Confirmed Hearing Loss Research Articles

Tinnitus update: what can be done for the ringing?

Physicians will frequently encounter patients who report tinnitus. Tinnitus is a symptom whereby there is the perception of sound or sounds in the ear or head in the absence of an external source of sound. Most individuals experiencing tinnitus will have a neutral reaction to the percept, but in a small proportion of patients, tinnitus can be a debilitating symptom. When it causes burden, patients can be affected in multiple different facets of life, including impairment in sleep, hearing cognition and psychological and psychiatric well-being, often resulting in high healthcare utilisation and societal costs. Hence, chronic, disabling tinnitus is a complex condition with multifactorial causes and multiple perpetuating biopsychosocial factors. Despite efforts to increase knowledge about its pathophysiology and research into treatments, little impact on real-world clinical practice has been seen. There are no proven effective pharmacological treatments or complementary medicines specifically for chronic, disabling tinnitus. Despite this, there is a role for treating this condition through a multidisciplinary approach specifically targeting comorbid active psychiatric conditions, using hearing aids in appropriate clinical settings such as in those with a coassociated confirmed hearing loss, and specialised cognitive behavioural therapy for patients reporting bothersome tinnitus. Cognitive behavioural therapy remains the most valuable evidence-based intervention in this regard. This narrative review attempts to summarise the current understanding in terms of pathophysiology, assessment and treatment of tinnitus for the internal physician who may encounter patients with disabling, chronic tinnitus.

Hearing Screening of Newborns and Infants by Otoacoustic Emission

Introduction: This study was undertaken to assess the hearing of newborns and infants using otoacoustic emission as a screening tool. Screening of hearing impairment in infants is done before the third month of life, and intervention, if any, required by the age of 6 months is of prime importance. The aim of this study was to screen 215 newborns for hearing.Materials and Methods: About 215 neonates and infants were included after a thorough ENT examination and history taking. Two stage DPOAE was done on the newborns with 2, 3, 4, and 6 kHz frequencies in both ears. Newborns who failed the first OAE in any ear were tested for a second OAE in both ears within 15 days to 3 months.Result: Out of 215 cases, 10.2% (n = 22) babies had referred results for 1st DPOAE in which low birth weight, prematurity, respiratory distress syndrome, post-natal sepsis, and maternal anemia and pregnancy-induced hypertension were among the identified infantile and maternal risk factors respectively. For these infants, repeated DPOAE screening was done. On repeat DPOAE testing, all babies passed the DPOAE 2nd test.Conclusion: This study was an attempt to show the importance of developing a hearing screen with DPOAE when repeated appropriately. The hearing of all infants should be screened at no later than one month of age; those that do not pass screening should have a comprehensive audiological evaluation at no later than three months of age, and infants with confirmed hearing loss should receive appropriate intervention by six months of age.

Screening of New-Born through Otoacoustic Emission Device in a Rural Setting for Early Detection of Hearing Impairment - An Observational Study

BACKGROUND
 Hearing is a special sense needed for proper speech, language, mental development and academic performance. The objectives of our study were to screen neonates at rural area with otoacoustic emission device for detecting hearing impairment and develop a comprehensive rehabilitation model for hearing impaired children in rural areas. Hearing loss initial signs are subtle and therefore for early detection routine neonatal hearing screening is the most effective tool. At first otoacoustic emissions are done, failing which neonates undergo BERA as final screening method to identify hearing impairment as a confirmatory test. In a country like India where neonatal hearing tests are not a part of the routine screening program, the chances of missing early detection and possible rehabilitation are large. In this context, the current project envisages to develop a practical and pragmatic screening model for detecting early neonatal hearing impairment using otoacoustic emission technique in a rural sector reaching every door step possible.
 METHODS
 From the government health records collected for a duration of one and half years, all babies delivered in PHC were included for the screening. The babies were screened every Thursday (Immunisation day) with OAE devices by an audiologist at the PHC. The total number of infants screened were 1737. The first screening test done was otoacoustic emission, failing which patient was referred for BERA as a confirmatory test.
 RESULTS
 The total number of babies screened by OAE in our study was 1737. Out of the total infants screened, 1st OAE referrals were 327 babies. All the 327 infants were screened by OAE for the 2nd time, out of which 10 infants were referred for the 2nd consecutive time. 317 infants were passed on 2nd OAE screening. All the 10 infants who were referred for the 2nd time were subjected to BERA at Ramaiah medical Hospital. Out of these 10 infants, BERA showed severe to profound bilateral hearing loss in 7 infants (Confirmed Hearing loss). Other 3 infants had normal BERA reading hence normal hearing. The statistics showed that the overall rate of hearing loss prevalence was 4.03 per thousand.
 CONCLUSIONS
 In order to diagnose hearing loss in neonates, it is critical to identify hearing loss at birth and provide a screening test for it. OAE screening of neonates should be done at the earliest to detect hearing impairment so that hearing and speech rehabilitation can be started at the earliest. It should be done at each & every health care centre on a large-scale basis to cover all the babies.

