Balance disorders in hearing impaired children: prevalence, current possibilities of diagnostics and rehabilitation (scoping review)

Year 2000 Position Statement: Principles and Guidelines for Early Hearing Detection and Intervention Programs.

Determine the prevalence of vestibular and balance disorders in children, rate of complaints of imbalance, and odds ratio of related diagnoses. Retrospective review of pediatric health system during a 4-year period for International Classification of Diseases, 9th Revision, codes related to balance disorders. Identified records were searched for chief complaints related to balance and for codes of related otologic and neuro-otologic diagnoses. A total of 561,151 distinct patient encounters were found. Unspecified dizziness was diagnosed in 2,283 patients (0.4%). Also, 22% presented with balance complaints. Peripheral disorders were diagnosed in 159, and central disturbances were diagnosed in 109 (prevalence < 0.0002%). Cumulative prevalence of diagnoses related to balance was 0.45% (2,546/561,151). Of all patients, 5,793 (1.03%) had chief complaint related to balance, and 2,076 (35.84%) were also diagnosed with vestibular disorder. Moreover, 38% with peripheral disturbances and 21% with central disturbances had balance complaints. Odds ratio of syncope was 21× higher than the general pediatric population in patients with unspecified dizziness, and sensorineural hearing loss was 43 times higher in those with peripheral vestibular disorders. In patients with central disorders headache was 16× higher (p < 0.05). The prevalence of balance disorders in children is low. Children diagnosed with these disorders typically do not present with chief complaint related to balance. Significant associations exist between sensorineural hearing loss, syncope, and headache in children diagnosed with balance disorders.

Background: Disabling hearing loss has serious implications in a child's development as it reduces the intelligibility of speech thereby interfering with the learning process. This will negatively impact on their vocational choices.&#x0D; Study objective: To determine the prevalence of hearing loss in primary school children in the central zone of Lusaka district, Zambia.&#x0D; Methodology: 1277 children, aged 6 to 13 years, from public primary schools in the central zone of Lusaka district were randomly selected and examined. Clinical otologic assessment, tympanometry and audiometry screening were conducted on all participants. Children who failed the audiometry-screening test underwent a pure tone audiometry to determine the extent and nature of their hearing impairment.&#x0D; Results: The prevalence of hearing impairment was 11.5% consisting of conductive hearing loss (87.8%), sensorineural hearing loss (6.8%) and mixed hearing loss (5.4%). Hearing impairment was more common in male children (13.8%) compared to female children (9.3%) (P value= &lt; 0.05). Thirty six point eight percent children had ear disease. The commonest ear disease was wax impaction (66%)found in children followed by otitis media with effusion (20%), foreign body ear (4%) and chronic suppurative otitis media (2%). The odds of developing hearing impairment in children with chronic suppurative otitis media was nine times greater than that of children without chronic suppurative otitis media OR = 9.9(95% CI, 2.33- 47.43).&#x0D; Conclusions: The prevalence of hearing impairment in school children in central zone of Lusaka district is high. This study shows that chronic suppurative otitis media has a statistical significant association with hearing loss and that the male children are at a higher risk of developing hearing impairment. There is urgent need for ear and hearing care awareness, and screening programs at the community level in the central zone of Lusaka district.

The effect of saccular function on static balance ability of profound hearing-impaired children

To systematically assess the data on the prevalence and causes of hearing impairment in Africa. Systematic review on the prevalence and causes of hearing loss in Africa. We undertook a literature search of seven electronic databases (EMBASE, PubMed, Medline, Global Health, Web of Knowledge, Academic Search Complete and Africa Wide Information) and manually searched bibliographies of included articles. The search was restricted to population-based studies on hearing impairment in Africa. Data were extracted using a standard protocol. We identified 232 articles and included 28 articles in the final analysis. The most common cut-offs used for hearing impairment were 25 and 30 dB HL, but this ranged between 15 and 40 dB HL. For a cut-off of 25 dB, the median was 7.7% for the children- or school-based studies and 17% for population-based studies. For a cut-off of 30 dB HL, the median was 6.6% for the children or school-based studies and 31% for population-based studies. In schools for the deaf, the most common cause of hearing impairment was cryptogenic deafness (50%) followed by infectious causes (43%). In mainstream schools and general population, the most common cause of hearing impairment was middle ear disease (36%), followed by undetermined causes (35%) and cerumen impaction (24%). There are very few population-based studies available to estimate the prevalence of hearing impairment in Africa. Those studies that are available use different cut-offs, making comparison difficult. However, the evidence suggests that the prevalence of hearing impairment is high and that much of it is avoidable or treatable.

