Access to Sudden Sensorineural Hearing Loss Care at Private Equity-Owned Otolaryngology Clinics.

OBJECTIVE: Characterizing access to sudden sensorineural hearing loss (SSNHL) care at private practice otolaryngology clinics of varying ownership models. STUDY DESIGN: Cross-sectional prospective review. SETTING: Private practice otolaryngology clinics. METHODS: We employed a Secret Shopper study design with private equity (PE) owned and non-PE-owned clinics within 15 miles of one another. Using a standardized script, researchers randomly called 50% of each clinic type between October 2021 and January 2022 requesting an appointment on behalf of a family member enrolled in either Medicaid or private insurance (PI) experiencing SSNHL. Access to timely care was assessed between clinic ownership and insurance type. RESULTS: Seventy-eight total PE-owned otolaryngology clinics were identified across the United States. Only 40 non-PE clinics could be matched to the PE clinics; 39 PE and 28 non-PE clinics were called as Medicaid patients; 39 PE and 25 non-PE clinics were called as PI patients; 48.7% of PE and 28.6% of non-PE clinics accepted Medicaid. The mean wait time to new appointment ranged between 9.55 and 13.21 days for all insurance and ownership types but did not vary significantly (P > .480). Telehealth was significantly more likely to be offered for new Medicaid patients at non-PE clinics compared to PE clinics (31.8% vs 0.0%, P = .001). The mean cost for an appointment was significantly greater at PE clinics than at non-PE clinics ($291.18 vs $203.75, P = .004). CONCLUSIONS: Patients seeking SSNHL care at PE-owned otolaryngology clinics are likely to face long wait times prior to obtaining an initial appointment and reduced telehealth options.

Exome Sequencing for Isolated Congenital Hearing Loss: A Cost-Effectiveness Analysis.

OBJECTIVES/HYPOTHESIS: To assess the relative cost-effectiveness of exome sequencing for isolated congenital deafness compared with standard care. STUDY DESIGN: Incremental cost-effectiveness and cost-benefit analyses were undertaken from the perspective of the Australian healthcare system using an 18-year time horizon. METHODS: A decision tree was used to model the costs and outcomes associated with exome sequencing and standard care for infants presenting with isolated congenital deafness. RESULTS: Exome sequencing resulted in an incremental cost of AU$1,000 per child and an additional 30 diagnoses per 100 children tested. The incremental cost-effectiveness ratio was AU$3,333 per additional diagnosis. The mean societal willingness to pay for exome sequencing was estimated at AU$4,600 per child tested relative to standard care, resulting in a positive net benefit of AU$3,600. Deterministic and probabilistic sensitivity analyses confirmed the cost-effectiveness of exome sequencing. CONCLUSIONS: Our findings demonstrate the cost-effectiveness of exome sequencing in congenital hearing loss, through increased diagnostic rate and consequent improved process of care by reducing or ceasing diagnostic investigation or facilitating targeted further investigation. We recommend equitable funding for exome sequencing in infants presenting with isolated congenital hearing loss. LEVEL OF EVIDENCE: N/A. Laryngoscope, 131:E2371-E2377, 2021.

The Potential Added Value of Novel Hearing Therapeutics: An Early Health Economic Model for Hearing Loss.

OBJECTIVE: To construct an early health economic model to assess the potential added value of novel hearing therapeutics, compared with the current standard of care. We use idiopathic sudden sensorineural hearing loss (ISSNHL) as a case example, because it is a lead indication for several emerging hearing therapeutics. METHODS: A decision analytic model was developed to assess the costs and effects of using novel hearing therapeutics for patients with ISSNHL. This was compared to the current standard of care. Input data were derived from literature searches and expert opinion. The study adopted a healthcare perspective of the UK National Health Service. Four analyses were conducted: 1) headroom, 2) scenario, 3) threshold, 4) sensitivity. RESULTS: The decision analytic model showed that novel therapeutics for ISSNHL have potential value both in terms of improved patient outcomes, as well as incremental net monetary benefit (iNMB). The base case analysis revealed an iNMB of £39,032 for novel therapeutics compared with the current standard of care. Results of the threshold and scenario analysis revealed that age of treatment and severity of ISSNHL are major determinants of iNMB for novel therapeutics. CONCLUSION: This article describes the first health economic model for novel therapeutics for hearing loss; and shows that novel hearing therapeutics can be cost-effective under NICE's cost-effectiveness threshold, with considerable room for improvement in the current standard of care. Our model can be used to inform the development of cost-effective hearing therapeutics; and help decision makers decide which therapeutics represent value for money.