Prevalence and Profiles of Late-Onset Hearing Loss in Preschool Children with Autism Spectrum Disorder Who Passed Newborn Hearing Screening in a South East Asian Population.

Prevalence of hearing loss in children with autism spectrum disorder (ASD) is uncertain, as it is more challenging to assess hearing function in children with developmental difficulties (DD). We aimed to determine the prevalence and profiles of hearing loss in preschool children with ASD in a Southeast-Asian population who passed newborn hearing screening. A retrospective study of preschool children with DD (ASD, Global Developmental Delay (GDD), and Speech and Language Delay (SLD)) attending the Child Development Unit (CDU) at our hospital was performed. Three hundred and thirty-three children (ASD: n = 129; GDD: n = 110; and SLD: n = 94) underwent hearing assessments. Of these, 10.8% of children (n = 36, comprising 15 with ASD, 12 with GDD and 9 with SLD) had confirmed hearing loss. Hearing loss was predominantly bilateral in children with ASD and GDD; in those with SLD, unilateral and bilateral hearing loss were equally common. Conductive hearing loss occurred as frequently as sensorineural hearing loss in children with ASD and SLD, but was the dominant subtype in those with GDD. Moderate to severe hearing loss (n = 2) was noted only in children with ASD. Children with ASD and GDD required significantly more audiology visits and procedures to obtain conclusive hearing test results, compared to those with SLD. The need to identify hearing loss and monitor for resolution is particularly important in vulnerable populations with communication deficits, such as in those with ASD.

Analysis of congenital hearing loss after neonatal hearing screening.

Neonates undergo neonatal hearing screening to detect congenital hearing loss at an early stage. Once confirmed, it is necessary to perform an etiological workup to start appropriate treatment. The study objective was to assess the different etiologies, risk factors, and hearing results of infants with permanent hearing loss and to evaluate the efficacy and consequences of the different screening devices over the last 21 years. We conducted a single-center retrospective cohort analysis for all neonatal hearing screening program referrals and performed an etiological workup in case of confirmed hearing loss. We analyzed the evolution of the etiological protocols based on these results. The governmental neonatal hearing screening program referred 545 infants to our center. Hearing loss was confirmed in 362 (66.4%) infants and an audiological workup was performed in 458 (84%) cases. 133 (24.4%) infants were diagnosed with permanent hearing loss. Ninety infants (56 bilateral and 34 unilateral) had sensorineural hearing loss, and the degree was predominantly moderate or profound. The most common etiology in bilateral sensorineural hearing loss was a genetic etiology (32.1%), and in unilateral sensorineural hearing loss, an anatomical abnormality (26.5%). Familial history of hearing loss was the most frequently encountered risk factor. There is a significant number of false positives after the neonatal hearing screening. Permanent hearing loss is found only in a limited number of infants. During the 21 years of this study, we noticed an increase in etiological diagnoses, especially genetic causes, due to more advanced techniques. Genetic causes and anatomical abnormalities are the most common etiology of bilateral and unilateral sensorineural hearing loss, respectively, but a portion remains unknown after extensive examinations.

School Hearing Screening With a Portable, Tablet-Based, Noise-Attenuating Audiometric Headset in Rural Nicaragua.

