Sensorineural Hearing Loss In Children Research Articles

Inner Ear Anomalies in Congenital Hearing Loss: Imaging, Genetics, and Associated Syndromes

Hearing loss is a common cause of childhood disability in the United States, often substantially impacting speech and language development. Unlike adults, sensorineural hearing loss in children frequently is associated with anatomic abnormalities of the inner ear, the cochleovestibular nerve, or the auditory processing centers of the brain, which can be identified with imaging. There are >300 syndromic causes of hearing impairment, and this review discusses a few of the common syndromes that have characteristic inner ear anomalies.Learning Objectives: 1) To provide knowledge on the genetic basis of common congenital ear anomalies; 2) to identify temporal bone anomalies on imaging studies, which may indicate a particular syndrome or genetic abnormality; and 3) to increase radiologist awareness that congenital ear anomalies, in the setting of hearing loss, may not exist as isolated imaging findings and often have associations with abnormalities in other organ systems throughout the body.

Prevalence of cytomegalovirus DNAemia and genotypic distribution among childbearing mothers and neonates in Taiwan

BackgroundCongenital cytomegalovirus (CMV) infection is the leading cause of neurologic disabilities and sensorineural hearing loss in children. However, in Taiwan, there is limited information on the genotypic diversity and prevalence of perinatal CMV infection in both mothers and neonates. The aim of this study was to screen samples from both mothers and umbilical cord blood for CMV at the time of delivery and to determine the CMV genotypic distribution. MethodsBetween June 2012 and July 2015, residual maternal and umbilical cord blood samples were collected from consenting participants admitted to the Chang Bing Show Chwan Memorial Hospital in central Taiwan. The blood samples were screened for CMV DNA using real-time PCR assay, and the genotypic classification of the CMV UL55, UL144, and US28 genes was determined by sequencing and phylogenetic analysis. ResultsA total of 1282 mother-neonate paired samples were enrolled in the study, 95.3% of whom were Taiwanese. CMV DNA was detectable in 6.2% of the maternal blood samples, with a significantly higher rate noted in non-Taiwanese mothers (11.7%,p=0.027). For the 1,282 umbilical cord blood samples, CMV DNA was detectable in 5.3% of the samples. The presence of CMV DNA in maternal blood was positively associated with the presence of CMV DNA in umbilical cord blood (p=0.01). In addition, the UL55, UL144, and US28 genotypic distribution was similar between mothers and neonates. ConclusionThe prevalence of CMV DNAemia in childbearing mothers and neonates is similar and their genotypic distribution implies potential CMV infection during pregnancy.

Congenital cytomegalovirus infection and its impact on the auditory function

Congenital cytomegalovirus infection contributes substantially to the incidence of sensorineural hearing loss, which may be late-onset, progressive or fluctuating. It leads to delayed diagnostics of hearing impairments in children. The aim is to assess the frequency and time of hearing loss occurrence in children with congenital cytomegalovirus infection and to develop the audiological follow-up algorithm adapted for those children. Materials and methods: 60 children with verified congenital cytomegalovirus infection have been involved into research as the main group. 61 children, with other sensorineural hearing loss risk factors, but excluding congenital cytomegalovirus, were included into the comparison group. The age of children ranged from 2 months to 7 years old. The follow-up duration was up to 4 years. Audiological assessment included: auditory brainstem response, auditory steady state response, otoacoustic emissions, impedancometry, pure tone audiometry (its type depended on the children’s age and development level) and speech tests. Results: Hearing loss was revealed in 17% of the main group, it varied from mild to severe degree. Among these children 30% developed late-onset hearing loss. 5% of children of the comparison group were identified with congenital hearing loss. Assessment of central auditory pathways function has been performed in children elder than 4 years old: 70% of children of the main group and 10% of children of the comparison one were identified with auditory processing disorders. The received data may be used by otolaryngologists, audiologists, pediatricians and infectious diseases physicians. The developed follow-up admits timely diagnostics of sensorineural hearing loss in children with congenital cytomegalovirus.

