Systematic review: Validity, reliability, and diagnostic accuracy of the electrogustometer.

Objective: What are the electrogustometer's (EGM) validity, reliability, and diagnostic accuracy in assessing taste sensation in adults compared to other taste tests? Data Sources: PubMed Medline, Elseviers's Embase, and the six databases of Cochrane Library. Methods: We conducted a systematic search on December 20, 2022, consisting of synonyms for EGM. We considered randomized controlled trials and observational studies with original data for inclusion if they included adults who underwent electrogustometry. Articles were excluded if no analysis regarding validity, reliability, or diagnostic accuracy had been performed or if these analyses could not be performed with the published data. Results: Nineteen articles discussing 18 studies were included for data extraction. The included studies carry a high risk of bias. Overall, the association between a variety of reference taste tests and EGM was moderate or weak with correlation coefficients ranging from -0.51 to 0.40 with one outlier of -0.74 found in one study correlating EGM and taste solutions. Test-retest reliability was good with reported correlation coefficients between 0.78 and 1.0. The sensitivity, specificity, PPV, and NPV of EGM in identifying abnormal taste function varied widely between the four studies on diagnostic accuracy. Conclusion: The included studies in this review lack the required standards regarding study design to draw firm conclusions about the validity, reliability, and diagnostic accuracy of the EGM. Future research is needed to assess these measurement properties. Based on the reported results, we would not recommend using the EGM as a screening test for taste disturbance in clinical practice. Level of Evidence: NA.

Endoscopic vs. microscopic stapes surgery: An anatomical feasibility study.

Objectives: To investigate the feasibility of the endoscopic approach vs. microscopic approach during stapes surgery, focusing on the visualization of the important anatomical structures of the middle ear, the volume of the resected scutum and chorda tympani (CT) injury. Methods: Fresh frozen human cadaveric heads underwent two stapes surgeries using an operating microscope on one ear and an endoscope on the other ear. The surgeon documented the visualization of critical landmarks, as well as exposure and injury of the CT. The volume of resected scutum was evaluated using cone beam computed tomography scanning and three-dimensional imaging. Results: We performed endoscopic stapes surgery in 10 ears and microscopic stapes surgery in 11 ears. A stapes prosthesis was placed in all ears. The volume of bony scutum resection was significantly lower in the endoscopic group (median = 2.20 mm3, IQR = 4.17) than in the microscopic group (median 13.25 mm3, IQR = 8.71). No scutum was removed in two endoscopic ears, while scutum was removed in all microscopic ears. The endoscopic and microscopic group had similar CT injury. Conclusions: This study showed that the endoscopic stapes surgery procedure is feasible and might be less invasive than microscopic stapes surgery. Future clinical prospective and functional studies will be needed to support our findings.

A Systematic Review on Factors Associated With Percutaneous Bone Anchored Hearing Implants Loss.

OBJECTIVE: To investigate factors associated with percutaneous bone anchored hearing implant (BAHI) loss. DATA SOURCES: Africa-Wide, Biosis, Cochrane, Embase, Global Health, LILACs, Medline, Pubmed, and Web of Science electronic databases. STUDY SELECTION: All studies reporting on adult and/or pediatric patients with a BAHI loss were identified. Retrieved articles were screened using predefined inclusion criteria. Eligible studies underwent critical appraisal for directness of evidence and risk of bias. Studies that successfully passed critical appraisal were included for data extraction. DATA EXTRACTION: Extracted data included study characteristics (study design, number of total implants and implant losses, follow-up), patient characteristics (sex, age, comorbidities, previous therapies), and information regarding BAHI loss (etiology of loss, timing of occurrence). DATA SYNTHESIS: From the 5,151 articles identified at the initial search, 847 remained after title and abstract screening. After full text review, 96 articles were eligible. Fifty-one articles passed quality assessment, however, due to overlapping study population, 48 articles reporting on 34 separate populations were chosen for data extraction. Three hundred one implant losses occurred out of 4,116 implants placed, resulting in an overall implant loss occurrence rate of 7.3%. Failed osseointegration was responsible for most implant losses (74.2%), followed by fixture trauma (25.7%). Most losses due to failed osseointegration occurred within 6 months of the implantation. BAHI implant loss occurred more frequently in pediatric patients (p < 0.005). CONCLUSION: The current systematic review identified factors associated with BAHI loss. These factors should be considered when assessing patients' candidacy and when investigating reasons for impeded implant stability and loss.

Congenital respiratory tract disorders in 22q11.2 deletion syndrome.

