Degree Of Sensorineural Hearing Loss Research Articles

OCENA PRZYDATNOŚCI KLINICZNEJ FONEMICZNIE ZRÓWNOWAŻONYCH LIST SŁÓW DLA DOROSŁYCH UŻYTKOWNIKÓW JĘZYKA KANNADA

ObjectiveThe present study aimed at standardizing the phonemically balanced word lists in the Kannada language for adults developed by Manjula et al. (2015).Materials and MethodsForty individuals with different degrees of sensorineural hearing loss were enrolled for the study. The word lists developed by Manjula et al. (2015) were presented monaurally under headphones at 40 dB SL (ref: PTA) in quiet. The number of correctly identified words was calculated for each list. The scores of all the lists in each group were statistically analyzed.ResultsThe results revealed that there is list equivalency within each group on repeated measures ANOVA. The statistical analysis also revealed that the speech identification scores reduced significantly with an increase in the severity of hearing loss on MANOVA.ConclusionsThe lists developed by Manjula et al. (2015) are sensitive to different degrees of hearing loss. Hence, the lists can be a good speech identification tool for testing adults with hearing loss in routine speech identification testing, assessing hearing aid benefits, and for research purposes where multiple word lists are required.

The relationship between tinnitus intensity and the degree of sensorineural hearing loss from the aspect of contribution of hyperbaric oxygen therapy

The relationship between tinnitus intensity and the degree of sensorineural hearing loss from the aspect of contribution of hyperbaric oxygen therapy

Differential effects of hearing loss on neural and behavioral measures of speech perception in noise

Understanding speech in background noise is difficult for many listeners, and those with hearing impairment tend to show considerable variability in performance. Event-related potentials (ERPs) and associated cortical oscillations are useful for examining neural responses to speech in various listening conditions and may represent neurophysiological markers of auditory perception. The present study examined the effects of hearing impairment on the neural coding and perception of speech in noise in a group of adult listeners with varying degrees of sensorineural hearing loss. This work was also designed to determine whether cortical ERPs and oscillatory rhythms in various frequency bands can predict the effects of hearing impairment on sentence recognition in noise. Passive N1-P2 and MMN responses were elicited with a double-oddball paradigm containing a consonant and vowel change in background noise. Speech perception was evaluated using phoneme discrimination and sentence recognition in noise tasks. Anal...

The peculiar features of vestibular function disorders associated with Meniere's disease

The objective of the present study was to reveal the special aspects of vestibular dysfunction in the patients presenting with unilateral Meniere's disease (MD) during the attack-free intervals. The study included 23 patients suffering from unilateral MD; all of them were examined with the use of tonal threshold audiometry, videonystagmography, the caloric test and video head-impulse test (vHIT). The study has demonstrated that the patients with MD are characterized, during the attack-free intervals, by the absence of latent spontaneous nystagmus in conjunction with the positive results of the head shaking test. Moreover, asymmetry of the results of the caloric test together with the normal results of the video head-impulse test is shown to be the characteristic feature of the patients with unilateral MD. The analysis of the degree of sensorineural hearing loss, the caloric test data, and coefficient of labyrinth asymmetry (CLAS) gave evidence of the tendency toward the elevation of the hearing threshold with increasing CLAS.

Bilateral versus unilateral hearing aids for bilateral hearing impairment in adults.

