Tinnitus and hyperacusis in children: clinic reports and basic research

Objective Tinnitus and hyperacusis are subjective symptoms which can be reported by people of any age. Although tinnitus and hyperacusis can have a negative effect on child development, these symptoms are commonly overlooked by their parents and clinicians. In this paper, we review clinical reports on tinnitus and hyperacusis in children and basic scientific studies on these disorders in order to provide updates of these disorders in the pediatric population. Recent studies have found that tinnitus and hyperacusis are not uncommon in children, especially in those with conductive and sensorineural hearing loss. The parents and clinicians should pay attention when children show abnormal behaviors and especially when they develop hearing loss. Since there is no objective measurement for tinnitus and hyperacusis, the diagnosis in children can be challenging. Tinnitus and hyperacusis are also common in Williams syndrome and autism but the mechanisms are still not clear. High doses of salicylate and noise exposure can induce tinnitus. Animal studies have determined lack of inhibition in the auditory cortex and the inferior colliculus may be critical for tinnitus and hyperacusis generation. The non-classic auditory system may also be involved in the awareness and tolerance of tinnitus and hyperacusis.     

Treatment of hyperacusis in Williams syndrome with bilateral conductive hearing loss

Williams syndrome (WS) is a rather rare congenital disorder characterised by a series of cardiovascular, maxillo-facial and skeletal abnormalities. It sometimes displays otorhinolaryngological symptoms because of the relatively high incidence of secretory otitis media and hyperacusis, which may be present in up to 95% of patients. The present paper describes a case of WS associated with bilateral conductive hearing loss which was not related to secretory otitis media. Hyperacusis was, moreover, present in spite of the conductive deafness. Surgical or prosthetic treatment of hearing loss was delayed because of hyperacusis. Treatment of the hyperacusis by acoustic training, instead, yielded excellent, long-lasting remission of the symptoms. Received: 14 July 2000 / Accepted: 12 April 2001     

Hyperacusis in Williams syndrome.

OBJECTIVE: To define hyperacusis in audiologic parameters and to further elucidate central and peripheral auditory pathways. DESIGN AND SETTING: Theories surrounding hyperacusis have always been highly debated. A group of children with Williams syndrome universally complain of hyperacusis. They have highly reproducible behavioural responses to noise and are thus hampered in their social interactions. Loss of inhibitory modulation to efferent sensory input to the cochlea is thought to be a possible mechanism. METHODS: Nine patients with Williams syndrome received a complete audiologic work-up, including audiogram, speech reception thresholds, acoustic reflexes, impedance, and transient evoked otoacoustic emissions (TEOAEs). MAIN OUTCOME MEASURES: Assessment of the efferent system is done by measuring changes in TEOAEs following stimulation of the contralateral ear. RESULTS: Three patients had high-frequency sensorineural hearing loss (SNHL) and thus, as expected, absent TEOAEs, indicating cochlear damage. Two had normal hearing and normal TEOAEs. However, four patients had normal hearing with absent TEOAEs. CONCLUSIONS: These findings are suggestive of cochlear disease and may, in fact, support the hypothesis of outer hair cell modulation by the ipsilateral medial olivocochlear system. Behavioural aspects of the syndrome make audiologic testing difficult. Thus, the diagnosis of SNHL may be hampered if it truly exists. The data show a preponderance of SNHL in the older age groups of our study population. This either reflects previously missed diagnoses or underlying cochlear disease, which may manifest later in life. Thus, this finding blurs the boundary between loudness recruitment and hyperacusis.     

Death following tonsillectomy in a child with Williams syndrome

Williams syndrome (WS) is an uncommon genetic syndrome due to a deletion of several genes on chromosome 7. The syndrome is associated with dysmorphic facies, neurological manifestations, idiopathic hypercalcemia, and cardiac abnormalities, particularly supravalvular aortic stenosis (SVAS). Children with Williams syndrome may have chronic serous otitis media and/or obstructive sleep apnea. Hyperacusis is also commonly seen in these children. We report a case of sudden death at the time of tonsillectomy/adenoidectomy and bilateral tympanostomy tube placement in a child with Williams syndrome. All children with Williams syndrome should have a thorough cardiac evaluation before undergoing general anesthesia for any otolaryngologic procedure.     

