Search

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

Objective: to evaluate the effect of antioxidant vitamins on hearing loss in diabetic patients. Patients and methods: Sixty type 2 diabetic patients with positive pure tone audiogram (PTA) enrolled in the study and divided into two groups: each group consists of thirty patients, group one was put on antioxidant therapy, and group two on placebo therapy, for 2 months duration. Thirty healthy individuals were kept as a control. Fasting blood sugar (FBS), and malondialdehyde (MDA) were measured in all groups. MDA and PTA were repeated after therapy. Results: The FBS and MDA are higher in diabetic patients, and beneficial effects of the administration of vitamin E and C combination on the oxidative stress and significant improvement, with mean differences of 12.5±7.79 decibel (dB), in the sensorineural hearing loss (SNHL) in diabetic patients were obtained. Conclusion: the present study demonstrated that diabetic patients have exaggerated oxidative stress, and administration of vitamin E and C combination had beneficial effect on the oxidative stress, and on the SNHL in diabetic patients with mean differences of 12.5±7.79 dB (p

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.

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

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.

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.

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.

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.

Introduction: Developmental speech and language disorders (DLD) constitute a group of disorders when children with normal intelligence and hearing fail to develop language in an age-appropriate manner. There is no definite or surrogate neurophysiologic laboratory marker to quantitate the extent of speech and language impairment. The current study was designed to evaluate the abnormalities in Auditory Brainstem Evoked Responses (ABER) in children with speech and language impairment who do not have a hearing deficit or autism. Materials and Methods: ABER recording was done in a cohort of 94 children (age 2-8 y) with DLD without overt hearing deficit or autism. The mean latencies for waves I, II, III, IV and V along with inter peak latencies for I-III, I-V, III-V and amplitude ratio of wave V/I was measured after click stimulus with intensities 110 db until 40 db and compared to age appropriate normograms. Results: The peak latencies for waves I, III & V, inter-peak latencies I-III & I-V, III-V and wave amplitude ratio V/I was found within normal limits in both ears of all the children when compared to age appropriate normograms. Conclusion: The current study therefore emphasizes the fact that ABER may not be used/recommended as diagnostic or prognostic tool in children with speech and language impairment without autism or hearing deficit. The results and the recommendations of this study will definitely reduce the burden on electrophysiologist, laboratories and also save time and financial resources.

In this study, the Audioscan test has been used to detect early signs of hearing abnormalities in 80 patients with King-Kopetzky syndrome. A significantly higher prevalence of Audioscan notches between 500 and 3,000 Hz was found for each age band and gender in patients with King-Kopetzky syndrome than in control subjects. This indicates that Audioscan notches between 500 and 3,000 Hz may represent a fine hearing deficit as an indicator of mild auditory dysfunction in patients with King-Kopetzky syndrome. However, there was no significant difference in the percentage of notches in the 3,001-8,000 Hz frequency band between the King-Kopetzky syndrome and control groups.

Background: Noise pollution or noise is an unwanted sound which is disturbing to human beings.However small or soft the sound, if it is undesirable it is considered as noise. Noise induced hearingloss is a sensorineural hearing loss that is commonly encountered second to presbycusis. Purpose: Toknow the effect of traffic noise exposure on hearing impairment to the employees of the Parking DistrictCompany of the Denpasar city and to improve diagnostic detection on hearing impairment caused bynoise. Method: A cross sectional study was conducted at the Parking District Company office. Thepopulations of this study were the employees of the Parking District Company. Samples of this study were the employees who were exposed to traffic noise and control samples were an employee who was unexposed. Samples were selected by simple random sampling. Results: From 40 parking attendants,27 persons (67.5%) aged above 35 years old. The parking attendants who had been working for ten to fifteen years were 36 persons (90%) and no history using ear protection when working. Seven persons(17.5%) had referred DPOAE upon examination with increase hearing threshold on audiogram result.In this study the parking attendants who had hearing deficit induced by noise were 7 persons (17.5%)and only one person (2.5%) in control group. There was a statistically significant effect of traffic noiseto hearing function deficit (p

Objectives:Soft surgical techniques are increasingly being used in an attempt to preserve residual low frequency hearing in adults undergoing cochlear implantation. We describe our experience of cochlear implantation with hearing preservation in adolescents.Methods:Attempts at cochlear implantation with hearing preservation in adolescents were identified from the Manchester Auditory Implant Centre, UK. A retrospective case note review was performed, with emphasis on: functional hearing deficit pre-operatively, surgical technique, age at implantation, type of electrode implanted, and functional hearing ability at sequential follow up. Functional outcomes were evaluated with the aim of evaluating both the potential benefit of preserving residual low-frequency hearing and the maintenance of preserved hearing over time.Results:Sixteen adolescents (10 girls, 6 boys) received 18 cochlear implants over a 5 year period. The average age at implantation was 13 years, 5 months (range 10.5 to 17 years, 11 months). Av...