To investigate the utility and effectiveness of a noise-attenuating, tablet-based mobile health system combined with asynchronous telehealth evaluations for screening rural Nicaraguan schoolchildren for hearing loss. Prospective population-based survey. Rural Nicaraguan communities. There were 3,398 school children 7 to 9 years of age. Diagnostic automated and manual audiometry, detailed asynchronous telehealth evaluations. Referral rates, ambient noise levels, and audiometric results as well as hearing loss prevalence, types, and risk factors. Despite high ambient noise levels during screening (46.7 dBA), no effect of noise on referral rates on automated audiometry or confirmatory manual audiometry in those who failed automated testing was seen. The overall audiometric referral rate was 2.6%. Idiopathic sensorineural hearing loss (SNHL) and cerumen impaction were the most common types of hearing loss in this population with an estimated prevalence of hearing loss (all types) of 18.3 per 1,000 children. SNHL was associated with both drug exposure during pregnancy (p = 0.04) and pesticide exposure in the home (p = 0.03). Hearing screening using a tablet-based, noise-attenuating wireless headset audiometer is feasible and effective in rural low-resource environments with moderately elevated ambient noise levels. The referral rate with noise-attenuating headsets was much lower than that previous reports on this population. In addition, manual audiometry resulted in much lower referral rates than automated audiometry. The confirmed hearing loss rate in this study is comparable to reports from other low-income countries that use some form of noise attenuation during screening. Pesticide exposure and drug exposure during pregnancy are potential causes of SNHL in this population.

Associations among hearing loss, multiple co-occurring symptoms, and quality of life outcomes in cancer survivors.

Evaluate for differences in demographic and clinical characteristics, occurrence of common symptoms, symptom severity scores, and quality of life (QOL) outcomes in survivors with (n = 155) and without (n = 118) audiometrically confirmed hearing loss. Survivors, who were recruited from throughout the San Francisco Bay area, completed the self-report questionnaires to obtain the information of demographic and clinical characteristics; the occurrence and severity of depression, anxiety, fatigue, decrements in energy, sleep disturbance, pain, and cognitive impairment; and the general and cancer-specific QOL outcomes. Parametric and non-parametric tests were used to evaluate for differences between the two survivor groups. Survivors with audiometrically confirmed hearing loss were older, more likely to be male, were more likely to be unemployed, report a lower annual household income, and had a higher comorbidity burden. Except for the severity of worst pain, no between-group differences were found in the occurrence rates for or severity of any of the symptoms. Survivors with hearing loss reported worse physical function and general health scores. While no between-group differences in symptom occurrence rates and severity scores were found, across the total sample, a relatively high percentage of survivors who were over 6years from their cancer diagnosis reported clinically meaningful levels of depression (25%), anxiety (50%), fatigue (40%), decrements in energy (70%), sleep disturbance (58%), cognitive impairment (57%), and pain (60%). Clinicians need to perform routine assessments of hearing loss, as well as common co-occurring symptoms and initiate individualized symptom management interventions.

Applications of the Grading Scales for the Detection of Ototoxicity in Children after Treatment of Neuroblastoma and Extracranial Germinal Tumor

