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Purpose: This study aimed to investigate the prevalence of hyperacusis and auditory processing (listening) difficulties (APDiff) in normal-hearing young adults and to explore the relationship between these two conditions., Methods: A total of 333 participants aged 18-24 underwent pure-tone audiometry tests and completed the Khalfa Hyperacusis Questionnaire (KHQ) and the University of Cincinnati Auditory Processing Inventory (UCAPI) online., Results: Hyperacusis was identified in 26.43% of participants, and APDiff in 36.04%. A strong, positive correlation was found between KHQ and UCAPI scores (r = 0.603, p < 0.001). Logistic regression models indicated that hyperacusis significantly increased the likelihood of having APDiff (OR 7.688, p < 0.001)., Conclusion: Despite the high prevalence of hyperacusis and APDiff in young adults, few seek help from audiology clinics. Our findings highlight a significant link between hyperacusis and APDiff, emphasizing the need for screening protocols for auditory processing skills in young adults due to these conditions' potential social and academic impacts., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objectives: To estimate the prevalence of tinnitus and hyperacusis in children aged 9-12 years in Flanders, as well as to explore the associations with hearing abilities and listening behaviours., Design: A cross-sectional survey was undertaken in four different Flemish schools. The questionnaire was distributed among 415 children, with a response rate of 97.3%., Results: The prevalence of permanent tinnitus was 10.5% and of hyperacusis was 3.3%. The hyperacusis prevalence was higher in girls (p < .05). Some children reported effects of tinnitus in terms of anxiety (20.1%), sleep (36.5%), and concentration (24.8%). When listening to personal listening devices, 33.5% of the children reported to listen for at least 1 h at 60% or higher of the volume range. Moreover, 54.9% of children stated to never wear hearing protection., Conclusions: Tinnitus and hyperacusis are prevalent in children aged 9-12 years. Some of these children might be overlooked and, as such, not receiving the required follow-up or counselling. Development of guidelines for the assessment of these auditory symptoms in children would help to determine the prevalence numbers with greater accuracy. Sensibility campaigns for safe listening are warranted, as more than half of the children never use hearing protection., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: This study aims to investigate the validity and reliability of the Khalfa's hyperacusis questionnaire (HQ) in Turkish tinnitus patients with hyperacusis using uncomfortable loudness levels (ULLs) and to determine a cutoff score for tinnitus patients specifically., Materials and Methods: One hundred and forty subjects with a mean age ± SD of 45.27 ± 14.43 years ranging from 18 to 75 participated in the study. HQ and ULLs were used as measures of hyperacusis. The participants were divided into two groups due to ULLs ≤ 90 dB in one or both ears (Group 1) and 56 participants have ULLs > 90 dB (Group 2)., Results: The mean HQ score of the participants was 19.55 ± 7.18 points, Group 1 (n = 84) HQ mean score was 21.97 ± 7.08, and Group 2 (n = 56) 15.91 ± 5.56 points, and the cutoff point was found 16.50. Statistically significant differences were found between the groups in total HQ (p < 0.001), attention, social, and emotional subscales of HQ., Conclusions: Hyperacusis questionnaire using with ULLs is a precise tool for the steps of identifying, categorizing, and managing the hyperacusis in patients with tinnitus. However, the effect of the tinnitus on hyperacusis should be considered, because it causes additional problems., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Hyperacusis is a reduced tolerance to sounds that often co-occurs with tinnitus. Both symptoms have convergent as well as divergent characteristics. Somatic modulation, changes in pitch or loudness during certain movements, is common in patients with a primary complaint of tinnitus. However, thus far, this is not documented in patients with hyperacusis., Objectives: This study aimed to examine the influence of somatic manoeuvres on the perception of external sounds in patients with a primary complaint of hyperacusis., Methodology: In this prospective cross-sectional pilot study, 18 patients with a primary complaint of hyperacusis were recruited at the Tinnitus Treatment and Research Center Antwerp (TINTRA). While patients listened to a 1 kHz broadband noise of 30 dB sensation level, six neck manoeuvres (flexion, extension, lateroflexion left/right, traction and compression), three jaw manoeuvres (protrusion, laterotrusion left/right) and one placebo manoeuvre (hand on head) were performed. The primary outcome measure was the change in the perception of the presented sound in terms of loudness and intrusiveness between baseline and each modulation measured by a visual analogue scale (VAS)., Results: No overall significant changes were found; however, individual results indicated that five patients presented a clinically relevant change