Prevalence and risk factors for reduced sound tolerance (hyperacusis) in children

Objective: To estimate the prevalence of reduced sound tolerance (hyperacusis) in a UK population of 11-year-old children and examine the association of early life and auditory risk factors with report of hyperacusis. Design: A prospective UK population-based study. Study sample: A total of 7097 eleven-year-old children within the Avon longitudinal study of parents and children (ALSPAC) were asked about sound tolerance; hearing and middle-ear function was measured using audiometry, otoacoustic emissions, and tympanometry. Information on neonatal risk factors and socioeconomic factors were obtained through parental questionnaires. Results: 3.7% (95% CI 3.25, 4.14) children reported hyperacusis. Hyperacusis report was less likely in females (adj OR 0.64, 95% CI 0.49, 0.85), and was more likely with higher maternal education level (adj OR 1.72, 95% CI 1.08, 2.72) and with readmission to hospital in first four weeks (adj OR 1.98, 95% CI 1.20, 3.25). Report of hyperacusis was associated with larger amplitude otoacoustic emissions but with no other auditory factors. Conclusions: The prevalence of hyperacusis in the population of 11-year-old UK children is estimated to be 3.7%. It is more common in boys.     

Uncomfortable loudness levels among children and adolescents seeking help for tinnitus and/or hyperacusis

Objective: To assess the prevalence of hyperacusis and severe hyperacusis among children and adolescents seen at an audiology outpatient tinnitus and hyperacusis service.

Design: This was a retrospective study. Hyperacusis was considered as present if the average uncomfortable loudness level (ULL) at 0.25, 0.5, 1, 2, 4 and 8?kHz for the ear with the lower average ULL, which is denoted as ULLmin, was ≤77?dB HL. Severe hyperacusis was considered as present if the ULL was 30?dB HL or less for at least one of the measured frequencies for at least one ear.

Study sample: There were 62 young patients with an average age of 12?years (SD?=?4.1?years, range 4–18?years).

Results: Eighty-five percent of patients had hyperacusis and 17% had severe hyperacusis. On average, ULLs at 8?kHz were 9.3?dB lower than ULLs at 0.25?kHz. For 33% of patients, ULLs were at least 20?dB lower at 8 than at 0.25?kHz.

Conclusions: Among children and adolescents seen at an audiology outpatient clinic for tinnitus and hyperacusis, hyperacusis diagnosed on the basis of ULLs is very prevalent and it is often characterised by lower ULLs at 8 than at 0.25?kHz.     

Probed serial recall in Williams syndrome: lexical influences on phonological short-term memory.

Williams syndrome is a genetic disorder that, it has been claimed, results in an unusual pattern of linguistic strengths and weaknesses. The current study investigated the hypothesis that there is a reduced influence of lexical knowledge on phonological short-term memory in Williams syndrome. Fourteen children with Williams syndrome and 2 vocabulary-matched control groups, 20 typically developing children and 13 children with learning difficulties, were tested on 2 probed serial-recall tasks. On the basis of previous findings, it was predicted that children with Williams syndrome would demonstrate (a) a reduced effect of lexicality on the recall of list items, (b) relatively poorer recall of list items compared with recall of serial order, and (c) a reduced tendency to produce lexicalization errors in the recall of nonwords. In fact, none of these predictions were supported. Alternative explanations for previous findings and implications for accounts of language development in Williams syndrome are discussed.     