Introduction: Advances in treatment have resulted in a significant increase in survival rates for patients cured of malignant diseases such as neuroblastoma (NBL) and extracranial germ cell tumor (GCT). NBL is one of the pediatric cancers during which potentially ototoxic cytostatic drugs (cisplatin and carboplatin) are used for treatment. Other cancers include germinal tumors, hepatoblastoma, sarcomas, and brain tumors. Often, this very aggressive treatment has a high risk of causing long-term side effects, including hearing loss. Hence, the present study aimed to evaluate the usefulness of the National Cancer Institute Common Terminology Criteria for Adverse Events (NCI CTCAE), Brock, Chang, and International Society of Pediatric Oncology (SIOP) Boston scales in terms of detecting the high-frequency nature of hearing loss induced by ototoxic drugs and monitoring hearing status in children after completion of oncological treatment. Additionally, the frequency of hearing loss in children treated for NBL and extracranial GCT was assessed, and the principles of monitoring hearing in these patients were indicated. Methods: The study group consisted of 78 patients diagnosed with NBL (n = 47) and GCT (n = 31). There were 23 boys and 24 girls in the NBL group, aged 0–16 years, and 21 boys and 10 girls in the GCT group, aged 0–18 years. The control group consisted of 54 patients who had never received oncological treatment, were not taking potentially ototoxic drugs, and appeared socially efficient in the subjective audiological assessment. Audiometric examinations and DP-acoustic otoemission measurements were performed. Additionally, impedance audiometry tests were done to exclude a possible conductive component of the hearing loss. Results: The analysis shows that ototoxicity-induced hearing loss was observed in 13.8–65.5% of children. 75.9% of patients showed hearing loss in the 16 kHz frequency range, and at least 56.8% of patients showed hearing loss in the frequency range above 12.5 kHz. Hearing impairment, relevant to speech understanding, was displayed by more than 40% of children treated for NBL and GCT. Conclusions: The confirmation of hearing loss in nearly 65% of cases in both patients indicates the necessity to monitor the long-term side effects of anticancer treatment. Acoustic otoemission measurements, the adoption of articulatory indices based on an audiogram, or the use of arbitrary ototoxicity assessment scales such as Brock, Chang, or SIOP Boston are fully justified techniques for studying ototoxicity induced by cytostatic drugs. However, they all require continuous improvement to increase their sensitivity and specificity, especially in the pediatric group.

Hearing outcomes in preterm infants with confirmed hearing loss

ObjectivesPremature infants are at high risk for hearing loss (HL). The aim of the present study is to assess the frequency of preterm infants affected by HL who experience hearing improvement over months and evaluate possible factors associated with hearing changes. MethodsThis retrospective study was conducted in a third-level referral audiologic center.Preterm infants with a confirmed diagnosis of sensorineural HL within the first 3 months of life were reassessed at 18 months corrected age using the click-evoked auditory brainstem response between January 1, 2012, and June 30, 2020. The frequency of hearing improvement and associations between possible risk factors and hearing changes were evaluated. ResultsA total of 138 preterm infants (71 male and 67 female; mean gestational age: 30+2 weeks) were assessed. The percentages of hearing improvement and hearing threshold normalization were 58.7% (81/138) and 35.5% (49/138), respectively. We observed a higher frequency of hearing improvement among preterm infants who had received exclusive breastfeeding or mixed feeding compared with those who had received exclusive infant formula (80% versus 29.3%, P < 0.001). ConclusionThis study confirms the importance of performing a long audiological follow-up and postponing the indication for cochlear implantation in children with a history of preterm birth.Because of the expression of mesenchymal stem cells and high total antioxidant capacity, breast milk might play a protective role in the auditory system of preterm infants. These findings could have important implications for clinical practice, positively impacting the long-term hearing outcomes of preterm infants.

Knowledge, Attitude, and Practice of Hearing Loss Among Physicians in Jordan

The aim of this study is to assess the knowledge, attitude, and practice of physicians regarding hearing loss and its management among children in Jordan as an example of developing countries. The cross-sectional survey composed of 18 questions was used to fulfill the aim of the study. The study population consisted of 335 physicians working in Jordan, with different specialties. The data were collected through site visits to a variety of health care facilities, as well as an online version of the survey to facilitate data collection and ensure participants' comfort. The majority of physicians (30.7%) were general practitioners or family doctors, followed by pediatricians (12.8%). Most of the physicians (88.7%) had experience ranging from 1 to 10 years; the majority of physicians (60.3%) were younger than 30 years of age. Most of the physicians identified the importance of newborn hearing screening. Physicians showed limited knowledge about the management and intervention of infants with permanent hearing loss. Only 11.5% of respondents would refer a child with confirmed hearing loss to an audiologist, and 1.9% of them would refer to a speech therapist. Even though the majority of the physicians (69.0%) reported not receiving any training to deal with deaf and hard of hearing children, they felt confident in talking with parents about hearing loss and its management. This study revealed that physicians have limited knowledge regarding hearing loss in children as well as its management and intervention. Furthermore, this study illustrates the need for more ongoing medical education programs regarding hearing loss in children.

Performance of Targeted Congenital Cytomegalovirus Screening in Newborns Failing Universal Hearing Screening: A Multicenter Study.

