Cognition in Children With Sensorineural Hearing Loss: Etiologic Considerations

Pierson, Suzanne K.; Caudle, Susan E.; Krull, Kevin R.; Haymond, Jody; Tonini, Ross; Oghalai, John S.

doi:10.1097/mlg.0b013e3180ca7834

Cited by 24 publications

(22 citation statements)

References 14 publications

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“…One exception may be in the case of children with suspected syndromic hearing loss—in which the independent diagnostic yield is 42%. In any case, congenital hearing loss of all types has been associated with decreased visual reception skills with age (20). Therefore, at the very least, the child's visual acuity should be evaluated and optimized to minimize his or her sensory disabilities and maximize the potential for normal development.…”

Section: Discussionmentioning

confidence: 99%

Comprehensive Diagnostic Battery for Evaluating Sensorineural Hearing Loss in Children

Lin¹,

Chowdhury²,

Mody³

et al. 2011

Otology &Amp; Neurotology

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Objective Selection of diagnostic tests for children with sensorineural hearing loss (SNHL) is influenced by clinical suspicion. Testing results reported in the literature are similarly biased. We evaluate the usefulness of a comprehensive diagnostic battery for each child. Study Design Retrospective review. Setting Tertiary care university hospital. Patients A total of 270 children referred for severe to profound SNHL between January 2002 and June 2009. Interventions Results of the following were reviewed: magnetic resonance imaging, computed tomography, renal ultrasound, electrocardiography, fluorescent treponemal antibody absorption test, connexin 26 sequencing, genetic consultation, and ophthalmologic consultation. Main Outcome Measure Diagnostic yield of each test was determined. Results Each diagnostic test or consultation was completed by at least 95% of patients for whom it was ordered. Magnetic resonance imaging revealed abnormalities explaining SNHL in 24% of patients. Computed tomography showed inner ear anomalies in 18% of patients. Biallelic connexin 26 mutations were found in 15%. Renal ultrasound found anomalies in 4% of patients. Electrocardiography found 1% of patients with prolonged QT intervals. Fluorescent treponemal antibody absorption test result was positive in 0.5%. Genetic consultation found a genetic cause for hearing loss in 25%. Ophthalmologic consultation found abnormalities associated with hearing loss in 8%. Conclusion Diagnostic radiologic imaging is the highest yielding test for evaluating children with SNHL. Connexin 26 sequencing identifies a nearly nonoverlapping subset of children compared with imaging. Specialty consultations, particularly from a clinical geneticist, can improve diagnostic yield. Other tests, although of lower diagnostic yield for SNHL, can identify important diseases that significantly affect patient health.

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Section: Discussionmentioning

confidence: 99%

Comprehensive Diagnostic Battery for Evaluating Sensorineural Hearing Loss in Children

Lin¹,

Chowdhury²,

Mody³

et al. 2011

Otology &Amp; Neurotology

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“…Even though the CI can be placed in very young children (Lin et al, 2010; Oghalai et al, 2009), it can be difficult to perform the necessary behavioral testing to program the CI, especially if there are co-existent developmental delays which are often found in this patient population (Beauchamp et al; Cristobal et al, 2008; Katzenstein et al, 2009; Kushalnagar et al, 2007; Pierson et al, 2007). Therefore, an objective method to measure brain activity would be give valuable additional information.…”

Section: Discussionmentioning

confidence: 99%

Neuroimaging with near-infrared spectroscopy demonstrates speech-evoked activity in the auditory cortex of deaf children following cochlear implantation

et al. 2010

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Cochlear implants (CI) are commonly used to treat deafness in young children. While many factors influence the ability of a deaf child who is hearing through a CI to develop speech and language skills, an important factor is that the CI has to stimulate the auditory cortex. Obtaining behavioral measurements from young children with CIs can often be unreliable. While a variety of noninvasive techniques can be used for detecting cortical activity in response to auditory stimuli, many have critical limitations when applied to the pediatric CI population. We tested the ability of near-infrared spectroscopy (NIRS) to detect cortical responses to speech stimuli in pediatric CI users. Neuronal activity leads to changes in blood oxy-and de-oxyhemoglobin concentrations that can be detected by measuring the transmission of near-infrared light through the tissue. To verify the efficacy of NIRS, we first compared auditory cortex responses measured with NIRS and fMRI in normal-hearing adults. We then examined four different participant cohorts with NIRS alone. Speech-evoked cortical activity was observed in 100% of normal-hearing adults (11 of 11), 82% of normal-hearing children (9 of 11), 78% of deaf children who have used a CI >4 months (28 of 36), and 78% of deaf children who completed NIRS testing on the day of CI initial activation (7 of 9). Therefore, NIRS can measure cortical responses in pediatric CI users, and has the potential to be a powerful adjunct to current CI assessment tools.

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“…Thus, we would expect his expressive language gains to be evident in future evaluations. His gains suggest that although he was diagnosed with Mild to Moderate Mental Retardation, his cochlear implant greatly increased his quality of life and improved his functional communication (Kushalnager et al, 2007; Pierson et al, 2007)…”

Section: Discussionmentioning

confidence: 99%

Neurocognitive functioning of a child with partial trisomy 6 and monosomy 21

Katzenstein¹,

Oghalai²,

Tonini³

et al. 2009

Neurocase

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This case study describes the neurocognitive presentation of a child with identified genetic abnormalities of trisomy 6 and monosomy 21 who was evaluated as part of a standard medical protocol for cochlear implantation following diagnosis of profound sensorineural hearing loss. This child received neurocognitive testing prior to cochlear implantation and approximately 12 months post-activation of his cochlear implant. While he has not fully developed oral language, his presentation suggested improvement in overall skills since the activation of the cochlear implant; however, less than would be expected for a typically developing child.

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Cognition in Children With Sensorineural Hearing Loss: Etiologic Considerations

Cited by 24 publications

References 14 publications

Comprehensive Diagnostic Battery for Evaluating Sensorineural Hearing Loss in Children

Comprehensive Diagnostic Battery for Evaluating Sensorineural Hearing Loss in Children

Neuroimaging with near-infrared spectroscopy demonstrates speech-evoked activity in the auditory cortex of deaf children following cochlear implantation

Neurocognitive functioning of a child with partial trisomy 6 and monosomy 21

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