Audiol. Res., Volume 14, Issue 1 (February 2024) – 19 articles

The aim of the present study was to investigate adverse effects of head injury, neck trauma, and chronic noise exposure on the complaint profile in people with Ménière’s disease (MD). The study used a retrospective design. Register data of 912 patients with MD from the Finnish Ménière Federation database were studied. The data comprised case histories of traumatic brain injury (TBI), neck trauma and occupational noise exposure, MD specific complaints, impact related questions, and the E-Qol health-related quality of life instrument. TBI was classified based on mild, moderate, and severe categories of transient loss of consciousness (TLoC). The mean age of the participants was 60.2 years, the mean duration of the disease was 12.6 years, and 78.7% were females. Logistic regression analysis, linear correlation, and pairwise comparisons were used in evaluating the associations. 19.2% of the participants with MD had a history of TBI. The phenotype of participants with TBI was associated with frequent vestibular drop attacks (VDA), presyncope, headache-associated vertigo, and a reduction in the E-QoL. Logistic regression analysis explained the variability of mild TBI in 6.8%. A history of neck trauma was present in 10.8% of the participants. Neck trauma associated with vertigo (NTwV) was seen in 47 and not associated with vertigo in 52 participants. The phenotype of NTwV was associated with balance problems, VDA, physical strain-induced vertigo, and hyperacusia. Logistic regression analysis explained 8.7% of the variability of the complaint profile. Occupational noise exposure was recorded in 25.4% of the participants and correlated with the greater impact of tinnitus, hyperacusis, and hearing loss. Neither the frequency, duration, or severity of vertigo or nausea were significantly different between the baseline group and the TBI, NTwV, or noise-exposure groups. The results indicate that TBI and NTwV are common among MD patients and may cause a confounder effect. Full article

Soft tissue conduction is a mode of hearing which differs from air and bone conduction since the soft tissues of the body convey the audio-frequency vibrations to the ear. It is elicited by inducing soft tissue vibrations with an external vibrator applied to sites on the body or by intrinsic vibrations resulting from vocalization or the heartbeat. However, the same external vibrator applied to the skin sites also excites cutaneous mechanoreceptors, and attempts have been made to assist patients with hearing loss by audio–tactile substitution. The present study was conducted to assess the contribution of the auditory nerve and brainstem pathways to soft tissue conduction hearing. The study involved 20 normal hearing students, equipped with ear plugs to reduce the possibility of their response to air-conducted sounds produced by the external vibrator. Pure tone audiograms and speech reception (recognition) thresholds were determined in response to the delivery of the stimuli by a clinical bone vibrator applied to the cheek, neck and shoulder. Pure tone and speech recognition thresholds were obtained; the participants were able to repeat the words they heard by soft tissue conduction, confirming that the auditory pathways in the brain had been stimulated, with minimal involvement of the somatosensory pathways. Full article

Background: Although hearing aids (HAs) can compensate for reduced audibility, functional outcomes and benefits vary widely across individuals. As part of the Danish ‘Better hEAring Rehabilitation’ (BEAR) project, four distinct auditory profiles differing in terms of audiometric thresholds and supra-threshold hearing abilities were recently identified. Additionally, profile-specific HA-fitting strategies were proposed. The aim of the current study was to evaluate the self-reported benefit of these profile-based HA fittings in a group of new HA users. Methods: A total of 205 hearing-impaired older adults were recruited from two Danish university hospitals. Participants were randomly allocated to one of two treatment groups: (1) NAL-NL2 gain prescription combined with default advanced feature settings (‘reference fitting’) or (2) auditory profile-based fitting with tailored gain prescription and advanced feature settings (‘BEAR fitting’). Two months after treatment, the participants completed the benefit version of the short form of the Speech, Spatial, and Qualities of Hearing Scale (SSQ12-B) and the International Outcome Inventory for Hearing Aids (IOI-HA) questionnaire. Results: Overall, participants reported a clear benefit from HA treatment. However, no significant differences in the SSQ12-B or IOI-HA scores between the reference and BEAR fittings were found. Conclusion: First-time users experience clear benefits from HA treatment. Auditory profile-based HA fitting warrants further investigation. Full article

Individuals with hearing loss are at risk for cognitive decline. The traditional approach to audiological care does not typically involve a team approach that addresses cognitive concerns. While cognitive screening is within the scope of practice in audiology, audiologists are not typically trained in interpreting screening results or providing rehabilitation that supports cognitive health. However, as growing evidence shows that hearing loss is tied to cognitive decline, a team approach is required to support whole-person care. Speech–language pathologists, who specialize in optimizing communication, are best situated to collaborate with audiologists to provide holistic aural rehabilitation. Audiologists and speech–language pathologists who partner to support a client’s communication skills and social relationships play an important role in the life of an individual with hearing loss. In this perspective, we describe relevant background information about hearing loss and cognition and present an interprofessional approach to aural rehabilitation for adults with hearing loss who have cognitive concerns. We also discuss implications for future research. Full article