Cytomegalovirus (CMV) is the most frequent cause of congenital infection and ≈20% of all infected neonates present or will develop sensorineural hearing loss. Targeted congenital CMV (cCMV) screening in newborns who failed universal newborn hearing screening has been proposed as a strategy to identify neonates with both hearing loss and cCMV infection who could benefit from antiviral treatment implemented within the first month of life. To evaluate the feasibility and performance of cCMV targeted screening in a French setting. Neonates were recruited in 5 maternity centers in greater Paris. A saliva sample for CMV polymerase chain reaction (PCR) testing was collected in neonates who failed newborn hearing screening. Outcomes including CMV PCR result and confirmation of hearing loss by an otorhinolaryngologist specialist were documented. Two-hundred thirty-six newborns were included and a saliva sample was collected in 98% (231/236) of them. The result of CMV PCR was available at a median of 9 days (7-10 days) of life and in 96% of cases within the first month of life. Two neonates were infected with CMV. The result of the otorhinolaryngologist assessment was available in 75% (178/236) of cases at a median of 16 days (9-26 days). Hearing loss was confirmed in 2.8% (5/178). The 2 infected neonates had hearing loss confirmed at 5 and 8 days of life and were treated with valganciclovir at days 9 and 16, respectively. The result of this study confirms that targeted cCMV screening is feasible in these French settings.

Emerging Data from a Newborn Hearing Screening Program in Sharjah, United Arab Emirates.

Objectives Newborn hearing screening (NHS) plays a critical role in early identification of hearing loss and subsequent early habilitation. Active parental involvement influences the success of NHS, particularly the initial NHS and follow-up. The current study reports the results of an NHS program in a cohort of babies born in a tertiary care hospital in Sharjah, United Arab Emirates (UAE). Further, it explores a two-stage NHS model to reduce false responses, thereby alleviating parental anxiety. Methods Retrospective observational study was conducted for a period of five years from January 2017 to December 2020. NHS was done as a two-stage model. All the healthy newborn babies were screened using Automated Auditory Brainstem Response (AABR) by trained audiology professionals. Babies who failed the first NHS were followed up after two weeks. Further, babies that failed the follow-up NHS were sent for diagnostic hearing evaluation and intervention as necessary. Results A total of 1821 newborn babies were screened during the study period. Eighty-one percent of babies passed the initial NHS. Four hundred and twenty-three (23.22%) babies were referred on the first NHS and were followed up after 2 weeks. Among these babies, 7.03% (24) failed second NHS. Nine (37.50%) of the 24 babies were confirmed to have hearing loss in both ears. The incidence of hearing loss in our cohort was 4.94 per 1000. Confirmed hearing loss was statistically higher in boys than girls (p < 0.05) Conclusion Current study was an attempt to report the emerging NHS data as part of the implementation of an NHS program. The study findings emphasize the need for a two-stage model of NHS to rule out false responses.

Results of a Protocol for Targeted Testing of Congenital Cytomegalovirus Infection in Babies who Fail the Newborn Hearing Screen

