Middle Ear Fibrosis Contributes to Hearing Loss in Patients With Myhre Syndrome.

Myhre syndrome (MS) is a rare genetic condition that presents with multiple genetic anomalies including cleft lip and palate and Eustachian tube dysfunction. These patients are at a high risk for airway scarring from intubation and mucosal inflammation. Hearing loss (conductive or mixed, of varying severity) is a common comorbidity in these patients, the exact etiology of which is still unclear. We present the cases of 2 unrelated children with MS who suffered progressive mixed hearing loss from fibrosis and obliteration of the middle ear spaces. Both patients had multiple sets of ear tubes that demonstrated early extrusion. The older patient underwent bone conduction implantation at age 11 which resulted in dramatic improvement of speech recognition and interactive skills. The other younger patient demonstrates a similar trajectory but has not yet undergone implantation. Otolaryngologists should take a cautious approach to surgery of the eardrum and middle ear to avoid unnecessary induction of fibrosis in this susceptible patient population. These cases highlight a newly described etiology for hearing loss and suggest a benefit to bone conduction implantation.

Incidentally Discovered 99mTc-MDP Uptake on Bone Scan in Otosclerosis.

Otosclerosis is a poorly understood clinical entity causing progressive conductive hearing loss. Here we present the first known evidence of otosclerosis demonstrating 99mTc-MDP uptake on bone scan. This presents an opportunity to explore the role of nuclear medicine imaging in early detection, staging, and even informing treatment and prognosis of this condition. Laryngoscope, 2024.

Piezoelectric Bone Conduction Hearing Implant: A Case Series of Audiological, Surgical and Patient-Reported Outcomes.

Objective: To examine the surgical, audiological and patient-reported outcomes of the Osia 2 implant. Methods: Data from 14 consecutive subjects undergoing implantation between April 2022 and November 2023 were reviewed. Ten subjects had conductive hearing loss, three had mixed hearing loss and one had single-sided deafness (SSD). Warble tone thresholds, Pure Tone Average (PTA4) and Speech Discrimination Score (SDS) in quiet and in noise were determined unaided and aided. The subjective outcome was determined from two standardized questionnaires: (1) International Outcome Inventory for Hearing Aids (IOI-HA) and (2) Speech, Spatial and Qualities of Hearing Scale 12 (SSQ12b). Results: Unexpected postoperative pain was found in four cases. The warble tone thresholds exhibited a consistent reduction across all frequencies, contributing to a mean decrease of 27 dB in the aided PTA4. SDS demonstrated notable improvements, with a 57.3% increase at 50 dB and a 55.6% increase at 65 dB. In noise, SDS exhibited a 43.9% improvement. The mean IOI-HA Score was 3.8, and the mean overall score for SSQ12b was 6.6, with consistent findings across the subgroups. Conclusions: The Osia device emerges as a promising recommendation for individuals with conductive or mixed hearing loss, possibly also for those with SSD. Its safety and efficacy profile aligns with the broader category of active transcutaneous devices, demonstrating a reduced risk of wound infection compared to percutaneous alternatives. Both audiological assessments and subjective evaluations revealed positive outcomes.

Assessment of Outer and Middle Ear Pathologies in Lilongwe, Malawi.

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.

Diagnosis and Management of Unexplained Conductive Hearing Loss With Intact Tympanic Membrane: A Systematic Review.