Diagnostic and therapeutic applications of genomic medicine in progressive, late-onset, nonsyndromic sensorineural hearing loss.

The progressive, late-onset, nonsyndromic, sensorineural hearing loss (PNSHL) is the most common cause of sensory impairment globally, with presbycusis affecting greater than a third of individuals over the age of 65. The etiology underlying PNSHL include presbycusis, noise-induced hearing loss, drug ototoxicity, and delayed-onset autosomal dominant hearing loss (AD PNSHL). The objective of this article is to discuss the potential diagnostic and therapeutic applications of genomic medicine in PNSHL. Genomic factors contribute greatly to PNSHL. The heritability of presbycusis ranges from 25 to 75%. Current therapies for PNSHL range from sound amplification to cochlear implantation (CI). PNSHL is an excellent candidate for genomic medicine approaches as it is common, has well-described pathophysiology, has a wide time window for treatment, and is amenable to local gene therapy by currently utilized procedural approaches. AD PNSHL is especially suited to genomic medicine approaches that can disrupt the expression of an aberrant protein product. Gene therapy is emerging as a potential therapeutic strategy for the treatment of PNSHL. Viral gene delivery approaches have demonstrated promising results in human clinical trials for two inherited causes of blindness and are being used for PNSHL in animal models and a human trial. Non-viral gene therapy approaches are useful in situations where a transient biologic effect is needed or for delivery of genome editing reagents (such as CRISPR/Cas9) into the inner ear. Many gene therapy modalities that have proven efficacious in animal trials have potential to delay or prevent PNSHL in humans. The development of new treatment modalities for PNSHL will lead to improved quality of life of many affected individuals and their families.

Assessment of the Clinical Benefit of Imaging in Children With Unilateral Sensorineural Hearing Loss: A Systematic Review and Meta-analysis.

IMPORTANCE: Imaging used to determine the cause of unilateral sensorineural hearing loss (USNHL) in children is often justified by the high likelihood of detecting abnormalities, which implies that these abnormalities are associated with hearing loss and that imaging has a positive contribution to patient outcome or well-being by providing information on the prognosis, hereditary factors, or cause of hearing loss. OBJECTIVES: To evaluate the diagnostic yield of computed tomography (CT) and magnetic resonance imaging (MRI) in children with isolated unexplained USNHL and investigate the clinical relevance of these findings. EVIDENCE REVIEW: Cochrane Library, Embase, PubMed, and Web of Science databases were searched for articles published from 1978 to 2017 on studies of children with USNHL who underwent CT and/or MRI of the temporal bone. Two authors (F.G.R. and E.N.B.P.) independently extracted information on population characteristics, imaging modality, and the prevalence of abnormalities and assessed the studies for risk of bias. Eligibility criteria included studies with 20 or more patients with USNHL who had CT and/or MRI scans, a population younger than 18 years, and those published in English. MAIN OUTCOMES AND MEASURES: The pooled prevalence with 95% CI of inner ear abnormalities grouped according to finding and imaging modality. FINDINGS: Of 1562 studies, 18 were included with a total of 1504 participants included in the analysis. Fifteen studies were consecutive case studies and 3 were retrospective cohort studies. The pooled diagnostic yield for pathophysiologic relevant findings in patients with unexplained USNHL was 37% for CT (95% CI, 25%-48%) and 35% for MRI (95% CI, 22%-49%). Cochleovestibular abnormalities were found with a pooled frequency of 19% for CT (95% CI, 14%-25%) and 16% for MRI (95% CI, 7%-25%). Cochlear nerve deficiency and associated cochlear aperture stenosis had a pooled frequency of 16% for MRI (95% CI, 3%-29%) and 44% for CT (95% CI, 36%-53%), respectively. Enlarged vestibular aqueduct (EVA) was detected with a pooled frequency of 7% for CT and 12% for MRI in children with USNHL. CONCLUSIONS AND RELEVANCE: Imaging provided insight into the cause of hearing loss in a pooled frequency of about 35% to 37% in children with isolated unexplained USNHL. However, none of these findings had therapeutic consequences, and imaging provided information on prognosis and hereditary factors only in a small proportion of children, namely those with EVA. Thus, there is currently no convincing evidence supporting a strong recommendation for imaging in children who present with USNHL. The advantages of imaging should be carefully balanced against the drawbacks during shared decision making.

