Otologic and audiologic findings in 22q11.2 deletion syndrome.

Hearing loss is frequently present in the 22q11.2 deletion syndrome. Our aim was to describe the audiologic and otologic features of patients with 22q11.2 deletion syndrome. We conducted a retrospective cohort study in a single tertiary referral center. We reviewed medical files of all patients with 22q11.2 deletion syndrome who visited an otolaryngologist, plastic surgeon or speech therapist, for audiologic or otologic features. Hearing loss was defined as a pure tone average (of 0.5, 1, 2, and 4 kHz) of >20 decibel hearing level. Audiograms were available for 102 of 199 included patients, out of which 163 ears were measured in the required frquencies (0.5-4 kHz). Median age at time of most recent audiogram was 7 years (range 3-29 years). In 62 out of 163 ears (38%), hearing loss was present. Most ears had conductive hearing loss (n = 58) and 4 ears had mixed hearing loss. The severity of hearing loss was most frequently mild (pure tone average of ≤40 decibel hearing level). In 22.5% of ears, otitis media with effusion was observed at time of most recent audiogram. Age was not related to mean air conduction hearing thresholds or to otitis media with effusion (p = 0.43 and p = 0.11, respectively). In conclusion, hearing loss and otitis media are frequently present in patients with 22q11.2 deletion syndrome. Moreover, our results suggest that children with 22q11.2 deletion syndrome remain susceptible for otitis media as they age.

Systematic review of the diagnostic value of laryngeal stroboscopy in excluding early glottic carcinoma.

OBJECTIVE: Laryngeal stroboscopy is an important diagnostic tool in the work-up of patients with phoniatric complaints. The aim of this article was to evaluate the diagnostic value of laryngeal stroboscopy in excluding glottic carcinoma in patients with suspected glottic carcinoma. DATA SOURCES: PubMed, Embase and the Cochrane Library. REVIEW METHODS: A systematic literature search was performed in PubMed, Embase and the Cochrane Library. Studies reporting original study data were included. Studies were selected based on predefined inclusion and exclusion criteria and were consequently systematically assessed for their relevance and risk of bias. Studies with low relevance, high risk of bias or both were excluded from analysis. The prevalences, sensitivities, specificities and post-test probabilities of laryngeal stroboscopy for detecting glottic carcinoma were extracted or calculated with data supplied in the articles. RESULTS: Seven studies, with high relevance and moderate risk of bias, were selected for data extraction. The baseline risk of glottic carcinoma was 25-49% in the included studies. Sensitivity ranged between 80% and 100% and specificity between 25% and 100%. Post-test probabilities of malignancy in case of an abnormal stroboscopy ranged between 35% and 100%. Post-test probabilities of malignancy in case of a normal stroboscopy ranged between 0% and 29%. CONCLUSION: A normal laryngeal stroboscopic result predicts the absence of invasive carcinoma. Laryngeal stroboscopy can therefore be suggested as a useful supplemental examination in the standard diagnostic work-up for all patients with suspected glottic carcinoma.

Retrospective complication rate comparison between surgical techniques in paediatric cochlear implantation.

