Hearing loss and cognition: A protocol for ensuring speech understanding before neurocognitive assessment.

INTRODUCTION: Many neurocognitive evaluations involve auditory stimuli, yet there are no standard testing guidelines for individuals with hearing loss. The ensuring speech understanding (ESU) test was developed to confirm speech understanding and determine whether hearing accommodations are necessary for neurocognitive testing. METHODS: Hearing was assessed using audiometry. The probability of ESU test failure by hearing status was estimated in 2679 participants (mean age: 81.4 ± 4.6 years) using multivariate logistic regression. RESULTS: Only 2.2% (N = 58) of participants failed the ESU test. The probability of failure increased with hearing loss severity; similar results were observed for those with and without mild cognitive impairment or dementia. DISCUSSION: The ESU test is appropriate for individuals who have variable degrees of hearing loss and cognitive function. This test can be used prior to neurocognitive testing to help reduce the risk of hearing loss and compromised auditory access to speech stimuli causing poorer performance on neurocognitive evaluation.

Congenital cytomegalovirus infection diagnostics and management.

PURPOSE OF THE REVIEW: Congenital cytomegalovirus infection (cCMV) is the most frequent congenital infection and a leading nongenetic cause of sensorineural hearing loss (SNHL) and brain disease. The purpose of this review is to highlight recent developments in the diagnosis and management of children with cCMV. RECENT FINDINGS: Progress is being made in the efforts to identify more infants with cCMV, especially those with asymptomatic infection. Largely due to efforts by various advocacy/parent groups, a number of states in the United States and many hospital systems have implemented hearing targeted CMV screening and mandated education of pregnant women about CMV. SUMMARY: cCMV is an important cause of SNHL and neurologic morbidity worldwide. Early identification of infected children is critical to improve outcomes by providing timely interventions and guidance for long-term follow up. The fact that most infants with cCMV have no abnormal clinical findings, and the need to obtain samples for diagnosis within the first 2-3 weeks of life, makes it challenging to identify a majority of infants with cCMV without universal newborn CMV screening.

Retrospective identification of congenital cytomegalovirus infection using dried blood samples - missed opportunities and lessons.

BACKGROUND: Congenital cytomegalovirus (cCMV) is the most common congenital infection in children, with a potential to cause neurodevelopmental delay and sensorineural hearing loss. Not only are most infected newborns asymptomatic at birth, even those who are symptomatic are not always diagnosed in time. Newborn dried blood-spot (DBS) specimens collected routinely at birth, have been recently used for retrospective diagnosis of cCMV. Our objective was to assess the clinical characteristics of children retrospectively diagnosed with cCMV using DBS polymerase chain reaction (PCR) testing, and report whether an early diagnosis could have been achieved. METHODS: A retrospective data collection study comprising all infants followed at a dedicated cCMV clinic diagnosed between 2014 and 2019 by the DBS PCR test RESULTS: During the study period, 436 children were born with cCMV and 19 (4.4%) were diagnosed with cCMV by the DBS PCR test. 9/19 were diagnosed before the age of 3 months; 3 of them were diagnosed after the neonatal period, although significant findings suggestive of cCMV were present at birth. 10/19 were diagnosed between 3 and 36 months of age; 6 of these 10 exhibited findings suggestive of cCMV at birth . In total, 8/19 children suffered from long-term sequela, including severe hearing loss or profound developmental delay. CONCLUSIONS: We report the numerous missed opportunities for early diagnosis and treatment of children with cCMV. Universal newborn screening for cCMV may have prevented poor hearing and developmental outcomes in 8 of the 19 children described herein.

Hearing impairment in Angolan children with acute bacterial meningitis with and without otitis media.

AIM: Bacterial meningitis (BM) is a common cause of hearing loss in childhood. Our aim was to investigate bacterial aetiology, hearing impairment and outcome in childhood BM with vs. without otitis media (OM) in Angola. METHODS: Hearing was tested by auditory brainstem response in 391 (76%) children with confirmed BM. The bacteria identified from the ear discharge were compared to those from cerebrospinal fluid (CSF). The hearing findings were compared among children with vs. without OM on days 1 and 7 of hospitalization, and at follow-ups of 1, 3 and 6 month(s). RESULTS: No correlation was found in bacteriology between the ear discharge and CSF. On day 7 in hospital, hearing impairment (>40 dB) was common, regardless of whether concomitant OM or not (in 27% vs. 30%, respectively). Any hearing deficit on day 7 was associated with a higher risk of complicated or fatal clinical course (OR 2.76, CI95% 1.43-5.29, p = 0.002). CONCLUSION: No significant difference prevailed in hearing thresholds between children with or without OM in hospital on day 7 or at later follow-ups. Any hearing impairment during hospital stay associated with a higher risk for complicated clinical course or death.