Defining outcomes following congenital diaphragmatic hernia using standardised clinical assessment and management plan (SCAMP) methodology within the CDH EURO consortium.

Treatment modalities for neonates born with congenital diaphragmatic hernia (CDH) have greatly improved in recent times with a concomitant increase in survival. In 2008, CDH EURO consortium, a collaboration of a large volume of CDH centers in Western Europe, was established with a goal to standardize management and facilitate multicenter research. However, limited knowledge on long-term outcomes restricts the identification of optimal care pathways for CDH survivors in adolescence and adulthood. This review aimed to evaluate the current practice of long-term follow-up within the CDH EURO consortium centers, and to review the literature on long-term outcomes published from 2000 onward. Apart from having disease-specific morbidities, children with CDH are at risk for impaired neurodevelopmental problems and failure of educational attainments which may affect participation in society and the quality of life in later years. Thus, there is every reason to offer them long-term multidisciplinary follow-up programs. We discuss a proposed collaborative project using standardized clinical assessment and management plan (SCAMP) methodology to obtain uniform and standardized follow-up of CDH patients at an international level.

Mini Review of the Cost-Effectiveness of Unilateral Osseointegrated Implants in Adults: Possibly Cost-Effective for the Correct Indication.

An osseointegrated implant (e.g. bone-anchored hearing aid, BAHA) is a surgically implantable device for unilateral sensorineural and unilateral or bilateral conductive hearing loss in patients who otherwise cannot use or do not prefer a conventional air conduction hearing aid (ACHA). The specific indications for an osseointegrated implant are evolving and dependent upon the country or regulatory body overseeing the provision of these devices. However, there are general groups of patients who would be likely to benefit, one such group being patients with congenital aural atresia. Given the anatomical aberrancies with aural atresia, these subjects cannot wear ACHAs. Another group of patients who may benefit from an osseointegrated implant over an ACHA are patients with chronically draining otological infections. As the provision of an osseointegrated implant requires a surgical procedure, there are inherent direct and indirect costs associated with its use beyond those required for an ACHA. Consideration of outcomes and cost-effectiveness for the osseointegrated implant versus the ACHA is prudent prior to making policy decisions in a setting of limited health care resources. We performed a mini review on all available cost-effectiveness analyses of osseointegrated implants published in Medline. There are only 2 contemporary cost-effectiveness analyses published to date. There is limited quality of life data available for patients living with an osseointegrated implant. As a result, the cost-effectiveness of the osseointegrated implant, specifically the BAHA, compared to conventional hearing aid devices remains unclear. However, there are clear indications for the BAHA when a standard hearing aid cannot be used (e.g. chronic draining ear) or in single-sided severe-to-profound hearing loss with reasonable hearing in the contralateral ear. The BAHA should not be considered interchangeable with the ACHA with regard to cost-effectiveness, but rather considered as an effective option for the patient for the correct indication.

GDP Matters: Cost Effectiveness of Cochlear Implantation and Deaf Education in Sub-Saharan Africa.

