-
The Laryngoscope Jun 2023To evaluate speech outcomes and facial nerve stimulation (FNS) rates in patients with far advanced otosclerosis (FAO) after cochlear implantation. (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
To evaluate speech outcomes and facial nerve stimulation (FNS) rates in patients with far advanced otosclerosis (FAO) after cochlear implantation.
METHODS
A systematic review was performed using standardized methodology of Medline, EMBASE, PubMed, Cochrane, and Web of Science databases. Studies were included if adults with FAO underwent cochlear implantation. Exclusion criteria included concurrent otologic history (e.g., Meniere's disease, superior canal dehiscence), non-English-speaking implant users, case reports, abstracts, and letters/commentaries. Bias was assessed using the Newcastle-Ottawa Scale for cohort studies and the National Institute of Health Scale for case series. The primary outcome measure was speech discrimination and the secondary outcomes were rates of partial insertion and FNS.
RESULTS
Twenty-seven studies evaluated cochlear implantation in FAO. Due to the heterogeneity of testing methods, statistical pooling of speech discrimination was not feasible, but qualitative synthesis indicated a positive effect of implantation. Pooled rates of FNS were 18% (95% confidence interval, CI 12%-27%) and the rate of partial insertion was 10% (95% CI 7%-15%).
CONCLUSION
Cochlear implantation in FAO demonstrates significant gains in speech discrimination scores with higher rates of FNS and partial insertion. Laryngoscope, 133:1288-1296, 2023.
Topics: Adult; Humans; Cochlear Implantation; Otosclerosis; Retrospective Studies; Meniere Disease; Facial Nerve; Cochlear Implants; Speech Perception; Treatment Outcome
PubMed: 36082830
DOI: 10.1002/lary.30386 -
The Journal of International Advanced... Sep 2022Progression of contralateral hearing loss following otologic and neuro-otologic surgeries is a distressing and rare complication. The aim of this study was to... (Review)
Review
BACKGROUND
Progression of contralateral hearing loss following otologic and neuro-otologic surgeries is a distressing and rare complication. The aim of this study was to systematically review the suspected etiologies and audiological findings in adults who experienced contralateral hearing loss.
METHODS
PubMed/MEDLINE, PsycINFO, CINAHL, ISI Web of Science, Cochrane Library, EMBASE, and Scopus databases were searched for this scoping review. The current review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. No limits were placed on language or year of publication.
RESULTS
Of a total of 46 studies, 43 studies met the inclusion criteria reporting contralateral hearing loss. The included studies were classified into 3 different categories: contralateral hearing loss after skull base surgeries (n=21), contralateral hearing loss after middle ear surgeries (n=17), and contralateral hearing loss after traumatic lesions (n=5). The cerebrospinal fluid leakage and drill-generated noise were reported as the most reported etiology of contralateral hearing loss following skull base and middle ear surgeries, respectively. The onset of contralateral hearing loss varied from immediately to 18 months after surgery. The severity of contralateral hearing loss varied from a slight to a profound degree of hearing loss.
CONCLUSION
Our results highlighted that contralateral hearing loss should be considered following the skull base and middle ear surgeries. Furthermore, this rare complication should be noticed after traumatic lesions.
Topics: Adult; Audiology; Deafness; Hearing Loss; Humans; Language; Noise
PubMed: 36063099
DOI: 10.5152/iao.2022.21563 -
Journal of the American Medical... Mar 2022To report the effectiveness of, and barriers and facilitators to, hearing rehabilitation for care home residents with dementia. (Review)
Review
OBJECTIVES
To report the effectiveness of, and barriers and facilitators to, hearing rehabilitation for care home residents with dementia.
DESIGN
Systematic review.
SETTING AND PARTICIPANTS
Care home residents with dementia and hearing loss.
METHODS
No restrictions on publication date or language were set and gray literature was considered. Eligible studies were critically appraised and presented via a narrative review.