Introduction: Children with global developmental delay (GDD) are at greater risk to have hearing impairments. These impairments interfere with developmental progress or rehabilitation effects. Hearing impairments may be correctable and if so, may improve developmental outcomes. We aimed to study the incidence, characteristics and probable risk factors of hearing impairment in children aged six months to two years with GDD. Methods: In this prospective study, an auditory evaluation was carried out by a trained audiologist in children with GDD. Transient evoked otoacoustic emissions (TEOAE) and brainstem auditory evoked response (BERA) were assessed in these children. Hearing loss was classified based on Goodmann's classification. Results: Of 113 children with GDD assessed, hearing impairment was identified in 35 (30.9%) children. Of 35 children, 22 (62.8%) had isolated sensorineural hearing loss (SNHL). Conductive hearing loss was identified in four (11.5%) and combined hearing loss in nine (25.7%). Of 31 children with SNHL, hearing loss was bilateral in 25 (80.6%). In SNHL, hearing loss was profound in one (3.2%), severe in 14 (45%), moderately severe in three (9.6%), and moderate in five (16.2%). Hearing impairment was associated with 31.7% (20/63) children with cerebral palsy. Kernicterus was another predominant risk factor associated with SNHL. Metabolic disorders, otitis media, tuberous sclerosis, and metachromatic leukodystrophy were other conditions associated with hearing impairment. Conclusions: Hearing impairment is accompanied by about a third of children with GDD. The hearing impairment in children with GDD tends to severe SNHL type in about 45% and bilateral in about 80%.

Background The clinical course and prognosis in sensorineural hearing loss (SNHL) may be even worse if the vestibular system is also involved, especially because of near location of the anatomic structures in the inner ear. However, vestibular function in children with SNHL appears to be under-reported. The aim of the study was to assess the vestibular function and to determine the prognostic value of some etiological, audiological, and demographic (age, sex) factors in predicting a possibility of vestibular impairment for very early identification of children with vestibular deficits. Materials and methods The control group consisted of 30 children with normal hearing (17 girls and 13 boys) and the study group consisted of 50 children with varying degrees of bilateral SNHL, aged between 5 and 15 years. All of them were subjected to the following: basic audiological evaluation (pure-tone, speech audiometry), immittancemetry and auditory brainstem responses, electronystagmography (ENG), and vestibular-evoked myogenic potential (VEMP). Results Hearing impaired (HI) children showed bilateral SNHL of various degrees ranging from moderate to profound hearing loss (HL) [moderate-severe (32%), severe (18%), and profound (50%)] and of different etiologies [heredofamilial (46%), acquired (38%), not known (16%)]. Abnormal ENG findings were recorded in 64% of HI children. Abnormal caloric test findings were found in 56% of the HI children with heredofamilial cause of HL, in 84.2% with acquired HL, and in 37.5% with unknown cause. These results according to the degree of HL were 37.5, 55.5, and 76% for moderate, severe, and profound HL, respectively. It was noticed that HI children with profound degree and acquired etiology of HL had the highest abnormal caloric findings. Abnormal VEMP was found in 72% of HI children, but this percentage varied according to the different etiologies, 56.5, 84, and 75% for heredofamilial, acquired, and unknown, respectively. Bilateral saccular affections were more common than unilateral. HI children with profound HL had the highest percentage of both bilateral and unilateral saccular affections in the absence of VEMP. Conclusion Vestibular deficits occur in a significant percentage of HI children. Abnormal ENG and VEMP findings in HI children varied according to the etiology and or the degree of HL; those with acquired etiology and/or profound degree of HL had the highest abnormal scores.

