Search

A 54-year-old female Nigerian presented with a 1-week history of sudden deterioration of vision in both eyes. There was no antecedent history of ocular trauma, floaters, nor flashes of light. However, she gave a history of a febrile illness associated with headaches and malaise 2 weeks prior to onset of ocular symptoms. She is a known retroviral-positive patient on treatment with highly active antiretroviral therapy for the past 5 years but is not a known hypertensive nor diabetic. At presentation, the best corrected visual acuity was hand movement in both eyes. Anterior segment examination of both eyes revealed fine keratic precipitates on the corneal endothelium with flare and inflammatory cells in the anterior chamber and grade 1 nuclear sclerosis. Dilated binocular indirect ophthalmoscopy of both eyes revealed pink disks with blurred margins total exudative retinal detachments. An assessment of Vogt–Koyanagi–Harada syndrome in a patient with human immunodeficiency virus/acquired immunodeficiency syndrome was made. Bilateral exudative detachment resolved with improvement of her best corrected visual acuity to 6/9 in both eyes after systemic treatment with steroids.&nbsp

OBJECTIVES: To compare clinical and neurodevelopmental outcome at the age of 6 months for neonates born to SARS-CoV-2-positive mothers. METHODS: Neonates of SARS-CoV-2 positive mothers, admitted in our hospital were assessed for growth, neurodevelopment by Amiel-Tison method, and Developmental Profile (DP3) at discharge as part of another study (July 2020). This data were retrieved and babies followed-up at the age of 6 months. Composite adverse outcome was death within 6 months post discharge or DP3 score

Objectives Although hearing loss is a well-known symptom of mitochondria-related disorders, it is not clear how often it is a congenital and cochlear impairment. The Newborn Hearing Screening Program (NHSP) enables to distinguish congenital cochlear deafness from an acquired hearing deficit. The initial aim of the study was to research the frequency of the congenital cochlear hearing loss among patients with various gene defects resulting in mitochondrial disorders. The research process brought on an additional gain: basing on our preliminary study group of 80 patients, in 12 patients altogether we identified two defected genes responsible for mitochondrial disorders, whose carriers did not pass the NHSP. Finally, these patients were diagnosed with the congenital cochlear deafness. Material and methods The results of the NHSP in the patients with mitochondrial disorders diagnosed in our tertiary reference center were analyzed. Only the cases with confirmed mutations were qualified for the study group. The NHSP database included 80 patients with mutations in 31 different genes: 25 nuclear-encoded and 6 mtDNA-encoded. We searched the literature for the presence of a congenital hearing impairment (CHI) in mitochondrial disorders caused by changes in 278 already known genes. Results For 68 patients from the study group the NHSP test indicated a proper cochlear function and thus suggested normal hearing. For 12 mitochondrial patients, the NHSP test indicated the requirement for the further audiological diagnosis, and finally CHI was confirmed in 8 of them. This latter subset included patients with pathogenic variants in RRM2B and SERAC1, known as “deafness-causing genes”. Contrary to our initial expectations, the patients carrying mutations in other “deafness-causing genes”: MPV17, POLG, COX10, as well as other mitochondria-related genes, all reported in literature, did not indicate any CHI following the NHSP test. Conclusion Our study indicates that the cochlear CHI is a phenotypic feature of the RRM2B and SERAC1 related defects. The diagnosis of the CHI following the NHSP allows to early distinguish those defects from other mitochondria-related disorders in which the NHSP test result is correct. Wider studies are needed to assess the significance of this observation.

Introduction: Oral Submucous Fibrosis (OSMF) is a slowly progressive chronic fibrotic disease of the oral cavity extending to pharynx. Function and patency of eustachian tube gets altered when the palatal and paratubal muscles which regulate the patency of pharyngeal orifice gets affected. This leads to pain in ear along with mild to moderate conductive loss of hearing. Aim: To evaluate hearing deficit in OSMF patients and to correlate clinical stages of OSMF with degree of hearing deficits in patients. Materials and Methods: It was a cross-sectional study. The study comprised of a total of 50 subjects (100 ears). Forty patients diagnosed with OSMF who reported to Department of Oral Medicine and Radiology constituted the study group. Ten normal individuals with no deleterious habits and without any previously diagnosed ear disorders constituted the control group. Pure Tone Audiometry (PTA) was used for evaluating all the subjects for air conduction and bone conduction hearing loss. Values on qualitative characteristics were shown as n (% prevalence) across five study groups. Values on quantitative variables were shown as Mean±SD across five study groups. Inter group comparison for qualitative and quantitative variables was done using chi-square test and analysis of variance (ANOVA) test respectively with Post-Hoc Bonferroni’s correction for multiple group comparisons. Results: The OSMF group showed a marked degree of hearing loss compared to the control group. The distribution of mean PTA in quantitative assessment was significantly higher in group D compared to group A and group B (p-value