RESULTS
Sixteen studies, most of low to moderate quality, were identified. Hearing rehabilitation, including hearing devices, communication techniques, and visual aids (eg, flashcards), was reported to improve residents' communication and quality of life and reduce agitation, with improvements in staff knowledge of hearing loss and job satisfaction. Residents' symptoms of dementia presented barriers, for example, losing or not tolerating hearing aids. Low staff prioritization of hearing loss due to time pressures and lack of hearing-related training for staff were further barriers, particularly for residents who required assistance with hearing devices. Adopting a person-centered approach based on residents' capabilities and preferences and involving family members facilitated hearing device use.
CONCLUSIONS AND IMPLICATIONS
Residents with dementia can benefit from hearing rehabilitation. Identifying and implementing efficient, individualized hearing rehabilitation is necessary for those with complex cognitive needs. Increased funding and support for the social care sector is required to address systemic issues that pose barriers to hearing rehabilitation, including time pressures, lack of training for staff and access to audiology services for residents.
Topics: Dementia; Family; Hearing; Hearing Loss; Humans; Quality of Life
PubMed: 34921761
DOI: 10.1016/j.jamda.2021.11.011 -
European Archives of... Nov 2023This paper was aimed at estimating the prevalence and risk factors of hearing loss (HL) among the middle-aged and elderly in China. (Meta-Analysis)
Meta-Analysis Review
PURPOSE
This paper was aimed at estimating the prevalence and risk factors of hearing loss (HL) among the middle-aged and elderly in China.
METHODS
Databases including the CQVIP (VIP) Database, Chinese National Knowledge Infrastructure (CNKI), China Biology Medicine disc (CBMdisc), Wanfang, PubMed, Web of Science, Excerpta Medica Database (Embase) and the Cochrane Library were comprehensively searched. In this review, random-effect models were used for pooling the prevalence of HL and the odds ratios (ORs) of potential risk factors.
RESULTS
34 studies were included in the meta-analysis. HL among the middle-aged and elderly in China had a pooled prevalence of 45% (95% confidence interval (CI) 40-51%). There were significant differences in the prevalence of HL between males and females (47% vs. 42%), between different screening methods by self-report and pure-tone audiometry (44% vs. 46%), between the middle-aged and the elderly (18% vs. 52%), and between the uneducated and the educated (49% vs. 36%). In urban areas, the prevalence was slightly higher than that in rural areas (50% vs. 48%). The findings suggested that the middle-aged and elderly in the South Central China region (61%, 95% CI 45-78%) and Northwest China (57%, 95% CI 55-58%) were more likely to develop HL. In addition, it was confirmed that advanced age, being male, noise exposure history, hypertension and hyperglycemia were related to a higher prevalence of HL among middle-aged and older adults.
CONCLUSION
The prevalence of HL among the middle-aged and older population in China is 45%, nearly half of the total population. It is urgent to take great efforts to raise people's awareness of HL prevention and early hearing screening.
Topics: Aged; Female; Middle Aged; Humans; Male; Prevalence; Hearing Loss; Deafness; China; Risk Factors
PubMed: 37439927
DOI: 10.1007/s00405-023-08109-3 -
The Cochrane Database of Systematic... Apr 2020Obstructive sleep-disordered breathing (oSDB) is a condition encompassing breathing problems when asleep due to upper airway obstruction. In children, hypertrophy of the... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Obstructive sleep-disordered breathing (oSDB) is a condition encompassing breathing problems when asleep due to upper airway obstruction. In children, hypertrophy of the tonsils and/or adenoids is thought to be the commonest cause. As such, (adeno)tonsillectomy has long been the treatment of choice. A rise in partial removal of the tonsils over the last decade is due to the hypothesis that tonsillotomy is associated with lower postoperative morbidity and fewer complications.
