Tympanomastoidectomy versus parenteral antibiotic therapy for pediatric otorrhea.

OBJECTIVE: To evaluate the efficacy of tympanomastoidectomy versus parenteral antibiotic therapy for otorrhea as a result of chronic suppurative otitis media (CSOM) without cholesteatoma in the pediatric population. METHODS: A retrospective review of 221 patients treated for otorrhea at a tertiary academic pediatric hospital was performed to evaluate the impact of tympanomastoidectomy versus parenteral antibiotic therapy on resolution of otorrhea. Inclusion criteria were age 0-18 years, prior treatment with otic and/or oral antibiotic, prior history of tympanostomy tube placement for recurrent otitis media, history of otorrhea, treatment with tympanomastoidectomy or parenteral antibiotic therapy, and follow-up of at least 1 month after intervention. Time to resolution was compared between the two modalities adjusting for age, bilateral ear disease status, and comorbidities using a Cox proportional hazard model. RESULTS: Eighty-three ears from 58 children met the inclusion criteria. Ears that initially underwent tympanomastoidectomy had a significantly shorter time to resolution of symptoms (median time to resolution) 9 months (95 % confidence interval CI: 6.2-14.8) vs. 48.5 months (95 % lower CI 9.4, p = 0.006). On multivariate analysis, however, only bilateral ear disease status was independently associated with time to resolution of symptoms (hazard ratio 0.4, 95 % CI 0.2-0.9, p = 0.03). There was no statistically significant difference in the rate of treatment-related complications when comparing tympanomastoidectomy to parenteral antibiotic therapy (p = 0.37). CONCLUSION: When adjusting for age, bilateral ear disease status, and comorbidities, there does not appear to be a significant difference in time to resolution of symptoms when comparing parenteral antibiotic therapy to tympanomastoidectomy. An informed discussion regarding risks and benefits of each approach should be employed when deciding on the next step in management for patients with CSOM who have failed more conservative therapies.

Cogan's syndrome is more than just keratitis: a case-based literature review.

BACKGROUND: Cogan's syndrome (CS) is a rare autoimmune disorder characterized by non-syphilitic interstitial keratitis (IK) and Menière-like cochlear vestibular symptoms, which may also have systemic effects. Corticosteroids are first-line treatment. DMARDs and biologics have been used to treat ocular and systemic symptoms of CS. CASE PRESENTATION: This is a case of a 35-year-old female who reported hearing loss, eye redness and photophobia. Her condition progressed to a sudden sensorineural hearing loss, tinnitus, and constant vertigo accompanied by cephalea. CS was diagnosed after excluding other diseases. The patient still developed bilateral sensorineural hearing loss after receiving hormone, methotrexate, cyclophosphamide, and a variety of biological agents. Joint symptoms were relieved after treatment with a JAK inhibitor (tofacitinib), and hearing did not deteriorate further. CONCLUSIONS: CS should be involved in the differential diagnosis of keratitis. Early identification and intervention of this autoimmune disease can minimize disability and irreversible damage.

Ear and hearing care programs for First Nations children: a scoping review.

