Hearing Rehabilitation Research Articles

Photobiomodulation Can Enhance Stem Cell Viability in Cochlea with Auditory Neuropathy but Does Not Restore Hearing.

Sensorineural hearing loss is very difficult to treat. Currently, one of the techniques used for hearing rehabilitation is a cochlear implant that can transform sound into electrical signals instead of inner ear hair cells. However, the prognosis remains very poor if sufficient auditory nerve cells are not secured. In this study, the effect of mouse embryonic stem cells (mESC) and photobiomodulation (PBM) combined treatment on auditory function and auditory nerve cells in a secondary neuropathy animal model was investigated. To confirm the engraftment of stem cells in vitro, cochlear explants were treated with kanamycin (KM) to mimic nerve damage and then cocultured with GFP-mESC. GFP-mESCs were observed to have attached and integrated into the explanted samples. An animal model for secondary neurodegeneration was achieved by KM treatment and was treated by a combination therapy of GFP-mESC and NIR-PBM at 8 weeks of KM treatment. Hearing recovery by functional testing using auditory brain stem response (ABR) and eABR was measured as well as morphological changes and epifluorescence analysis were conducted after 2 weeks of combination therapy. KM treatment elevated the hearing threshold at 70-80 dB and even after the combination treatment with GFP-mESC and PBM was applied, the auditory function was not restored. In addition, the stem cells transplanted into cochlea has exponentially increased due to PBM treatment although did not produce any malignancy. This study confirmed that the combined treatment with mESC and PBM could not improve hearing or increase the response of the auditory nerve. Nevertheless, it is noteworthy in this study that the cells are distributed in most cochlear tissues and the proliferation of stem cells was very active in animals irradiated with PBM compared to other groups wherein the stem cells had disappeared immediately after transplantation or existed for only a short period of time.

Spatial Hearing Questionnaire: Psychometric Properties of Turkish Version and Correlations with Hearing Skills

Objective: Self-report questionnaire is informative to assess general hearing disability. The aims of this study were to investigate the reliability of Turkish version of spatial hearing questionnaire (SHQ) and to analyze the validity of the SHQ by the correlation with speech, spatial, and qualities of hearing questionnaire (SSQ) and Turkish matrix sentence test (TMST). Methods: The first part of the study was the psychometric properties of the SHQ with 192 participants (137 with normal hearing, 55 with hearing loss). In the second and main part of the study, we applied two questionnaires (SHQ and SSQ) and TMST to people other than those included in the first part of the study (88 participants with bilateral sensorineural hearing loss). We compared the results of these two questionnaires and the TMST with the speech discrimination (SD) scores. Results: Turkish spatial hearing questionnaire’s internal consistency was 0.94 and 0.97 for individuals with normal hearing and for individuals with hearing loss, respectively. Moderate, positive, statistically significant correlation was observed between the SHQ and SSQ (r = 0:606, p = 0:001 in individuals with hearing loss who do not wear any hearing aid, and r = 0:627, p = 0:001 in hearing aid users), and SHQ and SD (r = 0:561, p = 0:032 in hearing aid users). According to TMST, moderate, positive, statistically significant correlation was found between SSQ and adaptive TMST in individuals with hearing loss who do not wear any hearing aid (r = 0:330, p = 0:033 for S0N90 and r = 0:364, p = 0:018 for S0N270). Conclusions: Turkish SHQ is a valid and reliable questionnaire for assessing hearing functions. SHQ, SSQ, and TMST are clinically beneficial measuring tools in planning the process of hearing rehabilitation and follow-up.

Paediatric amplification protocol, based on individualization of the hearing aids’ fitting parameters

