Clinical relevance of Scedosporium spp. and Exophiala dermatitidis in patients with cystic fibrosis: A nationwide study.

An increased prevalence of various filamentous fungi in sputum samples of patients with cystic fibrosis (CF) has been reported. The clinical significance, however, is mostly unclear. The aim of this study was to investigate the clinical relevance of Scedosporium spp. and Exophiala dermatitidis from sputum samples of patients with CF in the Netherlands. In this cross-sectional study, all CF patients of the Dutch national CF registry who were treated at five of the seven recognized CF centers during a 3-year period were included. We linked clinical data of the national CF registry with the national Dutch filamentous fungal database. We investigated the association between clinical characteristics and a positive sputum sample for Scedosporium spp. and E. dermatitidis, using logistic regression. Positive cultures for fungi were obtained from 3787 sputum samples from 699 of the 1312 patients with CF. Scedosporium spp. was associated with severe genotype, CF-related diabetes, several microorganisms, and inhaled antibiotics. E. dermatitidis was associated with older age, female sex, and Aspergillus spp. CF patients with and without Scedosporium spp. or E. dermatitidis seemed comparable in body mass index and lung function. This study suggests that Scedosporium spp. and E. dermatitidis are probably no major pathogens in CF patients in the Netherlands. Greater understanding of epidemiologic trends, risk factors, and pathogenicity of filamentous fungi in the respiratory tracts of patients with CF is needed.

The etiological evaluation of sensorineural hearing loss in children.

This study aims to evaluate the etiology of pediatric sensorineural hearing loss (SNHL). A total of 423 children with SNHL were evaluated, with the focus on the determination of causative genetic and acquired etiologies of uni- and bilateral SNHL in relation to age at diagnosis and severity of the hearing loss. We found that a stepwise diagnostic approach comprising of imaging, genetic, and/or pediatric evaluation identified a cause for SNHL in 67% of the children. The most common causative finding in children with bilateral SNHL was causative gene variants (26%), and in children with unilateral SNHL, a structural anomaly of the temporal bone (27%). The probability of finding an etiologic diagnosis is significantly higher in children under the age of 1 year and children with profound SNHL.Conclusions: With our stepwise diagnostic approach, we found a diagnostic yield of 67%. Bilateral SNHL often has a genetic cause, whereas in unilateral SNHL structural abnormalities of the labyrinth are the dominant etiologic factor. The diagnostic yield is associated with the age at detection and severity of hearing loss: the highest proportion of causative abnormalities is found in children with a young age at detection or a profound hearing loss. What is Known: • Congenital sensorineural hearing loss is one of the most common congenital disorders • Determination of the cause is important for adequate management and prognosis and may include radiology, serology, and DNA analysis What is New: • Using a stepwise diagnostic approach, causative abnormalities are found in 67% both in uni- and bilateral SNHL, with the highest diagnostic yield in very young children and those suffering from profound hearing loss • Bilateral SNHL often has a genetic cause, whereas in unilateral SNHL structural abnormalities of the labyrinth are the dominant etiologic factor.

The Otology Questionnaire Amsterdam: a generic patient reported outcome measure about the severity and impact of ear complaints. A cross-sectional study on the development of this questionnaire.

OBJECTIVE: Development of the 'Otology Questionnaire Amsterdam' (OQUA), a patient reported outcome measure (PROM), measuring the severity and impact of ear complaints of patients visiting an ENT surgeon. DESIGN: Multicenter, cross-sectional study. Phase 1: qualitative research. In-depth interviews (N = 16) to identify relevant types of ear complaints and to formulate items. Pilot study of the first and second draft of the OQUA (N = 32, N = 39). Phase 2: quantitative research. Field-testing of the OQUA (N = 352). Item reduction based on inter-item correlation, factor analysis and expert opinion. SETTING: Two secondary and two tertiary ENT clinics. PARTICIPANTS: Patients over the age of sixteen visiting an ENT surgeon with an ear complaint. MAIN OUTCOME MEASURES: Phase 1: meaning units and frequency of selected descriptions. Phase 2: inter-item correlation, factor loading and Cronbach's Alpha (α). RESULTS: Phase 1: eight relevant types of ear complaints were identified: earache, pressure in ear, hearing loss, tinnitus, otorrhoea, itch, dizziness and loss of taste. Phase 2: factor analysis generated a factor 'impact' (α = 0.913). The current version of the OQUA consists of 34 items, covers eight types of ear complaints and consists of two constructs: complaints and impact. CONCLUSION: The OQUA is a generic, otologic PROM designed to evaluate the severity of ear complaints and their impact on patients lives.

