Genetic and Non Genetic Hearing Loss and Associated Disabilities: An Epidemiological Survey in Emilia-Romagna Region

Hearing loss is one of the most common congenital sensory disorders. It can be associated with several comorbidities, in particular developmental disabilities (DD). In Emilia-Romagna (ER), a region in Northern Italy, Child and Adolescent Mental Health Services (CAMHS) provide the diagnostic framework and treatment for these conditions. The aim of the present study is to evaluate the prevalence of hearing loss, both isolated or in association with comorbidities, in the juvenile population. The study draws its data from the ER Childhood and Adolescent Neuropsychiatry Information System (SINPIAER), an Administrative Healthcare Database collecting the clinical data of all those who have attended CAMHS since 2010. The most frequent type of hearing loss was bilateral sensorineural hearing loss, which was present in 69–72% of the cases, while bilateral conductive hearing loss was the second most common type, ranging from 8 to 10%. Among DD, congenital malformations, mental retardation, visual impairment, and cerebral palsy were the most common. In particular, autism spectrum disorders show increasing incidence and prevalence among CAMHS users in ER region. In-depth knowledge of hearing loss epidemiology and related conditions, such as developmental disabilities, in the juvenile population is crucial for disease prevention, health planning, and resource allocation.

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Examining the Role of Language in Play Among Children With and Without Developmental Disabilities

Language Speech and Hearing Services in Schools ◽

10.1044/2020_lshss-19-00084 ◽

2020 ◽

Vol 51 (3) ◽

pp. 795-806 ◽

Cited By ~ 1

Author(s):

Elizabeth J. Short ◽

Rachael Cooper Schindler ◽

Rita Obeid ◽

Maia M. Noeder ◽

Laura E. Hlavaty ◽

...

Keyword(s):

Developmental Disabilities ◽

Autism Spectrum ◽

Free Play ◽

Group Differences ◽

Typically Developing ◽

Play Skills ◽

Critical Aspect ◽

Hyperactivity Disorder ◽

Better Than

Purpose Play is a critical aspect of children's development, and researchers have long argued that symbolic deficits in play may be diagnostic of developmental disabilities. This study examined whether deficits in play emerge as a function of developmental disabilities and whether our perceptions of play are colored by differences in language and behavioral presentations. Method Ninety-three children participated in this study (typically developing [TD]; n = 23, developmental language disorders [DLD]; n = 24, attention-deficit/hyperactivity disorder [ADHD]; n = 26, and autism spectrum disorder [ASD]; n = 20). Children were videotaped engaging in free-play. Children's symbolic play (imagination, organization, elaboration, and comfort) was scored under conditions of both audible language and no audible language to assess diagnostic group differences in play and whether audible language impacted raters' perception of play. Results Significant differences in play were evident across diagnostic groups. The presence of language did not alter play ratings for the TD group, but differences were found among the other diagnostic groups. When language was audible, children with DLD and ASD (but not ADHD) were scored poorly on play compared to their TD peers. When language was not audible, children with DLD were perceived to play better than when language was audible. Conversely, children with ADHD showed organizational deficits when language was not available to support their play. Finally, children with ASD demonstrated poor play performance regardless of whether language was audible or not. Conclusions Language affects our understanding of play skills in some young children. Parents, researchers, and clinicians must be careful not to underestimate or overestimate play based on language presentation. Differential skills in language have the potential to unduly influence our perceptions of play for children with developmental disabilities.

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Biological age and rates of aging of patients with vibration disease and bilateral sensorineural hearing loss

Russian Journal of Occupational Health and Industrial Ecology ◽

10.31089/1026-9428-2019-59-9-624-625 ◽

2020 ◽

pp. 624-624

Author(s):

S. I. Ereniev ◽

O. V. Plotnikova

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Sensorineural Hearing ◽

Biological Age ◽

Biological Aging ◽

Vibration Disease ◽

Bilateral Sensorineural Hearing Loss

Biological age and rates of aging of patients with vibration disease and bilateral sensorineural hearing loss were studied. The biological age of patients exceeded the calendar age by an average of 7.36±0.36 years and the proper biological age by 10.79±0.72 years. The rate of biological aging of the examined patients was 1.14±0.08 times higher than the rate of aging of their healthy peers.