Background. There are no paediatric amplification clinical practice guidelines in Russia at the moment. At the same time the technological progress of hearing aids (HA), new algorithms and functions requires the objective evidence of safety and effectiveness for the hearing impaired children. For these reasons paediatric amplification algorithm was developed by the authors. Besides the standard procedures the algorithm includes an objective electroacoustic verification of the output parameters and separate HA functions. The research is devoted to the investigation of the protocols advantages. 
 Aim development and evaluation the paediatric amplification protocol, based on objective electroacoustic verification of the output and functions of hearing aid. 
 Materials and Methods. Prospective, randomized, controlled clinical trial was performed. Two protocols effectiveness were compared. Standard protocol (control group) is based on the subjective verification. Experimental protocol suggests an electroacoustic verification of the output and different HA functions: feedback cancellation, amplitude and frequency compression, microphone directionality, digital noise reduction (experimental group). Each group included 56 children (317 years old) with permanent hearing loss from moderate to moderately-severe degree. Initial amplification was performed for each child; the results were estimated in 1, 3 and 6 months using PEACH and LIFE questionnaires, speech audiometry and phoneme testing. DataLogging and first fitting appointment time were also estimated. 
 Results. In preschool-age children of the experimental group post-amplification PEACH results were 6% better than in controls. In school-age children (LIFE questionnaire) results were 11% better comparing with control group. Speech intelligibility in quiet was 3.1% (p 0.05) higher for pre-schoolers in the experimental group and 9.3% (p 0.01) higher for school-age children comparing with control group. Speech intelligibility in noise was higher in experimental group than in control: 7.8% (p 0.05) in pre-schoolers and 13% (p 0.01) for school-age children. Phoneme recognition was better in experimental group as well: 4.5% (p 0.05) in pre-schoolers and 9.8% (p 0.01) in school-agers than in control group. After HA fitting following an experimental protocol DataLogging time was 1112% longer in comparison with standard procedure. On the other hand, experimental protocol took in average 1.5 hours for the first fitting and standard protocol 53 minutes. 
 Conclusions. Protocol of paediatric amplification based on objective electroacoustic verification of HA output and functions allows to significantly increase the effectiveness of hearing rehabilitation in children.

Effect of bilateral cochlear implants on language development in children aged 2-7 years

Aims: Pediatric patients with bilateral total sensorineural hearing loss have very poor or no language development compared to their peers. The hearing and language development of these patients is usually managed via cochlear implants (CIs). 
 Methods: This study examined the factors that affect the language development of children aged 24-84 months who have undergone CI surgery. The language development outcomes of patients with bilateral CIs and patients with unilateral CIs were compared. The participants were receiving regular hearing rehabilitation training and had undergone unilateral or bilateral CI surgery at various centers. Their language development was evaluated using the Turkish adaptation of the Test of Early Language Development-3 (TELD-3). 
 Results: The expressive language development of the patients with unilateral implants was delayed by 14.0±18.1 months, while the expressive language development of patients with bilateral implants was delayed by 2.8±8.7 months. This difference was statistically significant (p=0.025). 
 Conclusion: Although the levels of receptive language development of patients with bilateral and unilateral CIs were similar, the expressive language development of patients with bilateral CIs was better. We recommend that bilateral CI surgery be performed in a single session for patients with congenital bilateral total sensorineural hearing loss.

Comparing Speech Recognition Outcomes Between Cochlear Implants and Auditory Brainstem Implants in Patients With NF2-Related Schwannomatosis.

To compare cochlear implant (CI) and auditory brainstem implant (ABI) performance in patients with NF2-related schwannomatosis (NF2). Historical cohort. Tertiary academic center. A total of 58 devices among 48 patients were studied, including 27 ABIs implanted from 1997 to 2022 and 31 CIs implanted from 2003 to 2022. Three patients had bilateral ABIs, three had bilateral CIs, three had an ABI on one side and a CI on the other, one had a CI that was later replaced with an ipsilateral ABI, and one had an ABI and CI concurrently on the same side. CI or ABI ipsilateral to vestibular schwannoma. Open-set speech perception, consonant-nucleus-consonant word scores, and AzBio sentence in quiet scores. Among all patients, 27 (47%) achieved open-set speech perception, with 35 (61%) daily users at a median of 24 months (interquartile range [IQR], 12-87 mo) after implantation. Comparing outcomes, CIs significantly outperformed ABIs; 24 (77%) CIs achieved open-set speech perception compared with 3 (12%) ABIs, with median consonant-nucleus-consonant and AzBio scores of 31% (IQR, 0-52%) and 57% (IQR, 5-83%), respectively, for CIs, compared with 0% (IQR, 0-0%) and 0% (IQR, 0-0%), respectively, for ABIs. Patients with ABIs were younger at diagnosis and at implantation, had larger tumors, and were more likely to have postoperative facial paresis. Many patients with NF2-associated vestibular schwannoma achieved auditory benefit with either a CI or an ABI; however, outcomes were significantly better in those patients who were able to receive a CI. When disease and anatomy permit, hearing rehabilitation with a CI should be considered over an ABI in these patients. Tumor management strategies that increase the ability to successfully use CIs should be strongly considered given the high risk of losing bilateral functional acoustic hearing in this population.