OBJECTIVES
To assess whether partial removal of the tonsils (intracapsular tonsillotomy) is as effective as total removal of the tonsils (extracapsular tonsillectomy) in relieving signs and symptoms of oSDB in children, and has lower postoperative morbidity and fewer complications.
SEARCH METHODS
We searched the Cochrane ENT Trials Register; Central Register of Controlled Trials (CENTRAL); MEDLINE; EMBASE; CINAHL; Web of Science; ClinicalTrials.gov; ICTRP and additional sources for published and unpublished trials. The search date was 22 July 2019.
SELECTION CRITERIA
Randomised controlled trials (RCTs) comparing the effectiveness of (adeno)tonsillectomy with (adeno)tonsillotomy in children aged 2 to 16 years with oSDB.
DATA COLLECTION AND ANALYSIS
We used standard Cochrane methods and assessed the certainty of the evidence for our pre-defined outcomes using GRADE. Our primary outcomes were disease-specific quality of life, peri-operative blood loss and the proportion of children requiring postoperative medical intervention (with or without hospitalisation). Secondary outcomes included postoperative pain, return to normal activity, recurrence of oSDB symptoms as a result of tonsil regrowth and reoperation rates.
MAIN RESULTS
We included 22 studies (1984 children), with predominantly unclear or high risk of bias. Three studies used polysomnography as part of their inclusion criteria. Follow-up duration ranged from six days to six years. Although 19 studies reported on some of our outcomes, we could only pool the results from a few due both to the variety of outcomes and the measurement instruments used, and an absence of combinable data. Disease-specific quality of life Four studies (540 children; 484 (90%) analysed) reported this outcome; data could not be pooled due to the different outcome measurement instruments used. It is very uncertain whether there is any difference in disease-specific quality of life between the two surgical procedures in the short (0 to 6 months; 3 studies, 410 children), medium (7 to 13 months; 2 studies, 117 children) and long term (13 to 24 months; 1 study, 67 children) (very low-certainty evidence). Peri-operative blood loss We are uncertain whether tonsillotomy reduces peri-operative blood loss by a clinically meaningful amount (mean difference (MD) 14.06 mL, 95% CI 1.91 to 26.21 mL; 8 studies, 610 children; very low-certainty evidence). In sensitivity analysis (restricted to three studies with low risk of bias) there was no evidence of a difference between the groups. Postoperative complications requiring medical intervention (with or without hospitalisation) The risk of postoperative complications in the first week after surgery was probably lower in children who underwent tonsillotomy (4.9% versus 2.6%, risk ratio (RR) 1.75, 95% CI 1.06 to 2.91; 16 studies, 1416 children; moderate-certainty evidence). Postoperative pain Eleven studies (1017 children) reported this outcome. Pain was measured using various scales and scored by either children, parents, clinicians or study personnel. When considering postoperative pain there was little or no difference between tonsillectomy and tonsillotomy at 24 hours (10-point scale) (MD 1.09, 95% CI 0.88 to 1.29; 4 studies, 368 children); at two to three days (MD 0.93, 95% CI -0.14 to 2.00; 3 studies, 301 children); or at four to seven days (MD 1.07, 95% CI -0.40 to 2.53; 4 studies, 370 children) (all very low-certainty evidence). In sensitivity analysis (restricted to studies with low risk of bias), we found no evidence of a difference in mean pain scores between groups. Return to normal activity Tonsillotomy probably results in a faster return to normal activity. Children who underwent tonsillotomy were able to return to normal activity four days earlier (MD 3.84 days, 95% CI 0.23 to 7.44; 3 studies, 248 children; moderate-certainty evidence). Recurrence of oSDB and reoperation rates We are uncertain whether there is a difference between the groups in the short (RR 0.26, 95% CI 0.03 to 2.22; 3 studies, 186 children), medium (RR 0.35, 95% CI 0.04 to 3.23; 4 studies, 206 children) or long term (RR 0.21 95% CI 0.01 to 4.13; 1 study, 65 children) (all very low-certainty evidence).