Acquired hearing loss is common and its incidence increases markedly with age. In most people, 'age-related' hearing loss is sensorineural (due to the loss of cochlear hair cells) and bilateral, affecting both ears to the same degree. Hearing loss categorised as mild, moderate or severe is primarily managed with hearing aids. People with bilateral hearing loss may be offered one aid, fitted to one specific ear, or two aids fitted to both ears. There is uncertainty about the relative benefits to people with hearing loss of these different strategies. To assess the effects of bilateral versus unilateral hearing aids in adults with a bilateral hearing impairment. The Cochrane ENT Information Specialist searched the ENT Trials Register; Cochrane Register of Studies Online; PubMed; Ovid Embase; CINAHL; Web of Science; ClinicalTrials.gov; ICTRP and additional sources for published and unpublished trials. The date of the search was 8 June 2017. Randomised controlled trials (RCTs) comparing the fitting of two versus one ear-level acoustic hearing aids in adults (over 18 years) with a bilateral hearing impairment, both ears being eligible for hearing aids. We used the standard methodological procedures expected by Cochrane. Our primary outcomes were patient preference for bilateral or unilateral aids, hearing-specific health-related quality of life and adverse effects (pain or discomfort in the ear, initiation or exacerbation of middle or outer ear infection). Secondary outcomes included: usage of hearing aids (as measured by, for example, data logging or battery consumption), generic health-related quality of life, listening ability and audiometric benefit measured as binaural loudness summation. We used GRADE to assess the quality of the evidence for each outcome; this is indicated in italics. We included four cross-over RCTs with a total of 209 participants, ranging in age from 23 to 85 and with a preponderance of men. All the studies allowed the use of hearing aids for a total period of at least eight weeks before questions on preference were asked. All studies recruited patients with bilateral hearing loss but there was considerable variation in the types and degree of sensorineural hearing loss that the participants were experiencing.Three of the studies were published before the mid-1990s whereas the fourth study was published in 2011. Therefore, only the most recent study used hearing aids incorporating technology comparable to that currently readily available in high-income settings. Of the four studies, two were conducted in the UK in National Health Service (NHS - public sector) patients: one recruited patients from primary care with hearing loss detected by a screening programme whereas the other recruited patients who had been referred by their primary care practitioner to an otolaryngology department for hearing aids. The other two studies were conducted in the United States: one study recruited only military personnel or veterans with noise-induced hearing loss whereas about half of the participants in the other study were veterans.Only one primary outcome (patient preference) was reported in all studies. The percentage of patients who preferred bilateral hearing aids varied between studies: this was 54% (51 out of 94 participants), 39% (22 out of 56), 55% (16 out of 29) and 77% (23 out of 30), respectively. We have not combined the data from these four studies. The evidence for this outcome is of very low quality.The other outcomes of interest were not reported in the included studies. This review identified only four studies comparing the use of one hearing aid with two. The studies were small and included participants of widely varying ages. There was also considerable variation in the types and degree of sensorineural hearing loss that the participants were experiencing.For the most part, the types of hearing aid evaluated would now be regarded, in high-income settings, as 'old technology', with only one study looking at 'modern' digital aids. However, the relevance of this is uncertain, as this review did not evaluate the differences in outcomes between the different types of technology.We were unable to pool data from the four studies and the very low quality of the evidence leads us to conclude that we do not know if people with hearing loss have a preference for one aid or two. Similarly, we do not know if hearing-specific health-related quality of life, or any of our other outcomes, are better with bilateral or unilateral aids.

Using temporal bone computed tomography to predict sensorineural hearing loss in otic capsule-sparing temporal bone fracture

ObjectivesThe purpose of this study was to investigate the efficacy of otic capsule-sparing (OCS) length for the prediction of sensorineural hearing loss (SNHL) in patients with OCS temporal bone fracture. MethodsThirty-four patients with OCS temporal bone fracture were enrolled, and their temporal bone computed tomography (TBCT), audiogram, and medical records were reviewed. The TBCT measured the shortest length between the otic capsule and fracture line. This length was referred to as the OCS length and was used to predict SNHL. Ossicular dislocation and fracture were also evaluated. Patients were divided into two groups according to the presence of SNHL. Univariate and multivariate analyses were performed for age, gender, brain hemorrhage, mean bone conduction threshold on the contralesional side, OCS length, and ossicular dislocation and fracture on TBCT. A receiver operating characteristic (ROC) curve was produced to evaluate the efficacy of OCS length for the prediction of SNHL. To determine an association between degree of SNHL and OCS length, regression analysis was conducted in the SNHL group. ResultsThe mean OCS lengths of the SNHL and non-SNHL groups were 4.42±1.67mm and 8.00±5.71mm, respectively. In the univariate analysis, a relatively significant association (P <0.20) was found between SNHL and age, brain hemorrhage, mean bone conduction threshold on the contralesional side, OCS length, and incus dislocation. Multivariate analysis was performed using these factors. On multivariate analysis, OCS length (P=0.030, odds ratio=0.598; 95% confidence interval 0.375–0.952) was the only independent factor associated with SNHL. The area under the curve (AUC) was 0.747. When the cut-off value of OCS length was 5.27mm, the sensitivity and specificity for the prediction of SNHL were 71.4% and 69.2%, respectively. In the regression analysis, OCS length showed a significant association with degree of SNHL in the SNHL group (P=0.025, β=-12.822, SE=5.282). ConclusionsThe major finding of our study was that OCS length was significantly associated with SNHL in patients with OCS temporal bone fracture. Such patients with a short OCS length had a higher likelihood of SNHL.