Assessment and amelioration of hyperacusis in tinnitus patients

CONCLUSIONS: Tinnitus can be bothersome even without hyperacusis. The good correlation found in this study between the multiple-activity scale for hyperacusis (MASH) score and the overall annoyance of hyperacusis score supports the reliability of self-rating of hyperacusis. Objectives. A prospective study was undertaken to investigate the relationships between hyperacusis and tinnitus and to determine whether hyperacusis can be rapidly controlled over time. MATERIAL AND METHODS: All tinnitus patients seen over a period of 16 months with surnames beginning with the letters A L (n = 249) were assessed during a structured interview using (i) a scale ranging from 0 to 10 for assessing the annoyance of tinnitus and the overall annoyance of hyperacusis and (ii) the newly introduced MASH. RESULTS: The annoyance of tinnitus was greater in females. The prevalence of hyperacusis was high (79%). The annoyance of hyperacusis varied. Patients were categorized into five groups according to the hyperacusis annoyance score, as follows: no hyperacusis; mild hyperacusis (< or = 3); moderate hyperacusis (3.1-5.0); substantial hyperacusis (5.1-7.0); and severe hyperacusis (> or = 7.1). Individuals with severe hyperacusis were younger than those in the other groups. The correlation between the annoyance of tinnitus and the annoyance of hyperacusis was poor (r = 0.35). No audiometric difference was found between categories, whereas self-rated hearing deficiency increased with the annoyance of hyperacusis. A good correlation (r = 0.89) was found between the overall annoyance of hyperacusis and the MASH score. Changes over time, assessed in 32 patients investigated at least 3 times, showed more frequent improvement in hyperacusis (63%) than tinnitus (47%).     

Cleft palate and Williams syndrome

INTRODUCTION: Cleft palate is not one of the malformations in the Williams-Beuren syndrome. OBSERVATION: We report the case of a female patient affected by Williams syndrome, presenting with a cleft palate. The diagnosis of Williams-Beuren syndrome was confirmed by genetic analysis. DISCUSSION: Cleft palate with a Williams syndrome can be fortuitous given these two affections' frequency. The prevalence of Williams syndrome is 1/7500 to 1/10,000 births and palatine division 1/2000 to 1/2500. Cleft palate might also be a rare entity of Williams syndrome. We cannot be sure that the two are linked.     

孤独症儿童听力的初步探讨

孤独症是一种发生在低龄儿童严重的精神疾病，患儿有听力和言语发育障碍。其对声音的异常反应，表现为听觉减退和听觉敏感两个相互矛盾的方面。通过对９例１８耳孤独症患儿的ＡＢＲ检查，证实患儿有听力，且基本正常。但患儿的听觉敏感问题由于患儿的不合作，尚不能被证实，有待进一步探讨。     

Audiological and vestibular symptoms following SARS-CoV-2 infection and COVID-19 vaccination in children aged 5–11 years

PurposeThe present study assessed the prevalence of audio-vestibular symptoms following SARS-COV-2 infection or COVID-19 vaccination among children, comparing the two groups. A further aim was to evaluate whether children with pre-existing unilateral hearing loss were more prone to adverse events.Materials and methodsThis retrospective study included children aged 5–11 years with normal hearing or a proven history of unilateral hearing loss who contracted SARS-CoV-2 or received two doses of COVID-19 vaccine. Tinnitus, hyperacusis, aural fullness, otalgia, otorrhea, new-onset hearing loss, vertigo and dizziness were investigated as possible complications of SARS-CoV-2 infection or the COVID-19 vaccine.ResultsThis study included 272 children (143 boys, 129 girls), with a mean age of 7.8 ± 2.3 years. Among these, 120 were affected by pre-existing unilateral hearing loss.The most common audio-vestibular symptoms reported by children following SARS-CoV-2 infection and COVID-19 vaccination were aural fullness (33/132, 25 %) and dizziness (5/140, 3.6 %), respectively. All symptoms following COVID-19 vaccination resolved within 24 h.Compared to children who received the COVID-19 vaccine, those infected with SARS-CoV-2 had a higher prevalence of tinnitus (p = 0.009), hyperacusis (p = 0.003), aural fullness (p < 0.001), otalgia (p < 0.001), otorrhea (p < 0.001), and vertigo (p = 0.006). Two girls also experienced new-onset unilateral sensorineural hearing loss following SARS-CoV-2 infection.Children with a known history of unilateral hearing loss did not have a higher prevalence of audio-vestibular symptoms than children with normal hearing.ConclusionsOur results suggest that the COVID-19 vaccine is safe and can be recommended for children with unilateral hearing loss without fear of possible audio-vestibular sequelae.     