Background The importance of neonatal screening for congenital cytomegalovirus infection (cCMV) is widely shared in the world scientific literature. However, currently, no programs for universal neonatal screening for cCMV have been reported in the literature, and only a few experiences of hearing targeted cCMV screening have been published. In the Tuscany Region, Italy, screening for cCMV has been mandatory since 2008 for each newborn that result "refer" at the Transient Evoked Otoacoustics Emissions (TEOAE) test, and in our university hospital since 2012, it has been extended to some categories at risk of cCMV. Methods We present the results of the protocol for cCMV screening adopted at our Institution since 2012. Results From 2012 to 2017, 1615 newborns underwent cCMV screening. Twenty-five cases were positive for cCMV, (1.54% of all the newborn screened for cCMV and 0.19% of infants submitted to newborn hearing screening). Nineteen of these children (76%) had normal hearing, while 6 (24%) had a hearing deficit of variable degree. 2/25 (8%) cases presented a progression of the hearing deficit in the first months of life and no children had a late onset or fluctuating hearing loss. Conclusions Our findings show how cCMV screening in newborns that result refer at TEOAE allows the detection of many cases; but many are still missed. It would be, therefore, important to adopt a universal newborn cCMV screening program or a program extended to newborns at higher risk.

Purpose There is significant prevalence of overt and subclinical hypothyroidism in pregnant women in rural areas. Maternal hypothyroidism is known to cause congenital hypothyroidism resulting in sensorineural hearing loss. Anti-Thyroperoxidase antibodies are known to cross placental barrier. There is no literature on hearing assessment in infants born to women whose hypothyroidism was corrected during pregnancy. Do these infants suffer hearing loss? Our study addresses this question. Methods 140 infants born to women on treatment for hypothyroidism during pregnancy and 140 infants born to euthyroid women were evaluated for hearing by Brainstem Evoked Response Audiometrry at 1 and 4 months age. Anti-TPO antibodies were estimated at 4 months of age. Results There was no clinical hearing deficit or delay in neurological development in infants born to women undergoing treatment for hypothyroidism during pregnancy. However wave V latency on BERA was slightly prolonged in them compared to infants born to euthyroid women. There was absence of wave V when maternal subclinical hypothyroidism persisted till parturition. However within 6–8months of age the wave V latencies corrected to normal. Anti-TPO antibodies were within normal range at 4months age. Conclusion Maternal hypothyroidism when corrected before parturition does not affect hearing in the infants clinically. The mild delay in wave V on BERA corrects within first year of life. However larger studies to assess hearing in infants born to women having overt hypothyroidism during first trimester of pregnancy may be desirable to assess whether hearing is adversely affected in them.

Summary Chronic suppurative otitis media (CSOM) refers to the middle ear inflammation which is clinically characterized by the discharging ear, hearing deficit, fever and otalgia. Although a clinical diagnosis, imaging is imperative to rule out associated complications which apart from causing hearing deficit, may prove fatal at times. Both high resolution computed tomography (HRCT) and MRI are helpful in evaluating middle ear pathologies, usage being indication specific. Due to its excellent spatial resolution, HRCT is invaluable in assessment of chronically discharging ears, especially to look for bone erosion and the integrity of the ossicles. Due to its better spatial resolution, HRCT is preferred in suspected intra temporal complications whereas MRI is more useful in evaluating intracranial extension.