AUTHORS' CONCLUSIONS
For children with oSDB selected for tonsil surgery, tonsillotomy probably results in a faster return to normal activity (four days) and in a slight reduction in postoperative complications requiring medical intervention in the first week after surgery. This should be balanced against the clinical effectiveness of one operation over the other. However, this is not possible to determine in this review as data on the long-term effects of the two operations on oSDB symptoms, quality of life, oSDB recurrence and need for reoperation are limited and the evidence is of very low quality leading to a high degree of uncertainty about the results. More robust data from high-quality cohort studies, which may be more appropriate for detecting differences in less common events in the long term, are required to inform guidance on which tonsil surgery technique is best for children with oSDB requiring surgery.
Topics: Adolescent; Blood Loss, Surgical; Child; Child, Preschool; Humans; Pain, Postoperative; Palatine Tonsil; Postoperative Complications; Quality of Life; Randomized Controlled Trials as Topic; Recurrence; Reoperation; Severity of Illness Index; Sleep Apnea, Obstructive; Tonsillectomy
PubMed: 32347984
DOI: 10.1002/14651858.CD011365.pub2 -
The Cochrane Database of Systematic... Oct 2023Otitis media with effusion (OME) is an accumulation of fluid in the middle ear cavity, common amongst young children. The fluid may cause hearing loss. When persistent,... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Otitis media with effusion (OME) is an accumulation of fluid in the middle ear cavity, common amongst young children. The fluid may cause hearing loss. When persistent, it may lead to developmental delay, social difficulty and poor quality of life. Management of OME includes watchful waiting, autoinflation, medical and surgical treatment. Antibiotics are sometimes used to treat any bacteria present in the effusion, or associated biofilms.
OBJECTIVES
To assess the effects (benefits and harms) of oral antibiotics for otitis media with effusion (OME) in children.
SEARCH METHODS
The Cochrane ENT Information Specialist searched the Cochrane ENT Register, CENTRAL, Ovid MEDLINE, Ovid Embase, Web of Science, ClinicalTrials.gov, ICTRP and additional sources for published and unpublished studies to 20 January 2023.
SELECTION CRITERIA
We included randomised controlled trials and quasi-randomised trials in children aged 6 months to 12 years with unilateral or bilateral OME. We included studies that compared oral antibiotics with either placebo or no treatment.
DATA COLLECTION AND ANALYSIS
We used standard Cochrane methods. Our primary outcomes were determined following a multi-stakeholder prioritisation exercise and were: 1) hearing, 2) otitis media-specific quality of life and 3) anaphylaxis. Secondary outcomes were: 1) persistence of OME, 2) adverse effects, 3) receptive language skills, 4) speech development, 5) cognitive development, 6) psychosocial skills, 7) listening skills, 8) generic health-related quality of life, 9) parental stress, 10) vestibular function and 11) episodes of acute otitis media. We used GRADE to assess the certainty of evidence for each outcome. Although we included all measures of hearing assessment, the proportion of children who returned to normal hearing was our preferred method to assess hearing, due to challenges in interpreting the results of mean hearing thresholds.