Objective: To analyze the etiology, diagnosis, and treatment of unexplained conductive hearing loss (UCHL) with intact tympanic membrane. Methods: A systematic review was conducted based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A total of 642 articles were retrieved from databases such as PubMed, Embase, Web of Science, and Cochrane. Fifty-four research articles and 21 case reports were screened out according to the inclusion and exclusion criteria for analysis of the etiology of UCHL. Seven research articles with UCHL who underwent exploratory tympanotomy were selected for data extraction and analysis of clinical characteristics. Results: UCHL is a common manifestation of various diseases, including congenital ossicular anomalies (COA), otosclerosis (OTS), congenital middle ear cholesteatoma (CMEC), oval window atresia, superior semicircular-canal dehiscence, congenital stapedial footplate fixation, middle ear osteoma or adenoma, congenital ossification of stapedial tendon, and so on. A total of 522 patients were included in the 7 articles; among whom OTS showed a tendency to increase with age. The main symptoms were hearing loss, followed by tinnitus, dizziness, ear fullness, ear pain, facial paralysis. A total of 87.5% to 93.0% patients with COA manifested as nonprogressive deafness that occurred since childhood, with tinnitus incidence of 15.6% to 30.2%, and 86.4% to 96.4% patients with OTS presented with progressive hearing loss, with tinnitus incidence of 60.1% to 90.9%. The diagnosis positive rate of high-resolution computed tomography (HRCT) was 33.8% to 87.1%, and CMEC was higher than that of COA (83.3%-100% vs 28.6%-64%). All the articles reported good hearing recovery. The most common surgical complications included taste abnormalities, tinnitus, and dizziness. Conclusion: UCHL presents with similar clinical manifestations and poses challenges in preoperative diagnosis. Exploratory tympanotomy is the primary method for diagnosis and treatment, with good prognosis after removing the lesion and reconstructing hearing during the operation. Children can also safely undergo the surgery.

Long-term follow-up of otitis media with effusion in neonatal hearing screening.

OBJECTIVES: Increased neonatal referral rate of conductive hearing loss (CHL) related to otitis media with effusion (OME) following universal neonatal hearing screening (UNHS) may cause an unnecessary clinical, emotional, and financial burden. This study analyzes the long-term, audiological, and medical characteristics of CHL associated with OME in neonates in order to establish a standardized protocol following technology-driven improvements in detection and referral rates in UNHS. METHODS: A retrospective study of all neonates with OME-related CHL referred to the University Hospital of Leuven (Belgium) after failing UNHS with the MAICO devices between January 1, 2013 and December 31, 2021 was performed. Follow-up consultations, auditory tests, referral side, birth month, hearing loss degree, underlying pathologies and risk factors, time to normalization, and need for ventilation tubes were assessed. RESULTS: The incidence of CHL related to OME was stable between 2013 and 2021. Of all referred infants with OME, 52.3 % demonstrated spontaneous recovery. The average time to hearing normalization was significantly longer in children with underlying congenital pathologies compared to those without. Moreover, 74.4 % of these children received ventilation tubes compared to 32.0 % of children without underlying pathologies. No correlation was found between the incidence of OME-related CHL with either a hearing loss degree, admission to neonatal intensive care, or history of a nasogastric feeding tube. CONCLUSIONS: In children who failed UNHS due to OME, hearing recovers spontaneously without surgical intervention in 2/3 of the infants without underlying conditions within one year. In children with underlying congenital disorders, the time to hearing recovery is longer and the risk for surgical intervention is higher, underlining the need for implementing a UNHS standardized protocol.

An Ear Mystery: Conductive Hearing Loss with Normal Acoustic Reflexes and VEMPs.

The cause of sudden conductive loss in the absences of other vestibulo-audiologic abnormalities can be isolated to specific subsites of the ossicular chain using audiometric testing. In the absence of such abnormalities, a rare etiology may be the cause after an inciting trauma. Laryngoscope, 2024.

Late Onset Hearing Loss in Very Low Birth Weight Infants.

Objective: To determine the incidence of late onset hearing loss and associated risk factors in very low birth weight (VLBW) infants. Study Design: Retrospective study (2003-2015) of post-discharge hearing outcomes and risk factors in the VLBW infant population, before and after the institution of a standardized follow-up program. Results: Late onset hearing loss increased from 2.9 per 100 VLBW infants to 7.8 per 100 after instituting a monitoring protocol. The follow-up compliance rate nearly doubled. Both infants with late-onset sensorineural hearing loss and those with a conductive component were identified. The rate of conductive loss detection increased seven-fold. Conclusion: The institution of a standardized hearing follow-up program significantly increased the detection of late onset hearing loss in VLBW infants. A significant proportion of those with late onset hearing loss had a conductive component. Without identification and treatment, even conductive losses may negatively impact speech and language development.

Modification of skin incision for Baha Attract System Implantations.