Objective This study focused on the changes in the internal acoustic canal (IAC) caused by vestibular schwannomas (VSs) and their prognostic significance for postoperative hearing outcome. Methods A total of 125 consecutive cases of VS were included. We used a neuronavigation software to perform the following measurements on both the tumor side and healthy side: volume of the IAC (VIAC), maximal diameter of the IAC (DIAC), and length of the IAC (LIAC). A statistical analysis was realized using Spearman correlation to test the correlation of the morphometric measure of the IAC and postoperative hearing. Multivariate analysis was performed to test the impact of measurements of the IAC and preoperative hearing on postoperative hearing. Results The mean VIAC on the tumor side and on the healthy side was 0.271 and 0.169 cm3, respectively. The mean DIAC was 9.438 mm on the tumor side and 7.034 mm contralateral. The correlations tests showed significant correlations of both postoperative hearing deficit and degree of hearing loss with 1) VIAC on the tumor side, 2) difference between VIAC on the tumor side and healthy side, 3) DIAC on the tumor side, and 4) difference between the DIAC on the tumor side and healthy side. The multivariate analysis showed significant impact of the DIAC (P = 0.01) and preoperative hearing status (P = 0.02) on postoperative hearing. Conclusions Enlargement of the VIAC and DIAC are negative prognostic factors for hearing preservation. Reasons may be long-standing compression of the auditory nerve and an increased vulnerability of the inner ear structures during the drilling of the IAC.

Stahl ear deformity is a benign congenital malformation of the auricle characterized by an abnormal third crus. While there is no functional hearing deficit related to this deformity, they have been described as “elf like" or "satyr like” and can have social anesthetic ramifications. If recognized in the newborn period this deformity can be corrected noninvasively through the use of ear molds. However for those requiring surgical correction there is no standardized technique for repair. We present a case of a unilateral Stahl ear that was corrected using a combination of cartilage-cutting and -sparing techniques.

Mestrado em Gestão e Avaliação em Tecnologias da Saúde Submitted by Maria da Luz Antunes (mluz.antunes@estesl.ipl.pt) on 2019-07-17T11:12:06Z No. of bitstreams: 1 Qualidade de vida em indivíduos com perda auditiva_revisão sistemática da literatura.pdf: 3177512 bytes, checksum: 74beeacf39d509311facab695f2ea937 (MD5) Made available in DSpace on 2019-07-17T11:12:06Z (GMT). No. of bitstreams: 1 Qualidade de vida em indivíduos com perda auditiva_revisão sistemática da literatura.pdf: 3177512 bytes, checksum: 74beeacf39d509311facab695f2ea937 (MD5) Previous issue date: 2019 N/A

Objectives The optimal timing and technique for repairing chronic tympanic membrane (TM) perforations in pediatric patients remains controversial. The objectives are to determine the surgical and hearing outcomes of pediatric lateral graft tympanoplasty at a tertiary teaching hospital. Methods A retrospective review was conducted for pediatric lateral graft tympanoplasties performed for chronic TM perforations by a single surgeon over a four-year period. Primary and secondary outcomes were graft failure rate and hearing outcomes, respectively. Results 78 cases were analyzed. The mean age at time of surgery was 10.3 years (range 5–18 years). Mean follow-up was 11.0 months; 27 patients had follow-up >1 yr. Most patients were non-syndromic (85.9%), had a history of bilateral Eustachian tube dysfunction (ETD) (59%) and presented with marked myringosclerosis (73.1%). Thirty-three percent of cases were revision tympanoplasties. A learner surgeon (resident or fellow) was present in 89.7% of cases. Successful closure of the TM was achieved in 97.4% (76/78) of cases and 92.6% (25/27) of cases with >1-year follow-up. No obvious difference in graft failure was noted with regards to age at time of surgery, perforation size, history of bilateral ETD, presence of a learner surgeon, myringosclerosis, presence of syndromic features, or history of prior tympanoplasty. Ninety-one percent of patients either improved hearing or preserved their conductive hearing deficit. Poorer hearing outcomes were only associated with post-operative blunting. Conclusions Pediatric lateral graft tympanoplasty is effective in repairing chronic perforations with excellent hearing outcomes. Common quoted predictors of surgical outcome such as age at the time of surgery, syndromic features, history of previous myringoplasty, perforation size, and ETD dysfunction were not associated with graft failure in our series.