MAIN RESULTS
We identified 19 completed studies that met our inclusion criteria (2581 participants). They assessed a variety of oral antibiotics (including penicillins, cephalosporins, macrolides and trimethoprim), with most studies using a 10- to 14-day treatment course. We had some concerns about the risk of bias in all studies included in this review. Here we report our primary outcomes and main secondary outcome, at the longest reported follow-up time. Antibiotics versus placebo We included 11 studies for this comparison, but none reported all of our outcomes of interest and limited meta-analysis was possible. Hearing One study found that more children may return to normal hearing by two months (resolution of the air-bone gap) after receiving antibiotics as compared with placebo, but the evidence is very uncertain (Peto odds ratio (OR) 9.59, 95% confidence interval (CI) 3.51 to 26.18; 20/49 children who received antibiotics returned to normal hearing versus 0/37 who received placebo; 1 study, 86 participants; very low-certainty evidence). Disease-specific quality of life No studies assessed this outcome. Presence/persistence of OME At 6 to 12 months of follow-up, the use of antibiotics compared with placebo may slightly reduce the number of children with persistent OME, but the confidence intervals were wide, and the evidence is very uncertain (risk ratio (RR) 0.89, 95% CI 0.68 to 1.17; 48% versus 54%; number needed to treat (NNT) 17; 2 studies, 324 participants; very low-certainty evidence). Adverse event: anaphylaxis No studies provided specific data on anaphylaxis. Three of the included studies (448 children) did report adverse events in sufficient detail to assume that no anaphylactic reactions occurred, but the evidence is very uncertain (very low-certainty evidence). Antibiotics versus no treatment We included eight studies for this comparison, but very limited meta-analysis was possible. Hearing One study found that the use of antibiotics compared to no treatment may result in little to no difference in final hearing threshold at three months (mean difference (MD) -5.38 dB HL, 95% CI -9.12 to -1.64; 1 study, 73 participants; low-certainty evidence). The only data identified on the return to normal hearing were reported at 10 days of follow-up, which we considered to be too short to accurately reflect the efficacy of antibiotics. Disease-specific quality of life No studies assessed this outcome. Presence/persistence of OME Antibiotics may reduce the proportion of children who have persistent OME at up to three months of follow-up, when compared with no treatment (RR 0.64, 95% CI 0.50 to 0.80; 6 studies, 542 participants; low-certainty evidence). Adverse event: anaphylaxis No studies provided specific data on anaphylaxis. Two of the included studies (180 children) did report adverse events in sufficient detail to assume that no anaphylactic reactions occurred, but the evidence is very uncertain (very low-certainty evidence).
AUTHORS' CONCLUSIONS
The evidence for the use of antibiotics for OME is of low to very low certainty. Although the use of antibiotics compared to no treatment may have a slight beneficial effect on the resolution of OME at up to three months, the overall impact on hearing is very uncertain. The long-term effects of antibiotics are unclear and few of the studies included in this review reported on potential harms. These important endpoints should be considered when weighing up the potential short- and long-term benefits and harms of antibiotic treatment in a condition with a high spontaneous resolution rate.
Topics: Child; Humans; Child, Preschool; Anti-Bacterial Agents; Otitis Media with Effusion; Quality of Life; Anaphylaxis; Hearing Loss
PubMed: 37870130
DOI: 10.1002/14651858.CD015254.pub2 -
Frontiers in Human Neuroscience 2023First, to discuss sex differences in auditory function between women and men, and whether cyclic fluctuations in levels of female sex hormones (i.e., estradiol and... (Review)
Review
AIMS
First, to discuss sex differences in auditory function between women and men, and whether cyclic fluctuations in levels of female sex hormones (i.e., estradiol and progesterone) affect auditory function in pre-menopausal and post-menopausal women. Second, to systematically review the literature concerning the discussed patterns in order to give an overview of the methodologies used in research. Last, to identify the gap in knowledge and to make recommendations for future work.
METHODS FOR THE SYSTEMATIC REVIEW
Population, Exposure, Control, Outcome and Study design (PECOS) criteria were used in developing the review questions. The review protocol follows the guidelines of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) and was pre-registered in the Prospective Register of Systematic Reviews (PROSPERO; CRD42020201480). Data Sources: EMBASE, PubMed, MEDLINE (Ovid), PsycINFO, ComDisDome, CINAHL, Web of Science, Cochrane Central Register of Controlled Trials (CENTRAL) via Cochrane Library, and scanning reference lists of relevant studies, and internet resources (i.e., Mendeley) were used. Only studies published between 1999 and 2022, in English, or in English translation, were included. The quality of evidence was assessed using the Newcastle-Ottawa Scale (NOS).