of more than three points out of ten on VAS in terms of hyperacusis after at least one of the executed somatic manoeuvres., Conclusions: This pilot study did not demonstrate an overall significant change in hyperacusis after somatic manoeuvres but does not rule out the possibility of somatic modulation in some hyperacusis patients., Trial Registration: The protocol of this prospective cross-sectional pilot study was registered on clinicaltrials.gov with registration number NCT04693819., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Hyperacusis is the hypersensitivity to auditory stimuli that would typically not affect those with hearing sensitivity within normal limits. The prevalence of hyperacusis in the general population has been estimated to range from 9% to 15%. There is no universally accepted definition for what defines hyperacusis among kindred illnesses of poor sound tolerance. As a result, hyperacusis prevalence varies more, which has an impact on both assessment and treatment. This study aims to determine the efficacy of the different clinical assessment measures of hyperacusis in assessing hyperacusis through a systematic review., Methods: A review and reference of literature were done on hyperacusis, and assessment measures were used. A review of 23 articles was conducted to highlight these measures used. Most selected studies included retrospective, prospective, survey, and experimental designs. Study quality reported an overall low risk of bias., Results: This review reports the summary and the current evidence of clinical assessment measures used for diagnosing hyperacusis., Conclusions: This review highlights a protocol that may be used in the clinical diagnosis of hyperacusis. It also shows the necessity to standardize and validate assessment measures for the younger population., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To document whether patients with and without hyperacusis differ from each other on demographic, audiological, and clinical characteristics., Methods: Based on the Hyperacusis Questionnaire's (HQ) cut-off (HQ > 28), a total of 2301 participants were divided into patients with and without hyperacusis. Demographic data, scores on self-reported questionnaires [Tinnitus Functional Index (TFI), Visual Analogue Scale of tinnitus loudness (VAS loudness ), Hospital Anxiety Depression Scale (HADS)], and audiological parameters were retrospectively analysed to determine differential factors between the two groups., Results: In total, 10.9% of the patients was classified as hyperacusis patients (n = 251). They reported a significant, higher tinnitus severity (mean difference of 19 points on TFI) and mental distress (mean difference of 4 points on the HADS subscales) (p < 0.001) than patients without hyperacusis. Moreover, this group consisted of more women (45% % in hyperacusis group vs. 35% in non-hyperacusis group) and women scored significantly higher on the HQ (p < 0.001) and TFI (p < 0.01)., Conclusion: Patients with hyperacusis have distinctive characteristics. The presence of hyperacusis in combination with tinnitus can indicate a higher need for psychoeducation. Patients that present themselves with hyperacusis without tinnitus complaints remain a minority, yet might be underdiagnosed. Hence, future studies should disentangle tinnitus from hyperacusis. In clinical practice, greater efforts are required to increase knowledge about hyperacusis as a primary or secondary complaint and to provide individualized treatment for these patients., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: The oculocerebrorenal syndrome of Lowe (OCRL) is a rare X-linked multi-systemic disorder, almost always characterized by the triad of congenital cataract, cognitive and behavioral impairment and a proximal tubulopathy., Methods: Twenty-eight novel patients with suspected Lowe syndrome were studied., Results: All patients carried OCRL gene defects with mutational hot spots at CpG dinucleotides. Mutations previously unknown in Lowe syndrome were observed in ten of the 28 patients, and carriership was identified in 30.4 % of the mothers investigated. Mapping the exact breakpoints of a complete OCRL gene deletion revealed involvement of several flanking repeat elements. We noted a similar pattern of documented clinically relevant symptoms, and even though the patient cohort comprised relatively young patients, 32 % of these patients already showed advanced chronic kidney disease. Thrombocytopenia was seen in several patients, and hyperosmia and/or hyperacusis were reported recurrently. A p.Asp523Asn mutation in a Polish patient, associated with the typical cerebrorenal spectrum but with late cataract (10 year), was also evident in two milder affected Italian brothers with ocular involvement of similar progression., Conclusions: We have identified clinical features in 28 patients with suspected Lowe syndrome that had not been recognized in Lowe syndrome prior to our study. We also provide further evidence that OCRL mutations cause a phenotypic continuum with selective and/or time-dependent organ involvement. At least some of these mutants might exhibit a genotype-phenotype correlation.

