Search Results (29)

Objectives: Lermoyez syndrome (LS) is a rare variant of endolymphatic hydrops with a unique clinical presentation characterized by reversible sensorineural hearing loss preceding vertigo. This review aims to synthesize available literature on LS to clarify its clinical characteristics, diagnostic approach, management strategies, and outcomes, and to highlight the distinguishing features from Menière’s disease (MD). Methods: A systematic literature review according to PRISMA guidelines was conducted from 1919 to 2025. The extracted data included demographics, symptom profiles, audiovestibular testing, imaging findings, treatment approaches, and patient outcomes. Results: A total of 23 studies were identified, reporting 53 individual cases of LS. Patients ranged from 27 to 85 years of age, with a mean age of 50.34 years and a male predominance (64.1%). The hallmark of LS across cases was a reproducible clinical pattern of unilateral low-frequency hearing loss followed by vertigo and subsequent auditory recovery. Audiometry typically confirmed reversible sensorineural hearing loss, while vestibular tests and imaging were often unremarkable, primarily used to exclude alternative diagnoses. Treatment approaches varied and were often based on MD protocols, including dietary modifications, vasodilators, diuretics, and vestibular suppressants. Prognosis was generally favorable, with most patients experiencing both hearing recovery and symptom resolution. Conclusions: LS remains a clinically distinct but underrecognized inner ear disorder. Its defining feature—the paradoxical improvement in hearing after vertigo—distinguishes it from Menière’s disease and should prompt clinicians to consider LS in differential diagnosis. Due to the rarity of LS and the lack of standardized guidelines, diagnosis and treatment rely on careful clinical assessment and individualized management strategies. Full article

Background/Objectives: This study aimed to investigate the optimal sound presentation level for sound localization testing to assess the effect of hearing interventions in individuals with unilateral conductive hearing loss (UCHL). Methods: Nine participants with normal hearing were tested, and simulated two-stage UCHL was created using earmuffs and earplugs. We created two types of masking conditions: (1) only an earplug inserted, and (2) an earplug inserted with an earmuff worn. A sound localization test was performed for each condition. The sound presentation levels were 40, 45, 50, 55, 60, 65, and 70 dB SPL, and the results were evaluated using root mean square and d-values. Results: Both values showed little difference in masking Condition 2, regardless of the sound presentation level, whereas in masking Condition 1, the values were at their minimum at 55 dB SPL. In addition, comparing the differences between masking Conditions 1 and 2 for each sound presentation level, the greatest difference was observed at 55 dB SPL for both values. Conclusions: The optimal sound presentation level for sound localization testing to assess hearing intervention effects in UCHL was 55 dB. This result may be attributed to the effect of input from the non-masked ear, accounting for interaural attenuation; the effect was considered minimal at 55 dB SPL. Full article

Background: Hearing-related disease is a significant cause of disability worldwide. In resource-limited settings, prevention and early detection are critical for preventing severe disease. Understanding what a population knows and believes with regards to hearing health can be critical in identifying knowledge gaps and developing targeted interventions. Objective: To assess community awareness of hearing health and otitis media (OM) treatment, both modern and traditional, to inform educational programs. Methods: A retrospective review of clinical records from 52 patients (aged 1–79 years) diagnosed with OM during a 3-day hearing health clinic in Kasungu district, Malawi was conducted. Patients diagnosed with OM during the clinic were invited to provide additional details about their hearing health. Surveys contained open-ended questions to assess knowledge and beliefs regarding the cause of their infection and therapies they had previously used for treatment, including home remedies and prescribed medications from allopathic providers or traditional healers. A WHO adapted survey on hearing knowledge was also administered. Results: Hearing loss was identified in 60% of participants. Otoscopy revealed either bilateral or unilateral drainage in 69% of participants and perforation in 73%. Confidence in understanding the causes and treatments of OM was voiced by 60% of participants and 54% had used home remedies as treatment. Of the 11 home remedies used, none aligned with modern medical practice, and only two were recommended by local herbalists. Conclusions: Hearing-related disease contributes significantly to global disability, particularly in resource-limited settings. Educational campaigns to improve hearing health knowledge offer low-cost yet impactful solutions and implementation via partnerships with community leaders and traditional healers can be critical to addressing hearing health challenges. The use of nonantibiotic antimicrobials should be explored further, as these are low-cost and readily available. However, therapeutic alliance between patients and healthcare providers remains crucial. Full article

