Cholesteatoma in the pediatric aerodigestive population.

OBJECTIVES: To report the prevalence of cholesteatoma and related comorbidities in pediatric aerodigestive patients requiring tracheostomy or airway reconstruction procedures. To use study findings to inform clinical management of these complex patients. METHODS: A repository of clinical data drawn from our institution's electronic medical records was queried to identify airway reconstruction (airway) and complex hospital control (control) patient cohorts. Retrospective chart review was then performed to investigate the occurrence of cholesteatoma and related pathologies in these patients, as well as clinical management. RESULTS: The prevalence of cholesteatoma in airway and control patients was 6/374 (1.60 %) and 35/30,565 (0.11 %), respectively. The relative risk of cholesteatoma diagnosis in airway patients was 14.01 (95 % CI 6.06-32.14). Airway patients were more likely than control patients to have pressure equalization tube history (relative risk 3.25, 95 % CI 2.73-3.82). Age at cholesteatoma diagnosis and first surgical intervention was younger in airway compared to control patients (5.43 vs. 8.33, p = 0.0182, and 6.07 vs. 8.82, p = 0.0236). However, time from diagnosis to intervention and extent of surgery were similar between the groups. CONCLUSION: This is the first study to investigate the prevalence of cholesteatoma in the pediatric aerodigestive population. The relative risk of cholesteatoma diagnosis was found to be 14 times higher in patients with tracheostomy or airway reconstruction history. Underlying eustachian tube and palatal dysfunction are likely contributing factors to the elevated risk. Additionally, cholesteatoma in this population was diagnosed and required surgical intervention at a younger age, which may suggest a more aggressive disease course. Providers should maintain a high degree of suspicion for cholesteatoma in this complex population.

Colesteatoma del conducto auditivo externo: Experiencia de 5 años Hospital del Salvador / External ear canal cholesteatoma: 5-year experience Hospital del Salvador

Introducción: El colesteatoma del conducto auditivo externo (CCAE) es una estructura quística revestida por epitelio escamoso estratificado queratinizado que tiene la capacidad de invadir y erosionar localmente al hueso temporal. Su incidencia es de 0,19 a 0,3/100.000 habitantes siendo 60 veces menos frecuente que el de oído medio. Objetivo: Describir las características epidemiológicas, clínicas, imagenológicas y tratamiento de los pacientes diagnosticados con CCAE en el Servicio de Otorrinolaringología Hospital del Salvador. Material y Método: Se presenta una serie de ocho casos clínicos recopilados durante el período 2017 y 2021. Se realizó revisión de fichas clínicas, biopsias y tomografías computadas de oídos (TC oídos). Se describen los hallazgos y tratamiento efectuado. Resultados: El promedio de edad fue de 65,6 años, correspondiente a 5 mujeres y 3 hombres con presencia de tabaquismo y diabetes en la mitad de los casos. Los síntomas y signos más frecuentes fueron otalgia e hipoacusia seguido de otorrea. 7 pacientes se presentaron con tímpano íntegro y el compromiso de la pared inferior del conducto se evidenció en 6 de 8 pacientes. La TC oídos mostró erosión ósea del conducto, con o sin compromiso de estructuras adyacentes, en todos los casos y el diagnóstico histológico fue efectuado en el 100% de los pacientes. Se privilegió el tratamiento conservador mediante curaciones óticas periódicas asociado a ácido salicílico al 3% y/o antibióticos tópicos en 6/8 pacientes. Conclusiones: El CCAE es una entidad poco frecuente sin signos ni síntomas patognomónicos por lo que el diagnóstico histológico junto con el estudio imagenológico es perentorio. El tratamiento conservador es una alternativa terapéutica válida que ofrece buenos resultados en pacientes con adecuada adherencia al tratamiento y posibilidad de seguimiento estricto.

