of bone conduction after surgery (P=0.002). CONCLUSIONS Preoperative Carhart notch on audiogram and the severity of sensorineural hearing loss were associated with tinnitus and vertigo. However, preoperative Carhart notch was not associated with persistent postoperative tinnitus in patients with cochlear otosclerosis.

Sensorineural hearing loss and ulcerative colitis in remission.

Sensorineural hearing loss (SNHL) has been reported in association with inflammatory bowel disease (IBD). However, SNHL as an extraintestinal manifestation of IBD is frequently underreported. In the present study, we compared the prevalence and severity of SNHL among patients with IBD-ulcerative colitis (IBD-UC) in remission with controls to find out any association between SNHL and IBD-UC in remission compared to controls. This single-center hospital-based prospective observational study included outdoor patients with IBD-UC in remission and healthy age- and sex-matched controls. Eligible patients and healthy participants were subjected to a battery of audiological tests (otoscopy, tympanometry and pure tone audiometry [PTN]) after thorough systemic and ear, nose and throat (ENT) examination. A total of 100 patients were enrolled in the study: 50 in IBD-UC in the remission group and 50 in the control group. None of the demographic variables (age, gender, residence and habits) were significantly different between the two groups. Otoscopy and tympanometry were normal in all patients and controls. The difference between the two groups in respect to frequency and severity of SNHL onPTA and in respect to unilateral and bilateral distribution of the hearing loss was not statistically significant. There is no statistically significant difference in frequency and severity of SNHL between patients with ulcerative colitis in remission and healthy age- and sex-matched controls.

Prevalence and Pattern of Hearing Loss among Head and Neck Cancer Patients Receiving Chemotherapy with or without Radiation Therapy at Ocean Road Cancer Institute, Tanzania

Background Head and neck cancers are primarily treated by three main modalities; surgery, radiotherapy and chemotherapy administered alone or in combination. Radiotherapy and chemotherapy may cause complications to patients including hearing loss with various outcomes on types and severity. Broad objective To determine the prevalence and pattern of hearing loss among head and neck cancer patients receiving chemotherapy with or without radiation therapy at Ocean Road Cancer Institute, Tanzania. Methodology   This was a hospital based descriptive cross sectional study which included 138 histopathological confirmed   head and neck cancer patients from June to December 2017. Data collected using structured questionnaires included demographic characteristics, site of the tumor, treatment modality, type of chemotherapy groups used, dosage and duration.  Pure tone audiometry at a frequency range of 250HZ to 8000HZ using Amplivox 270 audiometer was performed to all patients after otoscopy.  Data was analyzed using Statistical Package for Social Science version 20 and a p value of <0.05 was considered to be statistically significant.  Results Out of 138 patients who were involved in the study, 98(71%) had hearing loss. Among 98 patients who had hearing loss, 53(54.1%) received chemotherapy alone and all had sensorineural hearing loss, the remaining 45(45.9%) patients received concurrent chemoradiation and 40(88.9%) had sensorineural hearing loss. Conductive and mixed hearing loss were observed in 3 (6.7%) and 2 (4.4%) patients who received chemoradiation, respectively (p value = 0.045).  The severity of sensorineural hearing loss was higher among patients who received chemoradiation than those who received chemotherapy alone (p value =0.682).  Use of a single drug group or combined drug groups had no significant difference in terms of outcomes on severity of hearing loss (p value =0.603) but high cumulative doses of platinum and taxane compounds had significant increase in terms of outcomes on severity of hearing loss. (p value =0.003, 0.021 respectively). Conclusion and recommendation Hearing loss following chemotherapy alone or with concurrent chemoradiation therapy is quite prevalent in our settings thus hearing evaluation pre, during and post chemotherapy/radiotherapy is encouraged for earlier detection of hearing loss and establishing prompt intervention.

Prevalence of Sensorineural Hearing Loss in Pediatric Patients with Sickle Cell Disease: A Meta-analysis.

To determine the prevalence of Sensorineural Hearing Loss (SNHL) attributable to Sickle Cell Disease (SCD) in the global pediatric population and to identify factors contributing to its severity. Meta-analysis. We performed a comprehensive literature search for scientific articles in PubMed, Scopus, CINAHL, Web of Science, and the Cochrane Library that reported the incidence of hearing loss in populations under 18 years of age with excluding studies analyzing patients on iron chelation therapy, adults, or those without objective audiological analysis. We identified 138 initial studies with 17 selected for analysis after applying the exclusion criteria. A total of 1,282 SCD patients and 553 controls were included in the meta-analysis. There was a statistically significant increase in the prevalence of SNHL in children with SCD compared to the general population with a cumulative risk ratio of 3.33. This is the first systematic investigation of the relationship between SCD and SNHL in pediatric patients across the globe. The increased prevalence of SNHL in the pediatric SCD population warrants future research into the predictors of SNHL severity and merits routine audiometric monitoring of SCD patients to reduce the social and developmental morbidity of hearing loss at a young age. PROSPERO Registration #: CRD42019132601. Laryngoscope, 131:1147-1156, 2021.