OBJECTIVE: To compare paediatric complication occurrence between the Mastoidectomy with Posterior Tympanotomy and the SupraMeatal Approach for cochlear implantation. DESIGN: Retrospective cohort study. SETTING: Children receiving a cochlear implant before 5 years of age between 1996 and 2014 in our tertiary center. PARTICIPANTS: A total of 144 patients receiving a cochlear implant (121 by Mastoidectomy with Posterior Tympanotomy and 23 by SupraMeatal Approach) operated on 165 ears (129 and 39 respectively). MAIN OUTCOME MEASURES: The severity (minor or major) using Cohen and Hoffman criteria and time of occurrence of complications (intraoperative, early postoperative or late postoperative) were identified. Intraoperative surgical challenges were correlated to complication occurrence. RESULTS: The mean age at implantation was 2.13 ± 1.14 years old. Patients operated by the SupraMeatal Approach (1.27 ± 0.69 years old) were significantly (P < .001) younger than those receiving a cochlear implant by Mastoidectomy with Posterior Tympanotomy Approach (2.40 ± 1.12). Most complications were minor (Mastoidectomy with Posterior Tympanotomy Approach: 64.0%; SupraMeatal Approach: 73.1%) and occurred early postoperatively (Mastoidectomy with Posterior Tympanotomy Approach: 61.5%; SupraMeatal Approach: 76.9%). More overall complications occurred in SupraMeatal compared to Mastoidectomy with Posterior Tympanotomy Approach cases (61.5% versus 20.6%; P < .001). Younger SupraMeatal Approach cohort patients (6 - 12 and 18 - 24 months; P < .008 and P = .016) most often developed these complications. When looking at specific complications, more infectious complications occurred in patients receiving a cochlear implant through the SupraMeatal Approach (P < .05). Logistic regression showed that the surgical technique and not the age at implantation was responsible for the documented complications. No relationship between complications and intraoperative difficulties was identified. CONCLUSION: In our institution, cochlear implantation in young patients through the SupraMeatal Approach resulted in significantly more (infectious) complications than those operated through the Mastoidectomy with Posterior Tympanotomy Approach. Outcomes from our institution recommends using the Mastoidectomy with Posterior Tympanotomy Approach when opting for a cochlear implant surgical technique in young children who are more prone to develop infectious complications.

Quality of life questionnaires in otorhinolaryngology: a systematic overview.

BACKGROUND: The importance of quality of life (QOL) as an endpoint and the use of validated QOL questionnaires have increased over time. OBJECTIVES: To evaluate health-related quality of life (HR-QOL) measurement instruments used in patients in otorhinolaryngology (ORL). We aimed to establish the use of QOL questionnaires in ORL over a period of time, establish the use of QOL questionnaires within different domains and determine the use of validated QOL questionnaires. MAIN OUT COME MEASURES: We performed a comprehensive search in PubMed up to 1 January 2014. Articles were included that measured HR-QOL questionnaires in clinical practice in children, adolescents or adults in 42 journals of ORL. Multiple unique QOL questionnaires, organised according to domain, time and survey of validation, were extracted from reported articles. RESULTS: Of 2442 articles, we utilised 1196 publications with a total of 2103 QOL questionnaires regarding ORL. We evaluated a variety of 363 unique QOL questionnaires in which 60% (n = 220) QOL questionnaires had been validated. We found a continuing increase in the amount of articles which used QOL questionnaires since the beginning of the 20th century, while the percentage of validated QOL questionnaires remained the same (76%). Most QOL questionnaires were used in the domains oncology (35%), otology (21%) and rhinology (20%). The domain otology had the largest amount of unique QOL questionnaires (n = 122). CONCLUSIONS: We identified and evaluated all unique HR-QOL questionnaires utilised in patients in ORL. Recently, the use of validated and non-validated HR-QOL questionnaires has increased within all domains of ORL. The assessment of QOL has become an important outcome measure in clinical practice, in medical research and for healthcare organisations.

Factors that influence outcomes in cochlear implantation in adults, based on patient-related characteristics - a retrospective study.

OBJECTIVE: Outcomes in speech perception following cochlear implantation in adults vary widely. Many studies have been carried out to identify and quantify factors that influence outcomes. This study adds a new dimension to pre-existing literature. DESIGN: Single-centre retrospective cohort study. SETTING: University Medical Center Utrecht, the Netherlands. PARTICIPANTS: A total of 428 adults with bilateral severe-to-profound sensorineural hearing loss, unilaterally implanted between February 1988 and March 2014. MAIN OUTCOME MEASURES: Univariable and multivariable linear regression analyses were carried out to identify factors that may influence outcome after cochlear implantation. Consonant-vowel-consonant word scores were recorded pre- and post-implant and were used as outcome measure in two groups of patients (prelingually and postlingually deafened adults). As an added dimension, multiple imputation was implemented and evaluated to tackle 4% (17/407) missing data. RESULTS: For postlinguals, pre-implant speech perception score and age at onset of deafness are positive predictors and meningitis and otosclerosis as cause of deafness are negative predictors of post-implant speech perception. This model accounted for 26% of variance. For prelinguals, pre-implant speech perception score is the only strong positive predictor (ß 0.524; P < 0.001). This model accounted for 31% of variance. Age at implantation was not a significant predictor in either group. CONCLUSIONS: Speech perception is predicted by pre-implant speech perception, age at onset of deafness and aetiology (meningitis and otosclerosis) for postlinguals and solely pre-implant speech perception for prelinguals. Age at implantation is of lesser importance in predicting speech perception outcome post-implant. Multiple imputation is a useful statistical technique when analysing incomplete data sets.