Introduction: External ear canal cholesteatoma (EECC) is a cystic structure lined by keratinized stratified squamous epithelium that has the ability to locally invade and erode the temporal bone. Its incidence is 0.19 to 0.3 / 100,000 habitants, being 60 times less frequent than that of the middle ear. Aim: To describe the epidemiological, clinical, imaging and treatment characteristics of patients diagnosed with EECC in the Hospital del Salvador ENT department. Material and Methods: A series of eight clinical cases collected during the period 2017 and 2021 is presented. A review of clinical records, biopsies and computed tomography of the ear (ear CT) was carried out. The findings and treatment carried out are described. Results: The average age was 65.6 years corresponding to 5 women and 3 men with the presence of smoking and diabetes in half of the cases. The most frequent symptoms and signs were earache and hearing loss followed by otorrhea. 7 patients presented with an intact eardrum and compromise of the inferior wall of the canal was evidenced in 6 of 8 patients. Ears CT showed bone erosion of the canal with or without compromise of adjacent structures in all cases and the histological diagnosis was made in 100% of the patients. Conservative treatment with periodic ear dressings associated with 3% salicylic acid and / or topical antibiotics was favored in 6/8 patients. Conclusion: EECC is a rare entity without pathognomonic signs or symptoms, therefore the histological diagnosis together with the imaging study is peremptory. Conservative treatment is a valid therapeutic alternative that offers good results in patients with adequate adherence to treatment and the possibility of strict follow-up.

The Risk of Cholesteatoma in Individuals With First-degree Relatives Surgically Treated for the Disease.

Importance: Cholesteatoma in the middle ear is not regarded as a hereditary disease, but case reports of familial clustering exist in the literature, as well as observed familial cases in the clinical work. However, the knowledge regarding cholesteatoma as a hereditary disease is lacking in the literature. Objective: To assess the risk of cholesteatoma in individuals with a first-degree relative surgically treated for the same disease. Design, Setting, and Participants: In this nested case-control study in the Swedish population between 1987 and 2018 of first-time cholesteatoma surgery identified from the Swedish National Patient Register, 2 controls per case were randomly selected from the population register through incidence density sampling, and all first-degree relatives for cases and controls were identified. Data were received in April 2022, and analyses were conducted between April and September 2022. Exposure: Cholesteatoma surgery in a first-degree relative. Main Outcomes and Measures: The main outcome was first-time cholesteatoma surgery. The association between having a first-degree relative with cholesteatoma and the risk of cholesteatoma surgery in the index persons was estimated by odds ratios (ORs) and 95% CIs through conditional logistic regression analysis. Results: Between 1987 and 2018, 10 618 individuals with a first-time cholesteatoma surgery (mean [SD] age at surgery, 35.6 [21.5] years; 6302 [59.4%] men) were identified in the Swedish National Patient Register. The risk of having a cholesteatoma surgery was almost 4 times higher in individuals having a first-degree relative surgically treated for the disease (OR, 3.9; 95% CI, 3.1-4.8), but few cases were exposed overall. Among the 10 105 cases with at least 1 control included in the main analysis, 227 (2.2%) had at least 1 first-degree relative treated for cholesteatoma, while the corresponding numbers for controls were 118 of 19 553 control patients (0.6%). The association was stronger for individuals under the age of 20 years at first surgery (OR, 5.2; 95% CI, 3.6-7.6) and for a surgery involving the atticus and/or mastoid region (OR, 4.8; 95% CI, 3.4-6.2). There was no difference in the prevalence of having a partner with cholesteatoma between cases and controls (10 cases [0.3%] and 16 controls [0.3%]; OR, 0.92; 95% CI, 0.41-2.05), which implies that increased awareness does not explain the association. Conclusions and Relevance: In this Swedish case-control study using nationwide register data with high coverage and completeness, the findings suggest that the risk of cholesteatoma in the middle ear is strongly associated with a family history of the condition. Family history was nevertheless quite rare and can therefore only explain a limited number of all cases; these families could be an important source for information regarding the genetic background for cholesteatoma disease.

Cholesteatoma in Congenital Aural Atresia and External Auditory Canal Stenosis: A Systematic Review.