Functional brain alterations following mild-to-moderate sensorineural hearing loss in children.

Auditory deprivation in the form of deafness during development leads to lasting changes in central auditory system function. However, less is known about the effects of mild-to-moderate sensorineural hearing loss (MMHL) during development. Here, we used a longitudinal design to examine late auditory evoked responses and mismatch responses to nonspeech and speech sounds for children with MMHL. At Time 1, younger children with MMHL (8-12 years; n = 23) showed age-appropriate mismatch negativities (MMNs) to sounds, but older children (12-16 years; n = 23) did not. Six years later, we re-tested a subset of the younger (now older) children with MMHL (n = 13). Children who had shown significant MMNs at Time 1 showed MMNs that were reduced and, for nonspeech, absent at Time 2. Our findings demonstrate that even a mild-to-moderate hearing loss during early-to-mid childhood can lead to changes in the neural processing of sounds in late childhood/adolescence.

Pediatric Autoimmune Inner Ear Disease: A Rare, But Treatable Condition.

Autoimmune inner ear disease (AIED) is a rare, but treatable cause of sudden sensorineural hearing loss in children. Most cases present acutely and involve both ears. The precise mechanism of hearing loss in AIED is not known. Many suspected etiologies have been proposed including infections, vascular abnormalities, and trauma. However, 70% of cases are defined as idiopathic. There are no standardized diagnostic criteria for AIED, and the diagnostic process may be challenging. Positive auto antibodies and response to immunosuppressive therapy support the diagnosis. Treatment may include corticosteroids and steroid-sparing immunosuppressive medications. A high index of suspicion is recommended as the hearing loss may be stabilized or even reversed with early treatment. Long-term medical treatment failures generally have good outcomes with cochlear implantation. [Pediatr Ann. 2019;48(10):e391-e394.].

The etiological evaluation of sensorineural hearing loss in children

This study aims to evaluate the etiology of pediatric sensorineural hearing loss (SNHL). A total of 423 children with SNHL were evaluated, with the focus on the determination of causative genetic and acquired etiologies of uni- and bilateral SNHL in relation to age at diagnosis and severity of the hearing loss. We found that a stepwise diagnostic approach comprising of imaging, genetic, and/or pediatric evaluation identified a cause for SNHL in 67% of the children. The most common causative finding in children with bilateral SNHL was causative gene variants (26%), and in children with unilateral SNHL, a structural anomaly of the temporal bone (27%). The probability of finding an etiologic diagnosis is significantly higher in children under the age of 1 year and children with profound SNHL.Conclusions: With our stepwise diagnostic approach, we found a diagnostic yield of 67%. Bilateral SNHL often has a genetic cause, whereas in unilateral SNHL structural abnormalities of the labyrinth are the dominant etiologic factor. The diagnostic yield is associated with the age at detection and severity of hearing loss: the highest proportion of causative abnormalities is found in children with a young age at detection or a profound hearing loss.What is Known:• Congenital sensorineural hearing loss is one of the most common congenital disorders• Determination of the cause is important for adequate management and prognosis and may include radiology, serology, and DNA analysisWhat is New:• Using a stepwise diagnostic approach, causative abnormalities are found in 67% both in uni- and bilateral SNHL, with the highest diagnostic yield in very young children and those suffering from profound hearing loss• Bilateral SNHL often has a genetic cause, whereas in unilateral SNHL structural abnormalities of the labyrinth are the dominant etiologic factor

Etiological factors of sensorineural hearing loss in children after cochlear implantation