Subjective ear noise and hyperacusis. Treatment with fluctuating currents

The prevalence of hyperacusis, and subjective ear noise in particular, tends to increase in the general population. The data available in the literature show that physiotherapeutical procedures are the most effective treatment of this abnormality. The techniques and devices using fluctuating currents were employed to treat 40 patients. A "SLUKH-OTO-1" (HEARING-OTO) device is of use now. This procedure proved to be the most effective in case of hyperacusis and subjective ear noise without hearing disorder, there was also a high percentage of positive results in chronic neurosensory hypoacusis. Treatment of subjective ear noise by this procedure is not so effective in otosclerosis and chronic otitis media.     

The hearing profile of Nigerian school children

The paucity of up-to-date and representative epidemiological data on hearing disorders in Nigeria has been observed as undermining the effective advocacy of prevention initiatives. This study attempts to address this problem by evaluating the prevalence and pattern of hearing impairment in school entrants. Parental interviews, otoscopy, pure-tone audiometric screening (frequency 0.5-4 kHz) and tympanometric examinations were conducted for a representative sample of 359 school children in an inner city area of Lagos. The prevalence of hearing loss was 13.9%. Middle ear abnormalities were noted in 20.9% of the study population, of which 18.7% were reported with otitis media with effusion. Impacted cerumen, documented in 189 children (52.6%), was the most common disorder. It showed significant association with hearing loss (P<0.001) and school performance (P<0.01). Tympanic membrane abnormalities were observed in 144 (40.1%) children. Of these, 45 (31.3%) showed abnormal tympanograms while 28 (19.4%) reported hearing loss (P<0.05). The early detection and management of hearing problems is relatively rare, thus precluding the determination of possible aetiological factors for the observed abnormalities. Poor public awareness, dearth of relevant facilities and the lack of early screening programmes are major known contributory factors. The well established national immunisation programme offers a cost-effective platform within the primary health-care system for addressing the high prevalence of hearing abnormalities in school children.     

An evidence‐based approach to the 12‐min consultation for a child with Down’s syndrome

Clin. Otolaryngol. 2012, 37 , 291–296 Background: Down’s syndrome is the commonest genetic condition. ENT problems are common in these children and can affect their development and quality of life. Method: English literature review and experience of the senior author in managing ENT problems in children and adults with Down’s syndrome. Search strategy: We carried out a Medline search on 31/03/2012 of English language publications using the following keywords: Down/Down’s syndrome, hearing loss, ear infections, sleep apnoea, thyroid. Results: The prevalence, presenting features and management of many common ENT conditions are significantly different in children who have Down’s syndrome. This is particularly true of otitis media with effusion and sleep‐disordered breathing. Conclusion: The outpatient consultation for a child with Down’s syndrome should assess a range of specific ENT problems affecting this patient group including hearing issues, sleep‐disordered breathing, recurrent upper respiratory tract infections, as well as other health conditions like hypothyroidism, atlantoaxial instability and cardiac issues, which can potentially affect the management of the patient.     

Language and cognition in two children with Williams syndrome

In this study, aspects of language, single-gesture retrieval, combinatorial play, and visual-spatial cognition are reported in two children with Williams syndrome who were in the single-word stage of language acquisition. Williams syndrome children are of particular interest because, when older (10-15 years), they display an unusual profile of abilities that may include superior linguistic performance compared with other aspects of cognition. The two children in the present study are compared to a group of normal children in the single-word stage of language acquisition with a mean age of 14.8 months, a group of single-word stage children who are 6-18 months delayed, and a group of older normal children with a mean age of 23.5 months. Results are compatible with the unusual cognitive profile displayed by older Williams syndrome children. We discuss the possibility that this profile involves mechanisms that extend beyond the boundaries of language proper.     