OBJECTIVE: Patients with congenital external auditory canal (EAC) abnormalities are at risk of developing cholesteatoma and often undergo surveillance imaging to detect it. The aims of this systematic review are to determine the incidence of cholesteatoma in patients with congenital aural atresia (CAA) and patients with congenital EAC stenosis and to investigate the most common age of cholesteatoma diagnosis. This information will help clinicians decide which patients require surveillance scanning, as well as the timing of imaging. DATA SOURCES: Ovid MEDLINE, Embase, CENTRAL, and Web of Science databases. REVIEW METHODS: A systematic literature review following the PRISMA guidelines was performed. The data sources were searched by 2 independent reviewers, and articles were included that reported on CAA or congenital EAC stenosis with a confirmed diagnosis of cholesteatoma. The selected articles were screened separately by 3 reviewers before reaching a consensus on the final articles to include. Data collection on the number of patients with cholesteatoma and the age of diagnosis was performed for these articles. RESULTS: Eight articles met the inclusion criteria. The incidence of cholesteatoma was 1.7% (4/238) in CAA and 43.0% (203/473) in congenital EAC stenosis. The majority of patients with congenital EAC stenosis that developed cholesteatoma were diagnosed at age <12 years. CONCLUSION: CAA is associated with a low risk of cholesteatoma formation, and surveillance imaging is unnecessary in asymptomatic patients. EAC stenosis is strongly associated with cholesteatoma, and a surveillance scan for these patients is recommended prior to 12 years of age with close follow-up into adulthood.

Risk of Acquired Cholesteatoma and External Auditory Canal Stenosis in Traumatic Brain Injury: A Nationwide Population-Based Cohort Study.

The aim of study is to investigate the risk of developing acquired cholesteatoma and external auditory canal (EAC) stenosis after traumatic brain injury (TBI) from the Taiwan National Health Insurance Research Database (NHIRD). Each subject was individually traced from their index date to identify those who received a diagnosis of acquired cholesteatoma and EAC stenosis. Cox regression analyses were applied to determine the risk of TBI-related acquired cholesteatoma and EAC stenosis. The follow-up data collected over 10 years were obtained from the TBI and comparison cohorts, of 455,834 and 911,668 patients, respectively. Multivariate analysis demonstrated that TBI significantly increased the risk of cholesteatoma (adjusted hazard ratio (HR), 1.777; 95% confidence interval (CI), 1.494-2.114, p < 0.001) and EAC stenosis (adjusted (HR), 3.549; 95% (CI), 2.713-4.644, p < 0.001). In our subgroup injury analysis, falls had the highest associated risk (4.308 times), followed by traffic injuries (66.73%; 3.718 times that of the control group). Otolaryngologists should not neglect the clinical importance and carefully investigate the possibility of subsequent cholesteatoma and EAC stenosis, which leads to hearing impairment in patients with TBI. Our research also shows the important role in preventing TBI, especially as a result of traffic injuries and falls.

Rate of chronic otitis media operations and cholesteatoma surgeries in South Korea: a nationwide population-based study (2006-2018).

The aim of this study was to estimate the total number and rate of chronic otitis media (COM) operations and cholesteatoma surgeries in South Korea, using a nationwide survey which analysed a 13-year trend (2006-2018). This study also analysed the trend of COM operations and cholesteatoma surgeries according to year, sex, and age using a nationwide population-based database, and the 13-year trend was analysed according to age groups. This study used nationwide data from the National Health Information Database (NHID), which is a government-affiliated agency under the Korean Ministry of Health and Welfare that supervises all medical activities in Korea. Retrospective medical data of patients of all ages were extracted from the NHID from January 2006 to December 2018 (NHIS-2018). This study was conducted by the Research Committee of the Korean Society of Otorhinolaryngology-Head and Neck Surgery, and the Korean Audiological Society reviewed and confirmed the study. There was a 1.5 fold increase in COM operation rates in 2018, compared to 2007 figures. The annual total number of COM operations was 5,935 in 2007, 8,999 in 2012 (peak), and 8,870 in 2018 (17 in 100,000). Meanwhile, the total annual number of cholesteatoma surgeries decreased from 3,502 in 2006 to 3,199 in 2018 (6 in 100,000). The rate of COM operations was higher (1.27 fold) in the female population than in the males in 2018. However, cholesteatoma surgery rates were higher (1.2 fold) in the male population than in the females in 2018. According to the 2018 data, COM operations were most commonly performed in patients in their 50s. COM operation rates increased rapidly in patients aged 51-80. In other age groups however, rates were constant or showed a decrease in figures, especially in the 40s age group (1st rank in 2006 to 3rd rank in 2018). According to the 2018 data, cholesteatoma surgery was most commonly performed in patients in their 50s. Cholesteatoma surgery rates increased dramatically from 2006 to 2018 in patients aged 0-10 years due to congenital cholesteatoma. Cholesteatoma surgery rates also increased in patients aged 61-80 years due to ageing population. Cholesteatoma surgery rates decreased in patients aged 41-50 years, ranking 1st in 2006 and 4th in 2018. In conclusion, the annual rate of COM operations was 0.017%, and no longer increases, but stabilizes/decreased after a peak point in the advanced country. The mean rate of cholesteatoma surgery was 0.006%, and decreased annually. There was female dominance in COM operations, but male dominance in cholesteatoma surgery. Major age groups of patients who underwent COM/cholesteatoma surgery were the 50s and 60s, and congenital cholesteatoma (0-10 years) accounted for about 20% of all cholesteatoma surgery.