BACKGROUND: Ear and hearing care programs are critical to early detection and management of otitis media (or middle ear disease). Otitis media and associated hearing loss disproportionately impacts First Nations children. This affects speech and language development, social and cognitive development and, in turn, education and life outcomes. This scoping review aimed to better understand how ear and hearing care programs for First Nations children in high-income colonial-settler countries aimed to reduce the burden of otitis media and increase equitable access to care. Specifically, the review aimed to chart program strategies, map the focus of each program against 4 parts of a care pathway (prevention, detection, diagnosis/management, rehabilitation), and to identify the factors that indicated the longer-term sustainability and success of programs. METHOD: A database search was conducted in March 2021 using Medline, Embase, Global Health, APA PsycInfo, CINAHL, Web of Science Core Collection, Scopus, and Academic Search Premier. Programs were eligible or inclusion if they had either been developed or run at any time between January 2010 to March 2021. Search terms encompassed terms such as First Nations children, ear and hearing care, and health programs, initiatives, campaigns, and services. RESULTS: Twenty-seven articles met the criteria to be included in the review and described a total of twenty-one ear and hearing care programs. Programs employed strategies to: (i) connect patients to specialist services, (ii) improve cultural safety of services, and (iii) increase access to ear and hearing care services. However, program evaluation measures were limited to outputs or the evaluation of service-level outcome, rather than patient-based outcomes. Factors which contributed to program sustainability included funding and community involvement although these were limited in many cases. CONCLUSION: The result of this study highlighted that programs primarily operate at two points along the care pathway-detection and diagnosis/management, presumably where the greatest need lies. Targeted strategies were used to address these, some which were limited in their approach. The success of many programs are evaluated as outputs, and many programs rely on funding sources which can potentially limit longer-term sustainability. Finally, the involvement of First Nations people and communities typically only occurred during implementation rather than across the development of the program. Future programs should be embedded within a connected system of care and tied to existing policies and funding streams to ensure long term viability. Programs should be governed and evaluated by First Nations communities to further ensure programs are sustainable and are designed to meet community needs.

Surgical results and quality of life after subtotal petrosectomy.

PURPOSE: Few data are available regarding subjective complaints and quality of life (QoL) after subtotal petrosectomy (STP). The purpose of our study was to assess long-term surgical results after STP, and to evaluate disease-specific, patient-reported outcomes including QoL and subjective hearing. METHODS: A retrospective cohort study, including a postal survey, was performed in the Amsterdam University Medical Centers (Amsterdam UMC) location Academic Medical Centre (AMC). All patients who underwent a STP between 1990 and 2018 were included. Patient characteristics, indication for surgery, adverse events, disease recidivism, and patient-reported health outcomes were evaluated. RESULTS: 181 patients (183 ears) underwent a STP for several indications. The main indication was chronic otitis media (COM) with or without cholesteatoma (69%). In the total cohort, 8 residual cholesteatoma (5%) and 6 inclusion cholesteatoma 4% were detected. Postoperative (functional) health outcomes showed a significant negative impact on QoL in the STP cohort compared to normative data. Compared to patients without ear problems, the postoperative STP patients scored worse on almost all domains of the Chronic Ear Survey (CES) (p < 0.001). SF-36 scores of postoperative STP data showed negative Z-scores in almost all subscales, indicating lower levels of QoL compared to Dutch reference values. Most subscales of the Amsterdam Inventory for Auditory Disability and Handicap (AIADH) demonstrate a large-effect size in disadvantage of the STP cohort when compared to Dutch reference values. CONCLUSION: STP is a suitable technique to tackle severe ear disease. Despite its favourable surgical results, STP has a negative impact on several domains of patient's QoL.

Giraffe skin disease: Clinicopathologic characterization of cutaneous filariasis in the critically endangered Nubian giraffe (Giraffa camelopardalis camelopardalis).

Giraffe skin disease (GSD) is an emerging disease of free-ranging giraffe recognized in the last 25 years in several species, including the critically endangered Nubian giraffe (Giraffa camelopardalis camelopardalis) of Uganda. Identifying the cause of GSD and understanding its impact on health were deemed paramount to supporting these vulnerable populations. Sixty-four giraffes were immobilized in Murchison Falls National Park, Uganda, from 2017 to 2019, and GSD lesions were opportunistically biopsied. Fifty-five giraffes (86%) had GSD lesions on the neck, axilla, chest, and cranial trunk. Lesions were categorized into early, intermediary, and dormant stages based on gross and histological characteristics. Early lesions were smaller, crusted nodules with eosinophilic and pyogranulomatous dermatitis and furunculosis. Intermediary lesions were thick plaques of proliferative and fissured hyperkeratosis and acanthosis with dense dermal granulation tissue and severe eosinophilic and granulomatous dermatitis. Lesions appeared to resolve to dormancy, with dormant lesions consisting of hairless plaques of hyperkeratosis with dermal scarring and residual inflammation. The periphery of early and intermediary lesions included follicular granulomas containing adult filarid nematodes, with myriad encysted microfilariae in the superficial dermis. Stage L3 larvae were common in early and intermediary lesions, and dormant lesions had remnant encysted microfilariae with no adult or stage L3 larvae. Nematodes were morphologically and genetically novel with close identity to Stephanofilaria spp. and Brugia malayi, which cause infectious filariasis. Identification of potential insect vectors, long-term monitoring of GSD lesions, and evaluating response to therapy is ongoing in the efforts to help conserve the Nubian giraffe.