<b><br>Introduction:</b> In an era of wide accessibility to various systems for hearing impairment prosthetics, there exists a broad spectrum of surgical techniques facilitating system implantation.</br> <b><br>Aim:</b> The aim of the study is to present the technique of vertical incision in the implantation of the Baha Attract magnetic system as an alternative incision, maintaining a compromise between optimizing the surgical procedure and preserving the functionality and quality of life of the patient.</br> <b><br>Materials and methods:</b> The vertical incision technique is presented based on 5 patients treated between December 2022 and March 2023 diagnosed with conductive or mixed hearing loss.</br> <b><br>Results:</b> There were no adverse consequences or complications resulting from the performed surgical incision. All patients experienced uncomplicated healing. Patients are using sound processor magnets ranging from 4-5 in strength and Baha 6 Max sound processors weighing 11.5 g.</br> <b><br>Conclusions:</b> The vertical incision technique serves as an alternative to the C-shaped perimeter incision, allowing for the optimization and standardization of the surgical procedure, resulting in a smooth scar formation and maintaining good audiological and aesthetic outcomes.</br>.

Active Bone Conduction Implant and Adhesive Bone Conduction Device: A Comparison of Audiological Performance and Subjective Satisfaction.

Introduction Atresia of the external auditory canal affects 1 in every 10 thousand to 20 thousand live births, with a much higher prevalence in Latin America, at 5 to 21 out of every 10 thousand newborns. The treatment involves esthetic and functional aspects. Regarding the functional treatment, there are surgical and nonsurgical alternatives like spectacle frames and rigid and softband systems. Active transcutaneous bone conduction implants (BCIs) achieve good sound transmission and directly stimulate the bone. Objective To assess the audiological performance and subjective satisfaction of children implanted with an active transcutaneous BCI for more than one year and to compare the outcomes with a nonsurgical adhesive bone conduction device (aBCD) in the same users. Methods The present is a prospective, multicentric study. The audiological performance was evaluated at 1, 6, and 12 months postactivation, and after a 1-month trial with the nonsurgical device. Results Ten patients completed all tests. The 4-frequency pure-tone average (4PTA) in the unaided condition was of 65 dB HL, which improved significantly to 20 dB HL after using the BCI for 12 months. The speech recognition in quiet in the unaided condition was of 33% on average, which improved significantly, to 99% with the BCI, and to 91% with the aBCD. Conclusion The aBCD demonstrated sufficient hearing improvement and subjective satisfaction; thus, it is a good solution for hearing rehabilitation if surgery is not desired or not possible. If surgery is an option, the BCI is the superior device in terms of hearing outcomes, particularly background noise and subjective satisfaction.

Binaural hearing in monaural conductive or mixed hearing loss fitted with unilateral Bonebridge.

Objective: To determine the benefits of binaural hearing rehabilitation in patients with monaural conductive or mixed hearing loss treated with a unilateral bone conduction implant (BCI). Methods: This monocentric study includes 7 patients with monaural conductive or mixed hearing loss who underwent surgical implantation of a unilateral BCI (Bonebridge, Med-El). An ITA Matrix test was performed by each patient included in the study - without and with the BCI and in three different settings - to determine the summation effect, squelch effect and head shadow effect. Subjective hearing benefits were assessed using the Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire. Results: The difference in signal to noise ratio of patients without and with BCI was 0.79 dB in the summation setting (p < 0.05), 4.62 dB in the head shadow setting (p < 0.05) and 1.53 dB (p = 0.063) in the squelch setting. The APHAB questionnaire revealed a subjective discomfort in the presence of unexpected sounds in patients using a unilateral BCI (aversiveness score) compared to the same environmental situations without BCI, with a mean discomfort score of 69.00% (SD ± 21.24%) with monaural BCI versus 25.67% (SD ± 16.70%) without BCI (difference: -43.33%, p < 0.05). In terms of global score, patients wearing a unilateral Bonebridge implant did not show any significant differences compared to those without hearing aid (difference: -4.00%, p = 0.310). Conclusions: Our study shows that the use of a unilateral BCI in patients affected by monaural conductive or mixed hearing loss can improve speech perception under noise conditions due to the summation effect and to the decrease of the head shadow effect. However, since monaural BCIs might lead to discomfort under noise conditions in some subjects, a pre-operative assessment of the possible individual benefit of a monaural BCI should be carried out in patients affected by unilateral conductive or mixed hearing loss in order to investigate the possible additional effect of the fitting of hearing aids.

Posterior Semicircular Canal Dehiscence with Vestibulo-Ocular Reflex Reduction for the Affected Canal at the Video-Head Impulse Test: Considerations to Pathomechanisms.