OBJECTIVE: Respiratory tract disorders have been reported in patients with 22q11.2 deletion syndrome, however infrequently. This study describes the respiratory tract disorders encountered in a cohort of 278 patients with 22q11.2 deletion syndrome. METHODS: We conducted a retrospective, cross-sectional, study at a single tertiary referral center. We identified the patients with 22q11.2 deletion syndrome and with an upper and/or lower respiratory tract disorder at our otorhinolaryngologic department. The different disorders were described. RESULTS: Out of 278 patients referred to the otorhinolaryngologic department, we identified 14 patients with a laryngeal and/or tracheal disorder. Nine patients had more than one congenital disorder in this anatomical area. Disorders included a choanal stenosis (n = 1), laryngeal web (n = 5), laryngeal cleft (n = 2), subglottic stenosis (n = 3), pharyngo-, laryngo-, tracheo- and/or bronchomalacia (n = 11) and tracheal stenosis (n = 1). CONCLUSION: Different types of respiratory tract disorders can be present in patients with 22q11.2 deletion syndrome. Clinicians should be aware of this clinical association for timely and accurate diagnosis and treatment. In addition, the diagnosis 22q11.2 deletion syndrome should be considered in patients presenting with a congenital respiratory tract disorder.

Middle ear abnormalities in Van Maldergem syndrome.

Van Maldergem syndrome (VMS) is a very rare syndrome that was first described in 1992. The main features of this syndrome comprise intellectual disability, blepharo-naso-facial malformation, and hand anomalies. Almost all nine described patients have been shown to be affected by conductive hearing impairment attributed to microtia, and atresia of the outer ear canal. Here, we present a VMS patient with congenital malformations of the middle ear as the main reason for severe conductive bilateral hearing impairment. To our knowledge, this is the first report to describe middle ear abnormalities in VMS. These malformations were seen on high resolution Computed Tomography scanning and during an exploratory tympanotomy. Due to the severity of the middle ear abnormalities and the risk for facial nerve damage, the patient was not offered an ossicular chain reconstruction but a bone conduction device after this exploratory tympanotomy. © 2016 Wiley Periodicals, Inc.

A Systematic Review on Complications of Tissue Preservation Surgical Techniques in Percutaneous Bone Conduction Hearing Devices.

OBJECTIVE: To investigate skin-related postoperative complications from tissue preservation approaches in percutaneous bone conduction device (BCD) implantations. DATA SOURCES: PubMed, Embase, and Cochrane Library. STUDY SELECTION: We identified studies on BCDs including the opted surgical technique and derived complications. Retrieved articles were screened using predefined inclusion criteria. Critical appraisal included directness of evidence and risk of bias. Studies that successfully passed critical appraisal were included. DATA EXTRACTION: Outcome measures included patient demographics, surgery time, follow-up time, and complications reported by Holgers classification. DATA SYNTHESIS: We selected 18 articles for data extraction; encompassing 381 BCDs implanted using non-skin thinning approaches. Four studies reported an implantation technique using the punch method (81 implants), 13 studies applied the linear incision technique without soft tissue reduction (288 implants) and one study used the Weber technique (12 implants). Holgers 3 was described in 2.5% following the punch technique, in 5.9% following the linear incision technique, and in no implants following the Weber technique. One patient was mentioned having Holgers 4, skin overgrowth was reported in 6 patients, and 10 studies compared their non-skin thinning technique with a skin-thinning technique. Overall, the soft tissue preservation technique had a similar or superior complication rate, shorter surgical time, and better and faster healing, compared with the soft tissue reduction technique. CONCLUSION: Tissue preservation surgical techniques for percutaneous BCDs have limited postoperative skin complication rates. Moreover, these techniques are suggested to have at least similar complications rates compared with skin-thinning techniques.

Congenital ossicular chain anomalies associated with a mobile stapes footplate: surgical results for 23 ears.

OBJECTIVES: We describe the audiometric results following surgery in a consecutive series of patients with a congenital ossicular middle ear disorder that was associated with a mobile stapes footplate. METHODS: We performed a retrospective analysis of patient charts from a tertiary referral center. A total of 23 patients (23 ears) underwent exploratory tympanotomy and ossicular reconstruction between 1986 and 2001. The main outcome measure was the audiometric results. RESULTS: Overall, we observed a mean gain in air conduction pure tone average of 17 dB (from 47 dB to 30 dB), a sensorineural deterioration of 3 dB, and a mean postoperative air-bone gap of 19 dB (mean preoperative air-bone gap of 38 dB). The air-bone gap closure was 20 dB or less in 15 of the 23 cases (65%), in agreement with the few results reported in the literature. Moreover, the audiometric results remained stable. In the syndromic group, the mean gain in air conduction was only 13 dB, which was worse than that observed for the nonsyndromic ears. CONCLUSIONS: Surgery for congenital ossicular chain anomalies with a concomitant mobile stapes footplate provides positive audiometric outcomes. Most ears had some sensorineural impairment (10 to 20 dB), which influenced the final hearing level attained after surgery. Preoperative assessment is mandatory to search for syndromal diagnoses, which might be important for patient counseling and prognosis.

Congenital stapes ankylosis associated with another ossicular chain anomaly: surgical results in 30 ears.

