Interaction of sensory processing and balance in adult cochlear implant users.

PUROPSE: The aim of this study was to evaluate the sensory processing skills, quality of life and balance performance in adult cochlear implant users. METHODS: A sample of 40 individuals was studied in two groups; 20 normal hearing individuals (37.84 ± 15.39 years old) and 20 cochlear implant users (35.58 ± 11.22 year old). Balance performance was assessed by Computerized Dynamic Posturography. The Adolescent/Adult Sensory Profile completed by the individual was used to assess sensory processing skills. Finally, quality of life was assessed with the Short Form-36. RESULTS: Among the different sensory processing parameters in the adolescent/adult sensory profile, a significant difference was found between the two groups in the parameters of low registration, sensory sensitivity and sensory avoidance (p < .05). Visual, vestibular and composite scores were found to be lower in cochlear implant users by Computerised Dynamic Posturography assessment (p < .05). When assessing quality of life, emotional well-being and social functioning parameters were found to be lower in cochlear implant users (p < .05). CONCLUSION: Factors such as the inability to reach sufficient auditory stimuli due to hearing loss and the occurrence of vestibular problems after cochlear implant surgery limit the quantity and quality of sensory stimuli from the environment. Individuals with cochlear implants may prefer to live isolated from society because they cannot adequately process incoming sensory stimuli due to hearing/balance problems, and this may negatively affect the quality of life of individuals. Our findings revealed the necessity of multisensory assessment and therapy protocols when rehabilitating individuals with cochlear implants.

Validity and reliability of the ERSA questionnaire in Turkish.

This methodological study aimed to assess the validity and reliability of the Turkish version of the Evaluation of the Impact of Hearing Loss in Adults (ERSA) questionnaire for individuals with treated hearing loss. The study involved 200 participants, and both exploratory factor analysis and confirmatory factor analysis were used to examine structural validity. External validity was assessed by correlating ERSA scores with the Abbreviated Profile of Hearing Aid Benefit (APHAB). Internal consistency and test-retest reliability were evaluated using Cronbach's alpha and the intraclass correlation coefficient, respectively. The Turkish ERSA demonstrated strong psychometric properties, with significant correlations between APHAB and ERSA scores and excellent internal consistency and test-retest reliability. The findings suggest that the Turkish ERSA is a valid and reliable tool for evaluating the impact of hearing loss in individuals.

Outcomes of Treatment of Multicanal Benign Paroxysmal Positional Vertigo by Means of TRV Chair.

INTRODUCTION: Benign paroxysmal positional vertigo (BPPV) is one of the most common causes of peripheral vertigo and can manifest in different forms. Multicanal BPPV is rare and difficult to treat. TRV Chair is a method that offers diagnosis and treatment at the same time. The aim was to analyze the outcomes of treatment of multicanal BPPV by means of TRV Chair and compare those results with manual maneuvers. METHODS: Forty-nine subjects aged 26-73 and diagnosed with multicanal BPPV were included. Appropriate maneuvers were performed on affected canals either by TRV Chair or manually. The number of maneuvers required by TRV Chair and manual maneuver were analyzed and compared. RESULTS: The mean number of maneuvers performed until the treatment was completed in manual treatment group was 2.57 ± 1.03, and in TRV group, it was 2.25 ± 1.16. No significant difference was observed between the number of maneuvers performed in either group until the end of the treatment (p > 0.05). DISCUSSION/CONCLUSIONS: Neither TRV Chair nor manual maneuvers were superior to the other in the treatment of multicanal BPPV cases.

Processes of Emotion Idioms Comprehension of Turkish-Speaking People with Wernice's Aphasia.

INTRODUCTION: Idioms are commonly used in everyday language to convey emotions figuratively. The ability to comprehend and use idioms that incorporate emotional elements is crucial for effective communication in daily life, particularly among people with aphasia (PwA). Despite the interest in understanding the process of emotion idiom comprehension in PwA, limited information is available in the literature. Therefore, this study aimed to investigate the process of emotion idiom comprehension in people with Wernice's aphasia (PwWA) and compare it with that of neurotypical individuals. METHODS: Sixty idioms were selected based on their syntactic and semantic features, and participants evaluated their imageability. Sixteen idioms were chosen for the study and two types of tasks were prepared: written idiom-picture matching and written idiom-written text matching. These tasks were administered to two groups: 11 PwWA and 11 neurotypical individuals. The results were analysed in terms of task performance, response type, syntactic and semantic features, and emotional content. RESULTS: The emotion idiom comprehension scores of PwWA group were significantly lower than those of the neurotypical participants. PwWA had greater difficulty with the written idiom-picture matching task and tended to rely on the literal meanings of the idioms. There were differences in the semantic features between the two groups. Among the emotional idioms, PwWA showed significant differences in the types of emotions they were able to comprehend. CONCLUSIONS: The findings of this study suggest that regardless of the syntactic content of idioms, PwWA's ability to comprehend emotion idioms is impaired, and they tend to interpret them more literally. This study provides a useful method for assessing emotional idiom comprehension in PwA.

