Hearing Loss in Pediatric Septo-Optic Dysplasia

2019 ◽  
Vol 128 (6) ◽  
pp. 485-489
Author(s):  
Brian William Herrmann ◽  
Campbell R. Hathaway ◽  
Michael Fadell
Keyword(s):  
Hearing Loss ◽  
Early Identification ◽  
Relevant Literature ◽  
Population Based ◽  
Cochlear Nerve ◽  
Tympanostomy Tubes ◽  
Predominant Form ◽  
Children With Hearing Loss ◽  
Cochlear Nerve Deficiency ◽  
Hospital Clinic

Objective: To identify and characterize hearing loss (HL) in children with septo-optic dysplasia (SOD). Methods: Otologic and audiometric data for patients less than 18 years of age identified as having SOD who were seen in the Children’s Healthcare of Atlanta-Scottish Rite Hospital clinic between 2013 and 2017 were collected and reviewed through a HIPAA-compliant medical record search. Relevant literature was also reviewed with the assistance of Medline. Results: Sixty-four patients with SOD were identified, and 7 of those patients (10.9%) were diagnosed with hearing loss. Type of hearing loss was sensorineural (SNHL) in 5 patients (63%), mixed (MHL) in 1(14%), and conductive (CHL) in 1(14%). Bilateral loss presented in 60% (3/5) of SNHL patients, while the rest demonstrated unilateral loss. Unilateral findings included cochlear nerve deficiency (1) and atresia/microtia (1). Tympanostomy tubes were required in 57% (4/7) of SOD children with hearing loss. Amplification was successfully implemented in 86% (6/7). Conclusions: Hearing loss was found in nearly 11% of SOD children, and SNHL was identified as (63%) the predominant form of loss. To our knowledge, this is the first retrospective review of hearing loss in a pediatric SOD cohort and the first to report of cochlear nerve deficiency and atresia/microtia in this population. Based on these findings, early identification of hearing loss with imaging when appropriate and treatment of otitis in this population is recommended.

Hearing loss due to mannosidosis and otitis media with effusion. A case report and review of audiological assessments in children with otitis media with effusion

2003 ◽  
Vol 117 (4) ◽  
pp. 307-309 ◽  
Author(s):  
A. U. Ahmmed ◽  
S. M. O’Halloran ◽  
N. J. Roland ◽  
M. Starkey ◽  
J. E. Wraith
Keyword(s):  
Hearing Loss ◽  
Otitis Media ◽  
Otitis Media With Effusion ◽  
Relevant Literature ◽  
Before And After ◽  
Newborn Hearing ◽  
Age Appropriate ◽  
Glue Ear ◽  
Hearing Assessment ◽  
Children With Hearing Loss

A case of a child with mannosidosis and bilateral otitis media with effusion (OME) is reported here along with some discussion of relevant literature to emphasize the need for age appropriate audiometricc assessment before and after insertion of grommets for glue ear (OME).There is a need for multidisciplinary teamwork in the management of children with hearing loss. If OME is treated surgically, age-appropriate hearing assessment is required before and after insertion of grommets. The need for audiological assessments will be relevant even if children had passed the newborn hearing screening test.

A Case of Cochlear Nerve Deficiency Without Profound Sensorineural Hearing Loss

2011 ◽  
Vol 32 (4) ◽  
pp. 529-532 ◽  
Author(s):  
Ikuyo Miyanohara ◽  
Keiichi Miyashita ◽  
Koji Takumi ◽  
Masayuki Nakajo ◽  
Yuichi Kurono
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Nerve ◽  
Sensorineural Hearing ◽  
Cochlear Nerve Deficiency

Cochlear Implant Outcomes for Children With Auditory Neuropathy Spectrum Disorder

2009 ◽  
Vol 19 (2) ◽  
pp. 75-84 ◽  
Author(s):  
Jaime Leigh ◽  
Gary Rance ◽  
Shani Dettman ◽  
Richard Dowell
Keyword(s):  
Hearing Loss ◽  
Speech Perception ◽  
Cochlear Implant ◽  
Cochlear Implants ◽  
Auditory Neuropathy ◽  
Cochlear Nerve ◽  
Spectrum Disorder ◽  
Language Outcomes ◽  
Outcomes For Children ◽  
Cochlear Nerve Deficiency

Abstract Cochlear implantation is currently the intervention option of choice for many children with auditory neuropathy spectrum disorder (ANSD) who are unable to obtain benefit from conventional amplification. The aim of this study was to review the speech perception and language outcomes for children with ANSD who had received a cochlear implant and highlight specific clinical considerations for working with this population of children with hearing impairment who are being considered for implantation. Finds for the group of 17 children with ANSD using cochlear implants were compared to previously reported outcomes for children with sensori-neural (SN) type hearing loss using cochlear implants. Two children, identified with cochlear nerve deficiency pre-operatively, received no useful auditory percepts from their cochlear implant and discontinued device use. The remaining children demonstrated speech perception and language outcomes comparable to those observed for SN hearing loss peers using cochlear implants. This paper highlights a number of considerations for clinicians to be aware of and the importance of careful counseling pre-operatively regarding the potential for less-than-optimal outcomes, particularly for those children identified with cochlear nerve deficiency.

scholarly journals The Influence of Parents Knowledge and Health Care Access to the Identification of Children with Hearing Impairment

2019 ◽  
Vol 15 (2) ◽  
pp. 121
Author(s):  
Asti Widuri ◽  
Muhammad Pringgo Arifianto
Keyword(s):  
Health Care ◽  
Hearing Loss ◽  
Early Detection ◽  
Hearing Impairment ◽  
Health Care Access ◽  
Early Identification ◽  
Parental Knowledge ◽  
Care Access ◽  
Children With Hearing Loss ◽  
Speech And Language Development

Abstract: Early identification and access to quality habilitative services designed for children with hearing loss is the key to developing language and communication skills commensurate with the children’s cognitive abilities. Early identification will provide the opportunity for improved outcomes for thousands of children with hearing loss. Objective: To review the role of parents knowledge and health care Access to the identification of children with hearing impairment. The study design was cross-sectional, with the sample were 45 parents of children at SLB – B Karnnamanohara Yogyakarta at the nursery-child and play group. Interviewer administered a questionnaire to the parents, for evaluations of their knowledge about deaf children and health care access. After obtained the data from the each variable, then test with chi-square statistic. The significant result shown at parental knowledge (p=0,028) and health care access (p= 0,02). This means that there were significant differences between parental knowledge about speech and language development and health care access to early detection of deafness in children. Parents knowledge and health care access influenced to the identification of children with hearing impairment. Keywords: Parental knowledge, speech and language development, early detection, children with hearing loss, health care access

Occipital meningocele and Mondini deformity of the cochlea

2001 ◽  
Vol 115 (1) ◽  
pp. 71-73 ◽  
Author(s):  
O. D. Smith ◽  
A. M. Neumann ◽  
K. S. Sirimanna
Keyword(s):  
Hearing Loss ◽  
Early Identification ◽  
Intimate Relationship ◽  
Children With Hearing Loss

We present two cases of a previously unreported association between occipital meningocele and Mondini deformity of the cochlea. The probability of the association is supported by the intimate relationship between the development of these structures; the existence of dysembryoplastic factors that can cause both abnormalities; and the universal existence of cochlear abnormalities in anencephalic foetuses. The importance of considering the association is emphasized, in terms of early identification and referral of children with hearing loss, and avoidance of duplication of investigations.

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