Posterior semicircular canal dehiscence (PSCD) has been demonstrated to result in a third mobile window mechanism (TMWM) in the inner ear similar to superior semicircular canal dehiscence (SSCD). Typical clinical and instrumental features of TMWM, including low-frequency conductive hearing loss (CHL), autophony, pulsatile tinnitus, sound/pressure-induced vertigo and enhanced vestibular-evoked myogenic potentials, have been widely described in cases with PSCD. Nevertheless, video-head impulse test (vHIT) results have been poorly investigated. Here, we present six patients with PSCD presenting with a clinical scenario consistent with a TMWM and an impaired vestibulo-ocular reflex (VOR) for the affected canal on vHIT. In two cases, an additional dehiscence between the facial nerve and the horizontal semicircular canal (HSC) was detected, leading to a concurrent VOR impairment for the HSC. While in SSCD, a VOR gain reduction could be ascribed to a spontaneous "auto-plugging" process due to a dural prolapse into the canal, the same pathomechanism is difficult to conceive in PSCD due to a different anatomical position, making a dural herniation less likely. Alternative putative pathomechanisms are discussed, including an endolymphatic flow dissipation during head impulses as already hypothesized in SSCD. The association of symptoms/signs consistent with TMWM and a reduced VOR gain for the posterior canal might address the diagnosis toward PSCD.

Clinical Outcomes of an Active, Transcutaneous, Bone Conduction Hearing Device: A Retrospective Study.

OBJECTIVE: The purpose of this study was to review patient demographics, indications, intraoperative findings, complications/adverse events, and audiological outcomes related to the implantation of the Osia 2 device. STUDY DESIGN: Retrospective case series. SETTING: Single tertiary institutional experience. METHODS: Patients who had undergone Osia 2 implantation by the senior author were identified from 2019 to present. Information was extracted from patient charts concerning patient demographics, indications for implantation, surgical findings, audiological outcomes, and adverse events. RESULTS: Sixty patients and 67 implants were included. The median age was 51 years (R: 11-92). Fifty-five percent of patients had mixed hearing loss (HL), 30% had single-sided deafness, and 15% had conductive HL. The mean operative time was 53.9 minutes. Only 4.5% of patients required bone polishing, and 3.0% required tissue thinning. The mean pure-tone averages 4 gain from unaided conditions was 41.2 dB. Mean gain at 6 and 8 kHz from unaided conditions was 35.42 and 40.67 dB, respectively. Mean improvement in speech recognition threshold and word recognition score was significant in noise and quiet conditions. The all-cause adverse event/complication rate in our series was 10.4%. The most common complications were infections (4.5%) and poorly controlled postoperative pain (3.0%). Hematomas occurred in 1.5% of patients. Reoperation was required in 4 patients; explant in 1. CONCLUSION: Use of the Osia 2 device in our series has resulted in good hearing outcomes, particularly in terms of high frequency gain. Complication rates were low. To our knowledge, this is the largest study to date reporting on Osia 2 outcomes.

Contemporary Mechanics of Conductive Hearing Loss.

The middle ear plays a critical role for the conversion of acoustic energy to mechanical vibrations that subsequently enter the cochlea. It is middle ear impedance matching through ossicular coupling that has enabled land-dwelling vertebrates to hear soft airborne sounds. Conductive hearing loss may result from damage to the delicate middle ear structures following infection, trauma or rapid pressure changes. An understanding of the mechanics of the middle ear significantly improves the oto-surgeon's ability to effectively diagnose conductive hearing loss, localize the responsible lesion and then effectively correct the conduction abnormality. This article reviews some of the basic knowledge of middle ear mechanics for sound transmission, highlights recent advances in developing new techniques to assist in diagnosis of middle ear disease, and finally sheds light on future research aimed at improving the diagnosis and management of middle ear pathology.

A homozygous SP7/OSX mutation causes osteogenesis and dentinogenesis imperfecta with craniofacial anomalies.