HYPOTHESIS: Cochlear implantation and deaf education are cost effective in Sub-Saharan Africa. BACKGROUND: Cost effectiveness of pediatric cochlear implantation has been well established in developed countries but is unknown in low resource settings, where access to the technology has traditionally been limited. With incidence of severe-to-profound congenital sensorineural hearing loss 5 to 6 times higher in low/middle-income countries than the United States and Europe, developing cost-effective management strategies in these settings is critical. METHODS: Costs were obtained from experts in Nigeria, South Africa, Kenya, Rwanda, Uganda, and Malawi using known costs and published data, with estimation when necessary. A disability adjusted life years (DALY) model was applied using 3% discounting and 10-year length of analysis. Sensitivity analysis was performed to evaluate the effect of device cost, professional salaries, annual number of implants, and probability of device failure. Cost effectiveness was determined using the WHO standard of cost-effectiveness ratio/gross domestic product per capita (CER/GDP) less than 3. RESULTS: Cochlear implantation was cost effective in South Africa and Nigeria, with CER/GDP of 1.03 and 2.05, respectively. Deaf education was cost effective in all countries investigated, with CER/GDP ranging from 0.55 to 1.56. The most influential factor in the sensitivity analysis was device cost, with the cost-effective threshold reached in all countries using discounted device costs that varied directly with GDP. CONCLUSION: Cochlear implantation and deaf education are equally cost effective in lower-middle and upper-middle income economies of Nigeria and South Africa. Device cost may have greater impact in the emerging economies of Kenya, Uganda, Rwanda, and Malawi.

Cost Effectiveness of Childhood Cochlear Implantation and Deaf Education in Nicaragua: A Disability Adjusted Life Year Model.

INTRODUCTION: Cochlear implantation (CI) is a common intervention for severe-to-profound hearing loss in high-income countries, but is not commonly available to children in low resource environments. Owing in part to the device costs, CI has been assumed to be less economical than deaf education for low resource countries. The purpose of this study is to compare the cost effectiveness of the two interventions for children with severe-to-profound sensorineural hearing loss (SNHL) in a model using disability adjusted life years (DALYs). METHODS: Cost estimates were derived from published data, expert opinion, and known costs of services in Nicaragua. Individual costs and lifetime DALY estimates with a 3% discounting rate were applied to both two interventions. Sensitivity analysis was implemented to evaluate the effect on the discounted cost of five key components: implant cost, audiology salary, speech therapy salary, number of children implanted per year, and device failure probability. RESULTS: The costs per DALY averted are $5,898 and $5,529 for CI and deaf education, respectively. Using standards set by the WHO, both interventions are cost effective. Sensitivity analysis shows that when all costs set to maximum estimates, CI is still cost effective. CONCLUSION: Using a conservative DALY analysis, both CI and deaf education are cost-effective treatment alternatives for severe-to-profound SNHL. CI intervention costs are not only influenced by the initial surgery and device costs but also by rehabilitation costs and the lifetime maintenance, device replacement, and battery costs. The major CI cost differences in this low resource setting were increased initial training and infrastructure costs, but lower medical personnel and surgery costs.

Hyperbaric oxygen therapy (HBO2) as an adjunct in the treatment of idiopathic sudden sensorineural hearing loss (ISSHL)--a case report on two patients.

We report on two cases of idiopathic sensorineural sudden hearing loss (ISSHL) successfully treated at our center. The patients were referred to us by a neurotologist for adjunctive hyperbaric oxygen therapy and were treated using a standard 2.4 atmospheres absolute (atm abs) 90-minute protocol with two five- minute air breaks. During this period the referring physician administered injections of steroids at his offices. The outcome in both cases was positive and produced significantly improved scores in both speech reception and speech discrimination. In October 2011, the UHMS added ISSHL to its list of approved indications. Despite this and an increasing body of literature that supports the use of hyperbaric oxygen therapy as an adjunct in the treatment of ISSHL, it has not yet been accepted for reimbursement by CMS and, in our experience, commercial carriers. We believe it would be beneficial in developing the case for reimbursement in this indication if all facilities record and submit their results to a national database perhaps organized and managed by the UHMS.