Introduction: Congenital sensorineural hearing loss is one of the most common sensory defects affecting 1-3 children per 1000 newborns. There are a lot of causes which result in congenital hearing loss, the most common is the genetic origin, but infection, cochlear malformation or other acquired causes can be reasons as well. Aim: The aim of this study was to establish the etiological factors of congenital profound sensorineural hearing loss in children who underwent cochlear implantation. Results: Our results show that the origin of the hearing loss was discovered in 62.9% of our patients. The most common etiological factor was the c.35delG mutation of the gap junction protein β-2 gene, the allele frequency was 38.7% in our cohort. Infection constituted to 10.1%, and meningitis and cytomegalovirus infection were the second most common cause. 79.9% of our patients received sufficient hearing rehabilitation before the end of the speech development's period (6 years old), but 11.2% of our cases were still diagnosed late. Conclusions: Based on our data we can state that genetic evaluation is crucial in the diagnostic process of congenital profound sensorineural hearing loss. Sufficient hearing rehabilitation affects the whole life of the child, and by late cochlear implantation the speech development falls behind. We can decrease the ratio of the late implantation with the new protocol of newborn hearing screening, and with sufficient information provided to the colleagues, so the children may be referred to the proper center for rehabilitation without delay. Orv Hetil. 2019; 160(21): 822-828.

Age of identification of sensorineural hearing loss and Characteristics of affected children: Findings from two cross-sectional studies in Saudi Arabia

ObjectivesTo identify the average age of identification (AOI) and characteristics of Saudi children with sensorineural hearing loss (SNHL). MethodsTwo cross-sectional studies were undertaken. Study A: the medical records of 1166 children aged 0–10 years old who visited the audiology clinics in four hospitals in Riyadh and Dammam during 2015 were reviewed. Study B: 174 carers of children aged 0–12 years who visited the audiology clinics in four hospitals in Riyadh during a three-month period were surveyed. ResultsThe mean AOI with SNHL in children was 3.2 years (SD = 2.5 years) and 3.1 years (SD = 2.6 years) with 14% and 16% not identified until after primary school age for Studies A and B, respectively. The presence of SNHL was positively associated with parental consanguinity, positive family history of SNHL, history of chemotherapy treatment, brain pathology and prior parental concern regarding their child's hearing. ConclusionAOI of SNHL among Saudi children is deemed high in relation to the likely age of onset, with about 15 in 100 children identified after school age. Childhood hearing screening programmes (at birth and at school entry) should be considered in order to intervene earlier.

Sudden Sensorineural Hearing Loss in Children: Clinical Characteristics, Etiology, Treatment Outcomes, and Prognostic Factors.

Objective:To investigate the clinical characteristics, etiology, treatment outcomes, and prognostic factors of sudden sensorineural hearing loss (SSNHL) in children to guide the clinical diagnosis and treatment of SSNHL in the pediatric population.Study Design:Retrospective case review.Setting:Tertiary referral center.Patients:Patients diagnosed with SSNHL from November 2011 to December 2017 with relatively complete clinical data.Intervention:Diagnosis and systemic treatment of SSNHL.Main Outcome Measures:Patients’ clinical characteristics, etiology, laboratory tests, imaging, pure-tone audiometry at admission, and discharge were analyzed.Results:A total of 25 children and 149 adults with SSNHL were included. Recent or previous viral infection rates (81.8%) and fasting blood glucose level (5.23 + 1.47 mmol/L) in children with SSNHL were lower than those in adult SSNHL patients (p = 0.033, p = 0.033). Autoimmune abnormalities (90.0%) and plasma fibrinogen abnormalities (27.3%) were higher in children with SSNHL than those in adult SSNHL patients (40.0%, 8.8%, respectively, p < 0.05). The recovery rate in children (38.4%) with SSNHL is comparable to that in adults (22.6%), but children have a higher complete rate compared to adults (26.9%, 11.3%, respectively, p < 0.05). Children with a profound audiometric curve had a worse prognosis in comparison to other types of audiometric curves (p = 0.041).Conclusions:Children with SSNHL have a lower rate of viral infection in comparison to adults with SSNHL. Fasting blood glucose levels, complement C3, C4, and fibrinogen may be closely related to childhood SSNHL. The recovery rate in children with SSNHL is comparable to that in adults, but children have a higher complete rate compared to adults. A profound hearing curve is an unfavorable prognostic factor in both children and adults with SSNHL.

