Hearing Outcomes Research Articles

RADT-05. MATURE OUTCOMES OF A PHASE II PROSPECTIVE STUDY OF PROTON RADIOTHERAPY FOR LOCALIZED BRAIN TUMORS

Abstract BACKGROUND Proton radiotherapy (PRT) is a highly targeted form of radiation that spares more normal brain tissue compared to modern photon radiotherapy. Therefore, we expect the late effect profile in children treated with PRT with curable brain tumors should be improved. METHODS Between 2010-2022, 100 patients were enrolled, treated with PRT, and then followed for disease control, hearing, neurocognitive and endocrine outcomes. Late effects of the tumor and treatment delivered were graded using CTCAE v4.0. RESULTS Median age at PRT was 8.0 years (1.1-20.8) and median follow up was 8.7 years (0.3-12.7). Tumor types included 40 (40%) ependymomas, 19 (19%) low grade gliomas (LGG), 21 (21%) craniopharyngiomas, and 20 (20%) other histologies. 26% of patients were treated with chemotherapy in addition to PRT. The location of PRT was 38 (38%) infratentorial, 56 (56%) supratentorial, and 6 (6%) both. The median radiation dose was 52.2 GyRBE (48.6-59.4). 8-year event-free survival (EFS) and overall survival (OS) was 71.7% and 86.2% for all patients. 8-year EFS and OS was 59.9% and 82.4% for ependymomas, 78.2% and 94.7% for LGG, 80.0% and 87.7% for craniopharyngiomas and 79.1% and 84.7% for all other tumor types. 19% of patients had new grade 3+ toxicities that were at least possibly related to radiotherapy. Baseline full-scale intelligence quotient (FSIQ) was 98.0 and follow-up FSIQ was 101.8 (p=0.14). 43% of patients had new endocrine deficiencies after radiation. 8 patients suffered new high-grade hearing loss after PRT. Dosimetry on critical brain structures was collected and correlated with late effects. More details will be presented at the conference. CONCLUSION PRT remains an effective treatment for children with curable brain tumors. While late effects of the tumor and treatment are still present, the overall burden of late effects is lower than other reports.

Electrophysiological Characteristics in Pediatric Cochlear Implantation.

Cochlear implantation is a potential intervention for individuals with severe to profound hearing loss, in particular in the pediatric population. This literature review aims to comprehensively evaluate the applications of electrophysiological tests in enhancing cochlear implant (CI) outcomes for children. A literature review searched Medline and PubMed databases for articles on electrophysiological tests in CI children, using the terms "electrophysiological tests," "children," and "cochlear implant." The systematic search leads to 72 eligible texts. Electrophysiological tests can be used to test CI children without the need for their active participation. These tests can be helpful in identifying and improving the health of deaf children in various ways, such as determining the CI functional status, the semantic integration effects in CI children, the effect of central auditory structures in speech stimulus processing, the development of lexical-semantic in CI children, and tracking the maturation of the central auditory system. CI enhances central auditory nervous system (CANS) maturation and auditory/language skills. The quality of electrophysiological tests can be improved to enhance hearing outcome prediction, postoperative physiology understanding, and hearing loss mechanisms. Electrophysiological tests study CANS maturation, identify lesions, aid CI programming, determine prognosis, and treatment outcomes.

Cochlear Implantation Outcomes: A 10-Year Single-Surgeon Experience.

Cochlear implantation (CI) is an appropriate management strategy for prelingual hearing loss. Rehabilitation programs are essential in enabling children to cope with devices and acquire language skills. Objectives: This study aimed to assess the hearing outcomes of CIs performed by a single surgeon in Erbil, Kurdistan region of Iraq, and compare them with the results of other CI centers worldwide. This is a prospective study implemented in Erbil from November 1, 2013, to October 30, 2023, on a convenience sample of 161 patients with prelingual hearing loss (HL) who underwent CI by a single surgeon. The following were collected: age at implantation, use of hearing aids before implantation, parent's educational level, and duration of rehabilitation. The effect of the previous variables on Categories of Auditory Performance (CAP) score and speech intelligibility rating (SIR) on the auditory levels of children was assessed by the researcher six months and one year following surgery. Implantation age showed significant associations with the CAP score examined at six months and 12 months post implantation (p-value 0.001). Speech intelligibility rating was also significantly linked to implantation age (p-value 0.001).