Oxygénothérapie hyperbare systémique dans le traitement de la surdité soudaine neurosensorielle idiopathique / Systemic hyperbaric oxygen therapy in the treatment of idiopathic neurosensory idiopathic deafness

La présente note informative traite de l'utilisation de l'oxygénothérapie hyperbare systémique (OHBS) dans le traitement de la surdité soudaine neurosensorielle idiopathique (SSNI). Il s'agit d'une revue de la littérature qui porte principalement sur l'efficacité de ce traitement. Elle répond à une demande du service de médecine hyperbare du Centre de santé et de services sociaux Alphonse-Desjardins (Centre hospitalier affilié universitaire Hôtel-Dieu de Lévis). Le recours à l'OHBS pour contrer la surdité en question est basé sur l'hypothèse suivante : la SSNI découlerait de dommages à l'oreille interne qui seraient dus à un manque d'oxygène, et la pression qui règne au sein d'un caisson hyperbare contribuerait à pallier ce déficit en facilitant la diffusion de l'oxygène dans les structures anatomiques endommagées. Ainsi, diverses publications récentes ont été retenues comme sources de données scientifiques et il ressort de leur analyse que l'efficacité de l'OHBS dans le traitement de la SSNI demeure incertaine. En effet: les auteurs de la revue systématique retenue ont réitéré leurs conclusions antérieures selon lesquelles la signification clinique des résultats des études n'est pas claire; le nombre d'études originales qui rapportent des résultats négatifs est sensiblement égal à celui des études qui sont favorables à ce traitement; tant les études analysées dans la revue systématique que les études originales qui ont été retenues ont été réalisées en suivant des méthodologies hétérogènes et défaillantes ainsi qu'avec des échantillons de petite taille. Toutefois, l'efficacité de l'OHBS dans le traitement de la SSNI fait présentement l'objet de travaux de recherche et l'INESSS assura une veille des résultats qui en découleront.(AU)

This information brief deals with the use of systemic hyperbaric oxygen therapy (s-HBOT) for the treatment of idiopathic sudden sensorineural hearing loss (ISSHL). It is a literature review that focuses mainly on the efficacy of this treatment and is in reply to a request from the Hyperbaric Medicine Unit of the Centre de santé et de services sociaux Alphonse-Desjardins (Centre hospitalier affilié universitaire Hôtel-Dieu de Lévis). The use of s-HBOT to counter this type of hearing loss is based on the following hypothesis: ISSHL results from damage to the inner ear attributable to a lack of oxygen, and the pressure in a hyperbaric chamber helps reverse this deficit by promoting the diffusion of oxygen to the damaged anatomical structures. Various recent publications were selected as sources of scientific data. An analysis of these publications shows that the efficacy of s-HBOT in the treatment of ISSHL remains uncertain, for the following reasons: The authors of the systematic review that was selected repeated their previous conclusions, which state that the clinical significance of the study results is not clear; The number of original studies reporting unfavourable results is more or less the same as the number of studies in favour of this treatment; and The methodologies used in both the studies that were examined in the systematic review and the original studies that were selected were heterogeneous and flawed, and the sample sizes were small. However, research concerning the effecacy of S-HBOT for the treatment of ISSHL is ongoing and INESSS will be monitoring the resulting data.(AU)

A pilot cost-of-illness study on long-term complications/sequelae of AOM.

OBJECTIVES: Acute otitis media (AOM) commonly affects young children and occasionally results in serious complications/sequelae. This pilot cost-of-illness study aimed to assess the economic burden of long-term AOM complications/sequelae in Belgium, and to establish a thorough methodology for a larger study. METHODOLOGY: We retrospectively reviewed charts of patients aged 10-20 years with long-term complications/sequelae considered to be AOM-related, and > or = 8 years of follow-up. From a list of 215 eligible patients, we selected 25 patients representing each of seven categories of complications/sequelae. RESULTS: Included patients had a mean age of 12.9 years; nine had chronic suppurative otitis media with cholesteatoma; six sensorineural hearing loss; six chronic perforation of the tympanic membrane; and one each with conductive hearing loss, facial paralysis, neurological impairment after intracranial complications, and complications of surgery. During 8-15 years of follow-up, the most common complications were hearing loss, chronic otitis media (OM), and cholesteatoma. These generally occurred > 5 years after the first AOM event, although chronic OM occurred after a mean time of 3.3 years. Yearly public health care payer (PHCP) costs ranged from Euro 119 to Euro 7957 per patient, and were highest for patients with sensorineural hearing loss. Yearly costs to the patients ranged from Euro 7 to Euro 289 per patient, and were also highest for patients with sensorineural hearing loss. CONCLUSIONS: Although complications/sequelae of AOM are rare, they can result in substantial costs. The applied methodology should be feasible for a larger study, with some minor adjustments.