Factors associated with delayed referral and hearing rehabilitation for congenital sensorineural hearing loss

ObjectivesTo identify the impact of social determinants of health and clinical status on referral and intervention for congenital sensorineural hearing loss. Study designRetrospective chart review of children with confirmed sensorineural or mixed hearing loss between 2013 and 2021 at a single academic medical institution. MethodsReferral rates and timing for hearing rehabilitation, rates and timing of completed evaluation, and rate and timing of amplification were recorded. Patient demographics included gestational age, race, ethnicity, sex, hearing loss severity, and CMV status. ResultsThere were 216 children with confirmed sensorineural or mixed hearing loss, of which 77 had a unilateral hearing loss and 89 a severe or profound hearing loss. Delayed referral for hearing aid evaluation was noted in premature patients (median 375 days premature, median 147 term; p < 0.01) and publicly insured patients (median 215 days, median 123 private; p = 0.04). Delayed time to hearing aid fitting was noted for non-white patients (median 325 days, median 203 white patients; p < 0.01), publicly insured patients (median 309 days, median 212 private insurance; p < 0.02), and premature patients (median 462 days, median 224 term; p = 0.03). White patients were more likely to be referred for cochlear implant (p = 0.03).Privately insured patients and patients with a positive CMV test were more likely to be referred for cochlear implant evaluation, be seen in the cochlear implant clinic, and undergo implantation (p < 0.05). Non-white patients had a delay in cochlear implantation referral (median 928 days, median 398 days white patients; p = 0.05). Prolonged interval between evaluation in cochlear implant clinic to implantation was noted for privately insured patients (median 125 days; median 78 days publicly insured; p = 0.05). ConclusionsSociodemographic factors were significantly associated with hearing amplification referral rates and time until amplification for children with identified congenital sensorineural hearing loss. For cochlear implantation, insurance type, CMV status were significantly associated with rate and timing of cochlear implant pathway.

A Retrospective Study of Clinical Profile of Patients with Autoimmune Inner Ear Disease.

Bilateral sensorineural hearing loss can be a very distressing symptom and can affect the efficiency of a person and one's quality of life. Conditions causing bilateral hearing loss are very few and autoimmune aetiology is one of them. Autoimmune ear disease is characterised by bilateral, mostly fluctuating audiovestibular symptoms and symptoms which respond to steroids. Diagnosis of AIED presents a unique challenge to clinicians due to the lack of standardized diagnostic criteria or reliable pathognomonic tests. The purpose of the study is to evaluate the patients who fit into criteria of autoimmune inner ear disease and understand the clinical features and response to medications for the same. A retrospective chart review of patients presenting with rapidly progressive bilateral hearing loss was done. The clinical presentation including detailed history and examination findings along with the blood investigation reports and audiograms were recorded in a tabular form. The study included 6 patients - 3 male and 3 female patients. Age of the patients at onset of hearing loss varied between 24-35 years. 3 of 6 patients presented with primary autoimmune ear disease and other 3 had hearing loss secondary to systemic autoimmune disease. All patients were treated with systemic steroids, but however showed a varied response. Patients with primary AIED were administered inner ear steroid therapy as well. AIED is thus a diagnosis of exclusion done with high index of suspicion. Patients with bilateral progressive sensorineural hearing loss should be evaluated for autoimmune etiology. Oral steroids with intratympanic steroids are currently the mainstay of treatment for AIED. Guarded prognosis of hearing improvement is noted in these patients. Hence, emphasis should be placed on early hearing rehabilitation for better quality of life.