Evaluation of ocular and cervical vestibular evoked myogenic potentials in patients with conductive and sensorineural hearing loss

Background Vestibular evoked myogenic potential (VEMP) test is an electrophysiological testing that can assess dysfunction of otolithic organs and the integrity of their pathways precisely. Aim Compare amplitude and latency of ocular and cervical vestibular evoked myogenic potentials evoked by air- and bone-conducted stimuli in three different groups. Materials and methods The control group (group 1) included ten healthy volunteers adults (20 ears) with normal hearing thresholds. Group 2 consisted of ten patients (20 ears) with conductive hearing loss (CHL) and group 3 comprised 10 patients (20 ears) with sensorineural hearing loss (SNHL). All participants underwent pure tone audiometry, immittancemetry ocular vestibular evoked myogenic potential (oVEMPs) and cervical vestibular evoked myogenic potential (cVEMPs) by air and bone conduction stimuli. Results The response rate of cVEMPs and oVEMPs elicited by both types of stimuli was 100% in control group and SNHL group. In the group of conductive hearing loss, air conduction(AC) (c VEMPs and o VEMPs) response rate was 60%. There was a statistically significant positive correlation between latencies of oVEMPs and cVEMPs evoked by AC and BC methods and degrees of hearing loss in SNHL group. Conclusion Ocular and cervical VEMPs elicited by bone conduction (BC) stimuli aren’t vulnerable to conductive hearing loss. Bc VEMPs provides assessment of vestibular system in patients with conductive hearing loss. Significant correlation was found between cVEMPs and oVEMPs abnormalities and severity of sensorineural hearing loss.

Characterization of Sensorineural Hearing Loss in Adult Patients With Sickle Cell Disease: A Systematic Review and Meta-analysis.

To determine the prevalence of sensorineural hearing loss (SNHL) attributable to sickle cell disease (SCD) in the global adult population and to identify factors contributing to its severity. Systematic Review and Meta-analysis. We performed a comprehensive literature search for scientific articles in PubMed, Scopus, CINAHL, Web of Science, and the Cochrane Library that reported the incidence of hearing loss in populations over 18 years of age with SCD. We identified 138 studies from the initial search, 12 of which met inclusion criteria and were utilized for data analysis. A total of 636 SCD patients and 360 controls were included in the Cochrane Review Manager 5.3 meta-analysis. There was a statistically significant increase in the prevalence of SNHL in adults with SCD compared with the general population with a cumulative risk ratio (RR) of 6.03. This is the first systematic investigation of the relationship between SCD and SNHL in adult patients across the globe. SNHL is more prevalent in patients with SCD, specifically those of the HbSS genotype, than the general population likely due to the pathophysiology of the disease and its effects on labyrinthine microvasculature. The increased prevalence of SNHL in the adult SCD population warrants future research into the predictors of SNHL severity and merits routine audiometric monitoring of adult SCD patients.

Progression of hearing loss and cochlear implantation in large vestibular aqueduct syndrome

ObjectivesLarge vestibular aqueduct syndrome (LVAS) is a congenital inner ear malformation that commonly results in progressive sensorineural hearing loss (SNHL) and cochlear implantation (CI). Though LVAS accounts for approximately 15% of pediatric SNHL, little is known regarding the rate and severity of SNHL in these patients. We sought to characterize the timing of SNHL progression to CI in patients with LVAS. MethodsWe performed a retrospective chart review at our institution from 2000 to 2018 using ICD-10 “large vestibular aqueduct syndrome,” and through identifying patients with CI who had LVAS. Demographic, surgical, and audiometric data were collected. Theoretical CI candidacy was approximated using a pure tone average (PTA) HL threshold of 70 dB. ResultsOf 103 patients, 96 had bilateral LVAS, and 7 had unilateral LVAS. Forty-one patients had bilateral implants, 52 had unilateral implants, and 10 were not implanted. The mean age at first implant was 8.62 years old [95%CI = 6.75,10.49], the mean age at second implant was 12.24 years old [95%CI = 8.33,16.15], and the mean time between implants was 4.37 years [95%CI = 3.02,5.73]. LVAS patients reached HL threshold of 70 dB at a mean age of 5.16 years old (SD = 3.04) for the “worse ear” and 9.08 years old (SD = 4.96) for the “better ear.” ConclusionsLVAS patients are a heterogenous population of patients, in which some may undergo progression of HL and some may not. Further, there may be a discrepancy in the timing between patients’ theoretical CI candidacy and when they undergo CI. In order to optimize timing of CI, individual monitoring and close observation of LVAS patients is recommended.