<strong>Background and objectives:</strong> Cytomegalovirus (CMV) is a major cause of sensorineural hearing loss in children [1]. Universal screening is controversial due to the low risk of CMV-related hearing loss [2]. The prevalence of CMV related hearing loss is 0.5 in 1000 children and is thought to be responsible for 15% to 20% of moderate-to-severe hearing loss in children [3, 4]. Since 2018, New York state law requires targeted CMV testing for newborns who “refer” on the Newborn Hearing Screen (NBHS) prior to discharge from the hospital at birth [5]. This is a report of a CMV testing protocol implemented at a community hospital for newborns who “refer” on the NBHS. <strong>Methods:</strong> Clinical records of all neonates born at BronxCare Health System (BCHS) who did not pass the NBHS between March 2019 and September 2020 were retrospectively reviewed. All newborns are screened using Auditory Brainstem Response (ABR) and those who do not pass are referred for audiology evaluation. Newborns who failed NBHS were tested for CMV prior to hospital discharge using Salivary Real-time Polymerase Chain Reaction (PCR) (Quest Diagnostics). Positive results were confirmed with serum PCR. We analyzed NBHS, audiology evaluation and CMV testing results. <strong>Results:</strong> A total of 3555 newborns had NBHS performed; 44 did not pass and were referred for audiology evaluation within 2 weeks of hospital discharge. Of these 44, CMV testing was done for 37 (84%) while 7 (16%) were not tested for CMV. Of those tested, salivary PCR detected CMV in two patients: the first had negative confirmatory serum PCR test; the second was confirmed positive and treated with ganciclovir. This patient, who is confirmed positive, had inconclusive results on follow-up audiology evaluation and was referred for Early Intervention services. Of the original cohort of 44 patients who were referred to audiology; thirty-three (75%) passed audiology evaluation and eleven did not. Of these eleven, two cases were followed successfully and have confirmed hearing loss, while 9 were lost to subsequent audiology follow-up. Of the two hearing loss cases; one was the confirmed CMV positive baby who started treatment with valganciclovir, while the other was CMV negative. Of the nine newborns who were lost to audiology follow up, 8 were tested negative for CMV while one baby was not tested for CMV. <strong>Conclusion:</strong> At BCHS, the prevalence of CMV related hearing loss is 0.28/1000 as compared to the reported prevalence of 0.5/1000. Eighteen months of targeted CMV testing in newborns who did not pass the NBHS found only one confirmed case of congenital CMV infection. This suggests that targeted CMV screening based on NBHS results misses some cases of congenital CMV infection. Of the eligible newborns who failed NBHS; seven were not tested for CMV. The testing protocol will need further refinement to avoid missing patients in the future.

Better late than never? Maternal biopsychosocial predictors of late follow-up from new Jersey's early hearing detection and intervention program

ObjectivesEarly hearing detection and intervention programs are designed to mitigate consequences of hearing loss in infants. Most research examining compliance with program protocols has examined factors related to being lost to follow-up. Another group that warrants study are babies who return for follow-up outside the timelines recommended by public health organizations. This research seeks to identify maternal factors that are associated with late follow-up at the point of diagnosis of hearing loss. Study designThe sampling frame for this study included all babies born in New Jersey in a two-year period. Our final sample consisted of 716 babies who needed diagnostic evaluations and completed them. Five hundred twenty-six babies completed their exams on-time while 190 completed them late. Logistic regression was completed to identify maternal factors related to late follow-up, and additional statistics were utilized to understand characteristics of babies who were late. ResultsIn the final modeling, maternal education (OR = 0.52), WIC participation (OR = 2.11), and health insurance status (OR = 2.04) were significantly predictive of being late (X2 (6) = 77.71; p < 0.01). Mothers for whom postpartum depression (OR = 1.89) was a concern were more also likely to have babies who were late. Needing to repeat a diagnostic audiologic exam was most predictive of lateness (OR = 5.32). Over one-third of babies who had confirmed hearing loss completed their testing late. ConclusionsLate completion of diagnostic hearing tests may contribute to delays in children hitting developmental milestones in a timely manner. Low socioeconomic status mothers and those with postpartum depression may have difficulty following up with recommended hearing tests. Limitations include data quality issues inherent in using administrative data.

Universal Neonatal Hearing Screening: An Experience at Tertiary Care Hospital

Background Hearing loss among neonates is one of the important health issue in pediatric population which may remain unnoticed until the child reaches a certain age. The importance of universal early screening, diagnosis and intervention in reducing the negative impact of congenital hearing loss has been described all over the world. Objective To observe the outcome of hearing screening by Automated Auditory Brainstem Response (AABR) in newborns delivered in Dhulikhel Hospital and neonates admitted in an intensive care unit (NICU) of Dhulikhel Hospital. Method A prospective study was done in neonates who were born at Dhulikhel Hospital, Kathmandu University Hospital from February 15th, 2017 to October 30th, 2019. AABR was used for their hearing assessment within 24 hours of birth and again at about 6 weeks of age in those neonates who failed the initial test. All the neonates admitted in NICU were studied regarding the risk factors based on Joint committee on Infant Hearing. Those who failed the test for the second time were referred for detailed audiological diagnostic work up. Result The screening rate was 92.6% of the total deliveries. A total of 5517 neonates comprising of 2800 males and 2717 females were screened from total deliveries of 5956 neonates in the study period. Among them, NICU (sick) babies were 422 (7.7%) and well babies were 5095 (92.3%). Out of them, 1675 failed the test in the first screening and 374 failed in the second screening. So, the total number of referred babies in second screening was 6.7% (374) out of 5517 screened. Amongst them, well babies were 6.59% (336), out of 5095 screened and sick babies were 9% (38) out of 422 screened. Low birth weight and prematurity were found to be the commonest risk factor present among them, followed by the use of ototoxic medications, hyperbilirubinemia and prolonged use of mechanical ventilation. Conclusion Automated Auditory Brainstem Response (AABR) is a very useful tool for hearing screening which should preferably be done in all the neonates where possible. It should be done within one month of life and those with confirmed hearing loss should receive early appropriate intervention for better hearing in future.