External auditory canal cholesteatoma and benign necrotising otitis externa: clinical study of 95 cases in the Capital Region of Denmark.

OBJECTIVE: To identify epidemiological and pathophysiological factors, and treatment strategies, in external auditory canal cholesteatoma and benign necrotising otitis externa. METHODS: A retrospective case study was conducted of patients suffering from external auditory canal cholesteatoma and benign necrotising otitis externa admitted to tertiary hospitals, in the Capital Region of Denmark, over a five-year period. RESULTS: Eighty-three patients (95 ears) with external auditory canal cholesteatoma or benign necrotising otitis externa were identified. A minimum incidence rate of 0.97 per 100 000 inhabitants per year was demonstrated. Sixty-eight per cent of cases had a history of smoking. Most lesions (74 per cent) were localised in the floor of the ear canal. Treatment time was 3.2 months for patients who had surgery and 6.0 months for those who received conservative treatment. CONCLUSION: It is suggested that external auditory canal cholesteatoma and benign necrotising otitis externa are in fact the same disease, and therefore the diagnosis of external auditory canal cholesteatoma should be changed to benign necrotising otitis externa. Microangiopathy has a leading role in the aetiology. Surgery should be conducted in most cases.

Bilateral Congenital Cholesteatoma.

OBJECTIVE: To report the first large case series of extremely rare bilateral congenital cholesteatoma (CC). STUDY DESIGN: A retrospective cohort study. SETTING: University hospital otology referral clinic. PATIENTS: Six hundred four children with surgically confirmed CC. MAIN OUTCOME MEASURES: The bilateral CCs were compared with the unilateral CCs. RESULTS: The incidence of bilateral CC was 3.6% (22/615) per case or 1.8% (11/604) per child. Bilateral CC did not differ from unilateral CC regarding its demographics, and invasiveness by the proportion of advanced CC as 31.8% (7/22) versus 28.2% (167/594). But the invasiveness or location randomly differed between the ears, that advanced CC per child was higher as 45.4% (5/11). Bilateral exploration was attempted with a concern for hearing loss, which featured a combination of laser myringotomy to treat early CC and endaural laser-assisted single-stage inside-out cholesteatoma surgery to treat advanced CC. Bilateral exploration was difficult in three children with initially negative otoendoscopy. Among the seven advanced CC, proportion of anterior type was 71.4% (5/7), who all exhibited more than 20 dB HL, but two posterior type retained normal hearing. Therefore, bilateral advanced CCs of anterior origin showed poorest hearing outcome as bilateral more than 20 dB HL, which were in two children. Six second-look operations and one third-look operation were required to treat six residual CCs (30%) in four children (40%), including bilateral residual CC in two (20%); such reoperations were significantly more frequent than in unilateral CC. CONCLUSION: The diagnosis of bilateral CC required high index of suspicion from TBCT, and early bilateral exploration.

No evidence of cholesteatoma in untreated otitis media with effusion in children with primary ciliary dyskinesia.