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The Use of Psychotropic Medication in Iranian Children with Developmental Disabilities

International Journal of Environmental Research and Public Health ◽

10.3390/ijerph18084120 ◽

2021 ◽

Vol 18 (8) ◽

pp. 4120

Author(s):

Roy McConkey ◽

Sayyed Ali Samadi ◽

Ameneh Mahmoodizadeh ◽

Laurence Taggart

Keyword(s):

Developmental Disabilities ◽

Psychotropic Medication ◽

Management Strategies ◽

Educational Interventions ◽

Autism Spectrum ◽

Developmental Needs ◽

Middle Income ◽

Middle Income Countries ◽

Iranian Children ◽

Children With Developmental Disabilities

The use of psychotropic medication in children is increasing worldwide. Children with developmental disabilities seem to be prescribed these medications at a higher rate compared to their non-disabled peers. Little is known about prescribing in non-Western, middle-income studies. In Iran, the file records of 1133 children, aged 2 to 17 years, assessed as having autism spectrum disorder (ASD) or an intellectual disability (ID) in Tehran City and Province from 2005 to 2019 were collated, and information from parental reports of medications was extracted. Upwards of 80% of children with ASD and 56% of those with ID were prescribed a psychotropic medication with around one quarter in each group taking two or more medications. The rates were higher among male children showing difficult-to-manage behaviors such as hyperactivity, but less so for children of fathers with higher levels of education. The lack of alternative management strategies may be a significant driver for the use of psychotropic medications in Iran and other Low and Middle Income countries, despite their known side effects, and their failure to address the developmental needs of the children. Rather, multi-disciplinary, behavioral, therapeutic, and educational interventions are required, but these are not available widely in Iran, although a start has been made.

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Sudden bilateral sensorineural hearing loss associated with urticarial vasculitis

The Journal of Laryngology & Otology ◽

10.1017/s0022215113001047 ◽

2013 ◽

Vol 127 (7) ◽

pp. 708-711 ◽

Cited By ~ 3

Author(s):

A C Hall ◽

A C Leong ◽

D Jiang ◽

A Fitzgerald-O'Connor

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Cochlear Implantation ◽

Skin Lesions ◽

Sudden Hearing Loss ◽

Sudden Onset ◽

Sensorineural Hearing ◽

Urticarial Vasculitis ◽

Wells Syndrome ◽

Bilateral Sensorineural Hearing Loss

AbstractBackground:Bilateral sensorineural hearing loss associated with recurrent urticarial skin lesions may be signs of underlying Muckle–Wells syndrome. Previous reports have described the hearing loss to be progressive in nature.Method:To our knowledge, this paper presents the first published case of sudden onset, bilateral sensorineural hearing loss associated with urticarial vasculitis due to underlying Muckle–Wells syndrome.Results:The patient underwent a cochlear implantation with a modest outcome.Conclusion:Cochlear implantation may help to rehabilitate sudden hearing loss associated with this condition, but early diagnosis may allow treatment with interleukin-1β inhibitors such as anakinra.

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Comprehensive medical evaluation of pediatric bilateral sensorineural hearing loss

Laryngoscope Investigative Otolaryngology ◽

10.1002/lio2.657 ◽

2021 ◽

Author(s):

Suat Kılıç ◽

Malek H. Bouzaher ◽

Michael S. Cohen ◽

Judith E. C. Lieu ◽

Margaret Kenna ◽

...

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Sensorineural Hearing ◽

Medical Evaluation ◽

Bilateral Sensorineural Hearing Loss

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Is simultaneous bilateral mastoidectomy ever advisable?