Clinical and audiological evaluation of post traumatic hearing loss

&lt;p&gt;&lt;strong&gt;Background&lt;/strong&gt;: Post traumatic hearing loss is one of the most common problems encountered among trauma victims. It can manifest as conductive, sensorineural or mixed hearing loss. It is against this background that the study evaluated the clinical and audiological outcome of 47 patients of trauma.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods&lt;/strong&gt;: A descriptive longitudinal study was conducted over a period January 2017 to March 2018. Follow up was done after 3 months of discharge. Study consisted of 47 patients presenting with features of trauma related injuries. After carrying out systematic clinical, audiometric and radiological evaluation, patients were managed conservatively.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results&lt;/strong&gt;: Of the 47 patients studied, 89.98% were related to road traffic accidents (RTAs); and 76.60% were under the influence of alcohol at the time of trauma. Among the 32 cases of the RTAs involving two wheelers, 29 patients (90.62%) were not using protective devices like helmet. Nearly 90% of patients had temporal bone fracture. Audiological evaluation confirmed hearing loss in 77% of patients at presentation. There was significant improvement of hearing thresholds with 51% attaining normal hearing at follow up with conservative management.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions&lt;/strong&gt;: Post traumatic hearing loss was very common, conductive hearing loss being the most common type. It resolved over a few days to few weeks post injury. Timely diagnosis and management with early steroid therapy showed encouraging results for patients with traumatic sensorineural hearing loss or mixed hearing loss.&lt;/p&gt;

An Integrative Review of Current Practice Models and/or Process of Family-Centered Early Intervention for Children Who Are Deaf or Hard of Hearing.

Over the past few decades, there has been an increasing shift toward emphasizing the importance of the child's family taking an active role in the habilitation process through family-centered early intervention (FCEI) programs. Accordingly, the Health Professions Council of South Africa recommends that early intervention services following confirmation of hearing loss must be family-centered within a community-based model of service delivery that is culturally congruent. The aim of this study was to explore and document current evidence reflecting trends in FCEI for children who are deaf or hard of hearing (DHH) by identifying and describing current practice models and/or processes of FCEI for these children. This study describes our first steps in formulating a framework for FCEI for children who are DHH in South Africa. An integrative literature review was conducted. Sage, Science Direct, PubMed, and Google Scholar databases were searched for studies published in English between January 2009 and January 2019 reporting on FCEI programs for children who are DHH. Studies that focused on the following were excluded from the study: speech and language outcomes of children, youth, and adults who are DHH; education for children who are DHH; universal newborn hearing screening; professionals' roles in early hearing detection and intervention; diagnosis of hearing loss; and sign language. Kappa statistics were performed to determine agreement between reviewers. Twenty-two studies were included in the review. Cohen's kappa revealed a substantial agreement (κ = 0.8) between reviewers for data extraction and synthesis in terms of the articles that met the criteria for inclusion in the review. Findings were discussed under 5 themes: caregiver involvement; caregiver coaching/information sharing; caregiver satisfaction; challenges with FCEI; and telehealth. Generally, there is sufficient evidence for FCEI, with caregivers indicating the need for full involvement in their children's care. Methods of caregiver involvement involving caregiver coaching/information sharing need to be culturally and linguistically appropriate, with sensitivities around time and manner. This increases caregiver satisfaction with intervention programs and improves outcomes for children who are DHH. Challenges identified by the studies raise implications for early hearing detection and intervention programs, as well as Departments of Health and Social Welfare. These included logistical challenges, professional-related challenges, and caregiver-related challenges. Various aspects of FCEI have been reported in the review. Findings of these studies have significant implications for the formulation of quality FCEI programs to ensure contextually relevant and contextually responsive care of children who are DHH.

