Long-term safety and subjective satisfaction of Bonebridge and Vibrant Soundbridge in congenital unilateral conductive hearing loss.

Purpose: The objective of this study was to assess and compare the long-term safety and subjective satisfaction levels of Bonebridge (BB) and Vibrant Soundbridge (VSB) in patients with congenital unilateral conductive hearing loss (UCHL).

Methods: The hearing effectiveness was measured using sound field hearing threshold (SFHT) and word recognition score (WRS). Long-term safety and subjective satisfaction levels were measured by questionnaires, including Speech, Spatial, and Qualities of Hearing Scale-12 (SSQ-12) and Abbreviated Profile of Hearing Aid Benefit (APHAB).

Improved radiological imaging of congenital aural atresia using flat-panel volume CT.

Background: Precise preoperative radiological evaluation of aural atresia is of utmost importance for surgical planning. Until now, multislice computed tomography (MSCT) has been used but it cannot adequately visualize small structures such as the stapes. Flat-panel volume CT (fpVCT) with its secondary reconstructions (fpVCT) offers a high-resolution visualization of the middle ear.

Management of Acquired Aural Atresia and Stenosis: A Case Series.

Acquired aural atresia and aural stenosis have high recurrence rates, which are challenging for ear, nose, and throat surgeons. This study aimed to discuss the management of acquired aural atresia and aural stenosis to obtain favorable outcomes. Four representative cases of different etiologies are presented, and a related literature review was conducted.

Clinical and molecular cytogenetic findings of cat eye syndrome and a 2-year-old patient with congenital aural atresia and hearing loss.

Background: Cat eye syndrome (CES) is a rare congenital disease frequently caused by a partial tetrasomy of the proximal long (q) arm of chromosome 22, due to a small supernumerary marker chromosome (sSMC). CES patients show remarkable phenotypic variability. Despite the progress of molecular cytogenetic technology, the cause of phenotypic variability and the genotype-phenotype correlations remain unknown.

Efficacy of vibrant sound bridge in congenital aural atresia: an updated systematic review.

Purpose: The indications of Vibrant Soundbridge (VSB) have been expanded to include patients with conductive and mixed hearing loss due to congenital aural atresia (CAA). However, the current evidence supporting the auditory outcomes of VSB is based mainly on case reports and retrospective chart reviews. Therefore, the present systematic review aims to summarize and critically appraise the current evidence regarding the safety and effectiveness of VSB in children and adult patients with CAA.

Active Bone Conduction Implant and Adhesive Bone Conduction Device: A Comparison of Audiological Performance and Subjective Satisfaction.

 Atresia of the external auditory canal affects 1 in every 10 thousand to 20 thousand live births, with a much higher prevalence in Latin America, at 5 to 21 out of every 10 thousand newborns. The treatment involves esthetic and functional aspects. Regarding the functional treatment, there are surgical and nonsurgical alternatives like spectacle frames and rigid and softband systems.

CMV-induced Hearing Loss.

Congenital cytomegalovirus (cCMV) infection is the most common fetal viral infection and contributes to about 25% of childhood hearing loss by the age of 4 years. It is the leading nongenetic cause of sensorineural hearing loss (SNHL). Infants born to seroimmune mothers are not completely protected from SNHL, although the severity of their hearing loss may be milder than that seen in those whose mothers had a primary infection.

Changes in Listening Effort Through Pupil Response After Atresioplasty in Children With Congenital Aural Atresia.

Objectives: This study aimed to determine whether the improvement of hearing by surgical treatment alleviates cognitive demands through pupil response in patients with unilateral congenital aural atresia (CAA).

Design: A prospective study was performed on patients with unilateral CAA who were scheduled to undergo primary atresioplasty between November 2017 and May 2020. Pure-tone audiometry, auditory digit span test, Korean Speech Perception in Noise test, pupil measurement during speech tests, and questionnaires (Sound-Spatial-Qualities of Hearing Scale; subjective listening effort rating) were performed before and 6 months after surgery.

Linear and Whorled Nevoid Hypermelanosis - A Case of Pigmentary Mosaicism.

Linear and whorled nevoid hypermelanosis is a rare skin pigmentation disorder, characterized by linear streaks and whorls of hyperpigmented macules along Blaschko's lines. Lesions are commonly restricted to the trunk, neck, and extremities, sparing the face, palms, soles, and mucosae. Associated with this, certain cardiovascular, musculoskeletal, neurological, and developmental anomalies have been reported in the literature.

Cause, severity, and efficacy of treatment for hearing loss in children with Trisomy 18: A single institution-based retrospective study.

Trisomy 18 is a common chromosomal aberration syndrome, characterized by variable clinical manifestations, including cardiovascular, pulmonary, genitourinary, and musculoskeletal findings, leading to a shorter survival and severe developmental delay in survivors. However, recently, intensive therapeutic intervention has allowed for prolonging survival. In terms of otological complications, only a limited number of relevant reports have been published.

Severe Unilateral Microtia with Aural Atresia, Hair White Patch, Stereotypes in a Young Boy with De novo 16p13.11 Deletion: Reasons for a New Genotype-Phenotype Correlation.

 Microtia is an uncommon congenital malformation ranging from mild anatomic structural abnormalities to partial or complete absence of the ear leading to hearing impairment. Congenital microtia may present as a single malformation (isolated microtia) or sometimes associated with other congenital anomalies involving various organs. Microtia has been classified in three degrees according to the complexity of the auricular malformation and to anotia referred to the total absence of the ear.