Narrow band CE-Chirp stimulus in auditory steady state response threshold estimation in normal hearers and patients with various degrees of sensorineural hearing loss

Background: Auditory steady-state responses (ASSRs) are periodic scalp potentials that arise in response to auditory stimuli. Narrow-band (NB) CE-Chirps stimuli have been developed to combine the advantages of compensation for the cochlear traveling wave delay and frequency specificity.Objectives: To measure the hearing threshold objectively using ASSR in adults with normal behavioural hearing thresholds and adult patients with different degrees of sensorineural hearing loss (SNHL) and its comparison to the behavioural thresholds.Methods: In the present study, 35 subjects (70 ears) were enrolled. Ears were grouped according to the level of hearing obtained by pure tone audiometry (PTA) into 7 equal groups. NB-CE-Chirp ASSR was done for all groups by means of auditory-evoked potential device.Results: The estimated ASSR audiograms configuration matched the behavioural curves. ASSR was equally accurate at all frequencies tested except for 1 kHz in the normal-hearing group who showed less accuracy compared to 500 and 4000 Hz in air conduction (AC) and in bone conduction (BC). Although the AC estimation was not equal among the different degrees of hearing compared to the BC estimation, the AC PTA-ASSR thresholds difference range was small. BC PTA-ASSR threshold difference was statistically significantly less than AC at all tested degrees of hearing loss, in most of the frequencies; which reflects that the ASSR was more accurate in estimating BC than the AC thresholds. There was a negative correlation regarding behavioural BC PTA thresholds with PTA-ASSR threshold difference and PTA-estimated audiograms threshold difference at all tested frequencies.Conclusion: ASSR using either AC or BC NB-CE-Chirp is a reliable objective method in estimating the behavioural threshold in normal hearers and patients with various degrees of SNHL, so it can be used in difficult-to-test cases where accurate behavioural thresholds could not be obtained.

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.

Sensorineural Hearing Loss in Patients with Chronic Suppurative Otitis Media

introduction: The relationship between sensorineural hearing loss and Chronic Suppurative Otitis Media remains a controversial. damage of the cochlea and high-frequency sensorineural hearing loss was suggested to arise from passage of toxic substances and the drugs from the middle ear through the round window membrane into the inner ear.Aim of study : To evaluate the incidence of sensorineural hearing loss in ears with chronic otitis media.Method: This is prospective a study, consisted of 120 patients. They were 90 males and 30 females . they had been assessed at Otolaryngology department in Al-Diwania Teaching Hospital , in Al-Diwania city , Iraq, between January 2013 to December 2013, the age range from 10-60 years . inclusion criteria are as follows: unilateral continuous otorrhea through a perforated tympanic membrane for at least 3 months, normal tympanic membrane in the contralateral ear based on otoscopy. We do pure tone audiometry( PTA) for every Patient Exclusion criteria are history of head trauma or meningitis, previous tympanomastoid surgery, history of noise exposure ,systemic ototoxic drug therapy, family history of congenital or acquired sensorineural hearing loss, and post-traumatic tympanic membrane perforation. The control used was the contralateral ear with normal otoscopy.Results: The mean bone conduction threshold in the diseased ear was 38.33 dB, and 10.1 dB in the control ear. The mean bone conduction threshold differences between the diseased and control ears range from 18.96 to 35.4dB across the frequencies 0.5,1.0, 2.0, and 4.0 kHz. These differences tended to increase with increasing frequency and were all significant (P &lt; 0.05).Conclusion: Patients with chronic suppurative otitis media had a significant degree of sensorineural hearing loss in this study. The higher frequencies were more affected