Profile of childhood hearing loss in the Western Cape, South Africa

ObjectivesTo describe the nature, associated risk factors and age of diagnosis for childhood hearing loss in a South African cohort from the Western Cape Province. MethodsA retrospective review of clinical data from children under six years of age with confirmed hearing loss at Red Cross War Memorial Children's Hospital (RCWMCH) was conducted between 1 January 2019 and 31 July 2019. Data collected included demographic information, type and degree of hearing loss, documented risk factors associated with hearing loss, and age of suspicion and diagnosis of hearing loss. ResultsThe study sample included 240 children with hearing loss, with a mean age of 42 months (21.8 SD; range 2–72). More than two thirds (68.3%) of the children presented with bilateral hearing loss. The majority presented with conductive hearing loss (64.6%), followed by sensorineural (28.7%) and mixed hearing loss (3.3%) or auditory neuropathy spectrum disorder (3.3%). More than half (51.8%) of the bilateral sensorineural hearing losses were of a profound degree. The most prominent risk factor for conductive hearing loss was otitis media, for sensorineural hearing loss it was a family history of childhood hearing loss, and for auditory neuropathy spectrum disorder it was hyperbilirubinaemia. Approximately one third of patients (27.1%) with sensorineural hearing loss did not have any associated risk factors. The mean age of diagnosis of permanent congenital or early-onset hearing loss was 31.4 months (22.8 SD; range 2–72), with a mean delay of nine months (13.2 SD; range 0–60) between age of suspicion and diagnosis of hearing loss (n = 93). ConclusionsThe large proportion of preventable hearing losses in this sample highlights the importance of maximising primary health care efforts to treat preventable causes timeously. Age of diagnosis of permanent congenital or early-onset hearing loss was severely delayed undermining prospects of positive outcomes through early intervention. Infant hearing screening services in the public health sector of South Africa should be prioritised alongside primary health care efforts to reduce preventable risks for hearing loss.

Development of in-house genetic screening for pediatric hearing loss.

ObjectivesTo evaluate the efficiency of in‐house genetic testing for mutations causing the most common types of inherited, nonsyndromic, sensorineural hearing loss (SNHL).MethodsRetrospective cohort study of 200 patients at a single, pediatric medical center with suspected or confirmed hearing loss who underwent either send out vs in‐house genetic testing for mutations in GJB2/GJB6, SLC26A4, and MTRNR1. Primary outcome measure was the difference in mean turnaround time for send‐out vs in‐house genetic testing. Additional outcomes included associations between audiometric findings and genetic test results.ResultsOne hundred four send‐out tests were performed between October 2010 and June 2014, and 100 in‐house tests were performed between November 2014 and November 2016. The mean turnaround time for send‐out testing was 53.7 days. The mean turnaround time for in‐house testing was 18.9 days. This difference was statistically significant (P < .001). The largest component of turnaround time was the amount of time elapsed between receipt of specimen in the lab and final test result. These intervals were 47.0 and 18.3 days for send‐out and in‐house tests, respectively. Notably, the longest turnaround time for in‐house testing (43 days) was less than the average turnaround time for send‐out testing. In addition, we identified two simple audiometric parameters (ie, bilateral newborn hearing screen referral and audiometry showing symmetric SNHL) that may increase diagnostic yield of genetic testing.ConclusionsThe development of in‐house genetic testing programs for inherited SNHL can significantly reduce testing turnaround times. Newborn hearing screening and audiometry results can help clinicians identify patients most likely to benefit from genetic testing.Level of EvidenceIV.

Delayed Confirmation of Hearing Loss in Infants

With required newborn hearing screening programs, the opportunity to confirm permanent hearing deficits within the first 3 months of life allows for