Unlabelled: Sensitivity to sound is one of the most commonly reported challenges in ASD. No compelling evidence shows that hearing of ASD individuals differs physiologically from normal peers. Superior semicircular canal dehiscence was found to be more common in ASD children with auditory hypersensitivity (29%) by means of high-resolution CT scan than the reported (14%) in normal pediatric population by other investigators. The increased prevalence of radiographic dehiscence might be due to inability of CT scan to visualize immature bone. We wished to determine whether ocular vestibular evoked myogenic potentials in ASD children with auditory hypersensitivity produces similar responses to those obtained in adult superior canal dehiscence, and whether it could help differentiate radiographic dehiscence due to bone immaturity from true canal dehiscence syndrome. A prospective study on 14 ASD children complaining of auditory hypersensitivity served as the study group. 15 ASD children without auditory hypersensitivity, age and gender matched, served as a control group. oVEMP and high-resolution CT scan of petrous and temporal bone were performed to all participants. Mean amplitude of n10 was 1.83 ± 0.11 and 1.79 ± 0.09 μV in the control group with mean peak latency of 9.79 ± 0.42 and 9.77 ± 0.30 ms for the right and left ears, respectively. Asymmetry ratio was 2.04 ± 1.37. In the study group, the mean amplitude of n10 was 2.07 ± 0.46 and 1.89 ± 0.30 μV, with mean peak latency of 9.52 ± 0.33 and 9.59 ± 0.21 ms for the right and left ears, respectively, with asymmetry 5.23 ± 6.93%. No statistically significant difference was observed for the studied parameters. In the study group, the number of ears showing an augmented amplitude (>2SD) of n10 was (N = 5). Furthermore, the study group demonstrated a radiographic SSCD in 6 ears. n10 was normal in the control group while radiographic SSCD was observed in 3 of them., Conclusion: oVEMPs show diagnostic ability in differentiating ASD children complaining of auditory hypersensitivity due to superior canal dehiscence from those with radiographic dehiscence only due to bone immaturity or atypical cortical development.

Autism is the third most common developmental disorder, following mental retardationand cerebral palsy. ASD children have been described more often as beingpreoccupied with or agitated by noise. The aim of this study was to evaluate theprevalence and clinical significance of semicircular canal dehiscence detected on CTimages in ASD children with intolerance to loud sounds in an attempt to find ananatomical correlate with hyperacusis.14 ASD children with auditory hypersensitivity and 15 ASD children without auditoryhypersensitivity as control group age and gender matched were submitted to historytaking, otological examination, tympanometry and acoustic reflex thresholdmeasurement. ABR was done to validate normal peripheral hearing and integrity ofauditory brain stem pathway. High resolution CT scan petrous and temporal boneimaging was performed to all participated children. All participants had normal hearingsensitivity in ABR testing. Absolute ABR peak waves of I and III showed no statisticallysignificant difference between the two groups, while absolute wave V peak andinterpeak latencies I-V and III-V were shorter in duration in study group whencompared to the control group. CT scans revealed SSCD in 4 out of 14 of the studygroup (29%), the dehiscence was bilateral in one patient and unilateral in threepatients. None of control group showed SSCD. In conclusion, we have reportedevidence that apparent hypersensitivity to auditory stimuli (short conduction time in ABR) despite the normal physiological measures in ASD children with auditoryhypersensitivity can provide a clinical clue of a possible SSCD.

The aim of this prospective clinical study was to test auditory function in patients with Laron syndrome, either untreated or treated with insulin-like growth factor I (IGF-I). The study group consisted of 11 patients with Laron syndrome: 5 untreated adults, 5 children and young adults treated with replacement IGF-I starting at bone age <2 years, and 1 adolescent who started replacement therapy at bone age 4.6 years. The auditory evaluation included pure tone and speech audiometry, tympanometry and acoustic reflexes, otoacoustic emissions, loudness dynamics, auditory brain stem responses and a hyperacusis questionnaire. All untreated patients and the patient who started treatment late had various degrees of sensorineural hearing loss and auditory hypersensitivity; acoustic middle ear reflexes were absent in most of them. All treated children had normal hearing and no auditory hypersensitivity; most had recordable middle ear acoustic reflexes. In conclusion, auditory defects seem to be associated with Laron syndrome and may be prevented by starting treatment with IGF-I at an early developmental age.

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.