Outcome assessment alternatives for young children during the first 12 months after pediatric hearing-aid fittings.

OBJECTIVE: Perform longitudinal evaluations of young children during the first 12 months after initial hearing-aid fitting. Document evidence of early prelingual auditory development (EPLAD), identify factors that affect EPLAD, and define performance milestones that can guide best practices. DESIGN: Unblinded, prospective, within-subject, repeated-measures design. Audiological measures and measures of EPLAD were taken at baseline, 3, 6, and 12 months after hearing-aid fitting. STUDY SAMPLE: Subjects were 45 pediatric patients initially fitted with hearing aids between 1 and 5.5 years of age. Four groups were formed for analysis purposes based on severity of hearing loss (moderate-to-severe and profound) and initial fitting age (≤ 30 months and > 30 months). RESULTS: All groups exhibited statistically significant increases in EPLAD within six months of hearing-aid fitting, and those with profound losses exhibited further statistically significant improvement between six and 12 months. Similar EPLAD levels were reached at 12 months regardless of severity of hearing loss. The EPLAD trajectory is similar to that following early cochlear implantation. CONCLUSIONS: Measures of EPLAD provide a means of evaluating outcomes following early pediatric hearing-aid intervention, supplementing behavioral audiological measures.

The clinical effectiveness and safety of prophylactic retinal interventions to reduce the risk of retinal detachment and subsequent vision loss in adults and children with Stickler syndrome: a systematic review.

BACKGROUND: Stickler syndrome, also known as hereditary progressive arthro-ophthalmopathy, is an inherited progressive disorder of the collagen connective tissues. Manifestations include short-sightedness, cataracts, retinal problems leading to retinal detachment and possible blindness. This is principally the case among individuals with type 1 Stickler Syndrome. It is the most commonly identified inherited cause of retinal detachment in childhood. However, there is no consensus regarding best practice and no current guidelines on prophylactic interventions for this population. OBJECTIVES: The aim of this systematic review was to assess the evidence for the clinical effectiveness and safety of primary prophylactic interventions for the prevention of retinal detachment in previously untreated eyes without retinal detachment in patients with Stickler syndrome. The primary outcome of interest was retinal detachment post prophylaxis. DATA SOURCES: A systematic search was made of 11 databases of published and unpublished literature, which included MEDLINE, MEDLINE In-Process & Other Non-Indexed Citations, EMBASE, the Cumulative Index to Nursing and Allied Health Literature and The Cochrane Library. There was no restriction by language or date. The references of all included studies were checked for further relevant citations and authors of studies with potentially relevant data were also contacted. REVIEW METHODS: Two reviewers double-screened all titles and abstracts of the citations retrieved by the search to identify studies that satisfied the inclusion criteria. Both reviewers also independently extracted and quality assessed all included studies. A narrative synthesis was performed. RESULTS: The literature search identified 1444 unique citations, of which four studies satisfied the inclusion criteria. The two principal studies were both retrospective cohort studies with control groups in populations with type 1 Stickler syndrome. One study evaluated 360° cryotherapy (n = 204) and the other focal or circumferential laser treatment (n = 22). Both studies reported a statistically significant difference in the rate of retinal detachment per eye between the groups receiving prophylaxis and the controls. However, both studies were subject to a high risk of bias. The results of the two supporting studies of Wagner-Stickler patients were either relatively inconsistent or unreliable. No study reported any major or long-term complications associated with the interventions. Despite the weaknesses of the evidence, the rate of retinal detachment in the intervention groups, especially the cryotherapy group, was lower than the rate either experienced in the study control groups or reported in other studies of untreated Stickler syndrome populations not exposed to prophylaxis. CONCLUSIONS: Only 360° cryotherapy and focal and circumferential laser treatment have been evaluated for the type 1 Stickler syndrome population, and then only by a single retrospective, controlled, cohort study in each case. Both of these studies report a significant difference between intervention and control groups (principally no treatment) and no major or long-term side effects or complications. However, there is a high risk of bias within these two studies, so the relative effectiveness of either intervention is uncertain. FUTURE WORK: A service priority is to determine reliably the prevalence of Stickler syndrome, i.e. how many individuals have type 1 or type 2 Stickler syndrome, and their risk of retinal detachment and subsequent blindness. A non-randomised, prospective cohort comparison study, in which eligible participants are treated, followed-up and analysed in one of three study arms, for no treatment, laser therapy or cryotherapy, would potentially offer further certainty in terms of the relative efficacy of both prophylaxis versus no prophylaxis and cryotherapy versus laser therapy than is possible with the currently available data. Alternatively, continued follow-up and analysis of existing study data, and data collection from relevant sample populations, are required to assess the long-term risks of blindness, retinal detachment and prophylaxis. FUNDING: This study was funded by the National Institute for Health Research Health Technology Assessment programme.