Agreement between health utility instruments in cochlear implantation.

OBJECTIVES: The objectives of our study were threefold: to compare health utility scores measured with different health utility instruments in adult patients with bilateral deafness, to compare the change in health utility scores after unilateral or bilateral cochlear implantation using the different health utility instruments and to assess which health utility instrument would be the most appropriate for future studies on cochlear implantation. DESIGN: A prospective study. SETTING: The data for this article were collected as part of a multicentre randomised controlled trial in the Netherlands on the benefits of simultaneous bilateral cochlear implantation compared to unilateral cochlear implantation. PARTICIPANTS: The study included 38 adult patients with severe to profound bilateral post-lingual sensorineural hearing loss. MAIN OUTCOME MEASURES: Participants completed various quality of life questionnaires (the EuroQol five-dimensional questionnaire (EQ-5D), the Health Utilities Index mark 3 (HUI3), a visual analogue scale (VAS) for general quality of life and a VAS for hearing) preoperatively, and one and two years postoperatively. The general health utility instruments (EQ-5D, HUI3 and VAS general) were compared. RESULTS: The EQ-5D, HUI3 and VAS general utility scores differed significantly. The intraclass correlation coefficients showed poor to no agreement between these instruments. A gain in health utility after cochlear implantation was found with the HUI3 and VAS general. The highest gain in health utility was found with the HUI3. CONCLUSIONS: A health utility score depends on the health utility instrument that is used in a specific patient population. We recommend using the HUI3 in future studies on cochlear implantation.

The value of postoperative anticoagulants to improve flap survival in the free radial forearm flap: a systematic review and retrospective multicentre analysis.

BACKGROUND: Free radial forearm flap (FRFF) reconstruction is a valuable technique in head and neck surgery, which allows closure of large defects while striving to maintain functionality. Anticoagulative drugs are often administered to improve flap survival, although evidence regarding effectiveness is lacking. OBJECTIVE OF REVIEW: To investigate the effectiveness of postoperative anticoagulants to improve survival of the FRFF in head and neck reconstruction. TYPE OF REVIEW: Systematic review and multicentre, individual patient data meta-analysis. SEARCH STRATEGY: MEDLINE, EMBASE, Web of Science and CINAHL were searched for synonyms of 'anticoagulants' and 'free flap reconstruction'. EVALUATION METHOD: Studies were critically appraised for directness of evidence and risk of bias. Authors of the highest quality publications were invited to submit their original data for meta-analysis. RESULTS: Five studies were of adequate quality, and data from four studies (80%) were available for meta-analysis, describing 759 FRFF procedures. Anticoagulants used were as follows: aspirin (12%), low molecular weight dextran (18.3%), unfractioned heparin (28.1%), low molecular weight heparin (49%) and prostaglandin-E1 (2.1%). Thirty-one per cent did not receive anticoagulants. Flap failure occurred in 40 of 759 patients (5.3%) On univariate analysis, use of unfractioned heparin was associated with a higher rate of flap failure. However, these regimens were often administered to patients who had revision surgery of the anastomosis. In multivariate logistic regression analysis, anticoagulant use was not associated with improved flap survival or flap-related complications. CONCLUSIONS: The studied anticoagulative drugs did not improve FRFF survival or lower the rate of flap-related complications. In addition, some anticoagulants may cause systemic complications.

Comparison of two cochlear implantation techniques and their effects on the preservation of residual hearing. Is the surgical approach of any importance?