Osteogenesis imperfecta (OI) is a heterogeneous spectrum of hereditary genetic disorders that cause bone fragility, through various quantitative and qualitative defects of type 1 collagen, a triple helix composed of two α1 and one α2 chains encoded by COL1A1 and COL1A2, respectively. The main extra-skeletal manifestations of OI include blue sclerae, opalescent teeth, and hearing impairment. Moreover, multiple genes involved in osteoblast maturation and type 1 collagen biosynthesis are now known to cause recessive forms of OI. In this study a multiplex consanguineous family of two affected males with OI was recruited for genetic screening. To determine the causative, pathogenic variant(s), genomic DNA from two affected family members were analyzed using whole exome sequencing, autozygosity mapping, and then validated with Sanger sequencing. The analysis led to the mapping of a homozygous variant previously reported in SP7/OSX, a gene encoding for Osterix, a transcription factor that activates a repertoire of genes involved in osteoblast and osteocyte differentiation and function. The identified variant (c.946C > T; p.Arg316Cys) in exon 2 of SP7/OSX results in a pathogenic amino acid change in two affected male siblings and develops OI, dentinogenesis imperfecta, and craniofacial anomaly. On the basis of the findings of the present study, SP7/OSX:c. 946C > T is a rare homozygous variant causing OI with extra-skeletal features in inbred Arab populations.

Congenital Absence of Stapes Suprastructure and Footplate: A Case Report.

INTRODUCTION: Congenital ossicular chain anomalies are rare conductive hearing loss conditions that remain difficult to diagnose even with high-resolution computed tomography (CT). The preoperative diagnosis is helpful for surgical planning and counseling patients regarding treatment outcomes. CASE PRESENTATION: We report a case involving a 14-year-old boy presenting with left conductive hearing loss without history of trauma for 5 years, physical examination showed normal otoscopic examination bilaterally and high-resolution CT showed absent of stapes suprastructure and footplate. Subsequent diagnosis was done via endoscopic middle ear exploration which revealed an absent long process of the incus, stapes suprastructure and footplate, but with intact oval window membrane. The residual incus was removed, and a tragal perichondrium graft was used over the oval window. A total ossicular replacement prosthesis was placed between the malleus and oval window to repair the chain. Postoperatively, the patient had no complications. Preoperative pure tone average revealed an air/bone result of 52/8 dB. Follow-up after surgery at 6 months showed a pure tone average air/bone result of 15/3 dB. The air-bone gap was reduced from 44 to 12 dB. CONCLUSION: Congenital absence of the stapes suprastructure and footplate remains a rare condition compared to the myriad of middle ear anomalies in the literature.

Does the Location of a Small Tympanic Membrane Perforation Affect the Degree of Hearing Loss in Adult Patients with Inactive Mucosal Chronic Suppurative Otitis Media?

BACKGROUND: Tympanic membrane perforation due to inactive mucosal chronic suppurative otitis media is a common problem in otolaryngology, with consequent conductive hearing loss. Still, there is controversy about the relationship between the location of the tympanic membrane perforation and the degree of hearing impairment. AIM OF THE STUDY: To assess the correlation between the location of a small tympanic membrane perforation and the degree of conductive hearing loss in adult patients with inactive mucosal chronic suppurative otitis media. PATIENTS AND METHODS: A prospective cross-sectional study enrolled 74 adult patients with small tympanic membrane perforations (perforation involves less than one quadrant of the tympanic membrane) and conductive hearing loss (airbone gap ≥ 20 dB HL) due to inactive mucosal chronic suppurative otitis media for at least 3 months. The locations of the tympanic membrane perforations were classified as anterosuperior, anteroinferior, posterosuperior, and poster inferior perforations. Audiometric analysis and a CT scan of the temporal bone were done for all patients. The means of the air and bone conduction pure tone hearing threshold averages at frequencies 500, 1000, 2000, and 4000 Hz were calculated, and consequently, the air-bone gaps were calculated and presented as means. The ANOVA test was used to compare the means of the air-bone gaps, and the Scheffe test was used to determine if there were statistically significant differences regarding the degree of conductive hearing loss in relation to different locations of the tympanic membrane perforation. RESULTS: The ages of the patients ranged from 20 to 43 years (mean = 31.9 ± 6.5 years), of whom 43 (58%) were females and 31 (42%) were males. The means of the air-bone gaps were 32.29 ± 5.41 dB HL, 31.34 ± 4.12 dB HL, 29.87 ± 3.48 dB HL, and 29.30 ± 4.60 dB HL in the posteroinferior, posterosuperior, anteroinferior, and anterosuperior perforations, respectively. Although the air-bone gap's mean was greater in the posteroinferior perforation, statistical analysis showed that it was insignificant (P-value=0.168). CONCLUSION: In adult patients with inactive chronic suppurative otitis media, the anteroinferior quadrant is the most common location of the tympanic membrane perforation, and there was an insignificant correlation between the location of a small tympanic membrane perforation and the degree of conductive hearing loss.