Clinical high-resolution imaging and grading of endolymphatic hydrops in Hydropic Ear Disease at 1.5 T using the two-slice grading for vestibular endolymphatic hydrops in less than 10 min.

BACKGROUND: Hydropic Ear Disease (Menière) is one of the most common inner ear disorders and one of the most common causes of vertigo attacks. The underlying pathology is a distension of the endolymphatic space of the inner ear, termed endolymphatic hydrops. However, the unequivocal morphologic confirmation of ELH has been restricted to post-mortem histologic analysis until 2007, when the first clinical MR imaging report demonstrated ELH in living patients with Menière's disease at 3 T combined with intratympanic application of contrast. Imaging techniques have since then evolved further. However, a high magnetic field strength of 3 T has consistently been mandatory for reliable clinical imaging of ELH. This limitation has significantly prevented ELH imaging from being widely available across different health care systems around the world. With the aim of filling this gap, in the present study, we aim to describe the feasibility of ELH imaging at 1.5 T in clinical practice and to develop a dedicated grading system for cochlear and vestibular ELH for MR imaging at 1.5 T. METHODS: In this retrospective study, we examined 30 patients with suspected hydropic ear disease undergoing diagnostic MR imaging. Contrast agent was diluted eightfold in saline solution and unilaterally applied by intratympanic injection as described previously. MRI scanning was performed using a 16-channel head coil on a 1.5 T Achieva Philips Medical Systems Scanner using a 3D FLAIR sequence. For the cochlea, a 3-stage grading was developed. For the vestibulum, a 4-stage grading based on two axial slices was developed by analysing both the superior and the inferior part of the vestibulum. The presence of hydropic herniation of the endolymphatic space into the posterior crus of the horizontal semicircular canal was evaluated. RESULTS: In all 30 patients, the perilymphatic fluid spaces of the inner ear showed clear and high signal intensity, while the endolymphatic space was not enhanced. In all patients, the vestibular endolymphatic space could be clearly delineated and differentiated from the perilymphatic space. Analysis of the cochlear endolymphatic space revealed no evidence of ELH in 7 patients, a grade 1 cochlear ELH in 11 patients and a grade 2 cochlear ELH in 12 patients. Analysis of the vestibular endoylmphatic space revealed no evidence of ELH in 8 patients, a grade 1 vestibular ELH in 5 patients, a grade 2 vestibular ELH in 9 patients and a grade 3 vestibular ELH in 8 patients. Three patients showed a clear hydropic herniation of the vestibular endolymphatic space into the posterior non-ampullated crus of the horizontal SCC. CONCLUSION: In summary, the findings presented in this study offer an easy, reliable and universally available technique of ELH imaging for diagnostic management of patients with suspected Hydropic Ear Disease.

Sensorineural Hearing Loss in Sjögren's Syndrome.

Sjögren's syndrome is a chronic autoimmune disease characterized by systemic dysfunction of exocrine glands, mainly the salivary and lachrymal glands. Sjögren's syndrome consists of two forms: primary Sjögren's syndrome, which is characterized by dry eyes and dry mouth without autoimmune diseases; and secondary Sjögren's syndrome, which is characterized by symptoms associated with other autoimmune diseases, such as systemic lupus erythematosus. Disease severities vary considerably from mild glandular dryness to severe glandular involvement with numerous extraglandular and systemic features. Sensorineural hearing loss is sometimes observed in both primary and secondary Sjögren's syndrome. This review article consists of (1) Pathology of Sjögren's syndrome, (2) Clinical manifestation of Sjögren's syndrome, (3) Autoimmune inner ear disease, (4) Histoanatomical features of the inner ear, (5) Immunological characteristics of the inner ear, (6) Pathophysiology of autoimmune inner ear disease, (7) Treatment for sensorineural hearing loss in Sjögren's syndrome, and (8) Future direction. Finally, we introduce a recently developed disease model of salivary gland inflammation and discuss future expectations for the treatment of sensorineural hearing loss in Sjögren's syndrome.