INTRODUCTION: Primary Ciliary Dyskinesia (PCD) describes a group of inherited disorders that result in abnormal ciliary motion leading to mucous stasis. Clinical features include almost universally otitis media with effusion (OME), particularly in infants. PCD patients provide us with a cohort of patients with OME that is not treated with ventilatory tube (VT) insertion as these have been shown to result in frequent complications including chronic otorrhoea, early extrusion and persistent perforation without significant improvement to hearing in the long term. This cohort was used to investigate whether children with PCD and OME not treated with VT were predisposed to cholesteatoma formation in the setting of a paediatric quaternary referral centre. METHODS: A retrospective chart review was performed of all the children attending a multi-disciplinary PCD clinic at a national quaternary referral centre with a diagnosis of OME. We reviewed otoscopic findings, and audiometry and tympanometry results. We assessed the children in four groups: Watchful waiting, hearing aids, VT, and VT and hearing aids. RESULTS: One-hundred-and-one of 107 patients included in the study had a diagnosis of otitis media with effusion. No child with OME and PCD was diagnosed with a cholesteatoma during the follow up period. The only children who had insertion of a ventilatory tube were those who had the procedure prior to the formal diagnosis of PCD. We found a significant complication rate in the children with VT insertion. Hearing improved over time. The prevalence of retraction pockets in untreated OME was 1.72% (3 out of 174 ears). CONCLUSIONS: In children with PCD, OME is an almost universal finding in younger children, but not in adolescents. The study supports the current preference to avoid VT insertion in children with PCD as it confers a significantly higher rate of complications. No cases of cholesteatoma were found in this cohort of PCD children with OME managed without VTs.

Increased risk of cholesteatoma among patients with allergic rhinitis: A nationwide investigation.

OBJECTIVES: No large population-based studies have reported on the risk of cholesteatoma developing after allergic rhinitis (AR). This study used a nationwide population-based claims database to investigate the hypothesis that AR may increase the risk of cholesteatoma. STUDY DESIGN: Retrospective cohort study. METHODS: Data from Taiwan's Longitudinal Health Insurance Database were analyzed to compile the following: 1) 15,953 patients newly diagnosed with AR between 1997 and 2000, and 2) a comparison cohort of 63,812 matched non-AR enrollees (with a ratio of 1 to 4). Each patient was followed for 10 years to identify cases in which cholesteatoma subsequently developed. The Kaplan-Meier method was used to determine the cholesteatoma-free survival rate, and the log-rank test was used to compare survival curves. Cox proportional hazard regressions were performed to compute adjusted hazard ratios (HRs). RESULTS: Among the 79,765 patients enrolled in this study, 45 (159,364 person-years) from the AR cohort and 88 (638,130 person-years) from the comparison cohort were diagnosed with cholesteatoma during the follow-up period (incidence rates 0.28 and 0.14 of 1,000 person-years, respectively). Patients with AR were more likely to develop cholesteatoma compared to those without AR (adjusted HR 1.57, 95% confidence interval = 1.05-2.34, P < 0.05). Patients with AR presented a significantly lower 10-year cholesteatoma-free survival rate than did those in the comparison group (log-rank, P < 0.001). CONCLUSION: This is the first study to demonstrate a link between AR and the development of cholesteatoma. We suggest that clinicians keep this association in mind and carefully investigate the possibility of development of cholesteatoma among patients with AR. LEVEL OF EVIDENCE: 3b. Laryngoscope, 128:547-553, 2018.

Cholesteatoma as a complication of Langerhans Cell Histiocytosis of the temporal bone: A nationwide cross-sectional analysis.

OBJECTIVE: To determine if patients with Langerhans Cell Histiocytosis (LCH) of the temporal bone have a higher risk of developing cholesteatoma. METHODS: Review of literature and cross-sectional weighted analysis of patients under 19 with a diagnosis of LCH from the National Inpatient Sample (NIS) and Kids' Inpatient Database (KID) from 2000 to 2013. ICD-9 codes and demographics were analyzed; pairwise comparisons and multivariate analyses were performed. RESULTS: Only seven cases of cholesteatoma after the treatment for LCH of the temporal bone have been documented in the literature. No significant association between cholesteatoma and LCH was seen (OR 0.747 [0.149-3.751]). Patients with LCH did have a higher incidence of chronic otitis media, chronic otitis externa, chronic sinusitis, hearing loss, and otitis media with effusion. CONCLUSION: Our results show that patients with Langerhans Cell Histiocytosis do not appear to have a higher risk of developing cholesteatoma. However they are more likely to be diagnosed with chronic otitis externa which should be differentiated from cholesteatoma or recurrence of LCH.