The goal of this work was to review the pre-and postsurgical auditory thresholds of two surgical implantation techniques, namely the mastoidectomy with posterior tympanotomy approach (MPTA) and suprameatal approach (SMA), to determine whether there is a difference in the degree of preservation of residual hearing. In a series of 430 consecutive implanted patients 227 patients had measurable pre-operative hearing thresholds at 250, 500, and 1,000 Hz. These patients were divided into two groups according to the surgical technique that was used for implantation. The SMA approach was followed for 84 patients in Amsterdam, whereas the MPTA technique was adhered to 143 patients in Maastricht. The outcome variables of interest were alteration of pre-and postoperative auditory thresholds after cochlear implantation. Complete or partial preservation of residual hearing was obtained in 21.4 and 21.7% in the SMA and MPTA group, respectively. No statistical differences could be found between the SMA and MPTA group (p = 0.96; Chi-square test). The SMA technique is correlated with a similar degree of hearing loss after cochlear implantation compared to the MPTA technique. However, both techniques were not able to conserve a measurable amount of hearing in patients with a substantial degree of residual hearing. Therefore, both surgical techniques need to be refined for patients in which residual acoustical hearing is pursued.

Surgery of the external nasal valve: the correlation between subjective and objective measurements.

OBJECTIVES/HYPOTHESIS: Minimum cross-sectional area of the nasal passage on CT (CT-MCA) is an objective computerised determination of the minimum cross-sectional area of the nasal passage on CT. CT-MCA was evaluated before and after surgery on the external nasal valve using the 'lateral crus pull-up' procedure (LCPU). The outcomes of CT-MCA were compared with other currently available objective tests for nasal valve patency. STUDY DESIGN: Prospective cohort study. METHODS: This study included 34 patients undergoing surgery on the external nasal valve with the use of the LCPU technique. CT-MCA was performed before and after surgery and compared with the subjective perception of nasal passage using the Nasal Obstruction Symptom Evaluation (NOSE) scale and with objective tests such as acoustic rhinometry (A-MCA), rhinomanometry (NAR) and peak nasal inspiratory flow (PNIF). RESULTS: This study showed a significant correlation between CT-MCA and the NOSE scale, PNIF and NAR. Paired-samples t-tests showed significant improvement after surgery on CT-MCA, PNIF and the NOSE scale. Multiple linear regression analysis showed that PNIF, CT-MCA and NAR were significantly associated with the NOSE scale. CONCLUSION: CT-MCA and PNIF were both significantly correlated and associated with the patient's subjective perception of nasal passage. The surgical procedure, the 'lateral crus pull-up', showed a significant improvement in the postoperative result both subjectively and objectively.

Benefit of sequential bilateral cochlear implantation in children between 5 to 18 years old: A prospective cohort study.

OBJECTIVE: To determine the benefit of sequential cochlear implantation after a long inter-implantation interval in children with bilateral deafness receiving their second implant between 5 and 18 years of age. STUDY DESIGN: Prospective cohort-study. SETTING: Tertiary multicenter. PATIENTS: 85 children with bilateral deafness and unilateral implantation receiving a contralateral cochlear implant at the age of 5 to 18 years. METHOD: The primary outcomes were speech recognition in quiet and noise (CVC) scores. The secondary outcomes were language outcomes and subjective hearing abilities, all measured before and 12 months after sequential bilateral cochlear implantation. Medians of the paired data were compared using the Wilcoxon signed-rank test. Univariable linear regression analyses was used to analyze associations between variables and performance outcomes. RESULTS: A significant benefit was found for speech recognition in quiet (96% [89-98] vs 91% [85-96]; p < 0.01) and noise (65% [57-75] vs 54% [47-71]; p = 0.01) in the bilateral CI condition compared to unilateral (n = 75, excluded 10 non-users). No benefit was seen for language outcomes. The subjective sound quality score was statistically significant higher in bilateral compared to the unilateral CI condition. Pre-operative residual hearing level in the ear of the second implant, the inter-implant interval and age at time of second implantation was not significantly associated with performance scores. CONCLUSION: After 12 months of use, sequential bilateral cochlear implantation showed improved speech perception in quiet and noise and improved subjective sound quality outcomes in children despite a great inter-implantation interval (median of 8 years [range 1-16 years]).