Advances in Evaluation of Electrode Insertion Trauma Induced Residual Hearing loss in Cochlear Implant Recipients and its significance- A Narrative Review.

Cochlear implant surgery has revolutionized the management of profound hearing loss, with a growing focus on preserving residual hearing, particularly low-frequency hearing. This review synthesizes existing literature on cochlear implantation techniques, surgical principles, and emerging therapies aimed at reducing post-implantation hearing loss. Methodologically, a comprehensive narrative literature review was conducted, encompassing various study designs and participant demographics. The search strategy involved major biomedical databases, focusing on articles in English. Surgical techniques such as "soft surgery" emphasize minimizing mechanical stress on the cochlea, while advancements in pharmacological agents explore the use of corticosteroids, neurotrophins, and growth factors to enhance hearing preservation. Electrode design and insertion strategies are evolving to minimize trauma and optimize hearing outcomes, including consideration of insertion forces and electrode array designs. Evaluating residual hearing loss involves sophisticated techniques like electrocochleography and radiological imaging. The impact of residual hearing on auditory verbal outcomes is variable, with studies indicating positive correlations with language development, particularly in speech production. Emerging strategies in electrode design, surgical techniques, and drug delivery hold promise for improving outcomes in cochlear implantation. However, challenges such as the lack of standardized guidelines and the need for further clinical trials remain. Future directions include the exploration of nanotechnologies, gene therapies, and stem cells for constructing bionic ears, although ethical and technical hurdles persist. This review underscores the ongoing efforts to enhance cochlear implant functionality and the need for continued research to optimize outcomes for patients with hearing loss.

Modified stapes surgery for otosclerosis: An evaluation of auditory results and postoperative complications.

The senior author Shiao, had introduced a modified version of the traditional stapes surgery, called minimally traumatic stapes surgery (MTSS), and explored its effectiveness in reducing postoperative vertigo. However, MTSS exhibited no significant breakthrough in terms of audiometric results. Building upon the original MTSS through slight modifications and the use of the Medtronic Big Easy ® Piston as the prosthesis, this study evaluates a refined version of the original MTSS technique. In particular, this research sought to investigate the impact of this refined approach on hearing outcomes and postoperative complications among patients diagnosed with otosclerosis. This research comprehensively examined the medical records of individuals diagnosed with otosclerosis who underwent MTSS. The postoperative outcomes assessed encompassed hearing outcomes and the occurrence of any new complications. Overall, 95 patients diagnosed with otosclerosis (comprising 106 affected ears) underwent MTSS. Significant improvements in both the average air conduction threshold (40 ± 16.82 dB after vs 60 ± 14.56 dB before surgery; p < 0.0001) and average air-bone gap (11.99 ± 7.24 dB after vs 29.65 ± 9.47 dB before surgery; p = 0.003) were observed after the surgery. Conversely, no significant change in the average bone conduction threshold was observed after the procedure (28 ± 13.81 dB after vs 29 ± 12.31 dB before surgery; p = 0.149). Among the 106 affected ears, 102 showed postoperative air-bone gap measurements <20 dB, indicating an impressive 96.2% overall hearing improvement. Notably, only a few postoperative complications were observed, including vertigo, chorda tympani injury, facial weakness, and a slight hearing deterioration. The refined MTSS technique promoted significant postoperative hearing improvements with minimal complications. This approach showed potential for addressing the surgical challenges in Asian patients with otosclerosis, emphasizing the importance of further research on this advanced procedural method.