Does glutaric aciduria type 1 affect hearing function?

This study aimed to evaluate audiological findings among patients with glutaric aciduria type 1 (GA-1). We used a large test battery for the audiological evaluation of 17 individuals with GA-1 (the study group) and 20 healthy individuals (the control group). Conventional audiometry (0.125-8 kHz), distortion product otoacoustic emissions (DPOAEs) (1, 1.5, 2, 3, 4, 6, and 8 kHz), contralateral suppression of otoacoustic emissions, and auditory brainstem response (ABR) ( 30, 50, 70 and 90 dB nHL) were measured for all participants (n = 37). Mild sensorineural hearing loss was found in 77.47% (n = 13) of the patients with GA-1, and normal hearing thresholds were seen in 23.53% (n = 4). There were three asymptomatic patients at the time of diagnosis [two developed mild mental motor retardation (MMR) and one developed severe MMR during the follow-up], one with a normal hearing threshold and two with mild hearing loss), and 14 symptomatic patients (three with normal hearing thresholds and 11 with mild hearing loss). Seven of the symptomatic patients diagnosed following an encephalopathic crisis required intensive care and showed significantly worse hearing thresholds than those without symptoms [20.86 ± 4.47 vs. 15.44 ± 3.96 decibel hearing level (dB HL), p = 0.039*], while five had mild-to-moderate hearing loss. Acute encephalopathic crisis had a negative effect on hearing function in the symptomatic patients. The emission and contralateral suppression amplitude values of the study group were significantly lower compared to the control group (p < 0.05). The I-V interpeak latency and absolute latencies of ABR waves I, III, and V of the study group were observed to be significantly prolonged and morphologically distorted compared to those of the control group (p < 0.05). Five patients had MMR, and three had moderate MMR; all eight had mild-to-moderate hearing loss. In addition, of the eight patients with mild MMR, four had mild hearing loss. In particular, the morphological findings of ABR waves were significantly worse in the patients with severe and moderate MMR (p < 0.05). There was a significant correlation between a macrocephaly history (12 patients) and hearing loss (p = 0.041*). Magnetic resonance imaging findings were evaluated in all the 17 patients with GA-1, and typical fronto-temporal atrophy and sylvian fissure enlargement were observed. Our findings support that GA-1 is associated with auditory impairment, primarily in symptomatic patients. Adequate audiological test battery evaluation is essential in this context, particularly for symptomatic patients with a history of encephalopathic crises.

Dysfunction of GRAP, encoding the GRB2-related adaptor protein, is linked to sensorineural hearing loss.

We have identified a GRAP variant (c.311A>T; p.Gln104Leu) cosegregating with autosomal recessive nonsyndromic deafness in two unrelated families. GRAP encodes a member of the highly conserved growth factor receptor-bound protein 2 (GRB2)/Sem-5/drk family of proteins, which are involved in Ras signaling; however, the function of the growth factor receptor-bound protein 2 (GRB2)-related adaptor protein (GRAP) in the auditory system is not known. Here, we show that, in mouse, Grap is expressed in the inner ear and the protein localizes to the neuronal fibers innervating cochlear and utricular auditory hair cells. Downstream of receptor kinase (drk), the Drosophila homolog of human GRAP, is expressed in Johnston's organ (JO), the fly hearing organ, and the loss of drk in JO causes scolopidium abnormalities. drk mutant flies present deficits in negative geotaxis behavior, which can be suppressed by human wild-type but not mutant GRAP. Furthermore, drk specifically colocalizes with synapsin at synapses, suggesting a potential role of such adaptor proteins in regulating actin cytoskeleton dynamics in the nervous system. Our findings establish a causative link between GRAP mutation and nonsyndromic deafness and suggest a function of GRAP/drk in hearing.

Transcutaneous Electric Nerve Stimulation in Chronic Subjective Tinnitus.