Prognosis of patients with idiopathic sudden hearing loss: role of vestibular assessment.

OBJECTIVE: To evaluate the correlation between caloric and vestibular evoked myogenic potential test results, initial audiogram data, and early hearing recovery, in patients with idiopathic sudden hearing loss. MATERIALS AND METHODS: One hundred and four patients with unilateral idiopathic sudden hearing loss underwent complete neurotological evaluation. Results for vestibular evoked myogenic potential and caloric testing were compared with patients' initial and final audiograms. RESULTS: Overall, abnormal vestibular evoked myogenic potential responses occurred in 28.8 per cent of patients, whereas abnormal caloric test results occurred in 50 per cent. A statistically significant relationship was found between the type of inner ear lesion and the incidence of profound hearing loss. Moreover, a negative correlation was found between the extent of the inner ear lesion and the likelihood of early recovery. CONCLUSION: In patients with idiopathic sudden hearing loss, the extent of the inner ear lesion tends to correlate with the severity of cochlear damage. Vestibular assessment may be valuable in predicting the final outcome.

Hearing assessment in high-risk congenital diaphragmatic hernia survivors.

OBJECTIVE: To report results of audiometric evaluations in high-risk congenital diaphragmatic hernia survivors and their exposure to audiological risk factors (mechanical ventilation, high frequency oscillation, aminoglycoside therapy and neuromuscular blocking agents). DESIGN: All newborns with high-risk congenital diaphragmatic hernia born between January 2003 and June 2009 were treated consecutively at the Neonatal Intensive Care Unit, Pediatric Hospital, University of Padova. Thirty-two survived and 26 of them underwent formal audiological evaluation (tonal and speech audiometry, otoacoustic emission, and immitance measurements) and follow up. RESULTS: Twenty-one children had normal hearing; 4 had conductive hearing loss, which was successfully treated; and 1 had severe sensorineural hearing loss and suffers from Turner syndrome. CONCLUSIONS: Our series revealed a lower prevalence of sensorineural hearing loss in high-risk congenital diaphragmatic hernia survivors than in other studies, suggesting that the association between hearing loss and congenital diaphragmatic hernia has yet to be accurately defined and fully elucidated.

Cost-effectiveness of implantable middle ear hearing devices.

OBJECTIVE: To assess the relation between cost and effectiveness of implantable middle ear hearing devices in patients with pure sensorineural hearing loss. DESIGN: Literature review. RESULTS: Four studies were identified that described the effect of middle ear implantation on quality of life in groups of at least 20 patients. Several different quality of life questionnaires were used. CONCLUSIONS: Our review demonstrated that middle ear implantation is a cost-effective health care intervention in patients with sensorineural hearing loss who suffered an additional therapy-resistant chronic external otitis.

Topics in pediatric head and neck imaging: self-assessment module.

OBJECTIVE: The educational objectives for this self-assessment module are for the participant to exercise, self-assess, and improve his or her understanding of radiology in pediatric congenital sensorineural hearing loss, posterior plagiocephaly, and midline nasofrontal mass. CONCLUSION: The activities in these articles guide the participant to understanding the key concepts of radiology in pediatric congenital sensorineural hearing loss, posterior plagiocephaly, and midline nasofrontal mass.