Background/Objectives: Hearing loss in childhood is associated with significant challenges in linguistic and cognitive development, particularly affecting language skills such as syntax, semantics, and pragmatics, which are essential for effective communication and social integration. This study aimed to analyze how different types and degrees of hearing loss impact linguistic development in children, and to identify clinical factors—such as age at diagnosis and years of language intervention—that may predict language performance. Methods: This study included a sample of 140 children aged 6 to 12, categorized into seven groups based on their hearing condition: unilateral and bilateral conductive, unilateral and bilateral sensorineural, unilateral and bilateral mixed hearing loss, and a control group with no hearing loss. Linguistic development was assessed using the Clinical Evaluation of Language Fundamentals-5 (CELF-5), a validated tool for diagnosing language disorders. Statistical analyses, including MANOVA and multiple regression, were conducted to evaluate differences in linguistic skills across groups and to determine the predictive value of clinical variables on total language performance. Results: The analysis revealed statistically significant differences across groups in all assessed linguistic domains (p < 0.001), with children with severe or bilateral hearing loss exhibiting notably lower scores compared to normohearing peers. The multiple regression analysis indicated that type of hearing loss was the strongest predictor of total linguistic performance (β = −0.674), followed by age at diagnosis (β = −0.285) and age of hearing device adaptation (β = −0.220). Years of language intervention also contributed significantly (β = 0.198) but to a lesser extent. Conclusions: This study highlights the critical impact of early and comprehensive auditory and language intervention on linguistic outcomes for children with hearing impairments. Early diagnosis and timely adaptation of hearing aids or cochlear implants are essential in mitigating language deficits, particularly in areas like syntax and pragmatic skills. These findings support the need for specialized, long-term interventions tailored to the severity and type of hearing loss to improve language development in this population. Full article

Introduction: Based on clinical practice guidelines, the application of corticosteroids as a first-line therapy is common. Although sudden sensorineural hearing loss (SSHL) etiology is primarily idiopathic, hearing loss can result from a perilymphatic fistula (PLF). Recent findings show evidence of a specific rate of PLF based on a cochlin-tomoprotein (CTP) detection test. Based on this rate of PLF treatment, the concepts of SSNHL need to be re-evaluated. The present study aimed to evaluate CTP in SSNHL patients and compare pre-surgical and post-surgical pure tone hearing thresholds after round and oval window sealing as first-line treatment. Material and Methods: A total of 30 patients with unilateral SSNHL with a pure tone average (PTA) (4 Freq. of 60 dB or more were enrolled in a prospective study. All patients underwent tympanoscopy for middle ear exploration as a first-line treatment. After intraoperative observation of a possible PLF, all patients obtained middle ear lavage to gain CTP samples for following ELISA-based CTP detection tests. All patients received round window and oval window sealing with fascia. PTA hearing thresholds were analyzed post-surgically 3 weeks after treatment based on 4-frequency bone conduction (BC). Results: The average preoperative pure tone BC threshold was 97.7 dB compared with the 69 dB postoperative BC threshold. Mean BC improved by 20.3 dB after middle ear exploration and window sealing. A total of 56% (17 of 30) of patients recovered at least 10 dB. The middle ear cochlin-tomoprotein detection rate was 70% positive. Conclusions: The combination of early tympanoscopy and inner ear-specific cochlin-tomoprotein as a detection tool for suspected PLF showed evidence of PLF as a key causative in SSNHL. Full article

Background: With bilateral hearing loss, the main problems for the patient are speech understanding in noise and, especially in asymmetrical hearing loss, an inability to correctly localize sound sources. There are multiple methods of treatment and rehabilitation for people with conductive hearing loss, and one of them is to use an active bone conduction implant. This case study is designed to evaluate the auditory benefits and sound localization accuracy with active bilateral bone conduction implants—in comparison to unilateral ones—in a patient with congenital bilateral conductive hearing loss caused by a congenital malformation. We assess subjective and audiological benefits (functional, directional hearing, speech comprehension in quiet and noise). Case report: This study describes the results in a 15-year-old patient with bilateral congenital malformation of the outer ears and associated conductive hearing loss who was treated with two Bonebridge active bone conduction implants. Speech recognition ability, hearing thresholds, and sound localization were tested under three conditions: unaided, unilateral on the right-side, and bilateral on both sides. The patient filled in an Abbreviated Profile of Hearing Aid Benefit questionnaire (APHAB) to evaluate limitations in daily life caused by hearing impairment. The results show an improvement in free-field hearing thresholds and the ability to discriminate speech, both in quiet and in noise after implantation. Subjectively, the patient had significantly fewer problems with two implants than with one (or with no implant) in terms of hearing in everyday situations. Conclusions: Unilateral use of the Bonebridge device in a patient with congenital bilateral conductive hearing loss did not provide full benefits. However, bilateral implantation improved speech understanding in noise and sound localization. Full article

