Development of otology specific outcome measure: Ear Outcome Survey-16 (EOS-16).

PURPOSE: An important outcome measure of patient care is the impact on the patient's health-related quality of life (HRQoL). Current ear-specific HRQoL instruments are designed for one diagnosis and emphasize different subdivisions such as symptoms, hearing problems, psychosocial impact, and the need for care. The optimal length of the recall period has not been studied. For these reasons, a new survey is needed that would cover most chronic ear diseases. METHODS: A preliminary 24-item survey (EOS-24) was created. Untreated adult patients (included n = 186) with one of seven different chronic otologic conditions from all university hospitals in Finland were recruited to respond to EOS-24 and the 15D general HRQoL instrument. The recruiting otologists evaluated the severity of the disease and the disability caused by it. A control group was recruited. Based on the patients' responses in different diagnosis groups, the items were reduced according to pre-defined criteria. The resulting survey was validated using a thorough statistical analysis. RESULTS: The relevance and necessity of the original 24 items were thoroughly investigated, leading to the exclusion of 8 items and the modification of 1. The remaining 16 items were well-balanced between subdivisions and were useful in all seven diagnosis groups, thus constituting the final instrument, EOS-16. The most suitable recall period was three months. CONCLUSIONS: EOS-16 has been created according to the HRQoL survey guidelines with a versatile nationwide patient population. The survey has been validated and can be used for a wide range of chronic ear diseases as a HRQoL instrument.

Cleft Lip and/or Palate and Auricular Malformations.

OBJECTIVE: To study the relationship between cleft lip and/or palate and auricular malformations in Finnish patients with cleft. DESIGN: Retrospective analysis of patients with an external ear malformation and either a cleft lip with or without a cleft palate (CL±P) or an isolated cleft palate (CP). SETTING: Tertiary referral clinic. PATIENTS: Review of hospital records of 100 patients from the register of 8200 patients with cleft in the Cleft and Craniofacial Centre at the Helsinki University Central Hospital. MAIN OUTCOME MEASURES: Proportions of variable auricular malformations among CL±P and CP patients. RESULTS: Microtia is the most common auricular malformation among patients with cleft and is almost equally prevalent with both CL±P and CP. The prevalence of microtia increases as the severity of CL±P increases. The combination of microtia and CL±P or CP is frequently found with both oculo-auriculo-vertebral spectrum and Treacher Collins syndrome. CONCLUSIONS: Microtia seems to be the most common auricular malformation among patients with cleft. The prevalence of microtia seems to increase as the severity of CL±P increases, whereas in isolated CP microtia seems to occur independently.

The learning curve in microtia surgery.

Reconstruction of the auricle is known to be complex. Our objective was to evaluate the improvement of the outcome of the lobulus-type microtia reconstruction. Patient satisfaction was also evaluated. There are no previous reports of the learning process in this field. Postoperative photographs of 51 microtia reconstructions were assessed and rated by a panel made up of six surgeons. The ratings were gathered to generate learning curves. Twenty-two patients assessed the outlook of their reconstructed ears, and the results were analyzed as a self-assessment group. The reliability of the rating by a panel was tested by intraclass correlations. There is a highly significant increasing trend in learning ( P = 0.000001). This trend is not constantly upward, and the steady state was not reached during the study. In the self-assessment group, females were significantly more critical than males ( P = 0.014). Intraclass correlation for six panel members was 0.90, and the rating was considered reliable. Thus, a long and gentle learning curve does exist in microtia reconstruction. To secure good quality and continuity, centralization of the operations and trainee arrangements are highly advisable. Outcomes of plastic surgery can reliably be rated by an evaluation panel.

[Microtia--not just an ear problem]. / Mikrotia--ei ainoastaan korvaongelma.

Microtia can be defined as a malformation of the auricle with varying severity. In the majority of patients it is combined with atresia or stenosis of the external auditory canal. The prevalence of microtia in Finland is approx. 4 out of 10000. Associated anomalies should be actively seeked and excluded. Approximately 70% of the patients seem to be non-syndromatic. Patients with microtia are known to more than usual have other structural abnormalities. With the exception of the mildest cases, the development of facial structure and dental occlusion should be monitored in all microtia patients, attempting to actively exclude any associated diseases.

Inheritance of microtia in the Finnish population.

OBJECTIVE: To study the inheritance of microtia in the Finnish population, identify families for genetic linkage analyses and compare the phenotype between sporadic and familial patients. METHODS: Retrospective case series and patient questionnaire of 109 microtia patients referred for reconstruction of the earlobe to the Helsinki University Central Hospital during the years 1980-2005. RESULTS: 22 out of the 109 patients had a relative with microtia or preauricular tag. The familial and sporadic patients did not differ in microtia phenotype or sex distribution. Urinary system anomalies were statistically more prevalent in familial patients (p<0.01). The analyses of the birthplace of parents or grandparents of familial or sporadic microtia patients resulted in no evidence for founder effect. CONCLUSIONS: The prevalence of familial microtia/OAVS in the Finnish population is higher than 20%. The sporadic and familial microtia/OAVS patients do not differ in the phenotype or sex distribution. The mode of inheritance seems to be autosomal dominant with incomplete penetrance.

Microtia in Finland: comparison of characteristics in different populations.

OBJECTIVE: To compare the characteristics of microtia in Finland and in other populations. METHODS: Retrospective case series and patient questionnaire of 190 microtia patients referred for reconstruction of the earlobe to the Helsinki University Central Hospital during the years 1980-2005. RESULTS: The prevalence in Finland is 4.34/10,000 and varied in other populations from 0.83 to 17.4/10,000. Microtia is seen more in males (58%), as unilateral (88.4%), right-sided (59.5%) and it is almost always associated with aural atresia or stenosis (93%). There is conductive hearing loss in 96% and sensorineural hearing loss in 8% of the affected ears. 11% of the patients had congenital heart defects, and 5% had anomalies of extremities. CONCLUSIONS: There is variation in the prevalence and characteristics of microtia in different populations.