Background: We propose a Suppression Head IMPulse (SHIMP) test method that provides for equipment only through the use of InfraRed Video-OculoScopy (IR-VOS) and allows horizontal and vertical semicircular canal function evaluation in bedside mode. We therefore named the test InfraRed clinical SHIMP (IR-cSHIMP). Methods: To check IR-cSHIMP diagnostic efficiency, we studied 22 normal subjects, 18 patients with unilateral, and 6 with bilateral deficient vestibulopathy. Each subject first underwent a vestibular examination and, only later, an IRc-SHIMP test. Results: When the IR-cSHIMP test was performed in the horizontal plane, all normal subjects showed anti-compensatory saccades. When the vertical semicircular canal function was evaluated, the same result was obtained in all normal subjects except three, which were considered false positives. In patients with vestibular deficits, the test performed in the horizontal and vertical planes were always pathological, with 100% agreement between clinical and instrumental tests. Conclusions: Our bedside method proved to be fast, simple, and effective in discriminating between healthy and pathological subjects. It required only the same skill as the better-known cHIT. For these reasons, we believe that the IR-cSHIMP should be part of daily clinical practice as a useful tool in the selection of patients to undergo more sophisticated investigations. Full article

Various stimulation-based rehabilitation approaches have been proposed to alleviate tinnitus. This study aimed to determine the efficacy of a rehabilitation approach that simultaneously provides microcurrent and sound stimulation for tinnitus relief. Twenty-eight participants with chronic sensorineural tinnitus were randomly assigned to one of two groups based on the rehabilitation approaches (sound therapy-only group and combined microcurrent and sound therapy group). Each participant underwent sound therapy or simultaneous stimulation for approximately 2 h daily for 3 months. The effectiveness of the rehabilitation approaches was determined based on changes in the Korean version of the tinnitus primary function questionnaire (K-TPFQ) and visual analog scale for loudness (VAS-L) scores at baseline, 1.5 months, and 3 months. For the K-TPFQ scores, both groups exhibited a large effect of rehabilitation; however, for the VAS-L scores, the simultaneous stimulation group demonstrated a large effect of rehabilitation, whereas the sound therapy group exhibited a small effect. Therefore, a rehabilitation approach that combines sound stimulation with microcurrent stimulation can improve response and perception in tinnitus. Full article

Background: The most frequent form of vertigo in pediatric age is represented by vertigo linked to migraine, with a prevalence of 32.7%. This group of pathologies has received a redefinition of the diagnostic criteria to adapt them to the pediatric age with a new classification of the clinical pictures. We have several kinds of problems with these conditions that often have a significant impact on patients’ and parents’ quality of life: the diagnostic approach involves different tools for the different age groups contained in the pediatric range; the treatment of this type of vertigo is not consolidated due to the limited availability of trials carried out on pediatric patients. Focusing on this topic, the aim of this review was to provide an update on the more recent clinical advances in the diagnosis and treatment of Vestibular Migraine (VM) in children. Methods: We searched the PubMed, Embase, and Cochrane library databases for articles published in English from January 2015 to April 2023. The secondary search included articles from reference lists, identified by the primary search. Records were first screened by title/abstract, and then full-text articles were retrieved for eligibility evaluation. The searches combined a range of key terms (“Pediatric” AND “Childhood” AND “dizziness” OR “vertigo” AND “vestibular”). Results: Migraine-related vertigo, in its most recent definitions and classifications, is the most frequent group of balance pathologies in pediatric age. The results from the various experiences present in the literature suggest a clinical approach to be integrated with the use of instrumental tests selected according to the age of the patient and the reliability of the results. Conclusion: Knowing the timeline of the applicability of vestibular tests and the information that can be obtained from them is fundamental for diagnostic accuracy. Therapy is strongly conditioned by the limited availability of pediatric trials and by the wide range it includes, from very young children to adolescents. Full article

If the term “genetics” is a relatively recent proposition, introduced in 1905 by English biologist William Bateson, who rediscovered and spread in the scientific community Mendel’s principles of inheritance, since the dawn of human civilization the influence of heredity has been recognized, especially in agricultural crops and animal breeding. And, later, in familial dynasties. In this concise review, we outline the evolution of the idea of hereditary hearing loss, up to the current knowledge of molecular genetics and epigenetics. Full article