Background: The reported data on European universal neonatal hearing screening (UNHS) practices tend to be scarce, despite the fact that the European Union project, EUScreen, collected unofficial data from 38 collaborating European institutions. The objectives of this systematic review were as follows: (a) to identify the most recent (in a 20-year span) literature information about UNHS programs in Europe and (b) to provide data on the procedures used to assess the population, the intervention policies, and on the estimated prevalence of congenital hearing loss with emphasis on the bilateral hearing loss cases. Methods: Queries were conducted via the Pubmed, Scopus and Google Scholar databases for the time period of 2004–2024. The Mesh terms used were “OAE”, “Universal Neonatal Hearing Screening”, “congenital hearing loss” and “well babies”. Only research articles and review papers of European origin were considered good candidates. The standard English language filter was not used, in order to identify information from non-English-speaking scientific communities and groups. Results: Very few data and reports were identified in the literature search. Eleven manuscripts were identified corresponding to eight UNHS programs. Except in Poland, most of the data refer to regional and not national programs. The screening coverage estimates of all programs exceed 90%; infants were mostly assessed by a three-stage protocol (TEOAE + TEOAE + AABR), followed by a clinical ABR test. The average prevalence (i.e., from well babies AND NICU infants) of bilateral hearing loss ranged from 0.5 to 20.94 per 1000 (Zurich sample). Infants presenting unilateral or bilateral hearing losses were first rehabilitated by hearing aids and consequently (>15 mo) by cochlear implants. Conclusions: Even though UNHS programs are well-established clinical practices in the European States, the amount of information in the literature about these programs is surprising low. The existing data in the timespan 2004–2024 corroborate the international UNHS data in terms of coverage and bilateral hearing loss prevalence, but there is a strong need to supplement the existing information with the latest developments, especially in the area of hearing loss rehabilitation. Full article

Outer and middle ear pathologies are known to disproportionately affect low-income countries but data is limited. We aim to quantify the prevalence rate of patients presenting with middle/outer ear pathologies at ABC Hearing Clinic and Training Centre in Lilongwe, Malawi. Audiological consultations (adult and paediatric) from 2018–2020 were reviewed for outer and middle ear pathologies. Secondary outcomes included patient type (private vs. community) compared to otoscopy findings, tympanometry findings, need for follow up, and follow up compliance. Out of 1576 patients reviewed, the proportion of abnormal cases’ was 98.2%, with 41.4% being unilateral and 57.4% bilateral. Eighty-three percent presented with outer/middle ear pathologies. 68% of those presented with a pathology often associated with some degree of conductive hearing loss (occluding wax, perforation, discharge, Type B/Type C tympanogram). Average age was 29 + 0.527 years; 41.6% private and 58.2% community patients. Cerumen impaction was most common finding (51%). Higher rates of otoscopic abnormalities and type B tympanograms were noted in community vs. private patient (~40% vs. ~30%; ~70% vs. ~30%). Adherence to follow up was higher for community vs. private patients (29% vs. 17%); ~70% reported subjective improvement upon follow up. The majority required multiple interventions on follow up. Secondary follow up was recommended in 64.8%. A significant disease burden of outer and middle ear pathologies was identified. Further research is required to understand the disease burden and promote health policy. Full article