Hearing Outcome After Type I Tympanoplasty Using Temporalis Fascia As Graft: A Prospective Study

Hearing Outcome After Type I Tympanoplasty Using Temporalis Fascia As Graft: A Prospective Study

Sialyllactose preserves residual hearing after cochlear implantation

In individuals with hearing loss, protection of residual hearing is essential following cochlear implantation to facilitate acoustic and electric hearing. Hearing preservation requires slow insertion, atraumatic electrode and delivery of the optimal quantity of a pharmacological agent. Several studies have reported variable hearing outcomes with osmotic pump-mediated steroid delivery. New drugs, such as sialyllactose (SL) which have anti-inflammatory effect in many body parts, can prevent tissue overgrowth. In the present study, the positive effects of the pharmacological agent SL against insults were evaluated in vitro using HEI-OC1 cells. An animal model to simulate the damage due to electrode insertion during cochlear implantation was used. SL was delivered using osmotic pumps to prevent loss of the residual hearing in this animal model. Hearing deterioration, tissue fibrosis and ossification were confirmed in this animal model. Increased gene expressions of inflammatory cytokines were identified in the cochleae following dummy electrode insertion. Following the administration of SL, insertion led to a decrease in hearing threshold shifts, tissue reactions, and inflammatory markers. These results emphasize the possible role of SL in hearing preservation and improve our understanding of the mechanism underlying hearing loss after cochlear implantation.

Validation of the Arabic language version of the Audio Processor Satisfaction Questionnaire (APSQ) for hearing implant users.

Audio processors (AP) are the external components of hearing implants. User satisfaction with the performance and comfort of their AP is a critical factor in ensuring daily use, which leads to improved hearing outcomes. The aim of this study was to construct and validate an Arabic language translation of the APSQ for use among Arabic-speaking clinicians and patients. The original APSQ was translated into the Arabic language using cross-cultural adaptation measures. The final questionnaire was administered to CI users in electronic form. 117 CI users (64 female) participated. A total of 179 ears were assessed. High levels of satisfaction with audio processors were observed among CI users. Item and scale analyses indicate that this version of the APSQ measure a homogeneous and valid construct. The Arabic version of the APSQ captures user satisfaction with hearing implant audio processors.

PENETAPAN AHLI WARIS DALAM TINJAUAN YURIDIS NORMATIF

This study focused on disparities between decisions made by first-instance and appeal courts in handling the same case. Failure to register marriages and divorces leads to legal division of property and inheritance shares. First-instance judges prioritize legal certainty of marriage/divorce certificates and their legal implications. However, appeal courts lean towards sociological facts, disregarding the evidential value of authentic marriage certificates. Furthermore, their decisions conflict with the Marriage Registration Act, which mandates valid divorces to be confirmed by court-issued certificates. This study was normative research using case, legal, and conceptual approaches. The legal material was decision number 651/Pdt.G/2020/PA.Tgr and number 54/Pdt.G/2021/PTA.Smd, as well as legal provisions in the law of evidence. Based on these legal materials, legal analysis was conducted using deductive and inductive methods to strengthen the analysis of legal reasoning results of different judges hearing the same case. This study found that: firstly, the High Court of Religion in Samarinda's decision to disregard the divorce certificate did not uphold legal certainty, as certificates hold significant weight in inheritance cases, serving as conclusive evidence. Hence, there was ambiguity regarding the divorce status of Kasmawati binti Talle and Tajang bin Abu Bagenda in case number 54/Pdt.G/2021/PTA.Smd due to the absence of fully evidential documentation. Secondly, the legal ramifications of an unregistered divorce render it invalid and necessitate annulment, binding the wife to her former husband, a principle supported by Imam As-Syafi'i in Islamic jurisprudence, aligned with other scholarly opinions.

An Observational Study to Assess the Outcome of Canal Wall Window Tympanomastoidectomy in Chronic Suppurative Otitis Media.