RESULTS
Sex differences: Women had more sensitive hearing (measured at the level of peripheral and central auditory system) than men. Cyclic fluctuations: Auditory function in women fluctuated during the menstrual cycle, while no such fluctuations in men over the same time period were reported. Hearing sensitivity improved in women during the late follicular phase, and decrease during the luteal phase, implying an effect of female sex hormones, although the specific effects of estradiol and progesterone fluctuations on the central auditory system remain unclear. Hearing sensitivity in women declined rapidly at the onset of menopause.
CONCLUSION
The review has shown the following. Consistent sex differences exist in auditory function across the auditory pathway with pre-menopausal women often showing better function than age-matched men. Moreover, pre-menopausal women show fluctuations in hearing function across the menstrual cycle with a better function during the peak of estradiol or when the ratio of estradiol to progesterone is high. Third, menopause marks the onset of hearing loss in women, characterized by a rapid decline in hearing sensitivity and a more pronounced loss than in age-matched men. Finally, the systematic review highlights the need for well-designed and -controlled studies to evaluate the influence of estradiol and progesterone on hearing by consistently including control groups (e.g., age-matched man), using objective tests to measure hormonal levels (e.g., in saliva or blood), and by testing participants at different points across the menstrual cycle.
SYSTEMATIC REVIEW REGISTRATION
https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42020201480, identifier CRD42020201480.
PubMed: 37151900
DOI: 10.3389/fnhum.2023.1077409 -
Otology & Neurotology : Official... Apr 2024To examine the otologic and neurotologic symptoms, physical examination findings, and imaging features secondary to hematologic malignancies. (Review)
Review
OBJECTIVE
To examine the otologic and neurotologic symptoms, physical examination findings, and imaging features secondary to hematologic malignancies.
METHODS
Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, databases, including PubMed, Scopus, and CINAHL, were searched for articles including patients with otologic manifestations of leukemia, lymphoma and multiple myeloma. Data collected included patient and study demographics, specific hematologic malignancy, timing and classification of otologic symptoms, physical examination findings, imaging features and methods of diagnosis. Pooled descriptive analysis was performed.
RESULTS
Two hundred seventy-two articles, of which 255 (93.8%) were case reports and 17 (6.2%) were case series, reporting on 553 patients were identified. Otologic manifestations were reported on 307 patients with leukemia, 204 patients with lymphoma and 42 patients with multiple myeloma. Hearing loss and unilateral facial palsy were the most common presenting symptoms for 111 reported subjects with leukemia (n = 46, 41.4%; n = 43, 38.7%) and 90 with lymphoma (n = 38, 42.2%; n = 39, 43.3%). Hearing loss and otalgia were the most common presenting symptoms for 21 subjects with multiple myeloma (n = 10, 47.6%; n = 6, 28.6%). Hearing loss and unilateral facial palsy were the most common otologic symptoms indicative of relapse in subjects with leukemia (n = 14, 43.8%) and lymphoma (n = 5, 50%).
CONCLUSION
Hearing loss, facial palsy, and otalgia might be the first indication of a new diagnosis or relapse of leukemia, lymphoma, or multiple myeloma. Clinicians should have a heightened level of suspicion of malignant etiologies of otologic symptoms in patients with current or medical histories of these malignancies.
Topics: Humans; Earache; Facial Paralysis; Multiple Myeloma; Hearing Loss; Hematologic Neoplasms; Deafness; Leukemia; Bell Palsy; Lymphoma; Recurrence
PubMed: 38437804
DOI: 10.1097/MAO.0000000000004141 -
Journal of Neuroradiology = Journal de... Mar 2024Cochlear implants have advanced the management of severe to profound deafness. However, there is a strong disparity in hearing performance after implantation from one... (Review)
Review
INTRODUCTION
Cochlear implants have advanced the management of severe to profound deafness. However, there is a strong disparity in hearing performance after implantation from one patient to another. Moreover, there are several advanced kinds of imaging assessment before cochlear implantation. Microstructural white fiber degeneration can be studied with Diffusion weighted MRI (DWI) or tractography of the central auditory pathways. Functional MRI (fMRI) allows us to evaluate brain function, and CT or MRI segmentation to better detect inner ear anomalies.