Validity, reliability and discriminative capacity of an electronic quality of life instrument (Pelican) for childhood asthma in the Netherlands.

PURPOSE: To assess psychometric properties of the Pelican instrument, an online Dutch self-administered Quality of Life instrument for childhood asthma for scientific and clinical use. METHODS: A cohort study was done in two asthma populations and healthy children. One asthma population had assessment at start, 4 and 8 weeks. The other asthma population and healthy children had one assessment. All children were aged 6-12 years. Children completed the Pelican instrument, Paediatric Asthma Quality of Life Questionnaire, feeling thermometer and Childhood Asthma Control Test. Lung function and fraction exhaled nitric oxide were measured. Parents completed Functional Status II, Asthma Control Questionnaire, Childhood Asthma Control Test questionnaires and symptom diaries. We assessed interpretability, structural validity, internal consistency, reliability, construct and discriminative validity of the Pelican instrument. RESULTS: Eighty-five asthmatic (mean age 8.5 years) and 49 healthy children (mean age 8.4 years) participated. The Pelican instrument has 5 domains with 21 items after factor analysis. Internal consistency was 0.89 (CI 0.85-0.92), domain reliability showed Cronbach's α's from 0.64 to 0.76 and item-to-scale correlations from 0.61 to 0.81. Test-retest reliability was confirmed ICC = 0.88 (CI 0.79-0.93). Construct validity was demonstrated by significant moderate correlations with other relevant asthma outcomes like PAQLQ (r = -0.59, p < 0.01). Discriminative capacity between controlled or uncontrolled asthma (t = 3.20, p < 0.01, Δ = 0.64) and asthma versus healthy subjects (t = 6.31, p < 0.01, Δ = 0.94) was found. CONCLUSIONS: The psychometric properties of the Pelican instrument were acceptable in Dutch paediatric asthma patients between 6 and 12 years old.

Barriers to and enablers of the use of the Otology Questionnaire Amsterdam in clinical practice-a qualitative post-implementation study.

BACKGROUND: The Otology Questionnaire Amsterdam (OQUA) is developed to evaluate multiple ear complaints and their impact on patients' daily lives. The current clinical use of this questionnaire is below the potential utilization. AIM: To identify the barriers and enablers of using the OQUA as perceived by ENT surgeons and patients and provide recommendations for an implementation strategy. METHODS: Prospective and qualitative analysis was performed using focus groups and interviews with ENT professionals (n = 15) and patients (n = 25) with ear complaints of one tertiary referral hospital and two regional hospitals. Barriers and enablers were identified and classified by using the Capability-Opportunity-Motivation-Behavior model and the Theoretical Domains Framework. Suggestions for an implementation strategy will be made accordingly. RESULTS: ENT professionals' barriers included lack of knowledge and skills to use the OQUA, inadequate technological support and perceived time constraints during consultation, uncertainty about the clinical relevance and lack of feedback on the outcomes of the OQUA. Enablers included beneficial consequences of the OQUA for the professional, organization and science. Patients' barriers included lack of knowledge about the objective and usefulness of the OQUA, perceived burden, difficulties in completing the questionnaire and insufficient feedback during consultation. Patient enablers included beliefs about beneficial consequences of the OQUA for the patient, health care and society. Suggested interventions involved education, training, environmental restructuring and incentivisation. CONCLUSION: Based on the findings, we propose an implementation strategy should focus on education and training about the objective, outcomes and relevance of the OQUA, environmental restructuring regarding the optimal use of the OQUA, and incentivisation with feedback on the valuable outcomes of the OQUA for the patient, professional and healthcare. Future research is needed to determine the feasibility of the implementation strategy.

Comparing Global Initiative for Asthma (GINA) criteria with the Childhood Asthma Control Test (C-ACT) and Asthma Control Test (ACT).