Long-term (16-26 years) follow-up outcome of steroid therapy in refractory autoimmune sensorineural hearing loss.

Autoimmune sensorineural hearing loss (ASHL) is a rare disease of uncertain etiology, with no established treatment strategy. The duration of morbidity is increased in refractory cases; and therefore, the preservation of hearing and the prevention of adverse effects with steroid therapy are serious long term issues to consider. Long-term follow up of patients treated for ASHL was performed retrospectively in order to elucidate the pathogenesis of ASHL, evaluate the consequences of steroid therapy, and determine a promising treatment course. The cohort in this study consists of four female patients with refractory ASHL that were followed for 16-26 years. Three patients already had profound deafness on one side, probably due to ASHL, before the initiation of steroid treatment. ASHL was managed with steroid administration and the hearing was evaluated through regular audiometric tests (173-212 times). The relationship between pure tone threshold average and steroid dose was reviewed over a long-term follow-up period for each patient. During follow-up, hearing deficit progressed rapidly several times in all patients, as did responsiveness to steroid therapy. Long-term high-dose steroid therapy was not required for hearing maintenance. Hearing thresholds were nearly maintained in three patients during the 16- to 21- year follow-up, and gradually declined over a 26-year follow-up period in one patient. Considering the progress due to presbycusis, the maintenance of hearing was considered sufficient in all patients. No serious adverse effects were observed in any of the patients. Management of patients affected by ASHL with regular audiometry allowed for hearing maintenance without the morbidity of prolonged steroid therapy. The current observations give insight into the pathogenesis of ASHL pathogenesis and establish an efficient course of treatment.

Autoimmune Inner Ear Disease among Patients with Selective IgA Deficiency.

OBJECTIVES: Autoimmune inner ear disease (AIED) is a distinct clinical entity from sudden sensorineural hearing loss. The purpose of this study was to investigate the clinical characteristics of AIED in patients with selective IgA deficiency (sIgAD). MATERIALS AND METHODS: This retrospective observational study was based on data from the Leumit Healthcare Services database in Israel. We searched all subjects aged ≥12 years who had undergone serum total IgA measurements during 2004-2014 for any reason. The sIgAD patients included all subjects with serum IgA of ≤7 mg/dL (0.07 g/L). A control group was randomly sampled from the full study population (n ≈ 730,000) with a case-control ratio of 10 controls for each case (1:10). RESULTS: Among 347 subjects with sIgAD, we identified 9 patients with concomitant AIED (sIgAD + AIED group). This group was characterized by a higher prevalence of allergic diseases (8 patients; 88.9%) than sIgAD patients without AEID (sIgAD + AIED group; 153 patients; 45.2%; p = 0.014). Both systemic diseases (3 patients; 33.3%) and organ-specific autoimmune diseases (7 patients; 77.8%) were more prevalent in the sIgAD + AIED group (sIgAD + AIED group: 19 patients 5.5%, p = 0.015; sIgAD - AEID group: 76 patients, 21.9%, p < 0.001), with an OR of 8.39 (1.94-36.19; p = 0.004). sIgAD patients with and without AIED were characterized by a higher prevalence of documented episodes of acute otitis media, allergic diseases, and autoimmune diseases than the control group. CONCLUSION: The study exposes a significant association between AIED and sIgAD. We believe that sIgAD has to be excluded in AIED patients.

Can an advanced audiology-led service reduce waiting times for paediatric ear nose and throat outpatient services?