This study was undertaken to assess the outcome of canal wall window tympanomastoidectomy in chronic suppurative otitis media in a tertiary care centre of central India. A total of 50 patients were taken for this study in which the youngest patient was 13-year-old male and oldest 55-year female. Maximum patients were in between age group 10 to 20 years, 20 (40%) patients and minimum 03 (6%) between age group 51 to 60 year. The study mainly emphasized on: To study the clinical profile of the cases of chronic suppurative otitis media, to study the hearing outcome after 3 months of Canal wall window tympanomastoidectomy technique, to study the post-operative condition of ear after 3 months whether dry or wet and to study the post-operative follow up of cases to assess the graft uptake. Average PTA & ABG improvement of different tympanoplasty was also calculated in our study in which Type I tympanoplasty had 12.20dB gain, Type II of 10.50 and Type III of 10.31dB.An overall average improvement in all patients with tympanoplasty was 11.00dB.At the end of the study 100% dry ears were achieved. The technique of canal wall window tympanomastoidectomy involves formation of a window in the outer attic wall to look at the disease extent which was then closed by a snuggly fitting cartilage.It is a novel technique & without compromising the structural integrity in case of a limited disease which is only extending till attic and aditus good anatomical and hearing results could be gained.

Cohort profile: longitudinal and population comparison of children who are HIV-exposed uninfected and children who are HIV unexposed in Kenya (HOPE study)

PurposeGlobally, the number of children/adolescents exposed to HIV but uninfected (HIV-exposed uninfected, HEU) is growing. The HEU outcomes: population-evaluation and screening strategies study was designed to provide population-level evidence of...

Predicting Postoperative Speech Perception and Audiometric Thresholds Using Intracochlear Electrocochleography in Cochlear Implant Recipients.

Electrocochleography (ECochG) appears to offer the most accurate prediction of post-cochlear implant hearing outcomes. This may be related to its capacity to interrogate the health of underlying cochlear tissue. The four major components of ECochG (cochlear microphonic [CM], summating potential [SP], compound action potential [CAP], and auditory nerve neurophonic [ANN]) are generated by different cochlear tissue components. Analyzing characteristics of these components can reveal the state of hair and neural cell in a cochlea. There is limited evidence on the characteristics of intracochlear (IC) ECochG recordings measured across the array postinsertion but compared with extracochlear recordings has better signal to noise ratio and spatial specificity. The present study aimed to examine the relationship between ECochG components recorded from an IC approach and postoperative speech perception or audiometric thresholds. In 113 human subjects, responses to 500 Hz tone bursts were recorded at 11 IC electrodes across a 22-electrode cochlear implant array immediately following insertion. Responses to condensation and rarefaction stimuli were then subtracted from one another to emphasize the CM and added to one another to emphasize the SP, ANN, and CAP. Maximum amplitudes and extracochlear electrode locations were recorded for each of these ECochG components. These were added stepwise to a multi-factor generalized additive model to develop a best-fit model predictive model for pure-tone audiometric thresholds (PTA) and speech perception scores (speech recognition threshold [SRT] and consonant-vowel-consonant phoneme [CVC-P]) at 3- and 12-month postoperative timepoints. This best-fit model was tested against a generalized additive model using clinical factors alone (preoperative score, age, and gender) as a null model proxy. ECochG-factor models were superior to clinical factor models in predicting postoperative PTA, CVC-P, and SRT outcomes at both timepoints. Clinical factor models explained a moderate amount of PTA variance ( r2 = 45.9% at 3-month, 31.8% at 12-month, both p < 0.001) and smaller variances of CVC-P and SRT ( r2 range = 6 to 13.7%, p = 0.008 to 0.113). Age was not a significant predictive factor. ECochG models explained more variance at the 12-month timepoint ( r2 for PTA = 52.9%, CVC-P = 39.6%, SRT = 36.4%) compared with the 3-month one timepoint ( r2 for PTA = 49.4%, CVC-P = 26.5%, SRT = 22.3%). The ECochG model was based on three factors: maximum SP deflection amplitude, and electrode position of CM and SP peaks. Adding neural (ANN and/or CAP) factors to the model did not improve variance explanation. Large negative SP deflection was associated with poorer outcomes and a large positive SP deflection with better postoperative outcomes. Mid-array peaks of SP and CM were both associated with poorer outcomes. Postinsertion IC-ECochG recordings across the array can explain a moderate amount of postoperative speech perception and audiometric thresholds. Maximum SP deflection and its location across the array appear to have a significant predictive value which may reflect the underlying state of cochlear health.