Several tools are useful in detecting uncontrolled asthma in children. The aim of this study was to compare Global Initiative for Asthma (GINA) guidelines with the Childhood Asthma Control Test (C-ACT) and the Asthma Control Test (ACT) in detecting uncontrolled asthma in children. 145 children with asthma filled in a web-based daily diary card for 4 weeks on symptoms, use of rescue medication and limitations of activities, followed by either the C-ACT or ACT. For predicting uncontrolled asthma, score cut-off points of 19 were used for C-ACT and ACT. According to GINA guidelines, asthma was uncontrolled in 71 (51%) children and completely controlled in 19 (14%) children. The area under the curve in the receiver operating characteristic curves for C-ACT and ACT versus GINA guidelines were 0.89 and 0.92, respectively. Cut-off points of 19 for C-ACT and ACT resulted in a sensitivity of 33% and 66% in predicting uncontrolled asthma, respectively. C-ACT and ACT correlate well with GINA criteria in predicting uncontrolled asthma, but commonly used cut-off points for C-ACT and ACT seem to underestimate the proportion of children with uncontrolled asthma as defined by GINA.

Improved treatment response to dornase alfa in cystic fibrosis patients using controlled inhalation.

Better treatment of obstructed small airways is needed in cystic fibrosis. This study investigated whether efficient deposition of dornase alfa in the small airways improves small airway obstruction. In a multicentre, double-blind, randomised controlled clinical trial, cystic fibrosis patients on maintenance treatment with 2.5 mL dornase alfa once daily were switched to a smart nebuliser and randomised to small airway deposition (n = 24) or large airway deposition (n = 25) for 4 weeks. The primary outcome parameter was forced expiratory flow at 75% of forced vital capacity (FEF(75%)). FEF(75%) increased significantly by 0.7 sd (5.2% predicted) in the large airways group and 1.2 sd (8.8% pred) in the small airways group. Intention-to-treat analysis did not show a significant difference in treatment effect between groups. Per-protocol analysis, excluding patients not completing the trial or with adherence <70%, showed a trend (p = 0.06) in FEF(75%) Z-score and a significant difference (p = 0.04) between groups in absolute FEF(75%) (L · s(-1)) favouring small airway deposition. Improved delivery of dornase alfa using a smart nebuliser that aids patients in correct inhalation technique resulted in significant improvement of FEF(75%) in children with stable cystic fibrosis. Adherent children showed a larger treatment response for small airway deposition.

Contralateral hearing loss in children with a unilateral enlarged vestibular aqueduct.

OBJECTIVE: To evaluate the long-term ipsi- and contralateral hearing of patients with a unilateral enlarged vestibular aqueduct (EVA). STUDY DESIGN: Multicenter retrospective cohort study. SETTING: Three tertiary otology and audiology referral centers. PATIENTS AND DIAGNOSTIC INTERVENTIONS: A total of 34 children with a unilateral enlarged vestibular aqueduct as identified on CT and/or MR imaging were evaluated with pure tone and speech perception audiometry. MEAN OUTCOME MEASURES: Radiologic measurements of the vestibular aqueduct, ipsi- and contralateral hearing loss, ipsi- and contralateral hearing loss progression over time and DNA test results. RESULTS: All patients in this cohort with unilateral EVA presented with hearing loss. Hearing loss was progressive in 38% of the ipsilateral ears. In 29% of the children, hearing loss was also found in the contralateral ear without EVA. In 90%, the contralateral hearing was stable, with a mean follow up of 4.2 years. We found a significant correlation between the severity of the hearing loss and the size of the EVA. A genetic diagnosis associated with EVA and/or SNHL was found in only 7%. CONCLUSION: About a third of the children with unilateral EVA are at risk of developing hearing loss in the contralateral ear. This indicates that at least in some patients with a unilateral EVA, a bilateral pathogenic process underlies the hearing loss, in contrary to what the imaging results suggest. These findings are important for counseling of EVA patients and their parents and have implications for follow up.

Reference ranges for interrupter resistance technique: the Asthma UK Initiative.

Measuring interrupter resistance (R(int)) is an increasingly popular lung function technique and especially suitable for preschool children because it is simple, quick and requires only passive cooperation. A European Respiratory Society (ERS)/American Thoracic Society (ATS) Task Force recently published empirical recommendations related to procedures, limitations and interpretation of the technique. However, for valid interpretation, high-quality reference equations are required and these have been lacking. The aim of the present study was to collate R(int) data from healthy children in order to produce more robust reference equations. A further aim was to examine the influence of methodological differences on predicted R(int) values. R(int) data from healthy children were collected from published and unpublished sources. Reference equations for expiratory and inspiratory R(int) were developed using the LMS (lambda, mu, sigma) method. Data from 1,090 children (51% males) aged 3-13 yrs were collated to construct sex-specific reference equations for expiratory R(int) and data from 629 children (51% males) were collated for inspiratory R(int). Height was the best independent predictor of both expiratory and inspiratory R(int). Differences between centres were clinically irrelevant, and differences between ethnic groups could not be examined. The availability of a large and generalisable sample and the use of modern statistical techniques enabled the development of more appropriate reference equations for R(int) in young children.