Objective: To determine the incidence of hearing loss after acute episode of meningitis in children Methods: A descriptive study carried out in the Department of Pediatric Medicine of The Children’s Hospital Lahore, Pakistan from January 2014 to July 2016. A total of 175 children one month to 13 years of age admitted with diagnosis of meningitis were included. Complete blood count, CSF cytology, biochemistry and culture sensitivity were sent. CT scan brain was done if required. Hearing assessment was done two weeks after admission using otoacoustic emissions in the patients having normal tympanogram. Hearing impairment was classified as sensorineural if otoacoustic emissions were absent while tympanometry was normal. Results: Of 175 children, 58% were males and 42% were females. Mean age was 2.1 years. Orientation as assessed by Glasgow comma scale (GCS) was normal in 63% while 5% had GCS

An immature female killer whale (Orcinus orca) stranded in the Wadden Sea in 2010 and was later transferred to Loro Parque, Tenerife, Spain, for rehabilitation. The killer whale, named “Morgan,” was suspected to have a hearing impairment. To test whether Morgan has a hearing deficit, auditory brainstem responses to short-duration, broadband click stimuli were recorded. The same procedure was conducted with five other killer whales at Loro Parque for comparative purposes. Stereotypical click-evoked responses were recorded in all of the killer whales except Morgan, even at the highest click level that could be projected. Reductions in the amplitude of the click-evoked response paralleled reductions in the stimulus amplitude of the clicks presented to all of the other whales. The lack of a click-evoked response in Morgan indicates that she suffers from a hearing deficit. The magnitude and frequency range over which the hearing deficit occurs cannot be specified with the techniques used here. Nevertheless, it can be concluded that Morgan’s hearing sensitivity to broadband signals is at least 20 to 30 dB worse than the hearing sensitivity of the other killer whales tested. Morgan potentially suffers from a profound hearing deficit or even a complete loss of hearing, but this cannot be determined through the electrophysiological tests used in this experiment.

UNLABELLED Superior canal dehiscence is a rare syndrome. The symptoms consist of hearing loss, dizziness and autophonia. The patient can be cured with surgery. A 28 year old woman went to several doctors for several months due to diminished hearing, dizziness and autophonia. The symptoms got worse. A work-up led to the diagnosis of superior canal dehiscence in the left ear. She underwent surgery and her symptoms improved. A latency in diagnosis is expected as the syndrome is rare. It's important to think of superior canal dehiscence when patients complain of these symptoms. KEY WORDS superior canal dehiscence, autophonia, dizziness, hearing deficit. Correspondence: Bryndis Baldvinsdottir, bryndisbaldvins@gmail.com.

Community-acquired acute bacterial meningitis is a fulminant disease with a mortality of 10–30% and high risk for permanent neurologic or hearing deficit [1, 2, 3, 4]. It is one of the most urgent treatable conditions because the patients may deteriorate to coma and death within 24–72 h, and early adequate treatment is pivotal for survival and a favorable outcome.

A 51-year-old female with mixed connective tissue disease presented with bilateral sensorineural hearing loss. The hearing deficit was gradually progressive over a period of 6 months. On evaluation, no obvious cause for hearing was evident. Due to ototoxic potential of hydroxychloroquine, we decided to stop the drug and observe for improvement. Over time, her hearing loss stabilized, with improvement in audiometric findings. Hydroxychloroquine induced auditory toxicity is rare. Increased awareness and early recognition may minimize damage.

Down syndrome (DS) is the leading cause of genetically defined intellectual disability. Although speech and language impairments are salient features of this disorder, the nature of these phenotypes and the degree to which they are exacerbated by concomitant oromotor dysfunction and/or hearing deficit are poorly understood. Mouse models like Ts65Dn, the most extensively used DS animal model, have been critical to understanding the genetic and developmental mechanisms that contribute to intellectual disability. In the present study, we characterized the properties of the ultrasonic vocalizations (USVs) emitted by Ts65Dn males during courtship episodes with female partners. USVs emitted by mice in this setting have been proposed to have some basic correlation to human speech. Data were collected and analyzed from 22 Ts65Dn mice and 22 of their euploid littermates. We found that both the minimum and maximum peak frequencies of Ts65Dn calls were lower than those produced by euploid mice, whereas the mean individual duration of “down” and “complex” syllable types was significantly longer. Peak, minimal and maximal, and the fundamental frequencies of short syllables generated by Ts65Dn mice were lower compared to those by euploid mice. Finally, Ts65Dn males made fewer multiple jumps calls during courtship and the mean total duration of their “arc”, “u”, and “complex” syllables was longer. We discuss the human correlates to these findings, their translational potential, and the limitations of this approach. To our knowledge, this is the first characterization of differences between adult Ts65Dn and euploid control mice with respect to USVs.