In Situ Otoacoustic Emission Monitoring to Assess the Effects of Noise Exposure on Hearing Health.

Noise-induced hearing loss (NIHL) is the largest cause of action for indemnity in North American industries despite the widespread implementation of hearing conservation programs. Possible causes behind the onset of NIHL are the intervals between hearing tests which are generally too long and the tests are insufficiently sensitive to detect temporary hearing changes to act and prevent permanent hearing damage. Moreover, current noise regulations might be too lenient as to the permissible maximum noise levels. Short-interval hearing assessment could help to observe temporary changes in hearing health and prevent permanent damage. This study investigates the short-term effects of noise exposure characteristics using repeated measurements of otoacoustic emission (OAE) growth functions and presents the most significant predictors of hearing health changes as observed in sixteen individuals equipped with OAE earpieces. The experimental results of this study show that the impulsiveness and frequency spectrum of the noise level could be a possible cause of the decline in OAE levels. As a consequence, hearing conservation programs should consider taking these noise metrics into account for proper NIHL risk assessment. Such noise exposure and hearing health monitoring could greatly improve hearing conservation practices in the workplace by acting faster and eventually mitigate occupational hearing loss.

Change to Hearing Loss-Related Risks and Screening in Preterm Infants.

Hearing loss is one of the most common congenital defects in infancy; it increases speech and language delays and adversely affects academic achievement and socialemotional development. The risk of hearing loss in premature infants is higher than that in normal newborns, and because of the fragility of the auditory nervous system, it is more vulnerable to different risk factors. The hearing screening guidelines in current use were proposed by the American Academy of Pediatrics and updated in 2007, but there are no uniform guidelines for hearing screening in preterm infants. This review focuses on the risk factors related to hearing loss in premature infants, hearing screening strategies, and reasons for failure. The aim is to provide a more comprehensive understanding of hearing development in preterm infants to achieve early detection and early intervention. At the same time, attention should be paid to delayed auditory maturation in preterm infants to avoid excessive intervention. KEY POINTS: · Hearing loss is very common in infancy, especially in premature infants.. · Genetic factors, infection, hyperbilirubinemia, drugs, and noise are the main causes.. · We should pay attention to the delayed hearing maturity of premature infants and avoid excessive intervention..

Aetiology of permanent childhood hearing loss at a population level.

AIM: To evaluate and describe results of aetiological investigations offered to a population level cohort of babies who had confirmed permanent hearing loss after they either (i) failed universal neonatal hearing screening or (ii) passed newborn screening but were detected with a permanent hearing loss in early childhood. METHODS: Descriptive analysis of results of investigations offered to neonates and young children in whom permanent hearing loss was detected as part of a statewide newborn hearing screening programme. A total of 306 285 newborns were screened between 2013 and 2017. The failed screening results were confirmed by a diagnostic audiological assessment battery. Medical evaluation for the identification of the cause of the hearing loss was performed by a paediatrician or otolaryngologist, investigations were ordered using a stepwise approach, and aetiology was assigned using a coding scheme. RESULTS: Permanent hearing loss was confirmed in 967 children (0.3%). Data were available for 873. An aetiological factor was identified or presumed in 61.3% of cases. Genetic causes were present in 26.8% and structural causes were present in 24.9% of cases. Congenital cytomegalovirus was present in 4.4%. CONCLUSIONS: Use of a coding scheme is feasible at a population level and allows collation of data from multiple sites and will allow outcome mapping and service planning.

Hearing loss and volumetric growth rate in untreated vestibular schwannoma.

OBJECTIVE: In this study, the authors aimed to clarify the relationship between hearing loss and tumor volumetric growth rates in patients with untreated vestibular schwannoma (VS). METHODS: Records of 128 treatment-naive patients diagnosed with unilateral VS between 2012 and 2018 with serial audiometric assessment and MRI were reviewed. Tumor growth rates were determined from initial and final tumor volumes, with a median follow-up of 24.3 months (IQR 8.5-48.8 months). Hearing changes were based on pure tone averages, speech discrimination scores, and American Academy of Otolaryngology-Head and Neck Surgery hearing class. Primary outcomes were the loss of class A hearing and loss of serviceable hearing, estimated using the Kaplan-Meier method and with associations estimated from Cox proportional hazards models and reported as hazard ratios. RESULTS: Larger initial tumor size was associated with an increased risk of losing class A (HR 1.5 for a 1-cm3 increase; p = 0.047) and serviceable (HR 1.3; p < 0.001) hearing. Additionally, increasing volumetric tumor growth rate was associated with elevated risk of loss of class A hearing (HR 1.2 for increase of 100% per year; p = 0.031) and serviceable hearing (HR 1.2; p = 0.014). Hazard ratios increased linearly with increasing growth rates, without any evident threshold growth rate that resulted in a large, sudden increased risk of hearing loss. CONCLUSIONS: Larger initial tumor size and faster tumor growth rates were associated with an elevated risk of loss of class A and serviceable hearing.