Respiratory medicines for children: current evidence, unlicensed use and research priorities.

This European Respiratory Society task force has reviewed the evidence for paediatric medicines in respiratory disease occurring in adults and children. We describe off-licence use, research priorities and ongoing studies. Off-licence and off-label prescribing in children is widespread and potentially harmful. Research areas in asthma include novel formulations and regimens, and individualised prescribing. In cystic fibrosis, future studies will focus on screened infants and robust outcome measures are needed. Other areas include new enzyme and antibiotic formulations and the basic defect. Research into pneumonia should include evaluation of new antibacterials and regimens, rapid diagnostic tests and, in pleural infection, antibiotic penetration, fibrinolytics and surveillance. In uncommon conditions, such as primary ciliary dyskinesia, congenital pulmonary abnormalities or neuromuscular disorders, drugs indicated for other conditions (e.g. dornase alfa) are commonly used and trials are needed. In neuromuscular disorders, the beta-agonists may enhance muscle strength and are in need of evaluation. Studies of antibiotic prophylaxis, immunoglobulin and antifungal drugs are needed in immune deficiency. We hope that this summary of the evidence for respiratory medicines in children, highlighting gaps and research priorities, will be useful for the pharmaceutical industry, the paediatric committee of the European Medicines Agency, academic investigators and the lay public.

A case promoting use of ultrasound-guided sampling techniques to correctly diagnose MDR-TB in children.

A paediatric case of multidrug-resistant tuberculosis in which endo-oesophageal ultrasound-guided fine-needle aspiration using an endobronchial ultrasound-guided bronchoscope was used to collect a sample for microbial analyses is presented. In our experience, ultrasound-guided sampling techniques, both endo-oesophageal and endobronchial, can be safely used for the diagnosis of paediatric intrathoracic tuberculous lymphadenopathy in children aged 3 years. Interventional pulmonologists with experience in using these techniques should be part of the multidisciplinary team treating these patients.

Recombinant human deoxyribonuclease for the treatment of acute asthma in children.

BACKGROUND: Airway obstruction in acute asthma is the result of airway smooth muscle contraction, inflammation and mucus plugging. Case reports suggest that mucolytic therapy might be beneficial in acute asthma. The aim of this study was to determine the efficacy of the mucolytic drug recombinant human deoxyribonuclease (rhDNase) in addition to standard treatment at the emergency department in children with an asthma exacerbation. METHODS: In a multicentre randomised double-blind controlled clinical trial, 121 children brought to the emergency room for a moderate to severe asthma exacerbation were randomly assigned to receive either a single dose of 5 mg nebulised rhDNase or placebo following the second dose of bronchodilators. An asthma score (scale 5-15) was assessed at baseline and at 1, 2, 6, 12 and 24 h. The primary outcome variable was the asthma score 1 h after the study medication. RESULTS: One hour after the study medication the asthma score in the rhDNase group showed an adjusted mean decrease from baseline of 1.0 (95% CI 0.5 to 1.6) points compared with 0.7 (95% CI 0.3 to 1.2) points in the placebo group (mean difference 0.4 (95% CI -0.2 to 1.0) points; p = 0.23). The asthma score over the study period of 24 h also did not differ significantly between the rhDNase and placebo group (mean difference 0.2 (95% CI -0.3 to 0.7) points, p = 0.40). The duration of oxygen supplementation and number of bronchodilator treatments in the first 24 h were similar in both groups. CONCLUSION: Adding a single dose of nebulised rhDNase to standard treatment in the emergency room has no beneficial effects in children with moderate to severe acute asthma.

Definition, assessment and treatment of wheezing disorders in preschool children: an evidence-based approach.