Section I: Fundamentals of audiological and vestibular medicine. Anatomy of peripheral auditory and vestibular systems. Aging in the auditory and vestibular systems. Pathology of the ear. Genetics of auditory and vestibular disorders. Immunology of cochlear and vestibular disorders. The epidemiology of hearing and balance disorders. Radiology of auditory and vesitibular disease. Ethics, law and related matters. Section II: Audiology. Development and plasticity of the human auditory system. Physiology of the auditory system. Central auditory anatomy and function. Acoustics, measurement of sound and calibration. The threshold of hearing. Psychoacoustic auditory tests. Otoacoustic emissions. Human auditory electrophysiology. Measurements of inner ear fluid pressure and clinical applications. The auditory processing of speech. Speech and language development in normally hearing and hearing-impaired children. Section III: Auditory disorders and their management. Screening programs for hearing loss. Clinical and audiometric assessment of hearing. Assessment of hearing-impaired children. Otitis media with effusion in children (Glue ear). Disorders of the inner ear in children. Management of the hearing-impaired child. Cochlear implants for adults and children. Disorders of the inner ear in adults. Noise-induced hearing loss. Central auditory disorders. Audiological rehabilitation. Auditory amplification in adults. The pathophysiology and assessment of tinnitus. Management of the tinnitus patient. Aspects of the paracuses. Psychological aspects of hearing impairment and tinnitus. Surgical management of hearing impairment in audiological medicine. Section IV: Vestibular science. The pathology of the vestibular system. Function and dysfunction of the vestibular system. Effects of aging on control of stability. Vestibular influences on cardiovascular control during movement. The physiology of the vestibulo-ocular reflex. Instrumentation and principles of vestibular testing. Clinical application of vestibular laboratory testing. Posturography. Emerging technologies. Section V: Vestibular disorders and their management. Normal and abnormal balance. Normal and abnormal eye movements. Peripheral vestibular disorders and diseases in adults. Neurological causes of balance disorders. Falls. General medical causes of disequilibrium. Balance disorders in children. Psychiatric consequences of vestibular dysfunction. Medical management of balance disorders and vestibular rehabilitation. The role of surgery in the management of the dizzy patient.

In a study published in this issue of Pediatrics, Pinninti et al1 report that vestibular, balance, and gaze disorders occur in up to 45% of children born with asymptomatic congenital cytomegalovirus (cCMV) infection. This study was based on the evaluation of 40 children from among the 449 newborns diagnosed with cCMV through the CMV & Hearing Multicenter Screening (CHIMES) study, which screened >100 000 newborns from 7 medical center sites across the United States from 2007 to 2012.2 The subjects in this study represent a sample from a single CMV & Hearing Multicenter Screening study site.This study represents the largest cohort identified and systematically tested for vestibular and balance disorders in children with asymptomatic cCMV. Previous smaller studies, which included symptomatic and asymptomatic children with cCMV and hearing loss, reported balance and vestibular problems, paving the way for this study.3–6 With this study, Pinninti et al1 are the first to report disorders of gaze in cCMV. The findings are a significant contribution to our knowledge about the sequelae associated with otherwise clinically unrecognized asymptomatic cCMV. Disorders of gaze, balance, and vestibular function may subtly impact the ability of children to perform activities of daily living and influence their reading, academics, and sports performance in school. Early identification of these subtle disorders and use of functional therapies may mitigate the impact they have on the daily lives of these affected children. Future research is needed to evaluate whether all infants with cCMV should receive vestibular testing. Another important consideration is the degree to which there are differences of age, hearing loss status, race, and ethnicity in the risk of vestibular disorders, as suggested in this study. If these findings are confirmed, future work is needed to assess the underlying causes of the disparities.It is critical to recognize that cCMV is not rare. It is a globally common congenital infection, with 0.4% to 0.6% (range 0.2%–2.2%) of all newborns reported to be infected, and is a leading cause of newborn illness and childhood sequelae including hearing loss; vision loss; speech, language and developmental delays; seizure disorders; and motor disorders such as cerebral palsy. Most (85%–90%) born with cCMV are asymptomatic and thus are usually undiagnosed. However, asymptomatic cCMV may contribute more to the overall public health burden of the disease than symptomatic disease on the basis of the differences in overall incidence.7 In addition to congenital and later-onset hearing loss and auditory processing disorders,8–12 clinicians should now consider gaze, vestibular, and balance disorders may occur in children with asymptomatic cCMV.1Much work is going on to evaluate strategies for newborn screening for cCMV and to evaluate treatment strategies for infants detected with cCMV.13–16 The study by Pinninti et al1 suggests that gaze, balance, and vestibular disorders should be included as key outcomes of clinical research.17,18 Ultimately, the hope is that early cCMV detection can treat or even prevent the many now recognized outcomes of this common congenital infection.

Perioperative Hearing Impairment

The purpose of this study is to demonstrate the incidence and the pattern of post-meningitis hearing impairment. A prospective study of 68 meningitis patients was performed. The patients' hearing was evaluated immediately following acute illness and periodically checked for two years. The incidences of the initial sensorineural and conductive deafness were 26.4% and 20.6%, respectively. Two patients (3%) suffered from mixed deafness. No consistent audiometric pattern was found in the cases of sensorineural hearing loss. Serial hearing evaluations demonstrated complete recovery of all cases of conductive hearing loss with only a slight improvement in the low tones noted in one patient with sensorineural hearing loss. No late development of sensorineural loss has occurred.