Auditory Brainstem Implantation: An Overview

An auditory brainstem implant (ABI) is a surgically implanted central neural auditory prosthesis for the treatment of profound sensorineural hearing loss in children and adults who are not cochlear implant candidates due to a lack of anatomically intact cochlear nerves or implantable cochleae. The device consists of a multielectrode surface array which is placed within the lateral recess of the fourth ventricle along the brainstem and directly stimulates the cochlear nucleus, thereby bypassing the peripheral auditory system. In the United States, candidacy criteria for ABI include deaf patients with neurofibromatosis type 2 (NF2) who are 12 years or older undergoing first- or second-side vestibular schwannoma resection. In recent years, several non-NF2 indications for ABI have been explored, including bilateral cochlear nerve avulsion from trauma, complete ossification of the cochlea due to meningitis, or a severe cochlear malformation not amenable to cochlear implantation. In addition, growing experience with ABI in infants and children has been documented with encouraging outcomes. While cochlear implantation generally remains the first-line option for hearing rehabilitation in NF2 patients with stable tumors or post hearing preservation surgery where hearing is lost but a cochlear nerve remains accessible for stimulation, an ABI is the next alternative in cases where the cochlear nerve is absent and/or if the cochlea cannot be implanted. Herein, we review ABI device design, clinical evaluation, indications, operative technique, and outcomes as it relates to lateral skull base pathology.

Childhood sensorineural hearing loss and adult mental health up to 43\u2009years later: results from the HUNT study

BackgroundHearing loss is a global public health problem putting millions of people at risk of experiencing impediments in communication and potentially impaired mental health. Many studies in this field are based on small, cross sectional samples using self-report measures. The present study aims to investigate the association between childhood sensorineural hearing loss and mental health in adult men and women longitudinally in a large cohort with a matched control group, and hearing is measured by pure-tone audiometry. Studies of this kind are virtually non-existing.MethodsThe present study combines data from two large studies; the School Hearing Investigation in Nord-Trøndelag (SHINT) carried out yearly from 1954 to 1986, and the second wave of the Nord-Trøndelag Health Study (HUNT 2) conducted from 1995 to 1997. The participants were 7, 10 or 13 years during the SHINT, and between 20 and 56 years old during HUNT 2. The total sample consisted of 32,456 participants (of which 32,104 in the reference group). Participants with a sensorineural hearing loss in SHINT of 41 dB or more were classified with moderate-severe hearing loss (N = 66), 26–40 dB as mild (N = 66) and 16–25 dB as slight (N = 220). Mental health in adulthood was measured in HUNT 2 by symptoms of anxiety and depression, subjective well-being, and self-esteem. The association between childhood sensorineural hearing loss and adult mental health was tested by means of ANOVA.ResultsThere was a significant relation between slight childhood sensorineural hearing loss and lowered subjective well-being in women (B = −.25, p = 0.038). Further investigation of the results revealed a significant association between slight hearing loss and symptoms of anxiety and depression (B = .30, p = 0.054) and between mild hearing loss and lowered self-esteem (B = .63, p = 0.024) among women aged 20–39 years. There were no significant relations between childhood sensorineural hearing loss and any of the three mental health outcomes among men.ConclusionsThis study suggests that women with slight or mild sensorineural hearing loss from childhood experience elevated levels of symptoms of anxiety and depression, lowered subjective well-being and lowered self-esteem. However, the results should be interpreted with caution due to a lack of power in some analyses.

Childhood Sensorineural Hearing Loss and Educational Attainment in Adulthood: Results From the HUNT Study.