The risk ratio for development of hereditary sensorineural hearing loss in consanguineous marriage offspring

ObjectivesThis study aims to define the relative risk of development of hearing loss in offspring of consanguineous marriages. Materials and methodsThis is a retrospective case-control study conducted in a tertiary referral center in Jeddah, KSA. The study group included 1600 probands (848 males, 752 females), with age range 0.5–12 years (6.6 ± 3.6). The study group comprised of two equal, age and sex matched subgroups; Hearing Loss (HL) group and Normal Hearing (NH) group. The children included in the HL group should have idiopathic or non syndromic genetic sensorineural hearing loss. ResultsThe HL Group comprised 800 children with variable degrees of sensorineural hearing loss. Profound and severe degrees of hearing loss were the most prevalent degrees (P <0.05%). The prevalence of consanguineous marriage offspring in the NH group was 42.5%, while in the HL group it was 68.9% (P < 0.05). The differences between both study subgroups regarding the distribution of different degrees of parental consanguinity (first, second, double first, and first once removed cousins) were insignificant (P > 0.05). The relative risk and 95% confidence interval (RR, 95% CI) for development of hearing loss in offspring of consanguineous marriage was 1.76 (95% CI 1.57–1.97, P < 0.001). ConclusionsThere was 76% increased risk for consanguineous marriage progeny to develop SNHL when compared to non consanguineous progeny.

Sensorineural hearing loss in patients with chronic suppurative otitis media: Is there a significant correlation?

IntroductionHearing loss as a sequel of chronic suppurative otitis media (CSOM) is often conductive, but recent studies have found an additional sensorineural component in these patients, thus demonstrating inner ear damage. The aim of the study was to determine the association between CSOM and sensorineural hearing loss (SNHL) and to assess the influence of patient’s age, duration of disease, and presence of cholesteatoma and ossicular erosion on the degree of SNHL.MethodsIn a retrospective study, the medical records of 119 patients who underwent surgery was reviewed. Seventy patients met the inclusion criteria of unilateral otorrhea, normal contralateral ear on otoscopy, and age between 10–65 years with no history of head trauma or ear surgery or familial hearing loss. Bone conduction (BC) thresholds for affected and contralateral ear were measured at frequencies of 500, 1000, 2000, and 4000 Hz. Data analysis was performed using SPSS 13 with independent-samples t-test, Pearson correlation test, and two-tailed analysis. A p ≤ 0.05 was considered statistically significant.ResultsSignificant higher BC thresholds were found in the affected ear than in the normal ear for each frequency (p < 0.001), which increased with increasing frequency (7.00 dB at the 500 Hz and 9.71 dB at the 4000 Hz). There was a significant correlation between age and degree of SNHL (r = 0.422, p < 0.001) but no significant correlation was in duration of the disease (r = 0.119, p > 0.05). There was no relationship between presence of cholesteatoma and ossicular erosion with SNHL (p > 0.05).ConclusionThese findings demonstrate that CSOM is associated with some degree of SNHL and cochlear damage, and higher frequencies are more affected. Aging can act as a precipitating factor in this pathological process.

Effects of Smoking on Eustachian Tube and Hearing.