Isolated Internal Auditory Canal Diverticula: A Normal Anatomic Variant Not Associated with Sensorineural Hearing Loss.

Bony internal auditory canal diverticula are relatively common, occurring in approximately 5% of temporal bone CTs. Internal auditory canal diverticula have historically been considered incidental; however, a recent publication reported that internal auditory canal diverticula are associated with sensorineural hearing loss. The objective of this study was to further characterize this potential association in a large cohort of patients. A total of 1759 patients undergoing high-resolution temporal bone CT were collected during a 6-year interval, and audiometric data were obtained from those with internal auditory canal diverticula. To assess any association of isolated internal auditory canal diverticula with sensorineural hearing loss, we excluded from further analysis patients with concomitant otosclerosis and bilateral diverticula and those without audiometric data, leaving 22 index cases. Audiometric data for the ear with a diverticulum was compared with that in the contralateral ear, to serve as an internal control. Of 1759 patients, 82 (4.7%) had either unilateral (n = 33, 40%) or bilateral (n = 49, 60%) internal auditory canal diverticula. The co-incidence of otosclerosis and internal auditory canal diverticula was 34% (n = 28). There was no correlation between patient age and diverticulum size on either side. Among the index cases with isolated unilateral internal auditory canal diverticula and complete audiometric data, word recognition scores and the prevalence and severity of sensorineural hearing loss were not significantly different comparing the internal auditory canal diverticulum side to its contralateral control. This study did not find a statistically significant association between ears with internal auditory canal diverticula and worsening sensorineural hearing loss or word recognition. Internal auditory canal diverticula most likely represent a normal anatomic variant in ears without otosclerosis.

Prevalence of sensorineural hearing loss among type-II diabetes mellitus patients attending KLES Dr. Prabhakar Kore Hospital and MRC: A cross-sectional study

INTRODUCTION: Diabetes mellitus (DM) is an important healthcare concern in the modern world. This disease and its complications can affect virtually every system in the human body. A possible complication of DM is sensorineural hearing loss (SNHL). AIM: The current study aimed to evaluate the prevalence of SNHL among the patients of type-2 DM attending the hospital as well as to correlate the severity of hearing loss to the duration of diabetes and the glycosylated hemoglobin (HbA1c) levels. A total of 125 known cases of type-2 DM were included in the study. METHODOLOGY: For each patient, a detailed history was taken, thorough clinical ear, nose, and throat examination was done, hearing thresholds were evaluated by means of pure tone audiometry and HbA1c levels were measured. RESULTS: SNHL was seen in 76.8% of the patients. Robust correlation was seen between the severity of SNHL and the duration of DM (P CONCLUSION: This study confirms that there is a high prevalence of SNHL in type-2 DM.

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.

Analysis of archived newborn dried blood spots (DBS) identifies congenital cytomegalovirus as a major cause of unexplained pediatric sensorineural hearing loss

PurposeCongenital cytomegalovirus (cCMV) infection is the most common non-genetic cause of sensorineural hearing loss (SNHL). However, accurate diagnosis of cCMV as the etiology of SNHL is problematic beyond the neonatal period. This study therefore examined whether cCMV infection could be identified retrospectively in children presenting with unexplained SNHL to a multidisciplinary diagnostic outpatient otolaryngology clinic at an academic medical center in Minnesota. MethodsOver a 4-year period, 57 patients with an age range of 3months to 10years with unexplained SNHL were recruited to participate in this study. Informed consent was obtained to test the archived dried blood spots (DBS) of these patients for cCMV infection by real-time PCR, targeting a highly conserved region of the CMV UL83 gene. Results were normalized to recovery of an NRAS gene control. Chart review was conducted to identify subjects who underwent genetic testing and/or neurodiagnostic imaging to investigate possible genetic, syndromic, or anatomical causes of SNHL. ResultsIn total, 15 of the 57 children with unexplained SNHL tested positive for CMV DNA in their DBS (26%). A mean viral load of 8.3×104 (±4.1×104) [range, 1×103–6×105] copies/μg DNA was observed in subjects retrospectively diagnosed with cCMV. No statistically significant correlation was found between viral load and SNHL severity. ConclusionsA retrospective DBS analysis demonstrated that 26% of patients presenting with unexplained SNHL in childhood had cCMV. DBS testing is useful in the retrospective diagnosis of cCMV, and may provide definitive diagnostic information about the etiology of SNHL.