A tailored approach in cholesteatoma surgery.

In recent years, new techniques have been added to cholesteatoma surgery, and established microsurgical approaches are being reconsidered. This study aims to present the importance of individualized decision-making for the selection of an intact canal wall (CWU) or canal wall down (CWD) surgical procedure for each patient. Using the "ChOLE" classification we categorized 264 operations retrospectively. 162 CWU and 102 CWD surgeries were performed. We focus to determine why a CWD procedure was chosen quite frequently despite some low-stage cases. Furthermore, we evaluated recidivism and hearing outcomes. Smaller cholesteatomas (Ch-stage 1a, 1b & 2a) were found in 182 patients (70%), ossicular chain status feasible for straightforward reconstruction (O-stage 0, 1 & 2) was present in 186 patients (70%), minor complications due to the cholesteatoma (L-stage 1) were infrequent with 28 cases (11%) and a well-pneumatized mastoid was found in 144 cases (55%). Recidivism rates were low (7%) without any difference in both groups and a mean follow-up time of 4 years and 8 months. In primary surgeries there was a significant difference (p < 0.05) in postoperative mean air-bone gap (ABG) between CWU (17dB) and CWD (27dB). The main goals of cholesteatoma surgery remain the avoidance of recidivism and optimal hearing rehabilitation. We recommend a tailored approach in the treatment of cholesteatomas and not a dogmatic one. Surgeons should not hesitate to perform a CWD procedure if required. Performed correctly it results in a dry ear and CWD surgery should remain in the skill set of the otologic surgeon.

Auditory brainstem implants: an analysis of adverse events in the MAUDE database

ObjectiveAuditory Brainstem Implants (ABI) are used to restore hearing in patients lacking appropriate cochlear anatomy and/or cochlear nerve. The objective of this study was to examine the Manufacture and User Facility Device Experience (MAUDE) database to analyze adverse events. Study designThis is a study of a multi-institutional database maintained by the US FDA. SettingA database analysis was performed via collaboration of multiple clinicians at tertiary referral centers. MethodsThe MAUDE database was queried for Medical Device Reports (MDRs) relating to ABIs. MDRs were identified using the advanced search term “Implant, Auditory Brainstem” and reviewing all reports with the basic search term “Brainstem Implant”. All collected reports were individually reviewed. ResultsA total of 265 individual patient reports were reviewed, of which 55 reports met inclusion criteria. Reports regarding audiologic outcome included failure to provide hearing benefit (n = 27), implant failure/device malfunction (n = 10), and device non-use (n = 6). Postoperative complications included local skin infection (n = 3), CSF leak (n = 3), elevated ICP (n = 1), surgical site dehiscence (n = 1), swelling (n = 1), seroma formation requiring drainage (n = 1), and meningitis (n = 2). Two patients had dislodged magnets during 1.5 Tesla MRI acquisition. There were 35 instances of full explantation of the device and 1 partial removal; 13 patients had a new device implanted following explantation. ConclusionsPoor hearing results, device failure, and non-use were commonly reported causes for explanation in this analysis. This information can aid physicians in counseling patients and family members and managing device expectations.

Evaluation and outcome of hearing in meningitis patients

Background: Meningitis remains the most common cause of acquired severe to profound SNHL in childhood. Likewise, deafness is the most common long-term neurological sequelae of the disease. Important consideration following meningitis is the development of labyrinthitis ossificans in which there is new bone formation within the lumen of the otic capsule. Ossification of the labyrinth can develop within a few days of meningitis which can make cochlear implantation difficult. Every newborn should be promptly screened for any hearing deficit along with the patients having meningitis. The most easily available investigation are OAE and BERA. Methods: Our ambispective study of 25 patients was undertaken in a duration of 2 years in ENT department of civil hospital, Ahmedabad. All the patients underwent a detailed clinical examination and all routine audiological, and radiological investigations. Results: The prevalence of hearing loss in post meningitis pediatric age group is 40%. Females are affected more than males. Median age group of presentation with hearing deficit is 4 years. Patients with complete immunization history have lower incidence of hearing loss associated with meningitis than incomplete immunization. Ossification of the cochlea can develop as early as 6 days from the development of first symptom. Conclusions: Ossification of the cochlea can develop as early as 6 days from the development of first symptom. These are the candidates who should be prioritized for cochlear implant surgery. Patients with mild hearing loss recovered completely whereas those with profound hearing loss underwent cochlear implant surgery and were benefited with it.