Early Results With the New Active Bone-Conduction Hearing Implant: A Systematic Review and Meta-Analysis.

OBJECTIVE: The bone conduction implant (BCI) 602 is a new transcutaneous BCI with smaller dimensions. However, limited patient numbers restrict the statistical power and generalizability of the current studies. The present systematic review and meta-analysis summarize early audiological and medical outcomes of adult and pediatric patients implanted with the BCI 602 due to mixed or conductive hearing loss. DATA SOURCE: Following the Preferred Reporting items for Systematic Reviews and Meta-analyses guidelines, 108 studies were reviewed, and 6 (5.6%) were included in the meta-analysis. REVIEW METHOD: The data on study and patient characteristics, surgical outcomes, and audiological test results were extracted from each article. Meta-analysis employed the fixed-effect and random-effects models to analyze the mean differences (MDs) between pre- and postoperative performances. RESULTS: In total, 116 patients were evaluated, including 64 (55%) adult and 52 (45%) pediatric patients. No intraoperative adverse events were reported, while postoperative complications were reported in 2 (3.1%) adult and 2 (3.8%) pediatric patients. Studies consistently showed significant improvements in audiological outcomes, quality of life, and sound localization in the aided condition. In the meta-analysis, we observed a significant difference in the unaided compared to the aided condition in sound field thresholds (n = 112; MD, -27.05 dB; P < 0.01), signal-to-noise ratio (n = 96; MD, -6.35 dB; P < 0.01), and word recognition scores (n = 96; MD, 68.89%; P < 0.01). CONCLUSION: The implantation of the BCI 602 was associated with minimal surgical complications and excellent audiological outcomes for both the pediatric and the adult cohort. Therefore, our analysis indicates a high level of safety and reliability. Further research should focus on direct comparisons with other BCIs and long-term functional outcomes.

Nasopharyngeal Cyst Causing Hearing Loss: Case Report and Review of Literature.

Symptomatic nasopharyngeal cysts are relatively uncommon. Here is a case report of 50 years of age female with hearing loss secondary to the nasopharyngeal cyst. She underwent endoscopic marsupialization and achieved normal hearing postoperatively.The study aims to determine the incidence of hearing loss, radiological patterns and histopathological findings associated with the nasopharyngeal cyst.

Approach to Sudden Hearing Loss Among Primary Care Physicians in Riyadh, Saudi Arabia.

INTRODUCTION: A medical emergency known as sudden sensorineural hearing loss (SSNHL) affects the ears suddenly, has a considerable probability of negative cognitive and functional outcomes, and can influence the patient's quality of life. Primary care physicians play a crucial role in diagnosing SSNHL and initiating prompt and efficient management since they are the ones who would likely encounter it initially. This study aims to evaluate the present knowledge, diagnostic, and management perspective of SSNHL among primary care physicians in Riyadh, Saudi Arabia. METHODS: A self-generated questionnaire with 17 questions was developed, and a link to the online survey was delivered to primary care physicians (PHPs) in Riyadh, Saudi Arabia, concerning the management of SSNHL. RESULTS: The knowledge level regarding SSNHL was evaluated, in which 21 (25%) of the participants had a low knowledge level, 34 (40.5%) had moderate knowledge, and 29 (34.5%) had a high knowledge level. Among 84 participants, 20 (23.8%) were confident in their ability to administer and understand the findings of tuning fork tests (TFT) to differentiate between sensorineural hearing loss and conductive hearing loss, whereas 64 (76.2%) were unsure about it. In addition, to distinguish between sensorineural hearing loss and conductive hearing loss, 62 (73.8%) participants were confident, and 22 (26.2%) participants were skeptical about their ability to interpret a formal audiogram. CONCLUSION: Considering SSNHL as a medical emergency, in our survey, many family doctors would make proper referral and treatment decisions. However, TFTs were underutilized for guiding management decisions compared to other ways to distinguish between conductive and sensorineural hearing loss.