OBJECTIVE: It is aimed to determine the therapeutic role of transcutaneous electrical nerve stimulation (TENS) on chronic subjective tinnitus with a randomized and comparative analysis. METHOD: 46 individuals with normal hearing, chronic subjective tinnitus, aged 20-65; randomly divided into the study (TENS therapy) and tje control groups. The control group (n = 23) was informed only about tinnitus, while the study group (n = 23) received TENS therapy (20 sessions in 4 weeks). Before TENS therapy, tinnitus-related evaluations of all participants [tinnitus frequency, loudness, minimum masking level (MMS), residual inhibition (RI)] were made and Tinnitus Handicap Inventory (THI), Visual Analogue Scale (VAS), Short Form 36 (SF-36), and The Quality of Life Scale were applied to the participants. These evaluations and questionnaires were repeated after 4 weeks of the therapy. RESULTS: There was a significant decrease in the tinnitus severity after TENS therapy in the study group (p = 0.003). Moreover, it was found that VAS (loudness and annoyance), THI (functional, emotional and total score), SF-36 (physical function, physical role, pain, general health, vitality, emotional role and social function) values improved compared to before TENS therapy and the difference was significant (p < 0.05). There was no significant difference between the first and last evaluations of the control group (p > 0.05). CONCLUSION: TENS is an effective, practical and reliable therapy method in reducing severity, loudness, and annoyance of tinnitus, and increasing the quality of life in individuals with chronic subjective tinnitus.

Audiological involvement in patients with systemic sclerosis.

OBJECTIVES: The aim of the present study was to evaluate hearing loss in patients diagnosed with systemic sclerosis (SSc) and to investigate the relation between hearing loss, subtypes of the disease, its duration and clinical findings, and antibody positivity. METHODS: The study included 47 patients with SSc and 44 healthy controls. Audiometric, tympanometric, and otoacoustic emission measurements were applied to both groups. RESULTS: The evaluation of the participants medical history showed that among the patients with SSc, 19.1% experienced ear fullness, 27.7% experienced vertigo, and 36.2% experienced tinnitus. Hearing loss was detected in 23.4% of the patients with SSc. The corresponding result was 4.3% in the control group with a statistically significant difference (p = 0.001). Transient-evoked otoacoustic emission amplitude values were significantly lower in the patients' both ears with SSc than the control group (p < 0.005). Duration of disease was significantly longer, and diffusing capacity of the lungs for carbon monoxide values were significantly higher in the patients with SSc and sensorineural hearing loss. CONCLUSIONS: The present study found that the incidence of hearing loss was significantly higher in the study group than in the healthy control group. In addition to other organ involvements, cochlear involvement occurs in these patients, and further studies are required.

A truncating CLDN9 variant is associated with autosomal recessive nonsyndromic hearing loss.

While the importance of tight junctions in hearing is well established, the role of Claudin- 9 (CLDN9), a tight junction protein, in human hearing and deafness has not been explored. Through whole-genome sequencing, we identified a one base pair deletion (c.86delT) in CLDN9 in a consanguineous family from Turkey with autosomal recessive nonsyndromic hearing loss. Three affected members of the family had sensorineural hearing loss (SNHL) ranging from moderate to profound in severity. The variant is predicted to cause a frameshift and produce a truncated protein (p.Leu29ArgfsTer4) in this single-exon gene. It is absent in public databases as well as in over 1000 Turkish individuals, and co-segregates with SNHL in the family. Our in vitro studies demonstrate that the mutant protein does not localize to cell membrane as demonstrated for the wild-type protein. Mice-lacking Cldn9 have been shown to develop SNHL. We conclude that CLDN9 is essential for proper audition in humans and its disruption leads to SNHL in humans.

Novel variant p.E269K confirms causative role of PLS1 mutations in autosomal dominant hearing loss.

Auditory reception relies on the perception of mechanical stimuli by stereocilia and its conversion to electrochemical signal. Mechanosensory stereocilia are abundant in actin, which provides them with structural conformity necessary for perception of auditory stimuli. Out of three major classes of actin-bundling proteins, plastin 1 encoded by PLS1, is highly expressed in stereocilia and is necessary for their regular maintenance. A missense PLS1 variant associated with autosomal dominant hearing loss (HL) in a small family has recently been reported. Here, we present another PLS1 missense variant, c.805G > A (p.E269K), in a Turkish family with autosomal dominant non-syndromic HL confirming the causative role of PLS1 mutations in HL. We propose that HL due to the p.E269K variant is from the loss of a stable PLS1-ACTB interaction.