Objectives: Simultaneous removal and cochlear implantation (CI) have been reported in intralabyrinthine and intracochlear schwannoma. A wide range of postoperative hearing outcomes have been reported after CI in these cases. This study evaluated the outcomes of performing a simultaneous resection of Schwannoma in cochlea and cochlear implantation (CI), aiming to assess the effectiveness of this combined surgical approach for hearing rehabilitation with CI. Methods: This retrospective case series was conducted at a tertiary care center. The study included four consecutive patients with profound sensorineural hearing loss due to a mass inside the cochlea. These patients underwent simultaneous single-sided CI and tumor resection performed by the same surgeon. Preoperative and postoperative audiological assessments were conducted to evaluate the patients’ hearing outcomes before and after the surgical intervention. Results: Simultaneous CI with tumor resection was successful in all cases. Two of the four patients had a unilateral tumor, while the other two had a bilateral tumor with the involvement of the internal auditory canal and cerebellopontine angle (neurofibromatosis type 2 (NF2)). In two cases of unilateral tumor, aided free-field pure tone average (PTA) was 26 dB, and 46 dB hearing level (HL), and word recognition score (WRS) at 65 dB was 40% and 68%, respectively, 3 months after surgery. In two cases of tumor with NF2, aided free-field PTA was 36 dB and 60 dB HL, and both cases showed 0% WRS at 65 dB 3 months after surgery. Conclusions: Simultaneous schwannoma excision and CI in patients with Schwannoma inside cochlea are surgically practical and safe. Postoperatively, there was a notable improvement in hearing in cases of sporadic schwannoma, regardless of the type of CI used. However, there was 0% WRS in the two NF2 patients with a mass in the internal auditory canal. Full article

Objective: Cochlear receptors are sensitive to vibratory stimuli. Based on this sensibility, bone-anchored hearing aids have been introduced to correct unilateral or bilateral conductive or mixed hearing loss and unilateral deafness. The vestibular system is also sensitive to the vibratory stimulus and this type of response is used in clinics to test its functionality. Being aware of this double separated sensibility, we wondered whether bone vibration, which activates the acoustic receptors of patients with bone conduction aids, can also influence the functionality of the vestibular system. Methods: To this end, we recruited 12 patients with a bone-anchored hearing aid and evaluated their vestibular function with and without an activated vibratory acoustic device. Results: Our results show that the vibratory stimulus delivered by the bone conduction aid also reaches and stimulates the vestibular receptors; this stimulation is evidenced by the appearance or modification of some nystagmus findings during bedside vestibular testing. Despite this, none of these patients complained of dizziness or vertigo during prosthesis use. Nystagmus that appeared or changed during acoustic vibratory stimulation through the prosthesis was almost all predominantly horizontal, unidirectional with respect to gaze or body position, inhibited by fixation, and most often consistent with vestibular function tests indicating peripheral vestibular damage. Conclusions: The findings of sound-evoked nystagmus seem to indicate peripheral rather than central vestibular activation. The occurrence of some predominantly horizontal and high-frequency induced nystagmus seems to attribute the response mainly to the utricle and lateral semicircular canal. Full article

Forty-nine children who started wearing cartilage conduction hearing aids (CC-HAs) before completing elementary school (17 with bilateral hearing loss and 32 with unilateral hearing loss) were followed-up and examined. The wearing and utilization status of the CC-HA and its progress to date were evaluated. In addition, 33 participants who purchased the CC-HAs were interviewed to assess the wearing effect. Eleven of seventeen children with bilateral hearing loss and 25 of 32 children with unilateral hearing loss continued to use the CC-HAs. In terms of wearing effect, a good wearing effect was reported, even by those with unilateral hearing loss. In cases where it was difficult to wear CC-HAs stably with pasting or ear tips, it was possible to fix them stably using commercially available hair bands and eyeglass vines. In two cases, the CC-HAs were worn from infancy. With ingenuity and appropriate educational and medical support, it is possible to wear CC-HAs from infancy. Full article

Congenital unilateral hearing loss (UHL) represents a contemporary audiologic challenge. Children with UHL can struggle with understanding speech in noise, localizing sounds, developing language, and maintaining academic performance, leading to low self-esteem, anxiety, and decreased social support. Two specific conditions related to UHL in children are single-sided deafness (SSD) and unilateral auris atresia (UAA). This was a retrospective observational study on a group of children with UHL. The Simplified Italian Matrix Sentence Test was used for the assessment of speech reception threshold (SRT) in different conditions: speech and noise from the front (S0N0), speech at 45° from the side of the better ear and noise at 45° from the opposite side (SbNw), and vice versa (SwNb). Each test was conducted unaided, with a bone-anchored hearing device (BAHD), and with a remote microphone (RM) system. The use of a BAHD and RM led to an improvement in SRT in S0N0 and SwNb conditions. The SSD subgroup demonstrated significant benefits with both devices in SwNb, and the UAA subgroup from the use of BAHD in S0N0. In conclusion, the study underscores the potential benefits of both devices in enhancing speech perception for UHL children, providing insights into effective intervention strategies for these challenging cases. Full article