Evaluating the benefits and outcomes of second ear surgeries in bilateral stapedectomies: a comprehensive analysis

Background: Objectives of the study were to evaluate the anatomical abnormalities and benefits of second ear surgery in patients with bilateral otosclerosis undergoing stapedectomy. Methods: A descriptive retrospective study was done in 43 patients undergoing bilateral stapedotomy in a tertiary care centre. Anatomical abnormalties presenting intraoperative were recorded in both operated ears. Hearing outcomes after the first and second ear surgeries were recorded. Chi square and paired t test was used to find the association between categorical and continuous variables between both groups. We correlated the post-operative air conduction (AC) averages, air bone gap (ABG) gain, AC gain between first and second operated ears. Results: Obliterative FP was the most common abnormality seen 7%, followed by facial nerve anamolies 5%. Bilateral variations were seen in 66% and 60% respectively. 66% required bilateral drill outs. After 1st ear and 2nd ear surgery 81% and 76% gained normal hearing. Average postop AC threshold was significantly higher after first ear surgery (p&lt;0.001). ABG and AC gain had no significant difference in both ears. Binaural symmetrical hearing with inter aural difference AB&lt;10 dB was achieved in 90% of patients. Conclusions: It is worthwhile to recommend the patients to undergo contralateral ear surgery if the first ear had achieved normal or socially acceptable hearing, as the chances of achieving a comparable hearing outcome as that of 1st ear is high. Following 2nd ear surgery an effective binaural symmetric hearing can be achieved.

Predicting Individual Hearing-Aid Preference From Self-Reported Listening Experiences in Daily Life.

The study compared the utility of two approaches for collecting real-world listening experiences to predict hearing-aid preference: a retrospective questionnaire (Speech, Spatial, and Qualities of Hearing Scale [SSQ]) and in-situ Ecological Momentary Assessment (EMA). The rationale being that each approach likely provides different and yet complementary information. In addition, it was examined how self-reported listening activity and hearing-aid data-logging can augment EMAs for individualized and contextualized hearing outcome assessments. Experienced hearing-aid users (N = 40) with mild-to-moderate symmetrical sensorineural hearing loss completed the SSQ questionnaire and gave repeated EMAs for two wear periods of 2-weeks each with two different hearing-aid models that differed mainly in their noise reduction technology. The EMAs were linked to a self-reported listening activity and sound environment parameters (from hearing-aid data-logging) recorded at the time of EMA completion. Wear order was randomized by hearing-aid model. Linear mixed-effects models and Random Forest models with five-fold cross-validation were used to assess the statistical associations between listening experiences and end-of-trial preferences, and to evaluate how accurately EMAs predicted preference within individuals. Only 6 of the 49 SSQ items significantly discriminated between responses made for the end-of-trial preferred versus nonpreferred hearing-aid model. For the EMAs, questions related to perception of the sound from the hearing aids were all significantly associated with preference, and these associations were strongest in EMAs completed in sound environments with predominantly low SNR and listening activities related to television, people talking, nonspecific listening, and music listening. Mean differences in listening experiences from SSQ and EMA correctly predicted preference in 71.8% and 72.5% of included participants, respectively. However, a prognostic classification of single EMAs into end-of-trial preference with a Random Forest model achieved a 93.8% accuracy when contextual information was included. SSQ and EMA predicted preference equally well when considering mean differences, however, EMAs had a high prognostic classifications accuracy due to the repeated-measures nature, which make them ideal for individualized hearing outcome investigations, especially when responses are combined with contextual information about the sound environment.