Although the educational achievement gap between people without hearing loss and people with hearing loss is well-documented, few studies are based on large, nonclinical samples. The present study aims to investigate the educational attainment among Norwegian adults diagnosed with sensorineural hearing loss as children, compared with a matched control group of people without hearing loss. A prospective cohort design was applied. Between 1954 and 1986, the children in the first, fourth, and/or seventh grade in all primary schools in Nord-Trøndelag County participated in the School Hearing Investigation in Nord-Trøndelag, in which they underwent audiometric screening. Those with positive results had their hearing further tested by means of pure tone audiometry at 0.25, 0.5, 1, 2, 4, and 8 kHz with air- and bone-conduction thresholds, as well as a full examination by an ear, nose, and throat (ENT) specialist. In the present study, 216 persons were classified with moderate-severe hearing loss (41 to 100 dB HL), 293 with mild hearing loss (26 to 40 dB HL), and 240 with slight hearing loss (16 to 25 dB HL). Age-matched controls were recruited from the Norwegian Health Study, which was conducted in the same county. A total of 48,606 people participated in the present study. Data on educational attainment up to 2014 was provided by Statistics Norway. Control variables comprised sex, age, mothers', and fathers' education. The relation between childhood sensorineural hearing loss and educational attainment was tested by means of multinomial logistic regression models; first for the total sample (born between 1941 and 1979), and then for two different birth cohorts born between 1941 and 1959 and between 1960 and 1979. Percentwise, the educational attainment level in general has increased, both among people without hearing loss and people with hearing loss, and especially for women. However, 27.5% of people without hearing loss obtained higher education, whereas the corresponding numbers for those with mild or moderate-severe hearing loss were 18.8%, and 21.3%, respectively. The results from the regression analyses showed that in the total sample, compared with having primary education, people with moderate-severe or mild hearing loss were about half as likely to achieve higher education as people without hearing loss (odds ratio (OR) = 0.63 and 0.49, respectively). An interaction term between sensorineural hearing loss and sex was specified but it was not significant. In the older cohort, we found a significant association between mild hearing loss and higher education (OR = 0.40), and between moderate-severe hearing loss and secondary education (OR = 0.65). In the younger cohort, there was a significant association between mild hearing loss and higher education (OR = 0.56) and between slight hearing loss and secondary education (OR = 0.61). The results from this study indicate that the achievement gap between people without hearing loss and those with hearing loss remains. Future studies should try to pinpoint what might be hindering people with slight, mild, moderate, or severe hearing loss in pursuing higher education. Parents, health personnel, institutions for higher education, and policy makers alike should take this into consideration when making plans and policies.

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

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

Sensorineural hearing loss in children with sickle cell disease

IntroductionSensorineural hearing loss (SNHL) has been reported to occur at increased frequency in the pediatric sickle cell disease (SCD) population, likely secondary to ototoxic medication regimens and repeat sickling events that lead to end organ damage. Risk and protective factors of SNHL in this population are not fully characterized. The objective of this study was to describe audiology results in children with SCD and the prevalence and sequelae of SNHL. MethodsA comprehensive clinical database of 2600 pediatric SCD patients treated at 1 institution from 2010-16 was retrospectively reviewed to identify all patients who were referred for audiologic testing. Audiologic test results, patient characteristics, and SCD treatments were reviewed. Results181 SCD children (97 male, 153 HbSS) underwent audiologic testing, with 276 total audiology encounters, ranging 1–9 per patient. Mean age at first audiogram was 8.9 ± 5.2 years. 29.8% had prior cerebrovascular infarct and an additional 25.4% had prior abnormal transcranial Doppler screens documented at time of first audiogram. Overall, 13.3% had documented hearing loss, with 6.6% SNHL. Mean pure tone average (PTA) among patients with SNHL ranged from mild to profound hearing loss (Right: 43.3 ± 28.9, Left: 40.8 ± 29.7), sloping to more severe hearing loss at higher frequencies. ConclusionsHearing loss was identified in a significant subset of children with SCD and the hearing loss ranged from normal to profound. Though the overall prevalence of SNHL in SCD patients was low, baseline audiology screening should be considered.

Risk factors associated with the diagnosis of Sensorineural Hearing Loss in children

Purpose: The hearing loss is the most frequent sensory alteration of the human being, with numerous medical, social, emotional and cultural implications.