Incidence, 10-year recidivism rate and prognostic factors for cholesteatoma.

OBJECTIVE: Cholesteatoma patients have a high risk of recurrence with complications, and knowledge exchange is a prerequisite for improving treatment. This study aimed to apply appropriate statistics to provide meaningful and transferable results from cholesteatoma surgery, to highlight independent prognostic factors, and to assess the incidence rate. METHODS: Incidence rates were assessed for the district of Aarhus, Denmark. From 147 patients operated on mainly with canal wall up mastoidectomies for debuting cholesteatomas, 10-year Kaplan-Meier recidivism rates were calculated and independent prognostic factors for the recidivism were identified by Cox multivariate regression analyses. RESULTS: Incidence rate was 6.8 per 100 000 per year. The 10-year cumulative recidivism rate was 0.44 (95 per cent confidence interval, 0.37-0.53). Independent prognostic factors for the recidivism were: age below 15 years (hazard ratio = 2.2; p > z = 0.002), cholesteatoma localised to the mastoid (hazard ratio = 1.7; p > z = 0.04), stapes erosion (hazard ratio = 1.9; p > z = 0.02) and incus erosion (hazard ratio = 1.9; p > z = 0.04). CONCLUSION: The recidivism rate is influenced by several factors that are important to observe, both in the clinic and when comparing results from surgery.

Depression and cholesteatoma: Preliminary findings from a nationwide population-based retrospective cohort study.

OBJECTIVE: To estimate the risk of developing depressive disorder (DD) following diagnosis with cholesteatoma. METHODS: In the study, we analyzed data from the Longitudinal Health Insurance Database of Taiwan. A total of 599 patients newly diagnosed with cholesteatoma between 1997 and 2007 were included with a comparison cohort of 2995 matched non-cholesteatoma enrollees. Each patient was followed for 3 years to identify the subsequent development of DD. Cox proportional hazard regression analysis was performed to compute adjusted 3-year hazard ratios. RESULTS: The incidence of DD per thousand person-years was approximately twice as high among patients with cholesteatoma (11.32) as among those without cholesteatoma (5.85). After adjusting for potential confounders, patients with cholesteatoma were 1.99 times (95% CI=1.18-3.34, P=0.010) more likely to suffer from DD within 3 years compared to those without cholesteatoma. CONCLUSIONS: This is the first study to demonstrate a link between cholesteatoma and subsequent DD within a three-year followup. We suggest that clinicians keep this critical but neglected issue in mind and carefully investigate the possibility of subsequent psychological problems among cholesteatoma patients.

Increased Acquired Cholesteatoma Risk in Patients with Osteoporosis: A Retrospective Cohort Study.

OBJECTIVE: Clinically, we found the increased incidence of acquired colesteatoma in the patients with osteoporosis. In this study, we used a retrospective cohort to examine this association and to investigate the possible mechanism. METHODS: We conducted a population-based retrospective cohort study by using the National Health Insurance Research Database (NHIRD). We identified an osteoporosis cohort comprising 37 124 patients newly diagnosed with osteoporosis aged 20 years or older. Patients in the comparison cohort had no history of osteoporosis and were frequency matched with the patients in the osteoporosis cohort according to sex, age, and index year. RESULTS: The acquired cholesteatoma incidence rates for the osteoporosis and comparison cohorts were 1.12 and 0.83 per 1000 person-years, respectively. After we adjusted for confounding factors, the osteoporosis cohort exhibited a 1.32-fold increased acquired cholesteatoma risk relative to the comparison cohort (hazard ratio [HR] = 1.32, 95% confidence interval [CI] = 1.11-1.57). In addition, patients with no history of otitis media (HR = 1.33, 95% CI = 1.11-1.59), cancer (HR = 1.34, 95% CI = 1.12-1.60), or COPD (HR = 1.26, 95% CI = 1.05-1.52) in the osteoporosis cohort exhibited an increased risk of subsequent acquired cholesteatoma relative to those in the comparison cohort. CONCLUSIONS: Our cohort study indicated that patients with osteoporosis had a 1.31-fold increased acquired cholesteatoma risk relative to the comparison cohort. This risk was further increased in patients with comorbid otitis media. Hence, we recommend that otolaryngologists evaluate the condition of the middle ear of patients with osteoporosis.