The incidence of sensorineural hearing loss among infants in the neonatal intensive unit (NICU) is higher than in normal infants. This study determined the rate of hearing loss in healthy newborns and in NICU patients before hospital discharge at a single institution in the Eastern region of the United Arab Emirates; 96.5% of all eligible infants were screened. Hearing deficit was diagnosed in 25/13 854 healthy newborns (0.18%; 95% CI: 0.12%-0.27%) and 14/826 infants in the NICU (1.7%; 95% CI: 0.9%-2.8%). Although hearing impairment was significantly more common in those admitted to the NICU (RR = 9.4; 95% CI: 4.9-17.9), healthy newborns accounted for 25 of the 39 cases with hearing loss. The rate of congenital hearing deficit was comparable to international data. Universal screening is recommended since selective screening of high-risk infants missed two- thirds of newborns with hearing loss.

The results of analysis of auditory brain- stem evoked responses (ABRS) are reported in 173 patients with delayed speech (DS). The mean age of the patients is 4.6 years (age ranges from 1.4 years to 10 years). The patients were classified into 5 groups based on ABR findings: Group I (62 patients) had normal hearing threshold and peak- interpeak latencies. The mean amplitude of wave I was however, not significantly low (p < 0.03). Group II (27 patients) had an increased hearing threshold (40 dB), mild delay in the mean absolute peak latency of wave I (p < 0.03), decreased I– IV interval (p < 0.03), but highly significant reduction of wave I amplitude (p < 0.004). There is also a significant latency delay (p < 0.001) and amplitude reduction (p < 0.05), when this wave is compared with that a Group I (as control). These observations are suggestive of mild degree of peripheral hearing deficit in this group. Group III (49 patients) had gross ABR abnormalities of various nature and hence may be sub- grouped into (a) SNHL cochlear type (55%) (b) SNHL retrocochlear type (4%) and (c) severed degree of SNHL undecisive group (41%). Ten patients (2.7%) among the sub- group (a) had unilateral hearing loss and another 3 had Down’s syndrome. Group IV (conductive deafness) had an increased hearing threshold and shifting of ABR waves towards right with normal I– V interval. Only 6 patients were found in this group. It may be that conductive deafness is less important as a cause. Group V (29 patients) had no responses at repeated ABR studies even at higher intensity of 95 dB, the ABR studies of this group correlates with the clinical evaluation of profound deafness. The delayed speech development in 84 patients (from Groups III, IV and V) may be caused by severe degree of hearing deficit as indicated by marked ABR abnormalities. If the mild peripheral hearing loss in Group II is added to the above groups, ABRs could identify 64.6% of our patients with hearing deficit. Hence, ABR test is most reliable and sensitive diagnostic test in detecting hearing loss, a common cause of delayed speech development in children.

Previous research has shown that elderly adults often exhibit intact priming effects on visual implicit memory tests, but little is known about auditory priming and ageing. We examined priming effects on auditory stem-completion and filter identification tasks in older and younger adults. Young subjects showed more priming when speaker's voice was the same as study and test than when it differed, but elderly subjects failed to exhibit this voice-specific priming effect in each of five experiments. The elderly did, however, show robust nonspecific priming. We attempt to rule out hearing deficit accounts of the priming impairment and consider alternative theoretical interpretations of the effect.

We report on 7 patients (6 M, 1 F) with Coffin-Lowry syndrome who have a sensorineural hearing deficit in addition to developmental delay and characteristic facial changes. One of the patients also had a history of premature exfoliation of primary teeth. These are previously unappreciated clinical signs that may aid in the early diagnosis of Coffin-Lowry syndrome. Early diagnosis and recognition of a hearing deficit in the patient can lead to the use of hearing aids to help the patient achieve his or her full potential. These "new" clinical manifestations expand the phenotype of Coffin-Lowry syndrome and constitute an additional indication of pleiotropy.

SummaryThe majority of reports on the phenomenon of musical hallucinations suggests that this abnormality is a complication of a progressive peripheral hearing deficit. The present case report and a review of the international literature would, however, indicate that this syndrome may be the result of a dysfunction of the temporal cortex.

Brainstem acoustic evoked responses were recorded from 37 infants with cleft palate. Only 35% presented normal auditory thresholds. 27% had a bilateral 22% a unilateral hearing deficit of more than 30 dB. Controls after operative closure of the cleft showed no improvement of the conductive hearing loss.