Prevalence and etiology of sensorineural hearing loss in children with down syndrome: A cross-sectional study

BackgroundThe prevalence and causes of sensorineural hearing loss (SNHL) in children with Down syndrome (DS) are poorly delineated. ObjectiveTo describe the prevalence, severity, laterality and underlying etiology of SNHL in a cohort of children with DS. MethodsA cross-sectional study was performed among all children with DS followed at the multidisciplinary Downteam of the Antwerp University Hospital. Patients' characteristics, risk factors for hearing loss, audiometric data and results of an etiological work-up were collected. ResultsAmong 291 patients in follow-up, 138 patients (47.4%) presented with hearing loss. In the majority this was caused by middle ear effusion and only 13 patients (4.5%) had sensorineural hearing loss, 7 boys and 6 girls with a mean age of 14.4 ± 7.4 years. Hearing loss was bilateral in 8 cases. Hearing loss severity was graded as mild in 38.5%, moderate in 30.8% and profound in 30.8% of the patients. An etiological work-up was completed in 9 children. Four patients presented with single sided deafness due to cochlear nerve deficiency. One patient had a genetic cause and in 2 patients the hearing loss was attributed to excessive noise exposure. The etiology of hearing loss was unknown in 6 patients. ConclusionSensorineural hearing loss is uncommon in children with DS with a prevalence of 4.5%. Etiological work-up may allow identifying a specific underlying cause. Cochlear nerve deficiency was found in 4 children with DS and single sided deafness.

Cochlear Implantation: An Overview.

A cochlear implant (CI) is a surgically implanted device for the treatment of severe to profound sensorineural hearing loss in children and adults. It works by transducing acoustic energy into an electrical signal, which is used to stimulate surviving spiral ganglion cells of the auditory nerve. The past 2 decades have witnessed an exponential rise in the number of CI surgeries performed. Continual developments in programming strategies, device design, and minimally traumatic surgical technique have demonstrated the safety and efficacy of CI surgery. As a result, candidacy guidelines have expanded to include both pre and postlingually deaf children as young as 1 year of age, and those with greater degrees of residual hearing. A growing proportion of patients are undergoing CI for off-label or nontraditional indications including single-sided deafness, retrocochlear hearing loss, asymmetrical sensorineural hearing loss (SNHL) in adults and children with at least 1 ear that is better than performance cut-off for age, and children less than 12 months of age. Herein, we review CI design, clinical evaluation, indications, operative technique, and outcomes. We also discuss the expanding indications for CI surgery as it relates to lateral skull base pathology, comparing CI to auditory brainstem implants, and address the concerns with obtaining magnetic resonance imaging (MRI) in CI recipients.

The Influence of Hearing Aid Gain on Gap-Detection Thresholds for Children and Adults With Hearing Loss.

The objective of this experiment was to examine the contributions of audibility to the ability to perceive a gap in noise for children and adults. Sensorineural hearing loss (SNHL) in adulthood is associated with a deficit in gap detection. It is well known that reduced audibility in adult listeners with SNHL contributes to this deficit; however, it is unclear the extent to which hearing aid amplification can restore gap-detection thresholds, and the effect of childhood SNHL on gap-detection thresholds have not been described. For adults, it was hypothesized that restoring the dynamic range of hearing for listeners with SNHL would lead to approximately normal gap-detection thresholds. Children with normal hearing (NH) exhibit poorer gap-detection thresholds than adults. Because of their hearing loss, children with SNHL have less auditory experience than their peers with NH. Yet, it is unknown the extent to which auditory experience impacts their ability to perceive gaps in noise. Even with the provision of amplification, it was hypothesized that children with SNHL would show a deficit in gap detection, relative to their peers with normal hearing, because of reduced auditory experience. The ability to detect a silent interval in noise was tested by adapting the stimulus level required for detection of gap durations between 3 and 20 ms for adults and children with and without SNHL. Stimulus-level thresholds were measured for participants with SNHL without amplification and with two prescriptive procedures-the adult and child versions of the desired sensation level i/o program-using a hearing aid simulator. The child version better restored the normal dynamic range than the adult version. Adults and children with NH were tested without amplification. When fitted using the procedure that best restored the dynamic range, adults with SNHL had stimulus-level thresholds similar to those of adults with normal hearing. Compared to the children with NH, the children with SNHL required a higher stimulus level to detect a 5-ms gap, despite having used the procedure that better restored the normal dynamic range of hearing. Otherwise, the two groups of children had similar stimulus-level thresholds. These findings suggest that apparent deficits in temporal resolution, as measured using stimulus-level thresholds for the detection of gaps, are dependent on age and audibility. These novel results indicate that childhood SNHL may impair temporal resolution as measured by stimulus-level thresholds for the detection of a gap in noise. This work has implications for understanding the effects of amplification on the ability to perceive temporal cues in speech.