Association between age-related hearing loss and cognitive decline

Background: Presbycusis, or age-related hearing loss, is a prevalent condition characterized by the gradual deterioration of hearing with age. The resulting impairments in auditory function lead to reduced speech perception in noisy environments and often have a negative impact on cognitive, behavioral, and psychosocial functions in the elderly population.Current Concepts: The proposed etiological mechanisms linking presbycusis to cognitive decline or dementia include the common-cause hypothesis, cognitive load hypothesis, cascade hypothesis, and overdiagnosis or harbinger hypothesis. Moreover, the challenges posed by the rapidly aging population in several countries, including Korea, highlight the increasing social impact of hearing loss and dementia. Consequently, interventions for hearing rehabilitation, such as the use of hearing aids and implantable hearing devices, have paramount importance in mitigating the adverse effects of hearing loss on cognitive impairment and dementia.Discussion and Conclusion: The implementation of prevention and rehabilitation programs for hearing loss in the elderly is crucial for alleviating the socioeconomic burden associated with the welfare of the aging population. However, many economically vulnerable elderly individuals reportedly do not use hearing aids, even though they experience considerable inconvenience resulting from hearing loss, primarily due to inadequate financial support. Therefore, implementing a comprehensive national policy to support hearing aid usage among elderly individuals with moderate hearing loss holds significant potential for reducing their risk of cognitive impairment and dementia.

Non-pharmacological therapy for tinnitus

Background: Tinnitus is often difficult to treat because it is closely related to hearing loss, central nervous system disorders, and emotional problems. Although various drug treatments have been attempted for patients with tinnitus, there is no drug that has been clearly proven to be effective. On the other hand, there are some studies showing the effectiveness of various non-pharmacological treatments for tinnitus.Current Concepts: Tinnitus is classified into subjective and objective tinnitus. Representative non-pharmacological treatments for chronic subjective tinnitus include sound therapy, hearing aids or implantable hearing rehabilitation devices, tinnitus retraining therapy, neuropsychiatric therapy, and neuromodulatory treatment. When objective tinnitus does not improve with conservative treatment, surgical treatment can be selected depending on the cause. Acupuncture treatment for tinnitus is not recommended due to lack of evidence on its effectiveness and safety.Discussion and Conclusion: Despite many studies, the mechanisms of tinnitus remain unknown. Thus, it is difficult to cure the cause of tinnitus. Because subjective responses to tinnitus vary according to the patient’s psychological state, a variety of approaches would be needed for the treatment of tinnitus.

Auditory Brainstem Response Testing in Children with Speech and Language Pathology: A Non-Randomized Observational Study

Introduction: The aim of this study was to compare auditory brainstem response (ABR) findings of normal-hearing preschool children with different types of speech and language pathology. Methods: This retrospective, non-randomized, cohort study was conducted at a tertiary speech and hearing rehabilitation institution according to STROBE guidelines. The study enrolled 123 preschool children diagnosed with speech language pathology and normal hearing. The participants included children with developmental language disorder, autism spectrum disorder, isolated articulation pathology, organic brain lesions, cognitive delay, and a group of very young children with clinically significant speech development delay. All patients underwent standard ABR procedures. Results: The latencies were the longest in the group of children with organic lesion, followed by the group of children with autism spectrum disorder, then the group with developmental language disorder, and the young children group. In the group of children with articulation pathology and the cognitive delay group, the latencies were the shortest. Conclusion: This study showed a connection between several groups of children with language pathology that includes comprehension problems and prolongation of ABR latencies.

The Complexity of Hearing Aid Fitting: Children with Congenital Hearing Loss and Middle Ear Dysfunction.

The early diagnosis of hearing loss (HL) and hearing rehabilitation facilitate language and communication development. Some children exhibit mixed HL due to middle ear effusion (MEE) or acute otitis media (AOM). Mixed HL can affect HL evaluation and hearing aid (HA) fitting. The present study retrospectively evaluated the prevalence of MEE/AOM among children with congenital sensorineural HL (SNHL) who were fitted with HAs and its effect on the HA fitting. Thirty-six HA fittings carried out between 2017 and 2020 at one rehabilitation center were examined. Medical and audiological information was retrieved for children between 6 and 32 months old. The number of appointments and HA fitting times were recorded. Twenty-eight children were included in the study. Eighteen children, in addition to SNHL, had a conductive component resulting from MEE/AOM. The children with these pathologies required significantly more HA fitting sessions and hearing tests, fewer real ear to coupler difference (RECD) measurements and longer HA fitting periods. The findings indicate that a large number of children fitted with HAs have an additional conductive component that makes the fitting process longer. Since early rehabilitation is necessary for language development, otolaryngologists should be aware of the adverse effects of MEE/AOE on the HA fitting process. It is important to inform parents that when there is a conductive component, the HA fitting process may take longer and that treatment by an otolaryngologist is vital. This study stresses the importance of multidisciplinary cooperation for optimal HA fitting.