P.096 Hearing preservation and quality of life outcomes in partial labyrinthectomy petrous apicectomy for microsurgical resection of large posterior fossa skullbase tumors

Background: The Partial Labyrinthectomy Petrous Apicectomy (PLPA) aims to give transpetrosal access whilst preserving hearing for challenging tumors such as petroclival meningioma. There are few studies assessing resection and morbidity and no large studies that document hearing preservation and quality of life (QOL). We present the first large series to do so. Methods: A retrospective review was performed of all PLPA cases between 2005 and 2023 at a tertiary center. Demographics, tumor characteristics, neuromonitoring, hearing and surgical outcomes were collected. QOL was measured with the 36-item short form survey (SF-36). Results: Of 73 PLPAs, data for 56 patients undergoing 57 surgeries was obtained. Petroclival meningioma (57.8%) and epidermoid tumors (21.0%) were common indications . The mean patient age and tumor size were 51.6 years and 44mm. Gross total resection was achieved in 40.3%, near total in 15.8% and subtotal in 43.8% of cases with no perioperative mortality and was not influenced by attempted hearing preservation (p=0.183). Of 39 hearing preservation cases, 27 (69.2%) were preserved, 10 (25.6%) were lost and 2 had unclear outcomes. Conclusions: Improved microsurgery and neuromonitoring during PLPA leads to decreased mortality and morbidity compared to historical cohorts while achieving a high rate of resection, hearing preservation and maintained QOL.

Debutul subliminal al surdităţii neurosenzoriale brusc instalate în hipotiroidism – revizuire a literaturii

Sudden sensorineural hearing loss (SSNHL) is a frequent oc­cur­rence in audiology and otolaryngology. It refers to a sen­so­rineural hearing loss of 30 dB or more across three con­se­cu­tive audiometric frequencies, developing within 72 hours. While the causes of SSNHL vary widely, most cases lack a clear origin. Studies have suggested a po­ten­tial re­la­tion­ship between thyroid dysfunction and the onset of SSNHL, yet establishing a definitive causal link re­mains un­cer­tain. Our review aims to meticulously explore the impact of thyroid function on SSNHL. The treatment for SSNHL with iden­ti­fiable causes typically targets the un­der­lying agent, often resulting in poor hearing outcomes due to inner ear hair cell damage. In cases of idiopathic SSNHL, steroid the­r­apy is the current standard of care. The prognosis for hea­r­ing recovery in idiopathic SSNHL hinges on factors such as the severity of hearing loss, age, presence of vertigo, and le­­­vels of thyroid hormones.

Evaluation of Cochlear Implantation in Children With Cochlear Nerve Absence or Deficiency.

To identify associations between cochleovestibular anatomy findings and hearing outcomes found in children with imaging evidence of an absent or hypoplastic cochlear nerve treated with cochlear implantation (CI). retrospective review. Cochlear implant program at tertiary care center. A retrospective review was performed to identify children with imaging evidence of cochlear nerve absence or deficiency who underwent CI evaluation. High-resolution 3-dimensional T2-weighted magnetic resonance imaging in the oblique sagittal and axial planes were reviewed by a neuroradiologist to identify cochleovestibular anatomy. Hearing was assessed pre and postoperatively with Speech Perception Category scores. Seven CI recipients were identified (n = 10 ears) who had bilateral severe to profound sensorineural hearing loss with lack of auditory development with binaural hearing aid trial and imaging evidence of cochlear nerve aplasia/hypoplasia. All ears had 2 nerves in the cerebellopontine angle (100%, n = 10), half of the ears had evidence of 2 or less nerves in the internal auditory canal (IAC). All children showed large improvement in speech perception after CI. Our experience with CIs for children with absent or hypoplastic cochlear nerves demonstrates that CI can be a viable option in select patients who satisfy preoperative audiological criteria. Radiological identification of a hypoplastic or aplastic cochlear nerve does not preclude auditory innervation of the cochlea. CI recipients in this subgroup must be counseled on difficulty in predicting postimplantation language and speech outcomes, and cautioned about facial nerve stimulation.