Screening for Hearing Loss in Older Adults: US Preventive Services Task Force Recommendation Statement.

Importance: Age-related sensorineural hearing loss is a common health problem among adults. Nearly 16% of US adults 18 years or older report difficulty hearing. The prevalence of perceived hearing loss increases with age. Hearing loss can adversely affect an individual's quality of life and ability to function independently and has been associated with increased risk of falls, hospitalizations, social isolation, and cognitive decline. Objective: To update its 2012 recommendation, the US Preventive Services Task Force (USPSTF) commissioned a systematic review on screening for hearing loss in adults 50 years or older. Population: Asymptomatic adults 50 years or older with age-related hearing loss. Evidence Assessment: Because of a lack of evidence, the USPSTF concludes that the benefits and harms of screening for hearing loss in asymptomatic older adults are uncertain and that the balance of benefits and harms cannot be determined. More research is needed. Recommendation: The USPSTF concludes that the current evidence is insufficient to assess the balance of benefits and harms of screening for hearing loss in older adults. (I statement).

Is There a Safe Level for Recording Vestibular Evoked Myogenic Potential? Evidence From Cochlear and Hearing Function Tests.

OBJECTIVE: There is a growing concern among the scientific community about the possible detrimental effects of signal levels used for eliciting vestibular evoked myogenic potentials (VEMPs) on hearing. A few recent studies showed temporary reduction in amplitude of otoacoustic emissions (OAE) after VEMP administration. Nonetheless, these studies used higher stimulus levels (133 and 130 dB peak equivalent sound pressure level [pe SPL]) than the ones often used (120 to 125 dB pe SPL) for clinical recording of VEMP. Therefore, it is not known whether these lower levels also have similar detrimental impact on hearing function. Hence, the present study aimed at investigating the effect of 500 Hz tone burst presented at 125 dB pe SPL on hearing functions. DESIGN: True experimental design, with an experimental and a control group, was used in this study. The study included 60 individuals with normal auditory and vestibular system. Of them, 30 underwent unilateral VEMP recording (group I) while the remaining 30 did not undergo VEMP testing (group II). Selection of participants to the groups was random. Pre- and post-VEMP assessments included pure-tone audiometry (250 to 16,000 Hz), distortion product OAE, and subjective symptoms. To simulate the time taken for VEMP testing in group I, participants in group II underwent these tests twice with a gap of 15 minutes. RESULTS: No participant experienced any subjective symptom after VEMP testing. There was no significant interear and intergroup difference in pure-tone thresholds and distortion product OAE amplitude before and after VEMP recording (p > 0.05). Furthermore, the response rate of cervical VEMP was 100% at stimulus intensity of 125 dB pe SPL. CONCLUSIONS: Use of 500 Hz tone burst at 125 dB pe SPL does not cause any temporary or permanent changes in cochlear function and hearing, yet produces 100% response rate of cervical VEMP in normal-hearing young adults. Therefore, 125 dB pe SPL of 500 Hz tone burst is recommended as safe level for obtaining cervical VEMP without significantly losing out on its response rate, at least in normal-hearing young adults.

Safe Use of Acoustic Vestibular-Evoked Myogenic Potential Stimuli: Protocol and Patient-Specific Considerations.