Clinical observation of genetically confirmed Kabuki syndrome type 1, with an undetermined type of inheritance, in a 7-year-old child

Kabuki syndrome is a rare hereditary disease that has a specific clinical picture: facial features, growth retardation, muscular hypotonia, skeletal abnormalities, dermatoglyphic abnormalities, mild to moderate mental retardation, and postnatal growth deficiency. Kabuki syndrome (Kabuki mask syndrome / Kabuki makeup syndrome) got its name for the specific features of the appearance of patients who have a facial expression similar to a special makeup that highlights the eyes and eyebrows of the characters of the Japanese Kabuki theater. The main reasons for the development of Kabuki syndrome in 75% are mutations in the KMT2D gene (KS type 1) and in 5% are mutations in the KDM6A gene (Kabuki syndrome type 2), the etiology of about 20% of cases remains unknown. Most mutations in type 1 Kabuki syndrome are formed de novo, and cases of autosomal dominant inheritance have also been described. Type 2 Kabuki syndrome has sex-linked (X-linked) inheritance. Because Kabuki syndrome is a low-prevalence, multisystem disease, patients require a variety of diagnostic and screening tests with multidisciplinary evaluation of outcomes at different stages of their lives. Early diagnosis of the disease is crucial for timely treatment and rehabilitation measures and improving the prognosis and quality of life of the child. The interest of the presented clinical observation of Kabuki syndrome lies in the importance of early recognition of clinical manifestations, timely surgical treatment, hearing rehabilitation and improving the prognosis of the patients life. The article describes a clinical observation, genetically confirmed type 1 Kabuki syndrome, in a child born in 2015 (type of inheritance not established). The interest of the presented clinical observation of Kabuki syndrome, manifested by characteristic facial features, skeletal anomalies, postnatal growth retardation, dermatoglyphic anomalies, intellectual retardation, lies in the need for a multidisciplinary approach to the diagnosis and treatment of the patient.

Hearing rehabilitation in children with malformed ears: The endoscopic-assisted approach for cochlear implantation

BackgroundCochlear implantation (CI) in children with malformed ears can be challenging through the standard surgical technique. Several alternative approaches have been described. The endoscopic-assisted approach can be chosen as an effective and safe surgical technique, overcoming the drawbacks of the traditional approach. MaterialWe further describe a combined technique based on a limited mastoidectomy with no posterior tympanotomy and an endoscopic transmeatal approach to the round window (RW): the electrode is driven from the mastoid to the middle ear through the attic. ResultsThe concomitant endoscopic assistance allows for improved surgical vision, reducing the risk of major complications. The main advantages of this technique are related to better visualization of the RW for safe insertion of the electrode; avoidance of damage to the facial nerve (FN), due to direct visualization, and sparing the posterior tympanotomy; avoidance of subtotal petrosectomy, if not necessary. ConclusionThe purpose of this article, supported with a video file, is to describe step by step this endoscopic-assisted procedure in a patient with middle ear malformation.

Correlation between cortical auditory evoked potential and auditory speech performance in children with cochlear implants.

This study aimed to explore the correlation between the characteristics of cortical auditory evoked potential (CAEP) of children with cochlear implants (CIs) and auditory and speech rehabilitation performance by an objective evaluation technique and subjective auditory and speech skills measurements. All participants were recruited from Beijing Children's Hospital, Beijing, China. 19 children with CIs had their responses to the CAEP and MMN recorded. The LittlEARs® Auditory Questionnaire (LEAQ), Categories of Auditory Performance (CAP), Speech Intelligibility Rating Scale (SIR), Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS), and Meaningful Use of Speech Scale (MUSS) measures were taken to assess the children's speech and hearing abilities. P1 and MMN of CAEP were negatively related to the duration of CI usage. The duration of CI usage and scores of auditory-verbal assessment questionnaires all showed significant relationships. Additionally, scores of these questionnaires were significantly inversely associated with the latency of P1 and MMN. P1 and MMN could be used as objective methods to evaluate the effectiveness of hearing and speech rehabilitation in children with CIs. In particular to those who cannot give effectively feedback of auditory and verbal effects, these methods might have a certain guiding significance.