Chronic suppurative otitis media in school going children

The morbidity and mortality associated with otitis media is really a challenge for the health care systems. This study is a small attempt to look into the magnitude of this problem in the society, where in school going children in Yavatmal city were studied. A total of 4104 students were studied. It was found that the overall prevalence of chronic suppurative otitis media in Yavatmal city is 3%. The incidence of acute otitis media was found to be 1.3% Chronic suppurative otitis media was commoner in students belonging to low socioeconomic strata and its association with adenoid hypertrophy was statistically significant. The authors recommend that a few more such studies should be carried out in different parts of the country to collect vital data for the health care system managers. Suppurative otitis media, school children, Yavatmal city.     

Early age conductive hearing loss causes audiogenic seizure and hyperacusis behavior

Recent clinical reports found a high incidence of recurrent otitis media in children suffering hyperacusis, a marked intolerance to an otherwise ordinary environmental sound. However, it is unclear whether the conductive hearing loss caused by otitis media in early age will affect sound tolerance later in life. Thus, we have tested the effects of tympanic membrane (TM) damage at an early age on sound perception development in rats. Two weeks after the TM perforation, more than 80% of the rats showed audiogenic seizure (AGS) when exposed to loud sound (120?dB SPL white noise, 相似文献   

FIRING PROPERTY OF INFERIOR COLLICULUS NEURONS AFFECTED BY FMR1 GENE MUTATION

Fragile X syndrome is the most common form of inherited mental retardation affecting up to 1 in 4000 individuals. The syn- drome is induced by a mutation in the FMR1 gene, causing a deficiency in its gene by-product FMRP. Impairment in the nor- mal functioning of FMRP leads to learning and memory deficits and heightened sensitivity to sensory stimuli, including sound （hyperacusis）. The molecular basis of fragile X syndrome is thoroughly understood; however, the neural mechanisms underly- ing hyperacusis have not yet been determined. As the inferior colliculus （IC） is the principal midbrain nucleus of the auditory pathway, the current study addresses the questions underlying the neural mechanism of hyperacusis within the IC of fragile X mice. Acute experiments were performed in which electrophysiological recordings of the IC in FMR1-KO and WT mice were measured. Results showed that Q-values for WT were significantly larger than that of FMR-1 KO mice, indicating that WT mice exhibit sharper tuning curves than FMR1-KO mice. We also found the ratio of the monotonic neurons in the KO mice was much higher than the WT mice. These results suggest that lack of FMRP in the auditory system affects the developmental maturation and function of structures within the auditory pathway, and in this case specifically the IC. The dysfunction ob- served within the auditory neural pathway and in particular the IC may be related to the increased susceptibility to sound as seen in individuals with fragile X syndrome. Our study may help on understanding the mechanisms of the fragile X syndrome and hyperacusis.     

Hearing thresholds,tinnitus, and headphone listening habits in nine-year-old children

Objective: Investigate hearing function and headphone listening habits in nine-year-old Swedish children. Design: A cross-sectional study was conducted and included otoscopy, tympanometry, pure-tone audiometry, and spontaneous otoacoustic emissions (SOAE). A questionnaire was used to evaluate headphone listening habits, tinnitus, and hyperacusis. Study sample: A total of 415 children aged nine years. Results: The prevalence of a hearing threshold?≥20?dB HL at one or several frequencies was 53%, and the hearing thresholds at 6 and 8?kHz were higher than those at the low and mid frequencies. SOAEs were observed in 35% of the children, and the prevalence of tinnitus was 5.3%. No significant relationship between SOAE and tinnitus was found. Pure-tone audiometry showed poorer hearing thresholds in children with tinnitus and in children who regularly listened with headphones. Conclusion: The present study of hearing, listening habits, and tinnitus in nine-year old children is, to our knowledge, the largest study so far. The main findings were that hearing thresholds in the right ear were poorer in children who used headphones than in children not using them, which could be interpreted as headphone listening may have negative consequences to children’s hearing. Children with tinnitus showed poorer hearing thresholds compared to children without tinnitus.