The purpose of this study was to evaluate the effect of tobacco use on the Eustachian tube and inner ear function. Case-control study. Thirty-one nonsmoking volunteers and 34 smoking subjects recruited in an University Hospital, submitted to an audiological evaluation including pure tone audiometry, basal tympanogram, stapedial reflexes analysis, and nine-step eustachian tube (ET) function test. Pure Tone Average (PTA) threshold at all frequencies tested was 12.5 dB in smokers and 3.7 in nonsmoking subjects. Nine smokers (27%) presented some degree of hearing loss versus none in the nonsmoker group. Linear regression analysis showed a higher degree of sensorineural hearing loss with age in smokers. Among the smokers, 20 subjects (59%) presented an impaired tubal function for the nine-step inflation/deflation tympanometric test, while only 6 (19%) subjects in the group of nonsmokers showed a tubal dysfunction. Tobacco use may reduce the ability to hear, mainly causing a sensorineural hearing loss for higher frequencies. We also found the presence of a high number of smokers suffering from tubal dysfunction. This has an important clinical relevance, not only because smoking increases the incidence of middle ear diseases, but also because tubal dysfunction may cause nonspecific symptoms characterised by ear fullness and difficulties in middle ear equalisation.

Inner ear dysfunction in patients with Behcet\u2019s disease

BackgroundBehcet’s disease (BD) is a rare immune-mediated small-vessel systemic vasculitis that often presents with mucous membrane ulceration and ocular problems. Alajouanine and colleagues first described inner ear involvement in BD in 1961. The cochlea is more frequently involved than the vestibular labyrinth or the central vestibular tracts. Hearing is commonly impaired in BD and rates vary between 24 and 62%.ObjectiveThe current study investigated the incidence, relationship, and characteristics of audiovestibular disturbances in patients with BD compared with age and sex-matched healthy individuals.Patients and methodsThe participants of this prospective study classified into two groups. The study group consisted of 15 patients (six men and nine women) with BD and the control group consisted of 15 healthy volunteers (five men and 10 women) without otological symptoms. A wide range of tests were conducted; all patients were evaluated by conventional pure-tone audiometry (0.25–8.0 kHz), high-frequency audiometry (8.0–16.0 kHz), and vestibular test battery in terms of bithermal caloric test and computerized dynamic posturography testing.ResultsThere was no significant differences between the two groups in terms of sex and age. Abnormalities of the pure-tone audiometry showed that 34% of patients demonstrated variable degrees of sensorineural hearing loss, while 60% of patients demonstrated abnormal audiograms at the high-frequency audiometry. In terms of vestibular assessment, 34% of patients demonstrated abnormalities at the caloric test, while 47% demonstrated abnormalities at the computerized dynamic posturography testing.ConclusionThe outcome of this study confirms the association between the inner ear dysfunction and the BD. Inner ear dysfunction in BD could be attributed to the presence of vasculitis or neuropathy that could happen on top of chronic inflammation process associated with BD. Therefore, patients with BD should be regularly subjected to otolaryngological evaluation to diagnose and for follow up of any audiovestibular involvement.

Long-term Incidence and Degree of Sensorineural Hearing Loss in Otosclerosis.

1) To evaluate the long-term incidence and degree of the sensorineural component of hearing loss (SNHL) in patients with otosclerosis after accounting for expected age-related hearing loss. 2) To identify variables that might predict development of sensorineural hearing loss due to otosclerosis. Retrospective audiometric database and chart review. Tertiary referral center. Consecutive patients with otosclerosis observed between 1994 and 2004, with ≥10 years follow-up, excluding patients with postoperative hearing loss or surgery before the initial audiogram. Bone conduction (BC) thresholds at 0.5, 1, 2, and 4 kHz and Word Recognition. BC threshold change (BCTC) over ≥10 years minus estimated age-related threshold change (ARTC) specific to age and sex for each patient (based on ISO 7029 reference population). Three-hundred fifty-seven ears (290 patients) met study criteria, including 217 ears that had undergone stapedectomy during the study period. Mean follow-up was 14.0 years. The average BCTC after subtracting estimated ARTC was 4.6, 2.6, 3, and 2.7 dB for 0.5, 1, 2, and 4 kHz frequencies, respectively. However, 34% of ears (122 ears) had clinically significant progression of SNHL during the study period (>10 dB BCTC beyond expected ARTC at ≥2 frequencies). Multivariate analysis demonstrated that the probability of developing clinically significant SNHL was higher for women (odds ratio 1.86, p = 0.018) and lower for operated patients (odds ratio 0.46, p = 0.002). The average long-term sensorineural hearing loss due to otosclerosis was statistically significantly more than for age alone at each frequency, but these average values (from 2.6 to 4.6 dB for tested frequencies) were clinically insignificant. Approximately one-third of patients with otosclerosis demonstrated a clinically significant progression of the sensorineural component of hearing loss, with the average BCTC above expected age-related changes ranging from 10.2 to 14.6 dB for tested frequencies among this subgroup.