AIM: Children with middle ear disease often experience lengthy delays waiting for outpatient paediatric ear nose and throat (ENT) services. This study aimed to investigate whether an alternative service delivery model using audiologists working in an expanded scope of practice reduced waiting times for children to access such services. METHODS: A total of 131 children consecutively referred to a large ENT outpatient service in Queensland, Australia, for middle ear and hearing concerns were prospectively allocated to either a standard ENT service or an advanced audiology (AA)-led service. Waiting times and attendance rates were collected and compared between the two patient groups. RESULTS: The median waiting time from referral to first offered appointment was 96 days for children in the AA-led service versus 417.5 days for children in the standard ENT service. Seventy-nine percent of children in the AA-led service attended their first offered appointment versus 61% in the standard ENT service. For children receiving grommets, the median waiting time from initial referral to grommet insertion was 226 days for children in the AA-led service versus 627 days for children in the standard ENT service. CONCLUSION: The AA-led service was an effective alternative pathway to reduce waiting times for children referred to ENT services with middle ear and hearing concerns.

Another 'BEE'? - Brain-Eye-Ear (BEE) Disease Secondary to HbSC Disease Masquerading as Multiple Sclerosis.

Recurrent episodes of neurological dysfunction and white matter lesions in a young adult raise suspicion for multiple sclerosis (MS). However, occlusive retinopathy, hearing loss and absence of CSF oligoclonal bands are atypical for MS and should make the clinician consider an alternative diagnosis. We describe a man with hearing loss, visual signs and symptoms, and an accumulating burden of brain lesions, who was treated for a clinical diagnosis of MS for nearly two decades. Genetic testing revealed a unifying diagnosis.

Training, supervision and performance of Community Health Workers in the delivery of ear and hearing care to 321 community members in rural Uganda.

OBJECTIVES: Community Health Workers are one way to address the shortage of ear and hearing care specialists in low-resource settings. However, there are few reports evaluating training and service delivery by Community Health Workers. DESIGN, SETTING AND PARTICIPANTS: We trained 13 Community Health Workers in primary ear and hearing care in Mukono District, Uganda. Community Health Workers attended a two-day training workshop and received remote supervision thereafter during service delivery in the community. An ear camp was held at the local health centre every two months, where a local ENT specialist could assess referred cases. MAIN OUTCOME MEASURES: Clinical and diagnostic skills and decision-making were assessed using an Objective Structured Clinical Examination, with scores recorded at baseline and six months. Service delivery was evaluated by analysing the following: (i) number of individuals evaluated; (ii) treatments delivered; (iii) cases referred for specialist opinion; (iv) proportion of appropriately referred cases; and (v) agreement between Community Health Worker and specialist diagnosis. RESULTS: Observed Structured Clinical Examination scores were high and stable for six months. 312 individuals were screened in the community by the Community Health Workers, with 298 classified as having an abnormality. Care was delivered in the community to 167 of these, and the remaining 131 referred to the ear camp. Diagnostic agreement was 39%, but 98% of referrals were deemed "appropriate" by the ENT specialist. 27 individuals self-presented to the ear camp without prior assessment by a Community Health Worker, and 97% of these were deemed appropriate. CONCLUSION: Trained Community Health Workers can play an important role in delivering ear and hearing services. Future work should look to explore this model in other contexts and/or compare it to other models of service delivery.

Exploring perceptions, barriers, and enablers for delivery of primary ear and hearing care by community health workers: a photovoice study in Mukono District, Uganda.