413 Percutaneous Bone Anchored Hearing Rehabilitation in Adults - the Nottingham Experience Over a 5 Year Period

Abstract Aim Percutaneous bone anchored hearing aid (BAHA) is an effective hearing rehabilitation option. However, complications including granulation, skin overgrowth and infections may lead to revision surgeries. This study aim was to assess the complication rate and potential contributing factors leading to revision surgeries. Method Adult patients who underwent BAHA connect (Cochlear Corp) operations between 2016 to 2021 were included. Patient notes were retrospectively reviewed. Results 166 patients received a BAHA connect. Twenty-nine (17.5%) patients required revision surgery; 2 for granulation, 10 for skin overgrowth, 9 for infection, 7 for traumatic extrusion and 1 for failure of osteointegration. Excluding traumatic extrusion patients, 22 (13.3%) required revision surgery. 56% of patients were performed under local anaesthetic, with no difference in complication rate compared to general anaesthetic. 70% patients with Holger’s grade 4 changes and 40% patients with grade 3 findings required revision. 31% cases required revision when primary surgeon was trainee, compared with consultant (17%). There was no difference in post-operative skin problems between skin incisions made in-line vs separate from abutment. Cases with intra-operative complications, including re-siting of initial hole due to bleeding or loose fixture, had higher rate of revision. Of revision surgery patients, 14% were active smokers and 21% ex-smokers. 31% required multiple revision operations. Conclusions Percutaneous BAHA insertion can be performed under local and general anaesthetic. Grade of primary surgeon and intra-operative problems appear to be risk factors for requiring revision surgery. Smoking and site of incision does not appear to be risk factors for postoperative skin problems.

Preoperative EABR evaluation of auditory pathway integrity in patients with different etiology and postoperative effect estimation.

To evaluate the effect of electrical auditory brainstem response (EABR) on the integrity evaluation of auditory pathway and the prediction of postoperative effect of cochlear implantation in patients with different etiology of hearing loss. A total of 580 patients with neuropathic deafness who underwent cochlear implantation surgery from August 2011 to December 2020 were selected for EABR test. The preoperative EABR waveform was analyzed, and parameters such as V wave amplitude, threshold, latency and interval of each wave, and slope of V wave I/O curve were measured. Neural response telemetry (NRT) test was performed during MAP 1month after operation, and C and T values of the machine were recorded. The total EABR extraction rate was 98.45% among 580 patients, including 100% for the normal structure group and enlarged vestibular aqueduct group (LVAS), 92.44% for other malformed group. The average threshold of V wave in patients with normal cochlear structure was significantly better than the malformation groups (p < 0.05). The total extraction rate of NRT was 78.62%, including 99.72% in the group with normal structure, 95.65% in the LVAS group, 1.85-88.24% in the group with other malformations, and 0% in the cochlear ossification group. The correlation analysis showed a statistically significant correlation between the average preoperative EABR threshold and the C value of NRT. Preoperative EABR could evaluate the integrity of auditory conduction pathway of patients with cochlear implantation and predict the postoperative hearing rehabilitation effect.

Cochlear implant: factors that impact hearing outcomes

Background: Cochlear implant has been providing hearing rehabilitation to patients with severe hearing loss. However, not all patients achieve optimal results, and the goal of this study was to evaluate the factors that influence hearing outcomes in cochlear implantation. Methods: This was a retrospective analysis of the patients who underwent cochlear implantation in our institution between 2018 and 2021. Review of clinical files and complementary tests, and analysis of hearing outcomes and complications were performed. Results: Sixty-nine cochlear implants were placed in 64 patients, with an average age of 49.3 years. In initial evaluation post activation and 1 year after implantation, the average pure tone average (PTA) was 37 dB, with a vocal discrimination of 48.7% at 50 dB, and 28, 6 dB, with a discrimination of 73.8% at 50 dB, respectively. Patients with postlingual deafness had better hearing outcomes at 1 year post surgery, in both PTA (27.9 compared to 33 dB) and discrimination at 50 dB (76.5 compared to 56.1%). Only one case had a major complication. Conclusions: In conclusion, we found that increased implant use by the patient, postlingual deafness, and in-creased surgeon experience, were factors that improved hearing outcomes in patients undergoing cochlear implantation.