Down syndrome is the most common chromosomal disorder in humans. Hearing impairment is a common feature of Down syndrome. To assess the prevalence of hearing impairment in children with DS attending the Mansoura University Children’s Hospital. The study is a descriptive cross-sectional study on 170 pediatric patients with genetically confirmed DS. Patients were recruited from the genetic outpatient clinic of the Mansoura University Children’s Hospital from October 2021 to October 2022. All infants and children were subjected to full history taking, and a lateral X-ray on the nasopharynx with open mouth and extended neck. The ears were examined and cleared from accumulated cerumen if present. The presence of middle ear pathology was assessed through an otoscopic examination of the tympanic membrane. Eustachian tube dysfunction and otitis media with effusion (OME) were assessed using tympanometry. Appropriate hearing tests including pure tone audiometry (PTA) and auditory brain stem response (ABR) were used. Conductive hearing loss (CHL) was observed in 48.8% of studied children with DS and 4.1% had sensorineural hearing loss (SNHL). Among patients with hearing impairment, 86.5% had bilateral affection. The severity of hearing loss was mild in 59.1% of patients with CHL and 71.4% with SNHL. Of the patients with CHL, HL remained stationary in 45.8%, regressed from moderate to mild HL in 15.7%, and normalized in 38.6%. SNHL was permanent in all 7 affected patients with a stationary course in 4 and a progressive nature in 3. OME, upper respiratory tract infections (URTI), and adenoid enlargement were commonly associated with CHL in infants and children with DS. Most children with DS have bilateral and mild hearing loss. HL is mostly conductive. Otitis media with effusion, adenoid enlargement, and recurrent upper RTI are common in patients with conductive hearing loss. Hearing assessment should be considered in all infants and children with Down syndrome.

Interestingly, children born with a congenital lack of normal vestibular function often have no balance disturbance at all although they may have mildly delayed motor milestones. The Moro response comprises a sudden bilateral extension of the upper limbs evoked by sudden jarring of the cot or dropping the head backwards by a few centimetres. Benign paroxysmal vertigo of childhood is not a positional vertigo and is quite different from benign paroxysmal positional vertigo. The seizure disorders are usually well controlled with anticonvulsants under the paediatric neurologist's guidance. In numerous conditions and syndromes there is sensorineural hearing loss with temporal bone anomalies. Perilymph fistulae in children are usually seen in association with temporal bone anomalies and pre-existing severe or total hearing loss in the affected ear. Refsum's disease is a disorder of lipid metabolism with pigmentary retinopathy, demyelinating neuropathy, ataxia and hearing loss. Suppurative ear disease and congenital or acquired cholesteatoma will require tympanomastoid surgery.

Evaluation of the use of a questionnaire to detect hearing loss in Kenyan pre-school children

Background: There is an increased risk of hearing impairment in permanent congenital hypothyroidism (CH). Only a few studies have explored the prevalence of hearing impairment in CH and their results vary widely. There is no data on hearing impairment in Indian children with CH. Setting and Design: Pediatric Endocrinology Clinic of a large tertiary care hospital located in Northwest India. Cross sectional study. Materials and Methods: Hearing evaluation of 77 children diagnosed with permanent CH was performed with a battery of tools that included Middle ear analysis, Pure Tone Audiometry (PTA), Behavioral Observation Audiometry (BOA) and Oto Acoustic Emissions (OAE). The choice of assessment tool was based on patient's age. Results: The mean age at diagnosis and at inclusion into the study was 3.0±2.7 years and 5.2±3.3 years respectively. The majority (63, 82%) of patients had symptoms suggestive of hypothyroidism; none complained of hearing loss. The etiological diagnosis was thyroid agenesis in 55 (71.4%), ectopia in 5 (6.5%), dyshormonogenesis in 9 (11.7%) and hypothyroidism with eutopic gland in 8 (10.3%) patients. Middle ear analysis, PTA, BOA and OAE was done in 48, 44, 43 and 53 patients respectively. Sensorineural hearing loss was detected in 1 out of 77 (1.2%) patients while conductive hearing loss was found in 5 (6.5%) patients. Conclusion: Hearing loss was present in a small proportion of patients with permanent CH. Further larger studies are required to determine the exact prevalence of hearing impairment in Indian children with permanent CH.