There is poor agreement on definitions of different phenotypes of preschool wheezing disorders. The present Task Force proposes to use the terms episodic (viral) wheeze to describe children who wheeze intermittently and are well between episodes, and multiple-trigger wheeze for children who wheeze both during and outside discrete episodes. Investigations are only needed when in doubt about the diagnosis. Based on the limited evidence available, inhaled short-acting beta(2)-agonists by metered-dose inhaler/spacer combination are recommended for symptomatic relief. Educating parents regarding causative factors and treatment is useful. Exposure to tobacco smoke should be avoided; allergen avoidance may be considered when sensitisation has been established. Maintenance treatment with inhaled corticosteroids is recommended for multiple-trigger wheeze; benefits are often small. Montelukast is recommended for the treatment of episodic (viral) wheeze and can be started when symptoms of a viral cold develop. Given the large overlap in phenotypes, and the fact that patients can move from one phenotype to another, inhaled corticosteroids and montelukast may be considered on a trial basis in almost any preschool child with recurrent wheeze, but should be discontinued if there is no clear clinical benefit. Large well-designed randomised controlled trials with clear descriptions of patients are needed to improve the present recommendations on the treatment of these common syndromes.

Evaluation of the outcome of CT and MR imaging in pediatric patients with bilateral sensorineural hearing loss.

OBJECTIVE: To evaluate the clinically relevant abnormalities as visualized on CT and MR imaging in children with symmetric and asymmetric bilateral sensorineural hearing loss (SNHL), in relation to age and the severity of hearing loss. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral otology and audiology center. PATIENTS AND DIAGNOSTIC INTERVENTIONS: From January 2006 until January 2016, a total of 207 children diagnosed with symmetric and asymmetric bilateral SNHL were included. They underwent CT and/or MR imaging for the evaluation of the etiology of their hearing loss. MAIN OUTCOME MEASURES: Radiologic abnormalities associated with SNHL. RESULTS: 302 scans were performed in 207 children (median age of 0.8 years old) with bilateral SNHL. The most frequently identified cause of bilateral SNHL was a malformation of the labyrinth. The combined diagnostic yield of CT and MR imaging was 32%. The diagnostic yield of MR (34%) was considerably higher than that of CT (20%). We found a higher rate of abnormalities in children with profound hearing loss (41%) compared to milder hearing loss (8-29%), and in asymmetric SNHL (52%) compared to symmetric SNHL (30%). CONCLUSION: Imaging is essential in the etiologic evaluation of children with bilateral SNHL. The highest diagnostic yield is found in children with bilateral asymmetric SNHL or profound SNHL. Based on our findings, MR is the primary imaging modality of choice in the etiological evaluation of children with bilateral SNHL because of its high diagnostic yield.

[Postmeningitis deafness in young children: action warranted before obliteration of the cochlea]. / Doofheid na bacteriële meningitis bij jonge kinderen: ingrijpen vóór de cochlea is geoblitereerd.

Meningitis may cause inflammation of the cochlea, which may result in deafness and also in rapid obliteration ofthe cochlea with fibrous tissue or even ossification, conditions that obstruct the placement ofa cochlear implant. In the first of two cases of postmeningitis deafness, in a boy aged 6 months and a girl aged 1 year and 9 months, ignorance about the time of audiological follow-up threatened the options for restoration of hearing. In the other case, a long diagnostic programme and an unsuccessful attempt at cochlear implantation caused a long delay in optimal restoration of hearing. Both cases illustrate the difficulties in connection with postmeningitis deafness in relation to the option of a cochlear implant operation. To increase the chances of a successful implantation, the time span between meningitis and audiological and radiological follow-up must be short. Auditory brain stem responses (ABR) and MRI are the keystones of the work-up.

High prevalence of abnormalities on CT and MR imaging in children with unilateral sensorineural hearing loss irrespective of age or degree of hearing loss.