BACKGROUND: Hearing loss is a prevalent but neglected disease, especially in low- or middle-income countries. The role of Community Health Workers (CHWs) to deliver primary ear and hearing care has been explored in several studies from a technical standpoint, but understanding perceptions, barriers, and enablers of such an approach from the perspective of CHWs themselves through a health equity lens has been less well documented. METHODS: This qualitative study used photovoice to explore the views and experiences of CHWs in the Seeta Nazigo Parish of Mukono District in the delivery of ear and hearing care in the community. CHWs were trained in ear and hearing care, and provided with digital cameras to capture photographs related to their work in the community over the following 3 months. Individual interviews regarding the photographs were held at the end of each month, in addition to one focus group discussion. A community workshop was convened at the end of the study to display the photos. Thematic analysis of photographs was conducted using Braune and Clarkes six-step framework. We also used the data to explore potential roles for key stakeholders in primary ear and hearing care, and how photovoice may facilitate their engagement. RESULTS: 13 CHWs participated in the study. Several themes were generated from analysis. CHWs perceived a high burden of ear and hearing disorders in their community and recognised the role they could play in tackling that burden. Potential barriers identified included a lack of equipment, training, and supervision of CHWs; logistical, financial, or psychological barriers to community participation; and the widespread use of traditional medicine. CHWs identified roles for the government and NGO bodies to enable and support delivery of ear and hearing care in the community. The community workshop was a useful method to engage key stakeholders in this topic. CONCLUSIONS: Photovoice is a powerful method to capture issues affecting CHWs. Here it was used to identify a number of perceptions, barriers and enablers to the delivery of ear and hearing care. Our results may inform future strategy in the field of ear and hearing care, and the potential use of photovoice to enact sociocultural change.

Evaluation of Subtotal Petrosectomy Technique in Difficult Cases of Cochlear Implantation.

OBJECTIVES: To assess the validity of the subtotal petrosectomy (STP) technique in problematic cases of cochlear implant (CI) surgery, and review indications, outcomes, and related controversies. STUDY DESIGN: This is a retrospective review of data from a private quaternary referral center of otology and skull base surgery. PATIENTS AND METHODS: A review of patients who underwent CI with STP (STP-CI) as the leading approach was performed. Demographics, indications, surgical details, and main outcomes were evaluated. The surgeries performed were usually single-stage procedures encompassing a comprehensive mastoidectomy, blind sac closure of the external auditory canal (EAC), and mastoid obliteration with autologous fat. RESULTS: A total of 107 cases were included. Mean follow-up was 7.1 years (range 1-13 years). The most frequent indication for STP-CI was chronic otitis media with/without cholesteatoma (32.7%), followed by open mastoid cavity (26.1%), and cochlear ossification (17.7%). Other difficult conditions where STP facilitates successful implantation include inner-ear malformations, temporal-bone trauma, unfavorable anatomic conditions, and revision surgery. A planned staged procedure was performed in 3 cases. The rate of major complications was 5.6% (n = 6). Three patients developed postauricular wound dehiscence which eventually resulted in device extrusion. No cases of recurrent/entrapped cholesteatoma, EAC breakdown, or meningitis were encountered. This is the largest single-center series of STP-CI reported in the literature. CONCLUSIONS: When CI is intended in technically challenging cases or associated with a high risk of complications, STP is effective and reliable. Safe implantation and excellent long-term outcomes can be achieved provided surgical steps are properly followed. Single-stage procedures can be performed in most cases, even when there is active middle-ear disease.

The social determinants of otitis media in aboriginal children in Australia: are we addressing the primary causes? A systematic content review.

BACKGROUND: Aboriginal and Torres Strait Islander children experience some of the highest rates of otitis media in the world. Key risk factors for otitis media in Aboriginal children in Australia are largely social and environmental factors such as overcrowded housing, poverty and limited access to services. Despite this, little is known about how to address these risk factors. A scoping content review was performed to determine the relationship between social determinants of health and otitis media in Aboriginal and Torres Strait Islander children as described by peer-reviewed and grey literature. METHOD: Search terms were established for location, population and health condition. The search terms were used to conduct a literature search using six health research databases. Following the exclusion process, articles were scoped, analysed and categorised using scoping parameters and a social determinants of health framework. RESULTS: Housing-related issues were the most frequently reported determinants for otitis media (56%). Two articles (4%) directly investigated the impact of social determinants of health on rates of otitis media within Aboriginal and Torres Strait Islander children. The majority of the literature (68%) highlights social determinants as playing a key role in the high rates of otitis media seen in Aboriginal populations in Australia. There were no intervention studies targeting social determinants as a means to reduce otitis media rates among Aboriginal and Torres Strait Islander children. CONCLUSIONS: This review identifies a disconnect between otitis media drivers and the focus of public health interventions within Aboriginal and Torres Strait Islander populations. Despite consensus that social determinants play a key role in the high rates of otitis media in Aboriginal and Torres Strait Islander children, the majority of intervention studies within the literature are focussed on biomedical approaches such as research on vaccines and antibiotics. This review highlights the need for otitis media intervention studies to shift away from a purely biomedical model and toward investigating the underlying social determinants of health. By shifting interventions upstream, otitis media rates may decrease within Aboriginal and Torres Strait Islander children, as focus is shifted away from a treatment-focussed model and toward a more preventative model.