Tracking tympanostomy tube outcomes in pediatric patients with otitis media using an electronic database.

OBJECTIVE: To implement and review a database for children with a diagnosis of otitis media (OM) to facilitate comparative outcomes and long-term prospective follow up of surgical outcomes. Specific aim is to review presenting symptoms, risk factors, tympanostomy tube outcomes and complications, and need for further procedures. METHODS: A web-based customized database was constructed to universally enroll all patients seen in consultation with a diagnosis of OM. Unique database fields include demographics, physical exam findings, risk factors, intervention, and long-term outcomes. Major surgical complications measured include: tympanic membrane perforation, retained tubes, chronic otorrhea, and cholesteatoma formation. RESULTS: Six hundred and thirty four unique patients have been prospectively enrolled. Five hundred and forty four tubes have been followed to extrusion. Outcomes demonstrate high prevalence of OM risk factors associated with surgical patients including: 63% in day care and 26% with a sibling requiring tympanostomy tubes. Complication rates; 1% developed perforations requiring surgical intervention, 2.6% required removal of retained tubes, 1% extruded early (<60 days), and 0.7% were surgically removed for other complications. Cholesteatoma was identified in 0.56%, all had ongoing chronic ear disease. CONCLUSIONS: Long-term, outcome driven investigations assessing the surgical management of OM are needed given the prevalence of this disease and the frequency of surgical intervention required. The current database represents the largest prospective cohort of patients enrolled and followed in this fashion and has generated data demonstrating a procedure associated with significant improvement in patient quality of life in the short-term with low complication rates in the long-term. This ongoing prospective investigation is providing data that have the potential to be important in treatment algorithms, procedure justification, and risk factor modification.

Quality of life of Nepali patients with ear disease before and after corrective surgery.

OBJECTIVES: Hearing impairment is a significant burden in the developing world. However, no suitable quality of life (QoL) measures exist for use in Nepal. We aimed to amend and translate the Glasgow Health Status Inventory (GHSI), assessing QoL at any given time, and the Glasgow Benefit Inventory (GBI), assessing change in QoL following intervention, into Nepali and to assess the impact of ear disease and effect of surgery on QoL. METHODS: The GHSI and GBI were translated into Nepali and independently verified. The GHSI was administered by interview to patients before surgery, and the GBI was administered 6 months after surgery. The Mann-Whitney U-test was used for hypothesis testing. RESULTS: The GHSI was administered to 242 patients. In total, 205 had chronic suppurative otitis media (CSOM) without cholesteatoma and 37 had cholesteatoma. The mean GHSI score was 47.9. There was no significant difference in GHSI scores between patients with CSOM without cholesteatoma and those with cholesteatoma. The GBI was administered to 161 patients, 73 of whom had also been in the GHSI group. In total, 130 had CSOM without cholesteatoma, 31 had cholesteatoma. The mean GBI score was +38.4 with no significant difference between disease groups. CONCLUSIONS: Ear disease in Nepal is associated with reduced QoL, and surgical intervention is associated with improved QoL. There is no difference in QoL or benefit following surgery for CSOM between patients with or without cholesteatoma. There are few QoL measures suitable for the developing world. It is essential to invest in these measures to guide health interventions.

Epidemiological study of cholesteatoma in Fukuoka City.