OBJECTIVE
This literature review aims to evaluate how helpful pre-implantation anatomic imaging can be to predict hearing rehabilitation outcomes in deaf patients. These techniques include DWI and fMRI of the central auditory pathways, and automated labyrinth segmentation by CT scan, cone beam CT and MRI.
DESIGN
This systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Studies were selected by searching in PubMed and by checking the reference lists of relevant articles. Inclusion criteria were adults over 18, with unilateral or bilateral hearing loss, who had DWI acquisition or fMRI or CT/ Cone Beam CT/ MRI image segmentation.
RESULTS
After reviewing 172 articles, we finally included 51. Studies on DWI showed changes in the central auditory pathways affecting the white matter, extending to the primary and non-primary auditory cortices, even in sudden and mild hearing impairment. Hearing loss patients show a reorganization of brain activity in various areas, such as the auditory and visual cortices, as well as regions involved in language and emotions, according to fMRI studies. Deep Learning's automatic segmentation produces the best CT segmentation in just a few seconds. MRI segmentation is mainly used to evaluate fluid space of the inner ear and determine the presence of an endolymphatic hydrops.
CONCLUSION
Before cochlear implantation, a DWI with tractography can evaluate the central auditory pathways up to the primary and non-primary auditory cortices. This data is then used to generate predictions on the auditory rehabilitation of patients. A CT segmentation with systematic 3D reconstruction allow a better evaluation of cochlear malformations and predictable difficulties during surgery.
Topics: Adult; Humans; Cochlear Implantation; Cochlear Implants; Deafness; Magnetic Resonance Imaging; Treatment Outcome
PubMed: 37806523
DOI: 10.1016/j.neurad.2023.10.002 -
International Journal of Pediatric... Apr 2021Sotos syndrome 1 (SOTOS1; MIM:117550) is rare genetic disorder characterized by excessive physical growth before and after birth, distinctive facial features, a large... (Review)
Review
OBJECTIVES
Sotos syndrome 1 (SOTOS1; MIM:117550) is rare genetic disorder characterized by excessive physical growth before and after birth, distinctive facial features, a large and elongated head, and intellectual disability (Sotos et al., 1964; Tatton-Brown et al., 1993). This systematic review aims to determine otolaryngologic conditions and complications of SOTOS1 based on existing literature through a review of current and past case reports and studies regarding SOTOS1.
METHODS
A systematic review of all published literature (1964-2020) describing otolaryngologic conditions and/or complications of patients with SOTOS1. Twenty journal articles met inclusion criteria. These articles included 160 patients diagnosed with SOTOS1.
RESULTS
Of the 160 individuals with SOTOS1 included in this review, 22 (14%) were reported to have otologic conditions. 4 (3%) individuals were reported to have conditions involving the thyroid and parathyroid glands. 2 (1%) individuals were reported to have head & neck tumors. 39 (24%) individuals were reported to have congenital malformations or abnormalities of the head & neck. 47 (29%) individuals were reported to have feeding difficulties. 16% of individuals were reported to have other otolaryngologic conditions.
CONCLUSIONS
Our review found multiple otolaryngologic conditions present in patients with SOTOS1, including hearing loss, otitis, hyperthyroidism, hypothyroidism, head & neck tumors, congenital malformations (high arched palate, cleft lip and palate, macroglossia), feeding difficulties, respiratory difficulties, and speech disorders. Additional studies should be conducted to further assess these associations.
Topics: Cleft Lip; Cleft Palate; Deafness; Humans; Intellectual Disability; Sotos Syndrome
PubMed: 33640723
DOI: 10.1016/j.ijporl.2021.110649