Is the cause of sensorineural hearing loss in patients with facial schwannomas multifactorial?

Facial nerve schwannomas (FNSs) and vestibular nerve schwannomas (VNSs) may occupy similar locations in the internal auditory canal (IAC). Risk factors for hearing loss in patients with FNS are unknown. Similar to VNSs, we hypothesize that the size and location of FNSs do not correlate with hearing loss. Retrospective cohort study and otopathology case review. A single institution retrospective review of all patients diagnosed with FNS was performed. Demographic data, tumor size and location, severity and type of hearing loss, and facial nerve function were analyzed. Otopathologic review of a case of FNS was also performed. Thirty-three patients with FNS were identified. Mean age was 44 years, and 61% (n = 21) were female. Nine patients had tumors involving the IAC facial nerve, whereas 24 had distal tumors without IAC involvement. Eleven patients (33%) had hearing loss, and 55% of patients with sensorineural hearing loss (SNHL) had tympanic segment tumors without IAC involvement. The presence of SNHL was not associated with IAC tumor location (P = 0.21). Maximal dimensions of FNSs were 3 mm to 42 mm, without correlation between size and SNHL severity (r = -0.017, P = 0.926). Otopathologic review of an untreated 5-mm IAC FNS demonstrated disproportionate spiral ganglion and hair cell loss within the ipsilateral cochlea compared to the contralateral ear. Tympanic segment FNS without IAC involvement may result in SNHL. Tumor size is not associated with the presence or severity of SNHL. Otopathologic review suggests degenerative cochlear changes in FNS. Mechanisms for SNHL in schwannomas are likely multifactorial and may be unrelated to cochlear nerve compression. 4. Laryngoscope, 127:1676-1682, 2017.

Aphasia and Auditory Processing after Stroke through an International Classification of Functioning, Disability and Health Lens.

Aphasia is an acquired language impairment affecting speaking, listening, reading, and writing. Aphasia occurs in about a third of patients who have ischemic stroke and significantly affects functional recovery and return to work. Stroke is more common in older individuals but also occurs in young adults and children. Because people experiencing a stroke are typically aged between 65 and 84 years, hearing loss is common and can potentially interfere with rehabilitation. There is some evidence for increased risk and greater severity of sensorineural hearing loss in the stroke population and hence it has been recommended that all people surviving a stroke should have a hearing test. Auditory processing difficulties have also been reported poststroke. The International Classification of Functioning, Disability and Health (ICF) can be used as a basis for describing the effect of aphasia, hearing loss, and auditory processing difficulties on activities and participation. Effects include reduced participation in activities outside the home such as work and recreation and difficulty engaging in social interaction and communicating needs. A case example of a young man (M) in his 30s who experienced a left-hemisphere ischemic stroke is presented. M has normal hearing sensitivity but has aphasia and auditory processing difficulties based on behavioral and cortical evoked potential measures. His principal goal is to return to work. Although auditory processing difficulties (and hearing loss) are acknowledged in the literature, clinical protocols typically do not specify routine assessment. The literature and the case example presented here suggest a need for further research in this area and a possible change in practice toward more routine assessment of auditory function post-stroke.

Assessment of hearing loss severity in patients with type 2 diabetes mellitus

Objective. The purpose of this study was to determine diabetes mellitus tip 2 influence on the incidence and severity of sensorineural hearing loss. Methods. The prospective case control study was done during 2013 among 132 patients, whose age range was 21-84 years, who were admitted to ENT department of the Health Center Kragujevac for evaluation of hearing loss. We performed detailed history, complete otorhinolaryngological examination including otoscopy, tympanometry and tonal liminal audiometry. All subjects were divided into two groups. The study group consisted of 67 patients with diabetes mellitus type 2 and the control group included age and gender matched subjects. We used tonal liminar audiometry for evaluation of the existence and the degree of hearing loss. The mean value obtained by tonal liminar audiometry at frequencies of 500, 1000, 2000 and 4000 Hz, greater than 25 dB was considered as a hearing loss. Data was statistically analyzed by using descriptive statistical methods, t-test and x2-test using standard statistical software package (SPSS for Windows, version 19.0). Results. There was sensorineural hearing loss in all patients with diabetes mellitus type 2. The frequency and degree of sensorineural hearing loss were statistically significantly higher in the group with diabetes mellitus patient compared to the control group (p < 0.05). Conclusion. The results indicate that diabetes mellitus type 2 patients often have significant hearing loss, which is very easy to establish by using tonal liminar audiometry, suggesting that these patients should be referred to the assessment of the impairment.