OBJECTIVE: To describe the audiometric results after stapes surgery in a consecutive series of patients with stapes footplate ankylosis combined with another ossicular middle ear anomaly. STUDY DESIGN: A retrospective analysis of charts collected between 1986 and 2001. SETTING: A tertiary referral center. PATIENTS: A total of 25 patients (30 ears) underwent exploratory tympanotomies and ossicular reconstruction. MAIN OUTCOME MEASURE: Audiometric results. RESULTS: Overall, a mean gain in air conduction of 18 dB (from 49 dB to 31 dB) and a mean postoperative air-bone gap (ABG) of 20 dB (mean preoperative ABG, 40 dB) were observed. The ABG closure was 20 dB or less in 70% of cases, which is in agreement with the few results reported in the literature. Moreover, the audiometric results remained stable. In the group of ears with a syndrome, the mean gain of air conduction was only 19 dB, which was comparable to that observed among nonsyndromic ears. CONCLUSIONS: Surgery for congenital stapes footplate ankylosis with a concomitant ossicular chain anomaly can provide worthwhile hearing improvement. The ABG closure was 20 dB or less in 21 of 30 ears (70%). Most ears had some sensorineural impairment (10-20 dB), which influenced the final hearing level after surgery. Over recent decades, the technique of the malleostapedotomy procedure has been improved. Preoperative assessment is mandatory for syndromal diagnoses, which might be important for patient counseling and prognosis.

Proximal symphalangism, hyperopia, conductive hearing impairment, and the NOG gene: 2 new mutations.

OBJECTIVES: To report on 2 families with proximal symphalangism syndrome and 2 new NOG gene mutations and to report on the outcomes of exploratory tympanotomy. STUDY DESIGN: Retrospective chart study. SETTING: Tertiary referral center. PATIENTS: A total of 6 patients, one of which underwent an exploratory tympanotomy, were examined from 2 families. INTERVENTION: Exploratory tympanotomy in 1 patient. MAIN OUTCOME MEASURES: Medical and otologic histories and postoperative hearing outcomes. RESULTS: In the patient that was operated upon, the preoperative air conduction hearing threshold of 55 dB was reduced to 41 dB with a residual air bone gap of 21 dB. Furthermore, deoxyribonucleic acid analysis revealed 2 different mutations: a heterozygous nonsense mutation in the NOG gene, c.391C>T (p.Gln131X), and a frameshift mutation in the NOG gene (NOG, c.304del (p.Ala102fs)). CONCLUSION: NOG gene mutations, which lead to aberrant noggin protein function, give rise to a large spectrum of clinical findings and different symphalangism syndromes. These syndromes are all allelic disorders within the Noggin phenotype spectrum. We report on 2 new mutations that are supplementary to those previously described in the literature.

Isolated congenital stapes ankylosis: surgical results in a consecutive series of 39 ears.

OBJECTIVES: We describe the audiometric results after stapes surgery in a consecutive series of patients with isolated stapes footplate ankylosis. METHODS: We performed a retrospective analysis of charts from 1986 to 2001 in a tertiary referral center, focusing on audiometric results. RESULTS: A total of 28 patients (39 ears) underwent stapedotomy or stapedectomy, and 5 patients (5 ears) had a syndromal diagnosis. Overall, we observed a mean gain in air conduction threshold of 21 dB (from 46 to 25 dB) and a mean postoperative air-bone gap of 14 dB (mean preoperative air bone gap, 36 dB). The postoperative air-bone gap was 10 dB or less in 46% of cases, in agreement with results reported in the literature. Moreover, the audiometric results remained stable. In the group of ears with a syndrome, the mean gain in air conduction threshold was only 12 dB (range, -13.8 to 32.5 dB), which was a worse outcome than that in the nonsyndromic ears. CONCLUSIONS: Surgery for isolated congenital stapes footplate ankylosis provides satisfactory audiometric outcomes. The postoperative air-bone gap was 10 dB or less in 18 of the 39 ears (46%) and 20 dB or less in 29 of the 39 ears (74%). Most ears had some sensorineural impairment (10 to 20 dB), which influenced the final hearing level after surgery. Preoperative assessment is mandatory to search for syndromal diagnoses, which might be important for patient counseling and prognosis.

Clinical presentation and the presence of hearing impairment in branchio-oculo-facial syndrome: a new mutation in the TFAP2A gene.

We report on the clinical presentation of branchio-oculo-facial (BOF) syndrome in 2 patients with mutations in the TFAP2A gene (OMIM 107580). This TFAP2A gene was recently shown to be involved in the causation of BOF syndrome. An overview of the literature on BOF syndrome is given based on clinical reports written in the period during which mutation analysis was not yet available for BOF syndrome. We also give descriptions of the mutations in the TFAP2A gene in our 2 new patients with BOF syndrome. Congenital conductive hearing impairments are described, including hearing rehabilitation and the results of ear surgery.