The third window syndrome, often associated with the Tullio phenomenon, is currently most often observed in patients with a superior semicircular-canal dehiscence (SCD) but is not specific to this pathology. Clinical and vestibular tests suggestive of this pathology are not always concomitantly observed and have been recently complemented by the skull-vibration-induced nystagmus test, which constitutes a bone-conducted Tullio phenomenon (BCTP). The aim of this work was to collect from the literature the insights given by this bedside test performed with bone-conducted stimulations in SCD. The PRISMA guidelines were used, and 10 publications were included and analyzed. Skull vibration-induced nystagmus (SVIN), as observed in 55 to 100% of SCD patients, usually signals SCD with greater sensitivity than the air-conducted Tullio phenomenon (ACTP) or the Hennebert sign. The SVIN direction when the test is performed on the vertex location at 100 Hz is most often ipsilaterally beating in 82% of cases for the horizontal and torsional components and down-beating for the vertical component. Vertex stimulations are more efficient than mastoid stimulations at 100 Hz but are equivalent at higher frequencies. SVIN efficiency may depend on stimulus location, order, and duration. In SCD, SVIN frequency sensitivity is extended toward high frequencies, with around 400 Hz being optimal. SVIN direction may depend in 25% on stimulus frequency and in 50% on stimulus location. Mastoid stimulations show frequently diverging results following the side of stimulation. An after-nystagmus observed in 25% of cases can be interpreted in light of recent physiological data showing two modes of activation: (1) cycle-by-cycle phase-locked activation of action potentials in SCC afferents with irregular resting discharge; (2) cupula deflection by fluid streaming caused by the travelling waves of fluid displacement initiated by sound or vibration at the point of the dehiscence. The SVIN direction and intensity may result from these two mechanisms’ competition. This instability explains the SVIN variability following stimulus location and frequency observed in some patients but also discrepancies between investigators. SVIN is a recent useful insight among other bedside examination tests for the diagnosis of SCD in clinical practice. Full article

For many individuals, music has a significant impact on the quality and enjoyability of life. Cochlear implant (CI) users must cope with the constraints that the CI imposes on music perception. Here, we assessed the musical experiences of young CI users and age-matched controls with normal hearing (NH). CI users and NH peers were divided into subgroups according to age: children and adolescents. Participants were tested on their ability to recognize vocal and instrumental music and instruments. A music questionnaire for pediatric populations (MuQPP) was also used. CI users and NH peers identified a similar percentage of vocal music. CI users were significantly worse at recognizing instruments (p < 0.05) and instrumental music (p < 0.05). CI users scored similarly to NH peers on the MuQPP, except for the musical frequency domain, where CI users in the children subgroup scored higher than their NH peers (p = 0.009). For CI users in the children subgroup, the identification of instrumental music was positively correlated with music importance (p = 0.029). Young CI users have significant deficits in some aspects of music perception (instrumental music and instrument identification) but have similar scores to NH peers in terms of interest in music, frequency of music exposure, and importance of music. Full article

Children with single-sided deafness (SSD) may experience delays in language and speech development. Reduced speech discrimination and poor sound localization abilities in young SSD patients may result in greater cognitive efforts required to focus and process auditory information, as well as increased listening-related fatigue. Consequently, these children can have a higher risk of academic failure and are often in need of extra help at school. Recently, cochlear implants (CIs) have been introduced as a rehabilitative option for these children, but their effectiveness is still a topic of debate. A literature review was performed according to PRISMA guidelines, searching the Medline database from inception to October 2023. The research identified nine papers that met the inclusion criteria. Data extracted from the selected studies included 311 children affected by SSD and cochlear implants. The reported audiological outcomes were further analyzed. Overall, a high level of satisfaction was described by parents of children with SSD and CI, and those who received a CI under the age of 3 presented better results. However, a proportion of patients did not use the device daily. Our review highlights the possible, and still controversial, role of CI for the hearing rehabilitation of children with unilateral deafness, underlining the need for further research in this field. To date, careful and comprehensive counseling with the child and the family is necessary before considering this option. Full article