Treatment of ear and bone disease in the Phex mouse mutant with dietary supplementation

HypothesisPhosphorus and vitamin D (calcitriol) supplementation in the Phex mouse, a murine model for endolymphatic hydrops (ELH), will improve otic capsule mineralization and secondarily ameliorate the postnatal development of ELH and sensorineural hearing loss (SNHL). BackgroundMale Phex mice have X-linked hypophosphatemic rickets (XLH), which includes osteomalacia of the otic capsule. The treatment for XLH is supplementation with phosphorus and calcitriol. The effect of this treatment has never been studied on otic capsule bone and it is unclear if improving the otic capsule bone could impact the mice's postnatal development of ELH and SNHL. MethodsFour cohorts were studied: 1) wild-type control, 2) Phex control, 3) Phex prevention, and 4) Phex rescue. The control groups were not given any dietary supplementation. The Phex prevention group was supplemented with phosphorus added to its drinking water and intraperitoneal calcitriol from postnatal day (P) 7–P40. The Phex rescue group was also supplemented with phosphorus and calcium but only from P20 to P40. At P40, all mice underwent auditory brainstem response (ABR) testing, serum analysis, and temporal bone histologic analysis. Primary outcome was otic capsule mineralization. Secondary outcomes were degree of SNHL and presence ELH. ResultsBoth treatment groups had markedly improved otic capsule mineralization with less osteoid deposition. The improved otic capsule mineralized did not prevent the development of ELH or SNHL. ConclusionSupplementation with phosphorus and calcitriol improves otic capsule bone morphology in the Phex male mouse but does not alter development of ELH or SNHL.

Establishment of a Flexible Real-Time Polymerase Chain Reaction-Based Platform for Detecting Prevalent Deafness Mutations Associated with Variable Degree of Sensorineural Hearing Loss in Koreans.

Many cutting-edge technologies based on next-generation sequencing (NGS) have been employed to identify candidate variants responsible for sensorineural hearing loss (SNHL). However, these methods have limitations preventing their wide clinical use for primary screening, in that they remain costly and it is not always suitable to analyze massive amounts of data. Several different DNA chips have been developed for screening prevalent mutations at a lower cost. However, most of these platforms do not offer the flexibility to add or remove target mutations, thereby limiting their wider use in a field that requires frequent updates. Therefore, we aimed to establish a simpler and more flexible molecular diagnostic platform based on ethnicity-specific mutation spectrums of SNHL, which would enable bypassing unnecessary filtering steps in a substantial portion of cases. In addition, we expanded the screening platform to cover varying degrees of SNHL. With this aim, we selected 11 variants of 5 genes (GJB2, SLC26A4, MTRNR1, TMPRSS3, and CDH23) showing high prevalence with varying degrees in Koreans and developed the U-TOP™ HL Genotyping Kit, a real-time PCR-based method using the MeltingArray technique and peptide nucleic acid probes. The results of 271 DNA samples with wild type sequences or mutations in homo- or heterozygote form were compared between the U-TOP™ HL Genotyping Kit and Sanger sequencing. The positive and negative predictive values were 100%, and this method showed perfect agreement with Sanger sequencing, with a Kappa value of 1.00. The U-TOP™ HL Genotyping Kit showed excellent performance in detecting varying degrees and phenotypes of SNHL mutations in both homozygote and heterozygote forms, which are highly prevalent in the Korean population. This platform will serve as a useful and cost-effective first-line screening tool for varying degrees of genetic SNHL and facilitate genome-based personalized hearing rehabilitation for the Korean population.

A novel mutation of the MITF gene in a family with Waardenburg syndrome type 2: A case report.