BACKGROUND: CSOM is a common disorder which causes hearing deficit. It can be due to perforation of tympanic membrane or ossicular erosion. AIM: To study the ossicular erosion and audiological profile in patients with CSOM. MATERIALS AND METHODS: A total of 30 patients with CSOM who underwent surgery were included in the study. RESULTS: Hearing loss was significantly higher in males and in patients with ossicular erosion. CONCLUSION: Ossicular erosion can give rise to hearing loss of more than 60dB.

The incidence of sensorineural hearing loss ranges from 1 to 3 per 1,000 live births in term healthy neonates, and 2-4 per 100 in high-risk infants, a 10-fold increase. Early identification and intervention with hearing augmentation within 6 mo yields optimal effect. If undetected and without treatment, significant hearing impairment may negatively impact speech development and lead to disorders in psychological and mental behaviors. Hearing screening programs in newborns enable detection of hearing impairment in the first days after birth. Programs to identify hearing deficit have significantly improved over the two decades, and their implementation continues to grow throughout the world. Initially based on risk factors, these programs identified only 50-75% of infants with hearing loss. Current recommendations are to conduct universal hearing screening in all infants. Techniques used primarily include automated auditory brainstem responses and otoacoustic emissions that provide noninvasive recordings of physiologic auditory activity and are easily performed in neonates and infants. The aim of this review is to present the objectives, benefits, and results of newborn hearing screening programs including the pros and cons of universal vs. selective screening. A brief history and the anticipated future development of these programs will also be discussed.

Noise-induced Hearing Loss (NIHL) is caused by exposure to noise, leading to progressive and irreversible hearing impairment, and the dentist is susceptible to this pathology. Objective: To verify the hearing profile and possible risk factors for hearing impairment of dentists working in Family Health Units (FHU) in Joao Pessoa, PB. Methods: We carried out interviews, audiological and instrumental noise assessments with 65 dentists, in order to characterize their hearing profile, using descriptive analysis and logistic regression. Results: It was observed that 43.07% of the dentists had decrease of at least one of the typical frequencies of NIHL. Otologic symptoms cited were: hearing deficit (55%) and tinnitus (50%). The most intense instruments were high speed turbine (61%) and compressor (55%). Conclusion: It was found that many dentists have hearing symptoms and are exposed to factors causing NIHL, requiring more knowledge for the prevention of this disease. Risk factors for NIHL are: age, working hours,noise level of the compressor, sensation of hearing impairment and tinnitus. These factors can be prevented or controlled through specific strategies. DESCRIPTORS: Hearing. Hearing Loss, Noise-Induced. Noise, Occupational. Noise Effects, Noise Measurement.

Hemifacial spasm (HFS) is caused by vascular compression of the VII cranial nerve at its root exit zone from the brainstem. Microvascular decompression (MVD) is the only treatment option that offers the prospect of a definitive cure for HFS. We conducted the first systematic review on the safety and effectiveness of MVD for the treatment of HFS.English-language studies on MVD for HFS published from 2000 to present were retrieved and summarized. The primary outcomes of interest were treatment success, recurrence rate and adverse events including mortality, stroke, cerebrospinal fluid leak, facial palsy (permanent and transient) and hearing deficit (permanent and transient).Twenty-two papers representing 5685 patients treated with MVD for HFS were included in this review. Complete resolution of symptoms following MVD was reported in 91.1% of patients over a median 2.9-year follow-up period. HFS symptoms recurred in 2.4% of patients and 1.2% underwent repeat MVD during the follow-up period. Transient complications included facial palsy (9.5%), hearing deficit (3.2%) and cerebrospinal fluid leak (1.4%). Permanent complications included hearing deficit (2.3%), facial palsy (0.9%), stroke (0.1%) and death (0.1%).MVD successfully relieves HFS in approximately 9 of 10 patients with low rates of symptom recurrence. Complications of this surgery are uncommon and generally transient.