Background: Since the discovery of the perilymphatic fistula (PLF), the diagnosis and treatment remain controversial. If successfully recognized, the PLF is surgically repairable with an obliteration of the fistula site. Successful treatment has a major impact on patient’s quality of life with an improvement in their audiological and vestibular symptoms. Objective: To prospectively investigate patients’ clinical and audiological evolution with PLF suspicion after middle ear exploration and obliteration of the round and oval window. Study Design: Prospective comparative study. Setting: Tertiary care center. Methods: Patients were divided into two groups: Group I consisted of patients where no PLF had been identified intraoperatively at the oval and/or at the round window, and Group II consisted of patients where a fistula had been visualized. Patient assessment was a combination of past medical history, the presence of any risk factors, cochlear and vestibular symptoms, a physical examination, temporal bone imaging, audiograms, and a videonystagmogram (VNG). Results: A total of 98 patients were divided into two groups: 62 in Group I and 36 in Group II. A statistically significant difference regarding gender was observed in Group II (83.3% of males vs. 16.7% of females, p = 0.008). A total of 14 cases (4 and 10 in Groups I and II, respectively) were operated for a recurrent PLF. Fat graft material was used in the majority of their previous surgery; however, no difference was found when comparing fat to other materials. In addition, no statistically significant difference was noted between Groups I and II concerning predisposing factors, imaging, VNG, symptom evolution, or a physical exam before the surgery and at 12 months post-operative. However, both groups showed statistically significant hearing and vestibular improvement. On the other hand, the air conduction (AC) and bone conduction (BC) at each frequency were not statistically different between the two groups before surgery but showed statistically significant improvement at 12 months post-operatively, especially for the BC at the frequencies 250 (p = 0.02), 500 (p = 0.0008), and 1000 Hz (p = 0.04). Conclusions: Whenever you suspect a perilymphatic fistula, do not hesitate to explore middle ear and do window obliterations using a tragal perichondrium material. Our data showed that cochlear and vestibular symptoms improved whether a fistula had been identified or not. Full article

Autism Spectrum Disorder (ASD) is a neurodevelopmental disorder characterized by stereotyped and repetitive behavior patterns. In addition to neurological and behavioral problems, individuals with ASD commonly experience otolaryngological comorbidities. Individuals with ASD often have auditory disorders including hearing loss and auditory processing disorders such as central auditory processing disorder (CAPD), as well as both chronic and recurrent otitis media. These challenges negatively impact a person’s ability to effectively communicate and may further impact their neurological functioning, particularly when not appropriately treated. Individuals diagnosed with ASD also have difficulty sleeping which contributes to increased irritability and may further aggravate the core behavioral symptoms of autism. The individuals with ASD also have a higher rate of sinusitis which contributes to the worsening of the autism behavior phenotype. The high prevalence of otolaryngological comorbidities in individuals with ASD warrants a better collaboration between their various healthcare providers and otolaryngologists with expertise in auditory, sleep, and sinus disorders in pursuit of improving the quality of life of affected individuals and their families/caregivers. Full article

The present study evaluates the response to betahistine in patients who presented vestibular drops attacks in the context of Ménière’s disease (MD) and the factors that can predict an unfavorable response to it. A total of 43 patients were analyzed, out of which 33 were diagnosed with MD. This is a descriptive, cross-sectional study with retrospective data collection. Data as regards age, accompanying symptoms, etiological diagnosis and response to MD treatment were collected. A statistical analysis was carried out, and we found that the disease evolution time and specific alterations in the vestibulospinal and oculomotor physical examination present an unfavorable response to betahistine. Failures for betahistine were treated with intratympanic gentamicin, with which symptomatic control was achieved in all cases. Full article

Waardenburg syndrome (WS) is characterized by hearing loss and pigmentary abnormalities of the eyes, hair, and skin. The condition is genetically heterogeneous, and is classified into four clinical types differentiated by the presence of dystopia canthorum in type 1 and its absence in type 2. Additionally, limb musculoskeletal abnormalities and Hirschsprung disease differentiate types 3 and 4, respectively. Genes PAX3, MITF, SOX10, KITLG, EDNRB, and EDN3 are already known to be associated with WS. In WS, a certain degree of molecularly undetected patients remains, especially in type 2. This study aims to pinpoint causative variants using different NGS approaches in a cohort of 26 Brazilian probands with possible/probable diagnosis of WS1 (8) or WS2 (18). DNA from the patients was first analyzed by exome sequencing. Seven of these families were submitted to trio analysis. For inconclusive cases, we applied a targeted NGS panel targeting WS/neurocristopathies genes. Causative variants were detected in 20 of the 26 probands analyzed, these being five in PAX3, eight in MITF, two in SOX10, four in EDNRB, and one in ACTG1 (type 2 Baraitser-Winter syndrome, BWS2). In conclusion, in our cohort of patients, the detection rate of the causative variant was 77%, confirming the superior detection power of NGS in genetically heterogeneous diseases. Full article

Surgery for cochlear implant is a traumatic procedure, with inflammatory responses leading to immediate and delayed intracochlear changes, resulting in newly formed fibrous and bony tissue. This newly formed tissue is thought to affect speech perception with cochlear implants and can also play a role in causing device malfunctioning and soft failures. We present a case of left cochlear implant explantation and reimplantation in a 15-year-old girl, who experienced deterioration of speech perception and device failure associated with osteoneogenesis of the round window, which could represent a cause of cochlear implant failure. To avoid surgical trauma of the cochlear lateral wall, enlarged round window insertion rather than a cochleostomy, soft surgical techniques, and the application of steroids are all important issues to prevent new tissue formation, although special attention should also be given to the trauma of round window borders. Full article