Waardenburg syndrome (WS) is an autosomal dominant disorder with varying degrees of sensorineural hearing loss, and accumulation of pigmentation in hair, skin and iris. There are four types of WS (WS1-4) with differing characteristics. Mutations in six genes [paired box gene 3 (PAX3), microphthalmia-associated transcription factor (MITF), endothelin 3 (END3), endothelin receptor type B (EDNRB), SRY (sex determining region Y)-box 10 (SOX10) and snail homolog 2 (SNAI2)] have been identified to be associated with the various types. This case report describes the investigation of genetic mutations in three patients with WS2 from a single family. Genomic DNA was extracted, and the six WS-related genes were sequenced using next-generation sequencing technology. In addition to mutations in PAX3, EDNRB and SOX10, a novel heterozygous MITF mutation, p.Δ315Arg (c.944_946delGAA) on exon 8 was identified. This is predicted to be a candidate disease-causing mutation that may affect the structure and function of the enzyme.

Neural Spike-Train Analyses of the Speech-Based Envelope Power Spectrum Model

Diagnosing and treating hearing impairment is challenging because people with similar degrees of sensorineural hearing loss (SNHL) often have different speech-recognition abilities. The speech-based envelope power spectrum model (sEPSM) has demonstrated that the signal-to-noise ratio (SNRENV) from a modulation filter bank provides a robust speech-intelligibility measure across a wider range of degraded conditions than many long-standing models. In the sEPSM, noise (N) is assumed to: (a) reduce S + N envelope power by filling in dips within clean speech (S) and (b) introduce an envelope noise floor from intrinsic fluctuations in the noise itself. While the promise of SNRENV has been demonstrated for normal-hearing listeners, it has not been thoroughly extended to hearing-impaired listeners because of limited physiological knowledge of how SNHL affects speech-in-noise envelope coding relative to noise alone. Here, envelope coding to speech-in-noise stimuli was quantified from auditory-nerve model spike trains using shuffled correlograms, which were analyzed in the modulation-frequency domain to compute modulation-band estimates of neural SNRENV. Preliminary spike-train analyses show strong similarities to the sEPSM, demonstrating feasibility of neural SNRENV computations. Results suggest that individual differences can occur based on differential degrees of outer- and inner-hair-cell dysfunction in listeners currently diagnosed into the single audiological SNHL category. The predicted acoustic-SNR dependence in individual differences suggests that the SNR-dependent rate of susceptibility could be an important metric in diagnosing individual differences. Future measurements of the neural SNRENV in animal studies with various forms of SNHL will provide valuable insight for understanding individual differences in speech-in-noise intelligibility.

A Case of Bacterial Meningoencephalitis Following Sudden Sensorineural Hearing Loss with Vertigo

Received August 12, 2015 Revised October 9, 2015 Accepted October 18, 2015 Address for correspondence Mee Hyun Song, MD, PhD Department of OtorhinolaryngologyHead and Neck Surgery, Myongji Hospital, Seonam University College of Medicine, 55 Hwasu-ro 14beon-gil, Deogyang-gu, Goyang 10475, Korea Tel +82-31-810-6360 Fax +82-31-810-6357 E-mail meehyun924@hanmail.net We report a case of sudden sensorineural hearing loss with vertigo in a 68-year-old woman, who developed bacterial meningoencephalitis during steroid treatment. The patient initially showed severe degree of sensorineural hearing loss on the left side with spontaneous nystagmus beating toward the contralateral side. Brain magnetic resonance imaging demonstrated no abnormal finding other than high signal intensity in parts of mastoid air cells and mild mucosal hypertrophy of the paranasal sinuses. During the course of steroid treatment, the hearing worsened to profound hearing loss, and on the 6th day of steroid treatment, the patient demonstrated dysarthria and disorientation with subsequent development of high fever. The patient was diagnosed with bacterial meningoencephalitis and treated with antibiotics. The patient recovered without any neurologic deficit but unilateral profound hearing loss persisted. The case is presented here along with a possible pathogenic mechanism of bacterial meningoencephalitis following sudden sensorineural hearing loss in this patient. Korean J Otorhinolaryngol-Head Neck Surg 2016;59(2):165-9

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.