Anatomical and audiological considerations in branchiootorenal syndrome: A systematic review.

Objective: Establish anatomical considerations, audiological outcomes, and optimal management in patients with branchiootic/branchiootorenal syndrome (BO/BOR). Methods: Databases reviewed: Medline, Pubmed, Embase, Web of Science, Cochrane Collection, and ClinicalTrials.gov. Clinical or radiological studies of patients with BOR syndrome describing either the audiological profile or anatomical changes were included. Articles in which BOR syndrome was associated with other syndromes, and those that were focused only on general and genetic aspects of BOR syndrome were excluded. Articles were assessed using Oxford Centre for Evidence-Based Medicine (OCEBM) grading system and the Brazzelli risk of bias tool for nonrandomized studies. Results: Searches identified 379 articles. Of these, 64 studies met the inclusion criteria, reporting outcomes in 482 patients from at least 95 families. In 308 patients, hearing loss was categorized as sensorineural (29%), conductive (20%), and mixed (51%). Hearing outcomes were variable in terms of onset, pattern, and severity; ranging from mild to profound deafness. One hundred sixty-nine patients presented with inner ear anomalies, 145 had middle, and 151 had external ear abnormalities. In 44 studies, 58 ear operations were described. Mixed outcomes were reported in patients managed with hearing aids or middle ear surgery; however, successful cochlear implantation was described in all five cases. Conclusion: The anatomical and audiological profiles of patients with BO/BOR are variable. A range of surgical procedures were described, however lacked objective outcome measures. Given the range of anatomical variants, management decisions should be made on an individual basis including full audiological and radiological assessment. Level of evidence: NA.

Cochlear Implantation in Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-Like Episodes Syndrome: Case Presentation.

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome is a multisystem, progressive neurodegenerative condition, and the most common mitochondrial cytopathy. While not a primary characteristic, sensorineural hearing loss is a common additional symptom reported in up to 78% of cases. This article presents 2 cases of cochlear implantation in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome. Both cases demonstrated significantly improved speech recognition, with results significantly better than previous case reports. Cochlear implants are an appropriate treatment for severe-profound hearing loss in mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome. While anesthetic risks and cognitive skills need to be taken into consideration, routine programming and rehabilitation pathways may be appropriate for this cohort.

Acute otitis externa: Consensus definition, diagnostic criteria and core outcome set development.

OBJECTIVE: Evidence for the management of acute otitis externa (AOE) is limited, with unclear diagnostic criteria and variably reported outcome measures that may not reflect key stakeholder priorities. We aimed to develop 1) a definition, 2) diagnostic criteria and 3) a core outcome set (COS) for AOE. STUDY DESIGN: COS development according to Core Outcome Measures in Effectiveness Trials (COMET) methodology and parallel consensus selection of diagnostic criteria/definition. SETTING: Stakeholders from the United Kingdom. SUBJECTS AND METHODS: Comprehensive literature review identified candidate items for the COS, definition and diagnostic criteria. Nine individuals with past AOE generated further patient-centred candidate items. Candidate items were rated for importance by patient and professional (ENT doctors, general practitioners, microbiologists, nurses, audiologists) stakeholders in a three-round online Delphi exercise. Consensus items were grouped to form the COS, diagnostic criteria, and definition. RESULTS: Candidate COS items from patients (n = 28) and literature (n = 25) were deduplicated and amalgamated to a final candidate list (n = 46). Patients emphasised quality-of-life and the impact on daily activities/work. Via the Delphi process, stakeholders agreed on 31 candidate items. The final COS covered six outcomes: pain; disease severity; impact on quality-of-life and daily activities; patient satisfaction; treatment-related outcome; and microbiology. 14 candidate diagnostic criteria were identified, 8 reaching inclusion consensus. The final definition for AOE was 'diffuse inflammation of the ear canal skin of less than 6 weeks duration'. CONCLUSION: The development and adoption of a consensus definition, diagnostic criteria and a COS will help to standardise future research in AOE, facilitating meta-analysis. Consulting former patients throughout development highlighted deficiencies in the outcomes adopted previously, in particular concerning the impact of AOE on daily life.

Outcomes of Cochlear Implantation in Patients with Superficial Siderosis: A Systematic Review and Narrative Synthesis.

This study aimed to establish outcomes following cochlear implantation (CI) in patients with superficial siderosis (SS). MEDLINE, Embase, Web of Science, Cochrane, and ClinicalTrials.gov databases were searched for this systematic review. No limits were placed on the language or the year of publication. The review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. Of a total of 46 studies, 19 studies met the inclusion criteria reporting outcomes in 38 patients. Of the 44 implants, 23 implants (52.27%) had good hearing outcomes at the last follow-up, 9 (20.45%) were initially beneficial for the patient, but then the performance deteriorated (4 of which were re-implanted), and 12 (27.27%) were not beneficial for the patient. All studies were classified as grade 4 studies using the Oxford Centre for Evidence-Based Medicine (OCEBM) grading system, being retrospective in nature and consisting of case reports and noncontrolled case series with a small number of patients. Of the 44 implants, 32 (72.73%) showed improved hearing outcomes following CI for SS at some point in time, of which 23 (52.2%) implants showed sustained benefit at the last follow-up (average follow-up time was 21.97 months). It is difficult to predict the longevity of benefit owing to the progressive nature of the disease or the patients in whom it may be beneficial, as the preoperative investigations inadequately predict benefit. Preimplantation and postimplantation counseling with the patient and their family regarding the potential limited benefit and eventual parallel decline with the neurological disease are crucial, along with thorough clinical investigations.