To elucidate the mechanisms responsible for deteriorated postural control in children with hearing deficit (CwHD), we measured center-of-pressure (COP) variability, mean velocity and entropy in bipedal quiet stance (feet together) with or without the concurrent cognitive task (reaction to visual stimulus) on hard or foam surface in 29 CwHD and a control group of 29 typically developing children (CON). The CwHD displayed an overall decreased postural performance as compared to the CON in the medial-lateral plane (p

BackgroundPresbyacusis is the most common type of hearing loss, oftenhaving profound effects on the quality of life in old age.The objectives of this study were:1. To analyse of the effect of demographic factors onpresbyacusis in Sri Lanka.2. To quantify the effect of non-demographic risk factors onpresbyacusis.MethodAn observational cross-sectional study was carried outinvolving all the patients who presented to the NationalHospital ENT clinic with hearing loss between April 2007 andApril 2010. Patients whose pure-tone audiometry resultswere suggestive of sensorineural hearing deficit and whosetympanometry testing did not show any middle earproblems were assessed using an interviewer-administeredquestionnaire.ResultsAmong the 567 participants studied, the mean age was63.4yrs. Fifty eight percent were females. The majority ofthe patients were from the Colombo district (59.3%),however, participants came from ten districts of Sri Lanka.Most of the study participants (64.2%) were married andliving with their partners, but 24.7% were widowed and therest were either unmarried or divorced. 6.1% hadcommunication difficulties leading to disrupted socialrelationships and 21% felt that hearing impairment (HI) hadadversely affected their psychological well-being. The onsetof impaired hearing, as perceived by the patients initiallyand later confirmed by pure-tone audiometry andtymanometry, had occurred at an average age of 62.9yrs.ConclusionThere was no significant association between presbyacusisand other demographic factors such as gender, educationlevel and marital status. A significant proportion of theparticipants had been widowed and that fact, too, canreduce their quality of life.

Thirty-three male canaries were reared as isolates in acoustic chambers. Each male's sexchromosomes and autosomal background were known to strain. The research included two experiments. One tested the hypothesis that the low-pitched songs of waterslager canaries is due to a partial hearing deficit in the high frequency range of their hearing, limiting learning high-pitched syllables. That hypothesis was falsified and the apparent cause for the low-pitched songs was uncovered in the second experiment. That experiment tutored the males with a tutor tape that offered six different canary songs to learn from. The experimental subjects included roller, waterslager, and border canaries, their hybrids and backcrosses. The selective learning of each genetically different group was closely associated with their specific genetic background, and the results revealed that roller and waterslager canaries have very different genetic mechanisms affecting their song learning, and that a waterslager, recessive, autosomal factor, when homozygous, was associated with that strain's low-pitched songs. On the other hand, the low-pitched songs of roller canaries were primarily affected by a recessive sex-linked factor. Border canary selective learning was biased toward learning the higher-pitched syllables and tours characteristic of the songs of border and other type canary strains.

Elderly persons with a physiologic hearing deficit (hearing impairment) are not necessarily socially or emotionally disturbed by the deficit in everyday life (hearing handicap). The self-perception of a hearing handicap in elderly people is a key element in seeking consultation for a hearing impairment or using hearing aids. Thus, it is important to determine the factors associated with the self-perception of a hearing handicap. The aims of the present study were to report the relation between a hearing impairment and the self-perception of a hearing handicap, and the factors associated with a self-perceived hearing handicap among a group of randomly recruited, community-dwelling elderly persons, aged 65 yr and older, in Taipei, Taiwan.A cross-sectional survey of community-dwelling elderly persons aged 65 yr and older (N = 1220) participating in an annual general purpose geriatric health examination in 2005 in Taipei. Pure-tone audiometry and a questionnaire including the Hearing Handicap Inventory for the Elderly-Screening Version (HHIE-S) were administered, after obtaining the subject's consent to participate in the study. Demographic information, lifestyle, self-report health status, and biochemical data were also collected.There was a moderate association (gamma(s) = 0.52) between hearing impairment and self-perceived handicap. Only 21.4% of the study subjects with moderate to profound hearing impairment (M4or=41 dB HL, N = 555) perceived themselves as hearing-handicapped (HHIE-S total scoreor=10). Besides hearing level, marital status (widowed) and self-perceived general health (bad or neutral) were factors that are significantly associated with a self-perceived hearing handicap among elderly subjects with moderate to profound hearing impairment. For study subjects with moderate to profound hearing impairment (M4or=41 dB HL), 5.0% of those with HHIE-S10 and 45.4% of those with HHIE-Sor=10 used or felt that they required hearing aids (chi2 test, p0.001). These data suggested that a self-perceived hearing handicap (HHIE-Sor=10) is an important indicator for referral of elderly persons for hearing-aid fitting.Our study findings, consistent with those of previous studies, demonstrated that not all elderly persons with impaired hearing function (hearing impairment) perceived a hearing deficit socially or emotionally in everyday life (hearing handicap). Marital status (widowed) and bad/neutral general health were nonaudiologically associated factors with a hearing handicap in the present study. Further, those with a self-perceived hearing handicap reported a higher rate of the use of, or requirement for, hearing aids. Because hearing deterioration is a common biologic process of aging, the results of this study can be used to identify the groups among elderly people with a greater need for hearing screening and hearing rehabilitation services.