Demography and etiology of congenital sensorineural hearing loss in children

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; The prevalence of sensorineural hearing loss (SNHL) is 2-3 per 1000 live births in India, and out of 1000 babies, 1 is having profound degree of hearing loss at birth or in the pre-lingual age group. Hearing loss is categorized as syndromic or non-syndromic hearing loss. The present study was done to identify and diagnose children with SNHL and study the etiological profile, so that all necessary timely precautions may be taken as SNHL in pediatric age group affects not only development of speech and language, but also emotional, social and behavioural development.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; A cross-sectional observational study was carried out in Department of ENT of a tertiary care centre, in 50 children with congenital SNHL. Data on epidemiological and etiological factors were collected. All cases were analyzed for detailed history and underwent clinical and audiological assessment. &lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; Majority of cases presented between 2 and 4 years of age, however age of detection of congenital deafness by parents was most commonly observed in 6-12 months of age. Out of 50 cases, the maximum number of cases [14 (28%)] were having history of antenatal complications, 11 (22%) cases were having adverse perinatal and postnatal history, while in remaining cases, the etiological factors remained undetermined. In one case associated syndrome was observed (Van der Hoeve’s syndrome).&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; The increased occurrence of etiologies in this study was of prenatal origin, followed by perinatal origin. The present study conducted at a tertiary centre reflects the need of 'High risk' registry maintenance with active surveillance and subsequent screening for early detection of hearing loss.&lt;/p&gt;&lt;p class="abstract"&gt; &lt;/p&gt;

Safety of Autologous Umbilical Cord Blood Therapy for Acquired Sensorineural Hearing Loss in Children.

Background and ObjectivesSensorineural hearing loss (SNHL) in children is associated with neurocognitive morbidity. The cause of SNHL is a loss of hair cells in the organ of Corti. There are currently no reparative treatments for SNHL. Numerous studies suggest that cord blood mononuclear cells (human umbilical cord blood, hUCB) allow at least partial restoration of SNHL by enabling repair of a damaged organ of Corti. Our objective is to determine if hUCB is a safe treatment for moderate to severe acquired SNHL in children.Subjects and MethodsEleven children aged 6 months to 6 years with moderate to severe acquired SNHL were treated with intravenous autologous hUCB. The cell dose ranged from 8 to 30 million cells/kg body weight. Safety was assessed by measuring systemic hemodynamics during hUCB infusion. Infusion-related toxicity was evaluated by measuring neurologic, hepatic, renal and pulmonary function before and after infusion. Auditory function, auditory verbal language assessments and MRI with diffusion tensor imaging (DTI) were obtained before and after treatment.ResultsAll patients survived, and there were no adverse events. No infusionrelated changes in hemodynamics occurred. No infusion-related toxicity was recorded. Five subjects experienced a reduction in auditory brainstem response (ABR) thresholds. Four of those 5 subjects also experienced an improvement in cochlear nerve latencies. Comparison of MRI with DTI sequences obtained before and after treatment revealed increased fractional anisotropy in the primary auditory cortex in three of five subjects with reduced ABR thresholds. Statistically significant (p<0.05) reductions in ABR thresholds were identified.ConclusionsTIntravenous hUCB is feasible and safe in children with SNHL.