The Journal of Laryngology & Otology ◽

10.1017/s0022215107006330 ◽

2007 ◽

Vol 121 (11) ◽

pp. 1041-1047 ◽

Cited By ~ 5

Author(s):

J J Klemens ◽

E Mhoon ◽

M Redleaf

Keyword(s):

Hearing Loss ◽

Decision Tree ◽

Sensorineural Hearing Loss ◽

Chart Review ◽

Historical Data ◽

Sensorineural Hearing ◽

Opinion Survey ◽

Potential Patient ◽

Simultaneous Procedures ◽

Bilateral Sensorineural Hearing Loss

AbstractIntroduction:We report our experience with bilateral, simultaneous tympanomastoidectomies and the results of an opinion survey of otologists.Methods:A chart review of 116 tympanomastoidectomies revealed 12 patients who underwent bilateral, simultaneous tympanomastoidectomies. An opinion survey generated 121 responses.Results:Of the 12 patients, none suffered any outcome which would have been avoided by staging the procedures. Twenty-three of 24 operated ears had the same or better hearing post-operatively. Of the survey respondents, 74 felt that performing bilateral, simultaneous tympanomastoidectomies was unsafe, largely because of the risk of bilateral sensorineural hearing loss.Discussion:Although bilateral, simultaneous tympanomastoidectomies carry double the risk of unilateral sensorineural hearing loss, compared with the unilateral procedure, the risk of bilateral sensorineural hearing loss is only 0.006–0.2 per cent, as derived mathematically from historical data. Respondents to the survey were mostly opposed to bilateral, simultaneous tympanomastoidectomies, but even those opposed gave indications for simultaneous procedures. This finding probably reflects an ambivalence about the theoretical risks of the operation versus the potential patient benefits. A decision tree for proceeding to the second case is presented.

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Cochlear nerve anomalies in paediatric single-sided deafness – prevalence and implications for cochlear implantation strategies

The Journal of Laryngology & Otology ◽

10.1017/s002221512000225x ◽

2020 ◽

pp. 1-4

Author(s):

K Pollaers ◽

A Thompson ◽

J Kuthubutheen

Keyword(s):

Magnetic Resonance Imaging ◽

Hearing Loss ◽

Magnetic Resonance ◽

Sensorineural Hearing Loss ◽

Cochlear Nerve ◽

Sensorineural Hearing ◽

Resonance Imaging ◽

Bilateral Sensorineural Hearing Loss ◽

Single Sided Deafness ◽

Cochlear Nerve Hypoplasia

Abstract Objective To determine the prevalence of cochlear nerve anomalies on magnetic resonance imaging in patients with unilateral or bilateral sensorineural hearing loss. Methods A retrospective case series was conducted at a tertiary referral centre. The inclusion criteria were paediatric patients with bilateral or unilateral sensorineural hearing loss, investigated with magnetic resonance imaging. The primary outcome measure was the rate of cochlear nerve hypoplasia or aplasia. Results Of the 72 patients with unilateral sensorineural hearing loss, 39 per cent (28 cases) had absent or hypoplastic cochlear nerves on the affected side. Fifteen per cent (11 cases) had other abnormal findings on magnetic resonance imaging. Eighty-four patients had bilateral sensorineural hearing loss, of which cochlear nerve hypoplasia or aplasia was identified only in 5 per cent (four cases). Other abnormal findings were identified in 14 per cent (12 cases). Conclusion Paediatric patients with unilateral sensorineural hearing loss are more likely to have cochlear nerve anomalies than those patients with bilateral sensorineural hearing loss. This has important implications regarding cochlear implantation for patients with single-sided deafness.

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Visual loss with chronic meningeal and systemic inflammation

Practical Neurology ◽

10.1136/practneurol-2021-003066 ◽

2021 ◽

pp. practneurol-2021-003066

Author(s):

Peter M Fernandes ◽

Lucy Kinton ◽

Stephen Madill ◽

Frederick R Burgess ◽

Euan McRorie ◽

...

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Annual Meeting ◽

Optic Disc ◽

Patient Outcomes ◽

Inflammatory Markers ◽

Visual Loss ◽

Targeted Treatments ◽

Infection And Inflammation ◽

Bilateral Sensorineural Hearing Loss

A 46-year-old man presented with worsening vision in the context of long-standing optic disc changes, bilateral sensorineural hearing loss, chronic aseptic meningitis, rash, arthralgia and raised inflammatory markers. The differential diagnoses of neoplasia, infection and inflammation are discussed. We highlight a condition that can present to adult neurologists, with successful diagnosis leading to targeted treatments that transform patient outcomes. This case was presented at the Association of British Neurologists Annual Meeting 2020 as a Clinicopathological Conference (CPC).

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