Prevalence, risk factors and management strategies for otological problems in girls with Turner syndrome.

AIM: To determine the prevalence and risk factors of ear disease in Turner syndrome (TS), propose an algorithm for future surveillance and recommend preventative strategies. METHODS: Review of TS patients seen in the West of Scotland between 1989 and 2015, with questionnaire follow-up in 2015. RESULTS: Of 168 girls, median age 27.3 (3.8-47.2) years, ear problems occurred more frequently with 45,X and 45,X/46,XiXq than other karyotypes: 71/103 (69%) versus 23/65 (35%). Recurrent acute otitis media (AOM) first developed at 0-5 years in 23 (40%) girls, persisting in 16 (10%) at 5-10 years; and first developing at 5-10 years in 11 (7%). Persistent otitis media with effusion (OME) first developed at 0-5 and 5-10 years in 23 (40%) and 14 (8%) girls. Recurrent AOM was significantly linked with cholesteatoma in 8 (4.9%) girls (7 aged >10 years). Permanent hearing loss was documented in 28 girls (16.7%), with 16 (9.5%) receiving hearing aids (bone-anchored in 3). CONCLUSION: Acute otitis media and OME occur commonly in preschool TS girls and may persist or newly develop in later childhood. Recurrent AOM predisposes to cholesteatoma. Strategies to reduce otological morbidity include: intensive patient education, annual audiology, vaccinations and a randomised trial of antibiotic prophylaxis in high-risk groups.

Hearing and hearing rehabilitation after obliteration of troublesome mastoid cavities.

PURPOSE: The purpose of this study is the evaluation of post-operative hearing threshold after revision surgery and obliteration of troublesome canal wall down mastoidectomy cavities (CWDMCs). The ability to use and tolerate conventional hearing aids (CHAs) was also evaluated. METHODS: A retrospective chart analysis of 249 patients with chronically draining CWDMCs who underwent revision surgery including obliteration of the mastoid cavity between 2007 and 2017 at the AMC location of the Amsterdam University Medical Centers (Amsterdam UMC) was performed. Patient characteristics, pre- and post-operative Merchant grade, surgical outcomes, pre- and post-operative hearing thresholds, and the ability/necessity to use a CHA or the ability/necessity to use a Bone Conduction Device (BCD) were recorded. RESULTS: Dry ears were found in 95% of the total cohort. Residual disease was detected in 1.6% during MRI follow-up with no residual cholesteatoma in the obliterated area. In 3.2% of the patients, recurrent disease was found. A significant improvement in mean air conduction level, mean bone conduction level, and mean air-bone gap (ABG) was found post-operatively (p < 0.05). For all types of ossicular chain reconstruction, a significant improvement in mean Pure Tone Average was observed (p < 0.05). The percentage of patients with an indication for CHA was similar pre- and post-operatively (67% both pre- and post-operatively). The ability to use a CHA improved from 3% pre-operatively to 57% post-operatively (p < 0.001). CONCLUSION: This study shows that revision surgery and obliteration of CWDMCs enable successful CHA rehabilitation post-operatively. Upon this type of surgery, hearing thresholds improve significantly, but the need for rehabilitation with a CHA remains necessary in most cases.

Mastoid cavity obliteration leads to a clinically significant improvement in health-related quality of life.