Bone conduction devices (BCDs) are widely used in the treatment of conductive hearing loss (CHL), but their applications on unilateral CHL (UCHL) patients remain controversial. To evaluate the effects of BCDs in UCHL, a systematic search was undertaken until May 2023 following the PRISMA guidelines. Among the 391 references, 21 studies met the inclusion criteria and were ultimately selected for review. Data on hearing thresholds, speech recognition, sound localization, and subjective questionnaire outcomes were collected and summarized. Moderate hearing threshold improvements were found in UCHL patients aided with BCDs. Their speech recognition abilities improved significantly. However, sound localization results showed wide individual variations. According to subjective questionnaires, BCDs had an overall positive influence on the daily life of UCHL patients, although several unfavorable experiences were reported by some of them. We concluded that the positive audiological benefits and subjective questionnaire results have made BCDs a credible intervention for UCHL patients. Before final implantations, UCHL patients should first go through a period of time when they were fitted with non-implantable BCDs as a trial. Full article

Background: To assess and compare binaural benefits and subjective satisfaction of active bone conduction implant (BCI) in patients with bilateral conductive or mixed hearing loss fitted with bilateral BCI and patients with monaural conductive hearing loss fitted with monaural BCI. Methods: ITA Matrix test was performed both on patients affected by bilateral conductive or mixed hearing loss fitted with monaural bone conduction hearing implant (Bonebridge, Med-El) before and after implantation of contralateral bone conduction hearing implant and on patients with monaural conductive or mixed hearing loss before and after implantation of monaural BCI. The Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire was administered to both groups of subjects and the results were compared with each other. Results: Patients of group 1 reported a difference of 4.66 dB in the summation setting compared to 0.79 dB of group 2 (p < 0.05). In the squelch setting, group 1 showed a difference of 2.42 dB compared to 1.53 dB of group 2 (p = 0.85). In the head shadow setting, patients of group 1 reported a difference of 7.5 dB, compared to 4.61 dB of group 2 (p = 0.34). As for the APHAB questionnaire, group 1 reported a mean global score difference of 11.10% while group 2 showed a difference of −4.00%. Conclusions: Bilateral BCI in patients affected by bilateral conductive or mixed hearing loss might show more advantages in terms of sound localisation, speech perception in noise and subjective satisfaction if compared to unilateral BCI fitting in patients affected by unilateral conductive hearing impairment. This may be explained by the different individual transcranial attenuation of each subject, which might lead to different outcomes in terms of binaural hearing achievement. On the other hand, patients with unilateral conductive or mixed hearing loss fitted with monaural BCI achieved good results in terms of binaural hearing and for this reason, there is no absolute contraindication to implantation in those patients. Full article

Clinical findings on cartilage conduction hearing aids (CCHAs) have gradually become clear; however, few reports include a large number of cases. This study included 91 ears from 69 patients who underwent CCHA fitting in our hospital. Their ears were divided into six groups (i.e., bilateral aural atresia or severe canal stenosis, unilateral aural atresia or severe canal stenosis, chronic otitis media or chronic otitis externa with otorrhea, sensorineural hearing loss, mixed hearing loss, and conductive hearing loss) according to their clinical diagnosis and type of hearing loss. Most clinical diagnoses were aural atresia or meatal stenosis (bilateral, 21.8%; unilateral, 39.6%). The purchase rate of CCHAs was higher in the closed-ear group (bilateral, 77.3%; unilateral, 62.5%). In the bilateral closed-ear group, air conduction thresholds at 1000, 2000, and 4000 Hz and aided thresholds with CCHAs at 4000 Hz were significantly lower in the purchase group than the non-purchase group. No significant difference was observed between the purchase and non-purchase groups in the unilateral closed-ear group. In the bilateral closed-ear group, air conduction thresholds and aided thresholds were associated with the purchase rate of CCHAs. In the unilateral closed-ear group, factors other than hearing might have affected the purchase rate of CCHAs. Full article