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

BackgroundInfant crying is an important cue for mothers to respond adequately. Inappropriate response to infant crying can hinder social development in infants. In rodents, the pup-mother interaction largely depends on pup's calls. Mouse pups emit high frequency to ultrasonic vocalization (2-90 kHz) to communicate with their dam for maternal care. However, little is known about how the maternal response to infant crying or pup calls affects social development over the long term.Methodology/principal findingsHere we used mice lacking acid-sensing ion channel 3 (Asic3(-/-)) to create a hearing deficit to probe the effect of caregiver hearing on maternal care and adolescent social development. Female Asic3(-/-) mice showed elevated hearing thresholds for low to ultrasonic frequency (4-32 kHz) on auditory brain stem response, which thus hindered their response to their pups' wriggling calls and ultrasonic vocalization, as well as their retrieval of pups. In adolescence, pups reared by Asic3(-/-) mice showed a social deficit in juvenile social behaviors as compared with those reared by wild-type or heterozygous dams. The social-deficit phenotype in juvenile mice reared by Asic3(-/-) mice was associated with the reduced serotonin transmission of the brain. However, Asic3(-/-) pups cross-fostered to wild-type dams showed rescued social deficit.Conclusions/significanceInadequate response to pups' calls as a result of ASIC3-dependent hearing loss confers maternal deficits in caregivers and social development deficits in their young.

Fifty patients attending a private psychiatric practice were chosen at random and examined with an audioscope combined with an audiometer that delivers a 40-dB tone at frequences of 500, 1000, 2000, and 4000 Hz. Thirty-five (70%) had hearing impairment, and 10% had severe bilateral deficits. Deficits were more common among older patients and among those with history of physical disabilities that usually affect hearing.

Hearing deficits have been observed in rodents that are susceptible to audiogenic seizures (AGS), including the genetically epilepsy-prone rat (GEPR). AGS susceptibility can be induced in normal animals by treatments that damage the cochlea. In this study, we measured the relative degree of hearing loss in animals from the GEPR substrains that exhibit different degrees of AGS severity and examined the relationship between the deficit and the AGS severity. Auditory brain stem response (ABR) thresholds to clicks in the GEPR substrain that exhibits exclusively maximal AGS severity (GEPR-9) were significantly elevated, and latencies for ABR peaks I, III, and IV were significantly increased as compared to normal Sprague-Dawley rats. ABR thresholds for the substrain of GEPRs were even higher than those in the GEPR-9, and ABR waveforms were distorted. ABR peak IV was significantly longer than normal in the GEPR-3 substrain, as were mean interpeak intervals and central conduction times. These data indicate that significant hearing deficits occur in the GEPR-3 substrain. In non-AGS-susceptible progeny of the GEPR-9 [GEPR-0(9)], ABR thresholds were not significantly different from normal. These data along with studies of ABR thresholds in thyroid-deficient rats suggest that an inverted U-shaped relationship exists between hearing deficit and AGS severity. That is, moderate threshold elevations are associated with increasing AGS severity, but when the hearing deficit exceeds a certain level, a decrement in AGS severity occurs.

As Ménière's disease progresses, the fluctuations becomes less marked and the hearing level gradually declines. In the more established and advanced stages of the disease, a permanent and progressive hearing loss develops. It has been reported, however, that hearing acuity in some patients with Ménière's disease significantly improved after having had the disorder for many years. Such observation suggests that the inner ear pathology in late stages of Ménière's disease may not be necessarily irreversible. The purpose of this investigation was to assess whether or not various audiological evaluations including EcochG may be of value in predicting the irreversibility of hearing deficit in patients suffering from advanced stages of Ménière's disease. The audiological studies included pure tone audiogram, SISI test, Bekesy audiometry, glycerol (or urea) test, speech discrimination test, and electrocochleography. A combination of the recruiting type of response in the EcochG and the narrowing of the tracing width of continuous sound in the Bekesy pattern seemed to be indicative for irreversible hair cell damages. Patients who had a low score in the speech discrimination test as well as an absence of ABR response were suspected of having retrocochlear lesions. Our investigations suggest that the irreversibility of hearing impairment in patients with later stages of Ménière's disease may be predicted to some extent in the use of various audiological evaluations including the EcochG.

A 12-year-old boy born of a nonconsanguineous marriage presented with dry rough skin and photophobia since birth. His growth and developmental milestones were normal and there was no history of any neurological problem, hearing deficit or scarring around the hair follicles. Cutaneous examination revealed diffuse thinning of scalp hair with loss of eyebrows and eyelashes and a sandpapery texture of the skin all over the body, suggestive of ichthyosis follicularis with alopecia and photophobia syndrome.