OBJECTIVE: To assess the change in health-related quality of life (HRQoL) in patients undergoing mastoid cavity obliteration. METHODS: Patients who had undergone canal wall-down mastoidectomy for chronic otitis media with creation of a persistent mastoid cavity and underwent revision tympanomastoid surgery including mastoid cavity obliteration using autologous material were included. Audiological measurements including air conduction (AC) and bone conduction (BC) pure-tone averages (PTA) and the air-bone gap (ABG) were assessed. Health-related Quality of Life (HRQoL) was assessed by the Zurich Chronic Middle Ear Inventory (ZCMEI-21) pre- and postoperatively. RESULTS: A total of 25 patients (16 females and 9 males; mean age 51.6 years, 14 right and 11 left ears) were included. Patients were reexamined after a mean follow-up period of 9.2 months (SD = 6.5) after obliteration of the mastoid cavity. Compared to the preoperative visit, patients showed a significantly reduced AC PTA at the postoperative visit (mean difference: - 4.1; SD = 10.4, p = 0.045). The mean ZCMEI-21 score changed from 31.7 (SD = 14.5) preoperatively to 17.4 (SD = 15.1) postoperatively (mean difference: - 14.3; SD = 19.1; p = 0.0002). The mean ZCMEI-21 score changes were neither correlated to the AC PTA shift (p = 0.60) nor to the ABG shift (p = 0.66). CONCLUSIONS: This is the first study reporting a highly significant and clinically important improvement in HRQoL after mastoid cavity obliteration in a prospective setting. The improvement in HRQoL was not correlated to the hearing improvement. As a clinical implication, we provide evidence for a substantial subjective benefit of the surgical obliteration of a symptomatic mastoid cavity and, therefore, encourage this surgical procedure.

Services for children with hearing loss in urban and rural Australia.

OBJECTIVE: To report satisfaction with services for children with hearing loss in urban and rural Australia. DESIGN: Mixed-method approach using surveys and semi-structured interviews. SETTING: Australian organisations that serve children with hearing loss. PARTICIPANTS: One hundred parents of children with hearing loss and 91 professionals that serve children with hearing loss completed surveys. Seven parents and eight professionals were interviewed. MAIN OUTCOME MEASURES: Comparison of satisfaction with services in rural and urban areas. RESULTS: Timing of initial hearing services was similar in rural and urban areas. Children with hearing loss in rural areas had less satisfaction with services than children in urban areas. Parents of children with hearing loss in rural areas had higher costs, mainly linked to travel, than parents in urban areas. Parents and professionals were concerned that advantaged parents received more services for their child than disadvantaged parents. Parents and professionals in urban and rural areas were satisfied with mainstream education, but less satisfied with education for children with hearing loss and additional disability. Professional satisfaction was lower in rural areas than urban areas. CONCLUSIONS: This small-scale study adds to research citing reduced services in rural areas. An exception is the newborn hearing screening program, with the limited data presented indicating the program is effectively overcoming the barrier of distance. However, children with hearing loss in rural areas have reduced access to ongoing services.

Fluctuating Sensorineural Hearing Loss.

BACKGROUND: Several otologic conditions can present with fluctuating sensorineural hearing loss, including Ménière's disease, autoimmune inner ear disease, and enlarged vestibular aqueduct. Although these 3 etiologies vary greatly, distinguishing between these conditions at initial presentation can be challenging. Furthermore, initial treatment of these conditions is often similar. In this review, we discuss historical and current perspectives on diagnosis and treatment of these conditions. SUMMARY: A literature search was performed regarding fluctuating hearing loss, and current treatment of these etiologies of fluctuating hearing loss was summarized. Immediate measures at the onset of acute hearing loss include corticosteroid therapy, while preventative and chronic therapies, which can limit disease severity and frequency, vary based on the specific condition treated. Key Messages: Fluctuating hearing loss can represent a range of pathologies, but the precise etiology may not be clear at initial presentation. Timely treatment and long-term follow-up, along with appropriate diagnostics, are necessary to optimize long-term hearing.