Nonmuscle Myosin Heavy Chain IIA Mutation Predicts Severity and Progression of Sensorineural Hearing Loss in Patients With MYH9-Related Disease.

MYH9-related disease (MYH9-RD) is an autosomal- dominant disorder deriving from mutations in MYH9, the gene for the nonmuscle myosin heavy chain (NMMHC)-IIA. MYH9-RD has a complex phenotype including congenital features, such as thrombocytopenia, and noncongenital manifestations, namely sensorineural hearing loss (SNHL), nephropathy, cataract, and liver abnormalities. The disease is caused by a limited number of mutations affecting different regions of the NMMHC-IIA protein. SNHL is the most frequent noncongenital manifestation of MYH9-RD. However, only scarce and anecdotal information is currently available about the clinical and audiometric features of SNHL of MYH9-RD subjects. The objective of this study was to investigate the severity and propensity for progression of SNHL in a large series of MYH9-RD patients in relation to the causative NMMHC-IIA mutations. This study included the consecutive patients diagnosed with MYH9-RD between July 2007 and March 2012 at four participating institutions. A total of 115 audiograms were analyzed from 63 patients belonging to 45 unrelated families with different NMMHC-IIA mutations. Cross-sectional analyses of audiograms were performed. Regression analysis was performed, and age-related typical audiograms (ARTAs) were derived to characterize the type of SNHL associated with different mutations. Severity of SNHL appeared to depend on the specific NMMHC-IIA mutation. Patients carrying substitutions at the residue R702 located in the short functional SH1 helix had the most severe degree of SNHL, whereas patients with the p.E1841K substitution in the coiled-coil region or mutations at the nonhelical tailpiece presented a mild degree of SNHL even at advanced age. The authors also disclosed the effects of different amino acid changes at the same residue: for instance, individuals with the p.R702C mutation had more severe SNHL than those with the p.R702H mutation, and the p.R1165L substitution was associated with a higher degree of hearing loss than the p.R1165C. In general, mild SNHL was associated with a fairly flat audiogram configuration, whereas severe SNHL correlated with downsloping configurations. ARTA plots showed that the most progressive type of SNHL was associated with the p.R702C, the p.R702H, and the p.R1165L substitutions, whereas the p.R1165C mutation correlated with a milder, nonprogressive type of SNHL than the p.R1165L. ARTA for the p.E1841K mutation demonstrated a mild degree of SNHL with only mild progression, whereas the ARTA for the mutations at the nonhelical tailpiece did not show any substantial progression. These data provide useful tools to predict the progression and the expected degree of severity of SNHL in individual MYH9-RD patients, which is especially relevant in young patients. Consequences in clinical practice are important not only for appropriate patient counseling but also for development of customized, genotype-driven clinical management. The authors recently reported that cochlear implantation has a good outcome in MYH9-RD patients; thus, stricter follow-up and earlier intervention are recommended for patients with unfavorable genotypes.

Association between pseudoexfoliation syndrome and sensorineural hearing loss

PurposeThe aim of this study was to investigate the sensorineural hearing loss (SNHL) in patients with pseudoexfoliation (PXF) syndrome and its correlation with clinical ophthalmological findings.MethodsA total of 25 patients with PXF and 15 age-matched and sex-matched normal controls were subjected to complete ophthalmological and audiological evaluation. The degree of hearing loss was compared between the two groups and correlated with the clinical findings including glaucoma, cataract, and laterality of PXS. The independent-sample Mann-Whitney U-test, the Kruskal-Wallis one-way analysis of variance, the related-sample McNemar test and Pearson's correlation were used for statistical analysis.ResultsA total of 22 patients had bilateral symmetrical SNHL. Overall, three ears (6%) had normal hearing sensitivity, 29 ears (58%) had mild SNHL, 15 ears (30%) had moderate SNHL, and three ears (6%) had moderately severe SNHL. On an average, patients had mild degree of SNHL in the frequencies 250-2000 Hz, moderate degree of SNHL in the frequency 4000 Hz, and moderately severe SNHL in the frequency 8000 Hz. SNHL was not affected by the association of cataract or glaucoma. A total of 28 ears of the controls had mild degree of hearing loss and two ears had normal hearing sensitivity.ConclusionThe prevalence and severity of SNHL increases in cases of PXF compared with age-matched controls. This is not affected by the laterality of ocular involvement, or the presence or absence of cataract or glaucoma. This confirms the systemic nature of the disease and may add to difficulties during ophthalmic surgery when performed under local anesthesia due to difficulty in communication.