Background The association between congenital cytomegalovirus (CMV) infection and sensorineural hearing loss (SNHL) was first described in 1964. Studies over the past four decades have further described the relationship between congenital CMV infection and SNHL in children. Objectives This manuscript will review the current knowledge of CMV-related SNHL and summarize the studies completed at the University of Alabama at Birmingham (UAB). Study Design A review of the series of studies at UAB that has led to a more detailed characterization of hearing loss due to congenital CMV infection. Results Approximately, 22%–65% of symptomatic and 6%–23% of asymptomatic children will have hearing loss following congenital CMV infection. CMV-related SNHL may be present at birth or occur later in childhood. Variability in the severity of CMV-related hearing loss ranges from unilateral high frequency losses to profound bilateral losses. Conclusions Congenital CMV infection significantly contributes to SNHL in many infant populations. Although, most children with congenital CMV infection do not develop hearing loss, it is difficult to predict which children with congenital CMV infection will develop hearing loss and, among those who do develop loss, whether or not the loss will continue to deteriorate.

Hemifacial microsomia is a rare form of craniofacial anomaly displaying abnormalities of the derivatives of the first and second branchial arches. A diagnosis of hemifacial microsomia is made if the patient presents with microtia, hypoplastic mandible, maxilla or the zygomatic arch on one side of the face. Bilateral involvement is rare. Here we present a rare form of the syndrome which presented with bilateral ear involvement and hearing deficit. Radiographs helped in diagnosing the case as hemifacial microsomia. The condition is extremely complex and heterogenous. Accurate diagnostic workup is necessary to differentiate this condition from other conditions of the first and second arch derivatives. We describe here the characteristic clinical and radiographic features which help to improve the knowledge on the disorder.

Screening for hearing loss in not a universal practice in current Indian scenario. Very few units have the required devices, manpower and awareness. As the number of neonatal units is increasing exponentially, there is an increasing desire to provide quality care and ensure intact survival. This article summarizes the pertinent research questions which need to be addressed in our country as we embark on our journey to provide hearing screening at least to NICU graduates.

The method for the diagnosis of the Pendred Syndrome in children by the Perchlorate discharge test using 123 I is described. The older child, who has the Pendred Syndrome and the obligatory hearing deficit, frequently has neither a goitre nor hypothyroidism, but other investigations (bone growth, scars and function tests) can also show changes. However a more certain diagnosis of this disorder in children is possible by the perchlorate discharge test using 123 I.

Otitis media and otitis externa are routinely and frequently diagnosed in office practice and are generally treated with few poor outcomes. They are usually easily diagnosed, and the organisms that cause them are well known. Yet, astonishingly, there remain several basic controversies in the treatment and care of individuals with these entities. Several large meta-analysis studies and multidisciplinary and specialist task forces have attempted to create a consensus on the treatment of these common disorders.

Hearing loss as a complication of non-otological surgery is rare. The majority of described cases have followed cardiac bypass surgery. To date there have been only 25 reported cases of non-otological surgery associated earing loss (NOSAHL) which have not involved cardiopulmonary bypass. Only two cases of bilateral NOSAHL have been previously described, and neither resulted in permanent severe bilateral hearing deficit. We describe a case of permanent bilateral severe hearing loss following metatarsal pinning in a patient with preexisting non-operated otosclerosis.

Background/Aim: To determine the incidence of visual impairment (VI) caused by ROP in The Netherlands in infants born between 2000 and 2009 and to determine the incidence of associated disabilities. To detect changes in data, we compared our data with data of three previous comparable studies conducted between 1975-1987, 1986-1994 and 1994-2000. Method: Data of children born between 1st of January 2000 and 31 December 2008 were retrieved from the Dutch institutes for the partially sighted and blind. Besides ophthalmologicdata, data on behavioral abnormalities, epilepsy, hearing deficit, developmental delay, and neurological handicaps were gathered. Results: Table Table Table Conclusion: There is a decrease in the incidence of VI caused by ROP in The Netherlands. Incidence of concomitant disabilities remained the same, except for a decrease in epilepsy. There was also a decrease of completely blind children due to ROP. Still 1/3 of the infants did not receive acute-phase ROP treatment.

Olfactory and hearing senses diminish with age, and cognitive dysfunction increases. The association between sensory deficits and cognitive dysfunction has not been well studied. This paper presents the results of testing a group of 50 community-dwelling patients of a family physician who developed a computer-assisted comprehensive geriatric assessment program. Olfactory dysfunction was present in 39% of subjects, with 18% being unable to detect smoke. There was no correlation between olfactory dysfunction and cognitive dysfunction. Hearing deficit, however, was found in 10% of subjects, all of whom also had either an associated olfactory or cognitive dysfunction. This study demonstrates the high prevalence of olfactory and auditory dysfunction in an ambulatory geriatric population, with a possible association between hearing deficit and cognitive dysfunction. The problem of multiple sensory deficits should be addressed in any long-term management decisions regarding elderly patients.