Hearing instruments for unilateral severe-to-profound sensorineural hearing loss in adults

Objectives: A systematic review of the literature and meta-analysis was conducted to assess the nature and quality of the evidence for the use of hearing instruments in adults with a unilateral severe-to-profound sensorineural hearing loss. Design: The PubMed, EMBASE, MEDLINE, Cochrane, CINAHL and DARE databases were searched with no restrictions on language. The search included articles from the start of each database until 11th February 2015. Studies were included that: (a) assessed the impact of any form of hearing instrument, including devices that re-route signals between the ears or restore aspects of hearing to a deaf ear, in adults with a sensorineural severe-to-profound loss in one ear and normal or near-normal hearing in the other ear; (b) compared different devices or compared a device to placebo or the unaided condition; (c) measured outcomes in terms of speech perception, spatial listening, or quality of life; (d) were prospective controlled or observational studies. Studies that met prospectively-defined criteria were subjected to random-effects meta-analyses. Results: Twenty-seven studies reported in thirty articles were included. The evidence was graded as low-to-moderate quality having been obtained primarily from observational before-after comparisons. The meta-analysis identified statistically-significant benefits to speech perception in noise for devices that re-routed the speech signals of interest from the worse ear to the better ear using either air or bone conduction (mean benefit 2.5 dB). However, these devices also degraded speech understanding significantly and to a similar extent (mean deficit 3.1 dB) when noise was re-routed to the better ear. Data on the effects of cochlear implantation on speech perception could not be pooled as the prospectively-defined criteria for meta-analysis were not met. Inconsistency in the assessment of outcomes relating to sound localisation also precluded the synthesis of evidence across studies. Evidence for the relative efficacy of different devices was sparse but a statistically significant advantage was observed for re-routing speech signals using abutment-mounted bone conduction devices when compared to outcomes after pre-operative trials of air-conduction devices when speech and noise were co-located (mean benefit 1.5 dB). Patients reported significant improvements in hearing-related quality of life with both re-routing devices and following cochlear implantation. Only two studies measured health-related quality of life and findings were inconclusive. Conclusions: Devices that re-route sounds from an ear with a severe-to-profound hearing loss to an ear with minimal hearing loss may improve speech perception in noise when signals of interest are located towards the impaired ear. However, the same device may also degrade speech perception as all signals are re-routed indiscriminately, including noise. While the restoration of functional hearing in both ears through cochlear implantation could be expected to provide benefits to speech perception, the inability to synthesise evidence across existing studies means that such a conclusion cannot yet be made. For the same reason, it remains unclear whether cochlear implantation can improve the ability to localise sounds despite restoring bilateral input. Prospective controlled studies that measure outcomes consistently and control for selection and observation biases are required to improve the quality of the evidence for the provision of hearing instruments to patients with unilateral deafness and to support any future recommendations for the clinical management of these patients

Right Paraduodenal Hernia in an Adult Patient: Diagnostic Approach and Surgical Management

Paraduodenal hernia, a rare congenital anomaly which arises from an error of rotation of the midgut, is the most common type of intraabdominal hernia. There are two variants, right and left paraduodenal hernia, the right being less common. We report the case of a 41-year-old patient with a right paraduodenal hernia with a 6-month history of intermittent episodes of intestinal obstruction. Diagnosis was established by CT scan and upper gastrointestinal series with small bowel follow-through. In a planned laparotomy, herniation of the small bowel loops through the fossa of Waldeyer was found. Division of the lateral right attachments of the colon opened the hernia sac widely, replacing the pre- and postarterial segments of the intestine in the positions they would normally occupy at the end of the first stage of rotation during embryonic development. Six months after the surgery, after an uneventful recovery, the patient remains free of symptoms

Genetic regulation of pituitary gland development in human and mouse

Normal hypothalamopituitary development is closely related to that of the forebrain and is dependent upon a complex genetic cascade of transcription factors and signaling molecules that may be either intrinsic or extrinsic to the developing Rathke’s pouch. These factors dictate organ commitment, cell differentiation, and cell proliferation within the anterior pituitary. Abnormalities in these processes are associated with congenital hypopituitarism, a spectrum of disorders that includes syndromic disorders such as septo-optic dysplasia, combined pituitary hormone deficiencies, and isolated hormone deficiencies, of which the commonest is GH deficiency. The highly variable clinical phenotypes can now in part be explained due to research performed over the last 20 yr, based mainly on naturally occurring and transgenic animal models. Mutations in genes encoding both signaling molecules and transcription factors have been implicated in the etiology of hypopituitarism, with or without other syndromic features, in mice and humans. To date, mutations in known genes account for a small proportion of cases of hypopituitarism in humans. However, these mutations have led to a greater understanding of the genetic interactions that lead to normal pituitary development. This review attempts to describe the complexity of pituitary development in the rodent, with particular emphasis on those factors that, when mutated, are associated with hypopituitarism in humans

Psychometric Validation of the FACE-Q Craniofacial Module for Facial Nerve Paralysis

Background: Systematic reviews have identified the need for a patient-reported outcome measure for facial nerve paralysis (FNP). The aim of this study was to determine the psychometric properties of FACE-Q Craniofacial module scales when used in a combined sample of children and older adults with FNP. Methods: Data were collected between December 2016 and December 2019. We conducted qualitative interviews with children and adults with FNP. FACE-Q data were collected from patients aged 8 years and older with FNP. Rasch measurement theory analysis was used to examine the reliability and validity of the relevant scales in the FNP sample. Results: Twenty-five patients provided 2052 qualitative codes related to appearance, physical, psychological, and social function. Many patient concerns were common across age. The field-test sample included 235 patients aged 8-81 years. Of the 13 scales examined, all 122 items had ordered thresholds and good item fit to the Rasch model. For 12 scales, person separation index values were ≥0.79 and Cronbach's alpha values were ≥0.82. The 13th scale's reliability values were ≥0.71. Conclusion: The FACE-Q Craniofacial module scales described in this study can be used to collect and compare evidence-based outcome data from children and adults with FNP

FACE-Q craniofacial module: Part 2 Psychometric properties of newly developed scales for children and young adults with facial conditions

Background: The FACE-Q Craniofacial Module is a patient-reported outcome measure designed for patients aged 8 to 29 years with conditions associated with a facial difference. In part 1, we describe the psychometric findings for the original CLEFT-Q scales tested in patients with cleft and noncleft facial conditions. The aim of this study was to examine psychometric performance of new FACE-Q Craniofacial Module scales. Methods: Data were collected between December 2016 and December 2019 from patients aged 8 to 29 years with conditions associated with a visible or functional facial difference. Rasch measurement theory (RMT) analysis was used to examine psychometric properties of each scale. Scores were transformed from 0 (worst) to 100 (best) for tests of construct validity. Results: 1495 participants were recruited with a broad range of conditions (e.g., birthmarks, facial paralysis, craniosynostosis, craniofacial microsomia, etc.) RMT analysis resulted in the refinement of 7 appearance scales (Birthmark, Cheeks, Chin, Eyes, Forehead, Head Shape, Smile), two function scales (Breathing, Facial), and an Appearance Distress scale. Person separation index and Cronbach alpha values met criteria. Three checklists were also formed (Eye Function, and Eye and Face Adverse Effects). Significantly lower scores on eight of nine scales were reported by participants whose appearance or functional difference was rated as a major rather than minor or no difference. Higher appearance distress correlated with lower appearance scale scores. Conclusion: The FACE-Q Craniofacial Module scales can be used to collect and compare patient reported outcomes data in children and young adults with a facial condition