We describe two new familial severe infantile spasm syndromes (ISSs) unrelated to Aristaless-related homeobox (ARX) gene mutation. Family A contains two male siblings each with dysmorphism, profound psychomotor delay, gastroesophageal reflux, infantile spasms, hypsarrhythmia, prominent independent central apneas, and early death. Family B contains two male siblings with dysmorphism, profound psychomotor delay, ambiguous genitalia, macular hypoplasia, neurosensory hearing deficit, gastroesophageal reflux, infantile spasms, no hypsarrhythmia, apneas, and early death in one sibling. Etiologic workup and ARX gene sequencing were negative. This indicates that several familial ISSs exist but are not genetically characterized.

The present study was carried out in order to analyze the clinical characteristics of tinnitus both in normal hearing subjects and in patients with hearing loss. The study considered 520 consecutive tinnitus sufferers. The following parameters were considered: age, sex, subjective disturbance caused by tinnitus, subjective judgment of tinnitus intensity, tinnitus laterality, tinnitus duration, tinnitus measurements, normal hearing or associated hearing loss. Among the patients considered, 223 have normal hearing while 297 have a hearing deficit. The hearing impairment was found to be in most cases of sensorineural type. The subjective discomfort is higher in presence of hearing loss. Subjects with hearing loss needed significantly higher masking levels. No evident differences in the residual inhibition (RI) result between the two groups were found. The present study confirms that tinnitus is most frequently associated with hearing loss. The characteristics of tinnitus in normal hearing subjects, except for the subjective judgment of tinnitus intensity, the pitch and the RI, are significantly different for those observed in subjects with hearing loss. The association of tinnitus and hearing deficit seems to increase the perceived severity of the symptom.

Listeners with impaired audiograms likely suffer from mixtures of peripheral hearing pathologies that include outer-hair-cell loss and the more recently discovered cochlear synaptopathy. These mixed pathologies pose challenges for current audiological practice as objective brainstem EEG metrics might not be able to isolate these different hearing deficits. By simulating different degrees of outer-hair-cell and auditory-nerve fiber loss in a functional model of the human auditory periphery, we studied which auditory brainstem (ABR) and envelope-following response (EFR) measures and stimuli are most sensitive to either type of hearing deficit. We validated our model predictions using recordings from 32 listeners with normal or elevated hearing thresholds. The simulations show that high-frequency outer-hair-cell loss steepens the ABR wave-V latency-vs-intensity curve as well as the wave-V amplitude-vs-intensity curve, such that grouping listeners according to the ratio of these metrics (i.e., the ABR growth ...

We describe an imported case of Lassa fever with both encephalopathy and bilateral sensorineural hearing deficit. Absence of fever during hospitalization, initially nonspecific symptoms, and onset of hearing deficit in a late stage of disease probably contributed to delayed diagnosis (14 days after admittance to hospital). The pathogenesis of neurological manifestations of Lassa fever is poorly understood and no specific treatment was given. A total of 118 personnel had close contact with the patient, but no secondary cases occurred. This case highlights the importance of considering Lassa fever as a differential diagnosis in patients with recent travel to endemic areas.