BACKGROUND: Vestibular-evoked myogenic potentials (VEMPs) are commonly used clinical assessments for patients with complaints of dizziness. However, relatively high air-conducted stimuli are required to elicit the VEMP, and ultimately may compromise safe noise exposure limits. Recently, research has reported the potential for noise-induced hearing loss (NIHL) from VEMP stimulus exposure through studies of reduced otoacoustic emission levels after VEMP testing, as well as a recent case study showing permanent sensorineural hearing loss associated with VEMP exposure. PURPOSE: The purpose of this report is to review the potential for hazardous noise exposure from VEMP stimuli and to suggest clinical parameters for safe VEMP testing. RESEARCH DESIGN: Literature review with presentation of clinical guidelines and a clinical tool for estimating noise exposure. RESULTS: The literature surrounding VEMP stimulus-induced hearing loss is reviewed, including several cases of overexposure. The article then presents a clinical calculation tool for the estimation of a patient's safe noise exposure from VEMP stimuli, considering stimulus parameters, and includes a discussion of how varying stimulus parameters affect a patient's noise exposure. Finally, recommendations are provided for recognizing and managing specific patient populations who may be at higher risk for NIHL from VEMP stimulus exposure. A sample protocol is provided that allows for safe noise exposure. CONCLUSIONS: VEMP stimuli have the potential to cause NIHL due to high sound exposure levels. However, with proper safety protocols in place, clinicians may reduce or eliminate this risk to their patients. Use of the tools provided, including the noise exposure calculation tool and sample protocols, may help clinicians to understand and ensure safe use of VEMP stimuli.

Sudden Bilateral Hearing Loss After Cervical and Ocular Vestibular Evoked Myogenic Potentials.

OBJECTIVE: Cervical and ocular vestibular evoked myogenic potentials (cVEMPs and oVEMPs) are commonly used in evaluation of neurotologic disorders. We present a case of sudden bilateral hearing loss immediately after oVEMP and cVEMP testing. The hearing loss did not recover. To our knowledge, no previous case reports discuss sudden hearing loss, especially bilateral, associated with VEMP testing. PATIENT: A single patient with sudden bilateral hearing loss that has persisted after cVEMP and oVEMP. INTERVENTION: The patient had a history of chronic daily dizziness. She underwent vestibular function testing that included cVEMP and oVEMP testing. A significant bilateral sensorineural hearing loss was noted immediately after cVEMP and oVEMP testing and confirmed with audiometric testing. Despite the use of oral steroids, her hearing loss did not recover. MAIN OUTCOME MEASURES: Serial audiograms, calculated maximum total sound energies to each ear. RESULTS: Pre-VEMP versus post-VEMP audiograms show increased thresholds and decreased word recognition scores; total sound energy delivered to each ear shows significant sound exposure. CONCLUSION: Although VEMP testing is thought to be safe and well tolerated, a significant amount of sound can be delivered to the cochlea, and certain individuals may be susceptible to acoustic trauma at these levels. We recommend limits for VEMP stimuli levels and attention to total sound exposure when multiple trials are used.

Screening for hearing loss in older adults: U.S. Preventive Services Task Force recommendation statement.

DESCRIPTION: Update of the 1996 U.S. Preventive Services Task Force (USPSTF) recommendation statement on screening for hearing impairment in older adults. METHODS: The USPSTF reviewed evidence published between 1950 and January 2010 on screening for age-related sensorineural hearing impairment in adults aged 50 years or older without diagnosed hearing loss in the primary care setting. POPULATION: This recommendation applies to asymptomatic adults aged 50 years or older. It does not apply to persons seeking evaluation for perceived hearing problems or for cognitive or affective symptoms that may be related to hearing loss. RECOMMENDATION: The USPSTF concludes that the current evidence is insufficient to assess the balance of benefits and harms of screening for hearing loss in asymptomatic adults aged 50 years or older (I statement).

Safety and clinical performance of acoustic reflex tests.

OBJECTIVE: Safety and effectiveness of acoustic reflex tests are important issues because these tests are widely applied to screen for retrocochlear pathology. Previous studies have reported moderately high sensitivity and specificity for detection of acoustic neuroma. However, there have been reports of possible iatrogenic hearing loss resulting from acoustic reflex threshold (ART) and decay (ARD) tests. This study assessed safety and clinical performance of ART tests for detection of acoustic neuroma. DESIGN: We report a case in which ARD testing resulted in a significant bilateral permanent threshold shift. This case was the impetus for us to investigate the clinical utility of ART and ARD tests. We analyzed sensitivity and specificity of ART, as well as asymmetry in pure-tone thresholds (PTT) for detection of acoustic neuroma in 56 tumor and 108 non-tumor ears. RESULTS AND CONCLUSIONS: Sensitivity and specificity were higher for PTT asymmetry than for ART. Ipsilateral ART at 1000 Hz had poor sensitivity and specificity for detection of acoustic neuroma, and involves some potential risk to residual hearing for presentation levels higher than 115 dB SPL. Approximately half of the acoustic neuroma group had ipsilateral ARTs that would require administration of ARD tests at levels exceeding 115 dB SPL. Therefore, we conclude that PTT asymmetry is a more effective test for detection of acoustic neuroma, and involves no risk to residual hearing. Future studies of contralateral reflex threshold and ARD in combination with PTT asymmetry are recommended.