OBJECTIVE: Evaluation of causal abnormalities identified on CT and MR imaging in children with unilateral sensorineural hearing loss (USNHL), and the association with age and severity of hearing loss. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral otology/audiology center. PATIENTS AND DIAGNOSTIC INTERVENTIONS: 102 children diagnosed with USNHL between 2006 and 2016 were included. They underwent CT and/or MR imaging for the evaluation of the etiology of their hearing loss. MAIN OUTCOME MEASURES: Radiologic abnormalities of the inner ear and brain associated with USNHL. RESULTS: Using CT and/or MR imaging, causal abnormalities were identified in 49%, which is higher than previously reported (25-40%). The most frequently affected site was the labyrinth (29%), followed by the cochlear nerve (9%) and brain (7%). No significant difference in the number or type of abnormalities was found for the degree of hearing loss or age categories. CONCLUSIONS: Imaging is essential in the etiologic analysis of USNHL because of the high prevalence of causative abnormalities that can be identified with radiology, irrespective of the patients' age or degree of hearing loss. CT and MR imaging are complementary imaging options. The ideal imaging algorithm is controversial. Based on our findings, we conclude that there is limited additional diagnostic value of simultaneous dual modality imaging over sequential diagnostics. We therefore perform a stepwise radiological workup in order to maximize the diagnostic yield while minimizing impact and costs. If the primary imaging modality does not identify a cause for USNHL, performing the alternative imaging modality should be considered. LEVEL OF EVIDENCE: Retrospective cohort study 2b.

The 'best method' of topical nasal drug delivery: comparison of seven techniques.

OBJECTIVE: To determine whether there is a 'best' technique for delivering drugs to the middle meatus. DESIGN: Single-blind cross-over study in healthy individuals using endoscopic video-imaging. PARTICIPANTS: A dyed test formulation was administered intranasally on seven non-sequential days to ten healthy individuals with no 'nasal' history. The participants were recruited through advertisement. MAIN OUTCOME MEASURES: Comparison of seven different techniques, 20 nostrils and 140 endoscopic videos for the deposition patterns of dyed test formulation. Analysis was possible in 90% of all endoscopic videos. Three head positions were tested for both nasal drops and nasal sprays. RESULTS: Deposition of dyed test formulation near the middle meatus was observed in 43% of all observations. No significant differences were observed in terms of delivery between any of the seven techniques. CONCLUSIONS: Our study suggests there may not be a single 'best' technique for topical nasal drug delivery. A more individual approach to topical nasal drug treatment, taking anatomy and head position into account would seem to be more appropriate.

[Airway malacia: not uncommon in children with atypical respiratory symptoms]. / Luchtwegmalacie: niet zeldzaam bij kinderen met atypische luchtwegsymptomen.

OBJECTIVE: To estimate the prevalence of primary airway malacia at birth, determine the predictive value of a clinical diagnosis of airway malacia compared with bronchoscopy results and describe the presenting symptoms. DESIGN: Retrospective descriptive study. METHOD: We reviewed the results of all bronchoscopies performed in the period 1997-2004 at the Erasmus MC-Sophia Children's Hospital, Rotterdam, the Netherlands, and the standardised status assessment of children diagnosed with primary airway malacia. RESULTS: A total of 512 bronchoscopies were performed. Primary airway malacia was diagnosed in 136 children (80 boys) with a median age of 4.3 years (range: 0-17). The prevalence of primary airway malacia at birth was estimated at approximately 1 in 2100. A diagnosis of probable airway malacia based on symptoms, patient history and targeted assessment of pulmonary function proved to be correct in 74% of patients. However, airway malacia was not suspected before bronchoscopy in 52% of patients. The symptoms were atypical and included: cough, recurrent airway infections, dyspnoea, wheezing and reduced exertional tolerance. The peak expiratory flow was more affected than the forced expiratory volume in 1 second value. CONCLUSION: Primary airway malacia occurs in an estimated 1 out of 2100 children and is difficult to recognise based on patient history and symptoms. Bronchoscopy should be considered to rule out airway malacia in patients with unexplained exertional intolerance, recurrent lower airway infections, or with 'atypical' or 'treatment-resistant' asthma.

Rare cause of bilateral sudden deafness.

In this paper, we describe the case of a 62-year-old female with recurring episodes of sudden deafness with vertigo and facial paresis. Within a month's time, this resulted in bilateral deafness and vestibular areflexia. Erroneously, the patient was diagnosed with sudden deafness of unknown origin and subsequently with neuroborreliosis (Lyme disease). The true diagnosis of relapsing polychondritis (RP) was revealed 9 months after initial presentation. The diagnostic delay is in part explained by the fact that, by definition, the disease has to relapse before the diagnosis can be made, but also by its pluriform clinical presentation. Timely identification of RP as the cause of this profound sensorineural hearing loss proved to be important. It was key in instigating adequate follow-up, and allowed for cochlear implantation before total cochlear obliteration, which might have hampered optimal hearing rehabilitation.