Hearing Aid in Vestibular-Schwannoma-Related Hearing Loss: A Review.

(1) Background: Several types of hearing aids are available for the rehabilitation of vestibular-schwannoma (VS)-related hearing loss. There is a lack of recently published papers regarding this theme. The aim of the present work is to organize current knowledge. (2) Methods: A review of the literature regarding the topics "vestibular schwannoma", "hearing loss", and "hearing aid" was performed. Nineteen studies were thus considered. (3) Results: Conventional hearing aids, contralateral routing of signal (CROS) aids, bone anchored hearing aids (BAHA), and others are available options for hearing rehabilitation in VS patients. The speech discrimination score (SDS) is considered the best measure to assess candidacy for rehabilitation with hearing aids. The best hearing rehabilitative conditions in VS patients when using conventional hearing aid devices are a mild-moderate hearing loss degree with good word recognition (more than 50% SDS). CROS-Aid and BAHA are reported to be beneficial. CROS-Aid expands on the area of receiving hearing. BAHA aids use direct bone-conduction stimulation. Unfortunately, there are no available studies focused specifically on VS patients that compare CROS and BAHA technologies. (4) Conclusions: Hearing aids, CROS, and BAHA are viable options for rehabilitating hearing impairment in VS, but require an accurate case-by-case audiological evaluation for rehabilitating hearing impairment in VS. Further studies are needed to prove if what is currently known about similar hearing illnesses can be confirmed, particularly in the case of VS.

Hearing Rehabilitation in Patients With Neurofibromatosis Type 2: The Quebec's Experience With Auditory Implants.

To review a single-center experience with hearing rehabilitation in patients with neurofibromatosis type 2 (NF2) and to describe the auditory outcomes of cochlear implants (CIs) and auditory brainstem implants (ABI) in this population. Retrospective case series. Tertiary referral center. Twelve adult patients with NF2 who received a CI (n = 10) and/or an ABI (n = 5) between 2000 and 2021. Insertion of a CI and/or an ABI in NF2 patients with bilateral vestibular schwannomas (VSs). Patients' demographic data, treatment history, hearing evolution, hearing rehabilitation methods, implant details, and auditory outcomes after implantation. Among those who received a CI, five patients had a stable untreated VS, one patient underwent a cochlear nerve preserving surgery, and four patients received radiotherapy treatments. Six patients became regular users of their device. The median open-set sentence recognition scores were as follows: 0.5% preoperatively, 60% at 1 year postoperatively, and 80% on the most recent audiological evaluation. All patients with an ABI were implanted concomitantly with VS surgical excision, and three of them also received radiotherapy treatments. The median open-set sentence recognition scores were as follows: 4% preoperatively, 26% at 1 year postoperatively, and 0% on the most recent evaluation. Three patients became regular ABI users. Despite major technological advances, auditory outcomes with ABIs remain deceiving. Considering the overall improvement in postoperative auditory performances provided by CIs compared with ABIs, cochlear implantation standouts as a primary mean of hearing rehabilitation in NF2 patients.

Influence of Bone Conduction Hearing Device Implantation on Health-Related Quality of Life for Patients with and without Tinnitus.

(1) Background: Tinnitus, often related to hearing loss, is an addressable public health concern affecting health-related quality of life (HRQoL). This study aimed to explore the influence of bone conduction hearing aid (BCHA) implantation on HRQoL and hearing disability in patients with hearing loss suffering from tinnitus. (2) Methods: Data were collected from an international hearing implant registry. Health Utilities Index Mark 3 (HUI-3), Spatial and Qualities of Hearing- 49 Questionnaire (SSQ) and self-reported tinnitus burden data for adult patients implanted with a BCHA (n = 42) who provided baseline as well as follow-up data 1-year post-implantation were extracted from the registry. Wilcoxon signed rank tests and paired samples t-tests were used to analyse outcomes data. (3) Results: Patients, with or without tinnitus, demonstrated clinically important mean improvements in HUI-3 multi-attribute utility scores, HUI-3 hearing attribute and SSQ scores. Hearing loss patients with tinnitus presented with a lower HRQoL than patients without tinnitus. (4) Conclusions: These findings demonstrate the importance of hearing rehabilitation in improving the quality of life and hearing disability of patients with or without tinnitus and in providing tinnitus relief in some patients with hearing loss and tinnitus.