OBJECTIVE AND DESIGN: To investigate the pathogenesis of cholesteatoma, we planned to conduct a cohort study. As a first step, we conducted an epidemiological study in Fukuoka City, Japan to determine the incidence of cholesteatoma treated both with and without surgery. We also conducted a case-control study to investigate the pathogenesis of cholesteatoma. RESULTS: The annual incidence of cholesteatoma, including cases treated without surgery, was 6.8-10.0 in a population of 100 000. The results of the case-control study suggested that a past history of otitis media and habitual sniffing caused by a patulous eustachian tube play a role in the pathogenesis of cholesteatoma. CONCLUSIONS: The annual incidence of cholesteatoma, including cases treated without surgery, was considered to not be high enough to perform a cohort study. The results of the case-control study suggest that otitis media and habitual sniffing due to a patulous eustachian tube, contribute to the onset of cholesteatoma.

Cholesteatoma risk in 8,593 orofacial cleft cases and 6,989 siblings: A nationwide study.

OBJECTIVES/HYPOTHESIS: To estimate the risk of surgically treated middle ear cholesteatoma in individuals with a nonsyndromic orofacial cleft and in their siblings compared with the general population. STUDY DESIGN: Historical cohort study. METHODS: Using the unique civil registration number for linkage, data from three national registers were used for the Danish 1936-2009 birth cohorts. Hazard ratios (HRs) were estimated with Cox regression analyses using age as the underlying time variable. Individuals were followed from January 1, 1977 until time of surgically treated cholesteatoma, and censored at emigration, death, or end of follow-up (December 31, 2010). RESULTS: A total of 8,593 individuals with nonsyndromic orofacial cleft and 6,989 siblings were identified, undergoing 201 and 21 first-time cholesteatoma surgeries, respectively. A 5% random sample of the Danish population comprising 249,708 persons without an orofacial cleft was created, and 175,724 siblings to these persons were identified. These controls underwent 485 and 332 first-time cholesteatoma surgeries, respectively. For individuals with cleft lip and palate the HR for cholesteatoma surgery was 14 (95% confidence interval [CI], 12-18) and for individuals with cleft palate the HR was 20 (95% CI, 16-24) when compared with the random sample. In siblings of individuals with cleft palate, the HR for cholesteatoma surgery was 2.1 (95% CI, 1.1-4.1) when compared with siblings of the random sample. CONCLUSIONS: A 20-fold increase in the risk of cholesteatoma was found in individuals with cleft palate, whereas cleft lip did not pose a risk of cholesteatoma. Furthermore, the study indicates an increased risk of cholesteatoma in unaffected siblings of individuals with cleft palate. LEVEL OF EVIDENCE: 2b Laryngoscope, 125:1225-1229, 2015.

Cholesteatoma in Danish children - a national study of changes in the incidence rate over 34 years.

OBJECTIVE: To describe temporal trends in the incidence rate of surgically treated middle ear cholesteatoma in Danish children from 1977 to 2010. METHODS: Data on surgically treated middle ear cholesteatoma was drawn from the Danish National Patient Register. A change in incidence rate over time was examined using Poisson regression analysis, while the cumulative incidence proportion was estimated using life-tables. RESULTS: A total of 5850 cases of surgically treated middle ear cholesteatoma distributed among 3874 children aged 0-15 years were identified. From 1977 to 2002 the age-standardized incidence rates for first-time surgically treated middle ear cholesteatoma increased from 8 to 15 per 100,000 person-years with an estimated annual increase of 1.8% (95% confidence interval (CI) 1.3-2.2%). From 2002 to 2010 the rates decreased from 15 to 10 per 100,000 person-years with an annual decrease of 5.4% (95% CI 3.2-7.5%). Age-specific incidence rates were at maximum around the age of 9 years during the whole period. The estimated cumulative incidence proportion at age 16 years based on the 2010 age-specific incidence rates was 0.16% (95% CI 0.09-0.32%) compared with 0.20% (95% CI 0.11-0.37%) based on the 2000 age-specific incidence rates. CONCLUSION: From 2002 to 2010 there was a decrease in the incidence rate of first-time surgically treated middle ear cholesteatoma. The decrease was preceded by a significant increase in the incidence rate of middle ear ventilation tube insertion. However, further studies are needed to find possible explanations for the decrease.