The Impact of Single-Sided Deafness upon Music Appreciation.

BACKGROUND: Many of the world's population have hearing loss in one ear; current statistics indicate that up to 10% of the population may be affected. Although the detrimental impact of bilateral hearing loss, hearing aids, and cochlear implants upon music appreciation is well recognized, studies on the influence of single-sided deafness (SSD) are sparse. PURPOSE: We sought to investigate whether a single-sided hearing loss can cause problems with music appreciation, despite normal hearing in the other ear. RESEARCH DESIGN: A tailored questionnaire was used to investigate music appreciation for those with SSD. STUDY SAMPLE: We performed a retrospective survey of a population of 51 adults from a University Hospital Audiology Department SSD clinic. SSD was predominantly adult-onset sensorineural hearing loss, caused by a variety of etiologies. DATA ANALYSIS: Analyses were performed to assess for statistical differences between groups, for example, comparing music appreciation before and after the onset of SSD, or before and after receiving hearing aid(s). RESULTS: Results demonstrated that a proportion of the population experienced significant changes to the way music sounded; music was found to sound more unnatural (75%), unpleasant (71%), and indistinct (81%) than before hearing loss. Music was reported to lack the perceptual qualities of stereo sound, and to be confounded by distortion effects and tinnitus. Such changes manifested in an altered music appreciation, with 44% of participants listening to music less often, 71% of participants enjoying music less, and 46% of participants reporting that music played a lesser role in their lives than pre-SSD. Negative effects surrounding social occasions with music were revealed, along with a strong preference for limiting background music. Hearing aids were not found to significantly ameliorate these effects. CONCLUSIONS: Results could be explained in part through considerations of psychoacoustic changes intrinsic to an asymmetric hearing loss and impaired auditory scene analysis. Given the prevalence of music and its capacity to influence an individual's well-being, results here present strong indications that the potential effects of SSD on music appreciation should be considered in a clinical context; an investigation into relevant rehabilitation techniques may prove valuable.

Cochlear implants in the management of hearing loss in Neurofibromatosis Type 2.

OBJECTIVE: Review of cochlear implant (CI) outcomes in patients with Neurofibromatosis Type 2 (NF2), implanted in the presence of an ipsilateral vestibular schwannoma (VS). Hearing restoration was combined in some cases with a Bevacizumab regime. METHOD: Retrospective review of 12 patients, managed over the period 2009-2016, at a tertiary referral multidisciplinary NF2 clinic. The patients are grouped by hearing outcomes to explore likely protective factors, and to generate a proposed decision-making tool for the selection of either CI or Auditory Brainstem Implant (ABI). RESULTS: Four of the 12 patients achieved speech discrimination without lip-reading. In these individuals there is reason to think that the mechanism of their hearing loss was cochlear dysfunction. A further four patients received benefit to lip-reading and awareness of environmental sound. For such patients their hearing loss may have been due to both cochlear and neural dysfunction. Two patients gained access to environmental sound only from their CI. Two patients derived no benefit from their CIs, which were subsequently explanted. Both these latter patients had had prior ipsilateral tumour surgery, one just before the CI insertion. CONCLUSION: Cochlear implantation can lead to open set speech discrimination in patients with NF2 in the presence of a stable VS. Use of promontory stimulation and intraoperative electrically evoked auditory brainstem response testing, along with case history, can inform the decision whether to implant an ABI or CI.

Ototoxicity (cochleotoxicity) classifications: A review.

OBJECTIVE: Drug-mediated ototoxicity, specifically cochleotoxicity, is a concern for patients receiving medications for the treatment of serious illness. A number of classification schemes exist, most of which are based on pure-tone audiometry, in order to assist non-audiological/non-otological specialists in the identification and monitoring of iatrogenic hearing loss. This review identifies the primary classification systems used in cochleototoxicity monitoring. By bringing together classifications published in discipline-specific literature, the paper aims to increase awareness of their relative strengths and limitations in the assessment and monitoring of ototoxic hearing loss and to indicate how future classification systems may improve upon the status-quo. DESIGN: Literature review. STUDY SAMPLE: PubMed identified 4878 articles containing the search term ototox*. RESULTS: A systematic search identified 13 key classification systems. Cochleotoxicity classification systems can be divided into those which focus on hearing change from a baseline audiogram and those that focus on the functional impact of the hearing loss. CONCLUSIONS: Common weaknesses of these grading scales included a lack of sensitivity to small adverse changes in hearing thresholds, a lack of high-frequency audiometry (>8 kHz), and lack of indication of which changes are likely to be clinically significant for communication and quality of life.

Treatment of superficial siderosis with iron chelation therapy.

Superficial siderosis is caused by recurrent haemorrhage in the subarachnoid space leading to haemosiderin deposition. It typically causes the triad of ataxia, deafness and myelopathy. We report a patient who developed superficial siderosis following neurosurgery for syringomyelia and who had an improvement in his hearing and mobility following treatment with a new iron chelation therapy that can penetrate the blood-brain barrier. It provides an intriguing insight into a therapy that could potentially modify the course of this rare neurodegenerative disorder. Further studies are required to assess the clinical efficacy of deferiprone in superficial siderosis.